**excludes two patients who remained in the neurology unit until they died, and 3 who died at home
Table 4 presents the numbers of families recalling input from a variety of different therapists and services, and receiving a variety of services and aids. No judgement is made about whether or not these inputs benefited the patient. Some patients were in hospital most of the time so the families did not require any aids and may not have known all the therapies the patients had.
INITIAL MANAGEMENT AND INVESTIGATION
Patients initially presented with non-specific symptoms, including personality change, apathy, anxiety, unexplained limb pains or cold limbs. This progressed over several months. As the condition progressed, they experienced short term memory loss, were unable to work and needed constant supervision. Some were aggressive or developed psychotic symptoms. The extent of the functional disability and memory loss was often not appreciated by professionals initially, as in the following example,
Family 'So we all went to this appointment with the GP and ... she wanted him to go away and get a foolscap piece of paper, sheet of paper, and write all his feelings down and bring it back the following week which we knew and I'm sure she must have known that that would be impossible for him because as soon as we left the surgery or as soon as he got to the door of the room he had forgotten, couldn't remember anything. And they knew he couldn't remember things. And we were very annoyed about that.'
Some patients tried to hide the extent of their problems from their doctors, and nearly all were eventually accompanied to the GP by family members who felt their concerns were not being addressed.
Most of the patients were given a psychiatric diagnosis initially, and 10 were treated in a psychiatric unit as an in-patient or day patient. Some were referred to the psychiatric services by the neurologist, or at the same time as a neurology referral, to exclude a psychiatric cause.
Many families were angry that their relative had been given a psychiatric label. Some reported that patients denied feeling depressed but were treated for depression. They felt they had been disbelieved; that their symptoms had not been taken seriously. Patients themselves were reluctant to see a psychiatrist or be treated in a psychiatric unit. Psychiatric hospitals had a stigma, and psychiatric illnesses were not perceived to be 'real' illnesses.
Family 'I'm offended that he wasn't believed. That they were trying to ... some of his symptoms seemed to fit some form of psychological disturbance, if I can put it that way and some of his symptoms didn't fit it but because they couldn't find something that fitted all of the symptoms they went with half of it and said right that's what we're going to treat and we won't believe him when he says this because that could just be part of the problem. They never actually looked at all the symptoms because I think they just didn't know what it was pointing to.'
Family 'I knew as soon as I told her that they wanted her to go to the [psychiatric] hospital then she would go mad because she doesn't agree with that. They're not mental institutions, really it's not a mental institution, it isn't, but it has this stigma ... once you go through the door you don't come out and it's wrong but people have that way you know. But there are a lot of people there that are mentally ill and that's a damn shame.'
Patients were often fearful of other disturbed psychiatric patients, or worried that being in an 'asylum' meant they would never get out of hospital. Families were also worried about leaving patients in a psychiatric unit with disturbed patients.
Family 'Because she knew that they were mental cases and she was not mental, if you know what I mean. She was scared stiff ... she was so upset at the ward, being in that ward, the head nurse, everybody could see. She didn't want to go out of her room. She had a room to herself and she kept asking me to look across and see that so and so wasn't near the door and all this carry on. It was terrible.'
Family 'Obviously we were worried about her being in a psychiatric unit with people that are really anxious and have break downs and all sorts of, you know, as well because she was like a little girl again, you know.'
Families thought that the psychiatric diagnosis had delayed neurological investigation and help. Some patients lost contact with neurology services during a period of inpatient psychiatric treatment. Psychiatric assessment and neurological investigation seemed to be mutually exclusive paths. Other families thought the psychiatric label closed the minds of professionals to other possibilities.
Family 'And I feel because they went down the route that they did for so long, expecting and then causing us to expect that it was a psychological problem that it shut down the avenues to be exploring other possibilities of what it could have been, I wonder if they would have got there sooner if they had kept looking at the whole thing.'
Family 'Yeah I mean we constantly told them of her physical symptoms. But you know they wanted to go down the path of it being psychological to begin with, and although they had started the neurological tests, but that kind of took it's time, once they had done that there seemed to be long gaps in between going to neurology, which I knew would be ... the cause of the problems, something neurological.'
In some cases, there was disagreement between the psychiatrist, who thought the patient had an organic condition, and the neurologist, who thought the symptoms were caused by anxiety or atypical depression. Some patients were sent more than once from the psychiatric unit for neurological assessment before their care was taken on by the neurology service.
Family 'There would be the hospital saying it was psychiatric help she needs and the psychiatric place was saying it's an actual illness she has got; it was very sort of like between the two.'
Psychiatrist 'There was a feeling of being abandoned, it wasn't a functional illness but no one else would accept that ... As a psychiatrist you often find you are the final common pathway for conditions no one else wants or can handle. '
Some families' anger at the initial psychiatric diagnosis led to later mistrust of professionals caring for the patient. One respondent had tried to bypass the GP in seeking help. It is notable that psychiatrists were rarely involved in later management despite continuing psychotic symptoms.
But despite these concerns, psychiatrists felt they did have a role to play. In particular, they offered expertise in mental state assessment and often recognised the extent of the cognitive loss when it had not been previously appreciated. Many patients also needed psychiatric nursing care to manage psychotic symptoms.
Psychiatrist 'From the clinical picture she presented the psychiatrist has a role because she presented with no marked physical symptoms and a clinical picture suggesting depression or pseudo-dementia. So a role in diagnosis and clinical management.'
Some patients sought informal referrals and used alternative therapies like counselling and homeopathy in the early stages of the condition. Two saw a psychiatrist privately. Seven patients were referred to a neurologist initially, 8 to a psychiatrist and 4 to a physician.
A few patients waited for an outpatient appointment with a neurologist. One family sought a private appointment with the neurologist to expedite a wait of several months and the longest wait was 6 weeks. But many patients were referred or admitted urgently, and some families commented on their surprise and alarm at the rapidity with which investigations were arranged once professionals realised the extent of patients' neurological deterioration.
Family 'And we was only in ten minutes when he said, "Right, I'm taking her in today". So I said, "What do you mean, you are taking her in?" Because to me she wasn't any worse than the weeks before you know. He said "She's in a very very bad way. I don't know how you have managed with her". I said, "phoo". I just laughed at him and said, "I know, but that's it isn't it". I didn't think we would ever sort of get any help and that day he took her for a brain scan. He said, "Right, I'm admitting her".'
Family 'And he came back and said "Right she's got to go into [neurology unit] tomorrow". And I thought, this is too fast it's just fast tracking all of a sudden. One minute it's depression now all of a sudden it's just, within three days she's been to the doctors and then into hospital.'
Family 'And he came in and he examined [patient] and he said to me "I'm going to have to go through for further tests" and I knew we were in bloody trouble because we're going to [neurology unit]. Why can't they do anything here? You know it must be quite serious that we're heading through there. So he took us in an ambulance the next morning.'
Many patients were seen by a neurologist early on for assessment but not followed up by the neurology service. In 4 cases the first neurologist seen found no neurological signs and attributed the symptoms to a psychiatric condition. Most of these were under the care of a psychiatrist at this stage. Some families were angry that the condition was missed at this stage.
Family 'So why wasn't the doctor picking up at that stage on those things that we had already described. [Neurologist] wasn't listening. So I was quite angry and I continued to be quite angry about that, you know, because those were the first signs.'
Another patient was lost to follow-up because the neurologist who initially saw her retired. In these situations, families felt abandoned and frustrated.
Family 'You feel nothing has been done, no diagnosis, no medication, no light at the end of the tunnel. You do become frustrated, you, I mean I say fell out with doctors and I was quite curt with other people because you know I always felt that they weren't doing enough.'
When the neurologist took over the care of the patient, families felt relieved that someone was taking their problems seriously and trying to address them. Two families expressed gratitude to the psychiatrists who took on the patients' management while seeking neurological follow-up. One respondent movingly described his hope that neurological investigation would find the solution to the problems,
Family 'So he then scheduled [patient] to come in for a week or two for further tests in April which I thought was the light at the end of the tunnel. I tried to keep, I was keeping everything together ... we thought that she would go in there they would find a solution a treatment would be given and she would begin to recover ... I pinned everything on that.'
All patients underwent in-patient investigation in a neurology unit at some point. Total duration of stay in acute neurology units varied between 5 days and almost four months. By this time, patients were cognitively impaired and often found the investigations frightening. In particular, many patients underwent one or more failed attempt at MRI scanning before being given sedation. Families felt their understanding of patients' fears and reactions could have helped prevent some of these problems.
Family 'I asked to go with him. He was going round, in the big machine, MRI scan. And I said I would go with him. They said, "No, he's all right" ... and if they'd let me go with him he'd have been all right because he could see me there. He wouldn't go in with the thing at all, wouldn't go in and they just wheeled him back.'
Family 'And I said to them ,"[patient] has not had an anaesthetic". I said, "She won't go in that machine". "You can go in with her". I said, "I've done all that". I said, "She will not go in that machine" I said, "and I'm not moving from here and she's not until you get someone down to give her an injection".'
The grand round was also frightening for patients,
Family R1: 'The registrar was presenting the case and we assisted and persuaded and helped [patient] to lie on a bed in full view of everybody. It's quite demeaning.'
R2: 'When you don't know what's going on you clutch at anything.'
R1: 'We felt it was for our benefit. I would now question whether its actually for the medical profession's benefit and whether [patient] would actually gain, was he being showcased ... detailed all the tests and examinations that he had been through, the history etc because it was all in medical jargon, which we now know intimately but then we didn't. So it didn't mean anything to us. It was probably very intimidating, quite intimidating for us but it certainly was intimidating for [patient].'
There was a perception that the programme of investigation prevented therapy and care being given. Some families thought that the trauma of the investigations had further weakened the patients.
Family R1: 'I think the volume of tests'
R2: 'Finding time out of the testing programme.'
R1: 'Became more important than the physio.'
Family 'But I think, wrongly I suppose, we all felt at the time, the first time he went through to [neurology unit], because there had been a deterioration, it ran through your head that all these tests and everything had taken it out of him and had actually worn him out. Which was probably not the case but because we didn't know what it was at the time. It sort of ran through your mind that you knew he was different, that you thought, maybe we shouldn't have put him through all the tests.'
Family R1: 'It was this at the end of all these tests [patient] had a liver biopsy, a further lumbar puncture, all those tests again I believe all the EEGs, all those tests again, lots of blood tests it was a very, very traumatic time for her.'
Int: 'It was traumatic undergoing all the tests?'
R1: 'Oh yes, it took, I mean she was very low at that time anyway physically and mentally it was very, very demanding on her because she was so weak at the end of that.'
Several families described their guilt that they had sanctioned the investigations. Some felt pressurised into agreeing to invasive investigations, and some felt that they were used as guinea pigs, that some of the procedures were not for the patient's own benefit.
Family 'So I was going to take her out of the hospital. I said, "Forget it. She'll come home" and then they all congregated the next morning giving me all the, "We ought to do this and do that". And you're torn then. Should I let her have it or shouldn't I let her have it and all. Anyway I gave in and I wish I hadn't because the poor little thing was in more discomfort ... I think it's as if you've been treated like a guinea pig you know.'
In 2 cases planned biopsies were postponed at short notice. In one case, it took several months to agree a policy on theatre procedures to allow a brain biopsy. Meantime the patient remained on the acute neurology ward until she was too ill to move to a more appropriate place for terminal care.
Care in acute wards
The organisation and type of care provided in psychiatric units were often inappropriate for patients with deteriorating physical problems. Similarly, acute medical and neurology units found it hard to manage patients with severe psychiatric disturbance.
Psychiatrist 'He was not appropriately placed in an acute psychiatric ward, as he deteriorated he became more and more dependent, with deteriorating mobility and needed help to walk or he would have falls. I recollect the ward was rather busy and had quite disturbed patients. He was vulnerable because of his lack of physical independence and staff constraints. It was difficult for the nursing staff, they were distressed at his rapid deterioration and it was outwith their day to day experience. They were trained to manage psychiatric disorder, not progressive physical disorders.'
Psychiatrist 'She needed physical as well as psychiatric support. Obviously on an acute psychiatric unit with disturbed patients, I wouldn't say patients with CJD could be adequately cared for on an acute medical or an acute psychiatric ward, their needs can't be fully met on either. There's no doubt about the difficulties, we are not geared to caring for patients with deteriorating physical conditions on a psychiatric ward and they are not geared to caring for patients with deteriorating mental states on medical wards.'
Patients deteriorated rapidly when in hospital, so their care needs changed rapidly. Most of the families were unhappy about the care provided in acute wards and there were numerous examples of substandard care. Often the staff caring for the patient did not seem to appreciate the extent of their rapidly increasing needs. For example, patients had poor motor coordination and difficulty eating, but food would be left at the end of the bed and taken away when the patient could not reach it.
Family 'I was there most mornings at 8.30 anyway, because I tended to think they were, I wouldn't say they were being slack down there, but she couldn't eat very well and they tended to leave things at the end of the table and she couldn't pick it up and eat the damn thing and then they would come in and say, "Oh you don't want that" and they would go away ... so I used to get down there early.'
Patients developed difficulty swallowing, but staff were unsympathetic when they couldn't swallow tablets.
Family 'And this orderly came over this day to give [patient] her pill and by this time she didn't want to take food or to drink, there was something wrong with her throat. So the orderly was trying to get her to take this pill and [patient] is going, didn't want it and that girl said to her, "Well you know what'll happen if you don't take this pill" ... she was doing this like you would do with a dog to make her swallow.'
Family 'And another time we went and he was having problems swallowing and she ... I think it was a staff nurse, she came and gave him a tablet and she didn't stay and check that he'd taken the tablet. She ... was just walking away and I said "He hasn't swallowed it" and she came back, she was quite annoyed with him and she said, "If you don't take that tablet you'll have an injection and you won't like that, will you?"'
Other families described finding their relative soaked with urine, when staff did not realise that they could not walk to the toilet.
Family 'Because they was trying to say that [patient] could go to the toilet and use the toilet. How many times we used to go up there and she was sitting in this chair. The pools would be underneath her and they were just leaving her in it and they just wasn't seeing to her.'
Family 'We had to tell them over and over that he was falling and afraid to get out of bed so eventually when they took notice of that, their reaction was to put up the sides of his bed and give him the buzzer and say, "When you want to get out of bed call the nurse". Now they did that very kindly but as soon as they'd gone he couldn't remember what they had said, you know, he didn't know what the buzzer was for.'
Family 'There were lots of times when he was wet, he hadn't been like changed and if you said to them, "Could you please come and change him" because he would be moaning, because like I said it would be burning him, it was like, "In a minute".'
Patients were often fearful of strangers, emotionally labile and became child-like. They were distressed at being left on the ward and did not understand why they were there or why they were undergoing all the investigations.
Family 'Trying to explain she had to stay was, for these tests, to find out what was wrong, she was getting upset, so I sat there till about six/seven o'clock and the way I had to come out was just say "Goodbye", give her a kiss goodbye and just walk. I couldn't turn round because if I'd turn round, I knew she was crying, I would walk back and end up stopping there most the night though I had to get back for the children.'
They needed reassurance and sympathetic handling, but this was not always forthcoming, as this comment demonstrates,
Family 'We would leave at night, you know, quite late at night and she would be clinging on, you know ... we just said, "Have you got a nurse that could just sit there and hold her hand, you know, just stroke her?" "No."'
Hospital staff didn't seem to understand how rapidly the patients' needs were increasing. Families commented that they noticed their relative's problems and pointed them out to the staff, but felt their views were undervalued,
Family R1: 'I think as well it's because [patient] walked in and looked quite capable of feeding and drinking himself.'
R2: 'But he wasn't. They didn't treat him as though ... they treated him as though he was in for tests and there was nothing wrong with him.'
Family 'We felt that we, I'm sure a lot of families feel like this about any kind of person that goes into hospital, but we felt that we had more knowledge about what had gone wrong with him although we didn't know the diagnosis and that we were teaching them but they were slow to pick it up ... It is as if they felt they were acting in isolation of us and they had no obligations towards us whatsoever.'
Acute wards did not have enough staff to provide the high level of care needed, and other ward crises prevented care of these dependent patients. Many families spent all day in the hospital ward to feed and look after their relative themselves, because they felt they would not otherwise be adequately cared for. One respondent recalled coming to visit her son and finding 'no staff to feed' written on his notes.
Family 'The staff on the ward were very minimal ... and we were getting things like relatives of other patients coming in and saying you know "[Patient] didn't eat anything, the staff didn't have time to feed him so I've been trying to help him" and one lady was bringing in trifles and things to try and get him to eat because the staff didn't have the time because it was taking about an hour to feed him then ... That's when we started to do 10 hour shifts in there ourselves.'
In this case another patient had also helped to supervise him when he repeatedly tried to jump out of his wheelchair and fell over.
Family R1: 'He would sit next to [patient] with his hand on [patient's] knee and he would doze and as soon as [patient] moved he grabbed him and hold him or pin him down. Otherwise he would have been on the floor. There was no nursing care.'
R2: 'The only alternative to that would have been some form of drug or restraint. So I mean that was brilliant. We owe that man a great debt.'
However, it was also important to families to be able to provide care themselves, and for staff to allow them to do so.
Family 'I knew that nobody could look after her like I would look after her because again I just felt she was my little girl so I had to look after her so I wouldn't leave her for a minute.'
Families felt that acute wards, where other patients got better, had little to offer patients who instead got progressively worse,
Family '[Neurology unit] was a medical hospital. There's nobody seriously ill. People were, the man beside him had lost the ability to walk and he was in there and he was getting physiotherapy. He left being able to walk out .... They were nursing in that situation, you know, where [patient] was the opposite. [Patient] things were disappearing. I don't think they were geared up for it to be honest.'
Some patients were very disturbed, and difficult to manage on an acute medical ward. Professionals found it a dilemma whether to sedate or restrain these patients, and families were distressed when they were over-sedated.
Neurologist 'Then there was a stage when he became agitated, restless, ataxic and falling over, this was the most difficult stage ... not knowing what was best, to sedate him or restrain with cot sides or to allow freedom to do the best he could.'
Family R1: 'I don't know what it was they gave him but we went in one day and it had just taken away so many of the functions that he had still got left that they had to put in a big square....'
R2: 'Brain damage cot.'
R1: 'And he was virtually crawling round it trying to find a way out and he was unrecognisable as a human being at that stage. I don't know what it was they gave him.'
There was an impression that patients became part of the hospital machine, rather than an individual who deserved to be treated with respect and dignity. Families appreciated professionals who interacted with patients and wanted them to able to wear their own clothes.
Family 'They never actually treated him like a human being.'
Family 'We at one stage had to say you know we've got to think of his dignity because they were leaving the curtains open.'
Family R1: 'I didn't want him in pyjamas all the time. I demanded that he should wear his jeans. That was what he was used to wearing ... But they would insist that they would put those damn jogger bottoms on him.'
R2: 'Because its easy for nurses.'
It was important to the families to maintain a sense of 'normality'. Keeping things 'normal' made them feel more in control, and it was distressing to see tubes or lines that reinforced how ill patients really were.
Family 'I remember going in there one day I had left her the night before in her bed with a telly in the room and it was quite sort of, you know, flowers on the table and that sort of stuff. And went in the next day and she was just on a mattress on the floor. And when I said to the nurse about it she had a bit of a go at me saying I didn't care, all I wanted, all I was concerned with was how it looked ... I think in a way you almost kid yourself you make them look as normal as you can just because it's something there to say, "Look, well it's not looking too bad".'
Sometimes ward policies were inflexible and unhelpful for families. For example, one family was prevented from spending time with the patient by rigid visiting hours. Another respondent commented on her distress at repeated telephone calls in the night, to report that her daughter had fallen out of bed.
Many families commented on their feelings of guilt at leaving the patient in a unit where they were not well cared for, and of their relief when they were transferred to a different unit for terminal care. One husband was so unhappy at the care his wife received in a psychiatric hospital that he discharged her.
Family 'We were so upset. The state he was in that we considered bringing him home from the [neurology unit] and they said you wouldn't be able to do that but I know families that have done it. I know it's very difficult but I really wanted him out of there.'
Family 'It was the time that you just felt it was so out of your control. You were doing everything you could but you just felt so awful, dropping her off at a place that she really didn't want to be, she was so anxious, so tearful.'
Family 'As soon as you left you could see her little old face, I used to say I've got to go because I've got to sort the kids out and get them, start work in the morning and you hated going, you know what I mean. You walked out the blooming place and you felt so guilty and when I walked in you used to see her sitting not interested in anything.'
Family 'And we were happier knowing that he was being really well cared for. That was one less stress, one less anxiety.'[Following transfer for terminal care]
Care at home
Seven patients were discharged home pending results of investigations at a stage when they were rapidly deteriorating. Where patients were discharged during the course of investigation, it was left to the GP to organise care at home and there was often a gap before services were arranged. Three families borrowed equipment, such as wheelchairs, to help them manage. It placed a great practical burden on the family, to care for patients with poor mobility and incontinence, who needed help to wash, dress, and feed. The following passage demonstrates this in one patient who was at home between admissions.
Family 'His walk was getting much worse. We were at the stage when he, to get him to the toilet, he used to put his hands on his shoulders and we used to hold him round the waist and walk him backwards to get him into the toilet upstairs. Well we didn't have a downstairs toilet. So he had to get up in the morning but [patient's father] used to have to sort of lift him a stair at a time really to get him up to bed. The bath by this time was a nightmare. It took 2 of us to get him in and out of the bath. He was frightened, he was really frightened wasn't he?'
The next passage, from the same respondent, describes the patient's rapid deterioration during the 6 weeks he was at home.
Family 'They brought a wheelchair and the day they brought it I made them put it in the garage. I said he wasn't going to use it. It lasted about 3 days I think and then we had to bring it in because we needed a wheelchair to get him to the toilet, to get him from room to room. It was that quick, you know, within days he'd lost the ability to walk basically.'
Others went home from the ward for short periods of time. One family described feeling that once off the ward, the patient was not the professionals' responsibility,
Family 'It's like, once you were off the ward, that was it ...You clocked out and clocked back in but in between ... Once I'd taken him off that ward they were quite happy. You know, it was one less to worry about.'
Time to diagnosis
The time from onset of progressive symptoms to referral to a neurologist ranged from 11-65 weeks, median 25 weeks. Time from onset to notification to the CJDU, which is a proxy for consideration of CJD as the diagnosis, was between 12 and 90 weeks, with a median of 46 weeks. Many families thought that the condition should have been picked up earlier by the GP. Several said that GPs should have guidelines to help them identify symptoms of nvCJD earlier,
Family 'Surely they should have cottoned on to that a bit earlier. I can understand it up to ten cases, or fifteen but ... they're still not picking it up fast enough at the GP's.'
Some of the GPs discussed their own discomfort that they had treated the condition as psychiatric before referral. But the early non-specific and psychiatric symptoms were common whereas nvCJD is extremely rare. Most thought that they had managed the patient appropriately according to the symptoms and signs presented, but appreciated that the family might feel unhappy that the patient had been treated for depression initially.
GP 'We see lots of people with [non-specific symptoms] and I'm sure most haven't got nvCJD ... There might be lots of people we would think might have nvCJD. It was distressing for the family going through the psychiatric symptoms, but they are very non specific symptoms aren't they ... It's a diagnosis of excluding lots of other diseases and the end point is this very rare disease.'
GPs recognised that the family's anger about an earlier psychiatric diagnosis might have been a barrier in later care of the patient and family. Interestingly, the GPs who discussed this potential problem included cases where families gave no indication that they were unhappy with care received from the GP.
GP 'I wonder if giving her a label which was psychiatric rather then neurological, if that's something which puts us in a bad light with the family later. I haven't seen her husband since the diagnosis and I made it plain I was happy to see him. I just wonder if he didn't find that particular consultation that helpful.'
GP 'It would've helped the family if we'd known sooner. I don't think we could have, but it would have helped him, he was very angry and bitter.'
PALLIATIVE AND TERMINAL CARE
Location of terminal care
Two patients remained in the unit where they had undergone neurological investigation until they died (these admissions were 6 and 15 weeks in duration). Three others were transferred from the neurology unit to be nursed in a medical ward, 3 to a hospice, 2 to nursing homes, 4 to other units including a respite unit and a community hospital. Five were nursed at home by their families but 2 of these were transferred to a hospice at the very end, where they died.
Several families commented on the difficulties of finding a suitable place for terminal care. Where available, hospices were thought very appropriate and provided very high quality care. But not all hospices would take patients who did not have cancer, and there was not always a hospice nearby.
Family 'The only thing that they were suggesting was that he would go into a hospice ... which is like miles away and when you've got young family is too much travelling all the time so I did try and get him into the nursing home .. but this was for the elderly which their age group I think starts off in their forties or something but they did say they could do like a one off deal because of what he's got, but he died before it even got going really.'
Four families mentioned that they felt obliged to leave the acute neurology ward because they were 'blocking' a bed. Acute wards also lacked the staff to provide the care needed.
Family 'But with [patient] in a general hospital we couldn't leave him there because they wouldn't put him in a side ward by himself because he had to have this nursing attention and they didn't have the staff to do it. They wouldn't special him. So he would always be to his dying day in the corner of that ward which is not right for a young person to die like that.'
Family 'We were told [patient] couldn't stay in hospital. We didn't want him in hospital any way, but we were told he couldn't stay in hospital because he couldn't be given any treatment and they needed the beds for patients who could be treated.'
One family found that the hospice director wanted them to move because of the long duration of the admission. It lasted 6 weeks when most patients in the hospice stayed there for 2 weeks at a time. The patient was hallucinating, shouting out and disturbing other patients. This family was offered an alternative place too far away for them to visit.
Some families felt strongly that the patient would have the best quality of life at home, especially where patients were very fearful of strangers. Some felt guilty that they were unable to provide care at home.
Family 'Quality of life for me is maybe in your own environment, maybe you might be able to cope with things differently if you are home and you can see people you know walking past the road, and I thought it might be better to bring her home.'
The trauma of visiting nursing homes and hospices to choose somewhere for a loved one to die is evident in the following extract,
Family 'We went up to see. It reminded me of things like going to school, looking for a school for [patient] when she was leaving primary school because we'd gone to a couple of schools, schools for her. So it sort of reminded me of doing something, it was strange, weird. We were going to this hospice and I couldn't believe we were going for what we were going for.'
In most cases, professionals took the lead in finding a suitable place. But one family had to find a nursing home themselves with no help from hospital social workers who clearly did not appreciate the urgency of the situation (the patient died 3 weeks after transfer). Arguments over funding also delayed transfer in this case. The health authority eventually agreed to fund the nursing home place but did not agree to 24 hour care, so that pads and an alarm were placed around the bed in case the patient fell out of bed in the night.
Family R1: 'He was deteriorating, there would be less and less nursing care, supervision. So they eventually broke at a deal whereby the health authority would accept a lower rate of charge if they didn't provide 24 hour nursing attendance but put pressure pads and an alarm beside the bed so that if he did get out of bed or anything they would immediately know.'
R2: 'He would be allowed to fall.'
Care from families
It was important to most families to be able to provide much of the care themselves, even when patients were in a hospice or ward. To meet families' need to care, they needed the patient to be nursed as close as possible to their homes and to have open access to the ward.
Family 'But best of all they didn't mind us being there all the time. Because I said, "There's no way we're bringing him in and leaving him and I'm not coming at visiting times. If that's what happens its no good" and she said, "No just treat it as your second home, come and go as you like. Nurse [patient] if you like, if you don't want help that's fine, if you do we're here".'
Families were totally immersed in caring during this time. They were spending most of their time with the patient and did much of the hands on nursing. Families expressed appreciation to nursing staff who supported them and allowed them to provide the care.
Family 'The night staff were angels. They really were good. They were friends weren't they. And I knew for a fact if [patient] woke, if there was any change at all she would be straight on the phone. It wouldn't be sort of a case of going to the next morning and saying he had a really bad night. That would never have happened. They would be on the phone straight away and let us know. That was very important with the people you trust. It doesn't matter if you're disturbed. You would rather be disturbed than think that you should have been there and you weren't.'
Family 'I will be eternally grateful to them for that because there was no way I could provide the care that they did. So I have given up work by this time. The pressure of it all with everything was too much at that time and I preferred to spend my time with [patient] now anyway. Because I knew that, you know, that the situation was so brief at that time, so I would come two or three times a day for the majority of the day.'
Family 'You don't want to know about anything else basically. We felt, the three of us, were in a glass cage and nothing else was getting through to you ... you just feel detached from anything else. There's only you two and your son or daughter. Everything is concentrated on that and everything else is just happening away from you.'
Family 'All the time you are trying to work out what's going on, what do they know, what will be helpful, what would make her feel less stressed, you know, physically, emotionally, mentally, psychologically. It is really, you know, you are measuring, all the time you are measuring, seeing changes taking place, trying to incorporate changes then you see an improvement, then it will go back. So it all was a tremendous observation all the time, sort of seeing every single little movement all the time. It was hard.'
All the families wanted to be treated as equal partners in the patient's care. They appreciated being able to do much of the care themselves with help from the staff.
Family 'That was a very daunting prospect for me to be changing the feeding thing. As it turned out I was actually shown how to do it and when ... it needed to be changed I actually did it. They let me do it, they let me become involved in that.'
Some felt guilty when they were not at the patient's bedside and needed support to get some respite from the emotional and physical burden of caring.
Family 'He'd wake up "mum". I'd say "I'm here love" and they'd say "don't get out of bed, you have your sleep, you need your sleep, we'll see to him. We can just say, look your mum's there, look, in that bed there" because this room had two beds. But it's hard when you are a mum. Your kids are shouting "mum" so you get up and go to them because it's their mum they want.'
Family 'When someone is so ill I mean you do want to do your utmost for them but when they do become so ill the person who is suffering does require such a high level of nursing care it needs to be done by professionals. There is only so much that, I mean the family obviously has emotional side of things to go through, and with this illness you have time to look forward to a death, you have to prepare yourself for a death. And so, having to take all that into consideration, it's so difficult to have that to worry about as well as caring for someone.'
Family 'We had years together and you don't just abandon someone because they get ill. I stayed with her 24 hours a day. I did start going out when my sister was here in the afternoons. She made me go for a walk for half an hour. But I know from what they said she'd never rest that's why I still say they do know because she was restless all the time I was gone. For half an hour she wouldn't rest. And so in the last couple of weeks I didn't bother going out at all.'
Care needs and care provided
Patients continued to deteriorate very rapidly until they were totally dependent. They needed intense nursing care round the clock. Some had hallucinations or were aggressive. These symptoms proved intractable and caused great disruption.
Family 'Can you imagine the nurses, what their job was like because every two minutes they had to go in and do something, turn him over, take clothes off, hug him, take him to the toilet ... There was always somebody going in and out.'
Family 'We knew we had to have somebody with her 24 hours a day. She also needed somebody with her in her bedroom and cushions, you know, at the floor in case she fell over. So somebody was around sort of at night and day, sometimes two people, sometimes more, because the person that was caring directly would also need support too, you know, because it is very draining.'
Family 'In that time he had to have a psychiatric nurse twenty-four hours a day because he was so violent and they tried to cope with him as best they could by sedating him. It was trial and error where you got it at the right balance and like quite a lot of times he was like too sedated.'
Nearly all the families expressed their appreciation to the nurses and carers who provided care during the terminal stage of the illness. Many contrasted this with the care they had witnessed in acute wards while the patient was being investigated. They commented on the personal attention received, and that staff seemed to care about both patient and family as individuals.
Family 'They must be handpicked these people. One couldn't be nicer than the other. They were just fantastic because they knew that that person was in there to die and they were going to make their final hours as comfortable as possible.'
Family 'They assigned him male nurses who would talk to him about football, take him out in a wheelchair round the grounds or chat to him about all sorts of things, cricket, you name it ... It was like he was still a person there ... And they would come in and if they were talking to us they would still be sort of touching his arm or something, he would be included in the whole circle. He wasn't an encumbrance to them was he?'
Families were grateful to staff who continued to assess and respond to each patient's changing needs.
Family 'They always seemed to be one step ahead and thinking about what his needs were going to be. We went in one day and he was having trouble sitting up. They brought him a reclining chair in. If he can't sit in the chair we'll make sure he can get out of bed and sit in this kind of chair. That mattress doesn't seem to be working, we'll get another mattress in.'
In the late stages of the illness, patients were unable to speak but families, who spent so much time with them, interpreted subtle changes in their expressions and felt they still had some awareness of their surroundings.
Family 'And his eyes used to light up when he saw them. You know he knew them. His friends used to come up all the time and I mean he was obviously delighted when they used to come didn't he, his face used to light up. So I really don't know how much he was aware of and how much he wasn't. Its extremely difficult to tell but he certainly wasn't deaf and he certainly wasn't blind. And he reacted to a joke. If you told him something funny he laughed. Right to the end.'
Family 'We didn't know what she knew, what was she thinking. We knew what we felt and we know that there's many different types of language apart from speech and we were connecting with all the different kinds of language, the body language, the sensitivity. The sort of the awareness, you know, the unspoken and that's what we were working with. That's the bit that we were learning about and that's hard actually because you are having to sort of learn it and use it at that time.'
Family 'I knew she knew what was what just by watching her eyes. The [nurse], because she sort of got attached, but if we said something to, like she'd joke with her about like me, you know, "blooming men", you know, "they're useless" and all that and you could see this change in her eyes. You knew she could understand. They say they can't understand but I'm sure she understood bits you said.'
Patients had changed in personality, some became psychotic and all were physically dependent, but of course to their families they were still the individuals they loved. It caused families great distress and anger when they were treated without dignity or respect, as in the following passages,
Family 'He was put into a side room on his own and what I didn't like about that was the door was left open. And right off see it was a ladies ward, and further down was the men's ward, and he had nothing on and he would kick the blankets off and sheets off, and he would be laying there with nothing on whatsoever. People could just look at him as they walked through to the ladies.'
Family 'My daughter was in with her boy friend ... And they come in, these two nurses came in and the boy friend was sitting watching the television ... and these two nurses walked in and proceeded to take [patient]'s clothes off while the boy friend was sitting in there ... We kept her dignity and then they tried to take it away from her.'
Family 'The first carer treated [patient] like a geriatric case you know. She toppled him about the bed. I can remember him responding to it. He was angry and he looked over at me one time and he started punching his fists and he was frustrated at this woman just hauling about in the way that she was.'
In most cases the staff who provided terminal care did treat each patient with dignity. They performed the necessary physical tasks sensitively and treated each patient as a person with individual needs.
Family 'I mean when he had these problems with his bowels they didn't make him embarrassed at all did they? They did it with such ... you know they left him so much dignity. It was "Right, we'll just do this, oh right, that's it done" sort of - not a problem - and talk about something else. No big deal.'
Family 'When he was still eating, sometimes he would refuse some of the food and we weren't sure if, I think we would leap to the conclusion that he was having problems eating. This is another downward step. Whereas [nurse] would come in and say "I'm not surprised you're not eating that muck" and put it in a human context, you would think, well, actually he's right, I wouldn't be eating that. And it made it seem as though he had still got some choice in what was happening.'
Many families said that staff spoke to patients as friends and treated them as if they were 'special'. They went to extra lengths to make both patient and family more comfortable. This often contrasted with the care they had received in acute wards and was deeply appreciated by the families,
Family 'Just their loving, compassionate way they had with her. Just that they treated her as special. She wasn't just a patient, she was a person.'
Family 'If there was any special food [patient] wanted they would have got it in for him. I mean the cook at the home, she used to bring his meals in because she, I mean everybody loved him didn't they.'
Family R1: 'And they used to tell [patient] everything. For all [patient] wasn't capable of answering them, she was the first one that knew about [nurse] being pregnant.'
R2: 'And they would go in and bath her or change her or turn her and they would chatting away saying, "oh my boyfriend last night blar blar blar we were out".'
Families felt there was more continuity of care in the terminal stage. This was important in allowing them to build relationships with the staff and for the patient who was often fearful of strangers. Often staff formed friendships with the family, had attended the patient's funeral and remained in touch with the family.
Family 'We became friends and that made the difference and they still are friends we still see them.'
Few of the professionals had managed cases of nvCJD before. Often families gave them information about the condition. This helped them to feel they were equal partners in the care.
Family 'I mean they had never had a patient with new variant CJD and they didn't know what to do. I mean they were asking me how to bath him, what have you, shave him. I did actually feed him because he could not feed himself, you know. I used to go every morning and feed him his breakfast.'
Some families felt disappointed by the lack of medical treatment available to help the symptoms. They felt abandoned by the doctors at this stage, or that the doctor's visits were token.
Family R1: 'They virtually gave up from there. There was nothing else done. The neurologist popped in once a week ... he would pop his head round the door and say how's [patient] been and I would say much the same or he's deteriorated and that was about it. He wasn't having any medical treatment.'
R2: 'And the nurses didn't sort of check his temperature and blood pressure every day.'
Family 'Only one prescription and it was for a suppository. It was only one. After June there was no medication for him, to ease things you know but there's no really we're talking about cures or pain. I thought that the medicine fell down on that because they weren't up to much.'
There was confusion over infection control procedures and some staff required reassurance that nvCJD was not contagious. Two patients were found to be colonised with MRSA, requiring infection control precautions. Families worried about being treated differently because of the diagnosis.
Family 'When [patient] was in hospital she used to have get suction and the nurses used to come and put the gloves on of course and suction her, and in the latter stages all of a sudden it was full face masks, different coloured overalls and rubber gloves. And the nurses were as confused as we were why all of a sudden it was like that.'
Family R1: 'Even when they knew what it was they didn't start treating you any different and like you were contaminated.'
R2: 'I mean to get as close as putting nappies on and things like that but there was no I'm not doing that, I'm not touching you ... I mean there was none of that.'
Professional 'It was quite difficult talking to the nursing home about her needs, a lot of fear about nvCJD, how to nurse them, can it be passed on, effect on other patients and staff. The sister came to the ward and we chatted and then it was fine.'
Nine patients received physiotherapy but families differed over its value. Some thought it was too late, or that it seemed to cause pain. Others found it helpful to relax and loosen the patient. It was seen as something positive they could do to help the patient.
Family 'So they did do quite a lot of exercises, different exercises to help her get out of bed without any problems and she used to pull faces at them all the time but it did help. I think it loosened her up but the involvement was ... and they were very good and they used to, you know, keep her quite cheery as well.'
Many families focused their anxiety on nutrition and the patients' weight loss. All patients lost their ability to swallow and 5 patients were fed by gastrostomy (PEG). Seven families reported some input from a speech therapist for advice on swallowing and only 3 reported input from a dietician.
Family 'Two weeks before he died was the last time he would eat. He wouldn't eat anything over and you know I face it. It really distresses me to know that my son was going to starve to death. There was nothing. Nothing they could have done. I always thought it could have been a more comfortable death.'
Family 'My main concern ... was because I didn't feel I was able to feed her. I tried and tried but it was very difficult due to her being so tired, due to her inability to swallow properly. I was, I felt that if I left it any longer then she may starve to death so I was hoping obviously as well as receiving some respite that they would perhaps help to build her up.'
Family 'Because he was literally starving to death. He wasn't having, he couldn't eat anything. So they took him down and put the PEG in. And that was a great relief. You could see they put a bag of liquid feed up and you could see it dripping in and at least he wasn't hungry.'
Others wanted help to discuss the implications of artificial feeding and other end of life decisions.
Family 'We had to go through the nightmare of deciding, do we feed through the stomach ... We all felt that we didn't want to interfere in that way because we were looking for quality of life and we didn't want to prolong something that was going to become a nightmare. We didn't know, you know, how long it could go on but we were trying to find out about it.'
Terminal care at home
The range of professionals involved, roles they played and care provided varied for the 5 patients nursed at home terminally. In two cases a social worker coordinated care and provided support to the family. In one a Macmillan nurse did so, with a social worker closely involved. In the other two the GP played a key role, but Macmillan nurses were also involved.
Most patients were sent home before the care package was organised. In one case a special arrangement was used to get funding quickly. Some families felt the care was not flexible enough to meet the patient's rapidly increasing needs and their own occasional need for respite.
Family 'I was really ill and I couldn't get out of bed. I wasn't allowed any extra help to come in to tide us over that period ... When you're nursing a terminally ill patient, especially with this disease, you need what you need and you need it then. You don't need it six months down the road.'
Family 'It happened that quick and it was changing that quick that their normal routine wasn't working. They put two carers to visit [patient] four times a day wasn't the answer. Somebody had to be there every day to see how it was changing. How his needs were changing.'
The families who provided care at home wanted to do much of the caring themselves. Some felt overwhelmed by the number of professionals involved, and wanted to minimise the number of strangers caring for the patient. One family was also very concerned about confidentiality and did not want all the professionals to know the presumptive diagnosis.
Family 'We made the decision together that we would actually look after [patient] ourselves and we would do as much of the care as we possibly could. Because of the person that we knew, that she loved her family around, she had reacted badly really with the hospital and they had been cold and indifferent in her care and support and we felt that we didn't want strange people dealing with her.'
Family 'I would never have, wouldn't like to see them totally taken away to someone else strange especially in that situation because they need someone they know and they do. Right to the last I'm sure [patient] knew who I was. I don't think you want total strangers.'
But families did appreciate professional advice.
Family 'I was pleased with their help, just the little things, young [nurse] one morning got her in the bath, and the sort of thing I wouldn't think of, she'd got a little bit of soreness under her boob, and you know that's the sort of thing I wouldn't have thought about, but it wasn't a sore as such but she just said, "Well we must get some cream put in there". She was really good.'
Families felt they had to fight for the care needed. Sometimes lack of funding prevented them getting the best care. Families felt they understood their relative's needs, and the best way to care for them. But they had to wait for professional assessments and argue for help.
Family 'I was allowed one nappy a night which was ridiculous. All we got was "It's too much money, it's too expensive". And I think personally I think it was because they was told I would only have her for a few weeks and they didn't want to go to the expense.'
Family 'Well then I got a fully qualified nurse seven nights a week. I had to fight for this as well but I got one. At once stage when [patient] was taken ill they let me have a qualified nurse while she had the pneumonia and then I had to go back to auxiliaries but I didn't like that because they wasn't trained.'
Family 'If you can see someone is completely helpless they don't need to be assessed do they, in my view. If they can't go to the toilet they need something to make them go to the toilet ... But it really got you down. I mean to keep arguing with people because you think to yourself, you know everybody, you're always up there making trouble as they call it but you're not, you're only sticking out for the person that's ill aren't you.'
Two families moved house and found that district nurses in the new location did not accept the previous assessment. One family had to wait 3 days without incontinence pads for reassessment and for care to be re-instituted. The other family was unhappy that the criteria for nursing tasks changed: district nurses in the new location were unwilling to wash hair, and that the family and home carers were now expected to do it.
The social workers discussed the need for specially trained and sensitive carers to help care for these very dependent patients and support their families.
Professional 'Because it was a special case, it needed a trained and sensitive carer ... In the future what's needed is 1-2 skilled carers trained up to deal with a situation like this. They would need great sensitivity. I was lucky to tap into this one carer. It needs extra money available for a couple of experienced skilled carers. They would need to be experienced in sensitivity and understanding of the terminally ill. And also some lifting and handling. And dealing with the health service. And also knowing if they needed to, training in nursing tasks. Skilled workers.'
Professionals involved in providing terminal care
The GP's involvement in terminal care varied. In many cases where the patient was in hospital the GP had no further involvement. Where the patient was cared for at home, or in a unit where the GP retained clinical responsibility, he/she was more involved. Some families reported that the GP only came if called, but in others the GP visited frequently and provided support to the family as well as medical care of the patient. In these cases the GP already had a good rapport with the family and they appreciated his/her support.
Family 'Well he listened. He also would contact you. He came in to see us every single day as things all got much worse. He never, ever made us feel that he hadn't got the time. He would always make sure that we had a contact number ... Even when we were in the waiting room afterwards when we were all feeling traumatised and needed to see him he didn't let us wait in the waiting room. He'd just say, "Come in straight away" you know, because again you feel so exposed.'
All of the GPs interviewed commented that they often play an important role in terminal care, including support to the family. But some thought they had lost the family's confidence in the initial stage of the illness. One GP expressed regret that he was not able to offer much support to the family but still visited the patient.
GP 'In terminal care I ... often don't do anything, visit under the auspices of pain relief but play a pastoral role for the family, with a physicians role in sorting out some medication now and again ... I didn't know what purpose I was serving, perhaps just my own need to do something, or guilt that I wasn't doing anything.'
The involvement of the neurologist in terminal care also varied, but many remained involved and offered advice on management. Most of the neurologists interviewed thought that, after diagnosis, care should be led by a palliative care or rehabilitation physician. But it depended on local circumstances.
Neurologist 'Its difficult, in the past we were seen as magicians, came in from afar, made the diagnosis then disappeared. Ideally we should be involved with patients, I happened to be involved with this case but we can't offer that to everyone.'
Neurologist 'I'm not sure there's a role for us in follow up, we know they are going to decline and probably its more appropriate that palliative care services are involved. Especially when they are young. They can do more to help the family come to terms with it on a 1 to 1 basis than I can in 10 minutes in outpatients. Neurological follow up in terms of support is not appropriate. We can't provide what's required, which is support and coping skills and terminal care.'
Specialist palliative care services were involved in 12 cases, to varying degrees. Five patients were in-patient in a hospice, 2 others had input from a consultant in palliative care medicine and in 5 cases nursing staff sought advice from a Macmillan nurse on the patient's management. All the palliative care professionals interviewed agreed that it was appropriate for them to be involved.
Professional 'We can provide total care, facilities for families, counselling if its needed. The families do need a lot of input ... it was very appropriate her being [in a hospice].'
Three of the families reported that they had felt pressurised to agree to a post mortem (PM) examination that was against their wishes, or that they had been asked to agree to the PM very insensitively. Two others were shocked when they heard that their relative's brain had been removed at autopsy for further study elsewhere. The PM results took 6 weeks in most cases, but some families said they had never been formally told the results. In one case there was a delay because the carriers refused to carry the remains.
Family 'I found out later, very much later, what had actually happened on the autopsy. Because they never told us what was going to happen. They didn't tell me they were going to take her brain out and all stuff like that so I was a bit annoyed about that.'
Most of the families were distressed that they were not allowed to view the body of their relative. Those who did insist on viewing the body were only allowed to do so for a short time and were shocked when they saw the changes in the body.
Family 'But you could only just see her face, no hands or nothing and she looked in a bad way. I was sorry I did go to see her really and then he said, "Scrub up. Don't touch anything, wash your face, wash your hands". Because he asked me, he said "Did you touch her?" I said, "Of course I did, you know, I stroked her head and that and stuff and gave her a kiss and that." So he says "Go and wash you face and stuff". So it made me feel a bit bad.'
Family 'Because I had worked myself up to go down ... I thought right I am going down to see [patient] just to say goodbye for the last time and I got a phone call to say I couldn't basically you know ... Even that was a phone call, there was nothing personal, nobody came and said.'
Family 'We wanted her in the chapel of rest for longer. We was told at first we could only have half a day and I said but that is ridiculous, because of contamination disease, and I went mad and I said, "No way. She didn't contaminate anybody when she was alive. She certainly ain't gonna do it now she's gone". I went mad and I had to fight that as well.'
One respondent was very angry that he was not permitted to pay his last respects after the PM although he had been told by hospital staff that he would.
Family 'I was assured, "No, no there will be no problem at all, nothing at all" so I sort of said, "Alright, OK, on your assurance" and all the rest of it. Anyway, she came back ... and the funeral director said, "I can't, she is in a body bag and we just can't open it." Which, I was just numb with anger really. They just took away the last the last time you see someone you love that's just and it still makes me so angry now just thinking about that.'
Families were told that their relatives' bodies had to be sealed in body bags, to prevent exposure to contaminated body fluids. This did not make sense to them when they had been in such close contact with the patients before death.
Family 'And the undertaker ... came up that night and he said, "I want to know what's going on". I said, "Well what do you mean?" and he said, "I had [patient] through to the hospital and I got a ticking off because he wasn't in a body bag". I said, "Well that's ridiculous. We've been nursing him. You know without any barrier nursing methods or anything for a year now. I said you know its nothing infectious".'
Family 'The head person in the morgue told us that we're to see that he was put in black body bags, the ones with the zip and we weren't to touch him whatsoever. So I said "well, why?" and he said, "Because of the body fluids". But, I said, "I don't understand this" I said, "Because when he was actually in hospital", I said, "He used to dribble quite a lot, I've been there wiping his mouth", I said, "no-one's told me I'm not to touch him" ... I said, "I've been changing his pads", I said, "with urine". I said, "They've not told me that I mustn't do anything. Mustn't touch him, or to put gloves on or anything. I've been doing that with my bare hands", I said, "so how come there's all of this big fuss?"'
In one case, the family wished a burial but were advised that cremation might be safer. When they resisted that, the grave was dug more deeply than usual, two employees of the council attended the burial in white coats and the grave was lined with lime. Liming is not necessary in these circumstances and the family still feels very hurt and angry about this.
Family 'At one stage they insisted that we cremate [patient] when we'd already bought a grave and they didn't give us a justifiable reason why we should cremate. We were promised that we could have a normal wake at home and a normal burial and we didn't get that. And [patient] came home we were told by the undertakers when they collected the body, we were told to go back that evening and seal the coffin. We were told that we could have the coffin open for a number of hours but we were to keep young children out of the room and that we were not to touch the coffin and not to touch the body or touch anything inside. So its a matter of public record ... At one stage we got a phone call to the family home insisting again that we cremate [patient] when we were almost taking him to the church. The family priest in the home refused and said "I'll bring this man to the church and keep him in the church." "You're not." That's when he was threatened by the police. The police would come and impound the body. We had to put all this off ... But the liming of the grave in particular we have never been given any kind of explanation for that. Its still, you've no idea, how much that still hurts you know, that they limed his grave. It's just as if they punished him for something you know ... They've committed an awful crime these people that have died of this you know.'
In many cases there was confusion over the arrangements for the undertaker or in arrangements for the death certificate. Hearing about confusion and misunderstandings between the undertaker and the doctors caused further distress to grieving families.
Family 'He said, "When I went through with [patient]'s body to the [neurology unit] where the post-mortem was done nobody there would sign the death certificate. So I came back here and contacted his GP and they wouldn't sign it because they hadn't seen him for months" and he said, "I got in touch with [hospital] and by chance the neurologist was there and he said I called him and he's signed it". He said, "I've had such a carry on. I've never had such a carry on getting a death certificate".'
Family 'The undertaker had to take [patient] back up to the hospital. The doctor would not sign this form until she had seen the body so had already seen the body but she wouldn't sign this form again until she had seen her again would she.'
Many of the families were angry that the death certificate did not state nvCJD as the cause of death. They felt it was important that nvCJD was recorded, so that its importance as a cause of death is recognised. One respondent said he wanted it recorded in case of future claims for compensation. There was a strong perception that under-reporting of nvCJD on death certificates was due to a cover-up by the government.
Family 'We wanted [patient]'s death certificate put right. It didn't say what he had died from and it seemed to me that this coroner was in cahoots with [neurologist]. The coroner was being obstructive.'
Family 'The government at this moment have said we cannot put that down as a reason for death. The reason for death is bronchial pneumonia, all be it, it is only bronchial pneumonia because she has got CJD and dementia.'
Waiting for the coroner or procurator fiscal to agree to the funeral was an added obstacle for these families. Other families wanted to avoid media exposure and thought an inquest would stimulate press interest.
Family '"I'll ring the coroner while you're here and see if he'll accept this". And of course as soon as she rang him he said "No. No we'll have to open an inquest" and I said, "What do we do now?" and she said, "Sometimes in these circumstances you have the funeral without the burial".'
But many families wanted to have an inquest. They wanted a 'death by misadventure' verdict, to apportion blame for the death. One family was angry that the coroner publicly stated his opinion that nvCJD and BSE were not related.
Family 'I think what you want is for somebody to put their hands up and say, "OK we were responsible" and unless you have that inquest and a death by misadventure, you're not going to have that.'
In a few cases, most of the funeral arrangements were made before the patient died. These families found it helpful not to have to plan practical details just after the death.
Family 'I've never arranged a funeral before, I wouldn't know where to start, but because you know that it is going to happen you can make these arrangements ... But we did feel awful ... Because you know when it happens you have got days to make these decisions and once you have made them and they are the wrong ones you can't go back and do it again ... So we did actually have time to grieve at that time because everything was in hand. It was like two phone calls and that was it, you know.'
PRACTICAL ADVICE AND HELP
The patients had very high and rapidly changing needs, placing a heavy practical burden on the families caring for them. Many families also faced financial difficulties, difficulties with transport to and from hospital, or logistical problems in arranging childcare and managing ordinary activities like shopping and housework while also caring for their relative. It was particularly hard to deal with these practical problems at a time of great emotional stress.
Practical problems when patients were in hospital
Often patients were investigated in a tertiary centre far from their home. Families spent considerable time and expense travelling to hospitals. Several found parking at the hospital a major expense, though 3 were offered free parking as a special arrangement. One respondent relied on friends to take her to hospital to see her daughter. Some respondents were working full time as well as trying to spend as much time as possible with the patient.
Family 'I could only get in evenings because of my job. I used to start ... in the morning and I won't get back there until sometimes 10 o'clock at night you know the time I got in.'
All the families spent as much time as they could with the patient in hospital, and many spent the whole day there,
Family 'Every single day I was there, all day long. I was getting picked up 10 or 11 o'clock at night in the car to bring me home and was going up and getting up and going up there the next morning and I was there all day.'
Many reported that they lived on take-away food or food from the hospital canteen. In 3 cases hospital staff helped the family get hospital food more cheaply, which was greatly appreciated.
Family 'Actually to save me coming back and cooking I was getting food from their canteen, and when one of the nurses found out I was actually paying sort of visitor prices for it, they got me a "to whom it may concern" note you know. So I got staff price food which was a bit of a bonus as my culinary skills are pretty limited so that was good.'
Some slept in the hospital, usually on a chair in the patient's room. Where patients were too far from home for the family to travel daily to see them, families were given no help with accommodation or transport.
Family R1: 'We didn't have any approach from any hospital social worker. Bearing in mind ... most people commute, travel to it. Its nobody's local hospital. There should have been some form of infrastructure that sort of sat down with you as part of the induction saying how are you going to cope with this and nobody did.'
R2: 'We couldn't get a sandwich or anything when we got there.'
Family 'I was on a Parker Knoll chair thing and the damn thing if you sat up the bed came up with me and all and I could never get comfortable in it.'
One family found that they could not take their son to the hospital cafeteria because it was not accessible for wheelchairs.
Five families mentioned difficulties with unsuitable transport to hospital. Patients were poorly mobile, uncoordinated, sometimes hallucinated and unpredictable. But families were expected to take them to hospital in their own car without any help. In one case, a volunteer driver came to transfer the patient between hospitals because there were no ambulances available. Other families described their fear that the disturbed patient would jump out of the car while it was moving.
Family 'We took him into the [neurology unit]. It's a massive place and we parked miles from where we should have been. We had to more or less hold him up, drag him, hold him up, till we got onto the right section and the hospital.'
Family 'If he'd had a frustration attack in the car we could have all been killed ... He once tried to jump out of the car. I was taking [patient] out just for a ride and he didn't want to go but we knew we couldn't leave him in the house .. and he tried to jump out of the car ... He's been in the car and he's clenched his fist and punched at the roof in the car.'
Family 'He had to be helped into the car and helped out and indeed they were on the motorway and he tried to get out of the car. He was just delirious.'
In 4 cases there were delays in investigation or care because of delayed transport, often for several days. In one case the ambulance was cancelled twice before it finally arrived to take the patient to hospital for an MRI scan, and on the third trip the MRI scanner was not working. But even shorter delays were difficult for emotionally labile patients and stressed families to deal with.
Family '"The ambulance has not come for him yet, it'll be here in a few minutes". It got to 10 o'clock. He rang again. He rang at half past 10. He said, "Look, I'll come and fetch him, we've promised him he can be home this morning". She said, "Well he is going on a bit, saying I want to go home, he said this morning". And [patient's father] said, "Look, I'll come and fetch him". "No, no, they'll be here in a minute". Quarter past two he arrived here and he sulked for about an hour.'
Family 'And all the cards I just packed them all up and packed up all the bag and everything and of course there was no damn ambulance. So the next day they say there'll be one the next day. So the next day we were up and ready again and, "Excuse me, there's no ambulance available again" and that was Friday. And I thought Saturday, Sunday nothing happens in a hospital. You know what its like the bare staff isn't it. There's nothing happening at all and I thought I can't stand it. I can't stand it.'
Practical problems when patients were at home
The practical problems of managing patients at home started from the early stages of the illness. Some patients were at home between hospital admissions or had terminal care at home. Most of these were discharged before practical arrangements were made for their care at home, and it was left to the GP to arrange the necessary support. When patients were at home, the burden of care fell on their families.
The early features of the disease presented insidiously and none of the families said they had had useful help at this stage. Patients displayed changes in personality, unpredictable behaviour, and loss of short term memory. Most of the patients required constant supervision from an early stage, because they would forget what they had just done, needed reminded and helped to wash and eat, and needed the reassurance of a familiar person with them.
Family 'She would ring up at about 10, 10.30 in the morning crying down the phone things like ... "The house is on fire" .. I mean it's frightening but you can't leave it you can't just say, "Look forget it love it's not happening". You have to come home, you have to get back here as fast as you can to put her mind at rest ... and she would be quite happy as long as there was somebody there.'
Family 'Well she would just despair ... she would cry she would be upset you know at lot of the time. And it was understandable because she found simple tasks in the house difficult to do you know making a cup of tea or anything like that it was very difficult at that time you know. I did practically all the duties in the house ... as well as going to work.'
Family 'She wanted as well she wanted to stick with me. She wanted to be with me all the time and she wanted to be sleeping in my room with me.'
Many became hallucinated, deluded and/or aggressive. These symptoms were very frightening but none of the families reported receiving any useful advice to manage them, and some commented that professionals were unsympathetic when they asked for help.
Family 'He went to grab my arm ... he was right up very close into your face, very frightening to see this because his teeth were clenched and his eyes were terrible, staring very wide eyes staring. I thought this is the end. I've got to ring the doctors and ask someone to come round and the receptionist said there were only two doctors on that morning so she would ring me back ... The doctor rang and the doctor shouted at me on the phone. He said "What are you ringing me up for? You brought him round here yesterday".'
All the patients eventually needed help to feed, to wash and to dress. Some patients developed hydrophobia which made it even more difficult to wash them. Sometimes families wanted to give personal care themselves, rather than having a stranger do so, but one father was uncomfortable about having to help his grown up daughter to dress.
Family 'There was a couple of mornings when I was getting [patient] dressed and [nurse] came down and said, "Do you need a hand" and I said, "oh, no" because it was bad enough for her me doing it, never mind a stranger.'
Patients developed increasing problems with mobility and many families said this was the most difficult problem for them to deal with. They had to help the patient in and out of bed, to bath them, to walk them to the toilet.
Family 'It was getting worse. I had to take her to the toilet all the time, then I prepared the meals; I did everything because she couldn't do it. She couldn't wash, couldn't bath, she couldn't do these things so I did it.'
Family 'And she was starting to need help. I mean she needed help. We used to run her bath for her and she would go in her bath and go out. We used to actually help dress her really to be honest. She started needing help more or less with everything. Her walking, I mean she had to hold onto one of our arms.'
None of the respondents said they had had any advice on lifting and handling, but were left to work out for themselves how to manage. Some said they could have asked for this advice, but it hadn't occurred to them, they were busy coping with the practical problems as they arose.
Family 'And we managed to get him upstairs didn't we and we sort of got to the top of the stairs coming down and I looked at [sister], she looked at me and I looked at [patient] and we all just cracked up laughing because obviously he had to lean to hold the banister. Well, he didn't weigh much but I mean he was 5 foot 10 so we are not that big. I said, "Tell you what, it's a bum job. All of us on us bum" so we all ... I mean we all came down in a fit of giggles, downstairs.'
Family 'It took three of us to lift her out of bed and sit her up in an armchair for a little while so that she was sitting up. I had a bed raiser but she was sliding down the bed so it was difficult just to keep her propped up in bed so we was physically getting her out and lifting her out of bed and putting her in the chair.'
Family 'Yeah there was just no advice which way to do it at all. If we had asked the district nurses they would have told us, you know. That was one of the things we didn't ask. Basically, we were managing.'
Many reported carrying their relatives upstairs manually. One family had been told not to lift the patient upstairs, but there was no bed or bathroom downstairs, so they found this advice unhelpful.
Family 'I did, I hurt my back at one time and I said, "No, I'd sooner carry her up to the bathroom" ... It used to frighten her a bit because I used to put her on my shoulder and I used to carry her up, run the bath and put her in and she was all right once she got in.'
Family 'She says, "Right, you will either have to keep her in the bedroom or make a bed down stairs for her" and I said, "How can I do that with two young children in the house? Their life is disrupted enough ... so I have got to try to keep a life as much as possible ... normal for the children". "But you might hurt your back. I don't want you to do that." I says "I have got no option I have got to lift her up the stairs." "Right, but we will take no responsibility if you hurt your back".'
Some were given equipment such as a wheelchair, a hoist, a toilet seat or a handrail to help the patient move around. But 3 families had to borrow a wheelchair. Because patients were deteriorating so fast, some of the equipment arrived too late to be of use.
Family 'She had brought a zimmer frame but after a week that was totally out of the question because he couldn't walk...'
Family 'They gave her special forks knives with big handles on them to grip and she got a mug with two handles on it. And prior to that they had given her a stand to put the kettle on so it made it easier for her to pour the water to make a cup of tea. But she wouldn't have been able to take a cup tea out anyway, she wasn't able to walk unaided then anyway. So these things I know they were given with the best of intention but they were of very little use to her by that time, by the time she actually got them, because she wasn't really able to feed herself and things like that you know.'
When patients became more dependent, it was difficult to care for them in an ordinary bed and some families did get a hospital bed which made it much easier. Patients were restless and agitated and would fall out of bed unless there were cot sides.
Family 'Beds just aren't made to cope with the patient. You know like the hospital beds were great. You could have them as high or as low as you wanted you know. They lift and lower. That would have been marvellous just to save your back a bit.'
Family 'I had a little down stairs room and I put a mattress in the floor and I used to put [patient] to one side and I used to sleep next to her and we coped like that. And then I think after weeks the doctor came out and we got our [hospital] bed ... It had the side-rails but no fittings so it was useless.'
Patients became incontinent, were uncoordinated when eating and drooled. They needed changed several times a day. Families had to do a lot of laundry for these patients during the illness, most also bringing washing home daily when the patients were in hospital. None was offered laundry services, but some had a home help who did some ironing and this was found very helpful.
Family 'Trying to get bedding dry is a nightmare if you've got an incontinent person in the house. Even just being given a sort of a waterproof sheet. I had to go out and hunt to find a fitted waterproof sheet for the bed. When you really haven't got the time to do it. I actually had to take time and go and hunt for this sheet because otherwise the mattress. I can change the bed but I can't change the mattress every time.'
Family 'I used to have to fetch all - oh they stunk and all didn't they - you know all his underwear and towels and everything get it washed and quickly dried and back to him.'
Two families came into conflict with health services over incontinence pads. By this stage, the patients were totally dependent, doubly incontinent and being cared for at home, but pads were rationed.
Professional 'Sometimes at the case conference we would discuss incontinence for up to 2 hours. The Health Authority had a strict line on it, they were over budget and assessed her as only needing 3 pads a day.'
Two families moved house when the patient was terminally ill, one to be nearer extended family, the other because the house was unsuitable. In the latter case, it took a long time to get suitable housing, they were first offered a place that was too small for the family and eventually the social worker intervened to expedite a place. This family was also sent a bill for the ambulance that took the patient to the new house.
Family 'But it was so practically on top of each other, you know, it was just impossible to get organised properly. I needed a bigger place ... It was a specially equipped place, shower walk-in, walk-in wet room, you know and facilities to nurse [patient] which would have been brilliant ... She expected me to... sleep in the special room with [patient] every night in which case if I had have done that, I would have lost my nurses, night nurses so therefore I would have been back to square one, nursing her twenty-four hours a day on my own which was impossible. No-one can do that. I had to turn the place down.'
Where patients were cared for at home terminally, families were overwhelmed with visitors, both friends and professionals, coming into the home. While they were grateful that so many people cared, the volume of visitors was sometimes another added strain. One family used the hospice for respite care once a week so that people could visit the patient there.
Family 'The house was like Piccadilly Station with people coming and going. It was draining. It was absolutely exhausting. We went and got the answering machine then. We went and got the answering machine when [patient] came home because we couldn't cope with the phone. You were trying to do jobs and you were trying to look after [patient] and it was just exhausting.'
It was important for patients to have familiar people around them, so families felt it was unhelpful to have too many or unfamiliar professionals caring for them.
Family 'The different home help I would come home to find with [patient] in the afternoon, it would be a stranger to [patient] and it would be a stranger to me. I would come home and find her with someone I would consider as a stranger. I always felt that wouldn't be helpful to [patient], I felt that she always required to have a familiar face.'
One family was also very worried about confidentiality and concerned the diagnosis should not be widely discussed, in case the media heard of it.
Family 'I was also worried about the publicity ... I didn't want to be chased by any reporters. I said "I don't want it on computers ... I don't want it written it on any papers because I just don't trust how people can be". So we were worried about that bit too.'
Some patients were disturbed at night, and families would have liked night nurses to allow them some rest. Several respondents said they needed some arrangements for respite or some flexibility in the care, for times when the family had extra pressure.
Family 'I think a night nurse would be marvellous just so you could get a good night sleep. You're able to cope during the day if you get a night's sleep. I mean even now the least bit noise and I wake up and it takes us straight back to 3 years ago.'
Family 'I was absolutely exhausted. You see I couldn't sleep at night because ... I had my bedroom door opened in case she started to wander.'
Patients' needs progressed rapidly. They needed support and equipment to be provided quickly, and to be reassessed regularly. The professional advice and support available varied greatly. Professionals lacked information on the prognosis to help predict what support would be needed. Families were left to cope with the problems as they arose. Most did not know what support and equipment was available and needed advice before, or as soon as, it was needed.
Family 'But suddenly you couldn't get him to eat anything and suddenly you couldn't get him to drink ... To describe it as rapid is a bit of an understatement. It was sudden. Changes would happen very suddenly within a day. He had good days and bad days.'
Family 'Actually there were lots of things we weren't told about really, like silly things like, you know, those little cups, you know ... I got one of those from Boots myself but nobody said "Oh, you know, this might be a good idea because it's got a little spout and you'll find that you don't drop a cup of tea all over you". So more the little practical things actually. I was surprised that we weren't told about that.'
Families said they had to argue with authorities for the help they did get. They felt they needed a professional to do the fighting for them. Several respondents said they also thought there should be special funding for these patients, because they progressed rapidly to become very dependent, so needed support quickly. Two respondents said they were promised help that didn't come.
Family 'All the time it's money, money, money. It shouldn't boil down to money. In cases like [patient]'s, it doesn't matter if she's there for a day, a week, a month, a year, what she needed should have been there right from the start.'
Family 'They said this is what we can do for [patient] and you know this is the help and this and that. And they asked me what I could do but I was doing practically everything anyway, and ended up that I did do most things ... So I always felt that they didn't provide the care they stated they were going to provide.'
Many of the patients had been a main breadwinner. Other family members had to continue to work to pay the bills, while also caring for the patient. Some gave up work to care for the patient but, as one respondent explained, people who give up work to care for a patient are not automatically entitled to benefits. There were also other financial costs to families in travelling to hospitals, extra laundry, hospital meals, funeral costs and many others.
Family '"You won't be entitled to anything because you are giving up your job" .That was the answer. So I went up to the Social Security office, I filled the forms in still and they phoned us up didn't they. I had to go in for an appointment and they were explaining all to us and they says, "seeing that it is exceptional circumstances we are awarding you your benefit but you are very lucky to get it".'
Family 'Obviously with [patient] not working, I was having to ... so I was having to work quite long hours to try and cover everything. So I couldn't be at home looking after her, so it was I used to take her out and bring her home at weekends. I used to see her obviously every evening and bring her home at weekends.'
Family 'Hard as it may seem, you've still got things to pay. I don't mean credit cards. I mean gas, electric, your mortgage, stuff like that. You've still got them to pay and to be able to pay them you've got to go to work.'
Finding out about complicated benefits and filling in detailed forms was an added burden to families who were grieving and trying to care for their relative. Four families reported that they got professional advice on benefits, but one of these families only got help after the patient died, their benefits were stopped and they were left with no income. Eight families reported that they got no benefits advice. One respondent said that it was difficult to get benefits for a patient with no diagnosis. Two commented on problems caused when the patient was on income support but was too uncoordinated to sign the forms.
Family 'It gets very difficult when you get to know you're dealing with a terminal illness, filling those forms in. I know that they have a fast-track system but it's a very difficult thing to have to do at the time you're dealing with a terminal illness.'
Family '[Patient] had applied for ... income support ... and at the very beginning in [hospital] she could sign but as time went on she couldn't. So it meant her book was, I couldn't get any money.'
The financial burden of caring for patients was very heavy for most families. The costs of caring are the subject of a separate study.
Other practical help
Some respondents discussed their difficulties in dealing with day to day practical issues while caring for their dependent and dying relative. Most families were spending all their time with the patient, in hospital or at home, and had little time for ordinary activities like shopping and housework.
Family 'I didn't want to ever leave her. You had to squash everything up, I had carers for two hours. In that two hours in the afternoon once a week I had to go get the money, do the shopping, do what I had to do and it was like this all the time because you had to be, what's the time, what's the time, what's the time.'
Some families who looked after patients at home terminally had home helps who helped with everyday tasks, and this was greatly appreciated. They felt they could better care for the patient because they didn't have to spend time on these things. But some of the other families would also have appreciated this kind of help.
Family R1: 'I mean the number of times, it sounds stupid, I went to the supermarket and left my trolley because I just couldn't cope with people.'
R2: 'You got to have food in when you come back. You don't spend 24 hours so you've got to come back. So you come and there's no milk.'
R1: 'And the last thing you want to do is go to the shop and buy milk.'
Family 'Mostly it was the ironing because obviously with the amount of washing I had to do with [patient] there was a lot of ironing to do, I mean, sometimes we had to change the bed three or four time in an hour. So there was a lot of sheets.'
Family 'I kept washing and just piling everything and the ironing was huge and I started crying over the ironing. I mean of all things an ironing. I was crying because the ironing wasn't done. So ... this woman came and she came twice a week and did my ironing and she said to me "do you want anything else done" and I said "no". I was quite happy just getting that job. I could hoover, clean, do all that jobs.'
Eight of the patients had young children, and 2 others had siblings with young children. Two families got a nursery place for a pre-school child to allow the other parent to spend more time with the patient. But most families relied on friends to help with childcare. It was a dilemma for families who wanted to give extra time and attention to children who were losing a parent, but also wanted to spend time caring for the patient. This often meant the other parent making several trips a day between and home, school and hospital so they could spend time with children and patient.
Family 'It was just a case of me working the days when I worked, picking the children up straight from school to the hospital, spending a few hours there, coming home, sort the children out, getting them to bed, ready for school and that is how I existed all the time. On the week-ends my friend used to come with me so she would walk the children off or whatever but that was it. There was no help in any way.'
The need for extra childcare has not ended with the death of the patient. The remaining parents have either given up work or work part time to look after the children, or have the expense of professional childcare. Some feel they have to be both father and mother and try to make up to the children for the loss of one parent, as in this example.
Family 'I was over compensating. I was trying to make every day like Sunday, you know Sunday dinners every day and in the end like, I needed somebody to go, "Don't do this cleaning up if you don't want to do it. It will still be there tomorrow".'
Again it was important to families to try to keep things 'normal'. To some this meant keeping to the same routine, especially for any children in the family. To others this meant maintaining the same standards of housework. Keeping other things the same as before seemed to help the families to feel in control of the situation. But it often added to the stress, as they tried to juggle caring for the patient with maintaining their usual routines.
Family 'Its like your world is falling apart and you think, well I can hold on. I can keep a grip on my house. I can keep my house to the standard that's normal. That's not going to slip away as well. So its like, just little jobs that you need done just to keep yourself ... because there were lots of people coming and I was trying to keep it tidy and I was trying to do everything.'
Family '[Husband] went to the hospital all day and every day cause we were in the quandary of obviously needing to be with [patient] but also having the children to think about and wanting to keep everything as normal as possible for them.'
One family had a very supportive social worker who helped them with practical problems. But after the patient died he was no longer available to help them, because he was allocated to the patient not the family.
Family 'He was allocated to [patient], he wasn't allowed to be involved with the family any more. Which I think is all wrong because there are things coming up now appertaining to when she was still alive and things that [social worker] was dealing with and now they are coming up and I'm not allowed to get in touch with him to ask him to help me ... Things like filling out forms that was done when [patient] was alive and then you don't know what's put on them forms.'
Families and professionals agreed that nvCJD was a particularly devastating condition. Not only did they lose someone they loved, but the disease had caused progressive disintegration of the person they knew. Patients changed in personality, often become violent or aggressive, and then progressively and very rapidly lost bodily functions.
Family 'But it was also like, you loved this person but you hated this person because of what he'd become but you didn't know this other person.'
Family R1: 'Seeing a fit young person go from running about with her kids, carrying on with her kids...to being able to do nothing within the space of a couple of months - it's just devastating totally.'
R2: 'Yeah, I think, as I say, it's not even the death, because when the death comes if anything it's a relief because it's over.'
Family 'We were in grief for all the time that [patient] had been different. We ... had lost the person that we knew and we had somebody else that we still loved but it's a loss and it's a continual grief and it's having to re-align all the time your knowledge of the person that you got familiar with the day before and then losing them again because they have moved on to another stage. It is really hard, I mean the continual perpetual loss ...'
Neurologist 'Looking after a young person with terminal disease, watching her deteriorate, talking the nurses who were also seeing her deteriorate, explaining to her parents. The problems were legion and will also be so ... We watched her degenerate, it was awful for everyone. And it will be awful the next time.'
Uncertainty about the diagnosis, why it had happened and about how the condition would progress, was difficult for families. Professionals also found it difficult to manage a condition they were unfamiliar with, and to be unable to advise families on what would happen.
Family R1: 'We could handle it if she walked out and got knocked over and got killed, we could understand that.'
R2: 'Then you can think, oh well you know she's dead you know we know why she died and how she died she got knocked over by a bus. But this, you just can't get your head around it.'
Respondents said they felt guilty because in the early stages they had not understood that the patient was ill and had argued with them over their strange behaviour. Many also felt in some way responsible for the disease, particularly patients' mothers who thought they must have caused it by giving them contaminated meat.
Family '[Patient] was never a violent person. She was lashing out at me. It made me angry. Although she didn't do it under normal circumstances but it still made me angry because I didn't know why she was doing it. And I got angry with her and then once I found out what was the matter with her I got angry with myself because I had got angry with her because she didn't know what she was doing and I didn't know what was the matter with her and I was gutted that I'd got angry with her instead of cuddling her. Being patient with her. And that makes me very bitter.'
Family 'I came to live here and you were reproaching yourself in one sense. Well if I hadn't come here, we were going to get married any how wherever we were, but if I hadn't come here would [patient] still have been alive and you tended to blame yourself for that.'
Family 'What did I do wrong that my daughter's dying?...You have to get through that. You blame yourself and I did do for a long, long, time, you know, have I done that? I wanted to be punished. It was terrible, it was awful. I mean I was the person that was feeding her, so it was probably something that I had dished up to her. It is a dreadful thing and obviously I know that isn't true now but I did feel that for a long time.'
Respondents also felt anger. They were angry with the authorities who had allowed contaminated meat into the food chain. They were angry that they had not been told of the possibility that BSE could cause human disease, so they felt they had not had a real choice about whether to eat beef or not. They were angry that the condition was not detected earlier, and had been attributed to depression. This anger compounded their grief, and sometimes led them to mistrust the professionals who could offer support.
Family 'Great anger that [patient] had died unnecessarily from a manmade disease that could have been prevented and also very bitter because ... the thought will always be in my mind that if I'd heard about it sooner I would have stopped buying [beef] sooner.'
Professionals found it hard to develop rapport with the family when they were mistrustful or angry about care the patient had received early in the illness.
Neurologist 'They were angry about their daughter having something horrible, probably filled with complete gloom. The social interaction was the most difficult thing, stretched communication skills to the limit. There was a lot of anger, why should it be my daughter.'
The high media profile of the disease meant that families were continually reminded of what had happened. Television footage of affected patients was particularly distressing.
Family R1: 'And the grieving process is longer with this because you might get over it a bit then all of a sudden something will come back on the news in the papers.'
R2: 'I have been worse these last three weeks than I have all...'
R1: 'Because it's coming up to the run up of [the BSE Inquiry].'
Family 'To actually see her walking. Its identical to what [patient] was to see her when she was seriously ill in bed. You know it just brings it back home again. I find it very distressing to see these programmes. I mean initially some of the programmes I'd say were very, very good explaining to us but to see the victim ill I find it very distressing because it brings it back to home again.'
Sometimes families felt stigmatised. One respondent mentioned her distress that people made jokes about 'mad cow disease'.
Family 'I think people keep their distance a bit and I've noticed sometimes with the kids as well, you know, they're a bit, you know, when they find out, they're a bit, you know, "Keep away from them, you might get mad cows disease" sort of thing.'
Family 'And you get these silly jokes that start creeping in about mad cows and things you know which is so hurtful. They don't make jokes about cancer. But by God they make jokes about this you know. You see greeting cards with it on. It's so hurtful.'
Different families reacted in different ways and wanted different kinds of support. Most appreciated getting support from professionals and from friends. But some felt receiving sympathy from other people would make them less in control of their feelings. Male respondents in particular coped by seeking active things they could do, rather than talking about their feelings.
Family 'You shove it in a box and sit on it and then you next get something that you can't do you just shove it in that box again and sit on it and that's how it was really. There was no, things were happening so quickly there was no time. There was two choices you could either give in to it and then it would just becomes all consuming, you just, I wouldn't be any good to [patient], it wouldn't be any good to me, financially it would just be absolutely horrendous. So that was that really.'
Family 'Throughout [patient]'s illness I always felt the need to be in control of the situation as much as possible. And therefore when things like counselling was suggested I rejected it because I felt that I was in control. Which I wasn't, I felt that because I had done so much for [patient] ... I was under the delusion that I was in control.'
To some respondents, finding out as much information as possible about nvCJD was one way to gain some control.
Family 'They were all mighty sympathetic but sympathy, I am not the sort of person who wants sympathy. I am not into that. I just want somebody to come up with an answer. But as soon as I am told by [neurologist] then I really start to turn it over and I want to know the ins and outs of everything I can and I read every bit of documentation I possibly could get my hands on.'
Different members of the same family also reacted differently. Some families said they were so grief stricken individually that they could not support each other, so they needed professionals to do so.
Family 'On frequent occasions I would just lose it, and have to come out of the room, and you know pull me back together again, and just go back and start again. And when he was upset you know we couldn't help each other because we were both dying inside so, [the nurses] more or less pulled it together.'
Others made efforts not to show their grief in order not to upset other family members or the patient.
Family 'I think different people handle it in different ways. It's what ever gets you through really, it's very much a personal thing. But I know that [patient]'s parents helped me and I liked to think that in some sort of way I helped them. It was very much a joint being strong effort. It was always like you had to be because you'd be not letting the side down but it would just sort of becomes you know one person would get upset and then it doesn't do any good for anybody else.'
Family 'I built this terrific wall around the three of us that nothing but nothing would touch us and it didn't.'
Family 'I always put on a front. She never ever saw us cry. And anybody that came to visit us in [neurology unit] I used to say, "Don't get upset. If you can't face her don't come in. Just don't come in because I don't want her upset at all".'
Families commented that, because patients were young and the illness was high profile and much feared, the deaths affected a large number of people.
Family 'But you know there's lots of kids out there. You think its only one person, [patient], but it has affected a thousand.'
Often parents busy looking after their ill son or daughter had no time to spend with their other children. Yet siblings of patients were also in need of support. They were losing a brother or sister and also had the trauma of seeing them deteriorate. Some were concerned that they might be at risk of getting the disease too, or felt guilty that they had been spared.
Family 'You know his first reaction was, "It should been me. I've been here more years." How do you cope with that? And because of what it is, we've as a family eaten the same meals so you live with this horror every day that it could happen to another member of your family. You know I think every family is the same. You live with this constantly.'
Professional 'I felt if there was anything I neglected it was [brother]. His needs were pushed to one side to care for [patient], he had problems which maybe I would have liked to talk to them about but it all happened so quickly.'
Some of the families with young children reported that the children had developed behavioural problems. Most had not been offered counselling for them, or been given advice on how to help them.
During the illness
Four families had some formal counselling while the patient was ill. However, none of these had found it very helpful. They were very concerned with the practical problems of caring for the patient and found it hard to take time away and engage with the counselling. One family resented advice from the counsellor to take some respite from caring when they wanted to spend as much time as they could with their son while he was still alive.
Family '[Counsellor's] only thought seemed to be to get us to take time off from being with [patient]. She said we needed time for ourselves. We would breakdown and we wouldn't be able to look after him and even just the time we spent with her was, we couldn't wait to get back. We just had to be, we knew we were losing him. We knew we hadn't much time. We had to stay with him. So if the counsellor was, I wouldn't recommend it, not with that attitude any way. You know in this condition the time's so short. You want to spend every minute with the patient.'
Family '[Psychologist] came to the house and we spoke for a little while but at that time [patient] was not well. I couldn't get my head round talking about when she would die and things and I just thought, "Forget it I'm not ready for this" and I didn't want it and I never made another appointment.'
Most families were too busy caring for the patient, and too physically worn out, to actively seek emotional support for themselves.
Family 'Every day we put every ounce of anything we had was put into that day because we didn't know what the next day was going to bring ... and of course on top of [patient] ... there was the kids.'
Family 'Because when I used to come back from the hospital the reasons why we didn't phone other people was because I was emotionally drained and physically drained sitting in that hospital room twelve hours a day.'
Many families were helped by informal emotional support from friends. Some were worried about confidentiality and were more isolated because they didn't want the diagnosis known.
GP 'I think his mum felt on her own quite a bit, because of the concerns over publicity, didn't like to talk too much to friends or neighbours. So quite isolated. I think a very realistic worry if it had got out.'
Most families had been helped by informal support from professionals, particularly nurses and carers. They greatly valued this support. They often commented on the special attention and kindness they had received, and described staff as friends, not just professionals. Where one professional had taken on a key worker role, they usually also provided emotional support.
Family 'When you would go in they would sit down and make you a cup of tea and that and sit and have a chat and if you had been to see [patient] and you felt that you wanted to go for a chat after it, I mean they were more than willing, the time was there and you didn't feel like you were imposing at all.'
Family 'They were very, very supportive to the point where a few of them actually came to [patient]'s funeral and one of them has actually popped up and seen [patient]'s parents from time to time. Very, very nice people.'
Professional 'The nurses got very attached very quickly and were caring and supportive to the family ... Gave more than nursing care, very emotional with the family, 90% of them were at the funeral, 3 or 4 still visit and [patient's mother] still comes up to the ward.'
This support was viewed as different from formal counselling, although it is likely that some of these professionals were trained in counselling skills. The families found informal support much more helpful than formal counselling during the time they were caring for patients.
Five families recalled being offered bereavement counselling soon after the death but only one family had taken up this offer. They attended 2 or 3 group meetings at the hospice, which they thought were helpful. But after that they found meeting other families and talking to the media helped them to talk through their experiences.
Family 'The thing you see, afterwards there was a lot of media attention on television and I talked the story over so many times, I basically counselled myself. Not deliberately, but I lived it all so many times that eventually my grief was actually coming out.'
Several respondents said they did not want counselling after the death, because it was important to get on with things and not dwell on what had happened.
Family 'They wanted me to go or they would come out and all the rest of it. But I didn't want anybody ... I was just a nervous wreck you know but my nature being I'm a doer. I don't lie down to things.'
But some found that the pain got worse. Initially they were busy with work but then, later on, had more time to grieve. They were continually reminded of what had happened by media stories about nvCJD.
Family 'I mean, I feel it more now, believe it or not, than what I did then, because I had things to do. Now I haven't got things like to worry about as much.'
Family 'It's to do with getting into a routine and at first we were so busy trying to keep everything normal whereas now we are getting into that bit of a routine. We have got time to think.'
Four respondents sought counselling some time after the death, and most found it helpful. Several had also required treatment with anti-depressants.
Family 'I found the pain was increasing rather than going away so I did eventually take up the offer of counselling and I used to go once a week to a counsellor which ... just started about the same time as the press became involved ... because it was a time where I did need support because of what was happening.'
Families felt dropped by friends and professionals after the death, when all the support vanished and they were left alone. This was more marked when there had been a lot of emotional and practical support while the patient was ill.
Family 'I think after [patient] we were dropped didn't we ... Sad really. You've got into a routine of visiting the person in hospital and the minute the person dies what do you do the next day. Its like with [patient] it was quite funny we were lost ... We had no idea what we were going to do because we had got into the routine of going to hospital to see her and even the following week it was like that.'
Family 'But sometimes now I think now we're more in need of someone to speak to now. Because I mean when it was all happening, everybody is around you and everybody is so shocked and they're all wanting to support you and everything. But everybody has got on with their own lives. I mean I don't hold that against anybody. I mean I would have been the same. You start getting on with your own lives. I mean they can't all be round me all the time. So now we're sort of left and you think to yourself now maybe is the time that you could do with someone, now maybe where you could just phone up and talk to them.'
Family 'I think afterwards, maybe families need support because it is like it's all ... that's it it's finished, you know. The GP is still there for us and it's difficult to know, but it's like ... sometimes you've got 20 people in your house, you know, things going on, coming and going and suddenly ... you have to get on with it and everything's gone and you're shattered. It is just that, it's having a connection still, it's ... I know we wanted to get on with our lives but it just sort of, it feels ... it will never go away but I think there should maybe be some more support afterwards.'
Professional 'At the end its difficult because it all stops, one day all these people traipsing in, the next day nothing.'
Support from other families
Several respondents said they felt alone, that no one else understood how they felt about the illness and death of their loved one. Several said they wanted support from others with the same experience.
Family 'When its happening to you, you feel as if you're alone on the planet. Because you're told that there's this illness that nobody has seen before.'
Many families had found support from the CJD Support Network and in particular the Human BSE Foundation. There was a strong belief that no one could help except other families of sufferers. The condition was so different from other conditions that only people who had been through the same experience could understand. The families involved in the Human BSE Foundation had formed a very close bond because of their shared trauma.
Family 'We would definitely have found it a great help to talk to somebody else who'd been through it. We couldn't liken it to anything else. It was no good. Counsellors they weren't any good. We couldn't get on the same wavelength you know. We couldn't explain to her what we were going through and needed. And we've found since [patient] while we've been in contact with the other families that was the most help.'
Family 'Just somebody who had been through the same thing to talk to ... and that's what has helped with this families group. Knowing that there is other people. As we say it's not a group of people who has lost twenty four or twenty seven loved ones now. It's one family that has lost twenty seven loved ones you know.'
Most found that they could empathise with other families, they were able to cry with them and felt other families would not grow tired of hearing about their experience. Some had found it helped them with their own grief when they were able to help other families.
Family 'Just to have somebody else to cry with. I think when you meet as a group you do a lot of crying and that helps even the men. You have to. God knows you couldn't go through anything worse. And I think that is important that you can. As I say all the professional carers in the world can't replace the families who have been through, the sufferers. You know hearts are in the right place. I think nobody can ever understand it, the depth of despair that it creates.'
Family 'And you would never tire of each other talking. You would just listen to each other. Whereas I haven't felt it but I keep saying to [patient's father] in years to come our friends might be thinking, "Oh no not again". So they might get tired but where I don't think the likes of [another patient's mother] will ever get tired of me and I won't get tired of listening to her because we've got nothing else really.'
Working with the Network and the Foundation gave some respondents a focus, something they could channel their grief and anger into,
Family 'I think being involved with all the families and being involved in the Network and Foundation has helped a bit. It has helped me. Its given me some kind of focus you know and our push for the Inquiry. We would never have got this Inquiry had we not pushed.'
But other families found it distressing to meet other families or visit other patients. It was harrowing to hear their experiences and brought back the memories of their own grief.
Family 'I didn't think that continually revisiting our situation, our experience of it and the families who would have similar experiences, would be helpful to me. I wanted to try and put things in the past, to move on, and I thought that it would not be beneficial to myself to talk about that. When I read a recent statement I found it very harrowing. It was yet another story of extreme tragedy and it was very upsetting to read it, so I don't know whether or not I would like to become involved in that way in, you know, going over these things again and again.'
Family 'Everybody's talking about the person they've lost and the thing so it all brings it back. Not that I forget about it but I get too, it gets me a bit emotional so I don't really want to be there talking to everybody else about it if you know what I mean. I shall never forget her, forget [patient] or forget what happened to her but when you're all together there it just wells, you know, it brings too many memories back. I can cope with it like this. But when you start talking about it, it brings too much back.'
Some respondents felt guilty burdening other families with their own sorrow, knowing it reminded them of their own grief.
Family 'I rang them quite a few times. But to be quite honest with you, although ... I go up and I go down but I don't ring them, because I feel that ... I know its easier to talk to them because they've gone through it, but I don't like to ring them when I feel down. Because I feel it's not fair because they've gone through it themselves, you know.'
Family 'It brought it all back to her and then I felt horrible because she had come down ... do you know what I'm trying to say? It's terrible really. I was so glad she'd come down though. I needed to talk to her.'
One professional commented that it was important to be sure about the diagnosis before telling the family because it was such a devastating condition with a hopeless prognosis. But this meant they were left without the support of other families before the diagnosis was more certain.
Support from professionals
Some respondents said they had had no support from their GP. But where GPs had offered emotional support, families found it helpful. They particularly appreciated GPs who made time for them and didn't distance themselves from the family's grief.
Family 'He was wonderful. He used to call round and he would sit and have a coffee with me. He was sitting crying there. He was wonderful, absolutely wonderful ... He wasn't a doctor. He was just a human. He was just lovely. Wonderful to us.'
Many GPs said that emotional support was the most important help they could offer in these circumstances,
GP 'The most important thing I can do to support a patient is to put an arm around them and say, "Cry here".'
But sometimes the family had lost confidence in the GP early on, when the patient was given a psychiatric diagnosis. And others thought the GP was too busy to offer the intensity of the support they needed.
Family 'We sort of felt [GP] fell out of favour with us because of the fact that she told [patient] to go away and pull himself together. At that point we saw that as the start of all the problems and good God if she can't get that right then we're not interested. So we changed allegiance and I then saw [another GP].'
Family 'Well when we've been to the GPs about different things she sort of said, "How are you managing, are you all right". That's it. If somebody says are you all right you just say, "yes". They've got 10 minutes you can't say, "Now sit down and when you've got 2 hours we'll go through it".'
Some families thought the neurologist or GP was indifferent. They were upset when the GP or neurologist didn't say they were sorry about the patient's illness, or ask after them.
Family 'Just enquire after his health. They didn't even enquire after his health.'
Many families built relationships with one professional, who was then able to understand the kind of support they needed. This could be a nurse, a social worker, the GP or the neurologist. Often this professional also coordinated the practical support and provided information, acting as key worker. In other cases the family developed friendships with and got emotional support from several of the staff.
Family 'She devoted far more of her time to me and my family and [patient]'s death than she really ought to have from her working schedule. That was why she became a friend. She was at his bedside when he died. You can't really ask much more than that from a professional.'
Professional 'My role developed to include day to day support by phone or visits, I'd see them to talk for an hour 3 times per week if necessary ... pull out all the stops and respond to needs as required. It was time consuming, it took 7-10 hours of my time a week in calls, visits, liaison. So on an informal basis they got support and counselling from myself.'
All the families who had input from palliative care professionals were grateful for their support. Where patients were nursed in a hospice the staff were very sensitive to the families' needs and provided support for each patient and family that met their individual needs. Professionals also commented that hospices were well equipped to deal with emotional needs, but some only accepted patients with cancer.
Family 'We asked if [patient] could go to the hospice. "No he doesn't have cancer. Can't go there". Can we see the Macmillan nurses? "No he doesn't have cancer you can't have anything to do with them". So we had nobody.'
After the death, families still needed emotional support, and the GP was often the professional the family could still contact easily. But one respondent said she didn't want to seek support from the GP because she needed reassurance that her feelings of grief were normal, not to feel she was ill. Another felt unable to visit the doctor's surgery after the patient's death because it held bad memories.
Family 'The GP's been good. But I don't always want to feel that it's a sickness. Having had so much sickness and illness and having this sickness destroying our lives it is just like ... you need something actually to help you keep going, you know. It's all right. That's how people feel, you know. That's actually an understandable feeling.'
Family 'I've never gone back to the doctors surgery and I can't go back. I don't feel I could walk in there. If there's anything wrong with me I don't know how I'm going to get to that surgery, if I have to walk in there again.'
Support for professionals
A striking finding from the interviews with professionals was that they also needed emotional support when looking after these patients and their families. The intensity of their involvement meant they were often deeply affected by it. Those with the closest involvement, especially nurses and carers, were most affected. Where palliative care services were involved, support for the professionals was already available. But in most other cases it was not.
Psychiatrist 'It did affect the staff looking after him, he was deteriorating in front of our eyes and there was nothing we could do to stop that. I'm not sure we did much to support the staff with that, I did speak to them and tried to support them as much as possible.'
Professional 'They were upset about it. There were photos of her when she was well, though they never knew her when she when was well, and we are used to terminal conditions ... We are acutely aware of the problem and support each other.'
Professional 'The nurses had to be counselled afterwards. Group counselling by the Macmillan nurse, just to talk it out, not the first time we've availed ourselves of that service.'
Professional 'Psychological needs, we all have these needs, the emotional side of it is all very draining, people tell you not to but you do get involved. You should learn to stand back but you can't help it. I've been involved in a few cases but this got the closest to my heart. So we should offer counselling to the carers. They get very closely involved, see them every day then suddenly they are gone.'
Some professionals commented on their own feelings of helplessness, that they had no interventions to offer to cure or alleviate the disease. This was compounded by their own feelings of uncertainty. They were unfamiliar with the condition and could not advise the family how it would progress.
Neurologist 'Here's something that can't be cured, its difficult to watch someone die and you can't to anything about it, there are parallels with motor neurone disease. You can offer no cure, I find that very difficult personally ... I'm sure it's the same for most neurologists, a feeling of impotence.'
Professionals who had children themselves often empathised with the parents of patients. This made it even more distressing for them.
Neurologist 'You think, "If that was my child." ... Maybe if I hadn't had kids it might not have touched me in the same way. My kids have eaten beef. We are all at risk I know.'
Professional 'Its stressful. I had a son the same age, how would I be feeling. I found this quite hard.'
One respondent commented that it was an additional stress to the family when professionals were visibly distressed. This reinforces how important it is to ensure professionals are equipped to deal with these distressing cases, which means emotional support should be available for them.
Family 'I was aware of [professional's] distress actually. She was ... quite traumatised by the whole situation. So as I said, the training for professionals is really important. They have got to know what they are facing because I picked it up. I've got to deal with my stress. I also was aware of hers. So it is actually doubly stressful.'
From the GP
Several families were unhappy with information from the GP before diagnosis. They felt they had been misled into thinking it was psychiatric problem, and then lost confidence in the GP's ability to help them. Some of the GPs wanted more information about early features to look out for, but others said it was too rare for guidelines for GPs to be useful.
GP 'How many patients do you see with anxiety and depression and personality change? I'm seeing them daily ... If they become neurologically unwell I would refer. The GP's job is to refer. It's extremely unlikely a GP would be making this diagnosis.'
None of the families identified the GP as a source of information after diagnosis, because the GP was unfamiliar with the condition. The GPs interviewed said that patients often use them to check what they have been told by the hospital consultants. However, they knew little about this condition. Several expressed their anxiety that they could not answer questions about it, especially how it would progress.
GP 'I'd have felt inept if they'd asked me about the disease process. The cause, at least you can say well nobody knows really, how infectious is it, baseline things really. You get a lot of information in the general press. Nothing too scientific, just basic information.'
About initial psychiatric diagnosis
Most patients were given a psychiatric diagnosis initially, and many families were angry about this. To many, psychiatric illnesses were not 'real' diseases. Some said a diagnosis of depression didn't make sense, because the patient denied feeling depressed or had other symptoms. Dementia also didn't make sense because it was a diagnosis given to older people. Families' understandings of these diagnoses didn't fit with their observations of the patient.
Family 'I knew that [patient] was never, never a depressive person ... I knew she wasn't, she wasn't suffering from depression as the root of the illness. That wasn't her illness, it was only the depression became a symptom of her illness. There was just no way, the type of person she was.'
Family 'He said it was dementia. Now can you imagine me sitting there in this waiting room and him telling me this. My daughter's got dementia. I couldn't, I'm telling you, I just couldn't understand. But he knew what he meant by dementia but nobody ever told me, no.'
These perceptions about psychiatric diagnoses made relationships with psychiatrists, GPs or others involved at that time difficult. Families had more confidence in those psychiatrists who said it was an organic problem early on and supported the family in seeking neurological investigation. But most psychiatrists did not feel their own input was inappropriate. As one commented,
Psychiatrist 'One of the problems is that people say nvCJD is often initially mistaken for a psychiatric condition, but dementia is a psychiatric condition. It happens to have a more noticeable aetiology than some psychiatric conditions.'
Families wanted to know what investigations were planned, what they involved, what they were looking for and the results.
Most of the families said they had been told what investigations the patient was having. But sometimes they were told about them in jargon. Several reported checking what the tests were with friends who were health care workers, or looking them up in dictionaries.
Family 'But nobody even said to us what an MRI was. Nobody, they would use these big long terms and we would come home and look in the dictionary.'
Family R1: 'We've got a friend that works for [hospital], he actually works in the labs and he explained most of the tests to us anyway.'
R2: 'So if we didn't understand we asked him and he explained in every day talk, like none of the big words.'
Family 'He said he'd rung for the results from the [hospital] and it was serious and I said "Well, what is it?" and he said this long word, very long word which I didn't know".'
Few felt they were given enough information about what the investigations entailed. Many commented on this, saying they might have been able to help overcome some of the practical problems. They felt the professionals didn't listen to their observations of the patient's behaviour and mental state. For example, MRI scans were frightening for patients and families thought they could have helped by accompanying the patient when they had the scan.
Family 'Because I actually went down with [patient] for the MRI scan, the first one and she wouldn't react so they couldn't do it. And the next day they took her down and gave her a sedative and I would stay and they put her inside, didn't explain anything to [patient] what was going to happen, and I actually got a shock when the machine started so I wouldn't have liked to have been inside the machine when it started and I was sitting outside. And they didn't explain any of this to [patient] nor me what was going to happen when the machine started so they done this test she still didn't lie still properly they didn't get a full one done did they?'
Most patients were not competent to consent to procedures, and families consented for them. They wanted to debate the pros and cons of individual investigations, but only brain or tonsil biopsy was actively discussed so they had a real choice whether to consent. One respondent reported that the patient consented to the brain biopsy, although he cannot have fully understood what he was signing. Two families felt pressurised to agree to biopsy. Three said they felt their relative was being used as a guinea pig; they thought the investigations were for scientific interest rather than their own benefit.
Family 'And [neurologist] asked us to have a word with us again so I went into his room and it was about the brain biopsy again and I said no again. And he says, "From a scientific point of view it would be good to do". And I thought no way are you using [patient] as a guinea pig.'
Family R1: 'I think they tried to explain about his biopsy, he had to sign didn't he?'
R2: '[Patient] had to sign for his brain biopsy.'
R1: 'I don't think he understood did he.'
R2: 'No, I mean he signed but he couldn't actually know what he had signed.'
Some of the patients were presented at a grand round. Two of the families had accompanied them to this. They found it a daunting experience and they were unprepared for it.
Many families were told the investigations were eliminating possible diagnoses, but many were not told what possible diagnoses were being considered, and some were told in jargon. Only 2 of the families said they were told that they were looking for nvCJD. Many were angry they had not been told the possible diagnoses earlier, and perceived that the possibility of nvCJD had been deliberately kept from them. One family first heard the possibility of CJD when their son was in recovery after a brain biopsy and they saw '?CJD' on the operation sheet.
Family 'Except for the brain biopsy. It was explained to us what it would be and the dangers, the pros and the cons but they felt, we went with them, that they felt this was the only solution to perhaps get a diagnosis. But probably the most critical factor was they didn't say what they were looking for and ... the first time we saw the letters CJD were on his operating notes.'
Family R1: 'They would not give anything away you know. I think deep down they knew it was new variant CJD but I think they were duty bound to put [patient] through all these tests.'
R2: 'To eliminate everything ... I think they were trying to throw us off the scent really.'
Family 'You know he referred to his text book of dystrophies. We didn't know what the words meant. Some of the Latin, Greek, you name it all foreign to us but then he mentioned two or three types of possible dystrophies. We then had to go back to the library and try and find out.'
One family vividly described how it was unbearable for them to wait for results of investigations,
Family R1: 'We said we'll take him up in the morning and they'll probably have the results by 10 or 11.'
R2: 'By 11 o'clock so we sat and then 11 o'clock came and 12 o'clock came.'
R1: 'We were in a side ward right next to the nurses station where they come in and look at the x-rays and things.'
R2: 'We waited and we just waited and waited.'
R1: 'Had some dinner and still no results.'
R2: 'And it was about 5 o'clock wasn't it? It was 5 o'clock and the neurologist just sort of breezed by, I'd gone for some sandwiches or something and he caught [patient's father] and I think you'd broken down hadn't you?'
R1: 'Just about.'
R2: 'And he said, "Oh the result of the MRI scan is fine. There's nothing on it". By this time we were, I can't describe what it was like. It was just a total nightmare.'
It didn't make sense to families that the investigations could be normal, when it was obvious that the patient was not normal. Several described feeling relief at normal results that excluded some conditions, but then realising that this left more serious possibilities.
Family 'All we were being told at the moment was that everything was normal and we could see nothing was normal.'
Family 'And he just said it was perfectly all right. Nothing there at all. As I say at first that was a relief and then when we came home we realised it wasn't because had it been a tumour it was something they could do something about but if nothing was showing up on the scan we realised we had a big problem.'
Three families said they had to demand to hear the results of investigations.
Family 'So we demanded that we saw [neurologist], who was very, not dismissive, but wasn't very forthcoming, said they hadn't come up with any diagnosis. They were still waiting for one or two tests and they would let us know, one of them being HIV.'
Family 'They told me about the AIDS one obviously because they asked my permission to do it. I asked what was the result because I just wanted to know. It came back negative. They done the test for Huntington's, something like that, because that's hereditary for the children. They never did tell me the result of that. All I know there was loads and loads of blood tests that they done and they just kept giving him pills.'
Some families were distressed when they were told expensive equipment would have to be discarded because it had been used on a patient with nvCJD. This made them feel stigmatised.
Family 'They had to wait for the man, the special person, coming to do it and she knocked it [PEG] out. So that was the end of [patient]. I said what do you mean. "Well that machine costs £20,000 and because of your daughter's illness its got to be thrown away because its so deadly the CJD".'
Perhaps because families did not feel they were kept informed of all the investigations patients were having, they sometimes assumed things had been done when they had not. One family assumed there were mechanisms for networking widely to identify other cases of unusual conditions. They were angry that the diagnosis was missed although there had been other cases at the same time.
Family 'And we presumed, the doctor sort of said he'd consulted as far as he could and nobody had ever seen it before. Well we presumed hospitals were all on a computer system that the secretary went to the computer, printed in [patient]'s symptoms and broadcast it all around the neurological hospitals in the country and people came back and said yes we've seen it or we haven't. So when he came back to us and said he'd asked everybody, we presumed this had been done.'
About the diagnosis
In some cases, especially the earlier ones, CJD was considered but thought unlikely until it was confirmed at post mortem. In others, families were told that CJD or nvCJD was the most likely diagnosis. Five patients had the diagnosis confirmed by brain biopsy. Uncertainty over the diagnosis was stressful and frustrating for families and many thought professionals were reluctant to state that it was nvCJD because it was being deliberately covered up.
Family 'Personally I think they were trying to hide it from us all the time you know'
Family 'We as a family can't accept that it had gone so far before we got them to admit that they were possibly looking at CJD ... That just doesn't make sense and has never made sense to us and we don't accept it. I don't think yet any of us accept that. I don't think they were lying. I think they were just, I don't know why, they were holding back. Maybe this could be the politics of it maybe, I don't know.'
Seven families recalled raising the possibility of nvCJD before the doctors did. Often the doctors then confirmed that it was one of the diagnoses being considered. This exacerbated families' perception that it was being covered up, that the doctors were reluctant to tell them although they already knew what it was.
Family 'It was just a little bit in the paper and it mentioned, like CJD. So we got down to the hospital for this case conference and we brought it up ... And then the doctor said "Well, we can't rule it out" ... so it seemed to me as if the cat was out of the bag, like when we had mentioned it, the cat was out of the bag.'
Several families were confused and angry that they were given conflicting information. In 4 cases, the professionals disagreed early on, and families were told it was nvCJD by one doctor, only to be told it was not by another. This was stressful for them. Some thought it was because it was being covered up. But one respondent was upset by a doctor who talked as if the patient had nvCJD before she had been told that it was definite.
Family 'I said, "One minute they are telling me he has got it and he's dying, the next minute there's another fellow saying it might not be". I said, "I just don't know where I am".'
Family 'When he was talking to me he was talking as if he knew she had it but he hadn't done tests and I was convinced that he didn't know she had it because she didn't have. So I didn't really gel with him very well because I just didn't like him because he was saying she had it and I just didn't think she had it.'
Many families were unhappy with the way in which they were finally told the diagnosis, or told that the condition was terminal. Some were told in jargon. Many said they were told in an unsympathetic way. Sometimes one member of the family was told when they were alone. No one offered to phone other family members for them and they were not offered support by the professionals. Few families recalled being given adequate time by the doctor who explained the diagnosis to them. Most said their minds went blank when they were first told, it was hard to take in the implications. They often wanted to ask questions later, when the diagnosis had sunk in, but most were not given anyone to contact. Some were told that they would get more information from the research registrar from Edinburgh, but that left them with nowhere to seek support meantime.
Family '"I've got to tell you I think [patient] has a progressive, degenerative neurological illness". And we sort of stood there and that didn't really mean an awful lot.'
Family R1: 'He never once said "I'm sorry".'
R2: 'I remember that. He didn't say "I'm sorry, but I'm afraid to tell you".'
R1: 'He never said that. He just said "I've got to tell you that we believe". Basically as soon as we sat down, "we've got all the test results back and I'm afraid we believe it's new variant CJD. It's either that or a measles virus which is also fatal". And that's basically how he told us.'
Family 'This particular day I walked up on the ward and the nurse looked at me and she said, "The doctors want to see you". So I was there totally on my own. I had no support from no family, no nothing. They didn't try to get hold of anybody to be there for me, to help me get through it, they just hit me with it, you know. This is what she's got, this is the prognosis and this is what to expect and I just went to pieces. The doctors left the room, the nurse made me a cup of tea and she went and left me on my own which I know she couldn't sit with me, I mean what can they do, they can't comfort you but there was no, "Can I get hold of your family for you. Is there anybody I can ring to get up here?" There was none of that.'
Family 'He said "It's all negative, all negative, all the tests have come back negative". I said, "What does that mean?" I said, "We know that something terribly wrong is happening with [patient]". He said "Well we are looking at possibly HIV or CJD" and he said "I think it is probably CJD" and I was gobsmacked. I was on my own and I said "Are you telling me my daughter is going to die?" although in my heart of hearts, you know, I guessed the sort of realisation of that anyway, but it was like being told in the most horrendous way, you know, possible ... and he said, "It's dreadful for the families", he said, "She won't feel a thing. Awful for the families". And that was how I learnt that [patient] had new variant CJD ... I remember, it is the flashbacks of that time, because I remember coming out of his office and going towards her bed and funnily enough the two friends who were there also have the flashback of my face, you know, they still have that memory of how I looked at that time and I said "I must go". "Are you all right?" and I just kissed [patient] goodbye and I went in the car and it was just a blur. I remember going down the motorway and all the lights flashing. It was just awful, terrible.'
Although most families were unhappy with the way they were told the diagnosis, a few said they had enough support available and felt they had had time to ask questions. These were invariably families who already had a good rapport with the neurologist.
Family R1: '[Neurologist] spent time with us as well at the time, I mean, obviously he didn't stay too long because he was letting us alone to let us digest what was going on. But he must have spent, I don't know, I don't know how long but he was willing to be there as long as we wanted him to be there.'
R2: 'I felt that we had so much support ... They all made a point of saying that we had, you know, if we wanted to speak to anybody at any time they were there.'
Some respondents described a need for support to tell the rest of the family the diagnosis. Some of the patients had some insight into their condition, and asked their family about it. How to respond to this posed a dilemma to families. None of the families had told the patient they were going to die, but one respondent described feeling guilty about this,
Family 'He said "You know what's wrong with me". I said "You know, you've got a brain disease". "Am I going to get better?" and I just had to tell him he was.'
Family 'She did say to me one day when one of her friends was coming round ... "Well does she know my situation?". I said "What do you mean, what sort of situation are you talking about?". She said, "Well does she know I'm going to die?". So that was a tremendous insight there ... but we don't know, she didn't say "Why am I going to die?".'
Family 'She asked me, "Am I going to die?" So I said to her, "Well to be honest with you I really don't know, they can't tell me, they can't me you know what the outlook's for you. They are unsure because they have no diagnosis for it now". I said that in all the best, in all good faith at the time ... I feel slightly guilty for that because obviously it is very difficult to tell someone that you know that they are going to die.'
Families wanted more information about the condition. They wanted to know what had caused it, especially as many felt it was somehow their fault. Many wanted to understand the pathology, what was happening to the brain. There was concern about the risk of transmission to other people and about the genetic risk to other family members.
Family 'And there was another question about, he at that time said it was a faulty gene. Now that has bothered me very much. To me a faulty gene, to me that is the logical explanation because we all ate the same dinners ... I thought that was the most logical explanation but that has been scrubbed now hasn't it? They don't believe that any more that it is a faulty gene or do they?'
Family 'You know we weren't aware. You know obviously there is a risk factor of handling a patient with nvCJD. It would have been better to have known what risks there were before and after but no-one knew did they because its so new. And things like that and [patient] used to soil her clothes a lot, we handled a lot of that, washed it ourselves. You know the contamination risk of that. Because in hospitals they just dispose of it don't they?'
Family 'What's the risk of me having it? If we have eaten the same contaminated food or been at risk of the same possible source what's the chance of me going down, what's the chance of my father going down with it. Because you know in reality you know if you've been susceptible or suspect, you know could have been at risk, there's a chance of any of us picking it.'
But some respondents were too shocked initially to take in any information about the disease, and would have found too much information early on an added burden.
Family 'At the time I don't know that I could have taken anything in. It was all that I could do just to continue normally. Alright for some people it may be a sort of way of sorting it out actually reading about it or finding out about it. All I knew was that [patient] was laying there and she was dying from something and it didn't, you know you could call it whatever you liked, it was just that. It was only really afterwards that I was, you know, left it a while and then sort of started finding little bits more about it.'
A neurological illness was particularly difficult for families to understand. Many felt they had some understanding of other diseases but neurology was an unfamiliar area. They found it difficult and distressing to imagine disease affecting the brain of someone they loved.
Family 'We've got a reasonable knowledge. But then you start venturing into areas you know nothing about and when neurology was mentioned I didn't know what neurology was. It had to be explained as the organic function of the brain.'
Family 'Because its hard enough to handle maybe without knowing what's actually happening to the brain. The thought of flaws in the brain, it's not a nice thing to contemplate.'
To one respondent, nvCJD didn't make sense as a diagnosis because it meant the patient must have eaten bad meat, and the family always ate good quality meat.
Family 'In my head it was just the burger thing ... I understood it, it was if you'd eaten bad beef. If you'd been eating cheap meat, tins of meat or things like that which we hadn't and it didn't make sense. I just couldn't believe it would be that.'
Four families recalled being told not to contact the media when they were told the diagnosis. They thought this was yet another example of professionals trying to cover up the extent of the disease.
Family 'He says, "By the way don't go to the press because they will make your lives hell". I think he had been told from higher up, you know, make sure these people don't go to the press.'
The perception of a cover up is so strong among the families that two respondents spontaneously commented during the interview that they did not feel anything was hidden from them.
Family 'Because, as we said, I mean, they were always open. I mean I don't think we ever got the impression that they were hiding things.'
It was important for the neurologist to develop a good rapport with the family, to communicate the uncertainties and later to tell them the diagnosis in a sympathetic way. Some of the families described a good rapport with the neurologist, and were happy with the information they received. Unfortunately, other neurologists were seen as being elitist, which placed a barrier between them and their patients. In these cases, families were unhappy with the information they were given and the way in which it was given.
Family 'Okay they were sort of showing us, you know, saying we've done tests but it was like they were removed. They, as neurologists, they were sort of, you know, you were elitist ... It was like, "Don't take up my time", you know, "I'm too busy, I'm too busy, I'm far too important".'
Many of the families said what they would value most from neurologists was 'straight talking'. Some thought that the professionals were embarrassed by their own lack of knowledge and this inhibited communication with the families.
Family 'I think just honesty. If he had just said, "Well I've never come across this before and its difficult for me but you know, I'll give you what support I can", at least you'd feel you know where you are and someone's being honest with you. But I think he was embarrassed by his lack of knowledge and frustrated by it and it made him brusque.'
Family 'The one thing I want when people are going to talk to me and tell me, nothing, don't waffle me, just hit me smack in the face with whatever it might be. And then I can accept it. If you go around the houses then I am afraid I don't believe all that rubbish. I just want, you know doesn't matter how traumatic in the end result going to be, give it to me from day one I can cope with it. You start waffling me and I don't I can't cope with that So [neurologist] assured me within ten days he would have the answer.'
About prognosis and care
Families wanted to know how the disease would progress, both to help them prepare emotionally and to allow them to plan how to care for the patient.
Family R1: 'If we had know more what was going to happen, prepare yourself for what was going happen because it was quite dreadful to watch him.'
R2: 'And also I think if you know what to expect you can start and discuss, well look at options of care, can't you? If you know what you are going to be dealing with, you know if you are harbouring false hopes about looking after somebody at home.'
Often the poor prognosis was apparent to professionals before any diagnosis was reached. One family described their bewilderment when their son's symptoms were ascribed to 'his condition' but the condition did not have a name.
Family 'Didn't get any explanation for it at all. "Just a symptom of the condition" which was mind-boggling because I didn't know what, and I thought how do they know its a symptom if they don't know what this condition is you know. That was the only answer we were getting was, "It's to do with the illness" but nobody knew what the illness was.'
It made no sense to families that their relative could die of something with no name.
Family R1: 'We tackled him again. Sometimes people do die of things that are unknown.'
R2: 'Got to have a name.'
R1: 'I couldn't understand that. I said even like the elephant man, the film of the elephant man, so long ago and they still had a name for that and it was so rare.'
Three families reported that the first person to tell them the patient was going to die was a junior doctor they had not met before. This was when the patient was very dependent but before the diagnosis was made. They were understandably shocked and felt a more senior and familiar doctor should have explained this to them.
Family 'I had been asked to come and speak to a doctor .. and this young person, we asked her if she had had any more information ... She said no, but she told us then, "You know, you're going to lose him and you better prepare yourself for it". You know, she was quite blunt about it ... Of course I just went to pieces and we were angry the way she had done it. Because we felt that she wasn't the one to tell us that if that was the case ... She didn't know anything about it. She had never been, to my knowledge anyway, she had never been involved.'
Family 'And then one of the junior doctors said, "Can I have a word with you" so I said "Yes, what's up?" He said "[Patient] is going to die, you know" so I said "What do you mean, she's going to die?" I said, "You don't even know what's up with her yet". So he says, "Well, she's in a very bad way".'
Family R1: 'The doctor came to see her, the house doctor wasn't it, she came out and said, "Can I have a word with you" took us into the office and at this point she hadn't even read [patient]'s records from [neurology unit] turns around and says..'
R2: '"I don't know if anybody has been this blunt with you before but of course you know people in [patient]'s position if the heart stops we don't resuscitate them and she is going to die".'
Families often felt overwhelmed by the rapidity of patient's decline. They wanted continuing information about what was happening as new problems developed. They wanted professionals to pro-actively offer advice and information, not just to be available to answer questions.
Family 'It was frightening because we were discovering these things. There was nobody there to say, "Well, you might find this after a period or this after a period" and for some kind of system where you can prepare yourself for it. But suddenly you couldn't get him to eat anything and suddenly you couldn't get him to drink and suddenly you couldn't ... We had no idea what to expect at all. It just seemed to be in free fall and we were just watching this and responding to it and we didn't, had no idea.'
Family 'If somebody had sat us down and said, "Look, eventually you're not going to be able to feed [patient] so we'll have to put some kind of strategy in place for dealing with that. You're not going to be able to lift him to bath him. You're not going to be able", even that I think might have helped but we had nothing like that at all.'
Often families found it difficult to know what to ask. They were too busy dealing with problems as they presented to ask about potential problems that might arise. Respondents who were working and only able to visit the hospital in the evenings had no opportunity to ask questions.
Family 'I would be there sometimes when they did their rounds and you know they would say bits to you and but there was never a time when they said, "look, you know, what is there anything you would like to know". It was all quite vague really.'
Family 'And then he kept saying, "Is there any questions you want to ask?" and like you just go, "I don't know" and like is there something I should be asking. But they won't tell you, not unless you ask.'
Some said they were not kept well enough informed about what happened to patients while in hospital.
Family 'Apparently he went blue one day and they laid him all down and everything else but he recovered. I didn't know about this until [relatives] started having a go thinking that I knew but never told them and I didn't know nothing about it.'
Practical advice was valued by families. One family was grateful for advice to be 'parents not nurses' in caring for their son. Many families said the most useful information about how the condition might progress, and how to care for the patient, came from other families. They thought people who had seen the disease first hand were best placed to offer advice. Often fairly simple advice on how to manage symptoms, or how long they might continue, was very helpful. It was also helpful to families just to talk to others who had experienced the same problems.
Family 'The doctors couldn't tell us anything. They couldn't tell us what was going to happen, how long it was going to be, what would happen next. They couldn't tell us because they just didn't know and [other families] were the only people we could get any advice from. So I could say "well [patient's mother] said this is what [patient] did and this is what happened next".'
Family 'Yes, and also they helped us in different ways because [patient] was losing a lot of saliva, continually he was soaking wet through with it and we couldn't keep pace with getting dry pieces of cloth and [other patient's mother] told me that, she said that will stop which it did ... They could say, well, they'd experienced that or they hadn't but if they hadn't experienced it then she could put you in touch with someone else that she knew that had gone through that.'
Families wanted to know if the patient was suffering any pain. Most were reassured that the patient was unaware of pain, but one disbelieved this reassurance.
Family 'When he was having those spasms, you know when he was stretching out like that and it was quite worrying to watch and you felt that he must be in pain or something and [nurse] could explain that that was a symptom of a brain injury so your worries were alleviated and she was very supportive.'
Family 'I said to them more than once that I feel that he's in pain and they kept saying, "No, no, no, that's muscular spasms in the back and all the rest of it but he's not in pain". It took a nurse in the end ... she said, "He is because otherwise he wouldn't be hunching" and ... she felt that he needed morphine.'
They wanted to know how long the patient would live, and were frustrated that doctors would not tell them a more precise prognosis. But where families were told what the timescale might be, it often differed from what actually happened and they felt misled. Some were also upset at being given conflicting information about the possible timescale.
Family 'And he wouldn't say, would he? He was just saying all the "We can't possibly know, we can't possibly know". And I was saying, "If this man is saying it's going to be days, are we looking at this kind of timescale or are we still looking at the months timescale. We need to know where we stand with this". And he was just backing off all the time and wouldn't give any information or any indication. Wouldn't commit himself to anything.'
Family 'So we have got this person telling us that he's got eight months, somebody telling us it might not be this, and then somebody else telling that we have got maybe three weeks.'
Family 'And I says "Well what if she has got it how long would you say she" and he said to me "It could be 12 months from the start of her illness" ... But as it was it was much, much shorter.'
As the disease progressed, families wanted to discuss the pros and cons of gastrostomy feeding and similar decisions. But sometimes professionals were unwilling to discuss it.
Family 'We actually said to [neurologist], and this was before [patient] left there, that "Look you know he's not eating, he's not drinking, what is the situation on artificially feeding [patient]. You must have some counsellors here". Because we didn't want to get into the situation of doing it and then regretting it.'
Professionals found it difficult to advise families about care when they had little knowledge themselves. They were anxious that families would ask them questions that no one could answer, and about managing the symptoms when they had not seen a case before. Many of the families commented that it must be difficult for professionals to manage a condition with no cure and so little knowledge about it.
Family 'How does a doctor comfort a family that he has just told that there's nothing he can do. How must he feel? As a professional doctor he must be gutted as well because doctors are there to save lives and it must be terrible for them to get a patient like [patient] and know they can't do anything for her to prolong her life, to make it any easier, or anything because it's so new, the disease is so new they know so very little about it.'
Family 'Because you ask the nurses and like to be honest with you and they would say "This is the first person we've nursed". So they didn't know either. They was like going blind, getting through it blindly as well as you were as a family you know.'
Professional 'I would want to know if his death was typical. At the moment I have experience of one, I would hate to say to relatives that it would be peaceful then they had chronic fits or something. Just because I would hate to give misinformation to relatives.'
Professionals also wanted practical information about management of patients. They needed clear information on infection control, and information about how the condition might progress.
Professional 'But we had never had one before so it didn't take us much further. At that point we were coping with her needs and she was obviously deteriorating and would die from it, so we didn't really need to know. Except was it infectious to the staff. When they found out they were anxious about it, and the surveillance unit were able to put minds at rest. They'd been caring for her for 4 months by then. I think the staff have to know the situation from the start.'
Professional 'It would be easier next time. At local level a policy on young people with nvCJD. We treated her as anybody with a terminal neurological illness.'
Professional 'We were all in the dark, didn't know what was happening, it was a new diagnosis, picked up stuff in the media only. There are many parallels with HIV in the early days ... There were scare stories, lack of information.'
The importance of having a diagnosis
Many of the families discussed what it meant to them to know the diagnosis. It helped them to make sense of what was happening. Sometimes they were bewildered when they were told the patient had depression or dementia. Their understanding of these diagnoses did not fit with their observations of what was happening to their relative. Similarly, being told the investigations were normal did not make sense.
Family 'But when [neurologist] said he was quite sure [patient] had nvCJD, that made more sense to me ... nothing could explain dementia to me in my head but nvCJD was a disease and I thought right that explains all ... It was the dementia that threw me.'
If the condition was just a mystery, it was impossible to come to terms with. Having a 'label' gave them something to focus on. It put ended the uncertainty over what was wrong and what had caused it. Importantly, having a name for the condition made it possible to begin to resolve their grief.
Family 'It is important to have an answer, however unpalatable the answer may be. It is important to have a disease, a tag or label, whatever you'd like to call it because even then you can focus and cope. You've got something to cope with. It's the threshold that you need to cross, the focus issue.'
Family 'There was nothing definite to get hold of, to grasp.'
Family 'It is important to know what they've got. It is important to know. I mean at the time you're thinking she can't have an illness that nobody else in the world has got. I mean that would be impossible. They must know what she's got.'
Many of the families said they felt alone and isolated when their relative suffered from an inexplicable condition that no one else had. Putting a name to it meant they were not alone. It also meant they could find others to fight with, because they wanted to fight against whatever had caused such a dreadful disease. Having a name for it also made it possible to find help. Some had tried to find self-help groups before the diagnosis was know, and found there were no groups for unknown conditions.
Family 'I think one of the most important things that would have helped us would have been to know that there was somebody else that had been through what we'd been through. I think that would have been a help.'
Family 'And you can ring up somebody. You can ring up a charity. You can get information. We had nothing ... Just somebody you could pick the phone up to. As it was we were completely alone, completely alone.'
Family 'But it makes you feel that you're not alone. I didn't know there was anybody else that had this disease and you do feel totally isolated and all the months that we had nursed [patient] beforehand, you think you're just on your own, there's nobody else that's been through this. Nobody else can know what you are feeling or how you are feeling ... knowing there was somebody there that knew exactly and had been through exactly the same thing and lost a child with this disease, it helped because you could turn to somebody and they knew what you was talking about.'
Knowing the diagnosis was the first step to finding out what would happen. This helped them to prepare emotionally and to plan the practical care that would be needed.
Family 'We needed to know what was going to happen. I think because everything was slipping away so fast. We needed somebody to say well you know just to be able to put some kind of hook on it so we could sort of say right we've got 5 months, 6 months.'
Family 'If we would have known earlier that what she was suffering with, we then could have sorted things out a little bit better and we wouldn't have been sort of wiser after each fact had come along, you know. And it was like a learning process for us because every week things were changing.'
Family 'It was distressing finding out but once you found out at least you know the end result so you prepare for that and you act according to that and that's moving him to a hospice and looking after him the best they could. You still want to know, don't you as soon as you can?'
Family 'Because then we knew what we was dealing with. We knew, we would know how to handle it. We would know the best way to care for her. Had it been a breakdown then obviously the care pattern would have been a different care package. It would have been a different way to look after her. Had it been a brain tumour and it was operable, again it would have been a different way of caring for her. Whatever it was there was a way of caring for her.'
Knowing the diagnosis and prognosis made it easier for professionals to plan management and to argue for the resources they might need.
Professional 'I might have thought twice about the PEG if I'd known the diagnosis. I didn't know how fast cognition would be impaired, I don't think after a week she would have felt hungry. Though prolonging was not the aim, it was palliating her symptoms, it might've been better not to. It prolongs things for the family.'
Professional 'Knowing the diagnosis would have been helpful, almost immediately I would have known what I was dealing with ... If you knew we were going to use a carer a lot of hours in a week, authorities are not worried if it's not prolonged. I got information that she was rapidly deteriorating so we knew it would be short. It probably helps if you go to someone and say this person has this disease, it makes it easier.'
It was also important to know what the disease was not. Other possibilities included a genetic condition, or HIV. These had implications for the other members of the family so it was important to know that they had been excluded. Professionals were anxious not to miss a treatable cause.
Family 'I know they tested for HIV as well but that was a pretty bad point because I remember thinking if she has got it then I've got it and if I am well I can look after her but if I am sick as well it becomes very, very awkward.'
Family 'If it was a genetically orientated thing [patient's brother] future would be blighted and I would say as a parent now and he must have felt the same way about his future from the point of view of the genetics side.'
Neurologist 'Deep down we knew what it was but there's always a nagging doubt, what if she dies and it's not that. I had to live with that.'
Some respondents mentioned their guilt that they had not understood how ill the patient was when they first showed behavioural changes. Often they had argued with them and been unsympathetic. They thought if only they had known the diagnosis earlier they would have been more sympathetic to the patient and helped them enjoy what was left of their life.
Family 'And to know what was going on and why. I mean, I don't suppose it would have been any easier to deal with had we known from the very beginning what was wrong with her. We would have looked after her differently. I mean, obviously when you're caring for somebody like that and you don't know what is wrong with them, you get frustrated.'
It was important to record cases, to show the extent of the problem. Only if it was recognised as a problem would scientists look for a find a cure for it.
Family 'It's not just because its on a piece of paper but I think for future medical records ...What could happen if all the cases that have died, all new variant CJD cases were not recorded and in 15 years time you've got 50, 60, 70 thousand people dying of new variant CJD, when did it first start. It's like the cattle.'
Lack of a clear diagnosis could make the families mistrust the professionals, or feel they were incompetent. Professionals recognised this.
Psychiatrist 'Because of having to wait for tests and for it to progress it was difficult for the family and for us, there was a sense of mistrust or incompetence; we don't know what we are doing or we are holding something back.'
Some families said the diagnosis itself was less important than knowing the prognosis.
Family 'The disease was just a name. It was the prognosis that was obviously the main bit so it wouldn't have really mattered what it was.'
Family 'They wanted a diagnosis and that is their agenda, that wasn't ours, we just wanted to take care of [patient] and to give her the best quality of life that we possibly could.'
Some families rejected brain biopsy when the poor prognosis was apparent, but others felt they still wanted to know for certain.
Family 'Leading up to the decision and the biopsy we were told at that time you know, we knew by that time that there wasn't any hope for him. He would just have a few months. And then we decided on the biopsy because we felt in our hearts we really needed to know.'
Professionals commented that the diagnosis made little different to the management, as they had no experience of the condition and were managing the symptoms as they presented.
Neurologist 'Again it is so difficult, patients always ask, "How long have I got". One can't ever give the prognosis in terms of time, can paint a picture of what the prognosis might be. It might help with decisions on PEG feeding, antibiotics, but I wouldn't need a diagnosis to make these decisions. It was self evident it was progressing and would be fatal.'
Two respondents said it might be better not to know the diagnosis, because it meant there was no hope.
Family 'In a way I think maybe we were lucky because we weren't getting a diagnosis. It didn't rub out hope altogether because it wasn't given a name. Maybe there was just that slight element of hope that you cling on to. I think now with them being diagnosed earlier its going to be really difficult because you know there's only one end.'
GP 'I think its better not to know too soon. It helps having a late diagnosis; its one of those dreadful conditions, if you know too early you just have to suffer for longer. If I got it I wouldn't want to know 2 years before it presented, I don't know about all these tonsil biopsies, it's a difficult one isn't it. I know it might have helped the family if he hadn't gone down the psychiatric route.'
Role of the CJD Surveillance Unit
Families and patients were visited by a registrar or consultant from the National CJD Surveillance Unit when the unit was notified of the patient. Six recalled completing a questionnaire but not receiving any further help. But others found the visit a useful source of information about nvCJD. Many viewed it as a second opinion, confirmation of the diagnosis. Six families had kept in contact with the registrar, who sent them papers and more information about nvCJD, and was available to talk to them by phone.
Family 'So after his test or after his taking blood or sample, whatever he did, he more or less confirmed it. He couldn't confirm but he said that's what he thought it was.'
Family 'He kept in, you know, regular contact with me. He provided as much information as he could at that time. He gave me the booklet and tried his best to explain things and as I say he kept in regular contact with me at that time.'
One of the GPs, a palliative care consultant, one psychiatrist and all of the neurologists had contact with the surveillance unit. Most thought it was useful for a doctor from the unit to visit and give a second opinion about whether CJD or nvCJD was the likely diagnosis. Some commented that they were well placed to discuss the condition with the family, as they had seen more cases. One neurologist said that the visit was seen as an important event by families and that a written report from them would be helpful. But neurologists recognised that it would be impractical for them to advise on the management of cases.
Neurologist 'It would be helpful to have rapid written feedback. It's a big thing for the relatives, someone from Edinburgh coming down, this will be the answer. We don't tend to get written feedback. A written report to discuss with the relatives. Basically its like a second opinion, with something at the end saying, "Yes we agree its typical of CJD" or, "No we don't", and "at the current time we think this test is of value or not". It would need to be comprehensive and rapid.'
Neurologist 'It would be difficult for the surveillance unit to advise on management of patients throughout the country. You need to strike a relationship with relatives, so you need someone who can spend a lot of time with them.'
Neurologists thought the most important role of the surveillance unit was collating epidemiological information. Most did not want more information about the disease or appropriate investigations, although the unit had helped to arrange the practicalities of some investigations. One neurologist suggested that the unit could prepare guidance on early features to help diagnosis, and another wanted advice on the value of different diagnostic tests.
Neurologist 'We wanted 2 things: reassurance and confirmation of the likely clinical picture and information about available diagnostic facilities. We sent CSF to the States through Edinburgh so it was helpful practically. I had read all that was published so I didn't need to know what was going to happen.'
Neurologist 'I feel without the surveillance unit the epidemiology would not have been collected and nvCJD would not have been recognised.'
Neurologist 'Going back to earlier diagnosis, I just wonder if it would be possible for the unit to put together markers for early diagnosis, they must have seen lots of cases of nvCJD and those that didn't turn out to be nvCJD. It would be very useful if they could publish something to give markers so you would know, for example, if you watched a case for 3 months and they didn't progress it might be very unlikely.'
One family, of a patient who lived for several months after diagnosis, was surprised that the surveillance unit did not follow her up.
Family 'I was surprised at the total lack of interest in the follow up because she stayed alive so long and they was all totally amazed about that but nobody followed it up. Nobody come to see her again.'
Most of the families had been given a booklet by the research registrar. Seven said they found it helpful, because it explained the investigations, went through what would happen, and, most importantly, it described features that tied in with their own experiences. This confirmed the diagnosis to them and helped them understand what might happen next.
Family 'That book told us exactly all the stages and everything that he was going to go through and that was a tremendous help to us because well we knew in advance what was going to happen and that the prognosis was that it would just be a few months.'
Family 'When I read that book it convinced me because everything through the book I'd seen it in [patient]. It was just like as if I'd written it you know. Every little item I was, I sat on my own, [patient] sat there and I read the book. It took me two or three days to get to read it because I probably didn't want to you know and it was just as if everything was written you was watching it and when I read the book I thought [patient]'s got it.'
Four of the families said the booklet was not helpful because the symptoms described in the booklet differed from their observations of their relative's symptoms. One respondent said he was too busy dealing with the problems presenting to want to read about the disease. Three families said the only helpful information in the booklet was the helpline number to contact the CJD Support Network.
Family 'He give us that yellow book and we read that and I just pulled holes in it left right and centre, you know, its not transmissible and all this and you know. I just ripped it to pieces because I'm not that stupid myself and I could see a lot of loopholes in it. I believe its been re-written again and again after that.'
Post Mortem results
In all cases it took several weeks to get the results of the post mortem, and some families said they had never formally been told the results despite asking several times. Four families heard the PM results in person, and appreciated the chance to discuss them with the neurologist. Others heard by letter or phone call.
Family 'I suppose he could have just wrote a letter to me and said well this was the results of it. What he did do he told me to actually come and see him and we sat for about an hour and we just talked about it and you know the implications and so on and so forth you know. As I say he was very good, very good.'
COORDINATION AND CONTINUITY OF CARE
Many patients were managed in a succession of different settings with little continuity between them. As the condition progressed, a number of professionals were involved and coordination of care was often difficult. These problems were exacerbated by uncertainty about the diagnosis and prognosis. Many of these problems are covered in earlier sections, but they are summarised below.
Coordination of investigations
Because of the non-specific nature of the presentation, some patients were referred to several different specialists. Others saw several private specialists or alternative practitioners. Sometimes this meant they were not in the 'system' and it was hard to organise follow-up,
Family 'It seemed because [patient] was seeing a psychiatrist privately he was outside the system and the psychiatrist recommended that he had the MRI scan and the EEG ... So it was up to us to follow that through and to work out how to get it done.'
Family 'And I mentioned to you earlier that we went to psychiatrists. By the way that guy disappeared from the face of the earth. Never heard from him again. I tried to contact him and couldn't. He wasn't associated to the [psychiatric hospital].'
It often seemed as if families were trying different sources of help but no one would 'take on' the problem. Often the neurologist would refer the patient to a psychiatrist or psychologist to exclude a psychological cause. The families viewed this as yet another diversion. In some cases there was no locally based neurologist, and this proved a problem in arranging follow-up. One patient got lost to follow-up when a neurologist retired.
Family 'I felt they weren't taking it seriously enough. She was being seen on a regular basis by her GP and he was trying, well he told me at the time he was trying, to get her an early appointment to go back to the neurologist ... This seemed to take quite a while to happen ... and I insisted then that she get some form of admittance to some hospital ... eventually she was admitted to the general hospital ... so they discharged her. I asked if they were able to transfer her and they said no, so back to the GP again...'
Many patients were in and out of hospital for investigations. Families felt that once the patient was off the ward, they were no longer the responsibility of the hospital staff. There were also numerous logistic problems in arranging investigations and transport between units.
Coordination of care
It was striking that few families recalled much contact with social services while the patient was undergoing investigation. Patients were becoming more and more dependent but usually no care package was arranged when they were discharged home between admissions. Where social work services were involved, it would be a different social worker while the patient was in hospital to that when the patient was at home.
One family was upset that care had not been arranged for the patient, when she was brought home 2 weeks after they thought the social services had been notified. They later discovered the social worker was actually notified just one day before the patient was discharged.
Family 'I brought her home and as far as I knew they had the two weeks to set up the care package. And it wasn't until months afterwards that I found out that they had only heard the day before I was going to bring her home.'
Many of the patients were fearful of strangers and needed to be cared for by familiar carers. Most families reported better continuity of care during the terminal stage of the illness. Often the nursing staff developed a close relationship with the patient and family, attended the funeral and remained in contact with the family.
Finding a suitable place for terminal care was difficult in many cases. One family had to find a place themselves without any help from the hospital social workers. They reported that it was more difficult because the diagnosis was unknown, and transfer was delayed by arguments over funding.
Family R1: '[Neurologist] wouldn't put a title to the disease. He said no he wouldn't put a title to it. So of course that meant a problem.'
R2: 'For the other agencies because they need to know what they're dealing with.'
R1: 'In three weeks this second meeting took place round the table meeting ... the social workers came with their shorthand notebooks completely blank. There wasn't a thing written on them.'
R2: 'Nothing to offer whatsoever.'
The time spent by his parents in visiting nursing homes detracted from time with their dying son.
Family 'We were devoting time, energy, emotion and stress into sourcing a place for [patient] to live. At the time we knew our son was dying and we didn't realise how quickly the sands of time were running out. We really shouldn't have been spending that time on those tasks. They're rightfully the tasks of other people. This is detracting from our primary need which is to care for our loved one whose time is expiring in front of us.'
The rapidity of progression meant that patients' needs changed rapidly and needed reassessed. They needed care to be available quickly, and the flexibility to allow changes in the care provided to respond to increasing needs.
The two families who moved when the patient was ill both experienced problems in transferring care packages to a new location. One had to manage a doubly incontinent patient for several days with no incontinence pads while awaiting reassessment.
Family 'I was expecting to move in and find out things were there. Nothing, not a thing and the nurse came out and when I mentioned it they said, "Oh we've got to have an assessment before we can give you anything" ... and I was again then literally cleaning her with my bare hands.'
Communication between professionals
There were several examples of poor communication between professionals on the wards where patients were investigated. For example, one family described an occupational therapist coming to see the patient for a home assessment, who did not know that he was unlikely to go home.
Family 'At that stage, and I can always remember it, an occupational therapist came in to see us. We sat round [patient]'s bed, taking into account at this stage he could do nothing for himself. "I've come to see you because we want to know what you want putting in your house." And we said, "We don't know whether he is coming home or not". "Of course he is, what's wrong with him?" And we said we don't know. "I'll find out then". So off she went and I said to her, "Well if you can find out you're doing better than we are". So off she went and about 10 minutes later she came back. "I think we'll have to leave it for a day or two, yes I'll come back and see you". Well we never saw her ever again.'
In another example, a nurse told a patient to sit up after a lumbar puncture, when the family had been told he should lie flat.
Family 'And the doctor came ... He said, "As soon as he has had his lunch lay him down flat again because he has had a lumbar puncture and unless they lay down for 4 or 5 hours they end up with a tremendous headache". But this little auxiliary told him to come and sit up in chair, "What are you doing in bed?".'
It was confusing for families when the doctors seemed to disagree about the diagnosis. This happened in several cases. It added to the family's distress, and they often thought it was because the diagnosis was being deliberately covered up.
In some cases, there was disagreement between a psychiatrist and a neurologist about the nature of the condition. The psychiatrists felt abandoned, without sufficient neurological support for what they thought was an organic problem. Some spent a lot of time trying to expedite neurology investigations. They also reported poor feedback from the neurologists once the patient was transferred to their care.
Family '[Psychiatrists] tried very hard to sort of speed it up for [patient] to be taken to the [neurology unit] and they were sort of chasing it up every day.'
Psychiatrist 'She had various scans and was referred to the neurologists who batted her straight back saying it was anxiety. We felt we could diagnose anxiety better than they could so we were unhappy about that.'
Psychiatrist 'We had no feedback at all from them, not even a discharge summary, we heard because [junior doctor] phoned to ask.'
Communication when patients were transferred for terminal care was also sometimes poor.
Family 'The first thing was that the sister from the ward before he was moved, went to the [neurology unit] and ... to meet him and to find out what was going on. They never got any information from [neurology unit], written information, no hospital notes or anything. We found this out later on.'
Many of the professionals caring for patients in the terminal stages said they lacked knowledge about the disease, and were unsure how to manage it. They wanted advice from professionals with experience of the condition, who knew what problems might present and what interventions helped.
Professional 'Someone else to talk to who'd treated someone in similar situation. We were in the dark really ... The more information you have the more help you are to the family, because they do keep asking you all the time, it would give them more confidence. What to expect, in terms of her mental state, it did change and fluctuate, especially towards the end she became aggressive, its nice to know to expect these things.'
Families expected professionals to share information about the patient. They were surprised that neurologists had not networked more widely to find other patients with similar symptoms. They were surprised that the GP did not hear the diagnosis when the family were told themselves.
Family 'So it happened to be [GP] who we went to see and he said, "Oh, how's [patient]?" so I said, "Well, haven't you had a letter?" So he said, "No". So I said, "Well apparently they think it is nvCJD" and he just looked at me ... like I'm lecturing a doctor which is not right, is it really?'
Some said that professionals should be able to get expert advice on nvCJD at the first suggestion of the diagnosis, avoiding referrals to local neurology and psychiatry units.
Family '[GP] should be able to pick up his phone and say right we've got a suspected case of this and then they go into where someone can really go for it ... Instead of pushing them off to the hospital it ought to be coming through to someone ... that's specialised.'
Key worker role
Families wanted one person to be available who knew about the condition and care needs, to coordinate care, to give information, and to make sure the family received both practical and emotional support. Professionals also identified a need for someone to take the lead in coordinating information and care.
Family 'I'm not saying you need be a doctor, I'm not saying you need be a nurse but somebody who is a listener and can allay your fears.'
Family 'It needs somebody that actually is able to network really as a professional and as somebody who knows exactly what happens with this illness.'
Neurologist 'Team care is fashionable but it depends who's talking the way they use it, it has its place but with a condition like this the strength of the relationship with the family is crucial and is diluted if delivered by a team rather than an individual ... The trust of the family and personal relationship are more important than knowledge.'
When patients were terminally ill, and dependent, many different carers were involved. It was important to coordinate care and communication between them. Concerns over confidentiality and the possibility of media interest made this more difficult. In 3 of the 5 cases managed at home, families identified a social worker or nurse who coordinated care. In the other 2 they thought the GP did so.
Family 'So I think the most of the care that was done, I mean we did at one stage, we had something like 20 professionals involved in different sort of, different areas. Some of them were very welcome but some of them were not at all.'
Where terminal care was in hospital or nursing homes, most families did not identify one person as key worker, but often developed relationships with several of the staff.
Family 'I think basically the GP was keeping a watching eye on her when she was in the [unit] ... But I think basically there again that the sister was my confidante sort of thing. She'd come and talk to me...'
Family 'The staff became friends basically over the time that [patient] was there. And they were always there and very friendly and very helpful and they always said that if there was anything at all I needed you know. They would constantly talk to me and ask me how I was, things like that as well, so it was the sole source at that time for both of us, for [patient]'s care and emotional support for myself.'
In most cases there was no one professional for the family to relate to earlier in the illness. There were many professionals involved in the care of patients from initial presentation. Most respondents said that only the family themselves provided any continuity between them.
The one professional who was involved from start to finish was the GP. In some cases the patient had terminal care at home or in a unit where the GP retained clinical responsibility. Where terminal care was provided in a hospital or hospice they were usually less involved.
GP 'Its organisational to a degree, make sure you get the right care in, depending what stage and what's possible ... There's a huge role in support for the family, its one thing to have a partner die, another to see them deteriorate in front of your eyes, become incontinent etc.'
Where the GP had a central role throughout, he/she already had a good rapport with the family, and could provide continuing support after the death. Many GPs saw continuing emotional support for the family as a main part of their role.
GP 'You are a little bit removed from clinical responsibility but continue to care for the rest of the family, help general support and preparation for death, start helping with the bereavement process early. Bridges to help through and ongoing support.'
Some GPs were less involved even if the patient was cared for at home terminally, and some families lost confidence in the GP because they were unhappy about the initial psychiatric diagnosis. Some GPs also said they were anxious about managing the condition and giving information to the family because it was unfamiliar to them.
Some of the neurologists remained clinically involved in the terminal stages of the illness. Many had very little input after the diagnosis was made, although they were available to give advice.
Family '[Neurologist] did say to me that don't feel like you've been slung out on a limb. I did phone him up a few times. He didn't actually come to see [patient] but he was interested in some aspects.'
Some of the neurologists interviewed thought they should be responsible for coordinating care, but others thought palliative care or rehabilitation physicians were better placed to do so. Some commented that there were too few neurologists to remain 'hands on', and they were often based in large centres far from the family's home. The roles played by different professionals depended on individual circumstances.
Neurologist 'Central role, it's sort of a team care but the neurologist has a central role. The major role in diagnosis, but also the focus for the patients' care, it can't really be delegated to anyone else.'
Neurologist 'Once the diagnosis is made and the relevant parties are involved, I expect the role of the neurologist fades into insignificance as carers like community and terminal care carers take over.'
Neurologist 'The neurologists' role is essentially excluding what's treatable. Degenerative disorders in young people are rare, there comes a stage when your input is of less value than somebody who is skilled at pulling together different disciplines, a specialist in young chronic sick, rehabilitation services, they have a package that they can offer. When the level of disability requires expertise in young disabled. It's why that speciality is there.'
Several of the professionals made suggestions about who should act as key worker. They needed to have some understanding of the condition, and a lot of knowledge of local services and how to access help quickly. One neurologist suggested that neurology liaison nurses could bridge the gap between hospital and community services. Another respondent suggested health visitors. It was also important for the family to like the key worker, as they would develop a close relationship.
Professional 'Role for someone to care for the family, would need nursing experience to understand nursing needs, social work experience to know what routes to go down ... Liaison officer of some kind between the various agencies. They could have had a social worker seconded, to deal with hospital, GP, nursing home, undertaker even. Needs to be compatible with family, they need to get on.'
Those professionals who did act as key workers found coordinating care for these patients very time consuming and emotionally demanding. There were rapid changes in the patient's abilities requiring reassessment of needs, and a large number of carers to coordinate and communicate with. The families were burdened by the emotional strain and the key workers took the lead in supporting them. They inevitably became close to the patient and family and were themselves distressed by the experience.
Professional 'I spent a great deal of time on the phone. We had a case conference with minutes ... We discussed what care was being provided and future plans. We also communicated on a weekly basis, I was aware of what everyone was doing, the central person.'
Professional 'At times I felt stretched what to suggest next ... You need to be a diplomat as well ... The key worker also needs support, its very stressful for them. The funeral was particularly stressful...'
DEALING WITH THE MEDIA
Many families were harassed by journalists, who repeatedly telephoned or came to their door seeking a story and refused to leave them alone. Three families were shocked by an unexpected prominent newspaper story, and one was angry that they published wrong information about the patient's eating habits. One family was particularly anxious that the press should not publish photographs of the patient.
Family 'That was a horrific time because there were reporters coming to the door, on the phone. Oh it was a nightmare. That was really terrible and they weren't interested in my daughter, they were just wanting sensationalism in the paper. You know mad cow and her face right in front of the paper. Mad cow disease victim ... The actual [newspaper]came to my door at half past eleven at night. Battered the door and of course I was here, the kids were up in bed and I didn't open the door. "Hello this is so and so from the [newspaper]", told me how much money I would get and I said, "Do you realise its half past eleven at night" ... He had a photographer, somebody with a camera, as soon as I probably opened the door they would take my picture.'
Family 'When I passed the news agents later and there was a full front page of [patient] staring out at me which was obviously a wee bit shocking. The other press they would come up and they would buzz and they just tried things, "Alright if you don't want to speak to us is there any chance we can just pop in for a cup of coffee". And you know just basically trying to get inside the door.'
Family 'And the headline was "Dead from CJD" and a big picture of [patient] on the front page. And we never went to bed. We never went to bed all that night. We waited until it was six o'clock, half past five to six o'clock until we knew the newspapers would be delivered to the paper shop and we went down and got one. And then we had to phone round. I had to phone everybody, to say that the story was on the front page of the paper in the morning in case they walked into the newsagents and saw her looking up at them.'
Some families thought they could deal with it by talking to one paper only, but this tactic did not work. Once the press was involved, they would not go away.
Family 'And he says, "I'll let you see what I'm going to print before I print it," he says, "I can also fax, once you have agreed on it, I could also fax that to other newspapers and say this is the only statement we will release and that will save us some hassle". It didn't work ... as soon as it was out, the reporters were waiting on us coming in. They were in cars waiting on us driving in and so, you know, right away they were wanting photographs. They went round about the town. They got in touch with his pals somehow or other, offered them money, his friends money to give them a quote.'
Family 'So we decided to speak to one paper rather than all of them which was a bad mistake because we should have got them all in like you do but I had never done anything like that before so we didn't know. I spoke to what I thought was the biggest one ... Did two interviews and I thought that will be it. It will get them away from the door but no, they kept coming, kept coming, kept coming, didn't stop.'
The worry about press interest started during the illness and was an added burden at an already very stressful time. Families and professionals both worried about the press disrupting hospital wards and invading their privacy and that of other patients. Sometimes families were so fearful of the press finding out that they did not tell friends what was wrong and missed out on support.
Family 'We had to say to the matron, for God's sake don't let the nurses or the carers say anything because we don't want the press here.'
Family 'We had also been warned at that stage about the publicity so we didn't know how we could trust other people coming into the home.'
Family 'The hospital said that there could be, he said, "Please don't go to the papers or anything because there's other patients. We have to protect their privacy".'
Families felt a need for someone to act as a filter to protect them from the press, especially around the time of the death. One family used a press agent and another a solicitor to do so. Others used the Human BSE Foundation. But many had no one to help them deal with the press.
Family R1: 'Yes, I mean we should have had somebody you know sort of organising the press because ... they were waiting outside for us.'
R2: 'And the phone never stopped it was ringing as soon as we put it down it was ringing it was like that all day.'
R1: 'Newspapers press agencies and things like that.'
R2: 'All week it was like that.'
Family 'I think everything went through the press agency. They were the contact ... We allowed one photograph and a few words and that's it. I think we wrote something, a few lines, about four or five lines.'
Families found that the press would come back whenever nvCJD was in the news again. Every time it was in the news it also brought back their grief.
Families wanted to guard the memory of their relative and control what information was published.
Family 'But I'm not very good with the media. I am not a very public person, you know ... Also you've got no control over the way that they, you know, what they pick up and the way that they want to report it.'
Family 'I felt the need to be in control and to protect my family. I didn't want people commenting on [patient]'s life. They didn't know her, they knew nothing about her ... I did then and I still do, guard [patient]'s memory very jealously and that's why I did not want to become involved with the press at all.'
Using the media
Some families welcomed and sought the press interest, because they wanted to warn others about nvCJD. They also thought the media attention had helped get the BSE Inquiry. Many were exasperated that people didn't understand the different types of CJD and wanted to raise awareness of that. They thought talking to journalists was one way in which the victims would not be forgotten. Some found talking to journalists helped to talk through their grief. One family used the press interest to argue for better care for the patient.
Family 'We were very angry about it and we'd actually gone to the press. We wanted to warn other people about what could happen if they fed their children on infected beef basically. You know we'd watched our son die, we didn't want other families to have to go through this.'
Family 'Yes I mean after so long everybody sort of loses interest if you like, you know, it sort of just drops off. But I believe that every so often it should be brought out into the open and try and let people realise you know about [patient], about the other victims. I don't think it should be forgotten. I don't' want people to forget his name you know.'
Family 'We wasn't getting what we wanted and we decided to go public. We knew that we'd hit the news. It wasn't an easy decision to take but we felt it was the only way to get what we needed for [patient] so we went public, we decided to go public.'
Most families avoided the press until after the patient died, often because of the fear that they would disrupt wards and invade other patients' privacy. They then sought media attention. Some described the contacts they have now developed with journalists.
Family 'You get to know the people in the media after a while. Because one or two came to do, "Can we do this article?" or "Can we do this programme?" and then you get to know a name and then anytime anything that you want that happens that you want to bring to their attention you ring them up and say "Look this is happening".'
Family 'So we've established a very wide network and I have a very wide network of media contacts. They ring me frequently even just to keep up-to-date with to sound me out with what's happening at the minute. Obviously so they can keep it on the back burner if they need to go into it again they'll give you a call you know.'
But the relationship with the media was fragile. Families were disappointed that they could not control the angle the press took. Some expressed their anger that the same papers that reported on patients who died of nvCJD also published stories about people flaunting the beef on the bone ban, or comments from a coroner who did not believe eating beef caused nvCJD. They were exasperated when the media confused nvCJD with other strains.
Family R1: 'I think somebody hadn't thought about it, in February they were saying that somebody had died and then in December they are telling you that this fellow's defying beef on the bone ban but they said why, because of this new variant CJD, which they weren't very...'
Family 'I mean they phoned us up when recently they started to get claims for the ... growth hormone, and they announced it was, you know, mad cow disease. They asked us for our opinion about, you know, about how we felt about that. And we said, "But it's got nothing to do with us, you know, that is something completely separate, it has not come through the same route, it is nothing to do with eating meat, it's, you know, for goodness sake go away and get your facts right".'
Families of patients who died before March 1996 when nvCJD was announced were angry that they were not told before the announcement was made.
Family 'I was annoyed about March because when they announced it, the government, that there was ten cases, obviously I knew [patient] was one of them by this time, nobody had let me know and I was just here trying to get things back to normal after getting sort of virtually rid of the press then they all come back, you see. So they was all bothering again. And then we had bother for a bit of time then.'
Some families were warned not to talk to journalists by professionals. They saw this as an attempt to cover up the extent of the disease. One family thought they were given the diagnosis late to stop them informing the press while the patient was in hospital. Another felt they were unwelcome to visit the patient on the ward after they had spoken to the papers.
Nearly all the professionals had refused to talk to journalists about the case. They were anxious that the press would disrupt wards, upset other patients, and get in the way of their day to day work. They were also concerned about breaking confidentiality. Some mentioned a need to help the family deal with all the press attention, or that they themselves needed a strategy to deal with it. Professionals thought the media attention was an added burden for the grieving families, made it more difficult to provide care for patients and made families suspicious of the professionals trying to care for them.
Neurologist 'The thing that would help most is if this condition was not given the sort of extra public scrutiny that it has been and parents were allowed to be in peace. They were phoned by radio and papers and so forth, it was unpleasant. It would be easier if there was not the notion that scientists and doctors were on one side and truth and journalists finding out all the mistakes.'
GP 'All the publicity added pressure to the family. I did discuss it with colleagues at the time ... we were very careful over confidentiality.'
Professional 'In the beginning we were frightened that the media would arrive, we were harassed at one time by telephone, worried that they would actually arrive and be intrusive. Suspicious of everyone that came to visit until we got to know them.'
GP 'There were some nuisance calls, journalists trying to get through. But we took action, all the receptionists were aware that any calls they didn't give anything away and referred them to me ... I've seen the publicity about other cases, I thought some were very insensitive and intrusive and jumping to conclusions.'
WHY nvCJD IS DIFFERENT
A striking finding from the interviews with families was the strength of their conviction that nvCJD is different from other conditions and should be managed differently, with special funding. The reasons why they believe it is different are summarised below.
Where it came from
nvCJD was considered a manmade disease, which was not an accident of fate but had arisen because of the actions of, or lack of action by, the government. As discussed above, there was a strong perception that the government was 'covering up' the disease, and families felt frustrated by this. They believed that the government owed a duty of care to patients.
Family 'If it is new variant CJD or human BSE, whatever you want to call it, then there should be this pot of money because at the end of the day we feel you know that this is a manmade disease, its been created and so the government should pay. It's not a disease that you just happen to get, it was given. The victims shouldn't have to suffer.'
Family 'I just think that the government has yet to, has continued to deny that they have a duty of care to all of these young people.'
They contrasted the financial burden many families carried because of the disease with the financial compensation paid to farmers to slaughter their cattle. They also contrasted the lack of care provision for patients with nvCJD with the special funding available for care of AIDS patients.
Family 'But they don't seem to be doing anything for us. If you look they've compensated farmers, meat rendering people and all that. So in a way they've sort of admitted they're in the wrong but the people that have really suffered, likes of us, they seem to have forgotten haven't they, and they're still sitting on this saying nothing is proven, nothing this. That side of it I really get a bit, you feel like going up there and shaking them by the throat or something.'
Family 'When they realised it wasn't AIDS they backed off double quick. "Oh, no can't do it." Couldn't do nothing because it wasn't AIDS. The funding wasn't there. The funding was there for AIDS patients.'
The anger, about the cause of the disease and perceived cover-up, was itself an added burden for grieving families. In early cases, doctors often didn't accept the link between nvCJD and BSE and this was a barrier between them and the families.
Family 'It's not like with most deaths the overwhelming feeling is sorrow. I think with this a great part of this is anger.'
Psychiatrist 'They were inclined to attribute it to BSE and I really tried to discourage them ... At the time I didn't accept that there was a strong case that that was the aetiology, didn't want them to get stuck with that. They might have seen me as unhelpful, it might have been a barrier.'
nvCJD was continually in the news, so families were continually reminded of what had happened. Many had traumatic experiences of being harassed by journalists, who would return whenever it was in the news again.
Family 'And the grieving process is longer with this because you might get over it a bit then all of a sudden something will come back on the news in the papers.'
Neurologist 'In neurology practice we deal with lots of conditions with a lot in common. That aspect is not altogether unknown, we are familiar with people deteriorating inexorably, when there is nothing we can do to stop it and we play a supportive role to the family. It's not unique from the caring point of view. What is unique is the press interest.'
Family 'Very different I would say from even a young person dying of cancer. No matter how sad that is and I mean tragic but they don't have these other 2 things to go along with it. They haven't got the anger, because it's a thing they don't know why cancer happens really, and they haven't got the press intrusion because the press are not interested. But with new variant CJD it is another world and that's what sets it apart.'
Most of the families had been involved in campaigning for the Inquiry, through the Human BSE Foundation. They found the CJD Support Network and Human BSE Foundation useful for support, but it was another way in which nvCJD was an ongoing problem for them, it was something they continued to live with.
Family 'Because it hasn't gone away. It's not like another, if [patient] had died from cancer or a heart problem you would have been getting a little bit away from it but because of what he's died from it, as I said, been all reporters in, television crews here and the phone. Everything has just been ongoing. The support group, going to meetings.'
Features of the condition
There can be no doubt that nvCJD was a particularly distressing condition for families to witness. Patients had undergone a change in personality, and rapidly deteriorated mentally and physically. In the early stages they were mentally unwell but often resisted seeking treatment and families didn't know where to get help. As it progressed, they had very great needs for psychiatric and physical care. It was as if they were progressively rubbed out as a person. Many families commented that it was more distressing because it killed young people, it was easier to accept the deaths of older people. It was also bewildering, because there was no reason why they should be at risk.
Family 'I can't think of anything as horrific as this. I mean even AIDS now, I said to the neurologist this condition is worse than AIDS. There's just no holding it. Everything is just slowing getting rubbed out. It can happen to anybody regardless of what they've done in the past.'
The uncertainties over diagnosis and prognosis, the strain of waiting for negative investigations, the lack of any treatment all made it harder for families to come to terms with what was happening. The lack of knowledge about the disease and rarity meant there was nowhere to seek information and support.
Psychiatrist 'Difficulty in managing something little understood, greatly feared and extremely rare condition ... Most similar experience in hospital in the early 80s with the first few AIDS cases, non understanding and fear although the risk of transmission there, not really sure what one is dealing with.'
Professional 'There's parallels with HIV, a great deal of confusion, anger, unknowns about what's likely to happen.'
Many families felt there was a stigma attached to the condition, that they were somehow contaminated.
Family 'It's a stigma with this as well you see, believe it or not.'
There were genetic risk factors and the families had agreed to genetic testing of the patients. Other family members could also have eaten the same contaminated meat. For these reasons many families worried that other members of the family might also get it.
Family 'You live with this constantly. As growth hormone people do. We live with this every day because we just don't know. [Patient] was probably very young when he contracted it. We don't know whether because we're older its taken on because there's no answers. Until we get some answers about incubation period, dosages, we live with this horror every day. You get up with it in your mind. You know every time you know if you forget something, you can't remember a name or just daft things.'
Some families felt patients were being punished, yet they had done nothing to deserve to be ill. Sometimes they wanted to be punished themselves, because they blamed themselves for the patient's illness. Mothers often blamed themselves for feeding their children contaminated meat. One mother said it was hard to come to terms with because it was a mother's instinct to feed her child, yet in so doing her child had got this dreadful disease.
Family 'It is not like anything else. It's an illness that they haven't got any medication for. There's no hope at the moment. They are still finding out exactly where it came from, how it happened to turn up in the food supply. It has actually interfered with the most fundamental thing in life which is food. If we don't eat we die. We eat and we're dying ... so it's unreal. There's no explanation.'
DIFFERENCES SINCE nvCJD WAS ANNOUNCED
Of the 19 patients and families who were the subjects of this study, 8 patients presented before nvCJD was announced in March 1996, and 11 presented afterwards. It seems intuitive that there should be differences in the families' experiences before and after the condition was recognised. But the numbers of cases are small, and conclusions about differences between the groups should be treated with caution.
Time to diagnosis
It might be hypothesised that the time to diagnosis would be quicker in the later cases. But the median time from onset to the time the National CJD Surveillance Unit was notified was similar in early and late cases, at 45 and 46 weeks respectively. The median time from seeing the first neurologist to the time of notification is markedly shorter in later cases, at 5 weeks compared with 14 weeks in early cases, although the number of cases is too small for this difference to be statistically significant. There were no differences in median times from onset to referral or from onset to seeing a specialist.
Knowing the diagnosis
The importance of having a diagnosis has been discussed above. Similar points were raised by families of early and later cases. In all the later cases and 5 of the 8 early cases the family was told that CJD was the likely diagnosis and the research registrar visited the family before the patient died. In the other 3 cases the family was aware that CJD was a possibility but the neurologist did not confirm to them that it was the likely diagnosis. These three families feel they suffered from greater uncertainty and missed out on support and information from the surveillance unit and the CJD Support Network.
Family 'We would have had a massive amount of knowledge that we could have drawn on and as it was we didn't have that.'
But there was also uncertainty about the diagnosis in later cases, as the diagnosis is only confirmed by biopsy or post mortem. Two of the early cases and 3 of the later cases were confirmed by biopsy while the patient was alive. One of the later families was told nvCJD was unlikely after the registrar had already visited because the CSF results were not typical.
Support from other families
Families of some early patients feel they missed out on help and advice from other families. Six of the early families and 7 of the later ones are now very actively involved in the Support Network, especially the Human BSE Foundation. Most of the other families have also had some contact with them. Several families discussed their feeling that they were alone in facing such a terrible disease. They believe that mutual support from other families would have helped them to cope with what was happening and to fight to find out why it had happened. Many of the later families also described feeling isolated until the diagnosis was made and they were able to find other patients and families suffering from the same thing.
Family 'But we thought we had a very lonely path to tread. If you were the only one that it had happened to, if we're unique and there was only [patient] that had died from eating infected beef we had a hell of a battle on our hands. But to know there was 5, 6, 7, 8 other people with the same symptoms, that would have helped.'
It is perhaps worth commenting that most of the issues discussed here were similar for families of early and later patients. They all suffered from the uncertainties early on in the disease, wanted earlier diagnosis and more information about the condition and how it would progress. Families of both early and later patients described poor care in acute wards, where staff didn't seem to appreciate the extent of the patients' disabilities. There remained great problems in finding a suitable place for a young person with a terminal illness, with both physical and psychiatric features. Coordination and continuity of care between settings was still difficult, and most families felt they themselves were providing the only continuity for patients. The rapidity of deterioration and great care needs of patients is of course the same. As one professional commented,
Neurologist 'Its such a ghastly disease that anything you provide isn't going to be enough.'
Families also felt the same anger about the disease, and suspicions that it was being 'covered up' by the government and by professionals. The problems in dealing with the intense media interest were common to families of early and later patients.
Families' expectations of change
Although it was difficult to discern differences in the experiences before and after March 1996, families did express expectations of what should change with greater recognition of the new variant strain of CJD.
Family 'People are still not getting the support that they need and I find that frightening. I had hoped that because we had been through this precedence that would help others through it and things would be in place.'
They think that professionals should have more knowledge about the condition, so that GPs can recognise it earlier and avoid referrals to psychiatrists.
Family 'But in all these practices I can't see why one doctor shouldn't at least have some sort of knowledge of it. In one way or another, however they do get knowledge, so it could be picked up a lot quicker.'
They also think that professionals should have better knowledge about disease progression in order to provide better information to families. They think a care package should be designed, including all the services needed to care for patients with nvCJD, and it should be centrally funded.
Family 'You need special teams of people that can go in on each individual case as it arises with specialist knowledge on money, on care, on places to put them, where they can go straight in and say right we'll organise it, we'll make sure, we'll take the worry and the burden off you straight away.'
Family 'People have different experiences and their needs are different, they are so different, that it needs to have somebody that is aware of the illness, how it is going to progress, the professionals that need to be involved and to actually to get that to be done ... To be the link person or a team or one person to go to the different places throughout the country and organise that care and have the connections to the social services, to talk about the implications, about the things like stair lifts, any physical aids that are needed, any emotional support that they feel may be needed for the family.'
Many of the families referred to the BSE Inquiry. They hoped it would identify why the disease had occurred, and alleviate some of the anger they feel. But some recognised that the anger in itself might give them a focus for their grief and it might even be more difficult for later families without this focus.
Family 'In a way I think maybe that helped us all with our angry. Maybe now we haven't got as much to push against. It might actually be harder because the Inquiry is in place.'
To families, nvCJD is very distinct from other strains of CJD. Some of the reasons why they think it is different from other conditions are discussed above. It is important to them that it is recognised as a separate condition to sporadic or iatrogenic CJD. Several were exasperated when the different strains were confused in media reports.
Family 'There's only human BSE that is the equivalent of BSE you know. But you have to keep repeating it over and over ... It's just these things in the paper. You see it every time. You know like sporadic CJD you know. Grandmother dies from mad cow disease you know. So I think public awareness is very important as well.'
Limitations of the study
There are important limitations to this study in identifying the needs of patients with nvCJD and their families. All the accounts were retrospective, often related many months after the patient had died. Families' retrospective accounts of patients' terminal illnesses have been found elsewhere to be moderately reliable50, but it is possible that the perceptions of both families and professionals had changed over time. Some of the families had told their stories to journalists or even appeared on television; their accounts had been told many times before and may have altered in emphasis in the telling. Conversely, before the interview, professionals sometimes said they were anxious they would not remember what had happened in enough detail.
The high media profile and knowledge of other families' experiences could have influenced respondents' perceptions of what had happened. Families meet and discuss their experiences through the CJD Support Network and the Human BSE Foundation. So the narratives were not independent. During the interviews families often compared their experiences to those of other families. Hearing other families' experiences and perceptions may have influenced their memories of events. For example two respondents commented that they had not felt anything was hidden from them. That this was relevant seems to reflect other families' stated perceptions that the diagnosis was 'covered up'.
Families' anger and grief that their relatives had died from the disease may have projected onto their perceptions of the care they received. But they described examples of good care as well as bad care. Where they were critical of patients' care they often described specific incidents, which were often corroborated in interviews with professionals.
Patients' views were necessarily missing from the study. Patients were obviously very distressed during the early stages of the illness, and very physically dependent as the illness progressed. But it also became clear that families themselves had great needs for emotional and practical support during the patient's illness. As one commented,
Family 'The patient lacks for nothing they get everything they need but the family that are left looking after the patient it's them that need more help I would say.'
Families' own needs could thus have overshadowed patients' needs in the interviews. This caveat could also apply to studies of other conditions, but carers have been found to be good proxies for the needs of patients with HIV48. Also, findings from interviews with professionals were similar to those with families, so it seems unlikely that patient's needs have been missed.
Families could have been inhibited from criticising medical care because they knew the interviewer was a doctor. Similarly, respondents may have been unwilling to criticise the National CJD Surveillance Unit because the study was based there. It was explained that the interviewer was not an expert in nvCJD, and the findings seem to show that respondents did not feel inhibited from criticising doctors or the surveillance unit.
A large number of issues were raised by respondents. We have tried to cover all the main areas in this report, but this means that few are covered in great depth.
Differences in the experiences of families of patients presenting before and after March 1996, when nvCJD was described, are discussed above.
Families and professionals gave different perspectives on patients' needs. Both families and professionals were asked to briefly go through what had happened. Professionals, not surprisingly, related events during the episode of care in which they were involved. But families' narratives often began with events some time before the patient's illness was apparent. They gave a picture of the patient's life before he or she became ill, and described the first insidious symptoms of the disease in that context. Perhaps for this reason, the perception on reading through the transcripts was that the initial stage of behavioural and psychiatric symptoms was much longer than the later stage after patients developed neurological signs. In fact both stages lasted a median of six months. Professionals had never known patients before they were ill, they only knew them when they were severely impaired and dependent. Despite these differences in perspectives, families and professionals recognised many of the same problems in managing the patients.
Similarly, to most of the professionals the episode has ended, but to families it is still continuing. They have the loss of a loved one and feelings of bereavement. They also re-live what happened when they meet together and talk over their experiences and are continually reminded by the high media profile of the disease. They still feel very much affected by nvCJD and live with it every day.
This study was limited to patients with confirmed nvCJD, but for every patient in whom nvCJD is eventually confirmed there are 5 others referred to the surveillance unit with suspected nvCJD (personal communication). These patients must have many of the same problems and needs as those described here.
Many of the needs identified here are similar to those described in other patient groups and their carers. The emotional strain of caring and benefit of informal support, 'someone to talk to', is commonly described in studies of the needs of carers35,43,46. Carers also often say they want more information about the condition their dependent suffers from, especially about how it will progress. They think this information helps them to cope better, and enables them to participate as equal partners in caring with professionals41,51. Stress caused by uncertainty about the diagnosis has been described in families of children with cancer and patients with dementia16,38,46. Needs for respite care and for help with practical tasks, like housework, have been described by patients with other neurological conditions and carers of patients with dementia12,36,37,43,46. Needs for financial help are also described in other groups, although less often40. Patients with a variety of conditions often say they value personal attention18,30. Families of other terminally ill patients describe a difficult balance between their needs for help to care for the patient and their desire to care independently45. Professionals caring for other terminally ill patients and those with HIV/AIDS have discussed problems in coordinating care39,46.
However, some of the needs of these patients with nvCJD are different from those described in other patients. The rapidity of their deterioration, and frequent need for reassessment, is unlike many other disorders. The combination of psychiatric and rapidly progressing physical symptoms is also atypical, and makes it particularly difficult to manage them on acute medical or psychiatric units. The high media profile, anger about the cause of the disease and suspicions of a 'cover up' also single out nvCJD. None of these problems is unique to patients with nvCJD, but they all add to the strain of caring and make management more difficult.
Presentation and early management
Many families expressed a hope that in future GPs will be able to recognise nvCJD earlier, and that patients will not be given psychiatric diagnoses initially. But this seems an unrealistic expectation. In most cases, patients genuinely had features of depression or psychosis so it was appropriate for them to be treated as such and to see a psychiatrist. nvCJD is very rare, whereas depression is very common. In fact, as the symptoms evolved, most of the psychiatrists recognised that the condition was organic and sought early neurological investigation. But this does not mean it was inappropriate for patients to be assessed by them. The psychiatric assessment often identified patients' cognitive deficit, suggesting an organic cause. Families' anger about a psychiatric diagnosis reflects the stigma that psychiatric illnesses have. Better public understanding of psychiatric conditions is needed, including recognition that they are 'real' illnesses52.
Most of the patients were seen by a neurologist very quickly once neurological signs were recognised. The median waiting time for a neurology appointment was only 2 weeks. This is much shorter than average waiting times reported for neurology appointments; for example in Scotland the median wait in 1997 was 9.6 weeks53. And it is likely that the waiting time reported here is an overestimate, because the date of referral was often missing and in many cases this was probably because the patient was seen urgently following a referral by telephone.
Families complained of poor care while patients were in acute neurology or psychiatric wards. This contrasted sharply with the better care most had when receiving terminal care. This was the most stressful time for families, when the diagnosis was uncertain and they felt that what was happening was out of their control. Their perceptions of poor care could partly reflect this. But many described specific examples of poor care. These examples suggest that staff often simply did not understand how dependent patients had become. Professionals also recognised that it was very difficult to manage patients with both psychiatric and physical needs. They were very vulnerable in psychiatric units because of their physical dependency and disruptive in neurology or medical wards because of their psychiatric symptoms. Clearly the nursing levels, ward environment and ward ethos were all inappropriate to care for these very dependent patients.
This study found that patients with nvCJD had very great needs for care, and many families felt they had to fight to receive the high level of support that was needed. Patients deteriorated very fast, requiring frequent reassessment of their needs. Coordination of care between different settings was often poor. In the early stages, patients travelled between different psychiatric and neurological 'systems' with little continuity between them. GPs were often not kept informed of what was happening, felt inhibited by a lack of knowledge about the disease, and sometimes had lost the family's confidence when they diagnosed a psychiatric illness early on. The lack of continuity was a particular problem for this group of patients, many of whom were fearful of strangers and needed familiar carers.
It was difficult to find an appropriate place for terminal care for many of these patients. Specialist palliative care services often exclude patients with diagnoses other than cancer, but some specialists have argued that they should accept patients on the basis of need rather than diagnosis54. Where available, specialist palliative care services seemed very appropriate for these patients. It was important for patients to be cared for near the family's home to allow them to visit and share the care. When a suitable place was found, most families reported that the staff were caring and sensitive to the needs of patients and families. This was especially so where specialist palliative care services were involved. Care given in the later stages contrasted sharply with care in acute wards.
Families were closely involved in caring for patients and it was important for them to be treated as partners in providing care. But it placed a great practical burden on them. They needed a high level of practical support, flexible enough to adapt to patients' changing abilities and families' needs for respite. Where patients were cared for at home a very high level of support was needed, but usually no care was arranged before discharge from hospital. Patients deteriorated so quickly that when aids were provided they sometimes arrived too late to be of benefit. The practical help needed was not just care and aids for the patient. Families were spending all of their time caring for the patient and had no time for household tasks. Some received practical help with domestic tasks such as ironing, and others would also have benefited from this kind of help. Families with young children also had logistic difficulties in arranging childcare, but a minority had any professional help with this. Many families also suffered a great financial burden, and few received professional advice on benefits. The financial costs to families are the topic of another study.
Throughout the illness, a common finding was that families sought to retain some control by keeping a sense of 'normality'. It is important for professionals to recognise this, to support them in maintaining their normal routines where possible.
It seems surprising that a minority of families reported that patients had services like physiotherapy or speech and language therapy. Some families may have been unaware that their relative received this service. Some reported that patients were too ill to benefit from it, but to others it was something positive to offer, sometimes contrasting with their perception that the doctors had 'given up' on the patient. Help from social workers was also patchy. Sometimes a social worker set up a care package and was seen as a great help. Other families had no contact with any social worker although they were in need of advice on benefits and other help. In some cases, care was hampered by financial constraints. In particular, incontinence pads were sometimes rationed.
All the stories related by the families were deeply moving, it was evident that they had suffered very great emotional strain during and after the illness. But none found formal counselling helpful during the illness, as they did not want to spend time away from the patient. They did appreciate informal support from nursing staff and carers. Many felt 'dropped' after the patient died and all the input disappeared. But although some were offered bereavement counselling, only one family accepted the offer. A few found they became increasingly distressed some time after the patient had died, and sought counselling then.
Many families had found useful advice and emotional support from the CJD Support Network and Human BSE Foundation. They feel strongly that nvCJD is very different from other conditions. So only other families can understand how they feel and give support and advice. Some found it helped them to offer help to other families affected by nvCJD. But it does place a burden on families to continue to do this, it reminds them of their own grief and means nvCJD is a continuing problem for them.
Some families were distressed by unwanted press attention, whereas others sought media coverage to warn others about the condition and found this helped to air their feelings of grief and anger. The professionals saw the media coverage as a threat and tried to avoid contact with them. They wanted to preserve patient confidentiality and sometimes commented that coverage was insensitive or unfairly critical. Sometimes professionals advised families to avoid the press, but this was interpreted as an attempt to 'cover up' the disease and was a barrier between families and professionals. Effectively most families had no help to deal with the press. They would clearly benefit from some help with this when press demands are intrusive, but this should perhaps be independent of the professionals, given their suspicions that professionals were trying to cover up the disease.
Information needs and communication
Nearly all the families wanted more information throughout the patient's illness. They wanted more information about investigations and results, about the diagnosis, prognosis, and available services. They wanted to be given information proactively, rather than being expected to know what questions to ask. It was too much to take it all in at once, they wanted to get more information about problems as they developed. Having this information was another way for families to feel more in control of what was happening and helped them to be equal partners in patients' care.
Most families lacked information on what the investigations entailed and what the results meant. Patients were not competent to consent to procedures, and families had to do so for them. So they needed a better understanding of what the investigations involved. Patients were often frightened of the investigations. Families thought they could help, by understanding how they might react, and sometimes by accompanying them.
There was a very strong perception among the families that the disease was being 'covered up' by the government. This was related to their belief that the government is responsible for the disease because it did not prevent the sale of beef contaminated with BSE. This perception made them mistrust professionals, and suspicious of any delay in reaching the diagnosis. This added to the stress caused by uncertainty about the disease, anger about early psychiatric diagnoses, and the very distressing symptoms these patients had.
A striking finding of this study is the very great importance of having a diagnosis. Families would value knowing the diagnosis earlier, but it is clear from the interviews with professionals that this may be impossible. Even after the development of neurological signs it is still a difficult diagnosis to reach. Diagnosis in the early cases posed a particular problem, before nvCJD was a recognised condition.
Many families recalled being told the diagnosis in an insensitive way. Of course their memory of this will be influenced by the great stress they were under at the time, their mistrust of professionals and the fact that it was such terrible news to hear. But the accounts do suggest that this could be improved by fairly simple measures like making sure family members are not told when they are alone, leaving time for questions and arranging follow up interviews to answer later questions. It also helped when families felt they had been kept well informed about the patient's investigations. Most importantly, where possible families should hear the news from a doctor with whom they already have a good rapport.
Families differed on the value of the information booklet. Where it was thought helpful, it was because it described symptoms that matched families' experiences of the patients. Many families found the most useful information and advice about how the disease might progress came from other families with first hand experience of it.
Most of the neurologists said they had enough information about the disease, but the other professionals wanted to know more. Many said they felt 'in the dark' and lacked confidence in managing patients' problems, although they often turned out to be similar to other patients with terminal conditions. There was particular confusion over infection control procedures and arrangements after death, which sometimes caused more unnecessary distress to families.
As the study originated from the National CJD Surveillance Unit, we should be cautious in drawing conclusions about its role. It was seen to give a second opinion and both professionals and families seemed to think this useful, although most recognised its main role was to study the epidemiology of the disease.
There was confusion about funeral arrangements, and many families were distressed that they were not allowed to view or touch the body after the patient died. Sometimes the families were aware of logistic problems, usually in signing the death certificate. These could have been dealt with by the professionals, without causing further distress to the grieving families. No rationale for the advice not to touch the body was given to families, who had had close contact in the months before death without being warned of any risk. Current guidance states that there is no risk from merely viewing a body, but there is a theoretical risk from touching the body after a post mortem examination, when the cranium has been opened. The dissemination of clear guidance about this would alleviate much of the confusion.
Appointing a key worker to each patient would help address the poor coordination of care between settings. Patients receiving terminal care in hospital or hospice would benefit from having a named key worker, as well those cared for at home. The ability to arrange services as they were needed could be devolved to a named professional. They could coordinate the large number of professionals involved and reassess patients' needs as they deteriorated. They could provide information about the disease as it progressed and address practical problems. They could build a relationship with patient and family, and provide informal emotional support. The key worker could continue to provide emotional support after the patient had died, when many families felt 'dropped'. In the cases included in this study, care was coordinated by social workers, nurses and GPs. Knowledge of local services and the ability to develop a relationship with the family was more important than the discipline. But the key worker role demanded a heavy time commitment. Whoever takes it on would require time to support patient and family and communicate with other professionals.
The key worker would need information about nvCJD, how it might progress and what services might be helpful. Some of the families suggested that there should be a national team of professionals to act as key workers for patients with nvCJD, so they could build up specialist expertise in their care. But they could not then have the knowledge of local services in each area. And it would be difficult for professionals who were centrally based to provide the intense input needed by patients and families. Patients deteriorated so rapidly they needed frequent reassessment and families needed a high level of emotional support, continuing after the patient died. It would be difficult to provide this level of support from a professional who was not local to the family. However there is certainly a need to develop expertise in the care of patients with nvCJD.
One solution might be to have one or two professionals, at national level, who would develop knowledge and expertise about the care of patients. They would act as coordinators, to work with, train and support individual key workers. They would develop links with national services and be a source of information and advice.
The professionals caring for patients with nvCJD need emotional support as well as information. It adds to families' stress if professionals are distressed. The key worker role could include making sure professionals had access to support, and key workers themselves would also require support, from their local managers or the coordinator.
Coordination of care
- All patients with a clinical diagnosis of nvCJD should have a key worker allocated to coordinate care for patient and family, regardless of the setting in which care is given.
- The key worker should be a professional with knowledge of local health and social services, who is able to develop a good relationship with the patient and family and is allocated time to devote to them. Their discipline will depend on local circumstances, but could be social work or nursing.
- Where patients are discharged home, the key worker should be appointed, and a care package arranged, before discharge.
The key worker's role should include:
- Assessment and reassessment of patients' care needs
- Assessment of the family's needs for other practical help and childcare
- Coordination of professionals providing care
- Advocacy for resources to meet the needs
- Ensuring flexibility of the care package, and availability of respite care
- Providing advice on financial help and benefits
- Providing information about the condition to the family, as it develops
- Providing emotional support to the family
- Providing information and helping the family with end of life decisions
- Helping with funeral arrangements
- Continuing emotional support after the patient dies, and assessment of need for formal counselling
- Regional or national coordinators should be appointed, to develop specialist expertise in nvCJD, and provide information and support to key workers.
- The value of advice and support from the other families involved in the CJD Support Network and Human BSE Foundation should be recognised. The coordinators should work with them in developing expertise on nvCJD and families should be informed about support available through their helplines.
Care on acute wards
- The rapidity of patients' changing needs should be recognised. Patients with suspected nvCJD should have their care needs frequently reassessed, and help given to mobilise, to feed and for incontinence as appropriate. Where they are disturbed on an acute ward, a registered mental nurse should be provided to help care for them.
- The urgency of placement for terminal care should be recognised, and transfer from acute investigation wards expedited.
- Terminal care should be provided as near as possible to patients' homes.
- Specialist palliative care services should be involved wherever possible.
- Families should be regarded as equal partners in caring for patients. They should be helped to provide as much of the care themselves as they wish. Their opinions about patients' changing abilities should be central to assessment of their needs.
- Families should be offered advice on practical care of patients, including movement and handling, and managing challenging symptoms. If caring for patients at home, they should have access to respite care and night nursing.
- Families should be offered practical help with household tasks and childcare as well as help with care of the patient.
- The rapidity of patients' deterioration should be recognised. Their needs should be frequently reassessed and any necessary aids or equipment provided rapidly. Provision of respite care and night nursing should similarly be provided promptly when needed, without undue delay.
- Familiar carers are needed, as patients are often fearful of strangers. A small number of familiar staff should provide care for each patient, rather than many, frequently changing, staff.
- End of life decisions, including feeding, should be openly discussed with families by informed staff.
- Clear guidance about handling the body after death should be disseminated. It should be provided to key workers to be discussed with families, carers and undertakers.
- Families need a high level of emotional support, during and after the illness. Professionals providing care of patients should provide informal support, continuing after the patient dies. They should also offer each family member more formal counselling, especially after the death.
- Professionals caring for patients also need emotional support. They should be offered counselling.
- Families should have access to advice on dealing with the media. This should be independent of the professionals providing care.
- Families need information about the condition, how it will progress and what therapies might be helpful. This should be offered in a proactive way, as the condition develops. The key worker should act as a point of contact to answer questions at any stage.
- Professionals caring for patients with nvCJD, or suspected nvCJD, should be aware of families' suspicions of a 'cover up'. They should offer clear and candid information at all stages of investigation and care.
- Patients and families should be given clear explanation of all investigations in lay language, including what the investigations involve and the results.
- Families should be told the diagnosis of nvCJD by a doctor they know and have a good rapport with. Care should be taken that they are not given conflicting information by different doctors. One family member should not be told when he/she is alone. They should be offered support, time to ask questions and a later interview to discuss it further.
- Patients' GPs should be kept informed of the patient's condition and care arrangements, because of their role in terminal care, and in continued emotional support to the family.
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INTERVIEW GUIDE FOR KEY CARERS
Explain reason for the interview: want to define the needs of patients and carers of new variant CJD
Will be used to make recommendations to improve care of future patients
Comments will be written down in full and may be quoted but will not be attributed to any individual
Can decline to answer any questions and can end the interview at any time
Seek permission to record the interview
Initial concerns and symptoms
- What first led up to [name of patient] seeking professional help?
- Who was the first professional that he/she sought help from?
- What advice/help was offered?
Go through stages of illness.
- What happened?
- What problems did [name] experience?
- What practical help was given?
- How helpful was it?
- What information was given?
- What emotional support was given?
- What help and support do you feel was needed at this time?
Investigation and diagnosis
- Were the investigations and results explained?
- What were you told about the diagnosis?
- What did you understand by what you were told?
- Were you given any information about the progression of the disease and what to expect?
- Were you given the booklet about CJD? [show booklet]Was it helpful?
- What did it mean to you to have/not have a diagnosis?
Later stage of illness and care
- Can you describe the symptoms and problems [name] experienced as the illness progressed?
- [prompt with symptom checklist]; for each ask problem ask: what help was offered, was it helpful
- Did you have any contact with CJD Support Network/HBSE Foundation?
- What services and support did you receive?
- [prompt with checklist of services]; for each ask what was helpful/unhelpful about it
- [if 'good' care]: what did you value about the care/what would it have meant to not have it?
- Did you receive any advice on benefits?
- Were any aids/adaptations made to the home to help care for [name]?
- Was there anyone in charge of co-ordinating services?
Management after death
- Were there any problems with the funeral arrangements?
- Were you offered bereavement counselling?
- What help/support would you have found useful at this time?
- Did you have any help to deal with the media?
- What would improve the care?
pain (ask where)
invol. movts/ unsteadiness
not eating/ nausea/vomiting
DN/ other nursing
hosp liaison nurse/ SW
help with laundry
other practical eg housework
TELEPHONE INTERVIEW SCHEDULE FOR PROFESSIONALS
Explain reason for the interview: want to define the needs of patients and carers of new variant CJD
Will be used to make recommendations to improve care of future patients
Comments will be written down in full and may be quoted but will not be attributed to any individual
Can decline to answer any questions and can end the interview at any time
Description of involvement
- At what stage of the illness were you first involved in the management of this patient/client?
- Could you briefly describe your involvement?
- What particular problems did you have in looking after the patient/client and family?
- Who else was involved?
[if relevant cover early, late and terminal stages of illness separately]
- Can you describe the symptoms and problems that the patient/client experienced?
- What practical and emotional support did the patient/client and family need?
- What support was available?
- Were there any organisational problems getting suitable care or advice?
Information needs of professional
- Where did you get information about the condition?
- What further information would have helped you care for the patient/client?
- Did you have any contact with the CJD surveillance unit?
- Did you have any contact with the CJD Support Network/HBSE Foundation?
- Were they helpful?
Communication between professionals
Roles: yourself and others
- What role do you think you should take in such cases
- Roles of others
- What would have helped you in the management/care of the patient/client?
[prompt with: information, sources of advice/expertise, practical help available, organisational issues]
CATEGORIES FOR SORTING DATA
- Initial management and investigation
- Palliative and terminal care
- After death
- Coordination and continuity of care
- Practical support
- Emotional support
- Importance of a diagnosis
- Dealing with the media
- Why families think nvCJD is 'different'
Glossary of Abbreviations
Bovine Spongiform Encephalopathy
New variant Creutzfeldt-Jakob disease
National Creutzfeldt-Jakob disease Surveillance Unit
Human BSE Foundation
Magnetic resonance imaging
Percutaneous endoscopic gastrostomy
Post mortem examination
Registered mental nurse