A fanily story
John Shelley died in January of Sporadic CJD. His family says of him "Our dad was a hardworking, generous and thoughtful man who was loved and respected by all who knew him. Dad always made sure us three children had everything we needed and took us on many great holiday".
John's story, as told by Angie, Mindy and his partner Donna, can be read here. John's story An American view
Florence Kranitz, President of the American CJD Foundation and Deana Simpson, founder of CJD Insight in
America have written an excellent article,
Using Non-Pharmacological Approaches for CJD Patient and Family Support as Provided by the CJD Foundation and CJD Insight in the CNS & Neurological Disorders journal.
Florence ’s husband died of Sporadic CJD and Deana’s family is affected by Genetic CJD.
Their article can be read here
A Family’s Guide to Surviving CJD.
A Brief History
My wife had Human Growth Hormone (HGH) treatment in her early teens. At the time both she and her parents were told it was completely safe and there was nothing to worry about. Her HGH treatment was halted in 1984/5 on the discovery of some deaths in the
US relating to HGH and CJD. She and her parents received a letter from doctors in the early 1990’s informing them of a slight risk but with no real cause for concern.
We got together in April 1994, and married in September 1995. Karen told me she had had HGH treatment and that there was a possibility that CJD may develop. We said we would worry about it if it happened. Towards the end of 2003 Karen was feeling unwell and she said she was not feeling right. This manifested itself as a persistent cough, sore throat and reduced hearing.
This feeling unwell, which she termed feeling wobbly, continued into early 2004 when she required my support to walk. From the end of April 2004 I would not let her drive. At this time she consulted our GP who diagnosed vertigo. She was given a course of medication which was useless. On two further occasions she saw the GP who continued with sea sickness pills and vertigo treatment. I went with her on 3rd June 2004, and informed the GP in no uncertain terms that this was not vertigo. At this time he looked at the back of her eye and at her gait. At this point was the first mention of CJD.
She was seen by neurologists at
our local hospital in June 2004, who confirmed a diagnosis of Iatrogenic CJD. Karen agreed to undergo therapy with pentosan polysulphate. This required the insertion of a shunt pump into her abdomen, and a catheter into a water ventricle in the brain. Narrow tubing up through the abdomen connected the two. .
Karen had regular pump refills but continued to deteriorate over forthcoming months and she passed away on 5th August 2006. Approximately 32 months after onset of the symptoms.
Make it go away
It has been just over a year since my mother died from sporadic CJD in June 2004. This has been an intense period of difficult reflection as I try to fathom what happened and why it happened. The first signs of the disease had only surfaced in November 2003 when Mum began to forget a word or two mid-sentence. Nothing too peculiar about that, I forget my words all the time. Mum and the family just put it down to age – she was 60. By early 2004 more symptoms were appearing. A good car driver all her adult life, Mum began to have difficulty in judging the distance of oncoming cars. So she stopped driving. Then she started to lose weight and had bouts of nausea. More forgetfulness followed and she was referred to a neurologist and on to a psychiatrist who could find no obvious condition that could be causing these problems. By April, Mum was having some difficulty walking. A return visit to her GP resulted in the frighteningly ironic diagnosis of “It’s all in the mind”. So near, and yet so far - it wasn’t in the mind it was in the brain.
May 2004 and in sheer frustration my stepfather changes Mum’s GP to one at another surgery in the small Welsh village in which they lived. This second GP agrees that whatever is happening needs more investigation and an appointment is made at an outpatient’s clinic at the local
Regional
Hospital . However, the rate of deterioration was becoming so rapid that Mum was admitted to hospital as an in-patient on Friday 14th May. She was able to walk onto the ward and in that first weekend she even helped some of the older patients whose mobility was worse than her own. She always offered a helping hand to strangers.
All sorts of tests were being done, including an MRI scan. All of these were throwing up negative results, yet all the time the patient continued to decline. Within those first two weeks in hospital Mum lost her mobility and the ability to swallow solid food and could no longer feed herself. Then the tremors started. With all testing options beginning to dry up an EEG (brain) scan was performed on Wednesday 26th May and the cause of the problem was immediately apparent. On Friday 28th May, my stepfather was called into an office by the specialist and was told that his wife had CJD – an acronym for a disease with a long, almost unpronounceable foreign name. At that time, no one in the family knew much about this disease. Now, we are lay-experts in something that to be frank, we had no desire to be experts in.
Stepfather was in a daze, but was assured that the family would receive help and support from a specialist unit in
Edinburgh . It was bank holiday weekend so we all gathered around in the hospital not really knowing what was ahead for Mum and ourselves. There is no treatment for CJD, only comfort can be offered to the patient. A rare drug called Piracetam had been prescribed to help alleviate the shaking and involuntary jerks but the
Regional
Hospital did not have any in stock. When questioned, they told us that they might not be able to get a supply 5 or 6 days. I really had to insist that the drug was supplied that very day and after several assertive conversations with the ward sister a packet of the drug was found at another hospital about 35 miles away. I was asked if I would go and collect it myself! Of course, I got into my car (luckily I have one) and got the drug.
Mum couldn’t swallow the tablets that night although the nursing staff begged her to. It was only 24 hours since we had been told of Mum’s illness and already it seemed that I knew more about the disease than the staff on the ward. Thank God for the Internet and thank God for the CJD Support Network whose website was by far and away the most informative. I told the staff that CJD patients have difficulty in swallowing and suggested that the tablet be crushed in lemonade. That is how we administered Piracetam from that point onwards. I stayed with Mum overnight and shared this shift with one of my brothers for the next few days, realising that perhaps this small
Regional
Hospital was out of its depth in caring for a patient with CJD. My Mother needed an advocate for her needs and I was concerned about that advocacy especially during the nighttime hours. The following day Mum was offered a Paracetomol tablet – her prescription had been misread!
By Wednesday Mum had deteriorated so much that she was transferred to a neurological unit some 50 miles away. I had to insist that she was sedated for the ambulance journey as she had developed a fright that is a characteristic CJD symptom. At this specialist unit we were told that a lumbar puncture would be performed to help establish the earlier CJD diagnosis. They said that there are some tropical conditions that can have similar symptoms. It seems strange now but I had this hope that Mum really had Malaria – because that can be cured and CJD cannot. A few days later a junior doctor sat me down and said, “Our tests on the lumbar fluid are negative”. I was delighted and asked if Mum does not have CJD, then what has she got? “No, when I say the tests are negative Mr.Bradley what I mean is that they are negative to anything else”. CJD it is then.
A week since the diagnosis and the family have still not received any advice as to what the future holds or what help might be available for stepfather in caring for Mum. We still have not heard from “
Edinburgh ” and we are not entirely sure who they are exactly, except that they do some sort of surveillance. In fact, it has taken a week for us to have another brief meeting with the specialist. If it wasn’t for the Internet we would have been very lost indeed.
That first weekend at the neurological unit and I am sitting with Mum when the nurse dispenses Paracetomol. Déjà vu does not kick in so I ask what it is for. The nurse says it is Mum’s prescription drug and for a second time I have to point out that it is not.
About a week after arriving here, Mum becomes very agitated indeed and even though she can hardly speak suddenly blurts out a clear request, grabbing stepfather by his collar. “Make it go away!” she says - by which she means her illness. I beg the doctor to sedate my Mother but the doctor is most reluctant to do this, saying, “We like the patients to find their own equilibrium”. However, in front of Mum we insist on sedation and each night thereafter Mum was sedated to help her (and consequently us) to sleep.
Mum is deteriorating rapidly. Each night when I start the long drive back home with stepfather we ask each other if she can possibly get any worse, knowing that the following day she will have declined yet further.
Two weeks after the diagnosis and we finally receive a visit from “
Edinburgh ”. These two weeks have been a hell for us, so
Edinburgh seems to us to be a bit slow. Besides, no help is being offered to us! There is though the matter of a question and answer session for a survey of theirs so this meeting is more a case of us helping them. At the time I felt angry about this lack of support coupled with a somewhat mercenary need for survey information. This anger was compounded by some basic errors of hospital care that did not seem to accommodate the critical condition of a CJD patient.
The following day Mum’s usual evening medication was not dispensed until midnight. Admittedly, the ward was busy that night but it seems unlikely that there was a patient on it so critically in need of her medication than the shaking jerking frightened woman that my Mother had become. We did not leave her until the Piracetam and the sedation began to take their effect.
That weekend, Mum continued a decline that was more and more pitiful to witness. I felt that she had lost her eyesight, as it seemed she could not see the utensils when she was being fed. The shaking and the jerking were getting worse, despite the stronger doses of the drug.
Within a week, Mum was transferred to a hospice in her locality. How relieved we were to get her closer to her home and in a quieter, calmer environment. She loved peace and quiet at the best of times. Morphine was administered within a day of arriving and mercifully Mum slipped into a coma. I wondered if Mum would drift back out of the coma but knew that would be cruel, the devastating march of CJD would be continuing and a coma was a much better place for her to be – if not for us. We combed her hair, put her make-up on and even painted her nails. She was the most fastidious of people and would have wanted us to make sure that she looked her best at all times.
Mum died on Wednesday 23rd June 2004 in stepfather’s arms. She just slipped away, her pulse and her breathing just got weaker and weaker. It was the best possible end to the worst possible of all diseases.
The Coroner ordered a post-mortem, to be followed by a full hearing. I had been prepared for this possibility in one of many conversations I had been having over the last few weeks with the help-line at the CJD Support Network. I knew that the idea of a post mortem would upset my stepfather so made sure that I was with him when the Coroner’s officer arrived. We considered the consent form for the post mortem and made some changes to it. There is a part of the form that states that photographs can be taken so we scribbled an instruction in the margin that photographs could only be taken of microscopic samples of tissue and not of the body.This is probably what is meant by ‘photographs’ anyway but the form is not specific about its meaning. Mum was camera and publicity shy and she would have insisted on similar safeguards whilst I also know that she would have approved of medical research. Another part of the post mortem form deals with the tricky issue of organ removal, never an easy subject for the bereaved family of a CJD victim. The form states that organs can be removed as part of the investigation but makes no mention of their return. Again, we scribbled in the margin a list of the organs that should be returned to the body after the investigation. That hand written list did not include the brain. We were not giving overt permission for the permanent removal of the brain, but we were not denying it either. This seemed to be the best way of dealing with the issue. Besides which - I rationalised with stepfather - that if the brain were removed from Mum’s body then the disease would also be removed. Whilst Mum approved of medical research and always used to say that she would like to leave her “body to science”, the decision to allow this is not easy for those who are left to give it.
The Coroner’s hearing was a long time coming and 6 months passed. At the hearing the Coroner made a number of ill-informed remarks about CJD, which only served to add to our distress at a public hearing. He stated that Mum had died of a variant of ‘mad cow’s disease’. This was reported on by one of the local newspapers. I wrote to the coroner and pointed out that Sporadic CJD was first discovered in the 1920’s and that the variant of CJD that has been linked to BSE in cattle was discovered in 1996. Therefore, Sporadic CJD is not the variant. Further, I objected to the use of tabloid English in a Coroner’s court, irrespective of what form of CJD the victim has died of. I asked the Coroner not to use this term again if any type of CJD case was being investigated, as it is not medically correct and would not be helpful to any family who are coming to terms with the devastating and cruel death they have been witness to. There was a reply to my letter, apologetic but vague. However, I am delighted to report that we did achieve a small victory at this time. We were able to contact another local newspaper with a much larger circulation figure and a later publication date. Stepfather voiced his concern to the editor about the amount of personal information in the other ‘paper’s reporting and about our objection to the term ‘mad cow’s disease’. This newspaper editor was a gentleman of the press, having listened to what stepfather had to say he agreed to print a version of the story that was acceptable to us and which would’ve been more acceptable to Mum!
So why write a brief history of my Mother’s dismal experience?
I write only with a spirit of constructive criticism, in the hope that the care and support of a person with Sporadic CJD can be urgently reviewed and improved. Also, I can make some recommendations that would improve the support offered to the victim’s carers. First, in this modern age, a family who are told of this terrible diagnosis should be assigned a Support Worker immediately - there and then. This person could be based remotely (the rare nature of CJD might dictate that this would be the case) and would be required to contact the family using the telephone (it’s what mobiles were invented for) on the day that the diagnosis is made (even if that day is a Sunday and the time is 8 p.m). The specialist making the diagnosis should be required to gather the contact details of the next of kin and these should be passed to the Support Worker’s team on a 24-hour national number. In other words, carer’s support should be ‘on call’. I would like to see help, advice and support becoming immediately available. Not everybody has access to the Internet and even those that do may not find the information rich repository that is the CJD Support Network website. A Support Worker could make sure that information and options for care are immediately available. More importantly, such a worker could answer the numerous questions that will obviously arise on an ongoing basis.
Secondly, a similar level of support and instant advice should be made to the nursing staff that will be caring for the patient. This action should also be the responsibility of the Support Worker’s team.
In all cases, I cannot over emphasise the need for urgency. The alarming deterioration of a person with CJD cannot wait for drugs to be found and delivered and a family should not have to wait for weeks for their questions to be answered. In 2005, in a developed country like ours there really can be no excuse.
Personally, I decided to get involved with the CJD Support Network – though this was difficult so soon after my Mother’s death. During my Mother’s illness we learnt a few things that might help to improve the comfort of the patient and no doubt other carers learnt good practise also by default. As a non-medical, non-professional lay person I have decided to compile practical advice in an easy to read booklet so that others who are plunged into this awful situation will have a better starting point. For instance, we learnt very quickly that Mum could not decipher the patterns on the clothing we were wearing and could in fact become frightened of them. So we decided to wear plain clothing at all times. Also, we found that switching her bed side light off at night did not help her as she became alarmed in the dark (I learnt that this is called ‘sun downing’) and so we always left her light on just in case she woke up and needed to see where she was. It’s little things like this that can make a big difference to the comfort of the patient. If you have experiences like this that you would like to share please contact me via the CJD Support Network
If you need any help or information about any type of CJD please contact Gillian Turner at the CJD Support Network help-line. I would like to state publicly that Gillian provided me with a support that I passed on to my family and hence on to my Mother during the terminal stages of her illness. This made an immeasurable difference to my Mother’s standard of care.
Finally, the marvellous work that is undertaken by the ‘Network always requires more money. Please, if you have any money raising ideas contact our Gillian. To all people who have been affected by this terrible illness I offer you my wholehearted support and solidarity.
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