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The Gauchers Association has long realized that the role played by those close to Gauchers sufferers, sometimes as carers, can have moments that are difficult, upsetting and challenging. Because of this, two members of the Association, Laura and Erica, both counsellors, decided to record a conversation about their respective experiences of Gauchers - Laura as a wife of a Gauchers patient and Erica as a mother of a sufferer. The names of these two members and the people to whom they refer have been changed. Laura started off by reminiscing how she and Erica had met.
Laura: We were at an early meeting of the Gauchers Association in August 1991. I caught your eye and thought to myself how knowledgeable and aware of this disease, Erica, you were, while to me it was a whole new experience.
My mind wandered back. Roger, my husband, had been told on his 60th birthday in August 1989, that he had cancer. The oncologist later said that he actually had two incurable diseases: the cancer that could be treated, and the Gauchers, which at that time could not. What on earth was this incurable unheard-of disease? We later found out almost by chance that it had been in Roger's family for years.
At that Gauchers meeting I felt aware that here at last was not only you but a whole group of people who understood how isolated and overwhelmed I was feeling.
Roger's brother, a psychiatrist no less, had carried his own Gauchers as a stigma, a guilty secret, to his grave. Whether the Gauchers had contributed substantially to his death is unclear; what was clear was that he had really suffered. He knew he had it and shared the information only with an uncle, also a physician, who, on hearing that Roger had it too, shared that secret not with us but with Roger's oncologist instead. His family could not acknowledge that we were adult enough to be told the truth and this made me furious. It was as if they were keeping a disgraceful secret tightly closed. Even to this day they will not discuss it - all the more shocking as some of them are doctors.
Erica: Laura, if you could sum up your feelings back in August 91....
Laura: Relief. A huge sense of relief. I felt that you were all here to listen and that anything that I would want to say could come tumbling out. I knew then that I'm not alone. At last I can be vulnerable. I can share my fears and anxieties and I know I'm being listened to and understood. And I know that in turn I will hear others' experiences and learn from them.
Erica: You know so much is being done with self-help groups, meetings, conferences and the like, yet we each have to live our lives between these meetings. There are the times of being alone, of being fearful, anxious, angry; how do you cope with that?
Laura: At this precise time Roger is more severely affected by the Gauchers than the cancer, and has started on Ceredase. Therefore I, being in a supportive role, have to live with the constant presence of doctors, drips, needles, hospitals. But it's a two-sided coin. There is the pain of seeing a loved partner having to cope with the invasion that the regular and frequent infusions become; against this there is the exhilaration of watching symptoms reduce at an amazing speed. Day to day it fluctuates. I do the best I can. And talking like this really helps.
Lives profoundly affected
Erica: Now that the Association has been going for nearly three years, you and I feel strongly that the position of those of us around Gauchers patients has not really been addressed. You as wife and I with a daughter Debra, who has suffered real effects for twenty-seven years, have had our own lives profoundly affected by Gauchers. As practising counsellors we know the value of really being listened to and we hope that describing our experiences and feelings will be a gain not only to ourselves but also to others in similar positions.
Laura: You can only talk about your own constant fears and great anxieties over the years - as a mother it is a totally different concept from what I have had to contend with. You've had your child look at you with eyes which says 'Mummy, make it better'.
Erica: That's the half of it - the other half is 'I would if I could but I can't'. When she was little I was prepared to take it over from her if it would only help her. I've spoken to other mothers and it seems that so many of us, when we first realized the nature of our children's illness, felt deeply and profoundly guilty for their condition.
Debra's Gauchers was diagnosed at age 19 in 1979, only after she had been very ill off and on for twelve years. Having a label for the awful, inexplicable symptoms and operations meant moving from an unnamed to a named terror. Giving it an identity enabled me to try and make sense of the seemingly meaningless jumble of symptoms I had achingly watched her endure so painfully for years.
I then felt and still feel great pain that I had contributed even unknowingly to the enormous difficulty and debilitation that the Gauchers has caused in Debra's life.
By 1985 I found my way to the annual conference of the Tay Sachs and Allied Diseases Association in New York. I sat in the huge glitzy hotel dining room and realized that virtually all the parents present had lost at least one child to that terrible illness, Tay Sachs. And here I was with a daughter who, though ill, was very much alive. I felt a fraud and started quite uncontrollably to cry but it was those mothers who held me and were generous in encouraging me to express my own grieving. Of course that happened BC (Before Ceredase!). At that time I was living with constant reminders that my daughter's Gauchers was chronic, progressive, untreatable and, in that form, almost certainly life-curtailing.
Laura: It must have been an awful time for you. How, if at all possible, were you able to make sense of it?
Erica: On diagnosis I realized that the only possible way was by being informed and in touch with those who knew about Gauchers. In the late 70s in England that was not easy. I experienced what others I have spoken to came across - 'the run-around'. It was so difficult that in order to have Debra's condition evaluated we had to go to New York. No one with experience of Gauchers who I was put into contact with in England would or could treat Debra knowledgeably.
The real breakthrough in this context was being introduced to the Tay Sachs people both here and in the States. Through them I finally had access in a systematic way to developments and trends in the treatment of Gauchers.
One of the most frustrating elements during the 80s was the impression given by the few relatively informed doctors that Debra was one of perhaps half a dozen sufferers in the UK. It was only when the Gauchers Association was formed that we realised that it wasn't so - there were many more. I know this impression has been experienced by others - as was the relief when we met one another. Somehow sharing the experience with others eased the burden.
Debra's illness fluctuated as a topic of discussion between us over the years. There were times she preferred to withhold all information about her health from me. Being an adult then she was fully entitled to do this and hurtful as it was to me I respected her position. At the same time I felt compelled to explore all avenues of information and feedback. Perhaps this was a way to alleviate the deep and terrifying agony of helplessness that I was living with. It was clear that I needed to know it all - regardless of when or if I could share it with Debra.
There were periods of hospitalization for Debra that were very painful, traumatic and exhausting. I had a sense of being on stand-by for her, hoping all the time, that science would one day catch up with her while she would still be well enough to benefit from a breakthrough in treatment. I read everything about Gauchers in journals, newspapers, magazines. Yes, Laura, you were correct, I was informed. It was my way of making the unbearable bearable.
Hard to watch your child suffer
It's so hard to watch your child suffer. Time doesn't make it any easier. But being able to discuss this with others who were doing the same was such a relief during the years when effective treatment for Gauchers was only a pipedream - or perhaps I should say only a test-tube dream!
Laura: It's really important to know it's OK to say that things are difficult, to express your pot-pourri of anger, loss, disappointments, fears, anxieties, and also hope, joy and relief of varying kinds. Sharing thoughts, feelings and expectations is OK wherever possible.
Erica: I couldn't agree more. My own situation, like that of so many others, involved years of hopelessness in terms of long-range expectation.
For me the advent of Ceredase was truly a lease on life - an unbelievable luxury within the anxieties we felt. And while certain fears still remain - medications bring their own difficulties and experts warn that Ceredase may prove to be no exception - people are getting better, their health improving, spleens getting smaller, pain reduced - even removed.
I was asked in April 92, after Debra had her very first Ceredase infusion, how I felt. My answer was 'I'll go to bed tonight with the certainty, for the first time in twenty-five years, that tomorrow morning she won't be a little bit worse'. Today when asked, yes there is a burden, there is still fearfulness of the known and the unknown, but above all there's life and there's hope.
Source: Gauchers News July 1994
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