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Matthew was eight years old when he had a bone marrow transplant to correct his Gaucher Disease at the Westminster Children's Hospital in 1985. Although now considered an inappropriate method to treat Gaucher Disease because of the high mortality rate caused by imperfect donor matches and the advent of enzyme replacement therapy, Matthew is 27 years old, healthy and has produced a daughter of his own.
I was diagnosed aged four years old. I was taken to my GP for an unrelated matter and the doctor noticed my enlarged spleen and liver. He organised tests and the diagnosis came back as Gaucher Disease.
My parents were told my future looked bleak, I would be in a wheel-chair by the age of 10 and I would be lucky to make it into my late teens. I also suffered from joint pains in my knees, legs and hips. They were described as growing pains and I even had an exploratory operation on one knee to see what the trouble was.
When I was about eight, my GP referred me to see Prof Hugh Jones at Westminster Children's Hospital where a new method to treat metabolic diseases called bone marrow transplantation was being performed.
My sister proved to be a 98% perfect match and while I was being prepared for the operation, she donated some of her own bone marrow taken from her pelvis. This was then infused into me it was like having a blood transfusion.
I had to be kept in complete isolation with everything sterilised. My room was very small but it even had a Baby Belling cooker in it so that special irradiated food could be cooked, usually tinned food. As it took one week for bread to be sterilised, it was pretty stale by the time it reached me. Even my teddy bear had to be irradiated and as this also took some time, he left home before me so that he could be at the hospital to greet me.
The doctors and nurses were wonderful but because they had to wear protective clothing, I only saw their faces from the bridge of their noses to their eye brows. One day my primary nurse Abigail walked into the room wearing normal clothes. I knew that this was wrong and shouted at her to get out. But she explained that my own immune system had successfully kicked in and I did not need barrier nursing any more. I was kept in isolation for about two months and in hospital for five months but my dad stayed with me throughout that time, camping out in a local nurses' home. He especially knew that bone marrow trans-plantation was a dangerous procedure and that children with other diseases in the ward did not survive.
Two weeks after I came out of isolation, a new state of the art isolation centre built specifically for bone marrow transplants was officially opened by Princess Anne. I was the only child well enough to be presented to her so I went back into isolation for her visit.
I have a video of her visiting me and there I was, a pale moon shaped faced, child lying on a bed Princess Anne was sitting on. I remember the new unit had no doors as the sterilised air blew out of the opening, preventing impure air from coming in.
Treated as an Adult
I was always kept fully informed and treated as an adult by the staff which helped a lot. I was told what was happening and what medication I was to take. I was told that my white blood count would be zero after the operation and together with the medical staff I watched it increase although sometimes there were dips too. I was aware that I was undergoing a dangerous procedure.
I can't remember any of my life before the transplant and when I speak about my earlier years before going into hospital, it is what my parents tell me. I had to take steroids and became quite chubby for a year. I also remember having to swallow some vile medicine which was put into a syringe and squirted into my mouth.
I used to be washed with Hibi Scrub and I hated the smell. To this day, if I smell anything like it, I feel strange.
Before the operation I was visited by a girl called Solette who had had a bone marrow transplant for Gaucher Disease. It was as if she said: 'Look at me. I got through it.' I think we were among the first children with a metabolic disease such as Gaucher Disease to have a bone marrow transplant. (Since the arrival of enzyme replacement therapy, the need for bone marrow transplants for Gaucher Disease is not considered appropriate for most sufferers).
Now I am a Father I am now 27 years old and have no symptoms. My enzyme level is completely normal. I have dry eyes which were caused when I developed a host donor graft reaction soon after the operation and I also developed dark patches on my skin at that time. I was told that, because of all the irradiation I had, I might not be able to have my own children. I met my partner Leila in 1998. She already had a son called Callum who I adopted as my own, so I was not concerned.
However we did discuss the possibility of having a child together and decided I should be checked. But before the appointment arrived, Leila became pregnant and we now have a lovely little girl called Grace who is 2 years old.
I consider the transplant team at the Westminster Hospital as my heroes and without them, I doubt if I would be here today. I cannot express enough my gratitude to them. If any of them remembers me, please contact me though the Gauchers Association. My wholehearted thanks must also go to my dad, Graham, who lived through all this with me and had his own fears to deal with. I also thank my sister, Emma, without whom I could not have undergone the transplant. n
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Source: Gauchers News October 2004.
© Copyright Gauchers Association 2004.