Steven attained the age of 17 last April, which was a real milestone in his life, as his doctors at Great Ormond Street Hospital, Moorfields Hospital etc. had not expected him to live beyond his 16th birthday. But he has always been, and always will be, a fighter. He amazes everyone around him with his determination to survive, regardless of how many operations ( 78 to date ) and whatever pain he has to endure. Somehow he manages to keep going. He is a truly amazing human being.
Until I met Steven 5 years ago, I hadn't heard of XP, and was taken aback when I learned, and saw for myself what the illness can do to a person. We have been best friends since our first meeting.

He has recently had what was left of both his ears amputated. He has cancer in both his eyes. He's had some extremely big tumours removed. Never before have I personally seen an illness literally eating someone. We were on a train one day and a passenger asked me when the fire was that Steven had been in. That may give you some slight indication of how he looks. But Steven turned to the passenger as calm as you like and said "It wasn't a fire, It's Cancer"

Steven was recently more poorly than he has ever been. He was in hospital for three months, and we all thought, including him, that he was going to die. Between 5 of us we stayed with him all the time he was there. As a result we all contracted Scabies. It was bad enough for those around him, but for Steven it was hell on earth. It was so bad the hospital had the Crash Team on stand-by to try to revive him if necessary.
But he has come through it all. How on earth he finds the strength to continue his fight is really quite incredible, but he does, and he always comes through things having retained a really wicked sense of humour.

No one knows now just how long Steven will be with us for. Everyday that passes is a wonderful bonus for both him and those who are fortunate to be in his company.
The way he looks makes some people feel physically sick, in school he has been described by some children as looking like a monster. But those blinkered people haven't taken the time to look beyond his physical appearance to see Steven as he really is. A kind, considerate, loving human being, with more heart and thought for others than any other 17 year old I've met (he was the same when I first met him).
To think that there are potentially many other people just like Steven, is both sad and wonderful. Sad, because they have this terrible illness, but wonderful, because if the world was full of people who thought and felt and gave so much just like Steven does, then the world would be amuch better place.

I only wish that Steven's life could be fuller and more enjoyable, but the XP is not that kind to him. We are constantly trying to find things for him to do in order to give him something to get up for each day. He has to have something ahead of him to look forward to. It is a constant battle to stop him turning his head towards the wall and wishing the world would stop so that he could get off.

For all that life has taken away from Steven, it has also given him so much in other ways.

When my son was first diagnosed with XP we were not told, all in one go, a great deal about the disorder. Over the next few weeks we asked our Doctor many questions (as the mothers amongst you will understand ). During genetic counselling we learned that we could pass it on to other unborn children. I had planned a larger family. It was also in that meeting that I learned that this condition was terminal. WOW.

I can't remember anything else that was said during the rest of that meeting. You might say I was in shock. Instead of catching the bus home, I caught a taxi cab. Stunned but composed, during the journey home, the taxi driver tried to get a chat going. I only yes'ed and no'ed in the right places (I think?). Getting out of the cab the taxi driver said "Cheer up, nothing can be that bad." I paid and said "I've just been told that my 15 month only baby was going to die." You could see he wanted to crawl in a hole somewhere. I felt bad about that comment later. He was only being nice. He didn't need me dumping on him.

It was a good six months until I came out of shock. Each birthday we treasure, each Easter, each Christmas. We never know if it will be his last. It made long term plans a problem. What if we decided to do something in 3 months time (a holiday, a family wedding etc). We always said yes but .... our family knew we were worried that by that time, we may be having problem and may have to pull out at the last minute.

My son has been in and out of hospital many times since. Making decisions about his care was a crash course in trusting your instincts. We had only our Doctors who could advise us. We had no group to turn to. We couldn't speak to an experienced family about it. My son was too young, to ask his opinion about his care. We treated our Doctors as team advisers but we, as parents, we made the final decisions. We decided that we would 'sleep' on most decisions, then whatever we woke up with was the way we went. Even if we didn't understand the wisdom in that decision, at the time. Sometimes we went against what would seem more logical. Our Doctor commented on our method of decision making because in hindsight we often made the wisest decision without enough information that was needed at the time.

My son is now 24 years old. One Doctor said that he was surprised that my son had survived all he has been through. Our 'sleeping-on-it' method had indeed given him many more quality years than he (the Doctor) could have ever expected.

He first had a Squamous Cell Carcinoma (SCC) when he was 11 months old. They didn't diagnose XP then. He has had many KA's, loads of Basal Cell Carcinomas (BCC's) and I've lost count of the SCC’s since that first one. The main kind of treatment he has had is surveillance and excision of lesions. Often needing a skin graft or two. His eyes have suffered, too. His vision is particularly restricted with sun damage to the corneas.

Living Down Under, here in Australia, where it is a sunny and hot climate seemed to us to be the wrong place to stay and raise our son. We considered moving to another climate but the more we found out about XP the more we realised that wouldn't fix it. My son was on the WRONG planet. UV affects the entire planet. One of my friends wrote a short story based on my son. The story was about how God had 'messed up' and put this child on the wrong planet. We found the story very amusing. I've often thought about that story since.

Television doesn't seem to cause him any problems but fluorescent light strips give off UV light (as we found out by him being burned) so we do not have these in the home. One night we had a lightning storm. WOW. 'Natures fireworks', it was a brilliant exhibition. We all went out and sat outside and watched the night sky. Being night time Gaelon could go outside. The next day Gaelon was intensely burned which showed us that lightening was intense UV. Who would have thought it. We found out the hard way, as usual.

Gaelon is quite contented in nature and nothing much phases him. He has been on TV once or twice and Women's magazines have run a story or two about him. We found that locally beneficial because then the neighbourhood who were in contact with him knew what he was living with. The man in the local deli gave him a free ice-cream, one day after he had been on TV and another man in the Video Shop let him hire a game for free. The story touched their hearts.
Because Gaelon is obviously physically affected people are usually curious as to what the matter is. Gaelon usually just tell them that he gets skin cancers and the Doctors cut them out. That's usually all they need to know. Gaelon is very quiet and very shy. But when people get to know him, his humorous side often has them laughing. Being in the church we often have members join us in the home. He isn't shy with them and will race them around the block smashing everything in sight (on the Sony Playstation ). Gaelon also likes the games where there are puzzles, like in the game 'Silent Hill'. Because his eyesight is reduced, sometimes he needs me to read the frames in the games to him. The TV magazine writing is too small for him to see, too. So I photocopy that for him and make the print larger by sizing-up on the photocopier. He subscribes to the game magazine and the print in that is too small, too. Sometime his sister comes around and visits him. She likes reading the magazine too, and helps him with the games. She likes to play the games where I'll help, where he needs it, but I don't like to play the games. I leave that to them.

It struck me a few years ago that while Gaelon is living with XP, his XP affects so many people around him. Not only the immediate family (obviously) but how he opens the hearts of those who come in contact with him. One of our rellies came over for a visit from England. Before she came she said that she wasn't sure she wanted to actually meet Gaelon because she was afraid she would become attached to him, and find it very hard emotionally when he died. Of course she will, we understand that, so I said to her that she may never have a chance again and that her fear should not make her choices for her. I suggested that she let her heart open and learn to love unconditionally and when the time comes she could let herself cry, 'with us' not 'for us'. She came. She fell in love. She is glad she did. And up to now she hasn't had to cry 'with us'.

I've grown so much since he was diagnosed (when I was 22 years old). I must say that taking care of him has made me a much better person. Meeting mothers in the hospital with all sorts of problems has made for a very interesting a varied life. I may not have planned it that way, but when life hands you a lemon you may as well make lemonade. Gaelon Joined CanTeen. That is Teenagers with Cancer. There motto is "Cancer is a work not a sentence". I just loved that. Gaelon is LIVING with XP, to the max ( ... well ... as much as he can).

Our profile this edition, is slightly different in that it comes from Miriam, who was a counselor at Camp Sundown this year.

Camp Sundown 2000: a shutting of curtains but opening of eyes

I hadn't heard much about Xeroderma Pigmentosum before reading last summer's Sunday Times article about Camp Sundown. Only a brief mention in a lecture on Medical Genetics where we were more interested in learning the genetic principles exhibited rather than studying its effects on actual people. This is often a problem at medical school; one is so busy cramming scientific facts to pass exams. that it's easy to forget that patients are people with lives of their own, not provided solely for the furthering of science. One of the things about the XP Society that has so impressed me is the way in which neither medical research nor patients are neglected. While money is raised to promote scientific research into the condition, equal funds are used to improve the every day living of those affected.

I volunteered at the camp for two weeks. The first of these was for older patients, usually in their twenties or thirties. I wasn't prepared for the disfigurement that some of them displayed, I had forgotten the harsh realities of XP in my excitement about volunteering. Several of them had surgery scheduled for the week after they left camp which added considerably to their reluctance to leave. It also came as a surprise to me to learn that nearly all the patients had lost siblings to the disease. This somehow added to the somber air of the whole week. Sometimes there was a feeling that the happiness was slightly forced, covering a deep depression that was felt by many.

The second week was entirely different. The guests were children from all over the world, usually accompanied by their parents and siblings. The school bus we used had been fairly empty in the previous week but suddenly we were finding ourselves squashed three to a seat in an attempt to accommodate everyone. I had been away for the weekend between the two weeks of camp and my train returning was delayed. As I opened the door to the conference room in which the first night festivities were taking place, I was met by the sight of dozens of youngsters running around with their faces painted, cowboy hats on and fake tattoos liberally applied. It could not have been more different from the previous week. There were children however, used to scorn and ridicule from classmates, who were unable to join in easily, fearing similar treatment. Some of the stories I heard from campers were horrifying; an Israeli family who ran a very successful restaurant that went bankrupt after rumours that a family member had a contagious skin disease; a native American lady who had been ostracised by her own people after her daughter was diagnosed with XP (in native American folklore such conditions are believed to result from wrong doing by the mother); a family whose affected son is being deprived of a proper education due to the local authorities failing to make adequate arrangements for his protection from UV light. While it was awful to hear these things, it must have been a huge relief for the parents to find themselves in the company of people who understood entirely what they went through on a day to day basis. The camp didn't just provide a holiday for the children.

The last night of the camp was jam packed with activities. Firstly dinner at a local pizza restaurant with an awards ceremony in honour of the children's achievements. Then a visit to the local baseball team, the Hudson Valley Renegades, where the campers were treated to expert training (and the young female volunteers to the sight of the players' tight trousers). Finally we went to Freedom Park where pony rides, fencing and much more were on offer. Everyone wore glow in the dark necklaces, and the memory of seeing scores of strips of light running around in the dark is one that will remain with me for a long time. Saying goodbye was hard, a lot can happen in a year, especially when exposed to the constant threat of UV radiation.

It was a truly great experience volunteering at the camp, humbling yet somehow empowering as I realised that I could make a difference, albeit a small one. The prospect of a Camp Sundown UK is wonderful, and exciting. There were four UK families in New York State, and I am sure that all will recommend the camp whole heartedly to other families. Media coverage of the condition is gaining momentum, and this can only help to raise funds for and public awareness of the XP Society. It is something in which I feel really privileged to be involved.

Health warning to potential volunteers: ensure that Holly and Alex W and Alex W are handcuffed and gagged when sleep is required. It is otherwise impossible.

I hadn't heard much about Xeroderma Pigmentosum before reading last summer's Sunday Times article about Camp Sundown. Only a brief mention in a lecture on Medical Genetics where we were more interested in learning the genetic principles exhibited rather than studying its effects on actual people. This is often a problem at medical school; one is so busy cramming scientific facts to pass exams. that it's easy to forget that patients are people with lives of their own, not provided solely for the furthering of science. One of the things about the XP Society that has so impressed me is the way in which neither medical research nor patients are neglected. While money is raised to promote scientific research into the condition, equal funds are used to improve the every day living of those affected.

I volunteered at the camp for two weeks. The first of these was for older patients, usually in their twenties or thirties. I wasn't prepared for the disfigurement that some of them displayed, I had forgotten the harsh realities of XP in my excitement about volunteering. Several of them had surgery scheduled for the week after they left camp which added considerably to their reluctance to leave. It also came as a surprise to me to learn that nearly all the patients had lost siblings to the disease. This somehow added to the somber air of the whole week. Sometimes there was a feeling that the happiness was slightly forced, covering a deep depression that was felt by many.

The second week was entirely different. The guests were children from all over the world, usually accompanied by their parents and siblings. The school bus we used had been fairly empty in the previous week but suddenly we were finding ourselves squashed three to a seat in an attempt to accommodate everyone. I had been away for the weekend between the two weeks of camp and my train returning was delayed. As I opened the door to the conference room in which the first night festivities were taking place, I was met by the sight of dozens of youngsters running around with their faces painted, cowboy hats on and fake tattoos liberally applied. It could not have been more different from the previous week. There were children however, used to scorn and ridicule from classmates, who were unable to join in easily, fearing similar treatment. Some of the stories I heard from campers were horrifying; an Israeli family who ran a very successful restaurant that went bankrupt after rumours that a family member had a contagious skin disease; a native American lady who had been ostracised by her own people after her daughter was diagnosed with XP (in native American folklore such conditions are believed to result from wrong doing by the mother); a family whose affected son is being deprived of a proper education due to the local authorities failing to make adequate arrangements for his protection from UV light. While it was awful to hear these things, it must have been a huge relief for the parents to find themselves in the company of people who understood entirely what they went through on a day to day basis. The camp didn't just provide a holiday for the children.

The last night of the camp was jam packed with activities. Firstly dinner at a local pizza restaurant with an awards ceremony in honour of the children's achievements. Then a visit to the local baseball team, the Hudson Valley Renegades, where the campers were treated to expert training (and the young female volunteers to the sight of the players' tight trousers). Finally we went to Freedom Park where pony rides, fencing and much more were on offer. Everyone wore glow in the dark necklaces, and the memory of seeing scores of strips of light running around in the dark is one that will remain with me for a long time. Saying goodbye was hard, a lot can happen in a year, especially when exposed to the constant threat of UV radiation.

It was a truly great experience volunteering at the camp, humbling yet somehow empowering as I realised that I could make a difference, albeit a small one. The prospect of a Camp Sundown UK is wonderful, and exciting. There were four UK families in New York State, and I am sure that all will recommend the camp whole heartedly to other families. Media coverage of the condition is gaining momentum, and this can only help to raise funds for and public awareness of the XP Society. It is something in which I feel really privileged to be involved.

Health warning to potential volunteers: ensure that Holly and Alex W and Alex W are handcuffed and gagged when sleep is required. It is otherwise impossible.