THE VON MEMERTY KIDS HELP FUND
Valeska von Memerty was born in October 1992, a healthy, strong baby who hit all her mile stones. At 18 months we took her for a hearing test for an erratic hearing problem, and after extensive traumatic testing period, she was diagnosed with MPS 6 (muco polysacharodisis 6) a.k.a. Maroteaux Lamy Syndrome. A genetic metabolic illness, which causes all the soft tissues to swell causing massive deformity, dwarfism, increasing paralysis, loss of motor functions, gradual blindness and deafness and proves fatal by the late teens. Viv was 4,5 months pregnant at this time, and we then went through the next round of tests to find out that our in-uteri boy was also likely to be affected, but it was too late in the pregnancy to consider termination and in August the fattest happiest bundle was born – Oscar.
As parents we had the terror and dilemma of having two children who faced a future of profound distress and severe trauma, and the only treatment that seemed viable were hugely expensive. June 1996 – both children are confirmed as having the serious form of the disease. Although enzyme replacement therapy is in development, it was recommended that we do Bone Marrow Transplants. because of the time frame. The sums needed for BMT are frightening , but after a massive fund-raising effort in 1997 we raised 1,25 million rand, and both children underwent unrelated bone marrow transplants in Manchester (UK) in 1997. We also had a rather public fight with the British Genetic Authority over break through developments which could ensure that child transplant patients might preserve their fertility after the severe chemo therapy. Oscar rejected his transplant after 6 months, and Valeska accepted her transplant but died in August 1998, from transplant complications. An agonising loss for us, and a horrible end to years of desperate planning, work, and hope.
Because of the high risks involved with a 2nd unrelated bone marrow transplant we had decided to just let the disease take its course, and simply love and support Oscar. We were also waiting to see if ERT (Enzyme Replacement Therapy) became available. We had since found out the ERT wasn’t likely to be available for another 3 to 5 years, and then would cost $ US 100 K to 150 K per annum FOR LIFE. But we then learnt of two new developments. The first, developed in Israel, is what is called a ‘lyte’ BMT. It involves no radiation, and hardly any chemo therapy – and from what we understood this new system called ‘non-ablative’ transplants worked very well on metabolic patients. The second development was that donor matching had progressed hugely since 1997. The HLA typing which is used to find a donor (to give you some idea in 1997 Oscar had one possible match on an international registry of over 3 million people) now underwent DNA testing on the most important part of the marrow. This meant that the rejection process and side effects are a lot less violent and difficult.
After two fund raising seasons with A Handful of Keys in South Africa, and a tour to Sydney and London we had almost scraped together the 120 000 pounds (2,3 million rands at the 2002 exchange rate) needed by Great Ormond Street Hospital in London. We had been advised that when it came to lyte transplants they were the best European hospital and as well as being one the finest paediatric hospitals in the world. Oscar underwent his transplant at the beginning of May 2002 and after a very unpleasant but fairly routine time went into a major crisis six weeks after transplant. He developed a massive lung infection culminating in a 10-minute heart stoppage. After resuscitating him, he was kept ventilated and paralysed in ICU for 9 days. We were told repeatedly that they had never been able to save a child in Oscar’s particular situation, and that he would almost definitely die within 72 hours, and that if he survived there would certainly be brain damage after such an extended cardiac arrest.
Miraculously and thanks to the magnificent work of the team in the hospital he survived, with ABSOLUTELY no brain damage at all. He is now 24 years old, though only 1, 17 cm high. He left home at 17 and lives in Joburg working in brand activation and as motivational speaker. Although he is unbelievably fit and healthy (a six pack nog-al and a very cool moustache)) he has had his leg broken and reset with US titanium steel ; two cornea transplants (Italian) and two cataract ops. He is cool, calm, funny, focused, smart and a veryyyyy trendy dresser.
The even better news is that in July 2000, we had a healthy little girl called Kasvia. She is not affected by the disease and it has been a real privilege and pleasure for us to have an unaffected child and to have to replace clothes because she has outgrown them! Now 19 years old she is a gorgeous, kind, smart, independent young woman who brings us great pride and joy.
We have toured all over the country with our two family shows “Celebration” and “Circle Of Life”, as well as 8 (!!!!!) New Years cruises to Mauritius as guest performers.