June 6, 2012

The tale of the 'boy in the bubble' & the 'Bubble Boy' disease.!

The boy in the 'bubble'
                   Have you ever thought of spending your life inside a plastic bubble?Well,David Vetter(1971-'84) a young boy from Texas did lead such a life in the real world.Born with a potentially fatal inherited disorder named 'Severe Combined Immuno Deficiency disease',Vetter had zero immunity to infections which made the doctors to put him in a germ- free isolator('the bubble').The isolator was supposed to be a temporary one as it was widely expected that the boy would develop a functioning immune system after a successful bone marrow (the only treatment for the disease at that point of time)transplant.However as the wait for the elusive matching bone marrow continued,the family and his medical team decided to go for an unmatched bone marrow transplant through I/V lines running into the bubble.The transplant was a failure as the boy succumbed to Burkitt's Lymphoma a couple of months post surgery.
                   In the year 2002,a panel of scientists identified the gene responsible for this condition and postulated that the replacement of the defective gene with a healthy/normally functioning gene could possibly cure the disease.This replacement surgery was successfully performed first on an 18 months old SCID patient Rhys,Evans (and was subsequently performed on 16 others) and turned out to be a landmark event in the history of modern medicine as more potentially fatal genetic defects could be cured using the technique of gene therapy.Though the success of the therapy still remains controversial,the possibilities that gene therapy put forwards are unarguably endless and it is something which we can look forward to in the near future.
For more on this dreaded disease,visit here and here.To read David Vetter's life story,click here



daniel said...

informative post