The Bubble Dog has the Cure

In June 2006 a new treatments was found to cure the life-threatening genetic disorder that disables the immune system, bubble boy disease, in basset hounds. The disease is an X-linked severe combined immunodeficiency (XSCID) that affects male dogs and 1 in 100,000 boys, all who share a faulty gene from one of their mother's x-chromosomes. New treatment will be available in the future. There is only one treatment for now and the boys are given bone-marrow transplants which replenish stem cells responsible for the renewing of new functional immune cells.

Gene therapy, done in the lab with the individual's own bone marrow cells and the normal gene, has shown some success in treating the disease. 10 out of the 11 boys were used in this 1999 French study but three of them developed leukemia because of the process. Peter J. Felsburg, professor of immunology at the University of Pennsylvania's School of Veterinary Medicine and leader of the recent work, stated that the new ex-vivo (outside the body) therapy has been proven successful in restoring normal immune function in XSCID boys but that it has several potential problems to it. His experiment consisted of injecting the corrected gene via an engineered virus into the bloodstream. Three of the four basset hounds used in the experiment have maintained a healthy immune system over a year after receiving the good genes and the fourth dog received a lower dosage proving that there is a lower limit of the dose before the gene can improve the immunity. Jennifer Puck, researcher in the Division of Immunology & Rheumatology and the University of California, San Francisco, is a leading expert in XSCID and she and her colleagues developed a way to diagnose the disease in young infants which will be presented in the conference Primary Immunodeficiencies: Past, Present, Future. She still believes it is too early to start the application in humans but seems very pleased with the results and she also stated that the French children who developed the leukemia got ill 30-34 months after the treatment. Puck wishes that research continues on this and other immunodeficiency disease that affect both girls and boys and that in the future we might be able to screen for diseases like these in infant children so that we reduce problems.

If this experiment proves to be effective it will eventually be used in humans and it is interesting how male dogs also show this disease. Even man's best friend gets the same illnesses.

Taken from Animal Planet: http://animal.discovery.com/news/briefs/20060612/bubbleboy.html

Posted by Carmen B. Arsuaga (1)