Jonathan Pitre, 16, has epidermolysis bullosa, a rare genetic disorder of connective tissue that causes your skin to be extremely fragile and prone to tears and blisters from minor trauma and friction. About 200 youngsters are born with this condition in the United States every year. They are sometimes known as butterfly children on account of their fragile skin. There is no therapy or cure for this painful and debilitating disease. Individuals must learn to manage the pain and use protective bandaging for wound care.
Stem Cell Transplant for Epidermolysis Bullosa
Stem cell transplant for epidermolysis bullosa involves replacing unhealthy cells in the bone marrow of a patient with healthy cells from a donor. Within Pitre’ s case, blood and marrow were attracted from his mother’ s hip with the hope that the come cells in her blood would colonize his marrow and alter the course of his disease.
The bone marrow is a soft, spongy tissue in the interior of bones that contains hematopoietic come cells, i. e., stem cells that can differentiate straight into blood cells. Intense chemoradiotherapy is used to kill the particular patient’ s own stem cells, followed by transplantation associated with donor stem cells, which hopefully will grow and offer a supply of healthy blood cells.
Complications of Stem Cell Transplantation pertaining to EB
Pitre’ t immune system has been wiped out by the effects of high-dose chemoradiotherapy. Physicians will know after several weeks if the procedure was a success. At the same time, the teenager is at high risk of infections and GVHD (graft-versus-host disease) if the donor stem cells develop into T-cells and attack normal cells.
It takes about two weeks for the donor cells in order to firmly establish themselves in the patient’ s marrow. The existence of immunity-providing white cells in Pitre’ s circulation may indicate that the transplanted stem cells are working. Any enhancement in his skin condition will also be a signal that the procedure is an achievement.
Pitre had formerly undergone a stem cell transplant which failed whenever his own stem cells re-colonized his marrow. This time, it really is Pitre and his doctors’ hope that his mother’ t stem cells will grow in his body, divide, plus make healthy blood cells that will contain the protein he or she needs to rebuild damaged skin.
1 . http://ottawacitizen.com/news/local-news/his-stem-cell-transplant-complete-the-wait-begins-for-jonathan-pitre
2 . http://www.debra.org/whatiseb