Prather Pediatric and Allergy Center - Ask Doctor Brent

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Title: Sick Cell Anemia - New Hope and Therapy

Category: Child Care


In the past, sickle cell disease was a devastating disease which shortened life and caused a tremendous amount of suffering for all people who had it. It is extrememly common throughout Louisiana, primarily in the black population. It usually starts in childhood with sickle cell crises. This is a jamming up of the sickled red blood cells in the blood vessels in the extremities and typically causes pain in the arms and legs, hands and feet. It occasionally causes severe pain in the abdomen. It rarely causes life threatening drops in the total red blood count because of blood pooling in the spleen or liver. There are several other complications which occur with sickle cell and, all in all, it is no fun for the patients who must struggle to live as normal a life as possible while carrying this dread disease. Fortunately, we have made great strides in the therapy of the disease. We now have early diagnosis, because of blood screening tests done right after birth. Also, we use close monitoring and therapy with multivitamin Folic acid, avoidance of high risk conditions such as certain viral infection exposures, especially Fifth's disease. For all these reasons patients have done much better over the past generation.

Now we have an exciting, potentially curative, therapy for this disease. The curative therapy involves an HLA autologous matched bone marrow transplant done at a major medical center such as St. Jude's in Memphis, Tennessee. There have been several cases of successful bone marrow transplants which have totally cured this disease. For any patients who have sickle cell disease, or a variant of sickle cell disease, and would like to be involved in potential bone marrow treatment or other study of their disease, they can contact St. Jude's Hospital by calling (901) 522-0431 and ask for Dr. Judy Wilimas.

In 1981, when I finished my training in allergy and immunology at LSU medical center in New Orleans, I had the privilege of serving with a team that did the first pediatric bone marrow transplant in Louisiana. It was done on a child with severe combined immune deficiency and it was successful. It is so gratifying to see the hundreds of cases of deadly diseases since that time cured with bone marrow transplant, including things like leukemia, sickle cell disease and numerous other diseases which were without hope of cure in the past. Although St. Jude's Hospital gets a lot of the credit and attention in bone marrow transplants and treatment of cancer and related diseases, there are many other wonderful centers which have been successful with bone marrow transplants including our own Children's Hospital of Louisiana in New Orleans.