Sickle Cell Disease: Treatment and Cures

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Sickle Cell Disease: Treatment and Cures




Guest:  Dr. Sherry Jackson – Pediatrics - Hematology/Oncology

Host:  Dr. Linda Austin – Psychiatry


Dr. Linda Austin:  I’m Dr. Linda Austin.  I’m talking, today, with Dr. Sherry Jackson who is Associate Professor of Hematology/Oncology at the Children’s Hospital, Medical University of South Carolina.  Dr. Jackson, in an earlier podcast, we talked about the genetics of sickle cell disease and the course of the disease.  I’m sure a question you get asked a lot is about cure.  Is there a cure for sickle cell and, if not, how do you go about treating it at least?


Dr. Sherry Jackson:  Linda, there is actually a cure, which is the use of bone marrow from a matched sibling donor.  And we would recommend, if there is an available donor, that a child, usually around the age of six would be an ideal age, undergo a bone marrow transplant.  The problem with that is, most of the children that are symptomatic from their sickle disease don’t have a matched donor, a matched sibling to serve as a donor, and the possibility of the bone marrow being rejected, if there’s not a perfect match, is a very high possibility.


So, right now, we don’t have the opportunity to transplant very many of our sickle cell patients.  What we have had some success with, and especially in the adult population, for the past 25 years, is a medication called hydroxyurea.  I’m not sure if many in the community have heard of it, but this is a capsule that can be taken everyday.  It causes the bone marrow to produce fetal hemoglobin.  The fetal hemoglobin is protected in babies.  And what we have found is that, taking hydroxyurea, in an appropriate dose, can cause production of fetal hemoglobin, even in adults. 


We’ve used this medication for nearly 25 years in adults.  We’ve found a significant response in terms of decreasing the number of painful episodes that a patient has, decreasing the episodes involving the chest, and increasing the hemoglobin, the blood count itself, so the anemia is not as severe as it is in patients who are not taking hydroxyurea.


Dr. Linda Austin:  So, that sounds like it really is an important piece of the treatment.


Dr. Sherry Jackson:  The hydroxyurea is.  Now, as it is with many medications, we have to use the medication in adults and gather experience and evaluate for side effects and complications from the medication before we try them in pediatric patients.  So, that’s why we’ve had a much longer experience with using hydroxyurea in adults than we have in children.  But, I’m happy to say that our sickle cell program, at MUSC, is part of two national studies that are ongoing, evaluating the use of hyroxyurea in children.


Dr. Linda Austin:  If somebody was interested in participating in one of those studies, what number would they call?


Dr. Sherry Jackson:  Well, these studies that we’re involved in would mainly include children who are in our practice, our sickle cell practice.  Our clinic number is:  876-0444.  But, at the visits, when patients come into to see me, the issues that we talk about include the availability of hydroxyurea and which of the patients are actual candidates to be on the medication.  It’s mainly depending on the frequency and severity of your symptoms.  I should mention, Linda, that we have, out of about 400 patients, I would say, maybe, 50, 60 percent are children who have been very healthy and have had very minimal complications of sickling, bone pain and hospitalizations, and so forth. 


But, remember, as we said, as the children get older, the likelihood is that they’re going to experience more in the way of sickling symptoms.  So, even if they’ve not been a candidate for hydroxyurea in the past, as those children approach their teenage years, many of them will begin to experience more symptoms.  And that’s when they would be eligible to be a patient taking hydroxyurea.


Dr. Linda Austin:  Dr. Jackson, thank you so much for talking with us about this.


Dr. Sherry Jackson:  Thank you so much.


If you have any questions about the services or programs offered at the Medical University of South Carolina or if you would like to schedule an appointment with one of our physicians, please call MUSC Health Connection:  (843) 792-1414.

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