Cell Disease: Treatment and Cures
Guest: Dr. Sherry Jackson – Pediatrics - Hematology/Oncology
Host: Dr. Linda Austin – Psychiatry
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?
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.
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.
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
Linda Austin: So, that sounds like it
really is an important piece of the treatment.
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.
Linda Austin: If somebody was interested
in participating in one of those studies, what number would they call?
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.
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
Linda Austin: Dr. Jackson, thank you so
much for talking with us about this.
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.