Treatment of Hemoglobin Sickle Cell Disease

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Sickle cell disease (SCD), specifically hemoglobin SC disease (HbSC), is a subtype of sickle cell disease with typically higher hemoglobin and milder or later disease complications. Sickle cell disease is a disorder in which red blood cells (RBCs) are abnormally shaped. This can result in painful episodes, serious infections, and damage to body organs. One medication used to treat sickle cell disease is hydroxyurea.

Hydroxyurea therapy offers significant benefits for infants, children, and adolescents with sickle cell anemia. These include a reduction in the frequency of pain crises and acute chest syndrome (inflammation of the lungs). Hydroxyurea has been given to many HbSC patients but HbSC patients were not included in the large clinical trials used to test hydroxyurea in SCD, so we don't know as much about how HbSC patients respond to hydroxyurea.

The purpose of this research study is to see if hydroxyurea, a medication given to many children with the most common type of sickle cell, HbSS, helps children who have HbSC, by giving a questionaire when the medication is started, and then each month at a clinic visit. The questionaire, called the PedsQLTM 3.0 Sickle Cell Disease Module, measures quality of life. Thia study will also evaluate how hydroxyurea changes laboratory test numbers, and blood thickness.

More information: clinicaltrials.gov, ITHANET Clinical Trials