Sickle Cell Disease
Treatments
Please talk to your health care provider about treatment options that are best for you or the person you are caring for.
Penicillin
Penicillin has been shown to prevent life threatening infections in children with SCD and save babies lives. When penicillin was first given to babies in the 1980s, it drastically reduced the rate of infant mortality (the number of babies who die) due to SCD. Newborns with SCD will be given liquid penicillin and older children can take tablets.
Hydroxyurea (Hi-Drox-Eee-u-Ree-uh)
Hydroxyurea is a medication that can reduce many of the complications of SCD. When we are born, we have a type of hemoglobin known as “Fetal Hemoglobin” that we get from our mothers. Research has shown that this Fetal Hemoglobin helps protect persons with SCD from some of the complications of the disease. As we get older, the amount of Fetal Hemoglobin naturally decreases and this protection is lost. Hydroxyurea acts to increase the level of Fetal Hemoglobin in patients who take it and thereby increases the protection against many of SCD’s complications.
Endari (L-glutamine powder)
Endari (En-Daa-Ree) was approved for the treatment of SCD in 2017. It has been shown to reduce the frequency of pain crises and reduce the number of hospitalizations due to pain crises.
Adakveo (crizanlizumab-tmca)
Adakveo (uh-dak-vee-oh) was approved for the treatment of SCD in November 2019. It is a once monthly infusion that has been shown to reduce the number of pain crises.
Oxbryta (voxelotor)
Oxbryta (Ox-Brye-Ta) was approved for the treatment of SCD in November 2019. It was shown to increase hemoglobin levels and decreased the breakdown of red blood cells (hemolysis).
Transfusion Therapy
There are different types of transfusion therapy used to manage SCD. One of the ways SCD is treated is with Therapeutic Apheresis (“Apheresis”). Apheresis replaces a SCD patient’s sickled red blood cells with healthy red blood cells. This is standard of care for people with SCD who have a stroke or other acute, life threatening complication of their disease. Apheresis is also used standardly in the chronic setting for people with SCD and a history of stroke, recurrent, life threatening complications of SCD or recurrent episodes of pain crises that are unresponsive to other therapies.
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