Medications

Medicines that treat sickle cell disease include hydroxyurea and various pain medicines. Some of these medicines require a prescription. Others are available over-the-counter. Pain medicine may work best when combined with pain management skills, such as distraction, guided imagery, deep breathing, relaxation, and positive, encouraging self-talk.

Hydroxyurea is approved by the U.S. Food and Drug Administration (FDA) for treating sickle cell disease in adults. Hydroxyurea increases fetal hemoglobin production, which can decrease the severity of sickle cell disease and can prolong life for severely affected adults. Research suggests that hydroxyurea also works well for children. But long-term studies are needed before hydroxyurea is routinely suggested for children.

Medication Choices

Hydroxyurea

Pain medicines

What To Think About

Hydroxyurea

• Hydroxyurea doesn't cure sickle cell disease, but it can reduce the number of serious sickling complications.
• Doctors recommend hydroxyurea treatment only for adults who have three or more painful events each year. Using it requires frequent blood tests and doctor visits. Most people who take this medicine have fewer pain events and acute chest events, and may also live longer.

Pain medicines

• Treatment for sickle cell disease pain varies depending on how bad the pain is and how long the pain lasts. Medicines that treat sickle cell disease pain include over-the-counter pain relievers such as ibuprofen and prescription opioids such as codeine.
• An increasing number of doctors and researchers recommend aggressive and continuous opiate pain medicine for severe painful events until the pain goes away.⁸

Sickle cell pain is often undertreated because:

• Some doctors underestimate the intensity of painful events.
• Some parents, doctors, and adults with sickle cell disease fear that use of opiate medicines (such as codeine and morphine) will lead to drug addiction. Research suggests that drug addiction is no greater among people with sickle cell disease than it is in the general population.⁸ It's important to use these medicines under careful medical supervision.