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Experts Recommend Best Treatments for Sickle Cell Disease

FILE - A sickle cell patient given an injection for pain relief.

A panel of experts is out with new guidelines for treating sickle cell disease. But not all the recommendations will be affordable for everyone.

Sickle cell disease - or sickle-cell anemia, as it’s sometimes called - is a genetic condition affecting millions around the world. It’s most common in tropical areas including sub-Saharan Africa, Saudi Arabia, and Central and South America. It’s also found in countries like the United States among migrants from those areas or their descendants.

The name comes from misshapen and sticky red blood cells which can cause severe pain when they block small blood vessels instead of flowing smoothly.

The new guidelines strongly recommend regular transfusions and a medicine called hydroxyurea. Barbara P. Yawn, MD, co-chair of the expert panel that developed the guidelines, says these treatments can really improve patients’ lives, “and they are just grossly underutilized. There are not nearly as many people using these therapies as there are people who are candidates for these therapies.”

Routine transfusions can be out of reach, especially in poor countries. Hydroxyurea, on the other hand, is a relatively inexpensive, generic pill.

Even more appropriate may be the recommendations to prevent pneumococcal infections, which can be fatal in young children with sickle cell disease. The guidelines recommend vaccination and a daily dose of penicillin for youngsters under age five.

“So that’s something that we would like to do. Is that feasible in a developing country? It should be, because penicillin is a generic drug. It is very inexpensive,” said Dr. Yawn in a telephone interview.

The guidelines also recommend using strong medicines like morphine to treat the acute pain of a sickle cell crisis.

The sickle cell treatment guidelines are meant for health care professionals, but Barbara Yawn says they’re also valuable for patients and their families to be aware of, to point their doctors to the best practices in treating the disease.

The expert panel that issued the sickle cell disease guidelines was organized by the U.S. National Heart, Lung, and Blood Institute, and their report was published in the journal of the American Medical Association, JAMA.