Sickle Cell Medication Helps Extend

A medication that reduces severe attacks in patients with sickle cell anemia has been found to extend patients’ lives, according to a study published in the Journal of the American Medical Association co-authored by Rita Bellevue, M.D., director of the Sickle Cell/Thalassemia Program at New York Me-thodist Hospital.

Benefits of the medication-hydroxyurea-were first reported eight years ago when researchers discovered that it reduces the number of painful crises caused by sickle cell disease.

In the latest study, researchers at 20 sickle cell disease centers, including New York Methodist, found that patients who had the fewest number of attacks as a result of taking hydroxyruea also had the lowest death rate. Patients on the medication for at least nine years experienced 40 percent fewer deaths than among patients who had not taken the medication.

"These finding are very exciting," said Dr. Bellevue. "In my clinical practice, the patients taking hydroxyurea experience few attacks, spend less time in hospitals, do not need blood transfusions as frequently, if at all, and are doing much better. Their average life expectancy has also increased significantly," she said.

Approximately 80,000 people in the United States are affected by sickle cell disease. A genetically transmitted blood disorder, sickle cell disease is cha-racterized by the presence of abnormal hemoglobin in the red blood cells, designated "Hemoglobin S."

Red blood cells carry oxygen to tissues throughout the body. The red blood cells in a patient with sickle cell disease take on a crescent (sickled) shape and become rigid once the cells have delivered oxygen. These sickled cells are unable to pass through tiny blood vessels and this clogging often causes pain and/or potential permanent organ dysfunction, such as brain or kidney or lung damage. Damage can be permanent.

"Hydroxyurea works by increasing the production of fetal hemoglobin. Whereas fetal hemoglobin is predominant at birth, this type of hemoglobin decreases significantly as we get older. The production of fetal hemoglobin makes it possible for red blood cells to flow normally and prevent many of the complications of sickle cell disease," said Dr. Bel-levue.

In addition to providing comprehensive care to pediatric and adult patients with sickle cell disease and thalassemia, The Hospital’s Sickle Cell/Thalas-semia Program offers a hydroxyurea clinic day each week. "We are following patients very carefully, so that we completely understand the long-term effects of hydroxyurea," said Dr. Bellevue.