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Sickle cell disease

Description

An in-depth report on the causes, diagnosis, and treatment of sickle cell disease.

Alternative Names

Sickle cell anemia

Highlights

Drug Warning

In 2006, the manufacturers of hydroxyurea (Droxia) updated this drug’s label to include more information on the risks for leg ulcers and gangrene in some patients. The new label also emphasizes that patients who use hydroxyurea should take precautions in handling the drug, including washing hands before and after contact with the bottles and capsules. People in the household who do not take hydroxyurea should use disposable gloves when handling this medication.

Pulmonary Hypertension

Pulmonary hypertension (high pressure in the blood vessels of the lungs) is a serious complication of sickle cell disease and a main cause of death. New research suggests that simple blood tests may help diagnose patients with sickle cell pulmonary hypertension and identify those who are at highest risk for dying from this condition. The proposed blood tests measure levels of:

• Brain natriuretic peptide (BNP). A 2006 study in the Journal of the American Medical Association indicated that higher levels of BNP are associated with pulmonary hypertension and increased mortality risk.
• Lactate dehydrogenase (LDH). A 2006 study in Blood found LDH levels rise with the severity of pulmonary hypertension. High LDH levels were also associated with leg ulcers, priapism (abnormal and painful penis erection), and early death.

Acute Chest Syndrome

Asthma increases the frequency and pain of acute chest syndrome in children, indicates a 2006 study in Blood . The researchers recommend that children who experience frequent bouts of acute chest syndrome should be screened for asthma. Acute chest syndrome is another serious, and common, complication of sickle cell disease.

• Review Date: 1/17/2007
• Reviewed By: Harvey Simon, MD, Editor-in-Chief, Associate Professor of Medicine, Harvard Medical School; Physician, Massachusetts General Hospital

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