New Guideline for Sickle Cell Disease

An expert panel has created a new evidence-based guideline for managing sickle cell disease (SCD), with a strong recommendation for the use of the drug hydroxyurea and transfusion therapy for many individuals with SCD, according to an article in the September 10th 2014 issue of JAMA. The researchers say that this is advisable even though high-quality evidence is limited, with few randomized clinical trials conducted for this disease.

A release from the publisher notes that sickle cell disease is a life-threatening genetic disorder affecting nearly 100,000 individuals in the United States.Most of those affected are of African ancestry or self-identify as black. This disease is associated with many acute and chronic complications requiring immediate medical attention. Care for persons with SCD often lacks continuity. Primary care and emergency care health professionals need up-to-date clinical guidance regarding care of persons with this disease, according to background information in the article.

In 2009, the National Heart, Lung, and Blood Institute convened an expert panel that developed the Evidence-Based Management of Sickle Cell Disease: Expert Panel Report 2014. Barbara P. Yawn, M.D., M.Sc., M.S.P.H., of the Olmsted Medical Center, Rochester, Minn., and colleagues produced a summary of this report and conducted a search of the medical literature to examine the strength of the quality of evidence for the recommendations.

Among the strong recommendations:

• For prevention: daily oral prophylactic penicillin up to the age of 5 years, annual transcranial Doppler examinations from the ages of 2 to 16 years in those with sickle cell anemia, and long-term transfusion therapy to prevent stroke in those children with abnormal transcranial Doppler velocity (elevated speed of blood flow in the cerebral arteries).

• To address acute complications: rapid initiation of opioids for treatment of severe pain associated with a vasoocclusive crisis (blockage of blood flow due to the abnormal “sickled” red blood cells getting stuck in the blood vessels) and use of incentive spirometry (a method of encouraging deep breathing with the use of an instrument to provide feedback) in patients hospitalized for a vasoocclusive crisis.

• For chronic complications: use of analgesics and physical therapy for treatment of avascular necrosis (a condition in which poor blood supply to an area of bone leads to bone death) and use of angiotensin-converting enzyme inhibitor therapy for microalbuminuria (a subtle increase in the urinary excretion of the protein albumin that cannot be detected by a conventional urinalysis) in adults with SCD.

• For children and adults with proliferative sickle cell retinopathy: referral to expert specialists for consideration of laser photocoagulation and for echocardiography to evaluate signs of pulmonary hypertension.