Updated guidelines for management of sickle cell disease released

1. Strong recommendations based on high-quality evidence are made supporting the referral of children with abnormal transcranial Doppler velocity to stroke-prevention specialists, with additional strong recommendations for simple or exchange blood transfusion in this cohort.

2. Strong recommendations based on high-quality evidence are made for the prompt use of parenteral opioids in adults and children with painful vasoocclusive crises. A consensus statement based on panel expertise was made to utilize sickle cell disease-specific or individualized pain management protocols created by the patient’s sickle cell physician.

3. Strong recommendations based on high-quality evidence are made for the use of hydroxyurea therapy in adults experiencing three or more pain crises (of moderate or severe nature) in a given year. Hydroxyurea therapy is strongly recommended based on high quality evidence in all infants (9 months and older), children, and adolescents with sickle cell anemia so as to reduce the risk of sickle cell-related complications.

Statement Rundown: Sickle cell disease (SCD) affects close to 100,000 individuals in the United States and is associated with organ damage, stroke, skin ulceration, vasoocclusive crises, and avascular necrosis. This article is a summary of recommendations made by the National Heart, Lung, and Blood Institute (NHLBI) expert panel on preventative measures and acute and chronic care for sickle cell disease patients.

These recommendations represent the first updated guidelines in 10 years and utilize a reader-friendly presentation in demonstrating both the strength of recommendation and the quality of evidence on which it is based. However, the formulation of these guidelines did overlook a few of the tips for high-quality guideline construction offered by the Institute of Medicine. These discrepancies include the absence of endorsement by all professional societies with expertise in SCD-related complications and the absence of a patient representative on the guideline construction panel. Moreover, given the absence of robust clinical trial data on the treatment of sickle cell disease, many recommendations are based on lower-quality evidence. Nevertheless, these guidelines impart a number of strong recommendations based on high-quality evidence that will aid in the management of this condition.

Click to read the study, published today in JAMA

Click to read the accompanying editorial, published today in JAMA

Relevant Reading: US Centers for Disease Control and Prevention. Sickle cell disease: data and statistics

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