New clinical guidelines for treatment of SCD

An expert panel of specialists from the National Heart, Lung, and Blood Institute (NHLBI) has issued new guidelines for the management and treatment of sickle cell disease (SCD). How will these latest evidence-based recommendations affect the care of your patients?

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Evidence-Based Management of Sickle Cell Disease: Expert Panel Report 2014 supports primary care physicians, nurses, and other healthcare professionals who provide inpatient, outpatient, or emergency care for adults and children with SCD.

The expert panel was comprised of specialists from pediatric and adult hematology, family medicine, internal medicine, obstetrics and gynecology, maternal/fetal medicine, emergency department nursing, psychiatry and mental health, transfusion medicine, and evidence-based medicine.

The researchers performed literature searches of multiple medical databases to identify relevant randomized clinical trials, nonrandomized intervention studies, and observational studies from 1980 through 2014. A final subset of 1575 original studies was used to summarize the findings and support the quality of evidence. In addition to the systematic searches, existing evidence-based clinical guidelines were incorporated into the new SCD recommendations.

Before its publication, the guidelines were reviewed by the NHLBI Advisory Council, a separate panel of SCD experts, and the National Blood Disorders Program coordinating committee; vetted during a period of public review and comment; and reviewed by the American Academy of Pediatrics, the American Society of Hematology, and the American Society of Pediatric Hematology/Oncology.

The SCD guidelines are divided into 5 sections that address health maintenance, including prevention and screening; diagnosis and management of acute and chronic complications; use of hydroxyurea therapy; and blood transfusion therapy.

Among the strong recommendations with the highest quality of evidence are:

• Daily prophylactic oral penicillin should be given to children aged up to 5 years.
• Children with conditional (170-190 cm/s) or elevated (≥200 cm/s) transcranial Doppler results should be referred to a specialist in long-term transfusion therapy to prevent stroke.
• Rapidly initiate parenteral opioids in adults and children for severe pain associated with vaso-occlusive crisis.
• Use simple or exchange transfusion for children with transcranial Doppler reading of 200 cm/s (time-averaged, mean-maximal cerebral blood flow velocity).
• In infants aged 9 to 42 months and older, children, and adolescents with sickle cell anemia, treat with hydroxyurea regardless of clinical severity to reduce complications related to SCD.

The researchers say that high-quality evidence is still lacking in many areas important for the care of patients with SCD. However, these new guidelines highlight the importance of collaboration between the primary care physicians and SCD experts to screen for and manage the disease as well as to reduce the high morbidity and mortality among these patients.

To read the full report and guidelines, log on to http://www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines/.

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