Management of Sickle Cell Disease: Recommendations from the 2014 Expert Panel Report

 


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Am Fam Physician. 2015 Dec 15;92(12):1069-1076A.

  Patient information: A handout on this topic is available at http://familydoctor.org/familydoctor/en/diseases-conditions/sickle-cell-disease.html.

Author disclosure: No relevant financial affiliations.

Family physicians are the primary and sometimes only health care resource for families affected by sickle cell disease. Recently published guidelines provide important recommendations for health maintenance, acute care, and monitoring of disease-modifying therapy in persons with this condition. This overview highlights some of the most important clinical activities that can and should be carried out in the community care setting. Children with sickle cell anemia should receive prophylactic penicillin from birth through at least five years of age, and all persons with sickle cell disease require vaccination to prevent invasive pneumococcal disease. Annual screening with transcranial Doppler ultrasonography is recommended for all children with sickle cell disease beginning at two years of age and continuing through adolescence to evaluate the risk of stroke and to initiate transfusion therapy in those at high risk. Vasoocclusive crises require immediate and adequate analgesia appropriate to the level of patient-reported pain. Antibiotics, hospitalization, and incentive spirometry are indicated for those with acute chest syndrome. There is strong evidence to support the promotion and use of hydroxyurea therapy in patients nine months and older who have sickle cell anemia because its use can decrease the frequency of vasoocclusive crises and acute chest syndrome with limited adverse effects.

Family physicians and family medical homes are essential to the care of children, adults, and families affected by sickle cell disease (SCD). Many of the approximately 100,000 U.S. residents who have SCD do not have regular or convenient access to comprehensive SCD centers. This summary of the recently published National Institutes of Health–sponsored SCD guidelines, Evidence-Based Management of Sickle Cell Disease, Expert Panel Report 2014,1 is intended to support, enhance, and expand the knowledge of basic aspects of care for patients with SCD. Specifically highlighted are several clinical actions to enhance preventive care, manage some of the most common acute and chronic complications of SCD, and initiate and monitor the two SCD-specific disease-modifying therapies, hydroxyurea and chronic blood transfusion therapy.

WHAT IS NEW ON THIS TOPIC: SICKLE CELL DISEASE

Offer hydroxyurea therapy to infants, children, and adolescents with sickle cell anemia regardless of clinical severity to reduce sickle cell disease–related complications.

Use an individualized prescribing and monitoring protocol or a sickle cell disease–specific protocol whenever possible to promote rapid, effective, and safe analgesic management and resolution of vasoocclusive crises in children and adults.

View/Print Table

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Administer prophylactic oral penicillin (125 mg twice daily for children younger than three years; 250 mg twice daily for those three years and older) until at least five years of age in all children who are homozygous for sickle hemoglobin.

B

911

Screen children with sickle cell anemia annually with transcranial Doppler ultrasonography from two until at least 16 years of age.

A

1214

Initiate rapid treatment with opioids in adults and children with sickle cell disease and a vasoocclusive crisis with severe pain.

B

2325

Initiate hydroxyurea therapy in adults with sickle cell anemia who have one or more of the following: three or more moderate to severe pain crises in a 12-month period; daily sickle cell pain that affects quality of life; severe or recurrent acute chest syndrome; or severe symptomatic chronic anemia.

B

1, 5, 3234, 37

Pregnant or breastfeeding women should discontinue hydroxyurea therapy.

C

27

Offer hydroxyurea therapy for infants nine to 42 months of age and for older children and adolescents with sickle cell anemia, regardless of clinical severity, to reduce sickle cell disease–related complications.

B

35, 36, 38


A = consistent, good-quality patient-oriented evidence; B = inconsistent or limited-quality patient-oriented evidence; C = consensus, disease-oriented evidence, usual practice, expert opinion, or case series. For information about the SORT evidence rating system, go to http://www.aafp.org/afpsort.

SORT: KEY RECOMMENDATIONS FOR PRACTICE

Clinical recommendationEvidence ratingReferences

Administer prophylactic oral penicillin (125 mg twice daily for children younger than three years; 250 mg twice daily for those three years and older) until at least five years of age in all children who are homozygous for sickle hemoglobin.

B

911

Screen children with sickle cell anemia annually with transcranial Doppler ultrasonography from two until at least 16 years of age.

A

1214

Initiate rapid treatment with opioids in adults and children with sickle cell disease and a vasoocclusive crisis with severe pain.

B

2325

Initiate hydroxyurea therapy in adults with sickle cell anemia who have one or more of the following: three or more moderate to severe pain

The Authors

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BARBARA P. YAWN, MD, MSc, MSPH, FAAFP, is director of the Department of Research at Olmsted Medical Center, Rochester, Minn., and adjunct professor in the Department of Family and Community Health at the University of Minnesota in Minneapolis....

JOYLENE JOHN-SOWAH, MD, MPH, is a medical officer at the Preventive Medicine Center for Translation Research and Implementation Science for the National Heart, Lung, and Blood Institute in Bethesda, Md.

Address correspondence to Barbara P. Yawn, MD, MSc, MSPH, Olmsted Medical Center, 210 9th St. SE, Rochester, MN 55904 (e-mail: byawn47@gmail.com). Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

REFERENCES

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