Management of sickle cell disease in the community

BMJ

2014; 348 doi: http://dx.doi.org/10.1136/bmj.g1765 (Published 10 March 2014)

Cite this as: BMJ 2014;348:g1765

Valentine Brousse, consultant paediatrician1,
Julie Makani, consultant haematologist23,
David C Rees, professor4

Author Affiliations

Correspondence to: D Rees david.rees2@nhs.net

Summary points

All children with sickle cell disease should take penicillin twice a day until 5 years of age at least
Acute neurological symptoms in children and adults with sickle cell disease necessitate urgent referral to hospital
All patients should receive annual vaccination against influenza and other appropriate vaccinations where available
Patients living in most parts of Africa should use insecticide treated bed nets and take malarial prophylaxis
Children with severe types of sickle cell disease (HbSS and HbS/β⁰thalassaemia) should be offered primary stroke prevention with annual transcranial Doppler scans and blood transfusion when resources allow this
Children and adults should be offered treatment with hydroxyurea if they have two or more episodes of severe acute pain in a year, or acute chest syndrome
Children, families, and adults should be offered education about sickle cell disease and managing its complications

Sickle cell disease is characterised by unpredictable episodes of acute illness, progressive organ damage, and a lack of effective treatments. It is one of the most common inherited conditions, although its prevalence varies widely. Median life expectancy is currently 40-60 years in high income countries but much less in low income areas.1 2 It is associated with protean clinical complications. Patients present to all medical specialties and increasingly to general practitioners. This review aims to provide an evidence based update on how to manage patients with this disease in the community. It does not consider sickle cell trait, which is largely asymptomatic.

Sources and selection criteria

We searched Medline, the Cochrane Database, and ClinicalTrials.gov using the term “sickle” together with certain complications including pain, infection, enuresis, renal failure, spleen, acute chest syndrome, leg ulcers, and stroke. Preference was given to randomised clinical trials, and when these were unavailable large case series were sought. We prioritised newer studies over older ones.