Clinical Review

Management of sickle cell disease in the community

BMJ 2014; 348 doi: https://doi.org/10.1136/bmj.g1765 (Published 10 March 2014) Cite this as: BMJ 2014;348:g1765
  1. Valentine Brousse, consultant paediatrician1,
  2. Julie Makani, consultant haematologist23,
  3. David C Rees, professor4
  1. 1Department of Pediatrics, Reference Centre for Sickle Cell Disease, Hôpital Universitaire Necker-Enfants Malades, APHP, Paris, France
  2. 2Department of Haematology and Blood Transfusion, Muhimbili University of Health and Allied Sciences, Dar es Salaam, Tanzania
  3. 3Nuffield Department of Medicine, Oxford, UK
  4. 4Department of Paediatric Haematology, King’s College Hospital NHS Foundation Trust, King’s Health Partners, London SE5 9RS, UK
  1. Correspondence to: D Rees david.rees2{at}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/β0 thalassaemia) 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.

What is sickle cell disease?

Sickle cell disease …

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