Anaemia, Sickle Cell
Description Vaso-Occlusive Episodes General Measures Medicine Treatment Referral D57.0-3/D57.8 DESCRIPTION Sickle cell disease (SCD) is a genetic, inherited condition resulting in abnormal red blood cells. Homozygous SCD is the commonest and most severe form, characterised by recurrent vaso-occlusive episodes (“sickle crises”) and chronic haemolytic anaemia. Adults develop hyposplenism, predispos
D57.0-3/D57.8
DESCRIPTION
Sickle cell disease (SCD) is a genetic, inherited condition resulting in abnormal red blood cells. Homozygous SCD is the commonest and most severe form, characterised by recurrent vaso-occlusive episodes (“sickle crises”) and chronic haemolytic anaemia. Adults develop hyposplenism, predisposing them to infection with encapsulated bacteria. Heterozygous SCD includes Haemoglobin S-C disease/HbSC, causing milder sickle cell disease, and sickle cell trait/HbS, who are generally asymptomatic.
VASO-OCCLUSIVE EPISODES
Vaso-occlusion can involve any part of the body, especially bone. Episodes may be triggered by dehydration, infection, stress or menstruation. The most common presentation is with acute episodes of pain that vary in severity, in the affected areas.
Investigations
- The diagnosis is suspected from the history, peripheral blood examination, and/or screening tests for sickling.
- Diagnosis is confirmed on haemoglobin electrophoresis.
GENERAL MEASURES
Severe vaso-occlusive episodes
- Keep well hydrated with intravenous fluids.
- Transfusion is only indicated for severe episodes with severe anaemia – discuss with a specialist.
MEDICINE TREATMENT
Severe vaso-occlusive episodes
- Maintain adequate saturation with oxygen supplementation.
To prevent venous thromboembolism:
- Low molecular weight heparin (LMWH), e.g.:
- Enoxaparin, SC, 40 mg daily.
OR
- Unfractionated heparin, SC, 5 000 units 12 hourly.
LoE:IIIbxi
In morbid obesity dosing of LMWH should be individualised, in discussion with a specialist.
LoE:IIIaxii
In renal failure (eGFR <30 mL/minute), the recommended prophylactic dose of enoxaparin is 20 mg daily.
LoE:IVbxiii
Analgesia
- Control pain adequately – See: Medical conditions associated with severe pain.
Chronic management
All patients:
- Folic acid, oral, 5 mg daily.
- Vaccination against infections due to pneumococci and haemophilus influenza type b.
Management of severe vaso-occlusive episodes:
- Indications for treatment:
- frequent painful vaso-occlusive episodes,
- severe vaso-occlusive episodes (e.g. acute chest syndrome, stroke)
- severe symptomatic anemia.
- Hydroxyurea is the mainstay of therapy in severe disease – refer for specialist initiation.
REFERRAL
- All patients, for chronic management in a specialised centre.
- Vaso-occlusive episodes should be managed in consultation with a specialist.
Standard Treatment Guidelines And Essential Medicines List For South Africa, Adult Hospital level_Version 1_30 March 2026
Chapter 2: Blood and Blood Forming Organs; 2.1.6 Anaemia, Sickle Cell