Blood, immune & lymphGeneticICD-10 D57
Sickle cell disease
An inherited blood disorder in which abnormal haemoglobin makes red cells stiff and sickle-shaped. They block small vessels, causing painful crises and organ damage.
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Causes
- Two copies of the sickle haemoglobin (HbS) gene
Risk factors
- African, Caribbean, Middle Eastern or Indian ancestry
Symptoms
- Severe pain crises
- Anaemia and fatigue
- Infections
- Jaundice
- Swollen hands and feet in infants
- Stroke
Diagnosis — tests and examinations
- Newborn screening
- Haemoglobin electrophoresis
Treatment
Medicines and medical treatment
- Hydroxyurea
- Pain management
- Penicillin prophylaxis and vaccinations
- Folic acid
- Crizanlizumab, L-glutamine
Operations and procedures
- Blood transfusions and exchange transfusion
- Stem cell transplant
- Gene therapies (exagamglogene, lovotibeglogene)
Self-care, home remedies and lifestyle
- Hydration, warmth, avoid overexertion
- Regular transcranial Doppler screening in children
Possible complications
- Acute chest syndrome
- Stroke
- Splenic sequestration
- Kidney damage
- Avascular necrosis
Prevention
- Carrier screening and genetic counselling
Outlook
Life expectancy has improved greatly; gene therapy may be curative.
When to see a doctor
Seek urgent care for fever, chest pain, breathlessness or stroke symptoms.
Sources and further reading
These sources cover this condition. They have not been checked against every statement on this page.
Related conditions
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