Overview
Sickle cell disease is an inherited blood disorder in which red blood cells become stiff and crescent-shaped, blocking small blood vessels and causing painful crises and anaemia. It occurs when a child inherits the sickle gene from both parents, and is most common in people of African, Caribbean, Mediterranean, Middle Eastern and South Asian ancestry. Knowing whether you carry the gene helps couples understand the risk to their children. With good routine care, people with the disease live increasingly full lives.
Symptoms
- Episodes of severe bone, chest or abdominal pain (crises)
- Chronic tiredness and paleness
- Yellowing of the eyes
- Swollen, painful hands and feet in young children
- Frequent infections
- Delayed growth in children
- Leg ulcers in older patients
Causes & risk factors
- Inheriting the sickle haemoglobin gene from both parents
- Crisis triggers: dehydration, infection, cold, stress and overexertion
- Infections such as malaria worsening anaemia and triggering crises
Treatment & self-care
Routine care includes daily folic acid, vaccinations, prompt treatment of infections, plenty of fluids and regular specialist follow-up; doctors may prescribe hydroxyurea to reduce crises. Painful crises are managed with prescribed pain relief, warmth, fluids and rest, with hospital care for severe episodes — seek emergency care for chest pain or stroke-like symptoms. Carrier testing helps couples understand the risk to their children.
See a doctor urgently if
- Pain crisis not controlled by usual home measures
- Fever in anyone with sickle cell disease
- Chest pain, cough or difficulty breathing
- Sudden weakness, slurred speech or one-sided numbness
- Sudden severe paleness or an enlarging spleen in a child