Sickle Cell Disease

Sickle cell disease is an inherited blood disorder in which abnormal haemoglobin (haemoglobin S) causes red blood cells to become rigid and sickle-shaped, leading to chronic haemolytic anaemia and episodes of vaso-occlusion. Tanzania has one of the higher burdens of sickle cell disease in the region.

• Chronic anaemia (fatigue, pallor)
• Recurrent painful crises affecting the bones, chest, or abdomen
• Jaundice
• Delayed growth in children
• Increased susceptibility to infections
• Over time, complications such as stroke, chronic organ damage, and leg ulcers

• Inherited when a child receives two copies of the sickle haemoglobin gene, one from each parent who carries the sickle cell trait.
• A genetic condition; it is not contagious and cannot be caught from another person.

• Newborn or early childhood screening using haemoglobin electrophoresis or similar laboratory testing confirms the diagnosis.
• A full blood count typically shows chronic anaemia.
• Family history of sickle cell disease or trait supports the diagnosis.

Long-term care includes daily folic acid supplementation, prompt treatment of infections, and penicillin prophylaxis in early childhood to reduce the risk of severe bacterial infection.

Painful crises are managed with adequate fluids and pain relief, escalating to stronger analgesia and hospital admission for severe crises.

Blood transfusion is used for severe anaemia or specific complications such as stroke or acute chest syndrome.

Hydroxyurea may be offered at specialist level for patients with frequent severe crises to reduce how often they occur.

• Genetic counselling for couples with known sickle cell trait.
• Newborn screening for early diagnosis.
• Routine childhood immunisation and penicillin prophylaxis to prevent infections.
• Prompt treatment of fever and infections.
• Avoid known crisis triggers such as dehydration, extreme cold, and high altitude.