Sickle cell disease is a group of inherited blood conditions that affect haemoglobin, the protein in red blood cells that carries oxygen. The best-known form is sickle cell anaemia. In sickle cell disease, red blood cells can become stiff and sickle-shaped, making them more likely to break down and block small blood vessels.
The condition can cause painful crises, anaemia, infection risk and organ complications. Severity varies widely. Some people have frequent hospital admissions, while others have milder disease with fewer symptoms.
Inheritance
Sickle cell disease is inherited when a child receives a sickle cell gene from both parents. A person with one sickle cell gene is usually described as having sickle cell trait or being a carrier.
Carrier status does not usually cause sickle cell disease, but it matters for family planning. If both parents are carriers, each pregnancy has a chance of producing a child with sickle cell disease.
Symptoms
Symptoms can start from a few months of age. Main problems include painful episodes, increased infections and anaemia.
Painful episodes, often called sickle cell crises, happen when sickled cells block blood flow. Pain can be severe, can last for days or weeks, and often affects the limbs, back, chest or abdomen. Triggers can include dehydration, cold, stress and strenuous exercise, although a clear trigger is not always found.
People with sickle cell disease are more vulnerable to infections, especially in childhood. They may also have chronic anaemia because sickled red cells break down sooner than healthy red cells.
Complications
Possible complications include acute chest syndrome, stroke, gallstones, leg ulcers, delayed growth, priapism, kidney problems, spleen problems, eye disease and severe anaemia after certain infections.
Acute chest syndrome is a serious lung complication that can cause fever, cough, chest pain and breathing difficulty. It normally requires urgent hospital treatment.
Diagnosis and Screening
In the UK, babies are offered newborn blood spot screening for sickle cell disease. Diagnosis may also be made through blood tests such as haemoglobin analysis. Carrier screening and genetic counselling may be offered to people with a family history or higher inherited risk.
Early diagnosis allows preventive care to begin before complications develop.
Treatment and Management
Sickle cell disease usually needs lifelong care from a specialist team. Management may include a personal care plan, vaccinations, antibiotic prevention, folic acid where appropriate, pain plans and advice on avoiding triggers such as dehydration and cold exposure.
Hydroxycarbamide, also known as hydroxyurea, may be used to reduce painful crises. People taking it usually need regular blood tests because it can lower white blood cells and platelets.
Blood transfusion or red cell exchange may be used for severe anaemia, stroke prevention, acute chest syndrome or other complications. People who receive many transfusions may need treatment to reduce excess iron.
Stem cell or bone marrow transplant is a potential cure, but it carries significant risks and is usually considered only for selected patients. In 2025, NHS England announced access to exagamglogene autotemcel, a gene-editing treatment for eligible older children and adults with severe recurrent crises who meet specialist criteria.
Living With Sickle Cell Disease
Regular review is important even when symptoms are quiet. People may be advised to keep hydrated, avoid sudden cold exposure, manage fever promptly, keep vaccinations up to date and seek help quickly for severe pain, breathing difficulty, signs of infection, neurological symptoms or sudden worsening anaemia.
The condition can affect school, work, travel, pain management, mental health and family life, so good care includes practical support as well as medical treatment.
See Also
References
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