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What is sickle cell anaemia?

Sickle cell anaemia, or sickle cell disease (SCD), is a genetic disease of the red blood cells (RBCs). Normally, RBCs are shaped like discs, which gives them the flexibility to travel through even the smallest blood vessels. However, with this disease, the RBCs have an abnormal crescent shape resembling a sickle. This makes them sticky and rigid and prone to getting trapped in small vessels, which blocks blood from reaching different parts of the body. This can cause pain and tissue damage.

SCD is an autosomal recessive condition. You need two copies of the gene to have the disease. If you have only one copy of the gene, you are said to have sickle cell trait.

What are the symptoms of sickle cell anaemia?

These include:

• excessive fatigue or irritability, from anaemia

• fussiness, in babies

• bedwetting, from associated kidney problems

• jaundice, which is yellowing of the eyes and skin

• swelling and pain in hands and feet

• frequent infections

• pain in the chest, back, arms, or legs

What are the types of sickle cell disease?

Hemoglobin SS disease

Hemoglobin SC disease

Hemoglobin SB+ (beta) thalassemia

Hemoglobin SB 0 (Beta-zero) thalassemia

Hemoglobin SD, hemoglobin SE, and hemoglobin SO

Sickle cell trait

How is sickle cell anaemia treated?

A number of different treatments are available for SCD:

• Rehydration with intravenous fluids helps red blood cells return to a normal state. The red blood cells are more likely to deform and assume the sickle shape if you’re dehydration.

• Treating underlying or associated infections is an important part of managing the crisis, as the stress of an infection can result in a sickle cell crisis. An infection may also result as a complication of a crisis.

• Blood transfusions improve transport of oxygen and nutrients as needed. Packed red cells are removed from donated blood and given to patients.

• Supplemental oxygen is given through a mask. It makes breathing easier and improves oxygen levels in the blood.

• Pain medication is used to relieve the pain during a sickle crisis. You may need over-the-counter drugs or strong prescription pain medication like morphine.

• Immunizations can help prevent infections. Patients tend to have lower immunity.

Bone marrow transplant has been used to treat sickle cell anaemia. Children younger than 16 years of age who have severe complications and have a matching donor are the best candidates.