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What is Sickle Cell Disease (SCD)
Sickle-cell Anaemia (also known as sickle-cell disorder or sickle-cell disease) is a common genetic condition due to a haemoglobin disorder inheritance of mutant haemoglobin genes from both parents. About 5% of the world's population carries genes responsible for haemoglobinopathies. Each year about 300 000 infants are born with major haemoglobin disorders including more than 200,000 cases of sickle-cell anaemia in Africa. Red blood cells are usually round and flexible. In sickle cell anemia, some red blood cells look like sickles used to cut wheat. These unusually shaped cells give the disease its name.
Healthy & Sickled Red Blood Cell
Causes of Sickle Cell Anaemia
Sickle cell disease is inherited from each of your parent leading to a faulty hemoglobin protein anomaly called hemoglobin S.
Sickle cell gene inheritance
Signs and Symptoms of Sickle Cell Anaemia
Symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Symptoms can include:
- Anemia. Sickle cells break apart easily and die. Typical red blood cells usually live for about 120 days before they need to be replaced. But sickle cells usually die in 10 to 20 days, leaving a shortage of red blood cells.
- Episodes of pain. Periodic episodes of extreme pain, called pain crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints
- Swelling of hands and feet. Sickle-shaped red blood cells block blood circulation in the hands and feet, which can cause them to swell.
- Frequent infections. The spleen is important for protection against infections. Sickle cells can damage the spleen, raising the risk of developing infections. Babies and children with sickle cell anemia commonly receive vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.
- Delayed growth or puberty. Red blood cells provide the body with the oxygen and nutrients needed for growth. A shortage of healthy red blood cells can slow growth in babies and children and delay puberty in teenagers.
- Vision problems. Tiny blood vessels that supply blood to the eyes can become plugged with sickle cells. This can damage the portion of the eye that processes visual images, called the retina, and lead to vision problems.
Management of Sickle Cell Anaemia
Stem cell or bone marrow transplants are the only cure for sickle cell disease, but they're not done very often because of the significant risks involved. Stem cells are special cells produced by bone marrow, a spongy tissue found in the centre of some bones. They can turn into different types of blood cells. For a stem cell transplant, stem cells from a healthy donor are given through a drip into a vein. These cells then start to produce healthy red blood cells to replace the sickle cells.
- Take over-the-counter pain relievers, such as paracetamol or ibuprofen (do not give aspirin to children under 16 unless a doctor has prescribed it)
- Dietary supplements like folic acid, which helps stimulate the production of red blood cells
- If anaemia is particularly severe or persistent, treatment with blood transfusions, hydroxycarbamide or voxelotor may be necessary
Preventions of Sickle Cell Anaemia
Preventing painful episodes
The main thing you can do to reduce your chances of experiencing a painful episode (sickle cell crisis) is to try avoiding possible triggers.
You may be advised to:
- Drink plenty of fluids to avoid dehydration
- Wear warm clothing to stop you getting cold
- Avoid sudden temperature changes, such as swimming in cold water
