I need information about sickle cell anaemia

We will like you to shed more light on sickle cell anaemia, and some preventive measures to avoid complications. As I have someone diagnosed with the problem.

Mr L P.

Thanks Mr. L. P. for your question. Sickle cell anemia is an inherited form of anemia, “a condition in which there aren’t enough healthy red blood cells to carry adequate oxygen throughout your body.” Normally, red blood cells are flexible and round, moving easily through the blood vessels. “In sickle cell anaemia, the red blood cells become rigid and sticky and are shaped like sickles or crescent moons.” There’s no cure for most people with sickle cell anaemia. But treatments can relieve pain and help prevent problems.

What symptoms?

• “Sickle

are the

Signs and symptoms of sickle cell anaemia, vary from person to person and change over time. Some of them are:

1. Anaemia. Sickle cells break apart easily and die, leaving one without enough red blood cells. 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 (anemia).

2. Episodes of pain. Periodic episodes of pain, called crises, are a major symptom of sickle cell anaemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to the chest, abdomen and joints. Pain can also occur in the bones.

3. Some adolescents and adults with sickle cell anaemia also have chronic pain, which can result from bone and joint damage, ulcers and other causes.

4. Painful swelling of hands and feet. The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.

5. Frequent infections. Sickle cells can damage an organ that fights infection (spleen), leaving one more vulnerable to infections.

6. Delayed growth. Red blood cells provide the body with the oxygen and nutrients one need for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.

7. Vision problems. Tiny blood vessels that supply the eyes may become plugged with sickle cells. This can damage the retina, leading to vision problems.

If one sees or notices any of the above, he/she must visit hospital.

Potential causes of anaemia

cell anaemia is caused by a mutation in the gene that tells the body to make the red, iron-rich compound that gives blood its red color (hemoglobin). Hemoglobin allows red blood cells to carry oxygen from the lungs to all parts of the body. In sickle cell anaemia, the abnormal hemoglobin causes red blood cells to become rigid, sticky and misshapen.

• The sickle cell gene is passed from generation to generation in a pattern of inheritance called autosomal recessive inheritance. This means that both the mother and the father must pass on the defective form of the gene for a child to be affected.

• If only one parent passes the sickle cell gene to the child, that child will have the sickle cell trait. With one normal hemoglobin gene and one defective form of the gene, people with the sickle cell trait make both normal hemoglobin and sickle cell hemoglobin. Their blood might contain some sickle cells and called carriers.”

Examples complications of

1. Stroke. A stroke can occur if sickle cells block blood flow to an area of the brain. Signs of stroke include seizures, weakness or numbness of the arms and legs, sudden speech difficulties, and loss of consciousness.

2. Acute chest syndrome. This life-threatening complication causes chest pain, fever and difficulty breathing. Acute chest syndrome can be caused by a lung infection or by sickle cells blocking blood vessels in the lungs.

3. Pulmonary hypertension. People with sickle cell anaemia can develop high blood pressure in their lungs (pulmonary hypertension).

4. Organ damage. Sickle cells that block blood flow through blood vessels immediately deprive the affected organ of blood and oxygen. Chronic deprivation of oxygen-rich blood can damage nerves and organs in the body, including kidneys, liver and spleen.

5. Blindness. Sickle cells can block tiny blood vessels that supply the eyes. Over time, this can damage the portion of the eye that processes visual images (retina) and lead to blindness.

6. Leg ulcers. Sickle cell anaemia can cause open sores, called ulcers onthe legs.

7. Gallstones. The breakdown of red blood cells produces a substance called bilirubin. A high level of bilirubin in the body can lead to gallstones.

8. Priapism. Men with sickle cell anaemia can have painful, long-lasting erections, a condition called priapism. Sickle cells can block the blood vessels in the penis. This can damage the penis and lead to impotence.