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I am a sickle cell affected person with anaemia

Kindly find a space in your committed and dedicated weekly column to highlight about anaemia in people affected by sickle cell – Salamatu V.

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Sickle cell anaemia is an inherited form of anaemia, a condition in which there are not enough healthy red blood cells to carry adequate oxygen throughout the 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 crescents.  

The symptoms

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

1. Periodic episodes of pain: Also 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.  

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

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

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

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

Causes of anaemia 

• Sickle 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 colour (haemoglobin). Haemoglobin allows red blood cells to carry oxygen from the lungs to all parts of the body. In sickle cell anaemia, the abnormal haemoglobin 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 haemoglobin gene and one defective form of the gene, people with the sickle cell trait make both normal haemoglobin and sickle cell haemoglobin. Their blood may contain some sickle cells and they are called carriers.”

Complications 

1. 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 in breathing. Acute Chest Syndrome can be caused by lung infection or by sickle cells blocking blood vessels in the lungs. 

3. 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. 

4. 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.

5. Leg ulcers: Sickle cell anaemia can cause open sores, called ulcers, on the legs.

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

7. Priapism: Men with sickle cell anaemia can have painful, long-lasting erections, a condition called priapism. 

How to reduce crises

• Exercise regularly but not so much that you become really tired. When you exercise, drink lots of fluid.

• Drink at least eight 12-ounce glasses of water a day during warm weather.

• Reduce or avoid stress. Talk to your doctor if you’re depressed or have problems with your family or job.

• Treat any infection as soon as it occurs. When in doubt, see your doctor.

• Wear warm clothes outside in cold weather and inside in air-conditioned rooms during hot weather. Also, don’t swim in cold water.

• If you have another medical condition, like diabetes, get treatment and control the condition.

• If you are pregnant or plan to become pregnant, get early prenatal care.


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