Sickle cell anemia is an inherited form of blood disorder — a condition in which there aren’t enough healthy red blood cells to carry adequate oxygen throughout your body.
Usually, the red blood cells are flexible and round (doughnut shape), moving easily via the blood vessels. In the case of sickle cell, the red blood cells are sickle in shape, they are not flexible rather are sticky and rigid. These c- shaped cells can get stuck in small blood vessels, which can reduce or block blood flow and oxygen to parts of the body.
SIGNS & SYMPTOMS
The symptoms and signs of sickle cell anemia vary from person to person over time.
It is very easy for the sickle cells to die, leaving no red blood cells. Usually red blood cells live for 120days before replacement, but sickle cell dies in 10-20days, leaving shortage of oxygen and red blood cells causing anemia & fatigue.
Episodes of pain called crises are a major symptom of sickle cell anemia. The pain develops when the sickle red blood cells flow through tiny blood vessels to their chest, abdomen and joins. Pain can also occur in their bones.
If crisis is severe, patients need to be hospitalized. Some adults and adolescents with sickle cell anemia also have chronic pain which can results to bone/joint damage, ulcer, stroke etc.
Painful swelling of hands and feet: The swelling is caused by sickle-shaped red blood cells blocking blood flow to the hands and feet.
Frequent infections: Sickle cells can damage an organ that fights infection (spleen), leaving you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.
Delayed growth: Red blood cells provide your body with the oxygen and nutrients you need for growth. A shortage of healthy red blood cells can lead to stunted growth in infants and children and delay puberty in teenagers.
Vision problems: Tiny blood vessels that supply your eyes may become plugged with sickle cells.
A sickle cell patient should see a doctor immediately or seek emergency medical care when he/she has:
- One-sided paralysis or weakness
- Trouble walking or talking
- Vision problem
- Swelling of the hand or feet
- Unexplained episodes of severe pain
MANAGEMENT OF SICKLE CELL
Sickle cell anemia is efficiently managed with Drepanostat (Hydroxymethylbenzoic acid).
This is a sickle cell management drug which comes in 10mg capsules. Drepanostat inhibits sickling of the red blood cells by enhancing its oxygen carrying capacity through increased production of Fetal Hemoglobin in the body. It is safe and well tolerated in infants and adults.
Drepanostat can be used during crisis and also as prophylaxis.
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