What is Sickle cell anemia? A disease affecting a lot of Kenyans

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By Selija Achaya

Sickle cell anemia is a disease that is disturbing many Kenyan households management is always so expensive, especially with constant hospital visits and pain management drugs. Many families have also lost their relatives and parents losing their young children.

But what is sickle cell anemia?

Sickle cell anemia is a genetic disorder that causes red blood cells to become misshapen and break down.
With sickle cell disease, red blood cells contort into a sickle shape from their normal disk shape. The disk feature makes them travel easily, even in the tiniest blood vessels. Unlike the crescent-shaped sickle cells that make them rigid and sticky and thus cannot easily travel in the blood vessels causing blockages.

When the blockage occurs, it makes it hard for blood oxygen and necessary nutrients to be transported across. That is what to leads to chronic pain and tissue damage.

4 common types of sickle cell anemia

-Hemoglobin SC disease

Hemoglobin SC disease is the known second most common type of sickle cell disease. It comes about when you inherit the Hb C gene from one parent and the Hb S gene from the other. However, the anemia is less severe.

-Sickle cell trait

People who have the sickle cell trait have only inherited a mutated gene (hemoglobin S) from one parent. Most of the time, people with sickle cell have no symptoms or reduced symptoms.

-Hemoglobin SB 0 (Beta-zero) thalassemia

Sickle beta-zero thalassemia also involves the beta-globin gene. It is similar to Hb SS anemia. But with this kind of sickle cell anemia, the symptoms are severe. 

-Hemoglobin SB+ (beta) thalassemia

Hemoglobin SB+ (beta) thalassemia is a type of sickle cell disease that affects beta-globin gene production. It is also where the size of the red blood cell is reduced because less beta protein is made. 

Those at risk of contacting sickle cell anemia

 Sickle cell disease is diagnosed in kids. For a child to have sickle cell, they must have inherited it from both parents who have sickle cell traits. A blood test called hemoglobin electrophoresis can also determine which type you might carry. Sickle cell is prone in areas where there are malaria cases as they are most likely carriers.

The areas include Africa, India, the Mediterranean, and Saudi Arabia.

Common symptoms of sickle cell anemia

Excessive fatigue or irritability from anemia.

Fussiness, in babies.

Constant bedwetting from associated kidney problems.

Jaundice is a condition that involves the yellowing of the skin and even the eyes. 

Swelling of hands and feet that’s painful. 

Suffering from frequent infections.

Pain in the chest area, back, arms, or legs.

Top complications of sickle cell anemia to a person

Delayed growth

Sickle cell RBCs that do not supply enough oxygen and nutrients lead to slow growth and even sexual maturation.

Acute anemia

Anemia comes about due to a shortage of Red Blood Cells. Sickle cells are easily broken down .normal Reb blood cells live up to 120 days and sickle cell red blood cells have a maximum of 20 days shelf life. 

Neurological complications 

Sickle cell patients are prone to strokes, seizures, and even comatose states. It is due to the loss of supply of oxygen and blood to the brain due to blockages.

Eye complications 

Partial blindness and full-blown problems in sickle cell patients is caused by blockage of vessels supplying essential nutrients for eye functions t the retina.

Heart diseases

Sickle cell disease interferes with oxygen and nutrient supply due to blockages. And due to these, a person is susceptible to heart and chest complications like heart attacks and heart failure.

Lung Disease

Prolonged loss of supply of oxygen and nutrients to the lungs leads to lung damage. Especially when high blood pressure occurs in the lungs, a condition known as pulmonary hypertension. And the worse case of scarring of the lungs, a condition known as pulmonary fibrosis.

Sickle chest syndrome

It is the most severe complication of the sickle cell crisis. Here patients experience severe chest pains accompanied by cough, fever, shortage of breath, and sputum production.

Treatment

Sickle cell anemia or sickle cell disease does not have a cure. But here are a few treatment options to help offer sickle cell anemia patients quality of life.

-Blood transfusions, packed red blood cells are removed from the donated blood. Blood transfusion increases oxygen and blood supply.

Administration of fluids. When rehydrated it helps blood cells to go back to their normal disk shape.

-Treating all infections arising or present before they worsen the situation.

-Offering pain medication, especially during the sickle cell crisis. Here patients tend to be in a lot of pain.

-Increase oxygen supply here you can connect a sickle cell patient e to an oxygen tank and mask. It makes breathing easier and increases blood oxygen supply.

Conclusion

Sickle cell anemia is a very chronic disease. Coupled with pain, swellings, blindness, and sickle chest syndrome it makes life hard for a patient. Sickle cell anemia has no cure.

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