Sickle cell is a group of disorders that causes red blood cells to become deformed or shrunken and breakdown.
Sickle cell disease is not an acquired disease but hereditary, it is seen mostly amongst African people or people of African ancestry.
An inherited disease or group of disorders, a sickle cell red blood have an abnormal or contorted crescent or sickle shape (hence the name “sickle” cell).
These cells die early as compared normal red blood cells leaving a shortage of healthy red blood cells and prevent blood from flowing causing sickle cell crisis.
General Causes of Sickle Cell
Sickle cell disease is caused by a problem in the hemoglobin-beta gene found on chromosome 11, the defect found forms abnormal hemoglobin.
For a child to be affected both parents must pass each of the sickle gene to the child. If a single gene is passed the person is healthy and said to be a “carrier” of the disease.
People with a single gene of sickle cell trait donot show symptoms, but can pass the gene to their child since they’re “carriers”.
Signs and Symptoms of Sickle Cell
Signs and symptoms usually show very early, they appear as early as 6 months of age.
1. Pain is one of the major sign of sickle cell anemia, this pain occurs when crescent shaped red blood cells restrict flow through tiny blood vessels to your chest, abdomen and joints. The pain is usually very excruciating and crippling, it last for hours or even days, some people encounter series of crisis in a year while others experience only a few in a year. A critical pain requires one to stay in a hospital.
2. A shortage in red blood cells can cause a slow growth in children and delayed puberty in teenagers as red blood cells provide the body with oxygen and nutrients required for growth and development of the body.
3. Tiny blood cells that provide the eyes can be mixed with sickle cells and cause damage to the retina leading to vision problems.
4. Restriction in the flow of blood causes the feet and hands to swell.
5. Red blood cells live as long as 120 days before they’re replaced but sickle cells die in 10-20 days causing shortage in red blood cells (anemia), when there’s not enough red blood cells the body runs low on oxygen and this can cause fatigue.
The Risk of facing Sickle Cell
People with sickle cell disease are prone to pulmonary hypertension, stroke, organ damage, blindness, pregnancy complication, joint deformity, Blindness.
How it can be treated
- Proper medications
- Blood transfusion
- This is rare and expensive but a bone marrow transplant helps with this disease.
Do not just see any doctor for check up, go to the right specialist, make sure to see a Haemotologist, cardiologist, pulmonologist, neurologist, paediatrician, primary Care provider (PCP) and Emergency medicine doctor.
There’s no cure for most people with sickle cell anemia. Treatments can relieve pain and help prevent complications associated with the disease. It is not a death sentence, people with sickle cell can live a happy life just as the rest of the world.
