Sickle cell anemia: A defective gene disease .


 

SICKLE CELL DISEASE OR SICKLE CELL ANEMIA

Sickle cell disease (SCD), also simply called sickle cell, is a group of hemoglobin-related blood disorders typically inherited. SCD is a hereditary blood disorder characterized by a mutation in the hemoglobin S gene, causing red blood cells to be crescent- or “sickle”-shaped, which can block blood flow to the rest of the body. 

Red blood cells are usually round and flexible. In sickle cell anemia, some red blood cells look like sickles used to cut wheat. These unusually shaped cells give the disease its name. 

Symptoms of sickle cell anemia usually appear around 6 months of age. They vary from person to person and may change over time. Symptoms can include: 

1. Anemia: This is a result of the fast destruction of sickle cells, which can’t get replaced fast enough, resulting in a shortage of red blood cells and a lack of oxygen for the body. 

2. Episodes of pain: Sickle cells blocking the blood flow of tiny blood vessels leads to pain crises, which can be severe and require hospitalization. 

3. Swelling of hands and feet: Sickle cells block blood circulation, causing the hands and feet to swell. 

4. Frequent infections: Sickle cells can damage the spleen, which is responsible for protecting against infections, making it harder for the body to fight off infections. 

5. Delayed growth and puberty: The lack of healthy red blood cells hinders the growth of babies and children and can delay puberty in teenagers. 

6. Vision problems: Sickle cells can block the blood supply to the eyes, damaging the retina and causing vision problems. 

7. Priapism: This is a painful obstruction of the blood vessels in the penis by sickle cells.

It is important to see your doctor right away if you or your child experiences any of these symptoms. Infections often start with a fever and can be life-threatening. Because children with sickle cell anemia are prone to infections, prompt medical attention should be sought for a fever greater than 101.5 degrees Fahrenheit (38.5 degrees Celsius). 

Sickle cell is an inherited disease caused by a defect in a gene. A person would be born with sickle cell disease if he inherits one gene each from the father and mother. 

If aperson inherits  one healthy gene and one defect gene then the person is said to be a “carrier”. If the carrier has a child with another carrier then they both have a high chance of having a child with the sickle cell  disease.  

Early diagnosis is of utmost importance in the treatment of sickle cell disease. Treatment may include pain medication, drinking a lot of water daily (8 – 10 glasses), blood transfusion, vaccinations and antibiotics, regular eye exams, folic acid, and bone marrow transplant. Sickle cell disease is a life-long condition.

 Though living a healthy lifestyle can reduce some of it’s complications, the greater part of it may not be entirely prevented. Sickle cell patients are faced with a lot of pain and this can either be due to the negligence or dismissal of partners and this is why it is important that partners are aware of their genotype before marriage.  


https://www.hopkinsmedicine.org/health/conditions-and-diseases/sickle-cell-disease

https://www.mayoclinic.org/diseases-conditions/sickle-cell-anemia/symptoms-causes/syc- 20355876


Compiled by Lkite Gold

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