Sickle cell disease is caused by mutations in the β-globin gene that result in the production of abnormal forms of the β-subunit of hemoglobin. It is the most common potentially fatal genetic disease. (Source: UNICEF records).
In simpler terms, in sickle cell anemia, the flexible, round disc-shaped red blood cells that move easily through blood vessels are absent. Through research, it has been discovered that in sickle cell anemia, the red blood cells are shaped like sickles or crescent moons. These stiff, sticky cells can get stuck in small blood vessels, which can slow or block the flow of blood and oxygen to certain parts of the body.
In Nigeria, 150,000 babies are born with sickle cell disease each year, which means that two out of every 100 children have this health problem.
Most of the world’s sickle cell cases are believed to be in Africa. Speculation is that this is because the region is a place where endemic malaria thrives.
The effects of sickle cell disease begin early in life and continue until the stage where it destroys cells.
Most deaths result from late diagnosis of the disease, that is, a situation where the disease is not detected in early childhood and therefore its special treatment is not implemented until much later in life. The life expectancy of people with sickle cell disease is generally 42 years, but most do not live that long unless they are given proper care and the environment in which they live is set up to suit them and not trigger complications.
Sickle cell crises are caused by complications of sickle cell disease that block blood vessels in different parts of the body.
an unstable environment.
When a crisis occurs, the person suffers excruciating pain, may have a stroke or develop organ damage in the spleen or any other organ.
Symptoms of sickle cell disease can be seen as early as four months of age, but are best recognized by the sixth month. These symptoms are as follows:
Enuresis as a result of kidney problems that are the result of the sickle cell disease deformity that attacks the system.
Pain in the chest, back, arms and legs resulting from a lack of oxygen in the blood.
Fatigue caused by sickle cell activity in the body.
Swelling of the hands and feet is the first pronounced symptom of sickle cell disease in infants.
Children are at risk of developing this disease when both parents carry the sickle cell trait and do not do their due diligence in testing to determine if they are carriers and therefore incompatible for childbearing.
The best-known treatment for sickle cell disease is bone marrow transplantation, where the stem cells responsible for forming red blood cells are transplanted to replace the cells that cause damage to the body. But parents are advised to be tested first to see if they are both carriers. Although treatment procedures are not yet complete in African countries, but with the help of UNICEF and NGOs dedicated to this cause, there is hope that in the coming years more procedures will be discovered to help fight this disease, but in the meantime, people are being made aware that it is essential in the long run to get tested to find out if one is a carrier. As a parent, you should check for the symptoms listed above to determine if the disease exists early in a child’s life.