Sickle cells: When you inherit life-long blood disorder

Sickle cells anemia is an inherited blood disorder that mostly affects people of African ancestry, but also occurs in other ethnic groups, including people who are of Mediterranean and Middle Eastern descent. It is a hereditary disease that affects the red blood cells and causes them to become sickled or crescent-shaped.

Sickle cells anemia is an inherited blood disorder that mostly affects people of African ancestry, but also occurs in other ethnic groups, including people who are of Mediterranean and Middle Eastern descent. It is a hereditary disease that affects the red blood cells and causes them to become sickled or crescent-shaped.

According to WHO, painful episodes can occur when sickled red blood cells, which are stiff and inflexible, get stuck in small blood vessels.

These episodes deprive tissues and organs of oxygen-rich blood and can lead to organ damage, especially lungs, kidneys, spleen, and brain. This makes it hard for oxygen to reach these organs.

The cells that make up these organs die when they fail to get oxygen.

Although the number of people with sickle cells in Rwanda is unknown, worldwide, it has been estimated that 250,000 children are born with sickle-cell anemia annually.

Dr Fabien Ntaganda, a pathologist at Rwanda Military Hospital, said people who suffer from sickle cells may require frequent blood transfusion, prevention of infection, pain management, chemotherapy, bone marrow transplant, and gene therapy, depending on the severity of the disease.

“Sickle cells is an incurable disease and the only tentative for cure is a bone marrow transplant and gene therapy although it isn’t successful for all patients,” Dr Ntaganda said, adding that women who have sickle cells can still give birth to babies free from the disease.

Symptoms

Signs and symptoms of sickle cell usually show up after an infant is four months old and may include anemia–shortage of red blood cells. Without enough red blood cells in circulation, the body cannot get the oxygen it needs to feel energised, according to Mayo Clinic.

Sufferers are at risk of frequent infections as they spleen, an organ that fights infection, is damaged.  Other symptoms include hand-foot syndrome, delayed growth, vision problems, and periodic episodes of pain among others.

People with sickle cell anemia can also experience complications from impaired blood circulation and infection-fighting problems. These include a higher risk of certain infections and stroke as well as a condition called acute chest syndrome, which is caused by inflammation, infection, or occlusions (blockages) of blood vessels in the lungs by sickled cells, according to kidshealth.org.

Sickle cell anemia is not contagious, so you can’t catch it from someone else or pass it to another person. People with sickle cell anemia have inherited two sickle cell genes, one from each parent.

A child who has inherited the sickle cell gene from only one parent will not develop the disease, but will have sickle cell trait. People who have sickle cell trait don’t have sickle cell anemia and usually don’t have any signs of the disorder, but they can pass the sickle cell gene on to their own children.

Because people with sickle cell trait don’t have the disease, they may never discover that they carry the gene. That’s why it’s recommended that teens who are unsure of their sickle cell status ask their doctors about testing.

 

Have Your SayLeave a comment