All you need to know about Sickle Cell Anemia/Disease (SCD)

All you need to know about Sickle Cell Anemia/Disease (SCD)

By Olaide Osayemi

According to Pan African Medical journal, Nigeria has the largest population of people with sickle cell disease in the world with over 150,000 new cases of sickle cell disease born every year. The country has 75% of sickle cell cases, about one out of every four persons in Nigeria are carriers of sickle cell anemia. While It is estimated that in the United States, some 90,000 to 100,000 Americans are afflicted with sickle cell anemia. Overall, current estimates is that one in 500 U.S. African American births is affected with sickle cell anemia.

WHAT IS SICKLE CELL ANEMIA/DISEASE?

Sickle cell anemia (sickle cell disease) is a blood disorder caused by an inherited abnormal hemoglobin (the oxygen-carrying protein within the red blood cells). The abnormal hemoglobin causes distorted red blood cells. The sickled red blood cells are fragile and prone to rupture. When the number of red blood cells decreases from rupture (hemolysis), anemia is the result. This condition is referred to as sickle cell anemia. The irregular sickled cells can also block blood vessels causing tissue and organ damage and pain. Sickle cell anemia is one of the most common inherited blood anemias. The disease primarily affects Africans and African Americans.

FACTS ABOUT SICKLY CELL ANEMIA/ DISEASE

Sickle cell anemia (SCD) is an inherited disorder of the hemoglobin in blood.

Sickle cell anemia requires the inheritance of two sickle cell genes.

Sickle cell trait, which is the inheritance of one sickle gene, almost never causes problems.

Virtually all of the major symptoms of sickle cell anemia are the direct result of the abnormally shaped sickled red blood cells blocking the flow of blood.

The current treatment of sickle cell anemia is directed primarily toward managing the individual features of the illness as they occur.

HOW IS SICKLE CELL ANEMIA INHERITED?

Sickle cell anemia is inherited as an autosomal (meaning that the gene is not linked to a sex chromosome) recessive condition. This means that the gene can be passed on from a parent carrying it to male and female children. In order for sickle cell anemia to occur, a sickle cell gene must be inherited from both the mother and the father, so that the child has two sickle cell genes.

The inheritance of just one sickle gene is called sickle cell trait or the “carrier” state. Sickle cell trait does not cause sickle cell anemia.

Persons with sickle cell trait usually do not have many symptoms of disease and have hospitalization rates and life expectancies identical to unaffected people. When two carriers of sickle cell trait mate, their offspring have a one in four chance of having sickle cell anemia. (In some parts of Africa, one in four persons is a carrier for sickle cell trait.)

MAJOR FEATURES AND SYMPTOMS OF SCD

Fatigue and Anemia

Pain Crises

Dactylitis (swelling and inflammation of the hands and/or feet) and Arthritis

Bacterial Infections

Splenic Sequestration (sudden pooling of blood in the spleen) and Liver Congestion

Lung and Heart Injury

Leg Ulcers

Aseptic Necrosis and Bone Infarcts (death of portions of bone)

Eye Damage

OTHER FEATURES

Some features of sickle cell anemia, such as fatigue, anemia, pain crises, and bone infarcts can occur at any age. Many features typically occur in certain age groups.

Sickle cell anemia usually first presents in the first year of life. Infants and younger children can suffer with fever, abdominal pain, pneumococcal bacterial infections, painful swellings of the hands and feet and splenic sequestration. Adolescents and young adults more commonly develop leg ulcers, aseptic necrosis, and eye damage. Symptoms in adult typically are intermittent pain episodes due to injury of bone, muscle, or internal organs.

Affected infants do not develop symptoms in the first few months of life because the hemoglobin produced by the developing fetus (fetal hemoglobin) protects the red blood cells from sickling. This fetal hemoglobin is absent in the red blood cells that are produced after birth so that by 5 months of age, the sickling of the red blood cells is prominent and symptoms begin.

The treatment of sickle cell anemia is directed to the individual features of the illness present. In general treatment is directed at the management and prevention of the acute manifestations as well as therapies directed toward blocking the red blood cells from stacking together. There is no single remedy to reverse the anemia. It is, therefore, important that affected individuals and their family members have an optimal understanding of the illness and that communication with the doctors and medical personnel be maintained.

 

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