Sickle cell anemia, or sickle cell disease is a genetic disease of the red blood cells (RBCs). Normally, RBCs are shaped like discs, which gives them the flexibility to pass through the smallest blood vessels but however, with this disease, the leukocyte have an abnormal crescent shape resembling a sickle and this makes them sticky, rigid and prone to getting trapped in small vessels, which blocks blood from reaching different parts of the body. This can cause pain and tissue damage.
Symptoms of sickle cell anemia.
Sickle cell anemia usually show up at a young age and they may appear in babies as early as 4 months old, but generally occur around the 6-months.
they are multiple types of scd and they all have similar symptoms, which vary in severity. These include:
excessive fatigue or irritability, from anemia
fussiness, in babies
bedwetting, from associated kidney problems jaundice, which is yellowing of the eyes and skin swelling and pain in hands and feet frequent infections
pain in the chest, back, arms, or legs. Other symptoms are Painful swelling of hands and feet,
Delayed growth and Vision problems.
Types of sickle cell disease.
Hemoglobin is the protein in red blood cells that carries oxygen. It normally has two alpha chains and two beta chains. The four main types of sickle cell anemia are caused by different mutations in these genes.
Hemoglobin SS disease which is the most common type of sickle cell disease. It occurs when you inherit copies of the hemoglobin S gene from both parents. This forms hemoglobin known as Hb SS. As the most severe form of SCD, individuals with this form also experience the worst symptoms at a higher rate.
Hemoglobin SC disease which is the second most common type of sickle cell disease. It occurs when you inherit the Hb C gene from one parent and the Hb S gene from the other. Individuals with Hb SC have similar symptoms to individuals with Hb SS. However, the anemia is less severe.
Hemoglobin SB+ (beta) thalassemia
Hemoglobin SB+ (beta) thalassemia affects beta globin gene production. The size of the red blood cell is reduced because less beta protein is made. If you inherite the Hb S gene, you will have hemoglobin S beta thalassemia. Symptoms are not as severe.
Hemoglobin SB 0 (Beta-zero) thalassemia
Sickle beta-zero thalassemia is the fourth type of sickle cell disease. It also involves the beta globin gene. It has similar symptoms to Hb SS anemia. However, sometimes the symptoms of beta zero thalassemia are more severe. It is associated with a poorer prognosis.
Hemoglobin SD, hemoglobin SE, and hemoglobin SO
These types of sickle cell disease are more rare and usually don’t have severe symptoms.
Who is at risk for sickle cell anemia?
Children are only at risk for sickle cell disease if both parents carry sickle cell trait. A blood test called a hemoglobin electrophoresis can also determine which type you might carry.
Complications of sickle cell anemia.
SCD can cause severe complications, which appear when the sickle cells block vessels in different areas of the body. Painful or damaging blockages are called sickle cell crises. They can be caused by a variety of circumstances, including:
changes in temperature
The following are types of complications that can result from sickle cell anemia.
Heart disease and chest. syndrome.
Sickle chest syndrome.
Long-term outlook for sickle cell disease.
The prognosis of the disease varies. Some patients have frequent and painful sickle cell crises. Others only rarely have attacks.
Sickle cell anemia is an inherited disease so it’s advisable you talk to a genetic counselor if you’re worried that you might be a carrier. This can help you understand possible treatments, preventive measures, and reproductive options.