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Sickle Cell Anemia

Sickle cell disease is an inherited blood disorder.Sickle cell anemia is a serious condition. It is characterized by defective hemoglobin. HbS molecules tend to clump together, making red blood cells sticky, stiff, and more fragile, and causing them to form into a curved, sickle shape. Red blood cells containing HbS can go back and forth between being shaped normally and being sickle shaped until they eventually become sickle shaped permanently.

Instead of moving through the bloodstream easily, these sickle cells can clog blood vessels and deprive the body's tissues and organs of the oxygen they need to stay healthy. Normal red blood cells are shaped like doughnuts with the centers partially scooped out and are soft and flexible. Instead of being flexible and round, these cells are more rigid and curved in the shape of the farm tool known as a sickle - that's where the disease gets its name. The shape is similar to a crescent moon.

Sickle cell anemia is inherited as an autosomal recessive trait. This means it occurs in someone who has inherited hemoglobin S from both parents. Sickle cell disease is much more common in certain ethnic groups, affecting approximately one out of every 500 African Americans. Someone who inherits hemoglobin S from one parent and normal hemoglobin (A) from the other parent will have sickle cell trait .

Someone who inherits hemoglobin S from one parent and another type of abnormal hemoglobin from the other parent will have another form of sickle cell disease, such as thalassemia . ickle cell anemia is an inherited, lifelong condition. People who have sickle cell anemia are born with it. They inherit two copies of the sickle cell gene, one from each parent. People who inherit a sickle cell gene from one parent and a normal gene from the other parent have a condition called sickle cell trait.

Causes of Sickle Cell Anemia

Common causes of Sickle Cell Anemia

  • Defective hemoglobin.
  • Genetic disorder( HBB gene).

Symptoms of Sickle Cell Anemia

Common Symptoms of Sickle Cell Anemia

  • Abdominal pain
  • Bone pain
  • Breathlessness
  • Delayed growth and puberty
  • Fatigue.
  • Fever.
  • Jaundice.
  • Paleness.
  • Rapid heart rate.

Treatment of Sickle Cell Anemia

Common Treatment of Sickle Cell Anemia

  • Hydroxyurea (Hydrea) was found to help some patients by reducing the frequency of painful crises and episodes of acute chest syndrome (which includes chest pain and difficulty breathing). It also decreases the need for blood transfusions.
  • Avoid alcohol , drugs , and smoking , which can aggravate sickle cell disease and its symptoms.
  • Avoid places low in oxygen. (For example, it's not a good idea to go hiking at high altitudes or spend lots of time swimming under water.).
  • Avoid extreme cold or heat.
  • Giving oral penicillin twice a day beginning at 2 months and continuing until the child is at least 5 years old can prevent pneumococcal infection and early death.
  • Hydroxyurea is a first effective drug treatment for adults with severe sickle cell anemia.




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