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Sickle Cell Anemia

INFORMATION ON SICKLE CELL ANEMIA
Sickle cell anemia is a blood disorder that affects hemoglobin; a protein found in red blood cells that helps carry oxygen throughout the body. Sickle cell anemia occurs when an abnormal form of hemoglobin is produced. Hemoglobin molecules tend to clump together, making red blood cells sticky, stiff, and more fragile, and causing them to form into a curved, sickle shape. Sickle cell anemia is an inherited disease in which the red blood cells, normally disc-shaped, become crescent shaped. As a result, they function abnormally and cause small blood clots. These clots give rise to recurrent painful episodes called "sickle cell pain crises.” Sickle cell anemia occurs when a person inherits two abnormal genes that cause their red blood cells to change shape.

SYNONYMS OF SICKLE CELL ANEMIA
Synonyms and related keywords:
1. Sickle cell anemia.
2. Sickle disease.
3. Sickle hemoglobinopathy syndromes.
4. Hemolytic anemia.
5. Aplastic anemia crisis.
6. Hemoglobin synthesis.

CAUSES OF SICKLE CELL ANEMIA
Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. The fragile, sickle-shaped cells deliver less oxygen to the body's tissues, and can break into pieces that disrupt blood flow. Sickle cell anemia is inherited as an autosomal recessive trait, which means it occurs in someone who has inherited hemoglobin S from both parents. Repeated crises can cause damage to the kidneys, lungs, bones, eyes, and central nervous system.

SYMPTOMS OF SICKLE CELL ANEMIA
Symptoms of Sickle Cell Anemia are:

1. Paleness, Yellow Eyes/Skin.
2. Fatigue, Breathlessness.
3. Rapid Heart Rate, Delayed Growth and Puberty.
4. Susceptibility to Infections.
5. Ulcers on the Lower Legs.
6. Jaundice, Bone Pain.
7. Attacks of Abdominal Pain.
8. Fever, Bloody Urine.
9. Frequent Urination.
10. Excessive Thirst.
11. Painful Erection.
12. Chest Pain.
13. Poor eyesight/blindness.

DIAGNOSIS OF SICKLE CELL ANEMIA
Tests for sickle cell anemia include:

1. Complete blood count.
2. Hemoglobin electrophoresis.
3. Sickle cell test.

TREATMENT OF SICKLE CELL ANEMIA
Patients with sickle cell disease need continuous treatment, even when they are not having a painful crisis. Painful episodes are treated with analgesics and adequate liquid intake. Treatment of pain is critical. Newer drugs are being developed to manage sickle cell anemia. Bone marrow transplants can be curative; this therapy is indicated in only a minority of patients. Antibiotics and vaccines are given to prevent bacteria infections, which are common in children with sickle cell disease.

Other treatment is:

1. Partial exchange transfusion for acute chest syndrome.
2. Transfusions or surgery for neurological events, such as strokes.
3. Dialysis or kidney transplant for kidney disease.
4. Irrigation or surgery for priapism.
5. Surgery for eye problems.
6. Gallbladder removal.
7. Wound care, zinc oxide, or surgery for leg ulcers.
 

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