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What is sickle cell disease?
Sickle cell disease is a genetic condition in which the quality of hemoglobin is defective. This condition can cause abnormal hemoglobin that can result in abnormally-shaped (sickled) red blood cells (see illustration). These abnormal red blood cells cannot easily pass through small blood vessels leading to inadequate oxygen for the tissues of the body.
Sickle cells also have a shorter life span than normal red blood cells (10 to 20 days compared to 120 days). This rapid turnover may result in inadequate time to replace the red blood cells and may result in anemia.
In sickle cell anemia, one defective hemoglobin gene is inherited from each parent. If only one gene is inherited from one parent, then the condition is milder and referred to as sickle cell trait.
Symptoms of sickle cell anemia vary depending on its severity. Patients with sickle cell trait may experience mild, if any, symptoms at all. In sickle cell disease, symptoms are more significant, especially in episodes of acute crisis. These symptoms can include:
- generalized body aches and pain,
- chest pain,
- bone pain,
- shortness of breath,
- ulceration of skin,
- blindness, and
- delayed growth and puberty.
“Hemoglobin Concentration (Hb).” Medscape.
“Anemia of Chronic Disease and Renal Failure.” Medscape.