Sickle cell anemia is an inherited disease in which the red blood cells, normally disc-shaped, become crescent shaped. Sickle cell anemia is a hereditary disorder that mostly affects people of African ancestry, but also occurs in other ethnic groups, including people who are of Mediterranean and Middle Eastern descent.
In many forms of the disease , the red blood cells change shape, usually looking much like that of a banana, upon deoxygenation because of polymerization of the abnormal sickle hemoglobin.
Hemoglobin molecules in each red blood cell carry oxygen from the lungs to body organs and tissues and bring carbon dioxide back to the lungs. Normal red blood cells are smooth and round like a doughnut without a hole.
They move easily through blood vessels to carry oxygen to all parts of the body. In sickle cell anemia, the body produces red blood cells that are shaped like a sickle (or crescent). These structures cause red blood cells to become stiff and assume a sickle shape.
Sickle cell anemia is characterized by a low number of red blood cells (anemia), infection, and periodic episodes of pain, usually beginning in early childhood. More than 70,000 Americans have sickle cell anemia.
The disease is chronic and lifelong. Individuals are most often well, but their lives are punctuated by periodic painful attacks. In addition to periodic pain, there may be damage of internal organs, such as stroke.
Red blood cells with normal hemoglobin ( HbA ) move easily through the bloodstream, delivering oxygen to all of the cells of the body. There are several common forms of sickle cell disease.
These are called SS (individuals inherit one sickle cell gene from each parent); SC (the child inherits one sickle cell gene and one gene for another abnormal type of hemoglobin called “C”); and S-beta thalassemia (the child inherits one sickle cell gene and one gene for beta thalassemia, another inherited anemia).
There’s no cure for most people with sickle cell anemia. However, treatments can relieve pain and prevent further problems.
Causes of Sickle Cell Anemia
The common causes of Sickle Cell Anemia include the following :
- Sickle cell anemia is caused by an abnormal type of hemoglobin called hemoglobin S. Hemoglobin is a protein inside red blood cells that carries oxygen.
- Cold weather (due to vasospasm).
- Hypoxia (flying in unpressurized aircraft).
- Sickle cell anemia is inherited as an autosomal recessive trait , which means it occurs in someone who has inherited hemoglobin S from both parents.
- Emotional stress.
- Dehydration (especially from exertion or during warm weather).
- Ingestion of bone marrow toxins (eg, phenylbutazone).
- Acute chest syndrome has been linked to fat embolism and infections.
- Alcohol intoxication.
Symptoms of Sickle Cell Anemia
Some signs and symptoms related to Sickle Cell Anemia include the following :
- Pain is the most common presentation of vasoocclusive crisis.
- Yellow eyes/skin.
- Susceptibility to infections.
- Ulcers on the lower legs (in adolescents and adults).
- Urinary symptoms (polyuria, hematuria, dysuria.)
- Shortness of breath or dyspnea (suggestive of acute chest syndrome).
- Rapid heart rate.
- Delayed growth and puberty.
- Delayed growth and puberty.
Treatment of Sickle Cell Anemia
Here is the list of the methods for treating Sickle Cell Anemia :
- Supplementation with folic acid , an essential element in producing cells, is required because of the rapid red blood cell turnover.
- If patients with sickle cell anemia are in crisis and are being transported by EMS, they should receive supplemental oxygen and intravenous hydration en route to the hospital.
- partial exchange transfusion for acute chest syndrome.
- transfusions or surgery for neurological events, such as strokes.
- Bone marrow transplant offers the only potential cure for sickle cell anemia. But very few people have a suitable donor for transplant.
- When severity of patient’s crisis is assessable, self-treatment at home with bed rest, oral analgesia, and hydration is possible.
- Standard treamtents for complications of sickle cell anemia include antibiotics, pain management, intravenous fluids, blood transfusion and surgery.