Information on Sickle cell anemia
Sickle cell anemia is a condition of the blood cells that affects mainly persons of African or mediterranean descent (Italian, Spanish, etc.). It is caused by an inherited abnormality of the oxygen carrying protein of the blood (hemoglobin).
Sickle cell anemia is an inherited form of anemia - a condition in which there aren't enough healthy red blood cells to carry oxygen throughout your body.
Causes of Sickle cell anemia
Sickle Cell Anemia (SCA) is caused by a change in the chemical composition of the protein (hemoglobin or Hgb) that carries the oxygen inside of the red blood cells (RBC's).
Normal Hgb is a round or ball-shaped folded molecule composed of 4 protein subunits - 2 alpha chains and 2 beta chains.
Symptom of Sickle cell anemia
The clinical course of sickle cell anemia does not follow a single pattern; some patients have mild symptoms, and some have very severe symptoms.
The basic problem, however, is the same: the sickle-shaped red blood cells tend to get stuck in narrow blood vessels, blocking the flow of blood.
- joint pain and other bone pain
- fatigue
- breathlessness
- rapid heart rate
- delayed growth and puberty
- susceptibility to infections
- ulcers on the lower legs (in adolescents and adults)
- Constipation
- bone pain
- attacks of abdominal pain
- fever
Because people with sickle cell trait don't have the disease, they may never discover that they carry the gene. That's why it's recommended that teens who are unsure of their sickle cell status ask their doctors about testing.
Treatment of sickle cell anemia disease
Patients with sickle cell disease need certain treatment and follow-up even when not having a painful crisis. Supplementation with folic acid, an essential element in producing cells, is required because of the rapid red blood cell turnover.
Treatment for sickle cell disease usually focuses on symptoms. While bone marrow transplant can be curative, this therapy is indicated in only a minority of patients, predominantly because of the high risk of the procedure and difficulty in finding suitable donors.
Therefore, the purpose of therapy is to manage and control symptoms resulting from crises and to try to limit the frequency of crises.
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