sickle cell anemia is an inherited blood disease which can cause episodes of pain, damage to vital organs, and for some, death in childhood or early adulthood.
Its effects vary greatly from one person to another, and from one time to another in the same person.
Most people with sickle cell anemia enjoy reasonably good health much of the time. Oxygen-carrying red blood cells are normally round and flexible. But under certain conditions, the red blood cells of a person with sickle cell anemia may change into a crescent or sickle shape within the blood vessels.
Sickled cells tend to become trapped in the spleen and elsewhere and are destroyed. This results in a shortage of red blood cells which, when severe, can cause the patient to be pale, short of breath, and easily tired.
People with sickle cell disease may be prone to certain infections which can worsen their condition, and their physical growth and development are often slower than normal.
Sometimes certain conditions may worsen a patient's anemia. This can happen when red blood cells are either destroyed, not produced in sufficient amounts, or are removed from circulation.
Infections and other problems can speed up destruction of red blood cells. A tell-tale sign of this is the yellowing of the whites of patients' eyes.
Reduced red blood cell production can be caused by a viral infection or a vitamin deficiency, especially during pregnancy. Large numbers of red blood cells may be trapped by the spleen. This occurs primarily in young children.
There is no cure for sickle cell disease. Treatment is symptomatic; preventive measures are employed to reduce the incidence of crises and to avoid infections.
It is also important that the patient receive all available immunizing agents. Nutritional deficiencies should be corrected when present (folate supplementation is especially important), and then a well-balanced dietary intake should be maintained.
When a sickle cell crisis is due to inflammatory changes, medications such as corticosteroids are sometimes administered to relieve pain in the joints and elsewhere.
Measures to improve or maintain the general well-being of patients include increasing the fluid intake to one and a half to two times the usual amount. The additional fluids increases the fluid volume and encourages mobilization of the abnormal red cells.
Adequate nutrition is necessary to optimize the patient's resistance to infection and resources for healing, and to manage the anemia that is characteristic of sickle cell disease.
How can problems with this disease be avoided?
What are treatment options?
Would nutritional supplements be helpful?
If I have children, will they have this disease?