Sickle-Cell Anemia - How to Cope up with Sickle-Cell Anemia?

In the inherited disease called sickle-cell anemia, the red blood cells contain an abnormal hemoglobin, called hemoglobin S. If you have this disease, you have no normal hemoglobin in your red blood cells, because you have inherited a sickle-cell gene from each of your parents. This condition must be distinguished from sickle-cell trait, in which you inherit only one sickle-cell gene from one parent. Then you have red cells that contain half normal hemoglobin and half hemoglobin S, and your health is not impaired. In addition to hemolysis, or premature destruction of red blood cells, hemoglobin S causes red cells of persons with sickle-cell anemia to become deformed in shape, or “sickled,” especially in parts of the body where the amount of oxygen is relatively low. These abnormal blood cells do not flow smoothly through the capillaries, or smaller blood vessels. They may clog the vessels, and prevent blood from reaching the tissues. This blockage causes anoxia, or lack of oxygen, which makes the sickling worse. Attacks of this kind are called sickle-cell “crises.” They can be very painful.

What are the symptoms?

If you have sickle-cell anemia, you will have all the symptoms of anemia . In addition, you may have occasional sickle-cell crises, which produce attacks of pain in the bones and abdomen. You may also develop blood clots in the lungs, kidneys, brain, and most other organs.
How often crises occur varies a great deal from one person with the disease to another. Crises are more likely to occur during infections and after an accident or injury. They also occur with anesthesia and surgery if appropriate precautions are not taken.
Both the sickle-cell trait and sickle-cell anemia are virtually unknown except in people of African descent and in persons from parts of Italy, Greece, Arabia and India. About 1 in every 1000 black Americans has sickle-cell anemia.

What are the risks?

There is virtually no risk from sickle-cell trait, but a man and woman who both have the trait can produce a child with sickle-cell anemia. If you have sickle-cell anemia, you risk painful crises. Abnormalities of bone growth and severe infection from certain bacteria may also occur. Severe sickle-cell crises can damage most organs in your body by impairing blood flow, and this damage can lead to death from heart failure kidney failure or stroke.

What should be done?

If you or your child displays any of the symptoms described, see your physician, who will consider the possibility of sickle-cell anemia, especially if the disease is known to run in your family. Analysis of a blood sample will disclose whether the disease is present.
If you are of African descent and you are considering getting married or having a child, it is wise for you to be tested for sickle-cell trait. Because the trait is relatively common in the black population, about 6 of every 1000 black couples will have the capability of producing a child with sickle-cell anemia.

What is the treatment?

There is now no cure for an inherited disease such as sickle-cell anemia, but the symptoms can be treated. Crises of acute pain are the most common problem. These are treated with painkillers, and you often have to be admitted to the hospital for them. It is extremely important that you do everything possible to maintain good health, and that you obtain prompt treatment for infections, injuries and other illnesses. Also, you should see a physician regularly who is thoroughly familiar with the disease. Special precautions are necessary before you have any surgery, including dental surgery. Also, you should not fly in an un pressurized airplane or be at altitudes above about 6000 feet, at least not without special precautions and instructions.