Important Facts About Sickle Cell Disease Prevention And Management Of Symptoms

By Lila Bryant


Sickle cell disease is caused by a genetic abnormality and results in misshapen red blood cells caused by abnormal hemoglobin. Instead of being circular, the cells take on a crescent shape which elongates them and prevents them from being able to move normally through the body's tiniest blood vessels which can lead to anemia, organ damage, cellular breakdown, bone marrow disorders, and considerable pain. While it isn't possible to prevent the incidence of this genetic condition, sickle cell disease prevention is largely designed to prevent crises and associated symptoms from occurring.

In order for someone to inherit this disease, both parents must have the disorder or carry the hemoglobin-S gene. If a person has this genotype, he or she may pass it on to any offspring. It is most prevalent amongst those of African or Mediterranean descent, but it is also found the Caribbean, Middle East, and Central and South America. Genetic counseling is advisable for these people before they have children.

Certain conditions increase the sickling of red blood cells including low blood volume, dehydration, elevated body acidity, low oxygen, injury, and anesthesia. A "crisis" occurs when the deformed cells begin to block the fine network of vessels that carry blood to the bones. Pain is typically felt in the back, legs, arms, chest, or stomach and it may last for hours or even days. If pain relievers don't help, one must seek treatment at a hospital.

Since the disease cannot actually be prevented, healthcare providers strive to provide patients with ongoing care that will subvert symptoms and lessen the incidence of a crisis. A crisis may be brought on by several factors, but in general patients can reduce its likelihood by not smoking, drinking little to no alcohol, exercising moderately, reducing stress, drinking lots of fluids, promptly treating infections, and keeping the body temperature normal.

The more the red blood cells maintain their round shape, the less likely symptoms will arise. It is important that patients do their part to make this happen including not placing oneself in situations with reduced oxygen levels such as high altitude locations or intense exercise, avoiding extreme temperatures, and drinking sufficient liquids. Doctors sometimes prescribe the drug hydroxyurea to control symptoms as well.

Folic acid supplements can be helpful in the synthesis of new red blood cells. In some cases blood transfusions will be given as a preventative measure against strokes. Children with this disorder seem to be especially prone to infections, in which case antibiotics will be given to prevent complications.

In cases where the condition has progressed further, it may be necessary to take more extreme action. This may mean dialysis or a kidney transplant, removal of the gallbladder if stones are present, hip replacement to correct avascular necrosis, eye surgery, and treatment of leg ulcers.

Patients who are fortunate enough to find a compatible bone marrow or stem cell donor may be eligible for a transplant, however it is usually very difficult to find a suitable match. It is also important for these patients to ensure that they receive the Hib, PCV, and PPV vaccines tot lower the risk of infection.




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