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.

If both parents have the disease or carry the hemoglobin-S gene, their children will also have it or be able to pass it on. Those with a Mediterranean or African heritage are most likely to have the condition, although it is also found amongst populations in the Middle East, Central and South America, and the Caribbean. These people are advised to attend genetic counseling if they are planning to have a family.

Sickling of the red blood cells is more likely when certain conditions are present including higher acidity, low oxygen levels, dehydration, low volume of blood, an injury, or use of anesthesia. When the malformed cells block the fine network of vessels through which blood moves to the bones, a crisis and pain result. Discomfort may be felt in the stomach, chest, arms, legs, or back and it can last from hours to days. When pain relievers offer little relief, it's necessary to go to the 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.

A bone marrow or stem cell transplant can usually eradicate the illness, however it is difficult to find a match for most patients. The risk of infection can also be lowered if patients are sure to receive the PCV, PPV, and Hib vaccinations.




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