Friday, June 6, 2014

What You Should Know About Sickle Cell Disease Prevention And Symptom Management

By Lila Bryant

Sickle cell disease is a potentially life-threatening disorder which results in the development of malformed red blood cells due to faulty hemoglobin. These irregular cells take on a sickle shape and are unable to flow freely through the body's smallest blood vessels, leading to organ damage, anemia, breakdown of blood cells, and bone marrow disorders, pain and suffering. Since the condition is genetic and cannot be eliminated, sickle cell disease prevention is primarily concerned with taking measures to prevent crises from occurring and reducing symptoms.

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.

Red blood cells are more likely to sickle when they are subjected to certain bodily conditions such as low oxygen, increased acidity, reduced blood volume, an injury or use of anesthetics. If the malformed cells start to block the delicate network of vessels supplying the bones with blood, a "crisis" occurs. During a crisis the patient may experience pain the arms, legs, back, chest, or stomach which may continue for a few hours or several days. Pain relievers may help, but if they don't medical attention is necessary.

There is no way of entirely preventing this disorder, but healthcare providers can offer these patients regular care to help lower the occurrence of a crisis and curtail symptoms. In most cases a crisis results from multiple factors, but patients can help reduce its incidence by refraining from smoking, drinking no or little alcohol, reducing stress, staying hydrated, getting moderate exercise, treating infections promptly, and maintaining a normal body temperature.

If the red blood cells stay round, this can stave off the symptoms of the illness. There are different measures the patient can take to help accomplish this, such as staying hydrated, avoiding extreme temperatures, and by not undergoing intense physical training or being in areas with high altitude that have low oxygen. The drug hydroxyurea can also be effective in preventing symptoms from manifesting.

Taking a folic acid supplement can be beneficial as it helps produce new red blood cells. Blood transfusions are also sometimes given preventatively to reduce the incidence of stroke. If infection is suspected, antibiotics will be prescribed as to avoid complications, children in particular are often susceptible to bacterial infections.

If the disease has progressed, it may be necessary to take more extreme measures. When kidney damage has occurred, dialysis or even a transplant may be needed. If gallstones have formed in the gallbladder it may be necessary to remove the organ. Avascular necrosis of the hip can be corrected with a hip replacement procedure, and eye disorders remedied with surgery. Leg ulcers will be treated with standard wound care.

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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