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.
Due to the genetic nature of this illness, it cannot be prevented, but healthcare providers can provide patients with continuous care to reduce the incidence of crises and manifestation of symptoms. When a crisis does happen, usually it is the result of compound factors, but patients can take steps to reduce this occurrence including exercising moderately, staying hydrated, reducing stress, not smoking, drinking little or no alcohol, treating infections in a timely manner, and maintaining normal body temperature.
Taking actions to keep the blood cells round, helps prevent symptoms. If the patient takes care to drink sufficient fluids each day, not put himself or herself in settings with reduced oxygen such as places with a high altitude or by taking part in intense physical activity, and by avoiding exposure to extreme temperatures. The medication hydroxyurea is also sometimes prescribed to curb symptoms.
Additional measures that may be helpful include taking a folic acide supplement, which stimulates production of new red blood cells, and prompt use of antibiotics for any bacterial infections that may occur. Blood transfusions are given to some patients as a preventative step against incidence of stroke.
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.
In rare instances, a patient may be fortunate enough to find a suitable donor for a bone marrow or stem cell transplant, but this seldom happens. It is advisable for patients to make sure they receive the PPV, Hib, and PCV vaccinations as a prophylactic measure against infection.
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.
Due to the genetic nature of this illness, it cannot be prevented, but healthcare providers can provide patients with continuous care to reduce the incidence of crises and manifestation of symptoms. When a crisis does happen, usually it is the result of compound factors, but patients can take steps to reduce this occurrence including exercising moderately, staying hydrated, reducing stress, not smoking, drinking little or no alcohol, treating infections in a timely manner, and maintaining normal body temperature.
Taking actions to keep the blood cells round, helps prevent symptoms. If the patient takes care to drink sufficient fluids each day, not put himself or herself in settings with reduced oxygen such as places with a high altitude or by taking part in intense physical activity, and by avoiding exposure to extreme temperatures. The medication hydroxyurea is also sometimes prescribed to curb symptoms.
Additional measures that may be helpful include taking a folic acide supplement, which stimulates production of new red blood cells, and prompt use of antibiotics for any bacterial infections that may occur. Blood transfusions are given to some patients as a preventative step against incidence of stroke.
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.
In rare instances, a patient may be fortunate enough to find a suitable donor for a bone marrow or stem cell transplant, but this seldom happens. It is advisable for patients to make sure they receive the PPV, Hib, and PCV vaccinations as a prophylactic measure against infection.
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