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.
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.
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.
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.
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.
When the disease has advanced, more extreme measures may be needed to prolong life. If the kidneys are damaged, transplant or dialysis may be indicated. Gallstones may form, which require removal of the gallbladder. Hip replacement can be recommended for cases of avascular necrosis of the hip, and eye surgery may be required for optical disorders. When leg ulcers are present, wound care will be administered.
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.
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.
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.
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.
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.
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.
When the disease has advanced, more extreme measures may be needed to prolong life. If the kidneys are damaged, transplant or dialysis may be indicated. Gallstones may form, which require removal of the gallbladder. Hip replacement can be recommended for cases of avascular necrosis of the hip, and eye surgery may be required for optical disorders. When leg ulcers are present, wound care will be administered.
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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