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.
When both parents of a child carry the hemoglobin-S gene, whether they are directly afflicted with the illness or simply carry the trait, their children will inevitably inherit it. People whose background is African or Mediterranean are most affected by this disease, but it is also seen in those with family origins in the Caribbean, Middle East, Central and South America. It is recommended that those with this trait seek genetic counseling before having 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.
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.
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.
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.
When both parents of a child carry the hemoglobin-S gene, whether they are directly afflicted with the illness or simply carry the trait, their children will inevitably inherit it. People whose background is African or Mediterranean are most affected by this disease, but it is also seen in those with family origins in the Caribbean, Middle East, Central and South America. It is recommended that those with this trait seek genetic counseling before having 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.
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.
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.
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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