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.
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.
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.
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.
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.
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.
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.
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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