When a person suffers from sickle cell disease, the hemoglobin is defective which causes malformation of the red blood cells. Instead of being a normal round shape, they are shaped like a sickle preventing them from being able to properly flow through smallest blood vessels. This problem can lead to anemia, organ damage, destruction of red cells, bone marrow disorders, and pain. This condition is genetic and cannot be cured, however sickle cell disease prevention is focused on preventing crises and symptoms from manifesting.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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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