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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.
If both parents have the disease or carry the hemoglobin-S gene, their children will also have it or be able to pass it on. Those with a Mediterranean or African heritage are most likely to have the condition, although it is also found amongst populations in the Middle East, Central and South America, and the Caribbean. These people are advised to attend genetic counseling if they are planning to have a family.
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.
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.
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.
Folic acid supplements can be helpful in the synthesis of new red blood cells. In some cases blood transfusions will be given as a preventative measure against strokes. Children with this disorder seem to be especially prone to infections, in which case antibiotics will be given to prevent complications.
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.
A bone marrow or stem cell transplant can usually eradicate the illness, however it is difficult to find a match for most patients. The risk of infection can also be lowered if patients are sure to receive the PCV, PPV, and Hib vaccinations.
If both parents have the disease or carry the hemoglobin-S gene, their children will also have it or be able to pass it on. Those with a Mediterranean or African heritage are most likely to have the condition, although it is also found amongst populations in the Middle East, Central and South America, and the Caribbean. These people are advised to attend genetic counseling if they are planning to have a family.
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.
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.
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.
Folic acid supplements can be helpful in the synthesis of new red blood cells. In some cases blood transfusions will be given as a preventative measure against strokes. Children with this disorder seem to be especially prone to infections, in which case antibiotics will be given to prevent complications.
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.
A bone marrow or stem cell transplant can usually eradicate the illness, however it is difficult to find a match for most patients. The risk of infection can also be lowered if patients are sure to receive the PCV, PPV, and Hib vaccinations.
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