All you need to know about sickle cell anemia
At least 1 in every 5000 persons is affected by sickle cell anemia, mostly among the American-African decedents, as claimed by the National Institutes of Health. It is estimated that at least 90,000 people are affected with sickle cell anemia. With most infants with the disorder born, it is now identified by routine neonatal screening. As of 2016, all 50 states have included screening for sickle cell anemia as part of their newborn screening.
Sickle cell anemia can make life more difficult particularly among children who will need to deal with delayed sexual maturity and stunted growth. An infection would call for immediate medical attention when self-medications fail. Also, one would need to have a principal health care provider who should be preferably a hematologist for frequent appointments. To maintain a healthy quality of life, it is utmost necessary to keep the number of sickle cell crisis in check. People with the symptoms should avoid certain medications that restrict blood vessels, high altitudes, extreme temperatures, alcohol and smoking, and strenuous exercise. Ensure to have plenty of water or fluids and relax as much as possible.
Treatments or management of sickle cell crisis involves a number of measures such as Penicillin; due to the immature immune system in children from the birth to 5 years of age, they are more prone to this childhood illness. Daily penicillin is recommended and dietary supplementation of folic acid. Preventive vaccine from malaria infection is strongly recommended for people living in malaria-infected locations.
A vaso-occlusive crisis is when most people with sickle cell anemia have intensely painful episodes. The frequency, severity, and duration of this crisis, however, vary tremendously and are treated based on the symptoms. In an acute chest crisis, which is similar to a vaso-occlusive crisis with the addition of antibiotics, oxygen supplementation and close observation are required. Sometimes, admission in an ICU may also be required. A simple blood transfusion or exchange transfusion is indicated if the oxygen requirement increases.
Hydroxyurea has shown to decrease the numbers and severity of attacks and was the first approved medication for the treatment of sickle cell anemia. As per studies, it has shown to possibly increase survival time which is achieved by reactivating healthy hemoglobin production in the place of hemoglobin S that causes sickle cell anemia.
In acute cases, blood transfusion is often used in the management of sickle cell anemia to prevent complication by decreasing the number of red blood cells and adding normal red blood cells. This reduces the risk of first or silent stroke as reports show abnormal cerebral blood flow and also reduce the risk of recurring strokes if sustained a prior stroke or silent stroke.
A bone marrow transplant has proven to be effective and currently the only cure for sickle cell anemia. But only a few have been successful due to the rarity of matches. Sickle cell disease is lifelong and its severities differ from person to person. Early diagnosis and regular medical care can prevent complications.