Sickle cell anemia is a hereditary blood disorder. It results in an abnormality of the hemoglobin, an oxygen-carrying protein that can be found in red blood cells. This causes the red blood cells to become a rigid and sickle-like shape in some circumstances. Symptoms of sickle cell anemia usually start around 5 to 6 months of age. In the developed world, life expectancy averages 40 to 60 years. Sickle cell anemia is an autosomal recessive condition which means it occurs when the individual inherits one abnormal copy of the hemoglobin gene from each parent (resulting in a total of two copies of abnormal genes). This gene can be found on chromosome 11.
An episode of sickle cell attack known as sickle cell crisis can be triggered by stress, dehydration, temperature changes, and high altitude. An individual with only one abnormal copy of the gene does not usually have symptoms and is said to have the sickle cell trait, otherwise known as carriers. The diagnosis can be made through a blood test. Some countries have made it a routine test for all babies at birth for the disease.
Management of the disorder include the prevention of infection through vaccination, and the use of pain medication, folic acid supplements, high fluid intake, and antibiotics. Blood transfusion, hydroxycarbamide, and bone marrow transplant are also options for patients. As of 2015, it has been estimated that 4.4 million individuals have sickle cell disease with an additional 43 million carriers. That year also resulted in 114,800 deaths. Approximately 80 percent of cases occur in sub-Saharan Africa. It is also frequently found in regions of the Arabian Peninsula, parts of India, and in people of African ethnicity in other parts of the world.
Symptom #1: Pain
In sickle cell crises, periodic episodes of pain can occur. The pain occurs when the sickle-shaped red blood cells cause a blockage in the flow of blood through the tiny blood vessels to the joints, chest, abdomen, and more. Pain can also be felt in the bones. Varying in intensity, pain that occurs during sickle cell crisis can last from a few hours to a few weeks.
While some patients experience only a few pain episodes per year, others can suffer a lot more. A more severe case of sickle cell crisis may require hospital admission. Patients with sickle cell disease also experience chronic pain due to ulcers or damage to the bone and joints.