Sickle cell anemia is an inherited blood disease in which red blood cells, which are normally round, become crescent or sickle-shaped under low oxygen conditions. In this condition, sickle cells are not only abnormally-shaped but also fragile and have a much shorter lifespan than normal red blood cells. These blood cells cannot be replaced quickly enough and a chronic shortage leads to anemia.
Many of the symptoms and complications arising from sickle cell disease are due to the blockage of blood vessels by the abnormal blood cells, preventing blood and oxygen from reaching vital organs. One of the main symptoms of sickle cell disease is painful episodes of severe pain caused by blockage of small blood vessels, often those in the bone. Hospitalization may be required. The disease is more common in those of African, Spanish, Middle Eastern, Mediterranean and Indian descent.
According to an article in the November 2008 issue of the New England Journal of Medicine, approximately 1 in 600 black Americans have the disease and 8 percent have the trait, meaning that they carry the gene but do not have the disease.
Organ Damage
Chronic blockage of small vessels feeding into organs such as the kidney and spleen can lead to organ damage and eventual failure. And repeated damage to the spleen eventually leads to fibrosis, a condition in which the spleen becomes small and non-functional. With the lack of functioning spleen, an organ instrumental in protecting against bacteria such as Salmonella and pneumococci, patients with sickle cell disease are at risk developing of life-threatening infections.
Stroke
A stroke can be caused by any condition that impedes blood from reaching the brain. While strokes usually occur in the older population by the build up of atherosclerosis in the blood vessels, in patients with sickle cell disease, blood flow to the brain is blocked by sickle cells and can occur at any age. Common signs and symptoms include numbness in the arm or leg, difficulty speaking and loss of consciousness.
Acute Chest Syndrome
Blockage of vessels in the lungs or a simple chest infection may lead to excessive inflammation and cause acute chest syndrome, a condition in which the patient develops fever, chest pain and difficulty breathing. Acute chest syndrome is one of the most common causes of death in sickle cell disease patients.
Pulmonary Hypertension
Approximately 30 percent of sickle cell patients develop some degree of pulmonary hypertension, a condition in which vessels in the lungs become narrowed from chronic oxygen deprivation. The right side of the heart has to work harder to pump blood against the higher pressure in the lungs and heart failure may ensue.
Considerations
Sickle cell disease can cause debilitating symptoms and lead to serious long-term complications. Effective management begins in childhood and includes immunization, regular antibiotics until six years of age to reduce the risk of infections, folate supplementation to help replenish blood cells, and medication to reduce painful crises.
References
- "New England Journal of Medicine"; Pulmonary Complications of Sickle Cell Disease; Mark T. Gladwin, MD, Elliott Vichinsky, MD; November 2008
- Mayo Clinic: Sickle Cell Anemia
- The Merck Manual for Healthcare Professionals: Sickle Cell Disease
