Because sickle cell disease or SCD — a group of blood disorders characterized by sickle-shaped cells — is inherited, it is present at birth. However, most newborns show no signs of the disease in the first four or five months of life. Over time, symptoms of the disease will develop, but these can differ from person to person, and can range from mild to severe manifestations that require hospitalization.
Among the symptoms are anemia, pain, swelling of the extremities, recurrent infections, slow growth, and vision problems.
Sickle cell disease is caused by the production of abnormal hemoglobin, the protein that carries oxygen in red blood cells. Normally, red blood cells are round, easily circulating inside blood vessels. The presence of abnormal hemoglobin, however, causes these cells to become deformed and take on a sickle-like shape.
This sickle shape makes red blood cells more prone to stick to each other and block blood flow, especially in small blood vessels, causing the disease’s symptoms and sometimes leading to pain crises. The following are the symptoms that occur most often.
Anemia, a condition caused by a shortage of red blood cells in the blood, is the most common symptom of sickle cell disease. This occurs because sickle-shaped red blood cells tend to break apart easily and die too quickly before the body can replace them. This lowers the number of red blood cells, causing insufficient oxygen to be delivered throughout the body, and also causing increased fatigue.
Patients with sickle cell disease tend to develop periodic episodes of pain, known as vaso-occlusive crises (VOCs). These occur when sickle-shaped red blood cells get stuck inside blood vessels and block blood flow to different parts of the body, including the chest, lower back, arms, and legs. These periods of pain can last anywhere from a few hours to a few weeks. If severe, these pain crises may require patients to be hospitalized. The frequency of these crises varies between individuals and depends on disease severity.
Some patients, including adolescents and adults, may experience chronic pain due to damage that has occurred over time to the bones and joints.
Patients with sickle cell disease may experience swelling in their hands and feet due to the blockage of blood flow to the extremities. This is often experienced along with a fever.
Swelling of the abdomen and the spleen, an organ that helps fight off infections, also may be observed, which may cause patients to have shortness of breath.
Sickle-shaped red blood cells can sometimes damage the spleen, lowering its ability to fight off infections. This can make patients more sensitive to mild infections as well as to life-threatening ones, such as pneumonia and meningitis. These problems are more likely to occur in younger patients.
Low red blood cell numbers cause tissues to become deprived of oxygen and nutrients. This may delay growth and development in children, as well as delay puberty in adolescents. Adults with sickle cell disease are usually shorter and thinner than the general population for the same reason.
Sickle-shaped red blood cells can obstruct vessels that supply blood and oxygen to the eyes. This can damage the retina — the region at the back of the eye that enables one to see — and cause vision problems. These include floaters, blurred vision, reduced vision at night, and possibly vision loss.
Bleeding in the eye also may occur, which may be followed by a buildup of pressure inside the eye, a condition called glaucoma.
Other symptoms and complications
Other symptoms and complications of sickle cell disease may include headaches, dizziness, jaundice (yellow tint in the skin or whites of eyes), gallstones, liver and kidney problems. Patients with sickle cell disease are also at a higher risk of having strokes, developing leg ulcers (painful, open sores) that do not heal, and acute chest syndrome — a serious lung condition that causes fever, chest pain, and breathing difficulties.
Last updated: Aug. 26, 2021
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