Sickle cell anemia is an inherited blood disorder caused by the production of abnormal hemoglobin, the protein that carries oxygen in the red blood cells. Normally, red blood cells are round, easily circulating inside blood vessels. The abnormal hemoglobin, however, causes those cells to become deformed and take on the shape of sickles or crescent moons. The cells then stick to each other, blocking blood flow — especially in small blood vessels.
Because sickle cell anemia is an inherited disease, it is present at birth. However, most newborns show no signs of the disease for at least four months.
Generally, symptoms of the disease — which range from mild to those requiring hospitalization — include anemia, pain, swelling of extremities, recurrent infections, slow growth and vision issues.
Anemia, which is a condition where there are lower numbers of red blood cells in the blood, is the most common symptom of sickle cell anemia. This occurs as sickle-shaped cells tend to break apart easily and die too quickly before the body can replace them. This leads to a shortage of red blood cells, causing insufficient oxygen to be delivered throughout the body leading to fatigue.
Patients with sickle cell anemia tend to develop periodic episodes of pain, termed “crises”. These occur when sickle-shaped red blood cells get stuck inside blood vessels and block the blood flow to the chest, abdomen, joints, and bones. These periods of pain can last anywhere from a few hours and a few weeks. If the pain is severe enough, patients may signal the need to be hospitalized. The number of occurrences of these crises varies between individuals depending on the severity of the disease.
Some patients, including adolescents and adults, may experience chronic pain due to damage that has occurred over time to the bones and joints.
Swelling of the extremities
Sickle cell anemia patients may experience swelling in their hands and feet due to the blockage of blood flow to the extremities.
Sickle-shaped red blood cells can sometimes damage the spleen, an organ that helps fight infection. That can make patients more sensitive to mild as well as life-threatening infections such as pneumonia.
Reduced oxygen flow may delay growth in children, as well as delay puberty in adolescents.
Sickle-shaped red blood cells can get stuck in the vessels that supply blood and oxygen to the eyes. This can damage the retina and cause vision problems.
Other symptoms and complications
Other symptoms and complications of sickle cell anemia may include:
- Abdominal swelling
- Pale skin or nail beds
- Jaundice (yellow tint in the skin or whites of eyes)
- Cold hands and feet
- Shortness of breath or dizziness
- Chest pain or a serious lung condition called acute chest syndrome
- Leg ulcers (painful, open sores) that do not heal
- Pulmonary hypertension
- Kidney or urinary problems
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