Signs and symptoms of sickle cell anemia often don't appear until an infant is at least 4 months old and may include:
- Anemia. Sickle cells are fragile. They break apart easily and die, leaving you without a good supply of red blood cells. Red blood cells usually live for about 120 days before they die and need to be replaced. But sickle cells die after an average of less than 20 days. This results in a lasting shortage of red blood cells (anemia). Without enough red blood cells in circulation, your body can't get the oxygen it needs to feel energized. That's why anemia causes fatigue.
- Episodes of pain. Periodic episodes of pain, called crises, are a major symptom of sickle cell anemia. Pain develops when sickle-shaped red blood cells block blood flow through tiny blood vessels to your chest, abdomen and joints. Pain can also occur in your bones. The pain may vary in intensity and can last for a few hours to a few weeks. Some people experience only a few episodes of pain. Others experience a dozen or more crises a year. If a crisis is severe enough, you may need to be hospitalized.
- Hand-foot syndrome. Swollen hands and feet may be the first signs of sickle cell anemia in babies. The swelling is caused by sickle-shaped red blood cells blocking blood flow out of their hands and feet.
- Frequent infections. Sickle cells can damage your spleen, an organ that fights infection. This may make you more vulnerable to infections. Doctors commonly give infants and children with sickle cell anemia vaccinations and antibiotics to prevent potentially life-threatening infections, such as pneumonia.
- Delayed growth. Red blood cells provide your body with the oxygen and nutrients you need for growth. A shortage of healthy red blood cells can slow growth in infants and children and delay puberty in teenagers.
- Vision problems. Some people with sickle cell anemia experience vision problems. Tiny blood vessels that supply your eyes may become plugged with sickle cells. This can damage the retina — the portion of the eye that processes visual images.
When to see a doctor
Although sickle cell anemia is usually diagnosed in infancy, if you or your child develops any of the following problems, see your doctor right away or seek emergency medical care:
June 11, 2014
- Unexplained episodes of severe pain, such as pain in the abdomen, chest, bones or joints.
- Swelling in the hands or feet.
- Abdominal swelling, especially if the area is tender to touch.
- Fever. People with sickle cell anemia have an increased risk of infection, and fever can be the first sign of an infection.
- Pale skin or nail beds.
- Yellow tint to the skin or whites of the eyes.
- Any signs or symptoms of stroke. If you notice any one-sided paralysis or weakness in the face, arms or legs, confusion, trouble walking or talking, sudden vision problems or unexplained numbness, or a headache, call 911 or your local emergency number right away.
- McCavit TL. Sickle cell disease. Pediatrics in Review 2012;33;195.
- Sickle cell anemia. National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/sca/. Accessed Nov. 6, 2013.
- Vichinsky EP. Overview of the clinical manifestations of sickle cell disease. http://www.uptodate.com/home. Accessed Nov. 6, 2013.
- Vichinsky EP, et al. Diagnosis of sickle cell disorders. http://www.uptodate.com/home. Accessed Nov. 6, 2013.
- Steinberg MH. In the clinic. Sickle cell disease. Annals of Internal Medicine. 2011;155:ITC3-1.
- Olowoyeye A, et al. Gene therapy for sickle cell disease. Cochrane Database Systematic Reviews. http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD007652.pub3/abstract. Accessed Nov. 6, 2013.
- Dong A, et al. Gene therapy for hemoglobinopathies: Progress and challenges. Translational Research. 2013;161:293.
- Sun K, et al. New insights into sickle cell disease: A disease of hypoxia. Current Opinion in Hematology. 2013;20:215.
- Gladwin MT, et al. Nitric oxide for inhalation in the acute treatment of sickle cell pain crisis: A randomized controlled trial. JAMA. 2011;305:893.
- Adam SS, et al. Potential role for statins in sickle cell disease. Pediatric Blood Cancer. 2013;60:550.