Signs & Symptoms of Sickle Cell Anemia
According to the Centers for Disease Control and Prevention (CDC), signs of sickle cell anemia usually develop during the first year of life - often after 4 months of age. Sickle cell anemia symptoms vary in severity, and from person. They may be related to anemia (low red blood cell count), pain, and/or complications of the disorder.
Sickle cell anemia symptoms caused by low red blood cell count include the following:
- Fatigue and weakness
- Pallor (paleness)
- Cold extremities (hands and feet), low body temperature
- Jaundice (yellowing of the skin or the whites of the eyes
Sickle Cell Anemia & Pain
Pain - sudden (acute) or long-lasting (chronic), ranging from mild to severe - is a common symptom of sickle cell anemia. Sickle cell anemia can cause pain in the bones and joints, lungs, abdomen, and other parts of the body.
Most people with sickle cell anemia develop a condition called sickle cell crisis at some point in their lives. This sudden pain throughout the body occurs when sickle cells block the flow of blood to the organs and limbs. Some people with SCD have crises infrequently (e.g., less than one a year) and others have them more often - once or more a month.
The exact cause for sickle cell crises is unknown, but several factors - including dehydration and infection - increase the risk. People with sickle cell anemia should drink plenty of fluids and follow their health care provider’s recommendations for reducing their infection risk.
Sickle Cell Anemia Complications
SCD can lead to bone and organ damage and other serious complications. Sickle cell crises, which occur more frequently in adults, increase the risk. Complications of sickle cell anemia include the following:
- Acute chest syndrome - Life-threatening condition similar to pneumonia; causes chest pain, shortness of breath, fever, and low blood oxygen levels
- Delayed growth/puberty - Causes smaller stature
- Eye/vision problems - Damaged blood vessels in the retinas can lead to vision loss
- Gallstones - Increased levels of hemoglobin and bilirubin can lead to stones in the gallbladder causing nausea, vomiting, fever, and other symptoms
- Hand-foot syndrome - Pain and swelling of the hands and feet and fever, usually in children under the age of 4
- Infections - Damage to the spleen can interfere with the body’s ability to fight infection; this complication is the leading cause of death in young children with SCD. Seek immediate medical care for fever, cough, difficulty breathing, bone pain, and headache.
- Leg ulcers - Sores on the lower legs; more common in males over the age of 10
- Multiple organ failure - Rare, serious complication that occurs when two major organs (lungs, liver, kidneys) fail, usually during sickle cell crisis; causes fever, rapid heart rate, difficulty breathing, and neurological symptoms like confusion
- Priapism - Blood vessel damage can cause painful erections in men with SCD
- Pulmonary hypertension - Caused by blood vessel damage in the lungs; symptoms include shortness of breath and fatigue
- Splenic crisis - Enlarged spleen caused by trapped red blood cells; may require blood transfusion
- Stroke - SCD increases the risk for ischemic stroke and hemorrhagic stroke
Sickle Cell Anemia Diagnosis
Sickle cell anemia is diagnosed with a simple blood test. Early diagnosis of SCD is very important. In the United States, a sickle cell anemia test is required as part of all newborn screening programs. If this test detects sickle hemoglobin in the blood, a second blood test is performed as soon as possible.
SCD also can be diagnosed before birth - as early as 10 weeks into the pregnancy. In this test, a sample of amniotic fluid or from the placenta is used to detect the gene for sickle hemoglobin.
Sources: National Heart, Lung, and Blood Institute of the National Institutes of Health (NIH); U.S. Centers for Disease Control and Prevention (CDC)