What Are the Potential Complications of Sickle Cell Anemia?
This inherited blood disorder can lead to many health problems, some of which can result in hospitalization or death. Learn more about the symptoms to watch out for.
Sickle cell anemia is a genetic disorder that impacts how red blood cells carry oxygen throughout the body. In the United States, more than 90 percent of people who have it are Black or African American.
The most severe form of sickle cell disease, sickle cell anemia causes red blood cells to be sickle- or crescent-shaped, rather than a disc. That distortion causes the cells to get stuck in the blood vessels or break down and die more quickly, which can cause severe anemia (very low red blood cell levels).
Sickle cell anemia can cause serious complications, most of which are medical emergencies that require immediate treatment. Learn more about them, so you can act promptly and receive the treatment you need.
Gallstones may develop in those with sickle cell anemia. They are hardened pieces of bile that can cause pressure, pain, and nausea if they block the exit from the gallbladder.
The breakdown of red blood cells produces a substance called bilirubin, and a high level of bilirubin in the body can lead to gallstones. People with sickle cell anemia often have a high bilirubin level because sickled red blood cells break down every 10 to 20 days — as opposed to 120 days for healthy red blood cells.
Symptoms of gallstones can include the following:
Pain in the right upper or middle upper abdomen that lasts at least 30 minutes
Sickle cells can plug up the tiny blood vessels that supply the eyes and damage the retinas, which are the light-sensitive layer of tissue at the back of the inner eye. This can lead to vision problems like blurred vision, floaters, flashes of light, blind spots, and even blindness.
People who have this blockage in blood flow may not notice any symptoms at first, but vision problems may come on suddenly and can become permanent.
People with sickle cell anemia have a higher risk of infections because the sickle gene itself impairs a person’s immunity, making them more susceptible to infection. Plus, the spleen, which normally helps fight infection, can get damaged by the sickle-shaped blood cells.
Infections primarily tend to affect infants and children with sickle cell anemia. Any infection can be life-threatening for a person with sickle cell anemia, so seek immediate medical attention for a child who might have one.
The symptoms of each type of infection vary significantly. But overall, symptoms of an infection can include the following:
Leg ulcers, which are wounds that are slow to heal or won’t heal at all, are a fairly common complication of sickle cell anemia, particularly in male patients and people age 10 to 50.
Leg ulcers tend to occur in people with sickle cell anemia as a result of poor blood circulation, trauma, infection, and inflammation. Typical symptoms of leg ulcers include the following:
Open, painful sores
Pain in and around the affected area
Pus or discharge
Swelling
An itchy or burning sensation
Priapism is a prolonged erection that lasts longer than four hours without sexual arousal or stimulation, and it can happen if the sickle cells block the blood vessels in the penis.
Priapism can be classified as major when episodes last four hours or more. It can also be called stuttering, when episodes last between a few minutes and three hours. Major priapism can cause permanent tissue damage in the penis and result in erectile dysfunction.
Pulmonary hypertension is high blood pressure in the arteries that carry blood to the lungs. It occurs in about 30 percent of adults with sickle cell anemia, potentially as a result of inflammation and reduced levels of nitric oxide, a substance that helps blood vessels relax. This causes the small arteries in the lungs to narrow, which over time can lead to pulmonary hypertension.
Symptoms of pulmonary hypertension include the following:
Shortness of breath, even at rest
Chest pain or discomfort
Fatigue
Dizziness or fainting
Swelling in the ankles, legs, or abdomen
Splenic sequestration is an acute condition in which large amounts of blood build up in the spleen, causing that organ to become swollen and inflamed. In sickle cell anemia, splenic sequestration occurs because sickle cells get trapped in the spleen and block the flow of blood.
Splenic sequestration most commonly affects children and requires prompt medical attention. Typical symptoms include the following:
Pain on the left side of the belly
Sudden weakness
Pale lips
Fast breathing
Rapid heartbeat
Acute chest syndrome is a life-threatening complication that occurs because the sickled cells block the blood and oxygen flow to the lungs. It can also be the result of an infection with a virus or bacteria. People with sickle cell anemia are more prone to infections because they have a weakened immune system.
The symptoms of this syndrome are similar to those of pneumonia and include the following:
Fever
Rapid breathing
Chest pain
Coughing
Shortness of breath
People with sickle cell anemia are at a higher risk of health problems that impact the heart, lungs, kidneys, and other vital organs because they’re not receiving enough oxygen-filled blood. In severe cases, sickle cell anemia can lead to acute multiorgan failure, which is life-threatening.
Signs and symptoms of organ damage can vary depending on the organ that’s affected. Overall, they include the following:
Trouble breathing
Irregular heartbeat
Nausea
Swelling in the hands and feet
Jaundice
A stroke is a serious medical emergency during which the flow of blood to the brain is blocked or a blood vessel bursts. It can occur if sickle cells impede the blood flow to the brain.
The signs and symptoms of a stroke include the following:
Numbness or weakness on one side of the body
Confusion understanding speech or difficulty speaking
Vision problems
Loss of coordination
Difficulty walking
Dizziness
Severe headache
It's also possible to have a silent stroke that doesn't cause the usual symptoms but can have lasting effects on the brain. It is best detected with an MRI imaging test.
The Takeaway
Many complications may arise from sickle cell anemia, and many of them can lead to hospitalization or even death.
Complications can impact the entire body, including infections, vision problems and loss, gallstones, pulmonary hypertension, and stroke.
Pay attention to the warning signs of these complications and seek prompt medical attention if you experience them or your condition gets worse.
