Signs and Symptoms of Sickle Cell Disease
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Medically reviewed by Steffini Stalos, DO
Sickle cell disease (SCD) is a group of inherited red blood cell disorders that causes symptoms such as anemia, pain, fatigue, jaundice, an increased risk of infections, and swelling in your hands and feet. In some cases, this disease can worsen and cause more serious symptoms like high fever, problems with vision, and organ damage.
This condition occurs due to a mutation in your hemoglobin. Hemoglobin is a type of protein in your red blood cells that carries oxygen through your body. Healthy red blood cells with normal hemoglobin are shaped like discs and are flexible enough to move through your blood vessels. In people with sickle cell disease, your hemoglobin doesn’t function properly and causes your red blood cells to change into a crescent-like shape and block the normal flow of blood.
About 20 million people have SCD globally. People who inherit the sickle cell gene from both parents develop symptoms of the condition while those who inherit the gene from just one parent have the sickle cell trait and do not develop the condition. Knowing the signs of this condition can help you understand when to seek care from your healthcare provider and get started on treatment sooner.
Common Symptoms
You are most at risk of developing sickle cell disease if both of your parents carry the sickle cell trait. Most people with this condition are born with the disease. However, infants do not show symptoms until they are five or six months of age.
The severity of symptoms varies from person to person. Some people may have mild symptoms like body aches or swelling, while others experience more severe symptoms that may require hospitalization.
Some common symptoms of SCD include:
Anemia: Sickle cell disease can cause your red blood cells to break down prematurely, which can cause you to have fewer red blood cells than your body needs to function. As a result of anemia, you can also experience fatigue, weakness, and shortness of breath.
Pain: Sickle-shaped red blood cells become stiff, which can make it difficult for blood to flow normally through small blood vessels in the abdomen and bones. This can cause chronic (or, long-term) pain.
Jaundice: The rapid breakdown of a large number of red blood cells can lead to yellowing of the skin and eyes.
Infections: SCD can increase your risk of other infections such as Salmonella osteomyelitis (bone infection), meningitis (inflammation of the brain), and pneumococcal septicemia (blood poisoning). Without proper treatment, these infections can cause serious complications, such as death.
Edema: This condition may also cause painful swelling in your hands and feet.
Severe Symptoms
Sickle cell disease can lead to various severe symptoms or complications which may arise at any age. More serious symptoms may include:
High fever: Fever may be the first sign of sickle cell disease-related complications. A fever of 38.5 degrees Celsius or 101.3 degrees Fahrenheit or higher requires immediate attention from a healthcare provider.
Acute chest syndrome: This condition occurs when sickle cells block the small blood vessels in the lungs, leading to a lack of blood flow to the lungs. The symptoms of acute chest syndrome are similar to pneumonia and include chest pain, difficulty in breathing, and cough.
Spleen sequestration: The spleen is an organ that helps your body fight infections. SCD can cause your spleen to become enlarged, painful, and filled with blood. Spleen sequestration can affect anyone with sickle cell disease, but is most common in children.
Priapism: If the red blood cells in your penis become affected, you may also experience prolonged and painful erections.
Stroke: This disease can interrupt blood flow to your brain and increase your risk of stroke.
Avascular necrosis: A lack of blood supply due to sickle cell disease can lead to the death of your bone tissue, leading to pain in your joints and bones.
Vision problems: Sickle cell disease can affect the blood vessels in your eyes, which may lead to problems such as sickled red blood cells blocking blood vessels of the eye can cause blurred vision, floaters, and sometimes blindness.
Organ damage: Because SCD causes a lack of blood flow to your major organs, your body may experience heart, kidney, or lung failure in severe cases.
Symptoms in Children
Approximately 275,000 children are born with sickle cell disease each year. A few common symptoms of SCD in children are:
Slow developmental growth
Delayed puberty
Irritability
Crying persistently
Swollen hands and feet
Severe infections
Children can also experience more serious symptoms similar to adults such as spleen sequestration, acute chest syndrome, and stroke.
When to Contact a Healthcare Provider
If you or your child experience any symptoms of SCD or have the trait for the condition, it’s good practice to see your healthcare provider for proper testing. Experts recommend seeing a healthcare provider before eight weeks of age if your infant was born with SCD.
It’s important to seek care from your provider urgently if you or your child are experiencing any of the following symptoms:
Difficulty breathing
A fever above 101 degrees Fahrenheit
Abdominal swelling
Sudden weakness
Loss of movement
Body aches
A Quick Review
Sickle cell disease is a group of inherited red blood cell disorders that occurs due to a mutation in your hemoglobin—a protein that carries blood and oxygen through your body. The severity of your symptoms can vary, but may include anemia, fatigue, shortness of breath, jaundice, and swelling.
In some cases, SCD can cause more serious symptoms such as fever, blurry vision, and organ damage. Without proper treatment, you may also experience complications such as seizures and loss of movement, which require immediate attention.
Frequently Asked Questions
What is the life expectancy of a person with sickle cell disease?
The average life expectancy of a person with sickle cell disease is 52.6 years. People assigned male at birth have a life expectancy of 49.3 years, and those assigned female at birth have a life expectancy of 55 years.
Do symptoms of sickle cell disease get worse as you age?
Yes, symptoms of sickle cell disease may worsen with age. You may experience additional complications such as pulmonary hypertension (high blood pressure in the lungs), leg sores, gallstones, and kidney damage.
What organs does sickle cell disease damage?
Sickle cell disease can damage all major organs of the body including the heart, lungs, kidney, spleen, liver, and brain.
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