People with sickle cell disease (SCD) have abnormally shaped red blood cells. This can cause symptoms of blocked blood flow and anemia. Symptoms vary for each person and can range from mild to severe.
Early signs and symptoms include swelling of the hands and feet, fatigue, and jaundice. Over time, people with SCD can develop many different complications. These complications are associated with specific symptoms. The most common symptom of SCD is pain, which can be acute or chronic.
What causes sickle cell disease symptoms?
Most of the signs and symptoms of SCD are related to complications of the disease. Complications usually occur because of blocked blood flow (vaso-occlusion) and low red blood cell count (anemia).
People with SCD have an abnormal form of hemoglobin in their red blood cells. Hemoglobin is a protein that carries oxygen from the lungs to the rest of the body. Abnormal hemoglobin, called “sickle hemoglobin,” has a mutation that causes the protein to form stiff bundles inside red blood cells.1
This causes red blood cells to become rigid and sickle-shaped. Sickle cells stick to walls of blood vessels and block blood flow. This can cause oxygen deprivation and acute pain crises in many organs.2
Sickle cells also tend to burst prematurely. Our bodies make new red blood cells but cannot keep up with the quick destruction of sickle cells. This means that people with SCD often experience symptoms of anemia. This includes:2,3
- Delayed growth
- Difficulty breathing
- Fatigue
- Irritability
- Jaundice
Early symptoms
People with SCD usually start showing symptoms around 5 or 6 months old. Some children will start to have problems earlier than others. The signs and symptoms will vary for each person and change over time.4
Early symptoms of SCD include:4
- Fatigue
- Swelling of hands and feet (dactylitis)
- Yellow skin and eyes (jaundice)
Jaundice and fatigue are caused by anemia. Dactylitis is caused by blocked blood flow in and out of the hands and feet.3
Pain
Pain is the most common symptom of SCD and the leading cause of hospital visits for people with SCD. It happens when sickle cells get stuck in blood vessels and prevent oxygen transport. Pain usually starts suddenly and can last for any length of time.3
People describe acute pain as sharp, intense, or stabbing. Acute pain can happen anywhere in the body, but it is most common in the:4
- Arms
- Back
- Chest
- Legs
- Stomach
Pain crises may be triggered by high altitudes, dehydration, or temperature changes. However, people often do not know what triggers the crisis.4
Many children and adults with SCD also experience chronic pain. This pain is usually different from acute pain crises.4
Other symptoms of complications
People with SCD experience other symptoms of complications they can develop. For example, people with SCD can develop a life-threatening condition called acute chest syndrome. This happens when a lack of oxygen damages the lung. Symptoms of acute chest syndrome are similar to pneumonia, such as:3
- Chest pain
- Coughing
- Difficulty breathing
- Fever
People with SCD also have a higher risk for stroke because of blocked blood flow in the brain. Stroke may occur with acute chest syndrome or by itself. Signs and symptoms of stroke include:4
- Confusion or trouble speaking
- Difficulty seeing
- Dizziness
- Numbness or weakness, especially on 1 side of the body
- Severe headache
People with SCD can also experience a severe complication of anemia called splenic sequestration. This happens when red blood cells get trapped in the spleen, making it grow larger than normal. Symptoms of splenic sequestration include:3
- Belly pain on the left side of the body
- Difficulty taking deep breaths
- Fast heartbeat
- Feeling full after eating very little
- Weakness
These are not all of the complications of SCD. People with SCD also have a higher risk for:4
- Gallstones
- Infections
- Problems with the heart, kidney, joints, and liver
- Vision loss
Each complication is associated with certain symptoms.4
Prevention and treatment of symptoms
Treatment focuses on managing symptoms and reducing the severity of complications. Some simple steps can help reduce the number of pain crises, including:3
- Avoiding extreme temperatures or high altitudes
- Drinking plenty of water
- Taking hydroxyurea or other FDA-approved medicines
Children are usually given additional vaccinations and antibiotics to prevent infections. Many people with SCD are also screened often for vision loss, lung function, and brain injury.1
Pain is usually treated with over-the-counter drugs. Severe pain may be treated with stronger pain drugs in a hospital. Anemia, acute chest syndrome, and stroke may be treated with blood transfusions. Swollen hands and feet are usually treated with pain medicine and increased fluids.1,3