Symptoms and Complications of Sickle Cell Disease
Reviewed by: HU Medical Review Board | Last reviewed: January 2021 | Last updated: December 2020
Sickle cell disease (SCD) is a lifelong illness caused by an abnormal protein in red blood cells. Red blood cells become rigid and sickle-shaped. Sickle cells can block blood flow and prevent oxygen from reaching many parts of the body. They can also burst prematurely, causing anemia (low red blood cell count).
These processes can cause many different symptoms and complications. Symptoms of SCD vary for each person. Symptoms can range from mild to severe, and they tend to worsen over time.
What is the difference between a symptom and a complication?
A symptom is a problem that someone experiences, which may be a sign of a certain disease. Symptoms can be acute or chronic. Symptoms can be similar for many different conditions, but doctors often use them when making decisions about diagnosis and treatment. For example, some symptoms of the common cold are a runny nose and sneezing, but these could also be symptoms of allergies.
A complication is an unfavorable result of a disease. Complications usually happen when a disease worsens. Complications can cause someone to experience certain symptoms. For example, a possible complication of a cold is developing bronchitis. Symptoms of bronchitis include a persistent dry cough, fatigue, and fever.
People with SCD experience many different symptoms and complications. A person is diagnosed with SCD at birth, before they show symptoms. Then, most of the symptoms that people with SCD experience are related to complications.1
What are common symptoms?
Most people with SCD begin to show symptoms when they are about 5 or 6 months old. Symptoms will vary from person to person and over time. Some symptoms occur when sickle cells block blood flow to a part of the body. Other symptoms occur when sickle cells burst prematurely, causing anemia.2
Early symptoms of SCD include:2
- Bossing of the skull (enlarged bones in the skull, especially the forehead)
- Fatigue and tiredness
- Swelling of hands and feet (dactylitis)
- Yellow skin or eyes (jaundice)
The most common symptom for people with SCD is pain. People with SCD can experience acute or chronic pain. Acute pain can occur anywhere in the body, but it commonly occurs in the stomach, chest, back, arms, and legs. On the other hand, chronic pain can be hard to describe but feels different than acute pain. Pain can be a symptom of many different complications of SCD.1
What are common complications?
People with SCD develop complications because of blocked blood flow and oxygen delivery. They also experience complications due to anemia. These complications can be acute or chronic, and they can range from mild to severe.3
Some of the most common complications of SCD include:2,3
- Acute chest syndrome (pneumonia-like condition caused by lung damage)
- Acute pain crisis (also called sickle cell or vaso-occlusive crisis)
- Delayed growth and puberty
- Eye problems
- Heart problems
- Joint complications
- Kidney problems
- Leg ulcers
- Liver complications
- Priapism (prolonged painful erection)
- Sleep disorders
- Splenic sequestration (enlarged spleen due to trapped red blood cells)
- Stroke and other brain complications
Each complication may cause someone with SCD to show certain symptoms. For example, someone who develops splenic sequestration may experience pain in the left side of the belly. Someone who develops gallstones may experience nausea and pain in the upper right side of the belly.1
Pregnancy and sickle cell disease
Pregnancy may increase the risk of experiencing complications of SCD, such as stroke, acute chest syndrome, and pain crises. SCD may also increase the risk of problems during pregnancy, such as birth defects. Women with SCD who want to have a baby should talk to their doctor about how to prepare for pregnancy and how to stay healthy with routine care.
Treatment and prevention
Treatment and prevention depend on what complications someone experiences. For example, children with SCD usually receive additional vaccinations and antibiotics to prevent infections. For most people, treatment and prevention includes some combination of medicines, such as hydroxyurea, and blood transfusions.1