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What Are the Symptoms of Sickle Cell Disease?

Medically Reviewed by Sabrina Felson, MD on June 13, 2021

Sickle cell disease changes the shape of the oxygen-carrying molecules (called hemoglobin) found inside red blood cells. Red blood cells  are usually round and flexible. This allows them to glide smoothly through your bloodstream. But when you have sickle cell disease, the abnormal hemoglobin creates stiff, sickle-shaped red blood cells.

If you have this disease, these abnormally shaped red blood cells can cause a number of health problems. For example:

  • The cells get stuck in the smaller arteries and block blood flow to organs and tissues.
  • The cells die sooner. Sickle cells live about 10 to 20 days. Healthy red blood cells live from 90 to 120 days. Red blood cells may burst open (hemolyze). Even though your body constantly makes new red blood cells to replace the old ones, sickle cell disease may prevent your body from keeping up with the demand.

These events trigger the symptoms of sickle cell disease.

Early Symptoms

Usually, they first appear when a child is about five to six months old. Early signs in babies are:

  • Extreme fussiness
  • Painful swelling of fingers and toes which is the definition of a condition called "dactylitis
  • "Yellow skin and whites of the eyes (also called jaundice or icterus). This is caused by the breakdown of hemogloblin released during the constant turn over of red blood cells.

Anemia and Other Symptoms

With sickle cell disease, you don't have enough healthy red blood cells. This is a condition called anemia. Symptoms can include:

Sickle cells can cause problems anywhere in your body by blocking blood flow. If you block the blood vessels to the eyes, you lose vision, to the skin - you get ulcers, to the bones - you get necrosis (bone death) and eventually osteoporosis. Recurrent  infarctions of the spleen, which has very high blood flow and whose job it is to remove damaged red blood cells, results in a non-working spleen. This leads to an increased risk of infections. 

Pain Crises

Pain is an unpredictable symptom of sickle cell disease. The deformed blood cells can get stuck in and block your blood vessels. When this happens, oxygen-rich blood cannot reach key organs and tissues. The result is a sudden pain attack, called a pain crisis.

The pain may feel sharp, stabbing, intense, or throbbing. Some people with sickle cell disease say it’s worse than childbirth or the pain after surgery.

You may have pain anywhere in your body and in more than one place. The most common areas are:

  • Arms
  • Belly
  • Chest
  • Legs
  • Lower back

A pain crisis can last anywhere from hours to weeks. If it’s severe, you’ll need to seek emergency care at a hospital. The number of pain attacks varies from person to person. Some people have them often. Others have only a few every now and then. Most kids are pain-free between attacks. But many teens and adults have long-term pain.

Symptoms May Change Over Time

Everyone is different. Some types of the disorder, like sickle cell anemia (also called hemoglobin SS), cause more severe symptoms than other types. Some people have only mild symptoms. Others have repeated flares that and often need to go stay in a hospital.  And your experience may change. You’ll want to work with your (or your child’s) doctor to best understand the disease, what to watch out for, and how best to take care of the condition.

WebMD Medical Reference

Sources

SOURCES:

National Human Genome Research Institute: “Learning About Sickle Cell Disease.”

National Heart, Lung, and Blood Institute: “Sickle Cell Anemia,” “What Are the Signs and Symptoms of Sickle Cell Disease?”

Mayo Clinic: “Sickle Cell Anemia.”

American Society of Hematology: “Sickle Cell Anemia.”

CDC: “Complications and Treatments.”

UpToDate: "Bone and Joint Complications in Sickle Cell Disease."

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