Symptoms and Complications of Sickle Cell Disease

Symptoms of sickle cell disease (SCD), a chronic and complex blood disorder, can vary. In young children the first sign of the problem is often painful swelling of the hands and feet. Learn more.

Sickle cells occur more often in pepole of African descentIn the United States, all newborns are screened for sickle cell disease which is most prevalent in African-American births. Symptoms and Complications

Sickle cell anemia (SCA), the most common form of sickle cell disease (SCD), is a chronic, complex, and frequently unpredictable disease. The severity and frequency of symptoms vary from person to person. In a healthy person, red blood cells live about 120 days; but hemoglobin S (sickle cells) only live 10 to 20 days, which means the bone marrow (where red blood cells are produced) goes into overdrive trying to replace those cells This frenzied activity can lead to a sickle cell crisis.

In babies, symptoms usually begin around six months of age. Often the first sign of SCA in children is a pronounced and painful swelling of the hands and feet (also known as dactylitis). SCA can also cause delayed growth during childhood and delayed puberty. Other symptoms include:

  • Severe anemia: While people with SCA usually have—and live with—mild to moderate anemia, at times they can experience severe anemia that can be life-threatening.
  • Splenic sequestration crisis: Located in the upper left side of the belly, the spleen is an organ that filters germs in the blood, breaks up blood cells, and makes a kind of white blood cell. A splenic sequestration crisis occurs when red blood cells get stuck in the spleen, making it enlarge quickly. This type of crisis occurs most often in young children between 10 and 27 months of age.
  • Aplastic crisis: This is usually caused by infection of parvovirus B19. Also known as fifth disease or slapped cheek syndrome, parvovirus B19 is a very common infection, which in people with SCA can cause the bone marrow to stop producing new red cells for a while, leading to severe anemia.
  • Jaundice: Yellow tint to the skin or whites of the eyes.
  • Fever: Due to infection or another reason.
  • Stroke: SCD is the leading cause of stroke in children. Symptoms of stroke include paralysis or weakness on one side of the body especially in the face, arms and legs, confusion, trouble walking or talking (slurred speech), numbness and a severe headache. If any of these symptoms occur, call 911 immediately. 
  • Pain crisis: (vaso-occlusive crisis): These episodes of severe pain happen when the sticky, sickled cells get stuck in blood vessels blocking blood flow. While these occlusions can occur anywhere, the back, chest, arms, and legs are affected most often.   
  • Acute chest syndrome: This potentially life-threatening complication resembles pneumonia and can include fever, pain, and a violent cough. In babies and young children, the symptoms of acute chest syndrome may be less defined. The child may appear restless or lethargic and listless.
  • Pulmonary hypertension: High blood pressure in the vessels that carry blood from the heart to the lungs.
  • Vision changes and problems: Diminished night vision, blurred or patchy vision, and occasionally rapid vision loss.
  • Priapism: Prolonged and painful erection, which can last for hours.
  • Infections: People with SCA are prone to infections such as pneumonia, meningitis, septicemia, and salmonella.
  • Shortness of breath and dizziness, feeling tired.

Triggers

The likelihood of a sickle cell crisis increases with any activity that raises the body's oxygen requirements. For some people, rain and wind can trigger a crisis. Other common triggers include:

  • Physical or psychological stress
  • Exposure to rapid or extreme changes in temperature. People with SCA are cautioned to avoid overheating or getting too cold. For example, swimming in cold water and then sitting on a warm beach can trigger a crisis. Extreme sports are not advised.
  • Dehydration: Drinking plenty of water—eight 12-ounce glasses a day and more if needed—helps decrease the risk of a crisis.  
  • High altitudes where the air is thin and oxygen levels are low. That means avoiding small aircraft that don’t have pressurized cabins, heli-skiing, or mountain climbing. 

Updated on: 12/06/16
Continue Reading:
Treating Sickle Cell Disease
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