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Symptoms of sickle cell disease may be noticed as soon as four months of age, or it may go undetected until later in the baby’s first year of life.

Oxygen deprivation is a result of blood vessels that are blocked by the misshapen red blood cells of sickle cell anemia. Periods of acute oxygen deprivation cause severely painful episodes called pain crises. The location of the pain and the types of symptoms depend on what tissues or organs of the body have been deprived of oxygen.

Symptoms of sickle cell disease include:

  • Fever
  • Swollen hands and feet
  • Pain in:
    • Chest
    • Abdomen
    • Arms
    • Legs
    • Joints
    • Bones
  • Enlarged organs, including:
    • Heart
    • Liver
    • Spleen
  • Increased risk of infection, especially pneumonia
  • Symptoms of anemia, including:
    • Severe fatigue
    • Headache
    • Lightheadedness
    • Shortness of breath
    • Heart failure
  • Yellowish tone to the whites of the eyes and the skin
  • Episodes of sickle cell crisis, including:
    • Severe chest pain
    • Shortness of breath
    • Severe abdominal pain
    • Severe bone pain
    • Nausea
    • Fever
    • In males, painful, prolonged erections of the penis which may result in impotence

Other medical conditions that can result from sickle cell disease include:

  • Leg sores
  • Gum disease
  • Damage to the retina of the eye, resulting in vision loss
  • Enlargement of the heart due to chronic anemia
  • Heart attack
  • Heart failure
  • Kidney infections
  • Kidney damage and eventual failure
  • Bone infections or infarctions
  • Gallbladder disease
  • Spleen damage and destruction, resulting in an increased risk of certain infections
  • Stroke
  • Abnormal bone growth
  • Delayed puberty
  • Learning and behavior problems in children who have had severe, chronic oxygen deprivation of the brain
  • Aplastic crisis or red cell aplasia

Sickle cell crisis can be provoked by certain triggers, including:

  • Smoking
  • Exercise
  • Travel to high altitudes
  • Drops in oxygen or changes in air pressure that can occur during airplane travel
  • Fever
  • Infection
  • Dehydration

References:

Owusu-Ofori S, Riddington C. Splenectomy versus conservative management for acute sequestration crises in people with sickle cell disease. Cochrane Database Syst Rev. 2002;(4):CD003425.

Sickle cell disease. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated June 6, 2013. Accessed July 1, 2013.

Sickle cell disease. Nemours' KidsHealth.org website. Available at: http://kidshealth.org/parent/medical/heart/sickle_cell_anemia.html. Updated September 2012. Accessed July 1, 2013.

Sickle cell disease (SCD). Centers for Disease Control and Prevention website. Available at: http://www.cdc.gov/ncbddd/sicklecell/index.html. Updated September 27, 2012. Accessed July 1, 2013.

What is sickle cell anemia? National Heart, Lung, and Blood Institute website. Available at: http://www.nhlbi.nih.gov/health/health-topics/topics/sca/. Updated September 28, 2012. Accessed July 1, 2013.



Last reviewed May 2014 by Michael J. Fucci, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

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