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by EBSCO Medical Review Board
(Sickle Cell Pain Crisis)

Definition

Sickle cell crisis is a bout of pain in people who have sickle cell disease . It can vary from mild to severe. The pain can happen anywhere in the body. It may last a few hours or few days.

Causes

Sickle cell disease changes the shape of red blood cells. The odd-shaped cells can get stuck in blood vessels. This blocks the flow of blood and oxygen to nearby tissue, which causes pain.

Risk Factors

A sickle cell crisis can be triggered by:

  • Not having enough fluid— dehydration
  • Smoking cigarettes or being around others who smoke
  • Stress or not getting enough rest
  • High altitudes—includes flying or hiking in mountains
  • Infection or illness
  • Alcohol—can increase loss of fluids from the body
  • Menstrual period—often at start of a period
  • Very hot or cold temps

Symptoms

Pain is the main symptom. It is most common in the arms, legs, back, or chest in adults, but it can be anywhere. Children may also have swelling in the hands and feet. Pain can be:

  • Mild to severe
  • Sharp or stabbing
  • Throbbing

Other symptoms depend on where a blockage has happened. They may include:

  • Fever
  • Headache
  • Feeling tired
  • Breathing problems
  • Joint pain
  • Belly pain or swelling
  • Painful erection of the penis that does not go away

Diagnosis

The doctor will ask about your health history and pain. A physical exam may be done to look for other causes of pain. The doctor may suspect a crisis based on your symptoms and history.

Blood tests or a chest x-ray can rule out an infection.

Treatment

The goal of care is to manage pain.

Mild Pain

Home care can help ease mild pain. This may include:

  • Over the counter medicine
  • Increase fluids
  • Heat therapy—a warm compress or heating pad
  • Massage

Severe Pain

Severe pain may be treated in a hospital with:

  • Pain medicine
  • IV fluids
  • Oxygen therapy—increases levels of oxygen in the blood

Long-term Pain

Coping with long-term pain can be stressful. Steps that may help to ease pain or intensity of pain include:

  • Cognitive behavioral therapy —to change thought patterns and improve coping skills
  • Alternative therapies such as:
    • Biofeedback
    • Massage therapy
    • Acupuncture
    • Hypnosis
    • Relaxation techniques

Prevention

Pain is often linked to certain triggers. To help prevent pain in the future:

  • Drink plenty of water throughout the day. Avoid drinks that can make you lose water such as alcohol.
  • Find ways to reduce stress.
  • Get enough rest.
  • Do not get too hot or too cold. Dress well for the weather.
  • Quit smoking. Talk to you care team about tools to help you quit.
  • Do not use drugs or drink alcohol.
  • Take steps to avoid infection. Wash your hands often. Talk to your doctor about which vaccines are helpful to you.

A care plan for sickle cell anemia may help prevent a pain crisis.

RESOURCES

American Society of Hematology  https://www.hematology.org 

Sickle Cell Disease Association of America  https://www.sicklecelldisease.org 

CANADIAN RESOURCES

Health Canada  https://www.canada.ca 

Sickle Cell Awareness Group of Ontario  http://www.sicklecellanemia.ca 

References

Adam MP, Ardinger HH, et al. Sickle cell disease. GeneReviews. Available at: https://www.ncbi.nlm.nih.gov/books/NBK1377. Seattle, Washington. University of Washington, Seattle; 1993-2019.

Buchanan GR, Yawn BP, et al. Evidence-based management of sickle cell disease. National Heart, Lung, and Blood Institute website. Available at: https://www.nhlbi.nih.gov/sites/default/files/media/docs/sickle-cell-disease-report%20020816%5F0.pdf. Accessed June 19, 2020.

Complications and treatments of sickle cell disease. Centers for Disease Control and Prevention website. Available at: https://www.cdc.gov/ncbddd/sicklecell/treatments.html#Pain. Accessed June 19, 2020.

Management of acute events of sickle cell disease. EBSCO DynaMed website. Available at:  https://www.dynamed.com/management/management-of-acute-events-of-sickle-cell-disease  . Accessed June 19, 2020.

Revision Information

  • Reviewer: EBSCO Medical Review Board James P. Cornell, MD
  • Review Date: 08/2020
  • Update Date: 10/07/2020