• Sickle Cell Crisis/Acute Chest Syndrome

    What is sickle cell disease?

    Sickle cell disease is a genetic disease characterized by red cells in the blood that are abnormally shaped. These red cells can cause blockages in the small blood vessels. Some complications caused by this disease are chronic, but others are acute and some may even be life threatening. One of the most common acute complications is called a sickle cell crisis. One of the more severe and life-threatening acute complications is acute chest syndrome.

    What is a sickle cell crisis?

    A sickle cell crisis is an episode of pain that can occur without warning in a person who has sickle cell disease. It is believed that a crisis occurs when the cells change shape and block blood vessels, which leads to reduced oxygen availability to the tissues served by that vessel and inflammation.

    What might trigger a sickle cell crisis?

    Common triggers include cold weather, dehydration, stress or sickness.

    What is acute chest syndrome?

    Acute chest syndrome is a potentially life-threatening infection and/or a blockage of blood flow to the chest and lungs in patients with sickle cell disease. It can result in lung injury, infection, breathing difficulty, low oxygen, pain to the rest of the body and death. Acute chest syndrome has many causes.

    Emergency Steps

    1

    Check the scene safety, form an initial impression, obtain consent and put on PPE, as appropriate.


    2

    Check for signs and symptoms.

    • Trouble breathing*
    • Cough*
    • Fever*
    • Chest pain*
    • Sudden pain in arm, leg, hand, feet, stomach, chest or back*
    • Pain in other body parts*

    *Note: Signs and symptoms with a * require immediate emergency medical treatment.


    3

    Call 9-1-1 and get equipment if the person requires immediate emergency medical treatment.


    4

    Give Care.


    General Care: Sickle Cell Crisis/Acute Chest Syndrome

    1. Allow the person to rest in a position of comfort.
    2. Apply a heating pad to painful areas.
    3. Encourage them to drink fluids.
    4. Assist with prescribed medication, if needed.
    5. Continue checking them as appropriate to determine if additional care is needed.
    6. Keep them from getting cold or overheated.
    7. Give care for shock, if necessary.
    8. Reassure them you will help and that EMS has been called (if appropriate).
    9. Watch for changes in condition, including breathing and responsiveness, and give care as appropriate and trained.

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    Information Provided the Scientific Advisory Council (SAC)

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