Sickle Cell Crisis

What is a sickle cell crisis?

A sickle cell crisis is a painful episode that occurs with people who have sickle cell anemia. It happens when your red blood cells (RBCs) sickle and block blood vessels. Blood and oxygen cannot get to your tissue, causing pain. A sickle cell crisis can also damage your tissue and cause organ failure, such liver or kidney failure. A sickle cell crisis can become life-threatening.

What are signs and symptoms of a sickle cell crisis?

Your symptoms may change each time you have a crisis. They will depend on the area of your body where blood flow has been blocked.

  • Fever

  • Pain

  • Weakness or fatigue

  • Abdominal pain and swelling

  • Headaches

  • A painful, erect penis (priapism)

  • Fast heart rate

  • Shortness of breath

What can trigger a sickle cell crisis?

  • Dehydration

  • Infection, such as a cold or the flu

  • Low oxygen levels from difficult exercise, flying, or high altitude

  • Getting cold or going from warm to cold quickly

  • Medical procedures, surgery, or having a baby

  • Strong emotions, such as anger or depression

How is pain managed during a sickle cell crisis?

  • Medicines decrease pain. Medicine may also be given to decrease sickling of your RBCs. You may also need medicine to prevent a bacterial infection or help you breathe more easily.

  • NSAIDs help decrease swelling and pain or fever. This medicine is available with or without a doctor's order. NSAIDs can cause stomach bleeding or kidney problems in certain people. If you take blood thinner medicine, always ask your healthcare provider if NSAIDs are safe for you. Always read the medicine label and follow directions.

  • Acetaminophen decreases pain and fever. It is available without a doctor's order. Ask how much to take and how often to take it. Follow directions. Acetaminophen can cause liver damage if not taken correctly.

How else is a sickle cell crisis treated?

  • IV fluids treat dehydration and help reduce sickling of RBCs.

  • Oxygen helps increase oxygen levels in your blood and make it easier for you to breathe.

  • A blood transfusion replaces blood with RBCs that are not sickle shaped.

  • Surgery may be done to remove a part of your spleen.

How can I prevent a sickle cell crisis?

  • Take vitamins and minerals as directed. Folic acid can help prevent blood vessel problems that can come with sickle cell anemia. Zinc may decrease how often you have pain.

  • Drink liquids as directed. Dehydration can increase your risk for a sick cell crisis. Ask how much liquid to drink each day and which liquids are best for you.

  • Balance rest and exercise. Rest during a sickle cell crisis. Over time, increase your activity to a moderate amount. Exercise regularly. Avoid exercise or activities that can cause injury, such as football. Ask about the best exercise plan for you.

  • Stay out of the cold. Do not go quickly from a warm place to a cold place. Do not go swimming in cold water. Stay warm in the winter.

  • Do not smoke or drink alcohol. These increase your risk for a sickle cell crisis. If you smoke, it is never too late to quit. Ask for information if you need help quitting.

  • Ask about what vaccinations you need. Vaccinations can help prevent a viral infection that may lead to a sickle cell crisis. You should get a flu shot every year. You may need a vaccine to protect you from the hepatitis B virus.

What are the risks of a sickle cell crisis?

A sickle cell crisis may cause severe pain. It may also cause jaundice (yellowing of your skin and eyes). A sickle cell crisis may harm organs, such as your kidneys or spleen. It may also lead to liver or kidney failure. A sickle cell crisis may cause trouble breathing or lung problems. A sickle cell crisis increases your risk for a heart attack or stroke. These conditions may become life-threatening.

When should I contact my healthcare provider?

  • You have any new signs or symptoms.

  • You have blood in your urine.

  • You are constipated or you have diarrhea.

  • You have changes in your vision.

  • You have increased fatigue.

  • You plan to travel by airplane or to a high elevation.

  • You have questions or concerns about your condition or care.

When should I seek immediate care or call 911?

  • You feel like you cannot cope with your pain, or you feel like hurting yourself.

  • You have a behavior changes, a seizure, or faint.

  • You have a fever.

  • You have abdominal pain, nausea, or vomiting.

  • You have an erection that is painful and does not go away.

  • You lose vision in one or both eyes.

  • You have a headache that is worse or different from those that you have had in the past.

  • You have new weakness or numbness in your arm, leg, or face.

  • You have new pain in any part of your body.

  • Your urine is dark in color, and you are urinating less than usual or not at all.

  • You have shortness of breath or chest pain.

  • You are dizzy, lightheaded, or faint.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

© 2014 Truven Health Analytics Inc. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or Truven Health Analytics.

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