Sickle Cell Crisis

Medically reviewed by Drugs.com. Last updated on Dec 2, 2024.

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What is a sickle cell crisis?

A sickle cell crisis is a painful episode that occurs in people who have sickle cell anemia. It happens when sickle-shaped red blood cells (RBCs) block blood vessels. Blood and oxygen cannot get to your tissues, causing pain. A sickle cell crisis can also damage your tissues 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) in men
• Fast heartbeats
• 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 may be given to decrease sickling of your RBCs. You may also need medicine to prevent a bacterial infection or help you breathe more easily.
• Prescription pain medicine may be given. Ask your healthcare provider how to take this medicine safely. Some prescription pain medicines contain acetaminophen. Do not take other medicines that contain acetaminophen without talking to your healthcare provider. Too much acetaminophen may cause liver damage. Prescription pain medicine may cause constipation. Ask your healthcare provider how to prevent or treat constipation.
• NSAIDs , such as ibuprofen, help decrease swelling, pain, and 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. Read the labels of all other medicines you are using to see if they also contain acetaminophen, or ask your doctor or pharmacist. 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 part of your spleen.

Treatment options

The following list of medications are related to or used in the treatment of this condition.

• hydroxyurea
• Droxia
• Siklos
• Endari
• Adakveo

View more treatment options

How can I prevent a sickle cell crisis?

• Take vitamins and minerals as directed. Folic acid may 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 as directed. Avoid exercise or activities that can cause injury, such as football. Ask about the best exercise plan for you.
• Wash your hands frequently. Handwashing can help prevent illness. Wash your hands before you prepare or eat food, and after you use the bathroom.
• Avoid quick changes in temperature. Do not go quickly from a warm place to a cold place. Get in a pool slowly instead of jumping in. Avoid getting too hot or too cold. Dress in light clothing in the summer and warm clothing in the winter.
• Do not smoke cigarettes or drink alcohol. These increase your risk for a sickle cell crisis. Nicotine and other chemicals in cigarettes and cigars can cause lung damage. Ask your healthcare provider for information if you currently smoke and need help to quit. E-cigarettes or smokeless tobacco still contain nicotine. Talk to your healthcare provider before you use these products.
• Ask about vaccines you may need. Vaccines can help prevent a viral infection that may lead to a sickle cell crisis. Get a flu shot as soon as recommended each year, usually in September or October. You may need pneumonia vaccines every 5 years. Your healthcare provider can tell you if you need other vaccines, and when to get them.

Call your local emergency number (911 in the US) if:

• You have shortness of breath or chest pain.
• You are a man and have an erection that is painful and does not go away.
• You lose vision in one or both eyes.

When should I seek immediate care?

• You feel like you cannot cope with your pain, or you feel like hurting yourself.
• You have behavior changes, a seizure, or faint.
• You have a fever.
• You have abdominal pain, nausea, or vomiting.
• You have a different or worse headache.
• 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 and you are urinating less than usual or not at all.
• You are dizzy, lightheaded, or faint.

When should I call my doctor?

• 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.

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 healthcare providers 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.

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