Sickle Cell Crisis

WHAT YOU SHOULD KNOW:

  • A sickle cell crisis is a problem that can happen when you have sickle cell disease. It is also called an episodic painful crisis. When red blood cells (RBC's) form abnormal curved shapes called sickles, it is called sickle cell disease. These sickle-shaped red blood cells break down faster that normal cells. A red blood cell count that is too low causes anemia, making you feel tired and weak. RBC's can also get stuck in your blood vessels, shutting off blood flow and oxygen to areas of your body. This causes pain and damage to body organs, such as your kidneys. A stroke and other medical problems can be caused by blocked blood flow. When these problems happen, it is called a crisis.

  • A sickle cell crisis can be very painful. You may also have trouble breathing, and other signs and symptoms. A crisis may go away, and then come back many times. Treatment may include medicines, intravenous (IV) fluids, blood transfusions, and surgery. Treatment for sickle cell crisis includes ways to help you cope with pain and other signs and symptoms. Treatments may decrease pain during a crisis, and may help prevent a crisis from growing to become life-threatening. It can also help prevent another crisis.

AFTER YOU LEAVE:

Take your medicine as directed.

Call your healthcare provider if you think your medicine is not helping or if you have side effects. Tell him if you are allergic to any medicine. Keep a list of the medicines, vitamins, and herbs you take. Include the amounts, and when and why you take them. Bring the list or the pill bottles to follow-up visits. Carry your medicine list with you in case of an emergency.

  • Pain medicine: Pain medicine will help you cope with pain. Tell caregivers what medicine has worked in the past to decrease your pain.
    Pain Scale


  • Steroids: Steroid medicine may be used along with pain medicine. It may help decrease the amount of pain that you are having. It can also make the pain go away sooner.

  • Anti-depressants: This medicine is used to decrease or stop symptoms of depression (deep sadness). It can also be used to help treat pain.

  • Antibiotics: This medicine fights infections that are caused by germs called bacteria. You may need this medicine to help fight an infection related to the crisis.

  • Hydroxyurea: This is medicine that can be used to treat cancer, but also may be used to treat sickle cell disease. Hydroxyurea can help your body make red blood cells that are less likely to sickle. Using this medicine may help decrease how often you have a painful crisis. This medicine may also help prevent a stroke. Tell caregivers if you are, or think you might be pregnant before using this medicine.

  • Bronchodilators: This medicine is used to help widen your airway so that you are able to breathe more easily.

  • Minerals: Caregivers may suggest that you take a mineral called zinc. This medicine may decrease how often you have a crisis.

Ask for information about where and when to go for follow-up visits:

For continuing care, treatments, or home services, ask for more information.

  • Talk to caregivers about your pain. Tell them how often you have it, and where the pain is in your body when it comes. Tell caregivers how bad your pain is, and what you do to cope with it. Tell caregivers about any new signs or symptoms, such as vision changes. Your caregiver may do tests to check your eyes, bones, liver, and other body parts. If you need to have ongoing blood transfusions, schedule them with your caregiver.
    Pain Scale


Sickle cell crisis triggers:

What causes a sickle cell crisis for you may be different than what causes it for someone else. You may not know what causes a sickle cell crisis to happen for you. The following are triggers that may make a sickle cell crisis happen:

  • Dehydration:

    • This is when your body has lost too much fluid. Losing body water makes it more likely that red blood cells will block your blood vessels. With sickle cell disease, your kidneys are not able to save as much body fluid as people who do not have the disease. This makes you more likely to get dehydrated.

    • This can happen when it is hot outside. It can also happen if you do not drink as much liquid as your body needs. You are more likely to get dehydrated if you are at high altitude (such as in the mountains). Dehydration is also more likely if you exercise without drinking enough liquid.

  • Infections: Getting an infection in your lungs can cause a sickle cell crisis.

  • Hypoxia: Hypoxia happens when your body does not get enough oxygen. Hard exercise such as running can cause hypoxia. Being at high altitude and flying in an airplane can also cause lack of oxygen.

  • Cold temperature: Being in a cold place may trigger a sickle cell crisis for some people. A crisis may also be started if you quickly go from a warm to a cold place.

  • Surgery: Having surgery or a medical procedure puts a strain on your body. Having a baby causes a woman's body to work very hard, and can trigger a sickle cell crisis.

  • Emotional stress: Strong feelings, such as anger, frustration and depression may trigger a sickle cell crisis.

Signs and symptoms of a sickle cell crisis:

You may have one symptom such as pain, or you may have many symptoms at the same time. Your signs and symptoms may change each time you have a crisis. Signs and symptoms can be mild or severe (very bad), and can get worse over time. You may have any of the following signs and symptoms:

  • Pain in a body part, such as your back, stomach, chest, or bones: The pain can stay in one body area, or spread to other places in your body. The pain can last for minutes, hours, or up to a week. In women, pain can come more often and worse during their menstrual cycle. The pain can also be worse when a women is near menopause (when the menstrual cycle stops). Women who are pregnant may have pain more often than when they are not pregnant. The pain may also be more severe.

  • Trouble breathing: This can happen when you are resting and when you are active.

  • Headaches: You may often have a headache. Headaches that are new or different can be a sign of a sickle cell crisis.

  • Signs and symptoms of anemia: These include feeling more tired than usual, headaches, and trouble breathing. Areas of your body may hurt. Your skin color may change to white or light-colored.

  • Priapism: This is when a man's penis is very painful when it is erect. This can be a sign that you are going to have a sickle cell crisis. This problem will usually go away without treatment. If it does not go away, the pain can be severe, and must be treated by caregivers right away.

  • If you have a problem called acute chest syndrome, you will have chest pain and trouble breathing: You may feel like your heart is beating fast, and you may have a fever (high body temperature).

  • If you are having a splenic sequestrian crisis, you will feel weak and short of breath: Your abdomen will swell and feel painful.

Ways to help prevent a sickle cell crisis:

  • Drink enough liquid: Drink at least 3 to 4 liters (about 14 to 16 eight-ounce cups) of healthy liquids every day. For most people this includes water, milk and juice. In hot weather, drink more than this amount. Talk to your caregiver if you drink alcohol, such as beer, wine, vodka, or other adult drinks.

  • Get vaccinated: Vaccinations can help prevent the infections that may lead to a sickle cell crisis. You should get a flu shot every year. If you do not have hepatitis B (a type of liver infection), get vaccinated against this virus. Ask your caregiver what other vaccinations you should have.

  • Balance rest and exercise: Rest during a sickle cell crisis. Over time, increase your activity to a moderate amount. Exercise regularly, such as every day. Avoid high-impact exercise or activities, such as playing football, which may cause injury. Avoid lifting heavy weights for exercise. Talk to you caregiver about the best exercise plan for you.

  • Avoid a sudden change of air pressure or lack of oxygen: Travel in airplanes with normal cabin pressure when flying. Avoid going to high altitude places, such as the mountains.

  • Avoid cold places: Keep your body warm in the winter, and at a temperature that feels right for you in the summer. Avoid quickly going from a warm to a cold place. Do not go swimming in cold water.

  • If you smoke, quit: It is never too late to quit smoking. Smoking can cause more trouble breathing, and damage your lungs. If you have a lung disease it is even more important to stop smoking. You will help yourself and those around you by not smoking. Ask your caregiver for more information about how to stop smoking if you are having trouble quitting. Tell caregivers if you use any street (illegal) drugs.

  • Eat a healthy diet: With sickle cell disease, you may need to be sure that you get enough folic acid. This vitamin is found in vegetables and fruit. Eat at least five servings a day of leafy vegetables and fruit. Get to and stay at a healthy weight. Ask your caregiver about the best eating plan for you.

Coping with pain:

  • Use medicine.

    • Talk to your caregiver about what medicines to use first to treat pain, and when they should be used. For menstrual cramps (in women), or a mild headache, you may use non-steroidal anti-inflammatory medicine (NSAIDs). You may be able to use acetaminophen for mild pain that you do not think is caused by a sickle cell crisis. If your pain is not better using these medicines, call your caregiver.
      Pain Scale


    • You may have pain medicine that has been prescribed (ordered) by your caregiver, such as narcotics. Use this medicine as directed by your caregiver. Do not use this medicine for reasons other than to treat pain, such as to decrease your worries. Talk to your caregiver if you are worried about growing addicted to the pain medicine. Know when the pain is bad enough that you need to go to the hospital.

  • Get counseling.

    • With sickle cell disease, you may fear that you will have a crisis. You may fear another crisis if you have already had one or more of them. You may feel like you cannot control what happens to your body. This can cause you to feel helpless and depressed. Pain that is ongoing can make you feel that it is hard to get through each day.

    • Meet with a mental health caregiver regularly. Talk to him about problems with friends and family. Ask caregivers for ways to cope with feelings of sadness and worry, and for more ways to cope with your pain. Cognitive-behavior therapy may be suggested by caregivers. Ask them for more information about this therapy.

  • Learn hypnosis. This therapy teaches you to change your level of awareness. This means that caregivers teach you to think about something other than your pain. You make yourself open to suggestions, like feeling happy and having more energy. Hypnosis can give long-lasting relief from depression without changing your normal activities. This treatment teaches you to have better control over your body. With practice, you may learn to do self-hypnosis without a caregiver helping you. For some people, hypnosis may help prevent a sickle cell crisis.

  • Take time to relax. Surround yourself with good smells and listen to your favorite music. Take a warm bath, and try to relax your body. While lying down on a bed, focus on relaxing your muscles. Start at your toes and feet, and relax those body areas. Slowly move up your body, relaxing body parts as you go. Doing these things may help decrease your pain.

  • Use distraction. This is a way of focusing your attention on something other than your pain. Playing cards or games, watching TV or taking a walk are all ways to do this. Other ways are visiting with friends, painting, petting animals, and writing down your feelings.

  • Try biofeedback. A biofeedback machine can help you learn when your body is tense and when it is relaxed. Learning this can also help you learn when and how to relax. Teaching your body to relax can help you feel more in control. You can also learn how to relax without using a machine. If you learn to check your own pulse, you can also learn how to slow it down. This can also slow down your breathing, and decrease your blood pressure.

Support and more information:

You may find it very hard to cope with the pain of a sickle cell crisis. You may feel very sad, angry, helpless, and out of control. Talk to caregivers about your feelings. If you and your partner want to have a child, talk to your caregiver. At all times, carry an identification card with you that says you have sickle cell disease. The card has the information that medical workers need in case you have a crisis emergency. Ask caregivers where to get this card. Contact the following for more information:

  • Sickle Cell Disease Association Of America
    231 East Baltimore St., Suite 800
    Baltimore , MD 21202
    Phone: 1- 410 - 528-1555
    Phone: 1- 800 - 421-8453
    Web Address: http://www.sicklecelldisease.org

CONTACT A CAREGIVER IF:

  • You have any new signs or symptoms.

  • There is blood in your urine.

  • You have new trouble breathing, even while resting, and are more tired than usual during the daytime.

  • You are constipated (having hard, dry stools less often than usual, that are difficult to pass), or you have diarrhea (loose, watery stools).

  • Your eyesight has changed in one or both eyes.

  • You are planning travel by airplane, or to a high elevation.

  • You have questions about sickle cell crisis or your treatment.

SEEK CARE IMMEDIATELY IF:

  • You feel like you can no longer cope with your pain, or feel like killing yourself.

  • Those around you say that you are not acting like yourself, or you have a seizure (convulsion) or lose consciousness (pass out).

  • You have a fever (high body temperature).

  • You have stomach pain, and you feel sick and are throwing up.

  • You are a male, and you have an erection that is painful and does not go away.

  • You cannot see out of 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 a new cough, trouble breathing and chest pain.

  • You have a new rash, itchy skin, or feel sick after starting to use a new medicine.

  • You suddenly feel weak, dizzy and have trouble breathing.

© 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.

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.

Learn more about Sickle Cell Crisis (Discharge Care)

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