Sickle Cell Pain Episode

Sickle cell anemia is also called sickle cell disease (SCD). It's an inherited disease that affects your red blood cells. It occurs most often in people of African descent. The most common symptom of sickle cell anemia is a pain crisis. This occurs when the red blood cells change shape and block blood flow in the smaller blood vessels. Pain crisis can affect the bones, joints, chest, or belly (abdomen). This may happen if you:

• Don't drink enough fluids (get dehydrated)
• Have large temperature swings from warm to cold
• Have an infection
• Drink too much alcohol
• Are stressed
• Are exhausted

Having too little oxygen in your blood may also trigger a pain crisis.

Home care

Follow these guidelines when caring for yourself at home:

• Drink at least 3 quarts of fluid (12 8-ounce glasses) over the next 24 hours. This will make sure you aren't dehydrated.
• Rest until all your pain is gone.
• Apply a heating pad to painful areas. Don't use ice as it can make the pain worse. Topical ointments such as Aspercreme, Bengay or lidocaine patches may provide relief to painful joints. Do not apply heat on top of topical medications.
• Take any prescribed pain medicines as directed. You may take acetaminophen or ibuprofen instead for milder pain. If you have long-term (chronic) liver or kidney disease, talk with your healthcare provider before using these medicines. Also talk with your provider if you’ve had a stomach ulcer or GI bleeding.
• Get treated for any infection (cold, flu, skin infection) as soon as it happens.
• Get early prenatal care if you are pregnant or plan to get pregnant.

Preventing future attacks

• Keep yourself well-hydrated. Drink at least 8 glasses of water and other fluids every day. Drink more when you have a fever, are driving at high altitudes or traveling by air.
• It is important you don’t overexert yourself or be exposed to very hot or cold temperatures. Don't do strenuous work or exercise, such as training for an athletic competition. These may make you very tired or dehydrated.
• Limit how much alcohol you drink. A drink once in a while may be OK when you don’t have symptoms. Always check with your healthcare provider.
• Attempt to avoid high altitudes such as flying, climbing or cities with high altitude. If you must encounter these scenarios stay well hydrated, when traveling on an airplane consider using compression stockings along with getting up frequently to move your legs and if traveling to high elevations try to acclimate to the elevation changes slowly along with plenty of hydration.
• Take your prescribed medicines.
• Adults with severe SCD may take medicines (such as hydroxyurea or L-glutamine) to help reduce the number of pain crises. Research shows that hydroxyurea therapy may also help babies and children with SCD.
• Some people have severe pain and might require an additional opioid medication daily.
• It is important to take the right vitamins and amount. Discuss with your provider if you should be taking daily calcium, vitamin D3, and or a multivitamin without iron. If you are pregnant you should be taking folic acid (folate) 4 mg daily, if you are not pregnant, 1 mg folate daily.
• It is important to keep your vaccinations up to date. Check your medical records to make sure your influenza, pneumonia, meningitis, and hepatitis are current. If they are not discuss with your provider.
• Discuss with your provider if voxelotor, crizanlizumab, a stem cell transplant or any other new treatments for SCD are right for you.
• If you smoke it is important you stop to reduce the number of pain crises. There are resources available, such as a non-smoking program, to help improve your chances of quitting. Consult with your provider on what resources are available to you. Don’t allow smoking in your home or car. Stay away from secondhand smoke.
• Keep all appointments for follow-up. If you use hydroxyurea, your healthcare provider will likely check you often. This is to prevent complications such as infections.
• Talk with your family and friends about SCD. People are often not aware of how painful the disease can be. 

If your pain crisis is severe, you may need to go to the hospital for intensive treatment.

Follow-up care

Follow up with your healthcare provider, or as advised. Keeping all routine follow-up appointments is key for managing sickle cell disease. Don’t forget to keep your appointments with your dentist and eye doctor. Talk with your provider if there are issues that make it hard for you to follow up (such as not having health insurance coverage for prescribed medicines). Wear a medical alert bracelet or necklace indicating you have “sickle cell disease.”

When to get medical advice

Call your healthcare provider right away if any of these occur:

• Pain that doesn’t get better after taking the medicines prescribed
• Fever of 100.4°F (38ºC) or higher, or as directed by your provider
• Cough with dark sputum or shortness of breath
• Blood (pink, brown, or red) in your urine
• Trouble with speech or vision
• Any painful joint that gets hot, swollen, or red
• Headache that is different from normal
• Not being able to move, or feeling like a part of your body is numb
• Sudden weakness or numbness
• Painful erection that doesn't go away

• New symptoms not seen before with the disease