Pain Management With Sickle Cell Disease

Acute pain episodes are the most common cause of hospitalization for people with sickle cell disease (SCD). However, most episodes are managed at home. Plan with your doctor how you will manage pain at home and when you will get emergency help.¹

This plan should include what medicines and techniques to use at home. It should also include when and how to go to the ER for severe pain. You can design this plan with your doctor and use it to make sure you get pain relief as fast as possible.

What is a pain management plan?

A pain management plan is a personalized document that details how to control pain when an episode starts. You can create this plan with your doctor and include it in your electronic medical record (EMR). This can help you treat pain in a controlled way. It also helps ER doctors who are less familiar with you give you the best possible care.²

A pain management plan should include:³

• How to prevent pain and what triggers to avoid
• How to treat mild pain at home
• How to use other pain management techniques
• When to go to the hospital and what to bring to the hospital
• How to treat pain in the hospital
• Additional explanations for doctors less familiar with you

Every person’s pain level and frequency vary. The treatments that work for 1 person may not work for you.

How is pain managed at home?

The techniques and medicines used to manage pain at home vary for each person. This should be included in your pain management plan so you can quickly do what works for you. Usually, at-home pain management consists of hydration and pain medicines.³

Pain medicines include non-steroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen and aspirin. Stronger NSAIDs may also be prescribed. Always tell your doctor about NSAIDs you take and ask them how to safely take them. In some cases, acetaminophen may be used instead of an NSAID.⁴

More severe pain episodes may be treated with oral opioids, such as codeine and morphine. Most at-home pain management includes opioids. Your doctor can prescribe certain types depending on the severity of your pain and what medicines have worked in the past.^5,6

Other pain management techniques are also often used, including:⁷

• Muscle relaxation
• Breathing exercises
• Massage
• Yoga
• Heat compresses

Talk to your doctor to determine which medicines and techniques to use and how to use them.

When should I go to the ER?

Your pain management plan should include when to get treatment in the ER. This plan should also include the details of how you will get to the ER. For children, this plan should be shared with teachers or daycare staff. Go to an emergency room right away if you experience:⁸

• Fever above 101°F
• Difficulty breathing
• Chest pain
• Abdominal swelling
• Severe headache
• Sudden weakness or loss of feeling
• Seizure
• Painful erection that lasts more than 4 hours
• Pain that will not go away with treatment at home
• Sudden vision problems

How can I receive better care in the ER?

Ideally, pain relief should begin within 30 minutes of arriving at the ER. However, many people with SCD experience delays in treatment for pain. These delays are largely due to systemic inequalities that limit access to healthcare for Black people with SCD. Some of these reasons include:^9-11

• Lack of laboratory tests to assess pain
• Lack of doctors experienced in treating SCD
• Overcrowding at nearby hospitals
• Perception of people with SCD as “drug seekers”

Many people with SCD report negative interactions with doctors in the ER. The best assessment of pain is the person’s own report. However, doctors who are less experienced in treating SCD often assume that people with SCD are just seeking drugs, yet there is little evidence of opioid misuse in people with SCD.¹²

Because of this, going to the ER can be extremely stressful. Your pain management plan should include what to bring to the ER and what to do in the ER to receive better care. Here are some tips to receive the best care in the ER:¹³

• Make sure your pain management plan is included in your EMR
• Bring a printed copy of the plan to the ER
• Bring a list of all of your medicines
• Tell the ER staff right away that you have SCD and encourage them to contact your SCD specialist
• Share your medical history and pain management plan with the ER staff
• Share your regular doctor’s contact information with the ER staff