Pain Medications and Sickle Cell Disease

Most people with sickle cell disease (SCD) experience an acute pain episode in their lifetime. The first episode can occur as early as 6 months old. Pain episodes occur with any frequency, usually in the hands, feet, chest, and back.¹

People with SCD treat most episodes at home. When home management is not enough, treatment in a hospital is necessary. However, racial disparities in access to healthcare and misconceptions about opioid use disorder often interfere with treatment.

How are pain medicines used to treat sickle cell at home?

About 1 in 3 people with SCD experience pain every day. Pain is treated at home about 10 times more often than in the hospital.^2,3

Home treatments include non-steroidal anti-inflammatory drugs (NSAIDs) and oral opioids. NSAIDs should not be used longer than 10 days to manage acute pain in SCD without talking to your doctor. You and your doctor should create a plan for treating pain at home. It should include when to go to the hospital.

NSAIDs are common pain and fever relievers. They block the production of chemicals in the body that causes inflammation. Some NSAIDs used to relieve sickle cell pain include:⁴

• Ibuprofen (Motrin, Advil)
• Aspirin (Bayer)
• Naproxen sodium (Aleve)

These NSAIDs are available over the counter. Acetaminophen (Tylenol) is not an NSAID, but it may also relieve pain. Stronger NSAIDs, such as diclofenac (Voltaren) can be prescribed. Tell your doctors about NSAIDs you take, and ask them how to safely take them.⁴

More severe pain episodes may be treated with oral opioids. About 80 percent of at-home pain management involves opioids. Depending on the severity and what medicines have worked, your doctor will prescribe you certain types. Some oral opioids used to treat sickle cell pain include:^2,3,5

• Codeine
• Fentanyl
• Hydrocodone
• Morphine
• Oxycodone/Oxycontin
• Hydromorphone (Dilaudid)

How are pain medicines used to treat sickle cell pain in the hospital?

When home management does not relieve pain, treatment in an ER is necessary. Pain relief should begin within 30 minutes of arrival. However, many people with SCD see delays due to societal racial inequalities. This includes limited access to quality healthcare for Black people with SCD. Some reasons include:^6-8

• Lack of lab tests to confirm acute pain
• Stigma surrounding SCD
• Doctors who are inexperienced in treating SCD
• Overcrowded hospitals
• Perceptions of people with SCD as “drug seekers”

The best assessment for pain is the person’s report of their own pain. However, doctors who are not experienced in treating SCD or aware of their implicit biases often assume people with SCD are just seeking drugs. Doctors often overestimate the rate of opioid use disorder in SCD.⁹

Doctors usually treat acute pain with intravenous (in the vein) opioids. They will reassess pain after the opioid is expected to work. If pain continues, your doctor may give repeat doses or increase the opioid strength.⁵

Once the pain subsides, opioids will be gradually reduced. After leaving the hospital, you should be given oral opioids to manage pain at home. Discharge from the hospital without opioid prescriptions increases the risk of hospital readmission.^5,10,11

What is patient-controlled analgesia?

If pain continues after multiple doses, you may stay for overnight patient-controlled analgesia (PCA). PCA is a continuous infusion that lets you control your own pain. PCA pumps allow you to add single doses of opioids during the infusion as needed.^5,7

Compared to occasional morphine dosage, young adults with SCD using PCA experience:^12-14

• Shorter pain duration
• Shorter hospital stays
• Less morphine used
• Less nausea and vomiting
• More satisfaction with care

What are the risks associated with pain medications?

NSAIDs and opioids can have severe side effects if used improperly. NSAIDs should not be used for longer than a week to manage acute pain in SCD. NSAIDs can increase the risk of heart attack or stroke. The risk for side effects increases if you take larger or longer doses of NSAIDs. The most common side effects of NSAIDs are:^4,15

• Gas and bloating
• Heartburn
• Stomach pain
• Nausea
• Vomiting
• Diarrhea or constipation

These are not all the side effects of NSAIDs. With your doctor, plan how to take NSAIDs to treat pain episodes. Call your doctor if you notice any changes in symptoms after taking NSAIDs.¹⁵

Opioids also have risks for people with SCD. These risks can be managed with other medicines. The risks include:⁵

• Sedation, which can increase the risk of acute chest syndrome
• Altered sleep quality, which can increase pain sensitivity
• Nausea and constipation
• Itchy skin

Opioid misuse is actually more uncommon in SCD than in other chronic pain conditions and the general population. Concerns about addiction should never interfere with pain treatment.⁶

What are other ways to manage pain?

Hydration may relieve pain and reduce the risk of other complications. Pain management at home and in the hospital should always include increased fluid intake.^1,16

Ketamine may treat severe pain that does not respond to opioids. Ketamine is commonly used as an anesthetic, but it can also relieve pain at low doses. It is a newer, off-label treatment for relieving sickle cell pain, and some practitioners may not be as experienced in using ketamine for treating sickle cell disease.¹⁷

Other therapies have also been used to treat pain. These include:^5,17

• Muscle relaxation or breathing exercises
• Massage and yoga
• Self-hypnosis
• Heat compresses
• Treatments for sleep-disordered breathing
• Antidepressants

Before beginning treatment for SCD, tell your doctor about all your health conditions and any other drugs, vitamins, or supplements you take. This includes over-the-counter drugs.