NSAIDs, Opioids, and More: What Medications Are Used for a Sickle Cell Crisis?
Key takeaways:
A sickle cell crisis is a painful event that occurs in people who have sickle cell disease (SCD). These episodes can cause intense pain that may last hours to days.
A sickle cell crisis is typically treated with pain medications such as acetaminophen (Tylenol), nonsteroidal anti-inflammatory drugs (NSAIDs), or opioids. The best choice will depend on your symptoms and pain severity, among other factors.
Several medications are also FDA approved to prevent sickle cell crises from happening in the future. This includes two gene therapy medications: exagamglogene autotemcel (Casgevy) and lovotibeglogene autotemcel (Lyfgenia).
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Table of contents
If you or a loved one have sickle cell disease (SCD), you're well aware how painful the condition can be. Sometimes, this pain can reach an overwhelming tipping point and cause what’s known as a “sickle cell crisis.”
A sickle cell crisis can cause a feeling of intense pain that needs immediate treatment. It happens when your red blood cells form a sickle shape — similar to the letter C — and clump together to block one or more blood vessels. This jam can happen suddenly and affect any part of your body.
If and when this happens, pain medications are a priority. They're crucial to controlling pain and discomfort from this complication of SCD. But what are these medications? And can any of them prevent sickle cell crises from happening in the first place?
Here, we’ll talk about what you need to know about medications used to treat and prevent sickle cell crises.
Why are medications given during a sickle cell crisis?
One of the main purposes of treating a sickle cell crisis is to ease your pain as soon as possible. Sickle cell pain may appear invisible, but it's a very real threat. It’s also important to treat it promptly to prevent organ complications (e.g., kidneys, heart, lungs) and other serious events that could occur.
Pain medications are a central part of treating a sickle cell crisis. You may also receive supplemental oxygen, fluids, and other non-medication treatments. At times, a blood transfusion may also be considered. Blood transfusions help filter out sickled cells and replace them with new ones.
If you have SCD, your healthcare professional (HCP) should give you specific instructions on what to do if you experience a sickle cell crisis. This plan should include guidance on when you can try to self-treat pain at home compared to when you should seek emergency care.
What pain medications are used to treat a sickle cell crisis?
Several medications can relieve pain and discomfort from a sickle cell crisis. Depending on your symptoms, one or more medications may be used. The best treatment(s) for you will depend on your symptoms, pain severity, and medical history.
In general, milder pain may be treated with a class of medications known as nonsteroidal anti-inflammatory drugs (NSAIDs). Ibuprofen (Advil, Motrin) is one common NSAID. Acetaminophen (Tylenol) may also be used for milder pain.
If your pain is more intense and won’t respond to medications like NSAIDs, opioid pain medications are more appropriate. They're preferred if a sickle cell crisis is causing more severe pain. Opioid medications can be used with or without NSAIDs or acetaminophen.
When used to treat a sickle cell crisis, opioid medications are often given in a hospital setting, but they can be taken at home too. Depending on your symptoms, you may receive them by mouth or as an injection. Morphine, oxycodone (OxyContin), and hydromorphone (Dilaudid) are three common examples.
If your pain still isn’t controlled, your care team may consider other options. For example, some health experts suggest that ketamine infusions may offer additional relief for some people. Nerve anesthesia can help, too.
Why are opioid pain medications typically given to treat a sickle cell crisis?
A sickle cell crisis is highly debilitating. Opioid pain medications may be the only way to adequately control your pain.
The benefits of opioids usually outweigh their drawbacks when treating a sickle cell crisis. But opioids do have risks — especially when taken at higher doses or for long periods of time. They’re usually used sparingly for a number of other health conditions for this reason. They’re also controlled substances.
Your care team will ultimately determine what type of treatment you need. But if this includes an opioid, they’ll choose which opioid to use depending on the severity of the crisis and other factors specific to your situation.
Are there any risks with using opioids for a sickle cell crisis?
Yes. Although opioids are often recommended for sickle cell pain, they have many possible risks and side effects.
Potential side effects of short-term opioid use include:
Constipation
Nausea
Vomiting
Drowsiness
If you’re experiencing any bothersome side effects from opioids, it’s recommended to talk to your HCP as soon as you can. No matter if you’re receiving opioids in the hospital or taking them at home, this is important so your HCP can make necessary adjustments to your treatment.
What’s more, chronic (long-term) opioid use has several risks too. These include medication dependence and misuse. These effects can happen to anyone due to the way opioids affect your brain chemistry. These risks also go up the longer you take opioids.
At any point throughout treatment, life-threatening opioid overdoses are also possible. This may include symptoms of slow or shallow breathing and altered consciousness. People at higher risk of an opioid overdose are recommended to carry naloxone (Narcan, Opvee) or have it at home. This is a life-saving medication that can reverse an opioid overdose.
Can any medications prevent a sickle cell crisis?
Yes. A number medications are available that can help lower the frequency of sickle cell crises:
Hydroxyurea (Droxia, Siklos): Hydroxyurea is a go-to oral prevention medication for adults with SCD. Hydroxyurea can help lower the frequency of painful crises. It has also been shown to lower the need for blood transfusions in adults.
Crizanlizumab-tmca (Adakveo): Crizanlizumab is an injectable medication. It can help lower the frequency of pain crises in people ages 16 and older with SCD.
L-glutamine powder (Endari): This oral medication can also help reduce the frequency of pain crises. It can be used by people ages 5 and older with SCD.
Exagamglogene autotemcel (Casgevy): This one-time gene therapy medication is infused into a vein. It’s a potential SCD cure for people ages 12 years and older. It’s designed to eliminate pain crises and hospitalizations from SCD.
Lovotibeglogene autotemcel (Lyfgenia): This is another one-time gene therapy medication. It has a lot of overlap with Casgevy. However, it uses slightly different technology in order to work.
In addition to medications, your HCP may also recommend other ways to help prevent sickle cell crises from happening. This may include drinking plenty of water, avoiding extreme temperatures, and avoiding places with low oxygen levels (e.g., high elevation).
The bottom line
Sickle cell crises can cause intense pain and discomfort. Acetaminophen (Tylenol), nonsteroidal anti-inflammatory drugs (NSAIDs), and opioids are all recommended sickle cell crisis treatment options. Supplemental oxygen and fluids are beneficial too. Other medications, such as hydroxyurea (Droxia, Siklos) and exagamglogene autotemcel (Casgevy), aim to prevent sickle cell crises from happening in the first place.
If you have any questions about treating SCD or a sickle cell crisis, it’s recommended to talk to your pharmacist or prescriber.
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References
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