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 lasts 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 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 acutely 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, also known as a vaso-occlusive episode.
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 managing 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 possible organ complications (as with the 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.
Handle sickle cell crises like a pro: Read about sickle cell triggers and how to manage your symptoms.
Concerned about side effects from opioids for sickle cell pain? Take control of your health with must-know opioid considerations.
Hydroxyurea and diet: Hydroxyurea is a common medication for managing sickle cell disease over time. Learn more about how your diet can impact this medication and its side effects.
If you have SCD, your healthcare professional 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, 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. But it’s not widely used as a treatment yet.
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 treat 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
Chronic (long-term) opioid use has additional risks. 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.
If you’re experiencing any bothersome side effects from opioids, it’s recommended to talk to a healthcare professional 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 healthcare professional can make necessary adjustments to your treatment.
Can any medications prevent a sickle cell crisis?
Yes. A number medications are available that help lower the frequency of sickle cell crises:
Hydroxyurea (Siklos, Xromi): Hydroxyurea is a go-to oral prevention medication for adults and children with SCD. Hydroxyurea can help lower the frequency of painful crises. It has also been shown to lower the need for blood transfusions.
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. It uses slightly different technology in order to work, though.
Your healthcare professional 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).
Good to know: Voxelator (Oxbryta) was an oral medication that helped lessen the severity of SCD. It did so by improving hemoglobin levels. It was discontinued by its manufacturer in September 2024, citing safety concerns.
Frequently asked questions
There isn’t one clear winner. The best medication for SCD depends on your symptoms, their severity, and your treatment goals. For example, medications such as hydroxyurea can help prevent crisis symptoms. On the other hand, NSAIDs and opioids manage pain during a pain crisis.
Yes, many people living with SCD take medication daily to manage symptoms and prevent complications. This includes medications such as hydroxyurea. But not all medications are meant for daily use. For example, Adakveo is administered monthly, and gene therapies like Casgevy are one-time infusions.
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 treatments. Supplemental oxygen and fluids are beneficial too. Other medications, such as hydroxyurea (Siklos, Xromi) 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
Adesoye, O. (2024). How I manage mild to moderate sickle cell pain at home. Sickle Cell Disease News.
Alshahrani, M. S., et al. (2021). Ketamine for sickle cell vaso-occlusive crises: A systematic review. Saudi Journal of Medicine and Medical Sciences.
Baldini, A., et al. (2012). A review of potential adverse effects of long-term opioid therapy: A practitioner’s guide. The Primary Care Companion for CNS Disorders.
Bluebird Bio. (2023). Bluebird Bio announces FDA approval of LYFGENIA (lovotibeglogene autotemcel) for patients ages 12 and older with sickle cell disease and a history of vaso-occlusive events.
Brandow, A. M., et al. (2020). American Society of Hematology 2020 guidelines for sickle cell disease: Management of acute and chronic pain. Blood Advances.
Centers for Disease Control and Prevention. (2024). Complications of SCD: Pain.
Howard, J. (2016). Sickle cell disease: When and how to transfuse. Hematology, the American Society of Hematology Education Program.
Karsenty, C., et al. (2022). Regional anesthesia for sickle cell disease vaso-occlusive crisis: A single-center case series. Pediatric Blood & Cancer.
McGann, P. T., et al. (2015). Hydroxyurea therapy for sickle cell anemia. Expert Opinion on Drug Safety.
National Heart, Lung, and Blood Institute. (2014). Evidence-based management of sickle cell disease: Expert panel report, 2014. National Institutes of Health.
National Heart, Lung, and Blood Institute. (2024). Sickle cell disease: Treatment. National Institutes of Health.
National Institute on Drug Abuse. (2021). Prescription opioids drugfacts. National Institutes of Health.
Novelli, E. M., et al. (2016). Crises in sickle cell disease. Chest.
Pfizer. (2024). Pfizer voluntarily withdraws all lots of sickle cell disease treatment OXBRYTA (voxelotor) from worldwide markets.
