Sickle Cell Crisis: Symptoms, Triggers, and Treatment

Sickle cell disease (SCD) is an inherited blood condition that affects millions around the world and about 100,000 people in the U.S. SCD — also known as “sickle cell anemia” — is most common among Black or African Americans and affects 1 out of every 365 births. 

SCD is a challenging condition that puts people at increased risk of developing serious health complications. This is because SCD causes the body to produce faulty hemoglobin, which carries oxygen in the blood. Red blood cells become stiff and change shape, leading to blocked blood vessels. 

In this article, we’ll discuss one of the most common and important health complications in SCD — the sickle cell pain crisis. We’ll cover symptoms, triggers, and when to seek treatment.

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What is a sickle cell crisis?

A sickle cell crisis, or pain crisis, occurs when the abnormal sickled blood cells lump together. In doing so, they block small blood vessels that supply various organs, bones, and muscles. This process is called “vaso-occlusion.” That’s why a sickle cell crisis is also known as a “vaso-occlusive crisis.” The end result is organ damage, pain, and inflammation. 

Sickled blood cells can block blood vessels that supply vital organs, including the brain and lungs. A blockage that happens in the brain can cause a stroke. If it happens in the chest, this can cause a type of sickle cell crisis called “acute chest syndrome.” Sickled cells block blood vessels in the lungs and cause some of the tissue in the lung to die. This can cause chest pain, shortness of breath, and decreased oxygen levels. The low oxygen levels cause the blood cells to sickle more, worsening the disease.  

Another type of pain crisis is when sickled cells clog blood vessels in the hands and feet. This is called dactylitis, or hand-foot syndrome. It can cause swelling and a lot of pain. Hand-foot syndrome is most common in infants and toddlers.

How painful is a sickle cell crisis?

Pain is a subjective and personal experience. That means everyone feels pain differently, which can make it hard to discuss. And the terms we use to talk about pain matter because they help to determine treatment. 

This is why some clinicians advocate for the use of the terms “acute painful episode” or vaso-occlusive “pain” in place of sickle cell or vaso-occlusive “crisis.” These advocates believe that this avoids underdiagnosing and undertreating people with SCD who are in pain but do not appear to be in “crisis.” But it’s still common to see and hear the term “sickle cell crisis.” We’ll use both terms in this article.

So, depending on the person and the specific event, a sickle cell crisis can range from mild to extreme pain. And the pain can last for hours to days. Sickle cell crises can start in people as early as 6 months old. Over 90% of people with SCD have a painful episode by 6 years old. 

The pain can affect almost any part of the body. It may start in one area but commonly involves at least two more sites, including the:

• Upper and/or lower back  

• Legs

• Knees

• Arms

• Chest

• Abdomen

Many people with SCD say that the pain is difficult to describe. Young kids can be irritable or fussy. Some describe the pain in more typical terms like aching, pounding, or tearing. Others describe the pain as: 

• Being “stabbed repeatedly”

• Having “a full body migraine”

• Being “electrocuted”

• Having “multiple fractures, broken bones, and glass flowing throughout [the] body”

• Feeling “like you just had surgery”

What are common symptoms of a sickle cell crisis?

In addition to pain in one or more parts of the body, there are other signs and symptoms that suggest an acute painful episode. They include:

• Weakness and fatigue

• Headache

• Dizziness or lightheadedness

• Yellowing of the skin and eyes (jaundice)  

• Swelling in the hands and feet, seen in dactylitis

What can trigger a sickle cell crisis?

The cause of a painful episode can be difficult to pinpoint. There’s often more than one cause or no clearly identifiable cause at all. 

Some of the triggers include:

• Drastic temperature changes, especially going from hot to cold 

• Dehydration

• High altitudes (where there’s less oxygen in the air)

• Infection

• Physical or psychological stress

• Extreme physical exercise

Sickle cell crisis treatment

The unpredictable nature of SCD episodes is one of the many reasons the condition can have such an effect on quality of life. And many people with SCD prefer to manage a mild or moderate painful episode at home, rather than depending on the emergency room (ER) every time. That’s why they work with their doctor, pediatrician, primary physician, or blood specialist (hematologist), to come up with a treatment plan. 

First steps for treating a painful episode at home can include warm packs or a heating pad on the sites that hurt. A warm bath, yoga, massage, and meditation may also be helpful. Staying hydrated with fluids (often at least 8 to 10 glasses a day) can help with circulation. 

Over-the-counter medications can also help with pain:

• Acetaminophen (Tylenol)

• Nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Advil, Motrin) or naproxen (Aleve) 

Many people with SCD develop problems with their kidneys from the disease. In that case, the American Society of Hematology (ASH) recommends a short course (5 to 7 days) of NSAIDs to protect the kidneys. Acetaminophen may be a better option than NSAIDs. 

For moderate to severe pain, most at-home plans for an acute painful episode include long-acting oral opioid medication such as morphine ER (MS-Contin) or oxycodone ER (Oxycontin). These can last for 8 to 12 hours. Short-acting opioids such as immediate-release oxycodone (Roxicodone) or hydromorphone (Dilaudid) can be helpful for breakthrough pain. These are all prescription pain medications, so you’ll need to discuss this plan with your healthcare provider.

When should you seek emergency care for sickle cell crisis? 

The most common reason people with SCD visit the ER or need hospitalization is a painful episode.

Most people with SCD have had pain crises in the past. If you’ve had a painful event before, you probably know when something is different about a crisis or when there’s cause for concern. But if you are unsure, here are some signs and symptoms to look out for:

• Ongoing pain: Pain that doesn’t get better with your at-home medications may need further attention.

• Difficulty breathing and/or cough: This could suggest acute chest syndrome or a lung infection.

• Chest pain: This could also be concerning for acute chest syndrome and/or problems with the heart.  

• Severe abdominal pain and swelling: This could suggest an issue with one of the organs in the belly, most commonly the gallbladder or the spleen. 

•  Fever over 101°F (38°C): People with SCD have a hard time fighting off infections. A fever can mean a bad bacterial infection.

• Sudden weakness or loss of feeling in the face, arm, or leg: These could be signs of a stroke, which needs emergency medical attention. 

• Severe groin or hip pain, or inability to walk or put weight on your leg: SCD can decrease blood supply and damage bones (commonly the hip) — called “avascular necrosis.” 

•  Prolonged erection: An erection of the penis that lasts more than 4 hours, called priapism, is a medical emergency.

• Very pale skin or inner eyelids: This can indicate severe anemia that requires a blood transfusion.

If you’re having any of these symptoms, it’s important to get medical attention right away.  

How is sickle cell crisis treated in the emergency room?

Treatment for an acute painful episode of SCD involves intravenous (IV) pain medications, usually opioids. Your healthcare team may consider other treatments such as IV ketamine and local nerve anesthesia if your symptoms don’t improve with standard treatments. For initial pain relief in kids, providers may recommend intranasal (in the nose) medication, such as fentanyl. Hydration (both oral and IV) can help with dehydration and circulation. 

Oxygen therapy and blood transfusions aren’t common. But they may help if your oxygen levels are low or you are very anemic.

If you have a pain management plan that you created with your doctor, take it with you to the ER. It’ll help because providers in the hospital often base opioid dosing on what you’re taking at home and things that have helped you in the past. If you aren’t getting relief from IV medications in the ER, your doctor may keep you in the hospital for additional pain control.  

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Written by:

Tiffany Chioma Anaebere, MD

Tiffany Chioma Anaebere, MD, is a medical writer and a practicing emergency medicine physician and educator in Northern California. Dr. Anaebere also researches and develops novel curriculum for physician learners.

Edited by:

Mandy Armitage, MD

Mandy Armitage, MD, has combined her interests in clinical medicine with her passion for education and content development for many years. She served as medical director for the health technology companies HealthLoop (now Get Well) and Doximity.

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