Pain is one of the most common symptoms of sickle cell disease (SCD). It can appear suddenly as an intense pain crisis or persist over time.

Pain that occurs on more days than not for six months or longer is considered chronic pain. The experience can vary widely from person to person.

Pain is also one of the most misunderstood symptoms. Many people with SCD report having their pain doubted, downplayed, or treated as a sign of drug-seeking behavior, and research supports that this happens. If that has been your experience, you are not imagining it.

Understanding why SCD causes pain can make it easier to recognize what’s happening in your body and discuss treatment options with your healthcare team. This article explains what causes sickle cell pain, how acute pain crises differ from chronic pain, ways to manage and prevent pain episodes, what to do when your pain isn’t taken seriously, and signs that may require immediate medical attention.

🗳️ What type of pain do you experience with sickle cell disease?

Sudden pain crises

Chronic pain

Both sudden and chronic pain

Pain not typical of my sickle cell disease

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How Sickle Cell Disease Causes Pain

SCD affects hemoglobin, the protein in red blood cells that carries oxygen throughout the body. People with SCD produce an abnormal form of hemoglobin called hemoglobin S (HbS). Red blood cells containing HbS can become rigid and sickle-shaped, making it harder for them to move through small blood vessels.

These cells can slow or block blood flow. SCD also causes inflammation that can make blood cells stick more easily to the walls of blood vessels, contributing to further blockage and tissue injury. The combination of blocked blood flow, reduced oxygen, and inflammation helps explain why pain is such a common and complex part of living with SCD.

Acute Pain or Pain Crises

When sickled red blood cells cause a blockage, tissues may not get enough oxygen. The reduced blood and oxygen supply can cause sudden, severe pain known as a vaso-occlusive crisis (VOC). Pain can occur throughout the body but is common in the chest, abdomen, lower back, arms, and legs.

A pain crisis can last for several hours or days and may affect several areas of the body at once. If your pain changes significantly from what you usually experience, tell your healthcare team so they can check for other causes.

There’s no single blood test or imaging test that can confirm or rule out a vaso-occlusive pain crisis. Tests may still be used to look for complications or other causes of pain. Because there’s no test that measures how much a crisis hurts, your description of your pain is a key part of assessing how severe it is.

Chronic Pain

Chronic sickle cell pain is pain that occurs on more days than not for six months or longer and may feel different from the sudden pain of a VOC. It may be related to long-term complications of SCD, such as damage to bones or organs, avascular necrosis, nerve-related pain, or changes in how the nervous system processes pain.

Both acute and chronic pain can interfere with daily activities and quality of life, but each person’s experience is different and may change over time.

Managing Pain During a Crisis

American Society of Hematology (ASH) guidelines recommend tailoring pain medication to the individual based on what has worked before and what you already take at home. A written individualized pain plan can record the medications and doses that have worked for you and be added to your electronic medical record to guide treatment during future crises.

Mild pain may be managed at home with self-care strategies and over-the-counter (OTC) medications. Drinking fluids to stay well hydrated and applying a heating pad or warm compress to the painful area may provide some relief.

OTC pain medications, such as acetaminophen (Tylenol) or ibuprofen (Advil, Motrin), may help manage mild to moderate pain. Take prescription medications according to your healthcare provider’s instructions.

If your pain is severe or does not improve with your home treatment plan, you may need care at a hospital or another acute care facility. Guidelines recommend that pain be assessed and pain medicine be given within one hour of arrival.

Your pain should then be checked again every 30 to 60 minutes so treatment can be adjusted as needed. If opioids are needed, the dose should be based on your usual treatment plan and what has worked for you before.

When Your Pain Isn’t Believed

Not being believed is a documented problem in sickle cell care. Knowing that this pattern occurs may make it easier to recognize in the moment and plan ahead.

Why It Happens

Several factors can overlap. First, there is no lab or imaging test that can measure how much pain you’re experiencing, so clinicians rely heavily on your description of your pain along with their clinical assessment.

Second, more than 90 percent of people with SCD in the U.S. are non-Hispanic Black or African American, according to the Office of Minority Health, and research has identified racism and implicit bias as important issues affecting sickle cell care. Third, severe crises are often treated with opioids, which carry their own stigma.

For example, in one survey of 66 internal medicine and emergency medicine residents at a single academic center, 38 percent believed that patients with SCD overreport pain and are “drug-seeking.”

According to the National Academies, racism and discrimination in healthcare have led some adults with SCD to avoid seeking care until they feel they have no other option.

What Can Help

These steps won’t solve bias in the healthcare system, but they may help you receive timely care:

• Ask your hematologist for a written, individualized pain plan. It should list your usual medications and doses, be added to your medical record, and be available on your phone or in print.
• Find out in advance whether your area has a sickle cell day hospital or infusion center.
• At triage, state that you’re having a vaso-occlusive crisis, rate your pain, and ask when you can expect medication and reassessment.
• Bring someone who can help ask questions and track time. If care is delayed, ask for the charge nurse or patient advocate.
• Afterward, record the dates, times, and names of the people involved, and share what happened with your hematologist or sickle cell center.
• Repeatedly having to prove your pain is real can be exhausting. Seek support from a therapist, peer community, or trusted member of your care team.

Preventing Future Pain Episodes

Recognizing symptoms of a pain crisis can help you respond when one occurs, and taking steps to prevent future episodes is vital for managing SCD. Common triggers can include dehydration, extreme temperature changes, infections, stress, and high altitudes.

Hydroxyurea can help prevent pain crises by changing how SCD affects the body. Taking hydroxyurea regularly can reduce pain crises, acute chest syndrome, hospitalizations, and the need for blood transfusions.

Other disease-modifying treatments may also help reduce vaso-occlusive pain episodes in some people with SCD. L-glutamine (Endari) is approved for people ages 5 and older, and crizanlizumab-tmca (Adakveo) is approved for people ages 16 and older. Your healthcare team can help determine which treatment options are appropriate for you.

When Pain Is an Emergency

Not every pain crisis requires emergency care. If your pain improves with your usual home treatment, follow your care plan. Seek immediate medical attention if severe pain doesn’t improve or you develop any of the following emergency symptoms:

• Difficulty breathing
• New chest pain
• Fever of 101.3 degrees Fahrenheit (38.5 degrees Celsius) or higher
• Sudden weakness or numbness or trouble speaking
• Priapism (a painful erection lasting four hours or more)
• Fainting or other rapidly worsening symptoms

Serious complications can develop quickly, so don’t delay care. Plan ahead by keeping your pain plan accessible and knowing which facility handles sickle cell emergencies best. If a past visit was dismissive, bring someone you trust when possible.