How to Treat a Sickle Cell Disease Crisis

Treatment for Sickle Cell Disease Crisis: What You Need to Know

Treatment for Sickle Cell Disease Crisis: What You Need to Know
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Sickle cell disease reshapes healthy round blood cells into a “sickled” C-shape. As these sticky cells clump up, they can block blood from getting to your tissues, so it can’t deliver needed oxygen. When this happens, it’s called a sickle cell crisis.

The most common symptom of a sickle cell crisis is severe pain, but it can also cause complications throughout your body.

Below, learn what causes sickle cell crises, where you can get treatment, and how to take care of yourself as you recover.

What Is a Sickle Cell Crisis?

In a sickle cell crisis, blood flow blockage from stiff, sticky red blood cells causes severe pain.

“Think of the blood vessels like a city highway,” says Elizabeth Margolskee, MD, MPH, a clinical pathologist and an associate professor of clinical pathology and laboratory medicine at the Perelman School of Medicine at the University of Pennsylvania in Philadelphia.
“In normal conditions, red blood cells flow smoothly through arteries and veins like small cars on the interstate,” says Dr. Margolskee. “Normal red blood cells are round, compact, and flexible. They circulate easily and deliver oxygen. During a sickle cell crisis, it’s almost as if the red blood cells transform into long, rigid trucks that can’t get around so easily and stop the flow of traffic.”

Each time this happens — especially in the bones — it feels very painful, says Sanjay Shah, MD, a hematologist, the section chief of hematology, and the director of the sickle cell and hemoglobinopathy program at Phoenix Children’s in Arizona. A sickle cell crisis can be triggered by factors like dehydration, infections, and stress.

You can prevent some crises by avoiding these triggers.

But once you’re in pain, you have options for treatment.
“The majority of patients with sickle cell disease manage their painful crises at home,” says Jennie Law, MD, a hematologist-oncologist and the associate section chief for classical hematology and sickle cell disease at the University of Maryland Greenebaum Comprehensive Cancer Center in Baltimore. But if your pain keeps getting worse, you can get a higher level of treatment at a medical facility.

Symptoms of a Sickle Cell Crisis

The most common symptom of a sickle cell crisis is severe pain.

During a crisis, you can feel pain anywhere, but it’s usually strongest in the limbs, lower back, chest, or stomach. This pain can go on for hours to weeks.

Other symptoms of a sickle cell crisis include:

  • Fatigue
  • Headaches
  • Yellow skin and eyes (jaundice)
  • Weakness

Some symptoms mean you need to get medical help right away, says Margolskee. “Keep an eye out for signs that the crisis is worsening, or not responding to home treatment.”

Call your healthcare provider or go to the emergency room if you have these symptoms:

  • Pain that’s not getting better with treatment at home.
  • Trouble breathing
  • Irregular heartbeat
  • Dizziness
  • Fever over 101.3 degrees F
  • Chest pain
  • Sudden weakness or numbness on one side of your body
  • Confusion
  • Speech or vision changes
  • An erection lasting four hours or more (priapism)

Where to Seek Treatment

Before a pain crisis, make a plan for where you want to get treatment. Your provider can tell you the best place to go in your area.

For the best pain crisis care, try to find a sickle-cell-specific acute care facility. This could include outpatient medical centers like these:

  • Day Hospitals You can get care during the day, then return home.
  • Infusion Centers These facilities have staff specially trained to care for sickle cell crises.

  • Comprehensive Sickle Cell Centers These facilities are solely for people with sickle cell disease.

Healthcare providers at these treatment centers may better understand what you’re going through and how to help you.

If you don’t have a facility like this near you, you can go to an emergency room.

If you have to visit an emergency room for a sickle cell crisis, bring a copy of your pain management plan. This detailed document provided by your sickle cell care team can help ER staff understand what you need more quickly.

Treatment for a Sickle Cell Crisis

Providers treat a sickle cell crisis with pain medication, hydration, and sometimes blood transfusions.

“Early pain management is the most critical step in helping prevent hospitalization,” says Dr. Shah. If you are admitted to the hospital, "pain is best managed with around-the-clock pain meds, adequate hydration, and supportive care,” says Shah.

For mild pain, your provider may tell you to take over-the-counter medications like acetaminophen (Tylenol) or ibuprofen (Advil). If you have more severe pain, you may need to stay in the hospital for a few days.

As an inpatient, you can get fluids through an IV and take stronger narcotic pain medications like oxycodone or morphine.

“In severe situations, blood transfusions to replace sickle cells with non-sickling red blood cells can be considered,” says Margolskee.

Self-Care Measures That Can Help

Besides medical treatment, you can ease a sickle cell crisis with self-care.

Drink fluids. “Staying well hydrated is number one,” says Shah. Drinking plenty of water helps your blood flow through your veins and arteries more easily.

Get plenty of rest. Avoid overexertion, says Dr. Law. Pushing yourself too hard too soon can make a crisis worse.

Apply heat. You can use heating pads or warm baths to ease pain in a sickle cell crisis, says Margolskee. The heat widens your blood vessels, allowing more blood flow.

Try massage. Gentle massage can be a helpful self-care measure for a pain crisis, says Law. Massage can boost your circulation and relax your muscles to ease pain.

Use mindfulness. Relaxation and meditation can reduce crisis pain, says Margolskee. Even short periods of mindfulness can distract from discomfort.

To practice mindfulness, try paying attention to your body and thoughts, or do deep breathing exercises.

After the Crisis

A sickle cell crisis typically resolves within two to four days, but recovery can look very different from person to person, says Shah. Your provider can give you a personalized recommendation on when you can return to work and school. For example, if your job is very physical, you may need to wait longer to go back.

Sickle cell crises cause pain, but they can also feel emotionally stressful.

“It is important to recognize that sickle cell can be very disruptive to a patient’s life, hindering their ability to maintain a job, attend school, [and] establish relationships,” says Law.

“The fact that so many patients with sickle cell are able to still do these things is a testament to their resilience and fortitude in the face of this chronic, life-altering illness,” Law adds.

After your pain crisis eases, you can focus on your mental health by speaking to a counselor, loved one, or support group. These people in your life can help you create and use coping strategies as you recover.

Resources We Trust

EDITORIAL SOURCES
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Tingting Tan, MD, PhD

Medical Reviewer

Tingting Tan, MD, PhD, is a medical oncologist at City of Hope National Medical Center.

Dr. Tan's research has been published in multiple medical and scientific journals, including...

Abby McCoy, RN

Author

Abby McCoy is an experienced registered nurse who has worked with adults and pediatric patients encompassing trauma, orthopedics, home care, transplant, and case management. She is...