Sickle Cell Crisis

What is a sickle cell crisis?

A sickle cell crisis is a painful episode that can occur if you have sickle cell disease. It happens when sickled cells (crescent-shaped red blood cells) get stuck in a blood vessel and slow or stop blood flow. Red blood cells carry oxygen throughout your body. A sickle cell crisis keeps oxygen from reaching your tissues and organs, resulting in pain and other complications.

You might be able to treat a sickle cell crisis at home. But if your symptoms become severe, you may need to stay in the hospital for a few days.

Types of sickle cell crises

The four types of sickle cell crises include:

1. Vaso-occlusive crisis: The most common type of sickle cell crisis, it happens when sickled cells block your capillaries or other small blood vessels, reducing blood flow to your tissues and organs. Other types of sickle cell crises affect specific areas of your body.
2. Aplastic crisis: When your bone marrow stops producing red blood cells.
3. Hyperhemolytic crisis: When your hemoglobin levels drop quickly. It can happen after a blood transfusion, a common treatment for sickle cell disease.
4. Splenic sequestration: When sickled cells get stuck in your spleen and cause painful swelling.

What are the symptoms of a sickle cell crisis?

Sickle cell crisis symptoms can vary depending on the areas of your body affected. They may include:

• Dizziness.
• Fatigue.
• Headaches.
• Jaundice.
• Pain.
• Shortness of breath.
• Weakness.

With a sickle cell crisis, pain might feel dull, intense, throbbing or stabbing. It can last a few hours or, in some cases, several days or weeks. Pain can occur anywhere in your body and one or more areas. During a sickle cell crisis, you might experience pain in your:

• Arms or hands.
• Chest.
• Legs or feet.
• Lower back.
• Stomach.

What causes a sickle cell crisis?

A sickle cell crisis happens when sickle-shaped red blood cells build up and block a blood vessel. But experts don’t always know what triggers this.

Possible causes of sickle cell crises are:

• Dehydration.
• High altitudes.
• Infections.
• Pregnancy.
• Smoking or vaping.
• Strenuous physical activity.
• Stress.
• Sudden, extreme temperature changes.
• Vitamin deficiencies.

What are the complications of a sickle cell crisis?

The severity of sickle cell crisis depends on the location of the blocked blood vessel. When vessels in your tissues or organs become blocked, it can lead to complications like:

• Acute chest syndrome.
• Blood clots.
• Chronic pain.
• Enlarged spleen and other spleen conditions.
• Leg ulcers.
• Liver disease.
• Lung conditions.
• Necrosis (bone or tissue death) due to lack of blood flow.
• Painful erections.
• Stroke.

How is a sickle cell crisis diagnosed?

If you have sickle cell disease, you likely already visit your healthcare provider every six to 12 months for routine blood tests. But if you’re in sickle cell crisis, you’ll need a full laboratory workup, including:

• Blood type test (in case you need a blood transfusion).
• CBC (complete blood count).
• CMP (comprehensive metabolic panel).
• Liver function tests.
• Reticulocyte count.

In addition, your healthcare provider will need to do a full physical examination and imaging tests, which might include:

• Doppler ultrasound.
• MRI.
• Ultrasound.

How is a sickle cell crisis treated?

If you’re in sickle cell crisis, you’ll need pain relievers to manage uncomfortable symptoms. For mild cases, you can do this at home with over-the-counter medications like acetaminophen (Tylenol^®) or ibuprofen (Advil^®).

In severe cases, you might need to stay a few days in the hospital, where healthcare providers can give you stronger pain medication and IV fluids.

Otherwise, sickle cell crisis treatment depends on the part(s) of your body affected and the severity of your symptoms. Healthcare providers may use medication, blood transfusions, bone marrow transplants or a combination of therapies.

Medications

The following are medications that healthcare providers use for long-term management of sickle cell crises:

• Crizanlizumab: A monoclonal antibody that can keep your red blood cells from sticking to the inside walls of your blood vessels.
• Hydroxyurea: A medication that makes your red blood cells larger and more bendable.
• L-glutamine: An amino acid that helps reduce red blood cell damage.
• Voxelotor: A medication that keeps your red blood cells from becoming sickled (crescent-shaped) and sticking together.

Transfusions

Healthcare providers may use blood transfusions to treat aplastic or hyperhemolytic sickle cell crises. This treatment can replenish your stores of healthy red blood cells and reduce your risk for sickle cell complications.

Transplants

A bone marrow or stem cell transplant can cure sickle cell disease in some cases. But it can take some time to find a matching donor. Bone marrow transplants can also cause graft vs. host disease (GvHD) and other complications. Because of this, healthcare providers rarely do bone marrow transplants when treating sickle cell crises. Instead, the main goals are treating your symptoms and getting you out of pain.

Can sickle cell crises be prevented?

You can’t always prevent sickle cell crises, but some things can help reduce your risk:

• Avoid smoking.
• Dress warmly in cold environments.
• Limit your alcohol intake.
• Stay hydrated.
• Take folic acid to boost red blood cell production.
• Wash your hands frequently to reduce your risk of infections.

How do I take care of myself?

If you or a loved one is in sickle cell crisis, doing these things might help ease symptoms:

• Apply heat. A hot bath or a heating pad can dilate (widen) your blood vessels and improve circulation. Warmth can also soothe aching muscles. (You should avoid ice packs. Cold temperatures can trigger a pain crisis, so it’s best to stay as warm as possible.)
• Drink lots of water. Staying hydrated keeps your red blood cells from sticking together so they can flow more freely through your vessels.
• Get a massage. Massage can ease pain by increasing circulation and relaxing your muscles.
• Practice mindfulness. Research shows that even brief mindfulness breaks can distract your brain and help ease pain related to sickle cell crisis.
• Take over-the-counter pain medications. This includes acetaminophen, ibuprofen or naproxen (Aleve^®).

When should I go to the ER for sickle cell crisis?

You should head to your nearest emergency room if you develop sudden intense pain that doesn’t get better with medication. Don’t wait. Starting treatment immediately can reduce your risk for long-term complications.

A note from Cleveland Clinic

If you have sickle cell disease, the looming possibility of a pain crisis can feel unsettling. These episodes can result in unpleasant symptoms and lifelong complications. If you’ve had sickle cell crises in the past, you know time is of the essence. If you have symptoms that don’t improve with over-the-counter pain relievers or your usual home remedies, it’s time to call your healthcare provider. They’re here to help.