Key takeaways:

A sickle cell crisis is a recurrent pain episode that lasts 5 to 7 days in some people with sickle cell disease. It often requires hospitalization and in severe cases can last several weeks.
There are many different triggers for a sickle cell crisis. This can include cold weather, inflammation, dehydration, and low oxygen levels.
There are short- and long-term consequences of a sickle cell pain crisis. Talk with your healthcare provider about identifying triggers for your pain crises and how to avoid them.

Sickle cell crisis is a serious complication of sickle cell disease that leads to episodes of severe pain. These pain events can last for several days to weeks in some cases and may require a hospital stay.

A sickle cell crisis isn’t always preventable, but there are ways to lower the frequency. Understanding what triggers your sickle cell crisis and learning to avoid those factors can greatly improve your quality of life.

What is a sickle cell pain crisis?

A sickle cell pain crisis happens when blood flow to tissues and organs gets blocked. This occurs because the red blood cells have an abnormal shape in sickle cell disease. Rather than having a round form, they’re shaped like a crescent or “sickle” and can easily clog the blood vessels and prevent blood flow. The lack of blood flow keeps the tissues from receiving the oxygen and nutrients they need to function and survive. This triggers intense pain.

About 5 out of 100 people with sickle cell disease will have a pain crisis up to 10 times per year.

What triggers a sickle cell pain crisis?

There are often no known triggers for a sickle cell pain crisis. But, conditions that cause the blood vessels to narrow can trigger a sickle cell crisis. That’s because narrowed blood vessels make it more likely for the sickled blood cells to get stuck.

Let’s review seven common triggers for a sickle cell crisis and how to avoid them.

1. Extreme cold

In cold weather, the body works to maintain body temperature. To do this, signals that your body temperature has dropped cause blood vessels to narrow. This narrowing leaves less room for the sickled red blood cells to pass, increasing the risk that the cells get stuck.

The decreased blood flow prompts the body to pump more blood to provide oxygen and nutrients. This further increases the risk of vessels being clogged by sickled cells.

2. Pain

If a person with sickle cell disease has an injury to an area of their body, the resulting pain can trigger a crisis in that location. When an injury occurs, the body releases chemicals that can cause blood vessels to constrict.

3. Dehydration

Dehydration can also trigger a pain crisis. When you don’t have enough fluid in your body, your cells — including the red blood cells — can shrink. This increases the risk of sickling and makes it more likely for them to clump together.

The body will also respond to dehydration by constricting the blood vessels. This can further increase the risk of a pain crisis.

4. Inflammation

Inflammation is the body’s response to heal itself from injury. This can occur in the presence of trauma or infection. But in sickle cell disease it can happen as a result of the disease process. This inflammation results in an increase of white and red blood cells to the area and narrowing of blood vessels.

5. Infection

Infection can cause obstruction of blood flow in several ways. White cells and cell debris at the infection site can decrease the pathway for blood flow. Fever can also increase the possibility of dehydration. All of these factors can trigger a pain crisis due to infection.

6. Low oxygen

Low oxygen levels in the blood can lead to inflammation and trigger a pain crisis. Common reasons to experience decreased oxygen levels include:

High altitudes
Strenuous exercise
Severe anemia
Lung disease
Heart disease

If untreated, lower oxygen levels can lead to other complications. The risk of major organ damage is of particular concern.

7. Stress

Stress is another important trigger for a sickle cell pain crisis. It can trigger the blood vessels to narrow directly. But it can also cause changes in the body like inflammation and increased heart rate. This can further contribute to having a pain crisis.

How serious is a sickle cell crisis?

A sickle cell crisis is a serious complication of sickle cell disease. These painful episodes can last 5 to 7 days on average and often require hospitalization. In severe cases, a sickle cell crisis can last for several weeks. It’s common to have pain in the hands, feet, chest, and back. But any part of the body can be affected. A sickle cell crisis can also be life-threatening in severe cases.

Having repeated sickle cell crises can also cause long-term complications. Risks include:

Chronic kidney disease
Lung disease
Heart disease
Impotence
Infertility
Increased risk of infections
Chronic pain
Brain injury or stroke

The bottom line

Sickle cell pain crisis is a serious complication of sickle cell disease. Many times there’s no known cause for these pain episodes. But there are some triggers that may make a pain crisis more likely. It may be possible to decrease the number of pain crises by avoiding these triggers. Talk with your healthcare provider about ways to decrease your risk of pain crisis and what triggers you need to avoid.

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Written by:

Alicia Stevens, MD

Alicia Stevens, MD, is a board-certified physician in the fields of internal medicine and pediatrics with over 15 years of experience. She graduated from the University of Cincinnati College of Medicine and completed her training at Tulane University.

Edited by:

Karla Robinson, MD

Karla Robinson, MD, is a medical editor for GoodRx. She is a licensed, board-certified family physician with almost 20 years of experience in health through varied clinical, administrative, and educational roles.

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