Sickle Cell Crisis Triggers

Sickle cell disease is a disorder that affects your blood’s ability to carry oxygen throughout your body. It is the most common gene blood disorder in the United States, meaning that the gene for sickle cell disease is passed from parent to child.^1,2

Sickle cell disease gets its name from the shape some red blood cells take on during the disease. The cells go from disc-shaped and flexible to sickle-shaped and rigid.^1,2

One of the major effects of sickle cell disease is a sickle cell crisis. These can be intense and difficult to manage. Sickle cell crises are often brought on by a set of factors known as triggers.¹

What is a crisis, and what is a crisis trigger?

A sickle cell crisis happens when the sickled cells block blood flow somewhere in the body. Sickle cell crises are also known as acute pain crises or vaso-occlusive crises.^1,3

When blood flow is blocked, it causes intense pain. This pain can occur anywhere in the body. It is often described as intense, sharp, or throbbing. Common sites of pain are the lower back, joints, and extremities like the hands and feet.^1,3

Sickle cell crises often have a period of 1 to 2 days when initial symptoms start to get worse. Pain usually peaks on day 3, with a crisis lasting 6 to 7 days total. Adults with sickle cell who are hospitalized for a sickle cell crisis usually stay in the hospital for 9 to 11 days depending on the severity.³

Crisis triggers are environmental or biological factors that may start a sickle cell crisis. Triggers happen when chronic inflammation in the bloodstream activates the immune system, cell clumping, and cells that send pain signals.³

Common triggers and how to avoid them

While each person may be more or less sensitive to certain triggers, doctors agree that some factors can generally trigger a crisis. The most common sickle cell crisis triggers include:¹

• High altitudes
• Illness
• Temperature changes
• Dehydration
• Stress

A crisis may happen because of only 1 of these things, or more of them may be at play. But many times people do not know exactly what brings on a sickle cell crisis.¹

There are some habits and tactics you can incorporate into your life to avoid these triggers. These can include:^1,2

• Being cautious of traveling to high altitudes, like when hiking or when in a city high above sea level (planes should not be a problem, because they are pressurized to provide consistent oxygen)
• Avoiding extreme temperatures or large temperature changes by dressing appropriately for the weather and, for example, not taking a cold shower or swimming in cold water
• Drinking plenty of fluids, especially when the weather is hotter
• Finding ways to manage stress, like relaxation techniques, counseling, or meditation
• Avoiding alcohol, as it increases your risk of dehydration
• Avoiding smoking, as it can cause a serious lung condition called acute chest syndrome
• Keeping exercise light, and finding ways to be active without becoming seriously out of breath or exhausted

How to manage crisis triggers and crisis onset

If you think you have encountered a crisis trigger, you may or may not be able to manage your pain at home. There are some at-home steps that may help soothe your pain:^1,2

• Drink lots of fluids
• Take a nonsteroidal anti-inflammatory (NSAID) medicine, like ibuprofen (or acetaminophen if you have kidney problems)
• Use a heating pad

If you cannot manage the pain at home, you can go to a sickle cell day center, outpatient hospital, or emergency room. You can receive stronger pain medicine and IV fluids in one of these settings. You may need to be admitted to the hospital to get a pain crisis fully under control. Doctors will send you home once your pain is resolved.¹