Triggers for Sickle Cell Disease Crisis

“It is the worst pain you’ve ever experienced in your life,” says Jeffrey Woodson, who has lived with sickle cell disease for many years in St. Louis. “At its worst, it feels like every bone in your body is broken as you’re trying to move around — like a dagger slowly being driven into every muscle and bone in your body.”

• Acute chest syndrome: A pneumonia-like illness
• Blood clots: When sickled red blood cells stick together
• Chronic pain: Pain that lasts longer than six months

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• Spleen complications: Swollen spleen with left-sided abdominal pain

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• Leg ulcers: Open wounds from blocked blood flow

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• Liver disease: Can include liver damage and gallstones

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• Necrosis: Bone or tissue death
• Painful erections: Can last four hours or more

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• Stroke: Blood flow blockage that causes brain damage

Dehydration

Dehydration is Woodson’s number one trigger. “The more I can stay hydrated, the better chances I have of not setting off a pain episode,” he says.

People with sickle cell disease lose water more easily because their kidneys aren’t as efficient, says Crawford Strunk, MD, a hematologist at Cleveland Clinic and the codirector of the Sickle Cell Medical Neighborhood, a lifespan sickle cell comprehensive center in Ohio. “So especially in hot weather, they need to make sure that they're staying appropriately hydrated,” says Dr. Strunk.

Extreme Temperatures

“Without fail, being cold and wet is an automatic ‘I’m going to have a real bad episode’ situation,” says Woodson, who rarely goes swimming during the summers, even on extremely hot days.

“Even if the pool is heated, and it’s been hot for several days, I am extremely cautious because a small gust of wind as I’m coming out of the pool or walking into air-conditioning can become problematic,” says Woodson.

Sudden shifts in weather can also prompt a crisis, says Strunk. “The change in temperature is what really drives it.”

High Altitudes

Physical and Emotional Stress

Poor Sleep

Infections

Sickness can be a crisis trigger, says Strunk. “Colds, flus, anything that's going to stimulate your immune response is going to overdrive your sickle cell disease as well.”

Once you learn your sickle cell crisis triggers, you can take steps to avoid them and prevent a crisis.

Dress for the weather. Dress in layers so you can regulate your temperature easily during the day. You have to plan for changing weather, especially in spring and fall, when temperatures can fluctuate, says Strunk.

You may not always know your triggers, but over time, you'll start to recognize them, says Strunk.

“It wasn’t until I was having more frequent episodes that I started taking inventory and noticing what was triggering me,” says Woodson.

“Since there are levels to an episode, some actions can trigger small episodes in a specific area, and if caught early enough with pain meds, hydration, and heating pads, it won’t last long and won’t spread to other areas of my body,” says Woodson.

• Drink plenty of water.
• Massage painful areas.
• Take over-the-counter medications approved by your hematologist, like acetaminophen (Tylenol) or ibuprofen (Advil).
• Place a warm pack on the painful area or take a hot bath. (Never use cold packs or ice to relieve pain.)

• High fever
• Trouble breathing
• Pain that doesn’t improve with treatment at home
• Severe vomiting or diarrhea
• Headache or dizziness
• Pale skin or lips
• An erection lasting over two hours
• Seizures
• Confusion or slurred speech
• Abdominal swelling that happens quickly
• Vision changes
• One-sided weakness

• A sickle cell crisis happens when red blood cells get stiff and sticky and block blood flow to parts of your body, causing severe pain.
• Sickle cell crisis triggers can include dehydration, sudden temperature changes, high altitudes, stress, poor sleep, and illness.
• Once you learn your specific triggers, you can try to avoid them, but if you do have a crisis, you can manage it with approved medication, warm packs, and help from your provider.