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An adrenal crisis is a life-threatening emergency that requires immediate medical care. This serious complication of congenital adrenal hyperplasia (CAH) can affect people of any age, including newborns just days old. It most often occurs in people with classic CAH, particularly the salt-wasting type. Without quick treatment, an adrenal crisis can be fatal.
Several factors can increase the risk of an adrenal crisis, and we’ll cover the most common ones in this article. If you or someone you care for is living with CAH, it’s important to understand what can trigger a crisis and how to spot the early warning signs. Being informed and prepared can help you take fast action — and may even help prevent a crisis from happening.
An adrenal crisis is a sudden, life-threatening condition that happens when the body doesn’t make enough cortisol. In the salt-wasting form of CAH, the body also doesn’t make enough aldosterone — a hormone that helps the body hold onto salt and water. People with CAH are more likely to experience an adrenal crisis because the condition affects the adrenal glands, which sit on top of the kidneys and help make hormones like cortisol, aldosterone, and androgens.
Because an adrenal crisis can come on quickly and become life-threatening, it’s critical to recognize the signs and symptoms early and get emergency medical care.
If you notice any of the symptoms below, seek medical help right away.
Cortisol and aldosterone are hormones that help keep blood pressure stable. When levels are too low, the body struggles to regulate blood pressure. During an adrenal crisis, blood pressure can drop quickly and dangerously.
Extreme fatigue is a common symptom of an adrenal crisis. This isn’t just everyday tiredness. It may feel like you’re too weak to stand, move, or even get out of bed.
Nausea and vomiting are often signs of an adrenal crisis. These symptoms can occur for many reasons, but if you live with classic CAH and aren’t sure what’s causing them, talk to a doctor right away.
Both back pain and abdominal (belly) pain may occur during an adrenal crisis. These symptoms can be mistaken for something else, so it’s important to know they can be part of a crisis.
Some people in an adrenal crisis become confused or delirious — or even lose consciousness. Any sudden changes in alertness or behavior should be treated as a medical emergency.
People in an adrenal crisis may have hypoglycemia (low blood sugar levels), which is usually confirmed with a blood test. Symptoms can include shakiness, sweating, or feeling faint.
Blood tests during an adrenal crisis often show very low levels of salt (sodium), a condition called hyponatremia. This can lead to confusion, headaches, or seizures if not treated.
Potassium levels may be too high during an adrenal crisis, a condition called hyperkalemia. This can be dangerous and affect heart rhythm, so it’s closely monitored during testing.
An adrenal crisis can be triggered by many different factors — even if you’re following your treatment plan closely. Knowing what can trigger a crisis may help you prevent one. However, studies suggest that around 10 percent of adrenal crises happen without a clear cause.
Cortisol plays a key role in how the body responds to stress. If cortisol levels aren’t well managed, both physical and emotional stress can trigger a crisis.
Surgery puts major stress on the body. The procedure itself — and especially anesthesia (the medication used to put you to sleep) — can raise your risk of an adrenal crisis. If you have CAH, your body may not produce enough cortisol to handle this stress, which can lead to a dangerous drop in blood pressure.
Keeping blood pressure stable during surgery is vital to protect your organs. Even dental procedures can trigger a crisis for the same reasons: physical stress, anesthesia, and challenges with blood pressure control.
Before any surgery, talk to your doctor about your CAH. Your care team can take steps to prevent an adrenal crisis, such as adjusting your medications before and after the procedure.
Stress — both physical and emotional — is a common trigger for adrenal crisis.
Physical stressors can include the following:
Emotional or mental stress — such as the death of a loved one, major life changes, or high anxiety — can also trigger an adrenal crisis. In fact, emotional stress has been linked to about 30 percent of adrenal crises in some studies.
Learning to manage stress may help reduce the risk of a crisis. If you’re feeling overwhelmed, talk to your doctor. They may recommend a counselor or therapist who can support your emotional health and help you build healthy coping strategies.
Infections — even common ones — can trigger an adrenal crisis. Fevers, which often accompany infections, are linked to about 17 percent to 24 percent of adrenal crises. During an infection, the body may struggle to produce and regulate cortisol, similar to what happens during surgery or physical stress.
Other illnesses can also raise the risk of a crisis. For example, stomach bugs like gastroenteritis have been shown to trigger adrenal crises in 35 percent to 45 percent of cases. Vomiting, diarrhea, and dehydration from these illnesses can make it harder for the body to stay in balance, especially for people with salt-wasting CAH.
For people living with CAH, daily glucocorticoid therapy — such as hydrocortisone (a corticosteroid that mimics cortisol) — helps to manage hormone levels and prevent adrenal crisis. This medicine is usually taken for life. Missing doses or stopping these medications can trigger an adrenal crisis.
Other medications may also increase the risk of an adrenal crisis. These include:
Always talk with your doctor or endocrinologist (a hormone specialist) before starting or stopping any medication. Make sure they know you have CAH so they can help you stay safe.
Although these are some of the most common triggers, adrenal crises can sometimes happen without a clear cause. That’s why it’s important to know the warning signs and have a plan in place.
Being prepared for adrenal crisis triggers is an important part of managing CAH. One common strategy is stress dosing, which involves taking extra hydrocortisone before or during a stressful event. For example, if you’re going through a difficult situation, like the death of a loved one, ask your doctor if you should temporarily increase your dose.
During illness, recommended daily doses of hydrocortisone may need to change, and the right amount can differ for adults and children. Follow your healthcare provider’s instructions carefully to reduce the risk of a crisis.
It’s also important to have an emergency kit that includes:
Wearing a medical ID bracelet or necklace, or carrying a medical alert card, can help others recognize your condition in an emergency. Be sure it includes emergency contact information for a trusted friend or family member.
As always, it’s best to discuss any questions and concerns about CAH or adrenal crisis with your doctor.
The main treatment for an adrenal crisis is fast-acting hydrocortisone. If a crisis is suspected, your doctor may recommend giving an emergency dose of hydrocortisone at home before going to the emergency room. This is often done as an intramuscular (into the muscle) injection.
At the hospital, the care team will examine you and may give additional hydrocortisone through an intravenous (IV) line. This is a small tube placed in a vein. They’ll also treat related symptoms, like low blood pressure, low blood sugar, or dehydration, to help stabilize your condition.
On CAHteam, people share their experiences with CAH, get advice, and find support from others who understand.
Have you or your loved one with CAH experienced an adrenal crisis? Let others know in the comments below.
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