An adrenal crisis is a life-threatening emergency that demands immediate medical attention. This serious complication of congenital adrenal hyperplasia (CAH) can happen at any age, including in newborns just days old.

The mortality rate for adrenal crisis is about 0.5 deaths per 100 patient-years, meaning that 0.5 percent of people with CAH may die from an adrenal crisis in one year. This means that, on average, for every 100 people with CAH being followed for one year, adrenal crisis causes 0.5 deaths. That number may seem small, but the risk is significant. Without fast treatment, an adrenal crisis can quickly become fatal.

Many factors can raise the risk of adrenal crisis, which we’ll cover in this article. It’s essential to understand the potential triggers and recognize the warning signs. Being prepared and aware can help you respond promptly in an emergency and, ideally, prevent a crisis from happening.

Adrenal Crisis

An adrenal crisis is a sudden, life-threatening condition that happens when the body doesn’t make enough cortisol, a hormone that helps regulate vital functions. People with CAH are more vulnerable to adrenal crisis because CAH affects the adrenal glands, which sit on top of the kidneys, and disrupts the body’s balance of hormones (such as cortisol, aldosterone, and androgen). Without proper treatment, these hormonal imbalances can lead to symptoms such as low blood pressure, weight loss, weakened immune response, low glucose (blood sugar), reduced energy, growth problems, and fertility issues.

Cortisol is crucial for maintaining blood pressure, and when levels are too low, the body struggles to regulate it. According to the National Library of Medicine, an adrenal crisis occurs when blood pressure drops too low, known as hypotension. This is defined as a systolic blood pressure (the top number in a blood pressure reading) lower than 100 millimeters of mercury (mm Hg) or a drop of at least 20 mm Hg from your usual systolic level.

To reduce the risk of an adrenal crisis, doctors may recommend “stress dosing” in certain situations. Stress dosing involves taking extra doses of cortisol medication, such as hydrocortisone, when the body is under stress from illness, injury, surgery, or emotional trauma. This helps the body handle the greater demand for cortisol during these times.

Without prompt treatment, an adrenal crisis can progress quickly and become life-threatening. It’s important to recognize the signs of an adrenal crisis so you can get immediate medical care. Symptoms include:

• Fatigue or weakness
• Digestive problems (nausea, vomiting, abdominal pain, or loss of appetite)
• Pain in the legs, arms, or back
• Dizziness, confusion, or loss of consciousness

If you notice any of these symptoms, seek medical attention right away.

Triggers for Adrenal Crisis

Many factors can trigger an adrenal crisis, even if you’re following your treatment plan. Understanding these triggers can help you take steps to prevent a crisis, but research suggests that up to 10 percent of adrenal crises occur without a known cause. Since cortisol plays a key role in the body’s response to stress, both physical and emotional stress can lead to a crisis if cortisol levels aren’t properly managed.

1. Surgery

Any surgery is a major stressor on the body, and the procedure itself, along with anesthesia (the medication that puts you to sleep), can increase this stress. If you have CAH, your body has difficulty producing enough cortisol to manage stress properly, making it harder to control blood pressure.

During surgery, maintaining stable blood pressure is crucial to help minimize blood loss and keep organs working properly. In the journal Cureus, researchers described a case of an adrenal crisis during a cesarean section. Doctors knew about the person’s condition and were able to manage the crisis with treatment.

In addition to surgery, anticoagulants (medications to help prevent blood clots during and after surgery) can increase the risk of an adrenal crisis by raising the risk of bleeding. Dental procedures can also trigger an adrenal crisis for similar reasons — stress on the body, anesthesia, and difficulty regulating blood pressure.

2. Stress

Physical stressors include:

• Injury, such as a broken bone or a wound that needs stitches
• Pregnancy and labor
• Dehydration
• Intense physical activity
• Exposure to extreme temperatures
• Digestive issues such as vomiting and diarrhea, which are more common in children

Mental, emotional, or psychological stress, such as the loss of a loved one, can also lead to an adrenal crisis. Emotional stress has been shown to cause an adrenal crisis in about 30 percent of cases.

3. Infection or Illness

Any type of infection — viral, fungal, parasitic, or bacterial — can lead to an adrenal crisis. Fevers, which occur with many infections, are known to trigger 17 percent to 24 percent of adrenal crises. During an infection, the body has a tough time regulating cortisol levels, similar to what happens during surgery and stressful events.

Illnesses can also cause an adrenal crisis. Gastroenteritis, a stomach illness, has been shown to trigger an adrenal crisis in 35 percent to 45 percent of cases.

4. Medications

For people living with CAH, glucocorticoid therapy, such as hydrocortisone (a corticosteroid that mimics cortisol), is a common treatment to help manage cortisol levels. This medicine is usually taken for life. Missing doses or stopping the medications can trigger an adrenal crisis.

Other medications may also lead to an adrenal crisis. These include certain cancer treatments, adrenal medications, and thyroid medications. It’s essential to discuss medication options with your health care provider or endocrinologist (doctor who specializes in hormone disorders) to make sure they’re aware that you have CAH.

Although these are some common triggers, it’s important to remember that an adrenal crisis can occur without any identifiable cause.

Stress Dosing and Emergency Preparation

Being prepared for potential triggers is crucial in managing CAH and preventing an adrenal crisis. Stress dosing is a common practice in which extra hydrocortisone is given before or during a triggering event. For example, if you’re experiencing significant emotional stress, such as the death of a loved one, it’s important to ask your doctor about adjusting your hydrocortisone dosage. During illnesses, the recommended doses may vary for adults and children, so follow your health care provider’s guidance to help prevent an adrenal crisis.

People with CAH should also have an emergency kit that includes extra medication and syringes. It’s also a good idea to wear a medical ID bracelet or necklace or carry a medical card that alerts others to your condition in case of an emergency. Make sure that the medical ID includes contact information for a family member or close friend.

As always, it’s best to discuss any questions and concerns about CAH or adrenal crisis with your endocrinology provider.

Talk With Others Who Understand

On CAHteam, the site for people with congenital adrenal hyperplasia and their loved ones, people come together to gain a new understanding of CAH and share their stories with others who understand life with the condition.

Have you had an adrenal crisis? Do you have any additional insights into the condition? Share your experience in the comments below.