Estimated reading time: 6 minutes
Medically reviewed and verified by Kate Burke, MD, MHA
Parents of children with congenital adrenal hyperplasia have a lot to deal with! You need to stay on top of your child's appointments, make sure they get the education and support they need, prepare them for adulthood and their changing responsibilities, all while living a full and engaged life. Of everything you need to be on top of, recognizing and managing an adrenal crisis should be at the top of your priority list. An adrenal crisis is a potentially life-threatening symptom of CAH. PatientsLikeMe is here to make sure you have the tools you need to navigate this symptom. What is an adrenal crisis? How does it happen and how does it affect the body? What can you do to manage and prevent it? Here is a complete guide.
CAH and Adrenal Crisis
CAH is a genetic condition that affects the adrenal glands’ ability to produce hormones like cortisol and aldosterone. Cortisol helps the body respond to stress, and aldosterone helps regulate salt and water balance. In children with CAH, the body’s inability to make enough cortisol or aldosterone increases their risk of an adrenal crisis. Those with the more severe forms of CAH can experience something called "salt wasting," where their body loses too much sodium. This is especially dangerous because it can cause dehydration and lead to a crisis very quickly. The symptoms of salt-wasting include poor feeding, vomiting, weight loss, and low blood pressure. If untreated, it can lead to an adrenal crisis.
What is an Adrenal Crisis?
An adrenal crisis is a serious, life-threatening condition that happens when the body doesn’t have enough cortisol. Cortisol helps manage stress, maintain blood sugar, and keep blood pressure at healthy levels. When cortisol levels drop too low, the body can’t function properly, which can lead to severe complications. For families dealing with congenital adrenal hyperplasia (CAH), understanding adrenal crises is especially important, as these events are more likely in people with this condition.
How Does an Adrenal Crisis Happen?
For people with CAH, an adrenal crisis is often triggered by stress on the body, such as an illness, fever, surgery, or infection. These stressors need more cortisol than the body can produce. A crisis can also happen if a person suddenly stops taking their prescribed medication for CAH. In children with CAH, adrenal crises can happen if they miss doses of their medications or if they experience a sudden health issue like a fever or dehydration. Even something as common as vomiting or diarrhea can lead to a crisis if it causes them to lose fluids and salt. This can make it harder for their body to regulate itself.
Acne? CAH Symptom. Gaining weight? Side effect. It can be tough to tell apart symptoms of congenital adrenal hyperplasia (CAH) and treatment side effects of steroids apart.
Watch now: Is it a symptom or a side effect?
Your body may feel out of whack when living with CAH. Too little cortisol can make you feel tired or unwell and high androgen levels can cause early puberty, premature growth, and more. Treating CAH with high-dose steroids can cause side effects like weight gain, diabetes, and bone density issues. See why it’s important to talk to your doctor about what you’re experiencing and your treatment plan.
Questions about CAH symptoms and/or side effects from high-dose steroids? Visit: WhatTheCAH to learn more.
Sponsored by Neurocrine Biosciences, Inc.
How Does an Adrenal Crisis Affect the Body?
When an adrenal crisis happens, the body starts to shut down due to the lack of cortisol. Some common symptoms are:
- Low blood pressure
- Low blood sugar
- Dehydration
- Fatigue and weakness
- Nausea and vomiting
Because these symptoms can escalate quickly, adrenal crises are a medical emergency. If untreated, the situation can become life-threatening, with risks including shock and even death.
Management
An adrenal crisis needs immediate medical attention. If your child is experiencing signs of a crisis, it is important to act fast! Here are some steps you can take.
- Hydrocortisone injection: One of the first treatments, for example, solu cortef, is giving the person hydrocortisone, either through an injection into a muscle or directly into a vein. Hydrocortisone replaces the missing cortisol and helps stabilize the body’s systems.
- Fluids: If the person is dehydrated, they may need fluids with added salts through an IV to help their body rehydrate and maintain healthy salt levels. This also improves blood pressure and prevents more dehydration.
- Blood sugar: If low blood sugar is a problem, doctors may give the person a sugar solution through the IV to help bring their levels back to normal.
- Electrolytes: During a crisis, the body may lose important minerals like sodium and potassium. Managing these levels is important to avoid heart problems or other complications.
The key to preventing long-term damage from an adrenal crisis is quick recognition and fast treatment. Once stable, they will need monitoring and possibly more treatments depending on the cause of the crisis.
Prevention
If you have a child with CAH, it’s important to be prepared for the possibility of an adrenal crisis. The first step is to take medications as prescribed. This is especially true for hydrocortisone and other hormone replacements that help their body function normally. Missing doses can increase the risk of a crisis. During times of illness or stress, your child may need more hydrocortisone than usual. Talk to your child’s doctor about “sick day rules” and how to adjust their medication when they are ill. Keep an eye out for symptoms like vomiting, dehydration, weakness, or confusion. If these symptoms show up, get medical help right away.
Many families keep an emergency injection of hydrocortisone at home to use if their child can’t take oral medication or if symptoms of a crisis start to appear. If you have this kit, make sure you and any caregivers know how to use it. Make sure you know where the nearest hospital is and have emergency contact information readily available. If your child starts showing signs of an adrenal crisis, call 911 right away.
An adrenal crisis is a serious complication of CAH. With the right knowledge and preparation, you can help reduce the risks and ensure your child gets the treatment they need if a crisis happens. By following your doctor’s advice, understanding the symptoms, and having a plan in place, you can make sure they live a healthy, active life.
Nearly 1 million people have joined PatientsLikeMe. You can track your health, monitor treatment efficacy, and talk to people experiencing the same thing as you. Creating an account is free! Join today.