Dealing with Adrenal Crises When You Have Adrenoleukodystrophy

Dealing with Adrenal Crises When You Have Adrenoleukodystrophy

An adrenal crisis, also known as acute adrenal insufficiency or Addisonian crisis, is potentially life-threatening event that can afflict people with a type of adrenoleukodystrophy known as Addison’s disease. These patients are not able to produce sufficient cortisol hormone from their adrenal glands.

An adrenal crisis is characterized by a variety of symptoms, including fatigue, headache, dehydration, high fever, chills, rapid heart and/or respiratory rate — and the possibility of shock or coma — and requires immediate medical attention.

Here are a few pointers for dealing with adrenal crises, and suggested steps you can take in the event of an attack.

Keep stress levels as low as possible

Stress is one of the major triggers of adrenal crises. It is important that you minimize stress as much as possible. Engage in activities that interest you, and take frequent breaks from work. Socializing, meditation, physical activity, and a positive outlook can help in managing stress.

If you are taking prednisone, talk with your doctor about possibly increasing your usual dose of this oral corticosteroid in times of stress.

Make sure you are well-hydrated

Dehydration can also trigger an adrenal crisis, so ensure adequate hydration at all times. You may want to speak with your doctor about the ideal concentration of daily salt and glucose necessary for you, as requirements can vary between individuals.

Learn about hydrocortisone therapy

An adrenal crisis requires immediate medical attention. If you know that you or someone else is at risk of an adrenal crisis, it’s best to prepare to tackle it in advance before rushing to the hospital.

Parents and caregivers of patients with Addison’s disease should familiarize themselves with giving hydrocortisone injections, which is usually the first-line treatment for adrenal crises. If you are the patient, you should know how to give yourself an emergency injection.

Your doctor may suggest an emergency medication kit that should always be within reach. The kit should contain two vials of hydrocortisone, along with the required syringes and needles, and an instruction booklet on how to give an emergency injection. Make sure such a kit is also available at school if you have a child with Addison’s disease.

Ask for the proper hydrocortisone dose

Typically during an adrenal crisis, 100 mg of hydrocortisone is given immediately as an injection into the bloodstream or a muscle, followed by 200 mg every 24 hours (or 50 mg every six hours).

A good idea for anyone at risk of an adrenal crisis is to ask their doctor for the appropriate dose of hydrocortisone that would need to be administered.

Have isotonic saline solution handy

Rehydration with a saline solution must be done immediately when a person has an adrenal crisis. Usually, 1 liter of isotonic saline solution needs to be given intravenously into the bloodstream within the first hour of the attack, followed by four to six liters over the next 24 hours. Check with your doctor for the recommended amount of saline, as it depends on the patient’s kidney function.

Request an adrenal insufficiency action plan

Parents and caregivers of people with Addison’s disease should request an adrenal insufficiency action plan (AIAP) from their doctor. An AIAP contains information about the patient, contact details for the endocrinologist, and a flowchart of steps to be taken in case of an adrenal crisis. It also details the age-relevant dose of hydrocortisone required, and tells you when you should be calling an emergency response team.

A copy of the AIAP should be kept with the patient at all times, as it is a ready reference for emergency responders.

Wear a medical alert ID at all times

If you have been diagnosed with adrenal insufficiency, wear a medical alert identification at all times so that first responders or emergency teams know you need hydrocortisone therapy. Medical IDs have information about your condition engraved on them. They are also globally recognized, so they can be useful if you are traveling.

Alternatively, you can also carry an emergency steroid wallet (ESW) card. An ESW is a credit card-sized card that has been endorsed by patient support groups. It contains information on how much hydrocortisone and saline should be injected, which can be useful for emergency responders.

Consult an endocrinologist

In most cases following an adrenal crisis, you may need to get admitted to a hospital for observation and follow-up. Consult with an endocrinologist for information on further courses of action, including appropriate rehydration measures that you can take at home. The endocrinologist will be able to tell if further doses of hydrocortisone are required, and look into the underlying cause.

 

Last updated: Nov. 12, 2019

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Adrenoleukodystrophy News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.

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Özge has a MSc. in Molecular Genetics from the University of Leicester and a PhD in Developmental Biology from Queen Mary University of London. She worked as a Post-doctoral Research Associate at the University of Leicester for six years in the field of Behavioural Neurology before moving into science communication. She worked as the Research Communication Officer at a London based charity for almost two years.