1. Brief In-service on Adrenal Insufficiency Joseph Lewis, M.D., Medical Director, Honolulu EMS Diplomat, American Board of Emergency Medicine and Former Fellow American College of Emergency Medicine

2. Purpose and Goals of this In-service  The purpose:  I want to familiarize you with a medical disorder with a growing population on Oahu.  The Goals:  You should be aware of the anatomical location and function of the adrenal glands.  You should be familiar with the terms adrenal insufficiency, adrenal crisis and their clinical signs and symptoms.  You should understand the physiologic reasons for the clinical signs and symptoms.  You should know the treatment for adrenal insufficiency and / or adrenal crisis.  You should be know which patients get this disease.

3. 1. Anatomy and Function of the Adrenal glands  The adrenal glands are small hormone secreting glands which sit on top of each kidney.

4. Anatomy and Function of the Adrenal glands The adrenal glands make two different types of hormones and release them into your blood. I call the first group of hormones the “every day hormones” because they are made and released in small amounts every day to maintain normal levels of electrolytes like sodium and potassium, but in larger amounts when your sick, for example dehydrated from a long hike or you spent to much time at the beach without drinking water and got dehydrated. I call the second group “ emergency hormones “ which are released only for emergency medical or traumatic conditions, like you get hit by a car, asthma attack or go in to shock from infection or blood loss. Essential fact the emergency hormones need the every day hormone Cortisol to work properly. The “every day” hormones made by the adrenal glands are also called steroid type Hormones and they are divided into three subgroups types of steroid hormones; Glucocorticoids: exert a widespread effect on metabolism of carbohydrates and proteins. Mineralocorticoids: are essential to maintain sodium and fluid balance and Sex hormones are responsible for turning boys into men and girls into women, at least on the outside, but the adrenals are normally a only a small secondary source. The testes and ovaries being the main source. The “ emergency hormones “ made by the adrenal glands are called catecholamine's and they include: norepinephrine, epinephrine & dopamine.

5. Adrenal insufficiency and Adrenal crisis. In adrenal insufficiency the patient doesn’t make enough of the every day hormones to maintain their normal fluid and electrolyte levels. If they stop taking their medications, which are basically hormone supplements, they can become hyponatremic, hyperkalemic, dehydrated, hypotensive and even have seizures. If they stop their medication it happens slowly over days. But if they get physiologically stressed they can’t make extra hormone to deal with the stress, so they get symptoms like they stopped taking their supplements, but they get them quickly over hours. We call this an adrenal crisis. Unfortunately the emergency hormones made by the adrenal glands don’t work very well in the absence of the everyday hormone Cortisol, which is also known as hydrocortisone. This contributes to their hypotension and shock, as they don’t get a very good emergency hormone response to life threatening stress like trauma or shock of both medical and trauma origin. This situation adds to their adrenal crisis. So as you can see, they don't make enough for everyday living, they get stressed and can't pump out any extra hormone like healthy people do and they get real sick to the point of an emergency. Then their emergency hormones don't work so well because they need an everyday hormone cortisol. Luckily in such an emergency we can give them the essential things they need to recover plenty of salt water, otherwise known as normal saline and is Solu-Medrol. It works like Cortisol and is a life saving intervention.

6. The clinical signs and symptoms of adrenal crisis.  Severe vomiting/diarrhea  Dehydration  Hypotension  Sudden, severe pain in back, belly or legs  Loss of consciousness  Can be fatal

7. Who gets adrenal insufficiency?. People can be born with adrenal insufficiency. People can develop it due to taking drugs like prednisone every day for long periods of time. People with autoimmune diseases like for example: Lupus, can develop it. People with certain infections like HIV or TB can suffer damage to their adrenals from the infection or the medications. The adrenals can shut down rarely in trauma due to hypotension or can die from loss of blood supply. Patients with pituitary tumors or brain injury with Pituitary damage. Big list, best thing is to ask. If your called to the scene of a sick child and the parent tells you they have adrenal insufficiency and that their child needs Solu-Cortef or Solu-Medrol or they will die, the parent is correct.

8. From an Endocrinologist ”Gland Specialist”  “…In adrenal insufficiency, because of the inability to produce Glucocorticoids and often Mineralocorticoids from the adrenal glands, there is a risk of life-threatening hyponatremia, hyperkalemia, hypoglycemia, seizures and cardiovascular collapse, in particular at times of physiologic stress to the body, such as in injury or illness…”

9. From the mother of James, a child with adrenal insufficiency, she describes how something like a simple fever which healthy kids deal with easily can bring her son to the brink of death.  “… People without adrenal insufficiencies naturally produce up to ten times the normal amount of Cortisol during times of physical stress. If an unaffected person is unresponsive, goes into cardiac arrest or is vomiting, you can treat the shock, heart, or dehydration and help them. For James, however, immediate, appropriate emergency response is vital. I have watched James, as a fever quickly spiked, go from alert and playful to grayish-white and lethargic, in a matter of minutes. It is scary. I have seen how a stress dose of Cortef quickly brought him back to where I could then manage his illness with the “common” treatment of Motrin and fluids…”

10. Closing notes We don’t have a standing order for adrenal insufficiency or crisis, but we have cell phones and radios. So communicate with the base station physician for the order. Questions? jlewis@honolulu.gov