1. ADRENAL INSUFFICIENCY Office of Emergency Medical Services & Trauma System

2. About This Presentation This presentation is intended for EMTs of all certification levels. We recommend that you review the slides from start to finish, however hyperlinks are provided in the table of contents for fast reference. Certain slides have additional information in the ‘notes’ section. This presentation was created by MA EMS for Children using materials and intellectual content provided by sources and individuals cited in the “Resources” section.

3. Table of Contents Objectives Anatomy & Physiology Epidemiology Presentation Management Medication Profiles Protocol Updates Resources

4. OBJECTIVES At the end of this program, EMTs will have increased awareness of: Epidemiology Anatomy & Physiology Pathophysiology Presentation Signs & Symptoms Treatment Family-centered care Effective medications

5. Adrenal Anatomy & Physiology The adrenals are endocrine organs that sit on top of each kidney

6. Each adrenal gland has two parts Adrenal Medulla (inner area) Secretes catecholamines which mediate stress response (help prepare a person for emergencies). Norepinephrine Epinephrine Dopamine Adrenal Anatomy & Physiology

7. Adrenal Cortex (outer area, encloses Adrenal Medulla) Secretes steroid hormones Glucocorticoids: exert a widespread effect on metabolism of carbohydrates and proteins Mineralocorticoids: are essential to maintain sodium and fluid balance sex hormones (secondary source) Adrenal Anatomy & Physiology

9. A person can survive without a functioning adrenal medulla A functioning adrenal cortex (or the steady availability of replacement hormone) is essential for survival Adrenal Anatomy & Physiology

10. The Essential Steroids Primary glucocorticoid: Cortisol (a.k.a. hydrocortisone) Primary mineralocorticoid: Aldosterone

11. Cortisol A glucocorticoid Frequently referred to as the ‘stress hormone’ Released in response to physiological or psychological stress Examples: exercise, illness, injury, starvation, extreme dehydration, electrolyte imbalance, emotional stress, surgery, etc.

12. Cortisol Critical actions on many physiologic systems, including: Maintains cardiovascular function Provides blood pressure regulation Enables carbohydrate metabolism acts on the liver to maintain normal glucose levels Immune function actions Reduces inflammation Suppresses immune system

13. Cortisol When cortisol is not produced or released by the adrenal glands, humans are unable to respond appropriately to physiologic stressors Rapid deterioration resulting in organ damage and shock/coma/death can occur, especially in children

14. Aldosterone A mineralocorticoid Regulates body fluid by influencing sodium balance The human body requires certain amounts of sodium and water in order to maintain normal metabolism of fats, carbohydrates and proteins

15. Water/sodium balance is maintained by aldosterone Without aldosterone, significant water and sodium imbalances can result in organ failure/death

16. Why we need cortisol Cortisol has a necessary effect on the vascular system (blood vessels, heart) and liver during episodes of physiologic stress

17. Who has Adrenal Insufficiency? Anyone whose adrenal glands have stopped producing steroids as a result of: Long-term administration of steroids Pituitary gland problems or tumor Head trauma Loss of circulation to adrenals/removal of tissue Auto-immune disease Cancer and other diseases (TB and HIV may cause)

18. Adrenal Insufficiency Can occur from long-term administration of steroids (over-rides body’s own steroid production) Examples: Organ transplant patients Long-term COPD Long-term Asthma Severe arthritis Certain cancer treatments

19. Why? Adrenal glands tend to get ‘lazy’ when steroids are regularly administered by mouth, I.M. injection or I.V. infusion To illustrate how quickly…Just 2-4 weeks of daily oral cortisone administration is sufficient to cause the adrenals to be slightly less responsive to stressors

20. Primary Adrenal Insufficiency = Addison’s Disease The adrenal glands are damaged and cannot produce sufficient steroid 80% of the time, damage is caused by an auto- immune response that destroys the adrenal cortex Addison’s can affect both sexes and all age groups

21. Congenital Adrenal Hyperplasia There is also an inherited form of adrenal insufficiency (CAH) Diagnosed by newborn screening; prior to successful screening techniques most children died Daily replacement oral hormones are required at a maintenance dose for LIFE I.M. or I.V. hormones necessary for stressors (illness, surgery, fever, trauma, etc.)

22. Vascular Reactivity In adrenally-insufficient individuals experiencing a physiologic stressor, the vascular smooth muscle will become non- responsive to the effects of norepinephrine and epinephrine, resulting in vasodilation and capillary ‘leaking’ The patient may be unable to maintain an adequate blood pressure The blood vessels cannot respond to the stress and will eventually collapse

23. Energy Metabolism In adrenally-insufficient individuals under increased physiologic stress, the liver is unable to metabolize carbohydrates properly, which may result in profoundly low blood sugar that is difficult to reverse without administration of replacement cortisol

24. Adrenal Insufficiency The speed at which patient deterioration occurs is difficult to predict and is related to the underlying stressor, patient age, general health, etc. Young children can be at high risk for rapid deterioration, even when experiencing a ‘simple’ gastrointestinal disorder

25. CARES EMS Campaign Video Click the link to view the video: http://documents.virtuoso.com/cares/cares_jessica _master_5_med_prog.wmv http://documents.virtuoso.com/cares/cares_jessica _master_5_med_prog.wmv

26. Presentation of Adrenal Crisis The patient may present with any illness or injury as the precipitating event A patient history of adrenal insufficiency warrants a careful assessment under specific protocols Children may deteriorate into adrenal crisis from a simple fever, a gastrointestinal illness, a fall from a bicycle or some other injury A mild illness or injury can easily precipitate an adrenal crisis in any age group

27. Critical Clinical Presentation The early indicators of an adrenal-crisis onset can be vague and non-specific. Some or all signs/symptoms may be present. Infants: Poor appetite Vomiting/diarrhea Lethargy/unresponsive Unexplained hypoglycemia Seizure/cardiovascular collapse/death

28. Critical Clinical Presentation Older Children/Adults Vomiting Hypotensive, often unresponsive to fluids/pressors Pallor, gray, diaphoretic Hypoglycemia, often refractory to D50 May have neurologic deficits Headache/confusion/seizure Lethargy/unresponsive Cardiovascular collapse Death

29. Clearly, the signs/symptoms of adrenal crisis are similar to other serious shock-type presentations. For these patients, standard shock management requires supplementation with corticosteroid medication. It is important to ANTICIPATE the evolution of an adrenal crisis and medicate appropriately under the specific protocols. Do not wait until a full adrenal crisis has developed. Organ damage or death may result from delays. Critical Clinical Presentation

30. Patient Management Follow standard ABC and shock management treatment. BLS: Transport without delay ALS: allow patient or caregivers to administer patient’s own steroid IM as soon as possible after initial life-threat and shock management have been initiated Transport without delay to appropriate hospital with early notification

31. It is important to note that you are caring for a patient with multiple issues: 1. The precipitating event (a trauma/illness that may be a critical issue on its own) and 2. The evolution towards adrenal crisis, which will result in organ failure/death if not reversed Patient Management

32. Administration of steroid medication should come as soon after appropriate A-B-C assessment and interventions as possible Your emergency management priorities remain the same. Patient Management

33. Profile: Solu-Cortef Trade name: Solu-Cortef Generic name: hydrocortisone sodium succinate Class: corticosteroid, Pregnancy Class C Mechanism: acts to suppress inflammation; replaces absent glucocorticoids, acts to suppress immune response

34. Solu-Cortef Side Effects: in emergency use, transient hypertension and/or headache, sodium/water retention may occur. Not usual in a 1-time dose Dosage: Adult: 100 mg IV, IM, IO Pediatric: 2 mg/kg to a max of 100 mg, IV, IM, IO

35. Solu-Cortef Administration route: IM or slow IV bolus. Give IV bolus over 30 seconds. IV infusion is not acceptable for emergency administration For young children, the preferred IM site is the vastus lateralis muscle

36. Solu-Cortef How supplied: self-contained Act-O-Vial Dry powder is in the lower of a two-chambered vial. Diluent is in upper chamber. Do not reconstitute until ready to use

37. Using Act-O-Vial Press down on plastic activator to force diluent into the lower compartment Gently agitate to effect solution Remove plastic tab covering center of stopper Swab top of stopper with a suitable antiseptic Insert needle squarely through centre of plunger- stopper until tip is just visible. Invert vial and withdraw the required dose.

38. Onset of action: for the indicated use (emergency steroid replacement in patient experiencing stressor) the onset of action is minutes. Do not delay transport. Solu-Cortef

39. Special thanks to MA Department of Public Health for Developing and Sharing this Program Dr. Jon Burstein, OEMS staff, and especially: Deborah Clapp, EMT-P, Program Manager EMS for Children MA Dept of Public Health 250 Washington Street 4 th floor Boston MA 02108 617-624-5088 Deborah.Clapp@state.ma.us

40. Resources CARES Foundation (www.caresfoundation.org) Review of Medical Physiology 17 th edition. Ganong, William F., Appleton & Lange Dr. W. R. Litchfield, President, NV Chapter of the American Association of Clinical Endocrinologists, letter of support to SNHD Medical Advisory Board; 2/12/09 Phone conference, Pfizer pharmacist, 2/25/10 Prescribing Information, Solu-Cortef, Sept 2009 Pharmacia & Upjohn (division of Pfizer) Prescribing information, Solu-Medrol, 2009, Pfizer Clark County EMS System BLS/ILS/ALS Protocols

41. Resources, continued “Management of Adrenal Crisis, How Should Glucocorticoids Be Administered?” Stanhope, et al, Journal of Pediatric Endocrinology Vol 16, Issue 8 pp 99-100 “Mortality in Canadian Children with Growth Hormone Deficiency Receiving GH Therapy 1967-1992” Taback, et al, Journal of Clinical Endocrinology & Metabolism Vol 81, #5 pp 1693-1696 Support petition, MA pediatric endocrinologists, 12/ 12/09, Medical Services Committee, on file, OEMS Personal communication, letters of support (Luedke, Smith, Clifford, Dubois, Bradley) Medical Services Committee 12/12/09, on file, OEMS