ADRENAL INSUFFICIENCY Office of Emergency Medical Services & Trauma System

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. Adrenal Anatomy & Physiology • 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

7. Adrenal Anatomy & Physiology • 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)

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

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. How Many in NV have some form of Adrenal Insufficiency? • Short answer: we don’t really know • The CARES Foundation estimates that the number of adrenally-insufficient persons in NV is more than 1,300 not including visitors to the state. • Numbers will most likely continue to increase as the number of successful organ transplants increases. Many children are being diagnosed with severe asthma, which increases the likelihood of long-term steroid use. Better screening tools allow CAH infants to survive to adulthood.

26. Endocrinologist Testimony… • “rapid therapy with intravenous glucocorticoid is a critical, life-saving intervention in patients with adrenal insufficiency in the midst of a medical emergency. Its absence will leave any EMS support rendered by the response team incomplete and inadequate” Support letter, Dr. W. Reid Litchfield, President, Nevada Chapter of the American Association of Clinical Endocrinologists, 2/12/2009

27. CARES EMS Campaign Video • Click the link to view the video: http://documents.virtuoso.com/cares/cares_jessica_master_5_med_prog.wmv

28. 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

29. 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

30. 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

31. Critical Clinical Presentation • 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.

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

33. Patient Management 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

34. Patient Management • 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, with the addition of steroid administration

35. Clark County EMS Protocol Update • This phrase has been added to the “Foreword” of the Clark County BLS/ILS/ALS Protocols concerning the administration of a patient’s own medications which are not part of the approved formulary : • “ (NOTE: telemetry contact is not required for the administration of the patient’s own Solu-Cortef in the treatment of adrenal insufficiency). “ • Many adrenally-insufficient patients carry an emergency Act-O-Vial of Solu-Cortef

36. 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

37. 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

38. 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

39. 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

40. 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.

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

42. 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 4th floor Boston MA 02108 617-624-5088 Deborah.Clapp@state.ma.us

43. Heartfelt Appreciation… • …is extended to the many people whose hard work helped make this protocol change possible, including: • Gretchen Alger Lin, CARES Foundation • Julie Tacker and son Bryce (NV CAH family advocates) • Southern NV endocrinologists: Drs. Asheesh Dewan, W. R. Litchfield, Lewis Morrow, Alan Rice, Rola J. Saad, and Sterling M. Tanner; and nurse practitioner Cathy Flynn • American Association of Clinical Endocrinologists-NV Chapter • SNHD Office of EMS & Trauma System staff and Medical Advisory Board members

44. Resources • CARES Foundation (www.caresfoundation.org) • Review of Medical Physiology 17th 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

45. 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