ADRENAL INSUFFICIENCY

1. ADRENAL INSUFFICIENCY MA EMS Protocol Update 2010

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

5. Objectives, continued • Treatment • Family-centered care • Effective medications • Medication Profiles • Protocol Updates • Relevant protocol changes

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

7. 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

8. 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)

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

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

12. 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.

13. 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

14. 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

15. 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.

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

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

18. 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

19. 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

20. 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.

21. Endocrinologist Testimony… • “…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…” • Support letter, Dr. Christine Leudke, Boston Children’s Hospital 12/12/2009

22. Who has adrenal insufficiency? • Anyone whose adrenal glands have stopped producing steroids as a result of: • Long-term administration of steroids • Pituitary gland problems, including growth hormone deficiency, tumor, etc. • Trauma, including head trauma that affects pituitary • Loss of circulation to adrenals/removal of tissue • Auto-immune disease • Cancer and other diseases (TB and HIV may cause) • There is also an inherited form of adrenal insufficiency (CAH)

23. Congenital Adrenal Hyperplasia • CAH is inherited (recessive gene, each parent contributes) • 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.)

24. More Information about CAH • Learn more about Congenital Adrenal Hyperplasia www.caresfoundation.org Learn more about EMS and CAH; watch a video about a 4-year old CAH patient National EMS Campaign

25. Parent testimony… • “… 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…” • Oral Testimony, Alex Dubois, December 12, 2009

26. 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

27. 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 4 weeks of daily oral cortisone administration is sufficient to cause the adrenals to be slightly less responsive to stressors.

28. Organ Transplant Patients • These individuals must take immunosuppressive medications (usually steroids) DAILY for life. • Their own adrenal glands stop producing cortisol because of external source of steroid.

29. Long-term Asthma and COPD • These individuals are at high risk of adrenal crisis from illness or trauma • Keep in mind that many children and teens with severe asthma take steroid medication every day and may be at significant risk of adrenal crisis. • A severely asthmatic teen may have been started on a steroid 10+ years ago

30. 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

31. Addison’s symptoms • This disease has a gradual onset and can be difficult to diagnose: • Chronic, worsening fatigue • Weight loss • Muscle weakness • Loss of appetite • Nausea/vomiting • Low blood pressure • Low blood sugar • Skin hyperpigmentation • Salt-craving

32. Acute manifestation of Addison’s is called Addison Crisis • Severe vomiting/diarrhea • Dehydration • Hypotension • Sudden, severe pain in back, belly or legs • Loss of consciousness • Can be fatal

33. How Many in MA 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 MA is more than 3800, 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.

34. 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

35. Parent testimony • “…In April of this year, we experienced how much the inability of emergency medical responders to help us impacts our lives. One of my daughters was at my sister’s home playing a game of tag with her cousins and two friends… Alissa was on a slight incline, lost her footing and fell head first onto a rock. She was unconscious and severely injured. My sister had not ever mixed, withdrawn or injected the medicine during an emergency. (She had practiced before, but never actually gave a shot to one to her nieces.)… Fortunately, she was able to inject it, but was unsure if she gave the correct dosage. As it turns out, Alissa was sent via ambulance … and needed to be admitted for three days with a concussion and some broken bones. My sister told me that she, herself, was pretty traumatized from having to give the injection and for having had that responsibility…” • Krupski letter of support, 12/12/09

36. 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

37. Critical Clinical Presentation(not all S&S may be present) 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

38. 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 (Solu-Cortef or Solu-Medrol) • 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.

39. Patient Management • Follow standard ABC and shock management treatment. • BLS/ILS: notify ALS intercept as soon as possible; transport without delay • ALS: administer 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

40. 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.

41. MA EMS Protocol Updates • This phrase has been added to Paramedic Standing Orders in certain ADULT treatment protocols: “For patients with confirmed adrenal insufficiency, give hydrocortisone 100 mg IV, IM or IO OR methylprednisolone 125 mg IV, IM or IO”

42. Link to main MA EMS Protocol page • Relevant ADULT treatment protocols: • 3.3 Altered Mental/Neurological Emergencies • 3.10 Shock (Hypoperfusion) of Unknown Etiology • 4.5 Multi-systems Trauma

43. MA EMS PEDIATRIC Protocol Updates • This phrase has been added to Paramedic Standing Orders in certain PEDIATRIC protocols: “For patients with confirmed adrenal insufficiency, give hydrocortisone 2mg/kg to maximum 100 mg IV, IM or IO OR methylprednisolone 2mg/kg to maximum 125 mg IV, IM or IO”

44. Relevant protocols: • 5.6 Pediatric Coma/Altered Mental/Neurological Status/Diabetic in Children • 5.8 Pediatric Shock • 5.10 Pediatric Trauma and Traumatic Cardiac Arrest

45. 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.

46. Please define “Confirmed Adrenal Insufficiency” Confirmation of a pediatric patient’s condition is determined by the presence of a medic-alert bracelet/necklace, OR by the child, parent or care provider verbally confirming a history of adrenal insufficiency In a school or daycare setting, it is acceptable for the school nurse or daycare provider to relay this information to you Document manner of confirmation on PCR

47. Adults • Confirmation of adrenal insufficiency in adults is achieved by viewing a medic alert bracelet/necklace, or medical record, or when the patient, family member or care provider verbally confirms that the patient has a history of adrenal insufficiency. • Be sure to document manner of confirmation on PCR

48. Patient’s Own Medication • Many adrenally-insufficient patients carry an emergency Act-O-Vial of Solu-Cortef. • Solu-Cortef is included in the required medication formulary, making it acceptable for paramedics to administer the patient’s own medication to the patient or to assist the patient in administering his/her own Solu-Cortef. • Only Paramedic-level EMTs may assist or administer the patient’s own medication.

49. 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

50. Solu-Cortef • MA EMS Indications: replacement of absent corticosteroid in identified adrenally-insufficient patients being managed under specific treatment protocol; many other uses as well • Contra-Indications: Do not use in the newly-born or any individual with a known hypersensitivity to Solu-Cortef