ADRENAL INSUFFICIENCY Office of Emergency Medical Services

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ADRENAL INSUFFICIENCY Office of
Emergency Medical Services Trauma System
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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.

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Table of Contents

• Objectives
• Anatomy Physiology
• Epidemiology
• Presentation
• Management
• Medication Profiles
• Protocol Updates
• Resources

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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

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Adrenal Anatomy Physiology

• The adrenals are endocrine organs that sit on top
  of each kidney

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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

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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)

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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

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The Essential Steroids

• Primary glucocorticoid
• Cortisol (a.k.a. hydrocortisone)
• Primary mineralocorticoid
• Aldosterone

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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.

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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

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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

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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

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

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Why we need cortisol

• Cortisol has a necessary effect on the vascular
  system (blood vessels, heart) and liver during
  episodes of physiologic stress

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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)

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Adrenal Insufficiency

• Can occur from long-term administration of
  steroids (over-rides bodys own steroid
  production) Examples
• Organ transplant patients
• Long-term COPD
• Long-term Asthma
• Severe arthritis
• Certain cancer treatments

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Why?

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

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Primary Adrenal Insufficiency Addisons 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
• Addisons can affect both sexes and all age
  groups

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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.)

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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

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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

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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

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How Many in NV have some form of Adrenal
Insufficiency?

• Short answer we dont 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.

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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

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CARES EMS Campaign Video

• Click the link to view the video
  http//documents.virtuoso.com/cares/cares_jessica_
  master_5_med_prog.wmv

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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

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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

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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

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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.

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Patient Management

• Follow standard ABC and shock management
  treatment.
• BLS Transport without delay
• ILS/ALS administer patients 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

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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

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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

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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 patients own
  medications which are not part of the approved
  formulary
• (NOTE telemetry contact is not required for
  the administration of the patients own
  Solu-Cortef in the treatment of adrenal
  insufficiency).
• Many adrenally-insufficient patients carry an
  emergency Act-O-Vial of Solu-Cortef

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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

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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

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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

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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

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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.

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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.

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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_at_state.ma.us

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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

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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

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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