Download Ma EMS Protocol

ADRENAL INSUFFICIENCY
MA EMS Protocol Update
2010
About This Presentation
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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.
Table of Contents
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Objectives
Anatomy & Physiology
Epidemiology
Presentation
Management
Medication Profiles
Protocol Updates
Resources
OBJECTIVES: at the end of this
program, EMTs will have
increased awareness of:
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Epidemiology
Anatomy & Physiology
 Pathophysiology
Presentation
 Signs & Symptoms
Objectives, continued
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Treatment
 Family-centered care
 Effective medications
• Medication Profiles
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Protocol Updates
 Relevant protocol changes
Adrenal Anatomy &
Physiology
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The adrenals are endocrine organs that sit
on top of each kidney
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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 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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A person can survive without a functioning
adrenal medulla.
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A functioning adrenal cortex (or the steady
availability of replacement hormone) is
essential for survival.
The Essential Steroids
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Primary glucocorticoid:
 Cortisol (a.k.a. hydrocortisone)
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Primary mineralocorticoid:
 Aldosterone
Cortisol
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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.
Cortisol
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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
Cortisol
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When cortisol is not produced or released
by the adrenal glands, humans are unable to
respond appropriately to physiologic
stressors.
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Rapid deterioration resulting in organ
damage and shock/coma/death can occur,
especially in children
Aldosterone
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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.
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Without aldosterone, significant water and
sodium imbalances can result in organ
failure/death.
Why we need cortisol
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Cortisol has a necessary effect on the
vascular system (blood vessels, heart) and
liver during episodes of physiologic stress
Vascular Reactivity
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In adrenally-insufficient individuals
experiencing a physiologic stressor, the
vascular smooth muscle will become nonresponsive 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
Energy Metabolism
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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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The speed at which patient deterioration occurs is
difficult to predict and is related to the underlying
stressor, patient age, general health, etc.
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Young children can be at high risk for rapid
deterioration, even when experiencing a ‘simple’
gastrointestinal disorder.
Endocrinologist Testimony…
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“…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
Who has adrenal insufficiency?
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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)
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There is also an inherited form of adrenal insufficiency
(CAH)
Congenital Adrenal Hyperplasia
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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.)
More Information about CAH
Learn more about Congenital Adrenal
Hyperplasia
www.caresfoundation.org
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Learn more about EMS and CAH; watch a video
about a 4-year old CAH patient
National EMS Campaign
Parent testimony…
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“… 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
Adrenal Insufficiency
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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
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Why?
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Adrenal glands tend to get ‘lazy’ when
steroids are regularly administered by
mouth, I.M. injection or I.V. infusion.
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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.
Organ Transplant Patients
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These individuals must take
immunosuppressive medications (usually
steroids) DAILY for life.
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Their own adrenal glands stop producing
cortisol because of external source of
steroid.
Long-term Asthma and COPD
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These individuals are at high risk of adrenal crisis
from illness or trauma
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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.
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A severely asthmatic teen may have been started
on a steroid 10+ years ago
Primary Adrenal Insufficiency=
Addison’s Disease
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The adrenal glands are damaged and cannot
produce sufficient steroid
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80% of the time, damage is caused by an autoimmune response that destroys the adrenal cortex
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Addison’s can affect both sexes and all age groups
Addison’s symptoms
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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
Acute manifestation of Addison’s
is called Addison Crisis
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Severe vomiting/diarrhea
Dehydration
Hypotension
Sudden, severe pain in back, belly or legs
Loss of consciousness
Can be fatal
How Many in MA have some
form of Adrenal Insufficiency?
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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.
Presentation of Adrenal Crisis
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The patient may present with any illness or injury
as the precipitating event.
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A patient history of adrenal insufficiency warrants a careful
assessment under specific protocols
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Children may deteriorate into adrenal crisis from a simple
fever, a gastrointestinal illness, a fall from a bicycle or some
other injury.
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A mild illness or injury can easily precipitate
an adrenal crisis in any age group
Parent testimony
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“…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
Critical Clinical Presentation
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The early indicators of an adrenal-crisis onset can
be vague and non-specific. Some or all
signs/symptoms may be present.
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Infants:
 Poor appetite
 Vomiting/diarrhea
 Lethargy/unresponsive
• Unexplained hypoglycemia
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Seizure/cardiovascular collapse/death
Critical Clinical Presentation
(not all S&S may be present)
Older Children/Adults
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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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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.
Patient Management
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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
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.
MA EMS Protocol Updates
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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”
Link to main MA EMS Protocol
page
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Relevant ADULT treatment protocols:
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3.3 Altered Mental/Neurological
Emergencies
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3.10 Shock (Hypoperfusion) of Unknown
Etiology
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4.5 Multi-systems Trauma
MA EMS PEDIATRIC Protocol
Updates
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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”
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Relevant protocols:
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5.6 Pediatric Coma/Altered
Mental/Neurological Status/Diabetic in
Children
5.8 Pediatric Shock
5.10 Pediatric Trauma and Traumatic
Cardiac Arrest
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Administration of steroid medication should
come as soon after appropriate A-B-C
assessment and interventions as possible
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Your emergency management priorities
remain the same, with the addition of
steroid administration.
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
Adults
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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.
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Be sure to document manner of confirmation on
PCR
Patient’s Own Medication
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Many adrenally-insufficient patients carry an
emergency Act-O-Vial of Solu-Cortef.
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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.
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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
Solu-Cortef
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MA EMS Indications: replacement of absent
corticosteroid in identified adrenallyinsufficient patients being managed under
specific treatment protocol; many other
uses as well
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Contra-Indications: Do not use in the newlyborn or any individual with a known
hypersensitivity to Solu-Cortef
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
Protect from heat
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Solu-Cortef
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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
Solu-Cortef
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How supplied: self-contained Acto-Vial
Dry powder is in the lower of a two-chambered vial.
Diluent is in upper chamber.
Do not reconstitute until ready to use
Using Act-O-Vial
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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 plungerstopper until tip is just visible. Invert vial and
withdraw the required dose.
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Onset of action: for the indicated use
(emergency steroid replacement in patient
experiencing stressor) the onset of action is
minutes. Do not delay transport.
Additional Notes
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This product contains the preservative Benzyl
Alcohol which is found in many medications. The
amount of Benzyl Alcohol is negligible in
comparison to other products and this medication is
considered very safe and effective for emergency
administration.
The exception is the newly-born and/or significantly
underweight neonates. In these groups there is
insufficient data; this medication may cause
‘gasping syndrome’, therefore use in this age-range
is not recommended for pre-hospital setting
Additional Notes
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Solu-Cortef is the first choice for
management of adrenal
insufficiency/adrenal crisis.
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The other approved medication, SoluMedrol, is an acceptable alternative choice
for specific management of adrenal
insufficiency/adrenal crisis
Solu-Medrol
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Generic: methylpredisolone sodium
succinate
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Trade:
Solu-Medrol
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Class:
steroid
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Pregnancy Class: C
Solu-Medrol
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Indications: Ma EMS Protocol: replacement of
absent corticosteroid in identified adrenallyinsufficient patients being managed under specific
treatment protocol; Other: many uses, including
acute bronchial asthma (not first-line); anaphylaxis
(not first-line); acute exacerbation of multiple
sclerosis
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Contraindications: any patient with systemic fungal
infection, any person with known hypersensitivity
to Solu-Medrol; the newly-born, underweight
neonates
Solu-Medrol
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Dose: Adult: 125 mg IM/IV/IO
Pediatric: 2mg/kg to a max of 125 mg
IM/IV/IO
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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-Medrol
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Onset of action: for the indicated use
(emergency steroid replacement in patient
experiencing stressor) the onset of action is
minutes. Do not delay transport.
Using the Act-O-Vial
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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 plungerstopper until tip is just visible. Invert vial and
withdraw the required dose.
Additional Notes


This product contains the preservative Benzyl
Alcohol which is found in many medications. The
amount of Benzyl Alcohol is negligible in
comparison to other products and this medication is
considered very safe and effective for emergency
administration.
The exception is the newly-born and/or significantly
underweight neonates. In these groups there is
insufficient data; the drug may cause ‘gasping
syndrome’ therefore use in this age-range is not
recommended in the pre-hospital setting
The End! (resources follow)
Please feel free to contact me:
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
[email protected]
Heartfelt Appreciation…
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…is extended to the many people whose hard work helped
make these protocol changes possible, including:
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Alex Dubois and son James (MA CAH family advocates)
Dr. Christine Leudke and the many other pediatric
endocrinologists across the state of Massachusetts
Dr. Jon Burstein, OEMS staff and members of the MA
Medical Services Committee
Gretchen Alger Lin, CARES Foundation
family members, state legislators and others for their letters
of support and kind words
Resources
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CARES Foundation (www.caresfoundation.org)
Review of Medical Physiology 17th edition. Ganong, William F.,
Appleton & Lange
Dr. Christine Luedke (pediatric endocrinologist, Children’s
Hospital of Boston ) letter of support to Medical Services
Committee; oral presentation, personal communication
12/12/09
Phone conference, Pfizer pharmacist, 2/25/10
Prescribing Information, Solu-Cortef, Sept 2009 Pharmacia &
Upjohn (division of Pfizer)
Prescribing information, Solu-Medrol, 2009, Pfizer
MA Statewide Treatment Protocols, version 8.03
Resources, continued
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“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 (Smith, Clifford,
Dubois, Bradley) Medical Services Committee 12/12/09, on
file, OEMS