ADRENAL INSUFFICIENCY

MA EMS Protocol
Update
2010

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.

Table of Contents

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

OBJECTIVES: at the end

of this program, EMTs
will have increased
awareness of:

Epidemiology
Anatomy & Physiology
Pathophysiology
Presentation
Signs & Symptoms

Objectives, continued

Treatment
Family-centered care
Effective medications
Medication Profiles

Protocol Updates
Relevant protocol changes

Adrenal Anatomy &

Physiology

The adrenals are endocrine

organs that sit on top of each
kidney

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 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)

A person can survive without a

functioning adrenal medulla.

A functioning adrenal cortex

(or the steady availability of
replacement hormone) is
essential for survival.

The Essential
Steroids

Primary glucocorticoid:
Cortisol (a.k.a.
hydrocortisone)

Primary mineralocorticoid:
Aldosterone

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.

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

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

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.

Water/sodium balance is
maintained by aldosterone.

Without aldosterone,
significant water and sodium
imbalances can result in organ
failure/death.

Why we need cortisol

Cortisol has a necessary

effect on the vascular system
(blood vessels, heart) and
liver during episodes of
physiologic stress

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

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

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.

Endocrinologist
Testimony

In adrenal insufficiency, because of

the inability to produce glucocorticoids
and often mineralocorticoids from the
adrenal glands, there is a risk of lifethreatening 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 Childrens Hospital 12/12/2009

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)

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.)

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

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

Adrenal Insufficiency

Can occur from long-term

administration of steroids (overrides bodys own steroid
production) Examples:
Organ

transplant patients
Long-term COPD
Long-term Asthma
Severe arthritis
Certain cancer treatments

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 4

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

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.

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

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

Addisons 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

Acute manifestation of
Addisons is called
Addison Crisis

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?
Short answer: we dont 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

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

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 sisters 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

The early indicators of an adrenalcrisis onset can be vague and nonspecific. Some or all signs/symptoms
may be present.

Infants:
Poor appetite
Vomiting/diarrhea
Lethargy/unresponsive
Unexplained hypoglycemia
Seizure/cardiovascular

collapse/death

Critical Clinical
Presentation
(not
all S&S may be
Older Children/Adults
Vomiting
present)
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

Clearly, the signs/symptoms of adrenal

crisis are similar to other serious shocktype 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

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 lifethreat 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

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

Relevant

ADULT treatment protocols:

3.3

3.10 Shock (Hypoperfusion) of

Unknown
Etiology

4.5

Altered Mental/Neurological
Emergencies

Multi-systems Trauma

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

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

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.

Please define
Confirmed Adrenal
Insufficiency
Confirmation of a pediatric patients

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

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

Patients Own
Medication

Many adrenally-insufficient patients

carry an emergency Act-O-Vial of SoluCortef.

Solu-Cortef is included in the required

medication formulary, making it
acceptable for paramedics to administer
the patients 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 patients own medication.

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

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

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

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

Solu-Cortef

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

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.

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

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 agerange is not recommended for pre-hospital
setting

Additional Notes

Solu-Cortef is the first choice

for management of adrenal
insufficiency/adrenal crisis.

The other approved medication,

Solu-Medrol, is an acceptable
alternative choice for
specific management of adrenal
insufficiency/adrenal crisis

Solu-Medrol

Generic: methylpredisolone
sodium
succinate

Trade:

Solu-Medrol

Class:

steroid

Pregnancy Class:

Solu-Medrol

Indications: Ma EMS Protocol: replacement

of absent corticosteroid in identified
adrenally-insufficient 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

Contraindications: any patient with

systemic fungal infection, any person with
known hypersensitivity to Solu-Medrol; the
newly-born, underweight neonates

Solu-Medrol

Dose:

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

Adult: 125 mg IM/IV/IO

Pediatric: 2mg/kg to
a max of 125 mg
IM/IV/IO

Solu-Medrol

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

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.

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
Deborah.Clapp@state.ma.us

Heartfelt
Appreciation

is extended to the many people whose hard

work helped make these protocol changes
possible, including:

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

CARES Foundation (www.caresfoundation.org)

Review of Medical Physiology 17 th edition.
Ganong, William F., Appleton & Lange
Dr. Christine Luedke (pediatric endocrinologist,
Childrens 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

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