ADRENAL INSUFFICIENCY

MA EMS Protocol Update

2010

About This Presentation

This presentation is intended for EMTs of all certification levels.
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 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 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 4year 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

(over-rides 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 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

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

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
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

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

Altered Mental/Neurological

3.10 Shock (Hypoperfusion) of Unknown

4.5

Emergencies

Etiology

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

Arrest

Cardiac

 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-OVial of Solu-Cortef.

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
glucocorticoids, acts to
response

absent
suppress immune

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
age-range 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:

Class:

Solu-Medrol

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 newlyborn, underweight neonates

Solu-Medrol
Dose: Adult: 125 mg IM/IV/IO
Pediatric: 2mg/kg to a max of 125 mg
IM/IV/IO

 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-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 17th 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
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