Adrenal Protocol

ADRENAL INSUFFICIENCY

Office of Emergency Medical Services & Trauma System

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:EpidemiologyAnatomy & Physiology

• PathophysiologyPresentation

• Signs & SymptomsTreatment

• Family-centered care• Effective medications

Adrenal Anatomy & Physiology

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

Each adrenal gland has two partsAdrenal 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 functionProvides blood pressure regulationEnables 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

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)

Adrenal Insufficiency

Can occur from long-term administration of steroids (over-rides body’s own steroid production) Examples:

Organ transplant patientsLong-term COPDLong-term AsthmaSevere arthritisCertain 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 quickly…Just 2-4 weeks of daily oral cortisone administration is sufficient to cause the adrenals to be slightly less responsive to stressors

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

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.)

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

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

How Many in NV 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 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.

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

CARES EMS Campaign Video

Click the link to view the video: http://documents.virtuoso.com/cares/cares_jessica_master_5_med_prog.wmv

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

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 appetiteVomiting/diarrheaLethargy/unresponsive

• Unexplained hypoglycemiaSeizure/cardiovascular collapse/death


Older Children/AdultsVomitingHypotensive, often unresponsive to fluids/pressors

• Pallor, gray, diaphoreticHypoglycemia, often refractory to D50May have neurologic deficits

• Headache/confusion/seizure• Lethargy/unresponsive

Cardiovascular collapseDeath

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.


Patient Management

Follow standard ABC and shock management treatment.

BLS: Transport without delay ILS/ALS: administer patient’s own steroid IM/IV/IO

as soon as possible after initial life-threat and shock management have been initiatedTransport 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)

and2. The evolution towards adrenal crisis, which will

result in organ failure/death if not reversed

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

Patient Management

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 patient’s own medications which are not part of the approved formulary :“ (NOTE: telemetry contact is not required for the

administration of the patient’s own Solu-Cortef in the treatment of adrenal insufficiency). “

Many adrenally-insufficient patients carry an emergency Act-O-Vial of Solu-Cortef

Profile: Solu-Cortef

Trade name: Solu-CortefGeneric name: hydrocortisone sodium

succinateClass: corticosteroid, Pregnancy Class CMechanism: acts to suppress

inflammation; replaces absent glucocorticoids, acts to suppress immune response

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

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 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

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.

Solu-Cortef

Special thanks to MA Department of Public Health for Developing and Sharing this ProgramDr. Jon Burstein, OEMS staff, and especially:

Deborah Clapp, EMT-P, Program ManagerEMS for ChildrenMA Dept of Public Health250 Washington Street 4th floorBoston MA [email protected]

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

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

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

Documents

Adrenal Protocol