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

PresentationSigns & Symptoms

Objectives, continued

TreatmentFamily-centered careEffective medications

• Medication Profiles

Protocol UpdatesRelevant 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 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

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 Children’s 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 (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 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= 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

Addison’s 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 Addison’s 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 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

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 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

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, diaphoreticHypoglycemia, 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 Emergencies

3.10 Shock (Hypoperfusion) of Unknown Etiology

4.5 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 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

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

Patient’s Own Medication

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

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.

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

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, IOProtect 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: Solu-Medrol

Class: steroid

Pregnancy Class: C

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: 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 ManagerEMS for ChildrenMA Dept of Public Health250 Washington Street 4th floorBoston MA 02108617-624-5088Deborah.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, 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

“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