Acknowledgements

We gratefully acknowledge the support of Organon, Inc., West Orange, New Jersey, for their generous unrestricted educational grant which made this program possible. We would especially like to thank Darlene Mashman, MD, Assistant Professor of Anesthesiology at Emory University School of Medicine, Atlanta, Georgia, for developing the concept for this manual. We sincerely thank Carolyn Greenberg, MD, for her input and expertise in making this a valuable educational tool for the Office-Based setting. A special thank you is extended to Andrew Antil, PACU Technician at St. Joseph's Hospital, Syracuse, New York, for the illustration indicating how to mix Dantrolene.

This manual was derived from a protocol developed by Dr. Darlene Mashman, Assistant Professor of Anesthesiology at Emory University School of Medicine in Atlanta, Georgia. Her original concept was used in developing an MH Procedure Manual for the hospital setting to be used as an educational tool to improve an operating room staff's efficiency in the treatment of malignant hyperthermia. A new instructional videotape is available to meet the needs of the Ambulatory and Office-based settings.

With the generous help of Dr. Carolyn Greenberg, Attending Anesthesiologist at Cornell/New York Presbyterian Hospital, formal MH Hotline Consultant and an active member of SAMBA, the hospital manual has been revised to fit the ambulatory settings.

We acknowledge the Malignant Hyperthermia Association of the United States as the originators of this material and thank them for allowing us to include it.

AMBULATORY SURGERY CENTER - M H PROCEDURE MANUAL

Mission Statement - TOP

Malignant Hyperthermia (MH) is an uncommon genetically determined and potentially lethal syndrome that is triggered in susceptible individuals by commonly used general anesthetics.

The Malignant Hyperthermia Association of the United States (MHAUS) is dedicated to reducing the morbidity and mortality of MH. One of its goals is to advise and prepare all medical facilities in the United States for prompt diagnosis and immediate treatment of MH episodes. This manual outlines a response plan and is intended to supplement the MHAUS treatment protocol.

The goal of this manual is to prepare all members of your medical/nursing staff to manage an MH crisis. Each member of the team will be assigned responsibility for specific tasks during an MH crisis. This type of coordinated team effort will allow the anesthesia care provider to focus primarily on the patient's medical care and, hopefully, minimize the risk associated with MH.

This manual assigns specific tasks to personnel, provides complete checklists and recommends periodic mock drills to improve your staff's efficiency in the treatment of Malignant Hyperthermia.

Main Goal

• Diagnose Early
• Treat Early
• Treat Efficiently
• Save A Life

Introduction - TOP

Nationally there continue to be several deaths per year from malignant hyperthermia. Our goal is to reduce this incidence to ZERO deaths annually from this treatable syndrome.

The successful treatment of a patient with malignant hyperthermia depends on four key factors

1. Early recognition of the signs and symptoms. This can be accomplished by educating and updating your staff about the syndrome and its treatment through "in-servicing".

2. Having a malignant hyperthermia cart/kit containing 36 vials of Dantrolene, sterile water (dilutent) and the other supplies in an easily accessible designated location known by all personnel. This allows for rapid treatment.
3. Having a response plan in place to implement the MHAUS recommended therapies quickly and efficiently. This plan should be reviewed and practiced prior to the crisis.
4. Conducting periodic drills to review the response program. Frequency of drills at your site may be determined by your staff experience and turnover.

There are several key points to remember when using the response plan described in this manual:

1. The anesthesia care provider must recognize the EARLY warning signs of malignant hyperthermia {hypercarbia, tachycardia, tachypnea, cardiac arrhythmia, unstable blood pressure, rigidity, mottling, mixed respiratory and metabolic acidosis, myoglobinuria and fever (often late)]. Periodic review will update and educate the entire staff.
2. A method of alerting your staff to initiate the plan and getting the MH cart/kit to the room must be put into place and tested before an emergency arises.
3. Multiple tasks need to be performed at the same time, quickly and efficiently. This is accomplished by assigning tasks to your staff which are outlined clearly on a worksheet using a checklist format. When a crisis occurs, the response plan is initiated by the anesthesia care provider. The responding staff picks up the appropriate worksheet(s) (kept on the MH cart/kit) and performs the tasks outlined. Task assignments may need to be adjusted to meet your staffing availability.
4. Periodic drills conducted by your anesthesia care provider are recommended to remind the staff of their role and the purpose of the worksheets. An instructional video is included in the manual to demonstrate a response3 plan.
5. Used supplies and Dantrolene must be restocked.
6. The patient and family should receive counseling, a letter describing the event, information on the MHAUS Medical ID Tag Program, and referral to MHAUS (1-800-98-MHAUS). The chart should be clearly marked "avoid succinylcholine and volatile anesthetic gases," and an Adverse Metabolic Reaction to Anesthesia (AMRA) Form (included in the manual) should be filled out.
7. The anesthesia care provider will keep your team updated on malignant hyperthermia. This personal should also maintain your response plan, check it periodically for accuracy, make changes as appropriate, and conduct periodic drills.

This manual contains a flowchart, staff worksheets, recommended MH cart/kit supplies and checklist, event drill form, posters, AMRA report, MHAUS poster, :MHAUS ID Tag" brochure, "What is MHAUS?" brochure, and MH Crisis Flowsheet.

Use the flowchart as a roadmap. It is color coded to indicate which staff has similar duties. Place a copy of the staff worksheets in your MH cart/kit for easy access during a crisis. Equip your office with a stocked MH cart/kit and cold supplies. Place a copy of the MH cart/kit supplies and checklist in the cart/kit for easy inspection of supplies. Use the event drill forms to document your response times and test your staff. Display an MHAUS treatment poster in the operating area. Additional posters outlining the MHAUS treatment guidelines are available by calling (1-800-98-MHAUS or 1-607-674-7901 outside the US). Use the MH Hotline for emergency questions at 1-800-MH-HYPER (1-800-644-9737) for the US and Canada, or 1-315-464-7079 outside the US.

Malignant Hyperthermia is an uncommon but treatable syndrome. It can strike at any time. BE PREPARED.

Malignant Hyperthermia Event - TOP

Location of Supplies

• Malignant Hyperthermia Cart/Kit
• Anesthesia Cold Supplies
• Nursing Cold Supplies
• Ice Machines

MHAUS HOTLINE:

1-800-MH-HYPER (1-800-644-9737)-US and Canada

1-315-464-7079-Outside the US

For cardiac arrest in children under 10 years of age without hypoxemia or anesthetic overdose-

ACUTE PHASE TREATMENT

DANTROLENE SODIUM: DOSE AND RECOMMENDATIONS - TOP

1 VIAL + 60 ML STERILE PRESERVATIVE-FREE WATER CONTAINS:

DANTROLENE 20 MG (0.33 MG/ML)
AND
MANNITOL 3 GM (50 MG/ML)

(The only solution to be used in mixing Dantrolene is sterile water.)

Be sure IV is functioning properly, otherwise place a 2^nd IV or central access.

Patient Weight = kg

Dose (2.5 mg/kg) = + 20 = # of vials

Dose is repeated until signs of MH are controlled. - TOP

Consider placement of CVP, if available, for monitoring in the hemodynamically unstable patient. Consider placement of CVP for monitoring volume status if clinically indicated.

4. Metabolic Acidosis:

Sodium bicarbonate

No blood gas available (arterial, femoral, or central)	Blood gas available (arterial, femoral, or central)
1-2 mEq/kg	0.3 x weight (kg) x base deficit 2
(Neonates require 1:2 dilution of NaH2CO3)

5. Hyperkalemia Treatment:
   • Hyperventilation
   • Calcium (for life-threatening hyperkalemia)

   CALCIUM CHLORIDE 10 MG/KG

   OR

   CALCIUM GLUCONATE 10-50 MG/KG
   (calcium gluconate is not compatible with bicarbonate in same solution)

   • Bicarbonate (above)
   • Intravenous glucose and insulin (follow serum glucose levels)

   10 units regular insulin and 50 ml 50% glucose (adult)

   OR

   0.15 units regular insulin/kg and 1 ml/kg 50% glucose (pediatric)

   • Epinephrine (repeat as necessary based on potassium values)

   0.5 mg IV (adult)
   
   10 micrograms/kg per IV (pediatric)

   4. Actively cool the hyperthermic patient:
   • Monitor closely to avoid hypothermia (cool to core temp of 38° C.)
   • Cold IV saline 15 ml/kg- repeat as needed
   • Surface cool with ice packs to groin and axilla and hypothermia blanket
   • Lavage: stomach (via NG tube)

wound (if applicable, via surgeon)

bladder (via irrigating foley catheter)

rectum (via rectal tube)

4. Dysrhythmias:
   • Usually respond to treatment of acidosis and hyperkalemia
   • If persistent and/or life threatening, use standing anti-dysrhythmic agents except calcium channel blockers which may cause hyperkalemia or cardiac arrest in the presence of Dantrolene Sodium.
     
5. Keep urine output greater than 2ml/kg/hour:
   • Aggressive hydration (may require CVP or PA catheter placement for monitoring volume status)
   • Furosemide 0.5-1.0 mg/kg IV
   • Since 1 vial of Dantrolene contains 3 gms of Mannitol, additional Mannitol is not usually necessary

   
   

   10. Laboratory tests:

   Recommendations

   COLLECT AND SEND 1^ST SAMPLE, IF POSSIBLE

   Blood Gas and Electrolytes Arterial, Central, or Venous Blood Gas Na Glucose K+ Ca+2

   Urine Dipstick Hb/Myoglobin CK/Myoglobin/SMA-19 Lactic Acid Level PT/PTT/FSP/D-DIMER/Fibrinogen CBC/Platelets

   • If urine dipstick is positive for hemoglobin, it may represent the presence of myoglobin.
   • Continuous end-tidal CO₂ and core temperature monitoring are strongly recommended

Anesthesia Care Provider

Acute Phase Treatment

Review Checklist

(See Anesthesia Care Provider Worksheet for Detailed Treatments)

Signs of Malignant Hyperthermia Identified:

Hotline in USA: 1-800-MH-HYPER (1-800-644-9737)

Hotline outside USA: 1-315-464-7079

Volatile anesthetic agents off and succinylcholine discontinued

Hyperventilation with 100% oxygen, high flows (at least >10 I/min)

Response Plan initiated

Malignant Hyperthermia Cart/Kit on the way

MH Cart/Kit in room and worksheets distributed

Dantrolene being mixed and administered

ECG, End-tidal CO₂ and core temp continuously monitored

Arterial, central, or venous blood gas sent

K+, Ca⁺², Na, Glucose

Acidosis being treated

Hyperkalemia being treated

Treating dysrhythmias (no calcium channel blockers)

Cool hyperthermic patient:

Cold saline infusion

Warming devices off

Hypothermia blanket on

NG Tube in place

Lavage with cold saline

Wound irrigation

Foley catheter placed

Urine sample for myoglobin obtained (if possible)

Lavage with cold saline, if needed

Don't overcool

Invasive lines in place if available/needed

Other labs drawn (if possible):

CPK

SERUM MYOGLOBIN

URINE MYOGLOBIN

PT/PTT, FIBRINOGEN, FSP, D-DIMER

CBC WITH PLATELETS

LACTIC ACID

URINE OUTPUT > 2CC/KG/HR

Anesthesia Care Provider Worksheet

Post Acute Phase Treatment - TOP

1) Ensure restocking of Dantrolene for MH cart/kit and supplies

2) Ensure MH cart/kit has been returned to designated location

3) Report to MHAUS and fill out Adverse Metabolic Reaction to Anesthesia (AMRA)* Report, which is included in this manual. For additional copies call 1-800-986-4287

4) Counsel family and patient:

• Explain implications of MH and further precautions
• Recommend follow-up muscle biopsy
• Provide a letter describing events
• Carefully mark the chart: include volatile anesthetic gases and succinylcholine in the allergies section of the chart
• Inform patient about MHAUS ID-Tag Program
• Refer patient to:

MHAUS
P.O. Box 1069
39 East State Street
Sherburne, NY 13460-1069
(607) 674-7901
(800) 98-MHAUS
E-mail: mhaus@norwich.net

 

* This information is entered into the North American MH Registry of MHAUS

Circulator/Scrub Nurse Worksheet

Malignant Hyperthermia Event

Procedures for a Malignant Hyperthermia Crisis - TOP

when completed

1) Bring MH cart/kit, nursing supplies (bag), code cart and defibrillator to the crisis area.
2) Get materials to help surgeon close wound.
3) Receive worksheet from the anesthesia care provider:

• help mix Dantrolene if necessary
• prepare and place ice bags to groin and axilla
• place foley catheter: obtain urine for myoglobin, then begin cold saline lavage, if necessary

4) Take Telephone Worksheet to the Secretary/Clerk.
5) Take PACU Worksheet to the PACU Nurse.
6) Await further assignment.

After patient is stable:

1) Restocks nursing supplies in refrigerator and on MH cart/kit.

PACU Nurse Worksheet

Malignant Hyperthermia Event

Procedures for a Malignant Hyperthermia Crisis

" when completed

1) Prepare monitors and bed space for the patient in recovery area (if applicable)

• ensure defibrillator, code cart and ice are available at the bed space

2) Offer assistance to Circulator/Scrub Nurse (if possible)

After patient is stable:

1) Confirms Receiving Hospital is ready to receive patient and updates the Receiving Hospital on patient transfer.

Secretary/Clerk Telephone Worksheet

Malignant Hyperthermia Event

Procedures for a Malignant Hyperthermia Crisis

" when completed - write contact person's name

1) Available Medical and Nursing Personnel:
2) Clinical Laboratory (if specimens are being sent):

Malignant Hyperthermia

Event Drill

Date

Drill Location (OR #)

Conducted by

Arrival of Items	Arrival Times
Malignant Hyperthermia Cart/Kit
Chilled 1000 ml normal saline bags for IV infusion
Bags of cold normal saline for wound irrigation
Ice
Regular insulin 100 units/ml
Responders
Surgeon	Anesthesia Care Provider
Nurse	Secretary/Clerk

Organization of Team: Poor Fair Good Excellent

Comments/Areas of Improvement:

Malignant Hyperthermia

Cart/Kit Supplies Checklist

Drugs - TOP

Expiration Date

1) Dantrolene 36 vials (each is diluted with 60 ml sterile H2))

2) Sterile water (without bacteriostatic agent) 1000 ml x 2

3) 8.4% sodium bicarbonate 50 ml x 2

4) Furosemide 40 mg/amp x 2 ampules

5) D50 50 ml vials x 2

6) 10% calcium chloride 20 ml vial x 2

7) Regular insulin 100 u/ml x 1 (refrigerated)

8) 2% lidocaine HCI: 20 ml vial x 2

General Equipment

1) 60 ml syringes x 3 (to dilute Dantrolene)

2) Mini Spike IV additive pins x 2 and Multi Ad fluid transfer sets x 2 (to reconstitute Dantrolene)

3) Angiocaths (for IV access and arterial line

4) NG tubes: sizes appropriate for your patient population

5) Pressure bag

6) Irrigating syringes x 2 (for NG irrigation)

7) Large clear plastic bags for ice

8) Bucket for ice

Malignant Hyperthermia

Cart/Kit Supplies Checklist

Airway Equipment (sizes appropriate for your patient population)

1) Anesthesia breathing circuit and rebreathing bag

2) Ambu bag (for transportation)

Monitoring Equipment

1) Esophageal temperature probes

2) A Line/CVP/Transducer kits

Intravenous Supplies

1) D5W 250 ml x 1

2) Microdrip IV set x 1

NOTE: Infusion pump preferable

Nursing Supplies on Cart/Kit

1) Large Steri-drape (for rapid drape of wound)

2) Three-way irrigating foley catheters: sizes appropriate for your patient population

3) 60 cc Toomy irrigating syringe x 2

4) Large clear plastic bags for ice x 4

5) Small plastic bags for ice x 4

6) Tray for ice

Malignant Hyperthermia

Cart/Kit Supplies Checklist

Laboratory Testing - TOP

1) 3 ml syringes or ABG kits
2) Blood specimen tubes
	CK/Myoglobin/SMA-19
	Lactic Acid Level
	PT/PTT/FSP/D-Dimer/Fibrinogen
	CBC/Platelets
3) Urine tube: myoglobin level
4) Urine dipstick: hemoglobin and myoglobin


Forms Folder - TOP

1) Laboratory Requests

2) Adverse Metabolic Reaction to Anesthesia (AMRA) Report included in manual (extras can be obtained from MHAUS)

3) Consult Form

Malignant Hyperthermia

Cold Supplies Checklist

Laboratory Testing

Anesthesia Cold Supplies

Expiration Date

1) From Refrigerator: Supplies labeled "for Malignant Hyperthermia Crisis only"
	1000 ml bags cold normal saline x 3

Nursing Cold Supplies

Expiration Date

2) From Refrigerator: Supplies labeled "for Malignant Hyperthermia Crisis only"
	Bags cold normal saline
3) From Refrigerator:
	Regular insulin 100 u/ml x 1 vial




MALIGNANT HYPERTHERMIA OFFICE-BASED SURGERY CENTER PROTOCOL FLOWSHEET - TOP

Anesthesiologist/Anesthesia Care Provider Suspects Malignant Hyperthermia and Alerts Surgeon/ Circulator/Scrub Nurse

Circulator (or Assigned Person) brings the Malignant Hyperthermia Cart/Kit including MHAUS' Treatment Protocol, and Code Cart to the Operating Area

SURGEON	ANESTHESIOLOGIST/ ANESTHESIA CARE PROVIDER	CIRCULATOR/ SCRUB NURSE	PACU NURSE	SECRETARY/ CLERK
COMMUNICATES VERBALLY WITH TEAM WHILE OPERATING: 1. Closes wound (consider cold saline irrigation of wound, if appropriate)	1. Focus is diagnosis and implementation of MHAUS treatment protocol. Directs resuscitation. STABILIZE PATIENT AND TRANSPORT TO NEAREST HOSPITAL. (D/C triggering agents. Hyperventilate with 100% oxygen, mix and administer Dantrolene, treat electrolyte & acid base abnormalities, if information is available) Assigns Worksheets to Team If available, sets up monitors and places additional lines. Ensures placement of second IV if necessary. Places NG tube for cold saline irrigation, if needed. Turns off any patient warming devices. Turn on cooling device, if available. Ensures blood & urine samples are sent for analysis, if possible.	Brings MH Cart/kit, nursing supplies, code cart and defibrillator to the crisis area. Gets materials to help surgeon close wound. Receives worksheet from the anesthesia care provider: helps mix Dantrolene prepares and places ice bags to groin and axilla places foley catheter: obtain urine for myoglobin, then begins cold saline lavage, if necessary Takes Telephone Worksheet to the Secretary/Clerk. Takes PACU Worksheet to the PACU Nurse. Awaits further assignments.	Receives PACU Nurse worksheet from the Circulator/ Scrub Nurse Prepares monitors and bed space for the patient in recovery area (if applicable) - ensures defibrillator, code cart and ice are available at the bed space. 3. Offers assistance to Circulator/ Scrub Nurse (if possible)	Receives Front Desk/ Clerk Telephone Worksheet from the Circulator/ Scrub Nurse. 2. Immediately calls the numbers on the worksheet.




SURGEON	ANESTHESIOLOGIST/ ANESTHESIA CARE PROVIDER	CIRCULATOR/ SCRUB NURSE	PACU NURSE
AFTER THE WOUND IS CLOSED: 1. Assists with evaluation and treatment plan via the MHAUS protocol.	AFTER THE PATIENT IS STABLE: Continues to mechanically ventilate patient while awaiting transport to the hospital. Continues sedation if patient is hemodynamic- ally stable, intubated and an amnestic is indicated. Calls receiving hospital to report the case to the ER physician and/or anesthesiologist. (Telephone numbers for the nearest hospital that can handle this type of emergency should already be identified and telephone numbers should appear on the Anesthesia Care Provider and Telephone Worksheets.) Consider accompanying the patient during transport. AFTER THE PATIENT HAS BEEN TRANSPORTED TO THE NEAREST HOSPITAL: Restocks Dantrolene and MH supplies. Reports event to MHAUS and completes AMRA form. Counsels family and patient: Explains implications of MH Patient letter Clearly mark chart Informs patient about MHAUS Medical ID Tag Program.	AFTER THE PATIENT IS STABLE: 1. Restocks nursing supplies on MH Cart/ Kit and refrigerator.	AFTER THE PATIENT IS STABLE: 1. Confirms receiving hospital is ready to receive patient and updates the receiving hospital on patient transfer..

 

Malignant Hyperthermia Event - TOP

Location of Supplies

• Malignant Hyperthermia Cart/Kit
• Anesthesia Cold Supplies
• Nursing Cold Supplies
• Ice Machines



MHAUS HOTLINE:

1-800-MH-HYPER (1-800-644-9737)-US and Canada
1-315-464-7079-Outside the US

For cardiac arrest in children under 10 years of age without hypoxemia or anesthetic overdose-

• assume subclinical muscular dystrophy and treat for hyperkalemia first -e.g., CaCI₂-see page 9 of worksheet.
• Consult neurologist for follow-up.

 

ACUTE PHASE TREATMENT

1. Discontinue all volatile inhalation anesthetics and succinylcholine. Consider giving amnestic sedatives.
2. Hyperventilate with 100% oxygen with flows at least 10 I/min. e.g., 10 ml/kg tidal volume x 15/min.
3. Give Dantrolene sodium:

 

PHARMACOKINETICS OF DANTROLENE	SIDE EFFECTS OF DANTROLENE
Onset 6-20 minutes	Cardiac arrest and hyperkalemia if given with calcium channel blockers
Duration 5-6 hours	Muscle weakness
Metabolized by liver	Drowsiness, dizziness
Excreted by kidney	Headache and nausea
	Hepatotoxicity with prolonged use




DANTROLENE SODIUM: DOSE AND RECOMMENDATIONS - TOP

1 VIAL + 60 ML STERILE PRESERVATIVE-FREE WATER CONTAINS:

DANTROLENE 20 MG (0.33 MG/ML)

AND

MANNITOL 3 GM (50 MG/ML)

(The only solution to be used in mixing Dantrolene is sterile water.)

DOSE: 2.5 MG/KG RAPID IV BOLUS

Repeat in increments up to a total dose of 10 mg/kg until signs of MH are controlled (e.g., heart rate, hypercarbia, acidosis, temperature, and rigidity). More than 10 mg/kg may be needed for some patients.

 

Be sure IV is functioning properly, otherwise place a 2^nd IV or central access. - TOP

Patient Weight = kg

Dose (2.5 mg/kg) = + 20 = # of vials

DOSE # MG GIVEN CUMULATIVE DOSE GIVEN SO FAR TIME DOSE COMPLETED RESPONSE #1 2.5 MG/KG       #2         #3         #4         #5

 


Dose is repeated until signs of MH are controlled.

4. Lines:

Consider placement of CVP, if available, for monitoring in the hemodynamically unstable patient. Consider placement of CVP for monitoring volume status if clinically indicated.

4. Metabolic Acidosis:

Sodium bicarbonate

No blood gas available (arterial, femoral, or central)	Blood gas available (arterial, femoral, or central)
1-2 mEq/kg	0.3 x weight (kg) x base deficit 2
(Neonates require 1:2 dilution of NaH2CO3)

5. Hyperkalemia Treatment:
• Hyperventilation
• Calcium (for life-threatening hyperkalemia)

CALCIUM CHLORIDE 10 MG/KG

OR

CALCIUM GLUCONATE 10-50 MG/KG

(calcium gluconate is not compatible with bicarbonate in same solution)

• Bicarbonate (above)
• Intravenous glucose and insulin (follow serum glucose levels)

10 units regular insulin and 50 ml 50% glucose (adult)

OR

0.15 units regular insulin/kg and 1 ml/kg 50% glucose (pediatric)

• Epinephrine (repeat as necessary based on potassium values)

0.5 mg IV (adult)

10 micrograms/kg per IV (pediatric)

4. Actively cool the hyperthermic patient:
• Monitor closely to avoid hypothermia (cool to core temp of 38° C.)
• Cold IV saline 15 ml/kg- repeat as needed
• Surface cool with ice packs to groin and axilla and hypothermia blanket
• Lavage: stomach (via NG tube)

wound (if applicable, via surgeon)

bladder (via irrigating foley catheter)

rectum (via rectal tube)