MH EMS-audio

Malignant Hyperthermia:

Malignant Hyperthermia Tina Moring-SRNA CheryllSt.Onge -SRNA University Of New England

:

Tina Moring - tmoring@une.edu CheryllSt.Onge – cstonge@une.edu Lisa Hogan- lhogan@une.edu Clinical Advisor Nurse Anesthesia Assistant Program Director

What’s Ahead:

History of Malignant Hyperthermia (MH) Pathogenesis and Etiology What role does calcium play? Triggers and non-triggers of MH How does anesthesia deal with MH patients Signs and symptoms of MH crisis Crisis response and control What is dantrolene ? What roles and responsibilities do staff have in an MH crisis? Hospitals vs Ambulatory surgery centers Transferring patients-what’s important to know MH testing-what is out there? What’s Ahead

This can happen….:

This can happen….

First Case Reports:

First Case Reports

What they recommended…1920’s :

Dr. Jones wrote a letter to the “sister of the house” Dear Miss….” in view of what occurred in the case of mother and brother….Family member should never have chloroform for an operation, but rather any operation should be with either gas and oxygen or ether by open method preceeded by morphine gr 1/6 and atropine gr 1/20 ….and the risks should be practically negligible….Yours truly” What they recommended…1920’s

Progress….:

MH was not a recognized phenomenon Monitoring = physical assessment Based on what we know now…1915 had it’s first case of MH Dantrolene -FDA approval 1979 Genetic testing in 1990’s Progress….

1960-MH family history:

Denborough and Lovell first described – July 2, 1960 21 yr old male – repair compound fractures- Dr James Villiers anesthesiologist 10 family deaths under anesthesia-attributed to ether 10 min into surgery – tachycardic , hypotensive , febrile, diaphoretic, and cyanotic Surgery ceased – patient cooled/blood transfusion Patient recovered Denborough and Lovell research inherited sensitivity anesthesia 1960’s MH mortality rate 80% 1960-MH family history

Research:

Research

Incidence:

1:15,000 children 1:50,000 adults Increased prevalence in Mid-west Incidence

Pathogenesis and Etiology:

Syndrome genetically transmitted-heterogenic (more than one gene) Mutation in Ryanodine receptor–abnormal Ca release Increased Ca release greater than Ca removal Hypermetabolic state-no compensatory mechanisms Depletes ATP and glycogen stores Accelerated metabolism no muscle relaxation increased O2 consumption excess CO2 production Pathogenesis and Etiology

Calcium in normal VS MH muscle:

Calcium in normal VS MH muscle

Calcium:

Calcium

Triggers for MH :

Succinylcholine Anesthetic Gases Halothane Isoflurane Enflurane Sevoflurane Desflurane Methoxyflurane Cyclopropane Others are potassium salts, curare and phenothiazines Triggers for MH

Non-Triggering Agents:

Non-depolarizing muscle relaxants Vecuronium Pancuronium Rocuronium Cisatracurium Atracurium Propofol Etomidate Narcotics Barbiturates Benzodiazepines Ketamine Local anesthetics Nitrous oxide Non-Triggering Agents

What’s the difference?:

Depolarizing Succinylcholine Continuous muscle contraction until Ca stores used up Leads to flaccid state Non-depolarizing Rocuronium Blocks the receptor at neuromuscular junction and does NOT allow muscle contraction What’s the difference?

Can we predict MH occurrence?:

NO …50% MH patients – surgery without incidence First MH crisis could be second or third exposure to a triggering agent Pre-op Assessments Is case elective or urgent? History of MH Patient and family history of anesthetic complications Any history of muscle disorders History of unexplained fevers-no diagnosis History of rhabdomyolysis -no diagnosis History of heat stroke History of dark colored urine Can we predict MH occurrence?

Holy MH…!!!:

Early Clinical signs sustained jaw rigidity Tachypnea Tachycardia Irregular pulse Changes in Monitored Signs Increased minute ventilation Rising end tidal CO2 Ventricular ectopy Peaked T waves Holy MH…!!!

Holy MH…!!!:

Succeeding Clinical signs Hot to touch Cyanosis Irregular pulse Changes in Monitored signs Rising T-core Falling SpO2 Ventricular ectopy Peaked T waves Holy MH…!!!

Holy MH…!!!:

Late Clinical Signs Generalized muscle rigidity Prolonged bleeding Dark urine Oliguria Irregular pulse DEATH Changes in Monitored signs Ventricular Ectopy Peaked T waves Holy MH…!!!

Holy MH…!!!:

Elevated end tidal CO2 Tachycardia-unexplained Hypoxemia Acidosis-(respiratory/metabolic) Tachypnea Hyperkalemia -think dysrhythmia Hypercalcemia Dysrhythmias Hypotension/Hypertension Cyanosis Muscle rigidity- masseter muscle spasm Holy MH…!!!

Crisis Response-GET HELP:

Immediate Therapy •Do not give additional succinylcholine •Hyperventilate with 100% O 2 •Bicarbonate 1-2 mg/kg as needed-Ph<7.2 • Dantrolene 2.5 mg/kg push, repeat PRN •Cool patient: gastric lavage , surface, wound •Treat arrhythmias – do not use calcium channel blockers •Arterial or venous blood gases Crisis Response- GET HELP

Crisis Averted:

Continued Care • Dantrolene 1 mg/kg every 4-6 hours for 24 – 48 hours •Monitor for return of symptoms – rate is 25% •Follow electrolytes, blood gases, CK, core temperature, urine output and color, coagulation studies • Biochemical markers –Blood gases – esp pCO 2 , pH – Myoglobin levels in serum and urine –PT, PTT, INR, fibrin split products –Liver enzymes, BUN •Monitor for signs of myoglobinuria and rhabdomyolysis and institute therapy to prevent renal failure Crisis Averted

Dantrolene-How does it work :

Blocks Ca release from sarcoplasmic reticulum Binds to ryanodine receptor decreasing intracellular Ca STOPS progression of hypermetabolic state by relaxing skeletal muscle Half life 6 hours requiring re-dosing Q4-6 hours $2,400.00/year to maintain 36 vials Dantrolene -How does it work

Roles and Responsibilities:

Roles and Responsibilities

Circulating Coordinator:

Call for available HELP and call 911 Assign staff to call MHAUS hotline Assist with patient preparation for transfer in house/external Start 2 Large bore IV’s Discontinue LR and start COLD Normal Saline Assist anesthesia or emergency care provider Change out breathing circuit Change out soda lime Circulating Coordinator

Medication Provider:

Bring MH/Crash cart to room Mix and administer meds as directed Record medications/events (ideally second person) Assist anesthesia Commonly Used Medications Sodium Bicarbonate if Ph<7.2 Lidocaine/Amiodarone 300mg Calcium Chloride Insulin/Glucose Lasix/Mannitol to maintain UO> 2ml/kg/hr Administration of medications requires physician orders Medication Provider

Cooling Provider:

Retrieve COLD saline, ICE and plastic bags Cool patient Monitor temperature Insert Esophageal temperature probe Nasogastric tube 3-way foley catheter Rectal tube Infuse cold saline IV Ice packs to head, neck, axilla , groin and under patient Lavage stomach, bladder, rectum via NG, foley and rectal tube Monitor and record temperature and urine output STOP cooling when temp < 38.5 Cooling Provider

Dantrolene Provider:

Mix and Administer Dantrolene Recruit as much help as possible to prepare & administer Will need at LEAST 2-4 people Ensure a minimum of 36 vials Dantrolene available 2 Liters preservative free Sterile water for mixing 60 cc syringes and spikes or 16 G needles Dantrolene 20 mg + 60 ml PF sterile water 2.5 mg/kg – GIVE RAPIDLY by large bore IV Dose may be repeated up to FOUR times as symptoms persist Dantrolene Provider

Mixing Dantrolene:

Mixing Dantrolene

Convincing Stats:

Mortality: Hospital vs. Ambulatory Settings January 2006-May 2008 MHAUS MH Hotline 503 calls from hospitals 28 determined MH-2 deaths - (7% mortality) 44 calls from ambulatory settings 13 determined MH-3 deaths- ( 21% mortality) Fulminant MH episode occurring outside of the hospital setting is more likely to lead to a bad outcome Convincing Stats

Watch Out…:

You may be the first responder MH can occur up to 24 hours post trigger exposure Can happen at home!! Ambulatory surgery patient may be home Early recognition and treatment is essential to survival Act FAST ! Watch Out…

Ambulatory Surgery Center (ASC) Key Actions…:

Recognition of suspected MH D/C of trigger agents Initiation of treatment Initiation of emergent MH transfer plan Transfer considerations/capabilities Implementation of transfer decision Notification of receiving health care facility-communication Ambulatory Surgery Center (ASC) Key Actions…

Ensuring Safe Transfer:

Transfer patient to facility with critical care team Transport considerations IV Dantrolene Non-depolarizing muscle relaxants Sedatives/hypnotics Analgesics Medications to treat hyperkalemia Life support measures Capabilities to communicate with accepting facility/MH hotline Ensuring Safe Transfer

Communication is KEY:

Patient Data End Tidal CO2 declining or normal Heart rate stable or decreasing-no dysrhythmias IV Dantrolene -point of administration Temperature declining Generalized muscle tone status Direct Personal Communication ASC anesthesia care provider and Critical care, primary or ER physician Communication is KEY

Can Testing be done?:

Caffeine Halothane Contracture Test Gold Standard Expose harvested skeletal muscle to caffeine and halothane Costly Limited sites for testing Complex procedure Most sensitive and specific test Genetic Testing analysis of DNA for specific mutations associated with disease Looks at ryanodine receptor gene – mutation Failure to identify a RYR1 mutation does NOT remove the risk of MH susceptibility Muscle contracture test recommended in addition to genetic testing Can Testing be done?

Bibliography:

Allen, G. L. (1998). Sensitivity and Specificity of the Caffeine-halothane contracture test. Anesthesiology, 88 (3), 579-88. ASC. (2011). Retrieved June/July 2011, Ambulatory Surgery Center Association. http:// ascassociation.org Christian, A. E. (1989). Is there a relationship between masseteric muscle spasm and malignant hyperpyrexia? Bristish Journal of Anesthesia, 62 , 540-44. Denborough , M. (1998). Malignant Hyperthermia. The Lancet, 352 , 1131-36. Deufel , T. G. (1992). Evidence for Genetic Heterogeneity of Malignant Hyperthermia Susceptibility. American Journal of Human Genetics, 50 , 1151-61. Ellis, F. H. (1990). Clinical Presentation of suspected malignant hyperthermia during anesthesia in 402 probands . Anaesthesia , 45 , 838-41. Fletcher, J. R. (1999). Comparison of European and North American Malignant Hyperthermia Diagnostic Protocol Outcomes for Use in Genetic Studies. Anesthesiology, 90 (3), 654-61. Glahn , K. E. (2010). Recognizing and managing a malignant hyperthermia crisis: guideline from the European Malignant Hyperthermia group. British Journal of Anaesthesia , 105 (4), 417-410. Gronert , G. A. Clinical Management of Malignant Hyperthermia. Hall, S. (2001). General Pediatric Emergencies Malignant Hyperthermia Syndrome. Anesthesiology Clinics of North America, 19 (2), 367-82. Harrison, G. I. (1992). Malignant Hyperthermia. Anaesthesia , 47 , 54-56. Hartung , E. K. (1996). Malignant hyperthermia (MH) diagnostics: a comparison between the halothane-caffeine-and the ryanodine -contracture-test results in MH susceptible, normal and control muscle. ACTA AnaesthesiologicaScandinavica , 40 , 437-44. Hommertzheim , R. S. (2006). Malignant Hyperthermia: the perioperative nurse's role. AORN Journal, 83 (1), 149-164. Hopkins, P. H. (1994). Diagnosing malignant hyperthermia susceptibility. Anaesthesia , 49 , 373-75. Hopkins, P. (2000). Malignant Hyperthermia: advances in clinical management and diagnosis. British Journal of Anaesthesia , 85 , 118-28. Bibliography

Bibliography:

Isaacs, H. B. (1993). False-negative results with muscle caffein halothane contracture testing for malignant hyperthermia. Anesthesiology, 79 (1), 5-9. Jurkat-Rott , K. M.-H. (2000). Genetics and Pathogenesis of Malignant Hyperthermia. Muscle and Nerve, 23 , 4-17. Larach , M. L. (1994). A Clinical Grading Scale to Predict Malignant Hyperthermia Susceptibility. Anesthesiology, 80 (4), 771-79. Larach , M. R. (1997). Prediction of Malignant Hyperthermia Susceptibility by Clinical Signs. Anesthesiology, 66 (4), 547-50. Malignant Hyperthermia: An OR Emergency. (2000). Plastic Surgical Nursing, 20 (4), 222-26. Martin, S. V. (2000). Malignant Hyperthermia: A case study. Seminars in Perioperative Nursing, 9 (1), 27-36. MHAUS . (2011). Retrieved January 2011, from Malignant Hyperthermia Association of the United States: www.MHAUS.org Nelson, T. L. (1996). Dantrolene Sodium can Increase or Attenuate Activity of Skeletal Muscle Ryanodine Receptor Calcium Release Channel. Anesthesiology, 84 (6), 1368-79. Ording , H. G. (1997). 4-chloro-m-cresol test-a possible supplementary test for diagnosis of malignant hyperthermia susceptibility. ACTA AnaesthesiologicaScandinavica , 41 , 967-72. Schick, L. Malignant Hyperthermia. Should We Use Muscle Biopsy to Diagnose Malignant Hyperthermia Susceptability ? (1993). Anesthesiology, 79 (1), 1-4. Stanton, C. (2010, September). Transferring patients with malignant hyperthermia . Retrieved from www.aorn.org Stolworthy , C. H. (1998). Malignant Hyperthermia: A Potentially Fatal Complication of Anesthesia. Seminars in Perioperative Nursing, 7 (1), 58-66. Tegazzin , V. S. (1996). Chlorocresol , an Additive to Commercial Succinylcholine , Induces Contracture of Human Malignant Hyperthermia-susceptible Muscles Via Activation of the Ryanodine Receptor Ca+ Channel. Anesthesiology, 84 (6), 1380-85. Bibliography

Slide 39:

Tina Moring tmoring@une.edu CheryllSt.Onge – cstonge@une.edu Lisa Hogan- lhogan@une.edu Clinical Advisor Nurse Anesthesia Assistant Program Director