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Malignant Malignant HyperthermiaHyperthermia

Presented By: St. James Healthcare Education Collaborative

With the support of: Surgical Services Leadership Team

June 2012

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Target Audience & Target Audience & ObjectivesObjectives

• Target audience is all associates who care for surgical Target audience is all associates who care for surgical patients undergoing general anesthesia and at risk for patients undergoing general anesthesia and at risk for malignant hyperthermia (MH). malignant hyperthermia (MH).

• The goal is to be able to assess the patient preoperatively; The goal is to be able to assess the patient preoperatively; be able to recognize signs and symptoms of MH, institute be able to recognize signs and symptoms of MH, institute prompt and appropriate treatment, and provide appropriate prompt and appropriate treatment, and provide appropriate treatment post-crisis.treatment post-crisis.

• A MedELearn Test may be assigned to associates by the A MedELearn Test may be assigned to associates by the Education Department. A pass score of 80 % is required. Education Department. A pass score of 80 % is required.

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What is Malignant What is Malignant Hyperthermia (MH)?Hyperthermia (MH)?

• It is a rare, genetic autosomial-dominate, life threatening It is a rare, genetic autosomial-dominate, life threatening disorder.disorder.

• A hypermetablolic disorder of the skeletal muscle, which A hypermetablolic disorder of the skeletal muscle, which left untreated will result in death.left untreated will result in death.

• Usually triggered through the use of commonly used Usually triggered through the use of commonly used general inhalation anesthetics and succinylcholine.general inhalation anesthetics and succinylcholine.

• These triggering agents cause a series of chain reactions in These triggering agents cause a series of chain reactions in the body that increases intracellular calcium ion the body that increases intracellular calcium ion concentrations.concentrations.

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Who Gets It?Who Gets It?

Patients who have an autosomal mode of inheritance Patients who have an autosomal mode of inheritance and of these:and of these:

• Males more frequently than females.Males more frequently than females.

• Highest incidence of MH is ages 18 years and under, with Highest incidence of MH is ages 18 years and under, with 52% of all reactions occurring in kids under the age of 15.52% of all reactions occurring in kids under the age of 15.

• Patients who are obese or have muscular physiques have Patients who are obese or have muscular physiques have higher occurrences.higher occurrences.

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Perioperative Plan of Perioperative Plan of carecare for for Malignant Hyperthermia Malignant Hyperthermia PatientsPatients

• Assess the patient preoperatively.Assess the patient preoperatively.

• Be able to recognize signs and symptoms of MH.Be able to recognize signs and symptoms of MH.

• Institute prompt and appropriate treatment.Institute prompt and appropriate treatment.

• Appropriate treatment post-crisis.Appropriate treatment post-crisis.

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Assess the Patient Assess the Patient PreoperativelyPreoperatively

• All patients should be interviewed for MH not only the All patients should be interviewed for MH not only the patients susceptible of MH.patients susceptible of MH.

• During the interview a risk assessment for MH should During the interview a risk assessment for MH should include assessments on caffeine intolerance, personal include assessments on caffeine intolerance, personal history or family history of MH, or prior complications form history or family history of MH, or prior complications form previous anesthetics.previous anesthetics.

• If the patient answers “yes” to your interview questions If the patient answers “yes” to your interview questions notify both MD and Anesthesia.notify both MD and Anesthesia.

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Patient’s Personal HistoryPatient’s Personal History

During the Interview be aware of any other personal During the Interview be aware of any other personal history such as:history such as:

• Any unexplained fever.Any unexplained fever.

• Presence of Cola colored urine.Presence of Cola colored urine.

• History of muscle weakness or cramps or muscle group History of muscle weakness or cramps or muscle group hypertrophy.hypertrophy.

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Pediatric population and Pediatric population and Malignant Hyperthermia Malignant Hyperthermia prevalenceprevalence

Perioperative nurses should be aware of MH being Perioperative nurses should be aware of MH being more prevalent in the pediatric populations with more prevalent in the pediatric populations with these congenital conditions at the fore front:these congenital conditions at the fore front:

• Arthrogryposis – joint contracturesArthrogryposis – joint contractures

• Muscular dystrophys – (Becker’s, Duchenne’s)Muscular dystrophys – (Becker’s, Duchenne’s)

• Kyphoscoliosis – abnormal spine curvatureKyphoscoliosis – abnormal spine curvature

• Osteogenesis – brittle bone diseaseOsteogenesis – brittle bone disease

• Myotonia Congenita – neuromuscular disorderMyotonia Congenita – neuromuscular disorder

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Preoperative Testing for Preoperative Testing for Malignant HyperthermiaMalignant Hyperthermia

• Caffeine Halothane contracture test- only definitive Caffeine Halothane contracture test- only definitive diagnostic test.diagnostic test.

• This test requires the removal of a muscle from the thigh. It This test requires the removal of a muscle from the thigh. It is expensive and not usually covered by insurers. is expensive and not usually covered by insurers.

• DNA test-but not all patients susceptible to MH show DNA DNA test-but not all patients susceptible to MH show DNA change (mutation). This test can not yet replace the change (mutation). This test can not yet replace the Caffeine Test.Caffeine Test.

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Clinical ManifestationsClinical Manifestations

• Increased body temperature is a late sign.Increased body temperature is a late sign.

• Unexplained tachycardia.Unexplained tachycardia.

• Rapid rise of CO2 levels frequently Rapid rise of CO2 levels frequently exceeding 80mmHg.exceeding 80mmHg.

• Generalized muscle rigidity. Most prominent Generalized muscle rigidity. Most prominent is the masseter muscle of the jaw.is the masseter muscle of the jaw.

• Cyanotic or mottled skin.Cyanotic or mottled skin.

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Differential Diagnosis or What Differential Diagnosis or What Mimics Malignant Mimics Malignant HyperthermiaHyperthermia

• Cocaine toxicityCocaine toxicity

• Hypoxic encephalitisHypoxic encephalitis

• Intracranial traumaIntracranial trauma

• Light anesthesiaLight anesthesia

• SepsisSepsis

• Thyroid stormThyroid storm

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Known Triggering Anesthetic Known Triggering Anesthetic AgentsAgents

• HalothaneHalothane

• EnfluraneEnflurane

• IsofluraneIsoflurane

• DesfluraneDesflurane

• SevofluraneSevoflurane

• SuccinylcholineSuccinylcholine

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Required Equipment for Required Equipment for Malignant HyperthermiaMalignant HyperthermiaDesignated Anesthesia Machine Required for MH Cases Designated Anesthesia Machine Required for MH Cases

or if Not Available Then:or if Not Available Then:

• Before induction change out the lime soda.Before induction change out the lime soda.

• Flush the machine with 5000ml/minute of vapor free Flush the machine with 5000ml/minute of vapor free oxygen for 20 minutes.oxygen for 20 minutes.

• If detected during procedure then stop gases, change If detected during procedure then stop gases, change machine or flush the CO2 line with 100%.machine or flush the CO2 line with 100%.

• May continue to administer nitrous oxide.May continue to administer nitrous oxide.

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Intraoperative Anesthesia Intraoperative Anesthesia Medication PreparationsMedication Preparations

• Administer Dantrolene 2.5mg/kg IV given through a large bore Administer Dantrolene 2.5mg/kg IV given through a large bore needle until crisis is resolved.needle until crisis is resolved.

• Dantrolene is mixed with sterile water (no baterostatic agent) Dantrolene is mixed with sterile water (no baterostatic agent) 60ml with a 20mg vial, shake vigorously. Upper dosage is 60ml with a 20mg vial, shake vigorously. Upper dosage is 10mg/kg, more may be needed.10mg/kg, more may be needed.

• Titrate Dantrolene as necessary until tachycardia, Titrate Dantrolene as necessary until tachycardia, hyperthermia, hypercarbia and rigidity is resolved.hyperthermia, hypercarbia and rigidity is resolved.

• Dantrolene should be administered for 24hrs. Infusing Dantrolene should be administered for 24hrs. Infusing 1mg/kg/hr.1mg/kg/hr.

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Additional MedicationAdditional Medication

• Sodium bicarb to correct acidosis with initial dose 1 to 2 Sodium bicarb to correct acidosis with initial dose 1 to 2 mEq/kg and repeat as indicated.mEq/kg and repeat as indicated.

• To treat hyperkalemia in adults use 10U Regular insulin IV To treat hyperkalemia in adults use 10U Regular insulin IV and 50ml 50% glucose. Kids then 0.1 U Regular insulin/kg and 50ml 50% glucose. Kids then 0.1 U Regular insulin/kg 50% glucose.50% glucose.

• Life threatening Hyperkalemia then adults give calcium Life threatening Hyperkalemia then adults give calcium chloride 10mg/kg or 10 to 50mg/kg calcium gluconate and chloride 10mg/kg or 10 to 50mg/kg calcium gluconate and check glucose hourly.check glucose hourly.

• Administer standard antiarrhythmic agents for the Administer standard antiarrhythmic agents for the treatment of acidosis and hyperkalemia.treatment of acidosis and hyperkalemia.

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The Medications Not to GiveThe Medications Not to Give

• Bacteriostatic WaterBacteriostatic Water

• Calcium Channel Blockers - may increase hyperkalemia and Calcium Channel Blockers - may increase hyperkalemia and react to the dantrolene causing death.react to the dantrolene causing death.

• Avoid solutions containing potassium such as LR which may Avoid solutions containing potassium such as LR which may contribute to the hyperkalemia and acidosis. Delegate extra contribute to the hyperkalemia and acidosis. Delegate extra personnel for both meds, labs and cooling needs.personnel for both meds, labs and cooling needs.

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Implementing Implementing ThermoregulationThermoregulation• Apply cooling blanket.Apply cooling blanket.

• Infuse cold saline IV.Infuse cold saline IV.

• Apply ice packs to the head, axillae, groin and underneath Apply ice packs to the head, axillae, groin and underneath the patient.the patient.

• Maintain basins of ice water and apply cold wash cloths Maintain basins of ice water and apply cold wash cloths exposed body parts.exposed body parts.

• Discontinue cooling measures when body temperature Discontinue cooling measures when body temperature reaches 38 C or 100.4 F .reaches 38 C or 100.4 F .

• If an open procedure then apply cold irrigation solution to If an open procedure then apply cold irrigation solution to the body cavity.the body cavity.

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Monitoring Monitoring ThermoregulationThermoregulation

• Standard Monitors include EKG, cardiac output, HR ,BP, O2 Standard Monitors include EKG, cardiac output, HR ,BP, O2 SAT, SAT, Arterial Line, and End Title CO2.Arterial Line, and End Title CO2.

• Core temperature done through esophageal, tympanic, Core temperature done through esophageal, tympanic, axillary, rectally and bladder.axillary, rectally and bladder.

• Esophageal and rectal lavage.Esophageal and rectal lavage.

• Inspect skin integrity and apply protective measures to the Inspect skin integrity and apply protective measures to the skin. Preop assessment of tissue perfusion.skin. Preop assessment of tissue perfusion.

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Monitor Renal FunctionMonitor Renal Function

• Muscle breakdown caused elevations in creatine kinase Muscle breakdown caused elevations in creatine kinase (CK) which may cause renal insufficiency.(CK) which may cause renal insufficiency.

• Monitor CK and know that it may be normal at first and may Monitor CK and know that it may be normal at first and may not peak until 16hrs after the crisis.not peak until 16hrs after the crisis.

• If urine is less than 1mg/kg/hr give lasix to prevent If urine is less than 1mg/kg/hr give lasix to prevent myoglobin induced renal failuremyoglobin induced renal failure

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Laboratory StudiesLaboratory StudiesMalignant Hyperthermia Order Set Entry – lab tests Malignant Hyperthermia Order Set Entry – lab tests

performed to detect the following imbalances:performed to detect the following imbalances:

• Increased Potassium, Calcium, Magnesium and SodiumIncreased Potassium, Calcium, Magnesium and Sodium

• Prolonged PTT & PTProlonged PTT & PT

• Decreased PlateletsDecreased Platelets

• Increased CPK (measure every 6hrs until decreased), Increased CPK (measure every 6hrs until decreased), Creatine and LactateCreatine and Lactate

• Increased GlucoseIncreased Glucose

• ABGsABGs

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Primary RN Role:Primary RN Role:

The primary or lead RN will delegate duties as follows: The primary or lead RN will delegate duties as follows:

• Notify Charge Nurse / Supervisors / Pharmacy.Notify Charge Nurse / Supervisors / Pharmacy.

• Have two to four RNs for Dantrolene Administration.Have two to four RNs for Dantrolene Administration.

• RN to document/record (lab results, urine output and color, fluid RN to document/record (lab results, urine output and color, fluid intake, types of irrigation and amounts, line placements).intake, types of irrigation and amounts, line placements).

• RN to lavage. RN to lavage.

• Care Aids / Anesthesia Tech / Housekeeping to run for cold Care Aids / Anesthesia Tech / Housekeeping to run for cold supplies, running for lab, and running for invasive equipmentsupplies, running for lab, and running for invasive equipment

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Delegation of Duties Delegation of Duties

Specific Duties During The MH Crisis That Will Be Specific Duties During The MH Crisis That Will Be Delegated To Associates:Delegated To Associates:

• Running for supplies not on the MH cart: Insulin in the Running for supplies not on the MH cart: Insulin in the fridge, Iced IV bags, cold IV solutions, cold irrigations.fridge, Iced IV bags, cold IV solutions, cold irrigations.

• Equipment for art. lines and 2 - 4 infusion pumps.Equipment for art. lines and 2 - 4 infusion pumps.

• Bringing deliberator into the room.Bringing deliberator into the room.

• Changing the anesthesia machine if directed by MD Changing the anesthesia machine if directed by MD Anesthesia or CRNAAnesthesia or CRNA

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Patient Susceptibility Without a Patient Susceptibility Without a Reaction: Post Acute PhasesReaction: Post Acute PhasesIn the Post Acute Phases for a patient who was susceptible to In the Post Acute Phases for a patient who was susceptible to

MH but did not react, the following is required: MH but did not react, the following is required:

• All these patients should be observed for at least 12 hours.All these patients should be observed for at least 12 hours.

• Sets up monitors including a chance for invasive monitors Sets up monitors including a chance for invasive monitors and assures crash cart is close.and assures crash cart is close.

• Prepares containers of ice for the possible need.Prepares containers of ice for the possible need.

• Counsel patient and family on MH and further precautions. Counsel patient and family on MH and further precautions. Refer them to MHAUS (Malignant Hyperthermia Association Refer them to MHAUS (Malignant Hyperthermia Association of the United States); the MHAUS Hotline Number (800) of the United States); the MHAUS Hotline Number (800) 644-9737 (the Malignant Hyperthermia Association of the 644-9737 (the Malignant Hyperthermia Association of the United States) can be reached 24 hours a day.United States) can be reached 24 hours a day.

• Educate patient on muscle biopsy follow-up.Educate patient on muscle biopsy follow-up.

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Post Acute Malignant Post Acute Malignant Hyperthermia PhaseHyperthermia Phase• Observe in ICU for at least 24hours due to the risk of Observe in ICU for at least 24hours due to the risk of

recrudescence.recrudescence.

• Maintenance dantrolene 1mg/kg or 0.25mg/kg/hr by Maintenance dantrolene 1mg/kg or 0.25mg/kg/hr by infusion for at least 24hrs. Further doses may be indicated.infusion for at least 24hrs. Further doses may be indicated.

• Frequent ABGs, and CK every 6hrs.Frequent ABGs, and CK every 6hrs.

• Monitor serum and urine pHMonitor serum and urine pH

• Physician follow-up with education on MHAUS as well as Physician follow-up with education on MHAUS as well as muscle bx. muscle bx.

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References:References:

1.1. American Society of Anesthesiologits (2012). American Society of Anesthesiologits (2012). http://www.asahq.org/. .

2.2. Malignant Hyperthermia Association of the United States Malignant Hyperthermia Association of the United States (2012). (2012). http://www.mhaus.org/. .