1 Malignant Hyperthermia Presented By: St. James Healthcare Education Collaborative With the support of: Surgical Services Leadership Team June 2012

2 Target Audience & Objectives Target audience is all associates who care for surgical patients undergoing general anesthesia and at risk for malignant hyperthermia (MH). Target audience is all associates who care for surgical patients undergoing general anesthesia and at risk for malignant hyperthermia (MH). The goal is to be able to assess the patient preoperatively; be able to recognize signs and symptoms of MH, institute prompt and appropriate treatment, and provide appropriate treatment post- crisis. The goal is to be able to assess the patient preoperatively; be able to recognize signs and symptoms of MH, institute prompt and appropriate treatment, and provide appropriate treatment post- crisis. A MedELearn Test may be assigned to associates by the Education Department. A pass score of 80 % is required. A MedELearn Test may be assigned to associates by the Education Department. A pass score of 80 % is required.

3 What is Malignant Hyperthermia (MH)? It is a rare, genetic autosomial-dominate, life threatening disorder. It is a rare, genetic autosomial-dominate, life threatening disorder. A hypermetablolic disorder of the skeletal muscle, which left untreated will result in death. A hypermetablolic disorder of the skeletal muscle, which left untreated will result in death. Usually triggered through the use of commonly used general inhalation anesthetics and succinylcholine. Usually triggered through the use of commonly used general inhalation anesthetics and succinylcholine. These triggering agents cause a series of chain reactions in the body that increases intracellular calcium ion concentrations. These triggering agents cause a series of chain reactions in the body that increases intracellular calcium ion concentrations.

4 Who Gets It? Patients who have an autosomal mode of inheritance and of these: Males more frequently than females. Males more frequently than females. Highest incidence of MH is ages 18 years and under, with 52% of all reactions occurring in kids under the age of 15. Highest incidence of MH is ages 18 years and under, with 52% of all reactions occurring in kids under the age of 15. Patients who are obese or have muscular physiques have higher occurrences. Patients who are obese or have muscular physiques have higher occurrences.

5 Perioperative Plan of care for Malignant Hyperthermia Patients 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.

6 Assess the Patient Preoperatively All patients should be interviewed for MH not only the patients susceptible of MH. All patients should be interviewed for MH not only the patients susceptible of MH. During the interview a risk assessment for MH should include assessments on caffeine intolerance, personal history or family history of MH, or prior complications form previous anesthetics. During the interview a risk assessment for MH should include assessments on caffeine intolerance, personal history or family history of MH, or prior complications form previous anesthetics. If the patient answers “yes” to your interview questions notify both MD and Anesthesia. If the patient answers “yes” to your interview questions notify both MD and Anesthesia.

7 Patient’s Personal History During the Interview be aware of any other personal 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 hypertrophy. History of muscle weakness or cramps or muscle group hypertrophy.

8 Pediatric population and Malignant Hyperthermia prevalence Perioperative nurses should be aware of MH being more prevalent in the pediatric populations with these congenital conditions at the fore front: Arthrogryposis – joint contractures Arthrogryposis – joint contractures Muscular dystrophys – (Becker’s, Duchenne’s) Muscular dystrophys – (Becker’s, Duchenne’s) Kyphoscoliosis – abnormal spine curvature Kyphoscoliosis – abnormal spine curvature Osteogenesis – brittle bone disease Osteogenesis – brittle bone disease Myotonia Congenita – neuromuscular disorder Myotonia Congenita – neuromuscular disorder

9 Preoperative Testing for Malignant Hyperthermia Caffeine Halothane contracture test- only definitive diagnostic test. Caffeine Halothane contracture test- only definitive diagnostic test. This test requires the removal of a muscle from the thigh. It is expensive and not usually covered by insurers. This test requires the removal of a muscle from the thigh. It is expensive and not usually covered by insurers. DNA test-but not all patients susceptible to MH show DNA change (mutation). This test can not yet replace the Caffeine Test. DNA test-but not all patients susceptible to MH show DNA change (mutation). This test can not yet replace the Caffeine Test.

10 Clinical 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. Generalized muscle rigidity. Most prominent Generalized muscle rigidity. Most prominent is the masseter muscle of the jaw. Cyanotic or mottled skin. Cyanotic or mottled skin.

11 Differential Diagnosis or What Mimics Malignant Hyperthermia Cocaine toxicity Cocaine toxicity Hypoxic encephalitis Hypoxic encephalitis Intracranial trauma Intracranial trauma Light anesthesia Light anesthesia Sepsis Sepsis Thyroid storm Thyroid storm

12 Known Triggering Anesthetic Agents Halothane Halothane Enflurane Enflurane Isoflurane Isoflurane Desflurane Desflurane Sevoflurane Sevoflurane Succinylcholine Succinylcholine

13 Required Equipment for Malignant Hyperthermia Designated Anesthesia Machine Required for MH Cases 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 oxygen for 20 minutes. Flush the machine with 5000ml/minute of vapor free oxygen for 20 minutes. If detected during procedure then stop gases, change machine or flush the CO2 line with 100%. If detected during procedure then stop gases, change machine or flush the CO2 line with 100%. May continue to administer nitrous oxide. May continue to administer nitrous oxide.

14 Intraoperative Anesthesia Medication Preparations Administer Dantrolene 2.5mg/kg IV given through a large bore needle until crisis is resolved. Administer Dantrolene 2.5mg/kg IV given through a large bore needle until crisis is resolved. Dantrolene is mixed with sterile water (no baterostatic agent) 60ml with a 20mg vial, shake vigorously. Upper dosage is 10mg/kg, more may be needed. Dantrolene is mixed with sterile water (no baterostatic agent) 60ml with a 20mg vial, shake vigorously. Upper dosage is 10mg/kg, more may be needed. Titrate Dantrolene as necessary until tachycardia, hyperthermia, hypercarbia and rigidity is resolved. Titrate Dantrolene as necessary until tachycardia, hyperthermia, hypercarbia and rigidity is resolved. Dantrolene should be administered for 24hrs. Infusing 1mg/kg/hr. Dantrolene should be administered for 24hrs. Infusing 1mg/kg/hr.

15 Additional Medication Sodium bicarb to correct acidosis with initial dose 1 to 2 mEq/kg and repeat as indicated. Sodium bicarb to correct acidosis with initial dose 1 to 2 mEq/kg and repeat as indicated. To treat hyperkalemia in adults use 10U Regular insulin IV and 50ml 50% glucose. Kids then 0.1 U Regular insulin/kg 50% glucose. To treat hyperkalemia in adults use 10U Regular insulin IV and 50ml 50% glucose. Kids then 0.1 U Regular insulin/kg 50% glucose. Life threatening Hyperkalemia then adults give calcium chloride 10mg/kg or 10 to 50mg/kg calcium gluconate and check glucose hourly. Life threatening Hyperkalemia then adults give calcium chloride 10mg/kg or 10 to 50mg/kg calcium gluconate and check glucose hourly. Administer standard antiarrhythmic agents for the treatment of acidosis and hyperkalemia. Administer standard antiarrhythmic agents for the treatment of acidosis and hyperkalemia.

16 The Medications Not to Give Bacteriostatic Water Bacteriostatic Water Calcium Channel Blockers - may increase hyperkalemia and react to the dantrolene causing death. Calcium Channel Blockers - may increase hyperkalemia and react to the dantrolene causing death. Avoid solutions containing potassium such as LR which may contribute to the hyperkalemia and acidosis. Delegate extra personnel for both meds, labs and cooling needs. Avoid solutions containing potassium such as LR which may contribute to the hyperkalemia and acidosis. Delegate extra personnel for both meds, labs and cooling needs.

17 Implementing Thermoregulation Apply cooling blanket. Apply cooling blanket. Infuse cold saline IV. Infuse cold saline IV. Apply ice packs to the head, axillae, groin and underneath the patient. Apply ice packs to the head, axillae, groin and underneath the patient. Maintain basins of ice water and apply cold wash cloths exposed body parts. Maintain basins of ice water and apply cold wash cloths exposed body parts. Discontinue cooling measures when body temperature reaches 38 C or F. Discontinue cooling measures when body temperature reaches 38 C or F. If an open procedure then apply cold irrigation solution to the body cavity. If an open procedure then apply cold irrigation solution to the body cavity.

18 Monitoring Thermoregulation Standard Monitors include EKG, cardiac output, HR,BP, O2 SAT, Standard Monitors include EKG, cardiac output, HR,BP, O2 SAT, Arterial Line, and End Title CO2. Core temperature done through esophageal, tympanic, axillary, rectally and bladder. Core temperature done through esophageal, tympanic, axillary, rectally and bladder. Esophageal and rectal lavage. Esophageal and rectal lavage. Inspect skin integrity and apply protective measures to the skin. Preop assessment of tissue perfusion. Inspect skin integrity and apply protective measures to the skin. Preop assessment of tissue perfusion.

19 Monitor Renal Function Muscle breakdown caused elevations in creatine kinase (CK) which may cause renal insufficiency. Muscle breakdown caused elevations in creatine kinase (CK) which may cause renal insufficiency. Monitor CK and know that it may be normal at first and may not peak until 16hrs after the crisis. Monitor CK and know that it may be normal at first and may not peak until 16hrs after the crisis. If urine is less than 1mg/kg/hr give lasix to prevent myoglobin induced renal failure If urine is less than 1mg/kg/hr give lasix to prevent myoglobin induced renal failure

20 Laboratory Studies Malignant Hyperthermia Order Set Entry – lab tests performed to detect the following imbalances: Increased Potassium, Calcium, Magnesium and Sodium Increased Potassium, Calcium, Magnesium and Sodium Prolonged PTT & PT Prolonged PTT & PT Decreased Platelets Decreased Platelets Increased CPK (measure every 6hrs until decreased), Creatine and Lactate Increased CPK (measure every 6hrs until decreased), Creatine and Lactate Increased Glucose Increased Glucose ABGs ABGs

21 Primary RN Role: 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 intake, types of irrigation and amounts, line placements). RN to document/record (lab results, urine output and color, fluid intake, types of irrigation and amounts, line placements). RN to lavage. RN to lavage. Care Aids / Anesthesia Tech / Housekeeping to run for cold supplies, running for lab, and running for invasive equipment Care Aids / Anesthesia Tech / Housekeeping to run for cold supplies, running for lab, and running for invasive equipment

22 Delegation of Duties Specific Duties During The MH Crisis That Will Be Delegated To Associates: Running for supplies not on the MH cart: Insulin in the fridge, Iced IV bags, cold IV solutions, cold irrigations. Running for supplies not on the MH cart: Insulin in the fridge, Iced IV bags, cold IV solutions, cold irrigations. Equipment for art. lines and infusion pumps. Equipment for art. lines and infusion pumps. Bringing deliberator into the room. Bringing deliberator into the room. Changing the anesthesia machine if directed by MD Anesthesia or CRNA Changing the anesthesia machine if directed by MD Anesthesia or CRNA

23 Patient Susceptibility Without a Reaction: Post Acute Phases In the Post Acute Phases for a patient who was susceptible to 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 and assures crash cart is close. Sets up monitors including a chance for invasive monitors 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. Refer them to MHAUS (Malignant Hyperthermia Association of the United States); the MHAUS Hotline Number (800) (the Malignant Hyperthermia Association of the United States) can be reached 24 hours a day. Counsel patient and family on MH and further precautions. Refer them to MHAUS (Malignant Hyperthermia Association of the United States); the MHAUS Hotline Number (800) (the Malignant Hyperthermia Association of the United States) can be reached 24 hours a day. Educate patient on muscle biopsy follow-up. Educate patient on muscle biopsy follow-up.

24 Post Acute Malignant Hyperthermia Phase Observe in ICU for at least 24hours due to the risk of recrudescence. Observe in ICU for at least 24hours due to the risk of recrudescence. Maintenance dantrolene 1mg/kg or 0.25mg/kg/hr by infusion for at least 24hrs. Further doses may be indicated. Maintenance dantrolene 1mg/kg or 0.25mg/kg/hr by 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 pH Monitor serum and urine pH Physician follow-up with education on MHAUS as well as muscle bx. Physician follow-up with education on MHAUS as well as muscle bx.

25 References: 1. American Society of Anesthesiologits (2012) Malignant Hyperthermia Association of the United States (2012).