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MH

Dr. Henry Rosenberg
Introduction
 Question patients about MH:
o Unexpected death
o Waking up “packed in ice”
o Readmission to hosp with signs of muscle breakdown after anesthesia
o Pain
o Brown or cola colored urine without exposure to anesthesia
 25% will experience recrudescence
 must be in icu for 36 hrs
 children/siblings 50/50 chance
 It is not wrong to admin dantrolene before definitive diagnosis
 Better to give early
 If MH develops
o Prepare dantrolene
o Obtain blood specimens
o Cool the patient if hyperthermic
 S/s
o Muscle rigidity (mild)
o Increased HR (gradual increase)
o Rapidly elevating ETCO2 (or gradual increase)
o Heart rate irregularity
o Rapidly rising body temp
 Once a patient enters the rapidly progressive stage:
o Sign accelerate
o Life threatening events within minutes
 Only a few moments to begin treatment with dantrolene
 Dantrolene must be available within 5 minutes

What is MH
 Inherited disorder
 Underlying problem: hypermetabolism
o Increased HR, RR and depth, body temp, sweating
o Normal body responses do not spin out of control
 Acid/base balance is disturbed
 K leaks out of cells
 Skeletal or voluntary muscle is the site during the crisis
 increased metabolism = increased acidity  acid leaks into the blood stream  lowers pH  affects
pumping ability of the heart
 Dantrolene
o Halts calcium release
o Ends MH reaction
o Initial dose 2.5 mg/kg quickly IV, may be repeated multiple times until reaction is under
control
o Comes 20 mg dantrolene, 3 grams of mannitol, mix with 60 mL of sterile water
o 1 mg/kg every 4-6 hrs for 24 hrs
 may be given longer if CK levels have not decreased

difficult to differentiate from MH (appendectomy example) o Rapid absorption of CO2 during laparoscopic procedures o Masseter muscle tightness with succs.stuck flow valve allowing rebreathing to occur. cardiac arrest  Masseter rigidity (MMR) may be an important warning sign o Jaw tension or jaws of steel after succs o Transient lasts less than 5 mins Discontinue elective surgery Switch to non triggering tech begin MH therapy Generalized rigidity: MH assured Most pts will develop rhabdo and myoglobinuria Pt hospitalized for 24 hrs  Increased RR secondary to hypercarbia  Hyperkalemia manifested by arrhythmia.occurs in postop period. misplaced LMA o Hyperthyroidism.muscle rigidity is not observed o Rhabdo. and other drugs involved May present at any time during the anesthetic even in the post op period Grading scale: o CO2 levels o Muscle breakdown o Temp elevation o Increased acidity and creatine kinase o High score = higher MH likelihood o Guide not definitive MH Outside the OR  Occasional reports of people with MH family history dying in “stressful” situations  Awake MH rare  < 20 cases reported in the US MH presentation  May resemble other syndromes  May develop immediately or gradually  if signs go unrecognized: o rapid decompensation resulting in hyperthermia.     Pt must be observed in ICU for 24 hrs since 25% of patients get MH again Labs o ABGs: Acid/base balance must be maintained every 6 hrs o K must be normal o Coag studies to see if bleeding o CK and myoglobin for detecting muscle breakdown every 6 hrs  Consult nephrologist concerning alkalinization of urine Factors affecting severity: o Duration. peaked T waves  immediately treat with insulin. HTN . acidosis.  O2 desat is unusual unless O2 stores are used up from hypermetabolism  Mottling skin due to high levels of catacholamines  Resemble o Sepsis. duchenne muscular dystrophy o Stimulants prior to anesthesia: tachycardia. muscle pressure with vascular compromise o Cardiac arrest in a young male patient: hyperkalemia should be suspected and treated. depth.increased muscle tone mistaken for jaws of steel o Equipment issues. hypercarbia.

6 fold per 3 mins delayed Mixing dantrolene  Diluent: sterile water ONLY – 60 mL  Rapid onset… less than 10 mins  Want to see a decrease in ETCO2  If muscle rigidity is present. If the MH episode is proceeding rapidly. the skeletal muscle. consider other causes of hypercarbia  If mix with saline  undesirable results  orange cloudy solution with insoluble particles. simply mix and inject. and cooling to target core temperature 38°C. amt of dantrolene soln per ml is reduced by 40% MH cart   Have at least 36 vials of dantrolene available Drugs o Therapy should be aimed at prompt treatment of hyperkalemia. not bags.  Do not use cold water o Sodium bicarbonate (8. We advise that the sterile water be stored in 100 ml vials. o Sterile water for injection USP (without a bacteriostatic agent) – Each vial of dantrolene should be reconstituted by adding 60 ml of sterile water for injection USP (without a bacteriostatic agent) and the vial shaken until the solution is clear. It is mandatory to get dantrolene to its effective site. should go away  HR should decrease and acidosis should resolve  Repeat doses until the desired effects  If no improvement. o Dantrolene – 36 vials should be available in each institution where MH can occur. hyperventilation. administration of dantrolene. each to be diluted at the time of use with 60 ml sterile water for injection USP (without a bacteriostatic agent). The post acute phase: ICU. predetermine MH transfer protocol. to avoid accidental IV administration of this hypotonic solution. see dantrolene stuff above MH and muscle disorders  Disorder of skeletal muscle  1 in 2000  1 in 15000 – 50000 anesthetics  Mortality 5%  Usual victim is young and healthy  Incidence higher in males  Mortality 20% higher when episode takes place out of a hospital  MH mortality o Age o gender o concomitant drug admin o Comorbidities  Pretreatment with dantrolene is not recommended  Risk of complications 1.4%) – 50 ml x 5 o For treatment of hyperkalemia  Dextrose 50% – 50 ml vials x 2  Calcium chloride (10%) –10 ml vial x 2 .

this may result in asystole. 24G. 20G. ACLS protocols.g. brown or red urine and heme positive dipstick) indicates that renal protection is mandated. o Urine collection container for myoglobin level.(60 ml x 2) with adapter for NG irrigation Monitoring Equipment o Esophageal or other core (e. CBC.5 Prepare anesthesia machine:  Ensure that anesthetic vaporizers are disabled by removing.000 ml of for IV cooling o * Lidocaine or procainamide should not be given if a wide-QRS complex arrhythmia is likely due to hyper-kalemia. 2-inch. i. rectal.g . bladder.(60 ml x 5) to dilute dantrolene o Intravenous catheters . o Blood specimen tubes for CK. Regular insulin – 100 units/ml x 1 (refrigerated) Lidocaine* for injection (2%) – 100 mg/5 ml or 100 mg/10 ml in preloaded syringes (3). General Equipment o Syringes . fibrin split products. pulmonary artery catheter) temperature probes o CVP kits (sizes appropriate to your patient population) o Transducer kits for arterial and central venous cannulation Nursing Supplies o Large sterile Steri-Drape (for rapid drape of wound) o Urine meter x 1 o Irrigation tray with piston (60cc irrigation) syringe o Large clear plastic bags for ice x 4 o Small plastic bags for ice x 4 o Bucket for ice o Test strips for urine analysis o Ten 60 mL Luer lock syringes o Toomy irrigation syringes for NGT w/ cold 0.16G.. or taping in the “OFF” position. Pigrnenturia (e. the coloration is due to red cells in the sample. thyroid studies). nasopharyngeal. 18G. and lactate. as prescribed by the AHA. o     Start active cooling if > 38. platelets. . would be followed when treating all cardiac derangements caused by MH. SMA 19 (LDH. samples should be kept on ice for later analysis. tympanic membrane.e. fibrinogen. Blood cultures are very useful and should be included to rule out bacteremia. Amiodarone (3 mL vial x 3) is also acceptable. If no immediate laboratory analysis is available. This may well prove useful on retrospective review and diagnosis. and the sample shows clear supernatant. 1-inch. o Lasix 40 mg per o Refrigerated cold saline solution – A minimum of 3. electrolytes.(sizes appropriate for your patient population) o Toomy irrigation syringes . ISTAT with TB syringes (the point of care ISTAT device has replaced lab blood gene and electrolyte measurement). 22G.. when the urine is centrifuged or allowed to settled. myoglobin. PT/PTT. 3/4-inch (4 each) (for IV access and arterial line) o NG tubes .9 NS o Temp monitors (do not rely on skin temp) o Blood pump infusion set Laboratory Testing Supplies o Syringes (3 ml) for blood gas analysis or ABG kits x 6 or point of care monitors.

consult manufacturer’s information and information on the MHAUS website. the filters should be replaced every hour. unused breathing bag should be attached to the Y-piece of the circle system and the ventilator set to inflate the bag periodically. Safe Anesthetic Agents for MH Patients:  Barbiturates / Intravenous Anesthetics o Diazepam o Etomidate (Amidate) o Hexobarbital o Ketamine (Ketalar) o Methohexital (Brevital) o Midazolam o Pentobarbital o Propofol (Diprivan) o Thiopental (Pentothal)  Inhaled Non-Volatile General Anesthetic o Nitrous Oxide  Local Anesthetics o Amethocaine o Articaine o Bupivicaine o Dibucaine . Some Hotline consultants recommend changing CO2 absorbent (soda lime or baralyme).         Most vaporizers have a significant reservoir of anesthetic that cannot be drained. Changing the CO2 absorbent (soda lime or baralyme) is not recommended if these procedures are followed. Use the expired gas analyzer to confirm absence of volatile gases. Not safe for use in MH-susceptible patients. Flow 10 L/min O2 through circuit via the ventilator for at least 20 minutes (refer to the consensus statement on page 36). the following anesthetic agents are known triggers of MH:  Inhaled General Anesthetics  Desflurane  Enflurane  Ether  Halothane  Isoflurane  Methoxyflurane  Sevoflurane  Succinylcholine (warning) All other anesthetic agents outside of these two categories of Volatile anesthetic agents and depolarizing muscle relaxants are considered safe. thus draining is not an acceptable choice. as some newer machines are not so easily cleaned of volatile agents. Newer anesthesia “work stations” may require up to 60 minutes for purging residual gases. Use new or disposable breathing circuit. Adding commercially available charcoal filters to the circuit will remove anesthetic gases and therefore obviate the need for purging the system as described However. During this time a disposable.

   o Etidocaine o Eucaine o Lidocaine (Xylocaine) o Levobupivacaine o Mepivicaine (Carbocaine) o Procaine (Novocain) o Prilocaine (Citanest) o Ropivacaine o Stovaine o Proparacaine Hydrochloride ALCAINE® o (proparacaine hydrochloride ophthalmic solution) Narcotics (Opioids) o Alfentanil (Alfenta) o Anileridine o Codeine (Methyl Morphine) o Diamorphine o Fentanyl (Sublimaze) o Hydromorphone (Dilaudid) o Meperidine (Demerol) o Methadone o Morphine o Naloxone o Oxycodone o Phenoperidine o Remifentanil o Sufentanil (Sufenta) Safe Muscle Relaxants o Arduan (Pipecuronium) o Curare (The active ingredient is Tubocurraine) o Gallamine o Metocurine o Mivacron (Mivacurium) o Neuromax (Doxacurium) o Nimbex (Cisatracurium) o Norcuron (Vecuronium) o Pavulon (Pancuronium) o Tracrium (Atracurium) o Zemuron (Rocuronium) Anxiety Relieving Medications o Ativan (Lorazepam) o Centrax o Dalmane (Flurazepam) o Halcion (Triazolam) o Klonopin o Librax o Librium (Chlordiazepoxide) o Midazolam (Versed) o Paxil (paroxetine) o Paxipam (Halazepam) .

o o o o Restoril (Temazepam) Serax (Oxazepam) Tranxene (Clorazepate) Valium (Diazepam) .