Malignant Hyperthermia Association of the United States and theNorth American Malignant Hyperthermia Registry of MHAUS

Volume 26 Number 3Summer 2008

The Communicator

Although great strides have been made in therecognition and effective management of a malignant hyper-thermia (MH) event, the recent death of an 18-year-old inSouth Florida (see article page 3) reminds us that our job toeducate everyone about MH is not yet complete.

While MH does not happen often, prompt recogni-tion and treatment is key to a successful outcome; one MHdeath is one too many!

One of the best methods used to combat this uncom-mon disorder with the propensity to destroy lives is throughholding an annual MH Mini-Conference. This year’s locationis being coordinated at the Tampa General Hospital in Tampa,FL, and is scheduled for September 27 and 28, 2008. Themini-conference is a great way for patients and medicalprofessionals to get up-to-date information and direct “hands-on” experience with the intricacies of dealing with MH byparticipating in a mock MH drill.

Our speakers include MHAUS President HenryRosenberg, MD, CPE, Saint Barnabas Medical Center inLivingston, NJ. We are also privileged to have Deanna Steele,a genetic counselor from Magee Womens Hospital Center forMedical Genetics in Pittsburgh, PA. Ms. Steele will provide aclearer understanding of the role genetics can play in theclarification of MH for both the patient and the medicalprofessional. Also speaking is Mohanad Shukry, MD,Childrens Hospital of Oklahoma, OK, Assistant Professor ofAnesthesiology at University of Oklahoma and a MH HotlineConsultant. The plan is to repeat the conference topics onboth days so those who are unable to make the Saturday datewill have opportunity to join us on Sunday.

There will be discussion on how to recognize MH,the initial signs to watch for, and time set aside for themedical professionals and MH-susceptibles to share experi-ences and brainstorm ways to improve the way they commu-nicate with one another (see article page 4). Ample time for

MH Mini-Conference Comes to Tampa,Florida, September 27 and 28

questions and answers will be an important feature of thepresentations as the goal is to send attendees home with abetter understanding of how to identify the signs of MH, reactquickly via a response plan that is in place and gain a clearerunderstanding of the various testing options MH-susceptibleshave available to them. The agenda for the conferenceincludes information MH-susceptible patients need to beaware of as well as specific tools they can use to share criticalfamily history with their anesthesia care provider. In addi-tion, plans include specific time to run through an actualsimulation of MH.

The mini-conference is coordinated by MHAUS’Patient Liaison Committee and on-site with the help of BrianCowin, RN, BSN of Critical Intervention Educators, Inc.,based in Tampa, which provides MH training and mock MHcodes, ACLS, PALS, and BLS to various outpatient surgicalfacilities throughout Florida and Georgia.

Join us for this opportunity to get answers to all yourquestions about MH and to experience the many simulta-neous tasks involved with caring for a patient experiencingMH, including mixingdantrolene, the antidote.

The cost toattend this valuableconference is $25.00 formedical professionals and$15.00 for MH-suscpetibles and theirfamilies. Registrationinformation is availableon the MHAUS website atwww.mhaus.org or bycalling the MHAUS officeat 607-674-7901.

In This Issue:

Speakers Bureau 2

Death of 18-Year-Old 3

What to Ask Your Doctor 4

Is Your Facility Prepared? 4

European MH Group 6

Meet Michael Wesolowski 7

Writing Award 8

Genetic Nondiscrimination 9

Werner Kalow 10

The Communicator is published four timeseach year by the Malignant HyperthermiaAssociation of the United States (MHAUS)and is made possible by a generous grantfrom Procter & Gamble Pharmaceuticals,manufacturers of Dantrium®. TheCommunicator is intended to serve theinformation needs of MH-susceptiblefamilies, health care professionals, andothers with an interest in MH.

EDITORBrian Kamsoke

Editorial Advisory PanelScott Schulman, M.D.Henry Rosenberg, M.D., CPEBarbara Brandom, M.D.Cynthia Wong, M.D.Lena Sun, M.D.Ronald J. Ziegler

FOR MHAUSHenry Rosenberg, M.D., CPEPresident

Ronald J. ZieglerVice President

Sheila Muldoon, M.D.Vice President, Scientific Development(MHAUS/NMSIS/Registry)

Stanley Caroff, M.D.Vice President – Director of NMSIS

Steven V. Napolitano, Esq.Secretary

Joseph R. Tobin, M.D.Treasurer

Dianne DaughertyExecutive Director

Gloria ArtistHotline Coordinator

Fay KelloggFulfillment Administrator

Nicole VieraAdministrative Assistant

Michael WesolowskiPublic Relations/Project Manager

Malignant Hyperthermia (MH) is aninherited muscle disorder which, whentriggered by potent inhalation anestheticsand succinylcholine, may cause a life-threatening crisis. The incidence of MH islow, but, if untreated, the mortality rate ishigh. Since the advent of the antidote drug,dantrolene sodium, and with greaterawareness of the syndrome, the mortalityrate has decreased. Great advances in ourunderstanding of MH have been made sinceit was first recognized in the early 1960s,but the nature of the fundamental defect(s)is still unknown. MHAUS advocates that all surgicalpatients undergoing general anesthesiashould receive continuous temperaturemonitoring, that adequate supplies ofdantrolene be stocked near the OR and thatthorough family histories be obtained.

Copyright 2008 by MHAUS

Speakers Bureau Helps You Prepare For MHA malignant hyperthermia (MH)

episode can happen at any time. Thebest way to protect your patients, andyour facility, is to be prepared before it’stoo late. MHAUS is ready to provideassistance through our Speakers Bureau.

MHAUS speakers are experts inMH. The most informed anesthesia careproviders in the world are affiliated withMHAUS as Hotline Consultants, mem-bers of the MHAUS Professional AdvisoryCouncil, or members of MHAUS Boardof Directors.

MH experts can come to yourfacility or meeting and speak to your staffabout malignant hyperthermia. MHexperts are ready to offer insight andsuggestions on the treatment regimen forMH should you find yourself facing apossible MH event. MHAUS SpeakersBureau answers your questions about thebasics of recognizing, diagnosing, andtreating MH.

The Speakers Bureau includes:❑ Speaker’s presentation on MH; but canalso include other topics unique to yourfacility❑ MH educational materials for up to 20attendees❑ Hands-on dantrolene mixing demon-stration❑ Mock MH drill event; what to do, andduties for individual staff members❑ Questions and answer session❑ In addition, the host receives: Alert

The Malignant Hyperthermia Association of the United States is a not-

for-profit organization dedicated to reducing the morbidity and mortal-

ity of malignant hyperthermia and other heat-related disorders by:

improving medical care related to MH; providing support information

for patients; and improving the scientific understanding and research

related to MH and other kinds of heat-related syndromes.

For more information or for materials on malignant hyperthermia or

MHAUS’ programs, call 607-674-7901; write MHAUS, PO Box 1069,

Sherburne, NY 13460; or visit us on the Internet at www.mhaus.org.

Band and Sticker Kits, Crisis Interven-tion Record, Dosage Chart, HotlineStickers, and MH Crisis Poster

The Speakers Bureau has giventalks or has talks pending as far south asFlorida as far west as California as fareast as Long Island and many places inbetween. Recent talks have taken placeat Disc Surgical Center in Marina DelRay, CA, (Dr. Jordan Miller), HalofaxMedical Center in Daytona Beach, FL,and Carolina Center for SpecialtySurgery in Charlotte, NC, (Dr. JosephTobin), Providence Hospital in Mobile,AL, (Dr. Mary Theroux), and TexasA&M College of Medicine in Temple,TX, (Dr. Gerald Gronert).

Talks are also scheduled for St.Mary’s Health Center in St. Louis, MO,by Dr. Theroux, and St. Vincent’sMedical Center in Bridgeport, CT, andBoulevard Surgical Center in LongIsland, NY, by Dr. Charlie Watson.Further talks are scheduled for Michi-gan, California, Wisconsin, Colorado,New Jersey, and Kansas.

To schedule a talk for yourfacility, contact Michael Wesolowski atthe MHAUS office at (607) 674-7901 oremail michael@mhaus.org.

Find out what you can do rightnow to keep your patients, and yourfacility, safer from MH emergencies.

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by Henry Rosenberg, MDMHAUS PresidentThe death of Stephanie Kuleba fromMH in March, 2008 during cosmeticsurgery in an office-based operatingroom was a striking reminder thatMH may occur wherever anesthesia isadministered. This event has causedanguish, pain and grief for the familyand friends of this young woman aswell as the providers of care. As aresult of this tragedy, Stephanie’sfamily has developed a website andcampaign to increase awareness ofMH. (www.stephaniejudekuleba.org)

One of the Kuleba family’sconcerns was that there was nodiscussion of the possibility of MHoccurring during surgery. Without afamily or personal history of MH, fewsurgeons or anesthesia providersinclude a description of this syn-drome when obtaining informedconsent for surgery. Certainly, unlessthere is an awareness of MH, fewpatients will ask their health careprofessional, “Are You Prepared forMH? MH Kills.”

Given that the data fromgenetic research shows that as manyas one in three thousand people arepotentially at risk for MH, it is timethat the blind spot regarding MHchanges. I believe that the promotionof preparedness and awareness of MHwill be assured when patients areasking the right questions.

Times are changing, andthere are many new initiatives in theinterest of patient safety which willaffect how patients communicate withtheir doctors. One of these initiativesis being spearheaded by The JointCommission (JC), the hospital andoutpatient center accrediting organi-zation. The JC has developed a“Speak Up” Campaign encouragingpatients to become actively involvedin medical decision-making forthemselves and their loved ones

(www.jointcommission.org/PatientSafety/SpeakUp/). Patients areencouraged to speak up, to not bereticent about asking challengingquestions, and to be open and honestwith their caregivers.

There is another majorinitiative (proposed regulation)underway in medical care that willcause providers to want to be awareof MH. Medicare and several insur-ance companies and states havedeveloped a list of medical events andnegative outcomes that will notqualify for reimbursement. These so-called “never events” include suchobvious problems as surgery on thewrong limb or leaving a foreign bodyin a patient during surgery. Of note:one of the listed events is intraopera-tive or immediately post-operativedeath in a normal, healthy patient(defined as aClass 1 patientfor purposes ofthe AmericanSociety ofAnesthesiolo-gists patientsafety initia-tive).

It sohappens thatthree MHdeaths in thepast two yearshave taken thelives of young

Death of 18-Year-Old a Harsh Reminder that MH Kills

Stephanie Kuleba

healthy individuals undergoingcosmetic surgery. Hence, this pro-posed regulation provides an impetusfor health care providers to askpatients the right questions, and itmay even encourage the providers tobe more receptive to patients’ ques-tions about MH.

Does this mean that deathfrom MH should never occur underany circumstances? Our expertclinicians agree that at this point, wedo not know enough about MH andits presentation(s) to make thisstatement. However, it is entirelyreasonable to claim that death fromMH in a patient who is known to beat risk for MH should not occur.

Because it is rare for there tobe outward signs of MH susceptibilityor a family history of MH, I believe allpatients about to undergo surgeryshould ask, “Are you Prepared forMH?” If the answer is a blank look,then ask more questions and consideralternatives.

By asking this question, youwill encourage all facilities usinggeneral anesthesia trigger agents tohave a full supply of dantrolene, awell-rehearsed protocol for treatingMH, and to spread the awareness ofthis potentially fatal disorder. (Seepage 4 for related articles).

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What Should You Ask Your Doctor Prior to Surgery?To assist you with your conversa-tion with your doctor, here is a listof some questions that should beanswered prior to you or yourloved one having surgery:

1.) Who will provide the anesthesiaand what is his or her qualifica-tions? A Physician Anesthesiolo-gist? A Nurse Anesthetist? AnAnesthesia Assistant? SedationNurse? Surgeon?

2.) Is the facility licensed by theState?

3.) Is the facility accredited by theJoint Commission or otheraccrediting agency?

4.) Is there a written protocol formanaging Malignant Hyperther-mia?

5.) Does the facility have 36 vials ofdantrolene?

6.) Does the facility have a cart orother location where additionalsupplies are kept to manage a caseof MH?

7.) Does the facility perform drills toprepare for MH each year? Haveall current employees beentrained?

8.) What hospital is back up for theambulatory center?

9.) Is there a written protocol fortransfer to a hospital in case ofemergency? How long does ittake to transfer?

10.) Does the facility perform bloodtests on site?

11.) How many physicians, nurses orother trained personnel areimmediately available to care fora patient in case of emergency?

12..) Is there a recovery area and is itstaffed by a nurse?

13.) Is there an anesthesia machinethat is used? If so, who main-tains the machine?

14.) Are patients undergoing generalanesthesia monitored by ECG(electrocardiogram)? Is oxygensaturation measured by using apulse oximeter? Is exhaledcarbon dioxide measured? Arethe concentrations of anesthesiagases monitored?

15.) Is body temperature measuredduring anesthesia? How?

16.) Is there a defibrillator present?17.) Are my records maintained by

the facility? For how long?

In addition, the patient’s obligationis to provide complete andadequate medical history. Besure to provide the following:

1.) Dose and frequency of all medica-tions that are taken.

2.) Mention all herbal medications andperformance-enhancing medica-tions that are being taken.

3.) Provide contact information foryour physician.

4.) List all allergies.5.) Describe any problems that you or

family members may have experi-enced during or after anesthesia.

6.) Describe any history of heat stroke.7.) Describe any personal or family

history of muscle disorders.8.) Describe any history of muscle

weakness, severe muscle pain afteranesthesia.

9.) Describe any history of brown ordark urine following anesthesia andsurgery, or occurring spontaneouslywith exercise.

Editor’s Note: The opinions expressed here areDr. Rosenberg’s and do not necessarilyrepresent the opinions of our Hotline Consult-ants or Professional Advisory Council.Comments can be addressed tohenryrosenberg@yahoo.com

Is Your Facility Prepared for an MH Emergency?by Henry Rosenberg, MDMHAUS President

The recent publicity concerning thedeath of a young patient from malig-nant hyperthermia (MH) in a Floridaoutpatient surgery facility reminds usthat this syndrome can appearsuddenly and explosively whenevergeneral anesthesia is administeredand that deaths still occur from MH

In this case, the MH episodebegan during cosmetic surgery in anoffice-based operating room. Eventhough MH was recognized after ithad begun some time during theprocedure and some amount of

dantrolene was administered, by thetime the patient was moved to thenearby emergency room, her bodytemperature was elevated to such anextent that DIC and death was inevi-table.

I would like to point out thatalmost exactly one year ago a youngwoman expired under almost exactlythe same circumstances in the LosAngeles area. Not to be an alarmist,we do hear regularly of patients whodevelop MH in the ambulatory orhospital setting who are treated andsurvive.

According to the latest articleon the risk of death from MH just

published in the April 2008 issue ofAnesthesiology based on data accumu-lated in the North American MHRegistry of MHAUS, the mortalityfrom a MH is in the range of 2%.(Cardiac Arrests and Deaths Associatedwith Malignant Hyperthermia in NorthAmerica from 1987 to 2006: A Reportfrom The North American MalignantHyperthermia Registry of the MalignantHyperthermia Association of the UnitedStates. Marilyn Green Larach, MD,FAAP.; Barbara W. Brandom, MD;Gregory C. Allen, MD, FRCPC.;Gerald A. Gronert, MD; Erik B.

Continued on page 5

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Lehman, MS, Anesthesiology 108;603-611,2008)

So, while MH does nothappen often and prompt recognitionand treatment is the key to a success-ful outcome, nevertheless, mortalityfrom MH still does occur. Further-more many of the patients who havea bad outcome are usually healthyyoung adults. This problem is farfrom solved.

❑ For any center or facility whereMH trigger agents are used a fullsupply of 36 vials of dantrolene withthe diluent of preservative-free sterilewater for injection should be avail-able within five minutes.

❑ A treatment plan needs to be inplace such as the posters, policymanual or other printed materialavailable from MHAUS.

❑ The telephone number for the MHHotline should also be immediatelyavailable.

❑ A drill simulating an MH caseshould be carried out once per year.It is important that this involve notonly anesthesia personnel but ORpersonnel as well, because themanagement of MH is a team effort.

❑ Identified personnel, who have theauthorization, should go through thedrill of mixing dantrolene. Gettingthe drug into solution takes time andmay take more than one person tomix an adequate dose. The recom-mended starting dose is 2.5mg/kg.For the average adult who nowweighs about 80 kg, that means 10vials of dantrolene.

❑ The team should NOT waste timechanging anesthesia machines oncethe crisis has been diagnosed. Rather,increase the fresh gas flow rates

maximally and discontinue all volatileagents.

❑ The full initial dose of dantroleneshould be administered and subse-quent doses titrated to heart rate, endtidal carbon dioxide and musclerigidity.

❑ Patients who develop MH willneed dantrolene treatment for 36hours to avoid recrudescence.

❑ If there is evidence of peaked T-waves on the ECG, calcium, glucoseand insulin should be used to lowerpotassium levels. Better still, ifpossible, measure potassium levels.

❑ In the event of a MH crisis, moni-tor core temperature (esophageal,nasopharyngeal, tympanic) probe inaddition to any other temperaturemonitor.

❑ If the patient is hyperthermic s/hemay have to have bags of ice placedon the body surface, particularly theneck and groin area. Lavage of thestomach with iced saline may also benecessary in cases of marked hyper-thermia.

If you are in a non-hospital set-tings, there are some specialissues:Make sure that the hospital where thepatient will be transferred knowsabout MH, that the ED physicianshave dantrolene available or at leastknow the basics of treating thedisorder.

Although it is tempting tomove the patient as rapidly as pos-sible to the hospital setting, the 20 ormore minutes that will take under thebest of circumstances may lead to theMH event spiraling out of control. Itis very difficult to be definitive onthis issue, but in my opinion, thepatient should be stabilized, i.e., theETC02 near normal, temperature at

or below perhaps 101F, and tachycar-dia resolving before transfer. You mayhave to hold the EMS people at baywhile this is happening.

In addition, the most knowl-edgeable person(s) about MH shouldaccompany the patient, withdantrolene drawn up and ready toadminister and monitor the patient asthoroughly as possible.

Blood specimens for CK,potassium, coagulation profile shouldbe drawn and measured as soon aspossible.

Once stabilized, the patientshould be given information aboutMH through MHAUS. Either theHotline Consultant or one of themembers of the MH ProfessionalAdvisory Council will be able to giveadvice concerning recommendationfor testing and confirming the episode.

Over the past year or so, theMH Hotline Consultants and membersof the Professional Advisory Councilhave come to realize that our educa-tional efforts need to be expandedfrom the anesthesia and surgicalspecialties to include Emergency andICU physicians as well as EMS person-nel who need to recognize that dealingwith MH is not the same as dealingwith many of the crises that they havebeen trained to manage. We hope toexpand our training and educationinto those areas as soon as possible,but resources are always a problem.

If you value the services andinsight of MHAUS and the 27 HotlineConsultants who volunteer their timeas well as the members of the Profes-sional Advisory Council, do supportMHAUS by becoming a member and/or donor. Visit the MHAUS web sitewww.mhaus.org for further details.

Editor’s Note: The opinions expressed here areDr. Rosenberg’s and do not necessarilyrepresent the opinions of our HotlineConsultants or Professional Advisory Council.Comments can be addressed tohenryrosenberg@yahoo.com

Continued from page 4

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by Henry Rosenberg, MDMHAUS President

Whenever I can, I try to attend themeeting of the European MH Group(EMHG). The group consists ofmany scientists and physicians whoare actively investigating MalignantHyperthermia and Central CoreDisease and their relation to othermuscle diseases. This year the meet-ing was held in the beautiful andcharming University town of Lund,Sweden, just south of Malmo whichis connected to Copenhagen by a newand extremely long bridge called theOresund Bridge.

The 50 or so participantscame from many European countries,Brazil, Australia, New Zealand, theU.S., and Canada. In some yearsattendees also come from the formerSoviet Republic and Asia, but not thisyear. Joining me from the U.S. wereDr. Barbara Brandom, the Director ofthe North American MH Registry andDr. Mary Theroux, a Hotline Consult-ant, and her colleague Dr. DivyaDixit from DuPont Children’s Hospi-tal in Delaware.

The meeting was hosted byDr. Gunilla Islander, a foundingmember of the EMHG, and Dr. KlausGlahn from Denmark. Matter of fact,this meeting celebrated the 25th yearof the founding of the EMHG and thefounding members were present:Richard Ellis from Leeds, HelleOrding, formerly from Copenhagen,Roger Fletcher from the UK, andGunilla Islander from Lund. Euro-pean hospitality was definitely inorder and greatly appreciated.

It was nice to be among aninternational group of scientists andphysicians committed to a betterunderstanding of MH and relateddisorders in the interest of better,safer patient care. I was impressedwith many of the presentations

related to the molecular genetics ofMH.

One of the most interestingwas presented by Dr. Natasha Kraevafrom Toronto. With the collaborationof the testing centers in Toronto andOttawa, they found that screening ofthe full length RYR-1 gene (the genemost clearly associated with MHsusceptibility) in 31 unrelated indi-viduals who were MH-susceptibles(25) or had Central Core Disease (6),mutations were found in 61% of thesamples. Seven of the 18 mutationswere new and predicted to be relatedto causing either MH or Central CoreDisease. This finding is similar tostudies done in the MH investigativeunits in Leipzig, Germany, Lund,Sweden and Leeds, UK.

Does this mean that thatthere are parts of the gene that havenot been explored and will havechanges that predispose to MH (e.g.,the so called non-coding section ofthe gene, the introns)? Or that thereare other genes that can predispose toMH (we know of one other, theDHPR gene, but that can account foronly a few percent of cases)? Ormight there be other genes thatinfluence the expression of the RYR1gene and be causal? Or is the biopsytest giving us false positives? Without

answers to these questions it will bedifficult to increase the sensitivity ofthe genetic test to make it a wide-spread screening test.

The MH group in Cologne,Germany, headed by Dr. FrankWappler, has been studying the effectof the drug Ecstasy on MH-suscep-tible swine. Ecstasy is an amphet-amine-like abuse drug that leads, incertain situations, such as “rave”parties, to high body temperature,acid base disturbance, and musclebreakdown. Hence, might MH-susceptibles be at special risk if theytake this medication? Although theMH-susceptible pigs seem to developgreater physiologic abnormalitieswhen given large quantities of thedrug, the difference is more quantita-tive than qualitative, i.e., MH suscep-tibility does not seem to play a role inthe response.

However, what was interest-ing is that dantrolene (or moreproperly, a new formulation ofdantrolene called Ryanodex) reversesthe MH-like signs. Therefore, there isa strong suggestion that patients whoappear in the Emergency Departmentsuffering from Ecstasy overdoseshould be treated with dantrolene.How dantrolene reverses the signs of

Continued on page 7

European MH Group Presents Many “Impressive” Abstracts

(Left) Richard Ellis, one of the EMHG founders, gave a talk about the history ofthe EMHG. (Right) The hosts of the EMHG meeting were Gunilla Islander and Dr.Klaus Glahn.

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MHAUSWelcomes MichaelWesolowski

Michael Wesolowski hasassumed the position of MHAUSPublic Relations/Project Coordina-tor. For over 20 years, Mr.Wesolowski has assisted variousorganizations with branding, grantwriting and public relations.

He has served as theExecutive Director of New BerlinHousing and Preservation Com-pany in New Berlin, NY, and asthe Director of Marketing Com-munications and Development forGallery 210 in Syracuse, NY, andas Agency Director of LM Sunrisein Buffalo, NY.

In his new position, Mr.Wesolowski will maintain andimprove the recognition ofMHAUS with the public, developand implement new ideas to helpthe educational and patient safetyefforts, assist the Executive Direc-tor with grant writing, and handleappeals and oversee and acknowl-edge various corporations andassociations.

“Everyone has been sohelpful and welcoming,” he says.“The transition into the positionhas been very smooth.”

Continued from page 6

Ecstasy overdose (which is really aform of Serotonin Syndrome) is notclear.

Another interesting abstractfrom the MH group in Leeds, En-gland, demonstrated that (at leastunder certain conditions) calciumlocated in the extracellular space canenter the cell and aggravate theongoing elevation of intracellularcalcium – the hallmark of MH, thatresults from the release of calciumfrom the sarcoplasmic reticulum.

The group also were unableto document new DNA variants inthe dihidropyridine receptor gene.

A progress report was pre-sented on the development of an MHtesting center in Czechoslovakia byDr. Stepankova. Their center hasbiopsied 120 individuals and identi-fied 14 families with RYR-1 muta-tions.

Dr. Kathryn Stowell’s groupgave several presentations based ontheir biochemical and genetic studiesof MH from Massey University, NewZealand.

Dr. Mariz Vainzof from theUniversity of Sao Paulo, representingthe group interested in MH and othermyopathies, described findingsdemonstrating a high frequency ofRYR1 variants in Brazilian patientswith Centronuclear myopathy(CNM). CNM is a diverse group ofcongenital inherited muscle diseasesthat share a common pathologicappearance: enlarged centrallylocated myonuclei surrounded by ahalo of disorganized cytoplasmicorganelles.

The abstract I presentedexamined cases reported to theMHAUS Hotline since January 2006that were likely or definitely MH,according to the consultants. Of the27 such calls from hospitals therewere two deaths, while of the 13 suchcalls from ambulatory centers oroffice surgery centers, there were

three deaths, or a mortality that isabout three times greater than in thehospital setting. A few caveats: thecalls may not represent all cases thatoccurred in such settings and therewere calls that could not be classi-fied as to whether they derived froma hospital or ambulatory setting.Nevertheless, the findings raiseconcern that when MH occursoutside a hospital setting, there is agreater likelihood of a bad outcome.While some may argue with thefindings, we at MHAUS are proceed-ing with developing protocols tomanage the treatment of MH when itoccurs in an outpatient setting.

This is a brief overview ofthe meeting. All the 50 or soparticipants were actively engaged inthe discussions of the papers.

Finally, there were a fewother interesting aspects to theprogram. For non-anesthesiologists,Dr. Islander had arranged a demon-stration of an actual MH case in theUniversity’s simulator laboratory.

Dr. A. Bom, a senior scientistat Organon Laboratories, a part ofSchering Plough, delivered a rivetingpresentation on his development of anew reversal agent for certainparalyzing drugs used by anesthesi-ologists and intensivists. The agent,Sugammadex, works by a novelmechanism, i.e., binding the para-lyzing agent so that it becomesinactive. The speed with which itworks implies that a relatively long-acting paralyzing agent can beturned into a short-acting agent bythis compound. This can effectivelyreplace succinylcholine, one of theknown triggers of MH, and eliminateother unpleasant complications ofsuccinylcholine.

Next year’s meeting will beheld in Australia and hosted byRobyn Gillies of Melbourne andother MH investigators in Australiaand New Zealand.

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The Lila and Jerry Lewis Memorial FundThere are many special people who take the time each year to remember their loved ones in a way

that helps MHAUS. The people below have made gifts during FY 07-08 (Oct. 2007 - May 2008) inmemory of Lila and Jerry Lewis. We are most grateful for their support and special tribute gifts.

Life BenefactorsDorothy GlassmanGregory Lewis GlassmanJacey Lila GlassmanMarilyn Lewis GlassmanSteve & Mickey LewisDr. Joseph SugermanBob & Dianne Winters

PatronsHillard FahnBrad & Julie Shames

SponsorsTerry & Cheryl DawesArline A. HammerBarry & Beverly KurtzGloria LeonardGreg & Laura Perloff

Lenny RobertsJudy SwartzMark Zamel

DonorsLarry & Linda BlumenfeldSy & Estelle OpperAllen JacobsSheldon & Doreen QueridoBill & Linda RouseLes & Diane SurfasErven & Beatrice Tallman

FriendsSam BirnbaumDoug BraunRob GloriaJon MatthewsJohn & Kerrie Sadler

Lois SoterGeorge & Miriam TrustmanMarilyn Tucker

HonorariaMarriage of Jacey Glassman & DanHayes and Greg Glassman &Courtney Grenfell by Marilyn Lewis GlassmanMemory of Renee Johnson’s UncleMelvin Rollin by Marilyn Lewis GlassmanMemory of Florence & DavidGoodman by Dorothy Glassman60th Birthday of Judy Levine &Steve Lewis by Marilyn Glassman

The Malignant HyperthermiaAssociation of the United States(MHAUS) is pleased to announce theavailability of an award in the amount$1500 to the author of a manuscriptrelated to malignant hyperthermia(MH).

MH is an inherited disorderof muscle, which is "triggered" bycommonly used anesthetic agents andmay lead to death or disability. Earlydiagnosis and prompt treatment is thekey to reducing morbidity andmortality related to MH. MH mayoccur at any time during an anes-thetic whether in a hospital, ambula-tory surgery center or an office-basedsetting. A large variety of programshave been developed by the scientificpanel at MHAUS in order to increaseawareness of the syndrome and itsmanifestations. These includeprocedure manuals for recognizingand treating MH (applicable to thehospital, ambulatory surgery center,and office-based surgery center) aCME-accredited slide show, and avariety of publications.

MHAUS Offers $1500 Writing AwardIn order to promote awareness of MHand its various manifestations and toencourage continued study of thesyndrome, Mr. George Massik, afounding member of MHAUS,graciously supports a writers’ award.The Daniel Massik Fund at TheFoundation for Jewish Philanthropiesin Buffalo, NY was established by Mr.Massik in memory of his son whodied from MH. This Award willprovide a stipend of $1,500 to ananesthesia resident/fellow or ananesthesiologist who is within fiveyears of ending his/her training toattend the annual meeting of theAmerican Society of Anesthesiologists(ASA) Meeting or, in special circum-stances, another meeting of similarmerit.

Award DetailsThe Award will be given to theprimary author of the best manu-script concerning, malignant hyper-thermia. The format may be a casereport, literature review or originalstudy.

❑ The document should address asignificant issue related to the prob-lem of malignant hyperthermia.

❑ Those participating must currentlybe a resident/fellow in anesthesiologyor an anesthesiologist who is withinfive years of ending his/her training.

❑ The paper must be a minimum ofthree double-spaced typed pages anda maximum of 10 pages. Author’s CVshould be included.

❑ The paper must not be in any stageof publication.

❑ Deadline for receipt of the manu-script in the MHAUS office is August1, 2008

The award will be presentedat the annual MHAUS RecognitionReception at the Annual Meeting ofthe ASA Meeting in Orlando, FL inOctober 2008.

The winner will be notifiedby August 31, 2008 to allow forcoordination of travel plans.

For further informationregarding the application process forthis award, please contact the Malig-nant Hyperthermia Association of theUnited States (MHAUS), attentionGloria Artist, either via regular mail atP. O. Box 1069, Sherburne, NY13460, via fax at 607-674-7910 oremail at gloria@mhaus.org.

CorrectionIn the genetic testing article appear-ing in the spring 2008 issue of TheCommunicator, Jenny Geurts, acertified genetic counselor with St.Luke’s Medical Center, should havebeen quoted as “...if the test comesback positive, at least we know now,”not negative. She has been a geneticcounselor for two years, not 15.

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President George W. Bush hassigned into law the first civil rightslegislation of the new millennium, theGenetic Information Nondiscrimina-tion Act (GINA). GINA is the firstand only federal legislation thatprovides protections against discrimi-nation based on an individual’sgenetic information in health insur-ance and employment settings.

“This is a tremendous victoryfor every American not born withperfect genes – which means it’s avictory for every single one of us,”said Representative Louise Slaughter(D-NY). “Since all of us are predis-posed to at least a few genetic-baseddisorders, we are all potential victimsof genetic discrimination.”

This means Americans canfinally take advantage of the tremen-dous potential of genetic researchwithout the fear that their owngenetic information will be usedagainst them. Dr. Barbara Brandom,of the Children’s Hospital and the

Genetic Nondiscrimination Act Becomes LawUniversity of Pittsburgh MedicalCenter, urges those having a genetictest performed for MH to have theirresults recorded in the North Ameri-can MH Registry. The informationstored in the Registry is vital tocontinue understanding the geneticsof MH.

Two clinical laboratories inthe U.S., PreventionGenetics, L.L.C.(www.preventiongenetics. com) andthe Center for Medical Genetics,University of Pittsburgh MedicalCenter (http://path.upmc. edu/divisions/mdx/diagnostics. html),offer clinical testing for MH diagnosis.

MHAUS provides a brochurethat explains the issues and proce-dures of genetic testing for MH, aswell as the muscle contracture test,which remains the “gold standard” forthe diagnosis of MH. The brochure isavailable on the MHAUS website atwww.mhaus.org or by calling theMHAUS office at 607-674-7901.

The new legislation protects

against genetic discrimination byhealth insurers or employers from:❑ Prohibiting group health plans andissuers offering coverage on the groupor individual market from basingeligibility determinations or adjustingpremiums or contributions on thebasis of genetic information.❑ Prohibiting issuers of Medigappolicies from adjusting pricing orconditioning eligibility on the basis ofgenetic information.❑ Prohibiting employers from firing,refusing to hire, or otherwise dis-criminating with respect to compen-sation, terms, conditions or privilegesof employment. Similar provisionsapply to employment agencies andlabor organizations.

The health insurance protec-tions offered by GINA are expected toroll out in less than 12 months,whereas the employment protectionswill be fully realized in about 16months.

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Dr. Werner Kalow, the “Father of Pharmacogenetics”Editor’s Note: This is the third in aseries of biographies related tothose who played an important rolein our understanding MH. Dr.Werner Kalow passed away onFebruary 16, 2008, at the age of 91.

When Dr. Werner Kalowallied his specialty, pharmacology,with the study of genetics, fewrealized that otherwise benign drugscould prove deadly in certain patientswith genetic defects.

Called “the father of pharma-cogenetics,” Dr. Kalow’s many years ofresearch into how genetic changescan alter a person’s response to drugseventually led to investigation intothe role of anesthetic agents intriggering malignant hyperthermia.

Born in Germany in 1917, hereceived an M.D. degree fromKoenigsberg University in 1941. Heserved as a surgeon in the GermanNavy during World War II and wasthe camp surgeon while a POW inArizona. Freed in 1947, he returnedto Berlin and began training inpharmacology.

Fluent in English, Kalowvolunteered to be an interpreter for agroup of American scientists visitingGermany. His detailed explanation ofa talk presented in English was soimpressive that he was invited by theChair of the Department of Pharma-cology of the University of Pennsylva-nia to visit for a year’s study.

In Philadelphia Kalowworked with a Beckman Spectropho-tometer, which uses the absorption oflight by drugs as a means to identifyand measure drug concentration, toinvestigate the metabolism of thelocal anesthetic procaine and later ofvarious other chemicals. Using thistechnique he was able to reveal thepresence of various forms of a specificenzyme that is responsible for the

metabolism of the paralyzing drugsuccinylcholine. For some patientswith an altered form of the enzymethe duration of action of the drug wasgreatly prolonged. This led to Kalow’spostulation that a genetic changealtered the structure of the enzyme.He then undertook investigation ofthe hereditary nature of this occur-rence. This eventually led to thedevelopment of a blood test that iscommonly used to investigate thosewith a prolonged response to succi-nylcholine and similar drugs.

Kalow joined the Departmentof Pharmacology at the University ofToronto in 1951, eventually becom-ing the Department Chair from 1966to 1977.

In the early 1960s Dr.Beverly Britt, an anesthesiologist atthe University of Toronto, wasinvestigating a newly recognizedsyndrome, malignant hyperthermia.She had shown that an acute MHcrisis could occur in some patientswhen certain anesthetic agents wereused. Furthermore, certain breeds ofpig manifested similar changes.

Her findings coincided withKalow’s interest in Pharmacogenetics,and in 1966 their long collaboration

began, continuing until her retire-ment in 1996.

Dr. Kalow’s and Dr. Britt’sdevelopment of the caffeine contrac-ture test (Lancet 1970, 2: 245-247)was an important indicator of MH.Their first studies showed thatexcised muscles from pigs exhibitingthe MH trait responded abnormally tocertain drugs.

Caffeine proved to be aseffective as the anesthetic halothanein causing muscles to contract,indicating susceptibility to MH. Laterstudies on leg muscle excised frompatients under anesthesia verified therole of caffeine in causing an abnor-mal increase in muscle contracture.This observation eventually led to thecaffeine halothane contracture test(CHCT), the standard for determin-ing MH susceptibility.

Drs. Kalow and Britt col-lected information on families of MHpatients in Canada, and broughtattention to the syndrome by manypublications describing the incidenceof the syndrome and its conse-quences. Along with the chair ofAnesthesia at the University, Dr. R.A.Gordon, they organized the firstInternational Symposium on Malig-nant Hyperthermia in Toronto in May1971. This symposium led to therecognition of MH as a distinctmedical syndrome and stimulatedboth laboratory and clinical researchthat continues today.

Dr. Kalow published over300 articles, was the recipient ofnumerous research awards through-out his distinguished career, and his1962 book, Pharmacogenetics, repre-sented the first systematic study of histhen emerging specialty. His workcontinues to stimulate new avenues ofpharmacological inquiry.

Werner Kalow 1917 - 2008

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Yes! I want to support MHAUS in its campaign to prevent MH tragediesthrough better understanding, information and awareness.

A contribution of: ❑ $35 ❑ $50 ❑ $100 ❑ $250 ❑ $500 ❑ $1000 (President’s Ambassador) or ❑ (other amount) $ ___________, will help MHAUS serve the entire MH community.

Please print clearly:Name: ______________________________________________________________________________Address: ____________________________________________________________________________

City: ____________________ State: _____________ Zip: _________________Phone: __________________________ E-mail: _________________________

❑ I am MH-Susceptible ❑ I am a Medical Professional

Please charge my ❑ Visa ❑ Mastercard ❑ Discover ❑ American ExpressName on card: ____________________________________________________Credit Card Number: _______________________________________________Expiration: ___________________________

Please clip out thishandy coupon, or feelfree to photocopy ifyou prefer to keepyour issue intact, thenmail to: MHAUS, POBox 1069, Sherburne,NY 13460-1069

Slide ShowPresentation For

MH Risk AvailableMHAUS offers a slide show kit

(CD-ROM and slide format) withlecture notes on “Managing MalignantHyperthermia Risk in Today’s SurgicalEnvironment.” This presentationreviews the risk of MH and assessescurrent trends in the management ofMH in the inpatient and outpatientsettings. Two CME credits areavailable.

This is a valuable tool to assistin developing standard of care practiceguidelines and algorithms to ensurepatients at risk will have access toappropriate interventions for treatingMH. The program is arranged so it canalso be used as a self-study program toenhance individual knowledge of MHand the risks involved.

Cost is $165 plus shipping andhandling for the slides and CD. Call607-674-7901 or visit www.mhaus.orgto order.

Every MH-Susceptible Should Wear AMedical ID Tag

MHAUS has help available for the MH-susceptibles whohave no insurance or cannot afford to purchase a medicalID tag.

The Sandi Ida Glickstein Fund was established for thepurpose of providing free ID tags for MH-susceptible patientswho qualify.

To take advantage of this program, please send us a letterindicating why you would like MHAUS to provide you with acomplimentary ID tag.

The goal of the free ID tag program is to ensure thesafety of MH-susceptibles during an emergency situation and toprevent a tragic outcome from MH.

For further information, please contact MHAUS atP.O. Box 1069, Sherburne, N.Y. 13460-1069; call 607-674-7901, or visit www.mhaus.org.

Have you visited us lately? Log on to www.mhaus.orgto get the latest information on MH, order materials,

post a message to the bulletin board orlearn about the “Hotline Case of the Month.”

MHAUSP.O. Box 1069Sherburne, NY 13460-1069www.mhaus.org

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PERMIT #10SHERBURNE, NY 13460

MHAUS Happenings, Events and Notices❑ THANKS! MHAUS is gratefulfor the financial support of thefollowing State Societies of Anes-thesiology: California, Connecti-cut, Florida, Illinois, Maine,Maryland, Michigan, Nevada,Ohio and Pennsylvania. Ourappreciation also goes to thefollowing state components of theAmerican Society ofPeriAnesthesia Nurses: Arkansas,Colorado, Delaware, DC, Illi-nois, Kansas, Maryland, Mis-souri, Nebraska, New Hamp-shire, New Mexico, NorthCarolina, Pennsylvania, Texas,Vermont and Wyoming. Call theMHAUS office to ask how yourgroup can join their ranks!

❑ Upcoming MHAUS MeetingDates and LocationsAANA, Minneapolis, MN, August

9-13; MH Mini-Conference,Tampa, FL, September 27-28;ASA, Orlando, FL, October 18-22.

❑ Joseph R. Tobin, MD, hasbeen appointed Chair of theDepartment of Anesthesiology atWake Forest University BaptistMedical Center by William B.Applegate, MD, MPH, FACP,Interim President of Wake ForestUniversity Health Sciences andDean of The Medical School.Tobin, professor of anesthesiol-ogy and pediatrics, was head ofthe Section on Pediatric Anesthe-siology and Pediatric CriticalCare. He is also the Director ofthe Malignant HyperthermiaBiopsy Testing Center at WakeForest University School ofMedicine.

❑ Fay A. Rozovsky, JD, MPH, wasone of six distinguished individualsto receive honorary degrees fromProvidence College during its 19thCommencement Exercises on May18, 2008, in Providence, RhodeIsland. Rozovsky is President ofThe Rozovsky Group, Inc. and anauthority in patient safety, consent,and healthcare risk management.An attorney, she has written morethan 600 articles and has written,co-written, or edited more than 20books. In 1990, the U.S. SupremeCourt cited her book, “Consent toTreatment: A Practical Guide,” aslegal authority in a critical patient’srights case. A faculty member atVirginia Commonwealth UniversitySchool of Medicine, Rozovsky is oneof the first women to attend Provi-dence College and graduatedsumma cum laude.