Postpolio Syndrome PDF
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HANLEY& BELFUS AnAffiliateofElsevier TheCurtis Center IndependenceSquareWest Philadelphia, Pennsylvania 19106 Notetothereader:Althoughthe techniques, ideas,and informationinthis book have been carefullyreviewedforcorrectness, neither the authors nor the publishercanac ceptanylegalresponsibilityforanyerrorsoromissionsthatmaybemade. Neitherthe authors nor the publishermakesanyguarantee, expressedor implied, with respectto the material containedherein. LibraryofCongress ControlNumber: 2003107490 POSTPOLIOSYNDROME ISBN 1-56053-606-3 Copyright©2004 byHanley& Belfus.Allrightsreserved.No partofthisbook may be reproduced, reused, republished, or transmitted in any form, or stored in adata baseor retrievalsystem,withoutwrittenpermission ofthe publisher. Printedin the UnitedStatesofAmerica Lastdigit istheprintnumber: 9 8 7 6 5 4 3 2 1 CONTRIBUTORS Jamesc.Agre,MD,PhD MinistryMedical Group, EagleRiver,Wisconsin DorothyD.Aiello,PT,MS Senior PhysicalTherapist, SpauldingRehabilitation Hospital, Framingham,Massa chusetts JohnR.Bach,MD Professorand ViceChairman, DepartmentofPhysicalMedicineand Rehabilitation; ProfessorofNeurosciences,UniversityofMedicine and DentisttyofNewJersey NewJerseyMedical School; University Hospital, Newark, NewJersey BrentBernstein,DPM DepartmentofPodiatry, Staten Island University Hospital, Staten Island, NewYork Kristian Borg,MD,PhD AssociateProfessorand Head, DepartmentofRehabilitationMedicine, Karolinska Institute; Huddinge UniversityHospital, Stockholm,Sweden NeilR.Cashman,MD Professor, DepartmentofMedicine, UniversityofToronto Faculty ofMedicine; Director, NeuromuscularClinic, Sunnybrookand Women's Health SciencesCen ter,Toronto, Ontario, Canada MariaH.Cole,OTR SeniorOccupationalTherapist,SpauldingRehabilitation Hospital, Framingham, Massachusetts DeborahDaCosta, PhD Assistant Professor, DepartmentofMedicine, McGill University; Medical Scientist, McGill UniversityHealthCenter, Montreal, Quebec, Canada MarijkeDallimore,PT ClinicalAssociate,Post Polio Clinic, UniversityofBritish Columbia,Vancouver, British Columbia, Canada Elizabeth Dean,PT,PhD Professor,School ofRehabilitationSciences,and Coordinator, Post Polio Clinic, UniversityofBritish Columbia,Vancouver, British Columbia, Canada LoisFinch,MSc School ofPhysicaland OccupationalTherapy, McGill University, Montreal, Que bec,Canada v vi Contributors AnneC.Gawne, MD (deceased) Director, Post-PolioClinic, RooseveltWarmSprings Institutefor Rehabilitation, Warm Springs, Georgia LauroS.Halstead,MD,MPH Clinical Professor, DepartmentofMedicine, Georgetown UniversitySchool of Medicine; Director, Post-Polio Clinic, NationalRehabilitation Hospital,Washing ton, DistrictofColumbia ClaireZ.Kalpakjian, PhD Research Fellow, DepartmentofPhysicalMedicineandRehabilitation, University ofMichigan HealthSystem,AnnArbor, Michigan BethKowall, MS,OTR Post-PolioResource GroupofSoutheasternWisconsin, Milwaukee,Wisconsin FrederickM.Maynard,MD DepartmentofPhysicalMedicineand Rehabilitation, MarquetteGeneral Hospital, Marquette, Michigan PimaS.McConnell, PT,ATP Director, Seating and WheeledMobilityClinic, RooseveltWarmSprings Institute for Rehabilitation, Warm Springs, Georgia SunnyRoller, MA ProgramManagerand ResearchAssociate, DepartmentofPhysical Medicineand Rehabilitation, UniversityofMichigan HealthSystem, AnnArbor, Michigan LauraA.Ryan, OTR OccupationalTherapist, SpauldingRehabilitation Hospital, Framingham,Massa chusetts JulieK.Silver,MD Assistant Professor, DepartmentofPhysicalMedicineand Rehabilitation, Harvard MedicalSchool,Boston,Massachusetts; Medical Director,SpauldingFramingham OutpatientCenter; Director, InternationalRehabilitation Centerfor Polio, Fram ingham, Massachusetts;Associatein Physiatry, Massachusetts General Hospital;As sociate in Physiatry, BrighamandWomen's Hospital, Boston,Massachusetts KathrynA.Smith, BS,MPT AquaticProgramDirector, Roosevelt Warm Springs Institutefor Rehabilitation, Warm Springs, Georgia BarbaraC.Sonies, PhD Chief, OralMotor Function Section, DepartmentofRehabilitation MedicineClin icalCenter, National InstitutesofHealth, Bethesda, Maryland;Adjunct Professor, Speech and HearingDepartment, GeorgeWashingtonUniversity,Washington, DistrictofColumbia;Adjunct Professor, Hearingand Speech Science Department, UniversityofMaryland, Baltimore, Maryland Contributors vii DeniseG.Tate,PhD Professor,DepartmentofPhysicalMedicineand Rehabilitation, University of Michigan HealthSystem,Ann Arbor, Michigan DariaA.Trojan,MD,MSc AssistantProfessor, DepartmentofNeurology, Montreal Neurological Institute, McGill UniversityFaculty ofMedicine; Physiatrist and Director, Post-Polio Clinic, Montreal Neurological Hospital, McGill UniversityHealthCenter, Montreal, Que bec,Canada JoseVega, MD, PhD DepartmentofPhysicalMedicineand Rehabilitation, University ofMedicineand DentistryofNewJersey-NewJerseyMedical School;University Hospital, Newark, NewJersey PREFACE During the course ofworking on this book, my co-editor, Dr. Anne Gawne told me that shewould liketo write the preface.Shesaid,"Therearesome things Iwant to say."I readilyagreed, knowing that whatevershewrote would be thoughtful and insightful. Unfortunately,Anne diedsuddenly inthe middleofpublishing thisbook, and none ofuswilleverknowwhat exactlyshehad in mind. I havefound it adifficult processto sit down and write something meaningful in herplace.Iknowforafact,however,that oneofthe thingsthatAnnewould want me tosayisaheartfelt thank-youto the peoplewho contributedto thisvolume.Thepo lio health carecommunityisasmallone. Most ofusknow eachother, and wework together, collaborating and sharing ideas. So, thank you to all of the authors who wrote chaptersforthisbook.Thankyouforyourwillingnesstodevoteyour time and energyyetagain to helping poliosurvivors. Anne and Ipublished this book with afocuson evidence-basedmedicine.Theau thors who contributed to this text are allextremely knowledgeable and have based their recommendationson what the current polio researchreveals. WheneverIwrite oreditabook, Ialwaysaskmyselftwoquestions: (1)Will thisbookhelp people?and (2)Will Ilearn somethingfrom working on this project?With respectto this book, the answer to both questions isa resounding yes.I am confident that this text will helphealth careproviderssharpen theirskillsinorder toprovidestate-of-the-artmed icaltreatment forpolio survivors.Polio survivors,too, can benefitfrom reading this book-even ifsome of the writing is rather technical. Moreover, I have learned a greatdealfrom mycolleagueswho collaborated with meon this project. I sincerelywish that Anne washere to write this preface.She did, however, write severalchapters in this book. Iencourage you to readher work and to consider the wonderful contributionshemadeto helping poliosurvivorsthroughoutthe world. For thoseofuswho continueour work with polio survivors,wewillremain stead fastinour goaltoimprove their health and quality oflifethrough excellencein med icalcareand thoughtful researchthatisevidencebased. Julie K.Silver,MD ix ACKNOWLEDGMENTS Aswith any authoritativework, there aremany peoplewho areresponsibleforthe finaltextand who aredeservingofrecognition. In thiscase,Ifirstwant to recognize those individuals who havedesignedand conducted the researchstudies that are the basisforthispublication.Itistrue that weneedmore researchon polio-related issues, but therearemanycliniciansand scientistswhoareadding toour currentknowledge aboutthe lateeffectsofpolioand postpolio syndrome. For thoseofuswho treat po liosurvivors,wearegratefulforthe diligent effortsoftheseinvestigators. Theauthors whowrote chaptersforthisbook haveprovided not onlyan insightful reviewofthe scientificliterature, but alsotheir own clinicaland researchexperiences that make the chapters vibrant and relevant.These contributors are heroesto many polio survivors-and for good reason. They are compassionate, intelligent, and ex tremely knowledgeable. They understand the researchand know how to translate it into aclinicalapproach that makessense.Iam deeplygratefultoallofthem. LauroHalstead haslongbeenateacher,mentor,and friend tome.Hisexpertiseon polio-related issuesisunsurpassed. Daria Trojan and her colleagues,LoisFinch and Deborah Da Costa, havemadewonderful contributions to this textand to the polio literature in general. Daria did alot of pinch hittingfor me in getting this book to gether, and sheissomeone that manyofusinthe poliohealth carecommunityknow wecan counton. Neil Cashmanworked with Daria, and havingcollaborated exten sivelyin the past, they continue to make a fine team. Fred Maynard is highly re spectedbyallofus.Hiscontributiontothisbook isextremelyvaluable.KristianBorg is an amazingly intelligent and gracious man. It was a pleasure to work with him. JohnBachisalwaysthedoctorIgotowith mymost complicated poliopulmonaryis sues.John isthe foremost authority on respiratory complications in neuromuscular diseases,and his chapter on postpolio pulmonary dysfunction, with colleagueJose Vega,isan importantsection in this book. Iam grateful toBarbaraSonieswho took time out ofher busyschedule at the National Institutes ofHealth to write aterrific chapteronspeechand swallowingissuesinpoliosurvivors.Jim Agreiscurrentlypur suinganumberofotherinterestsbut stilltook timeouttowriteachapter on exercise. This isacomplicatedtopic inthe polioliterature, andJim wrote athoughrful and in sightful analysisfor this text. Elizabeth Dean and Marijke Dallimore are expertson physical therapy and exercisein polio survivors, and their chapter is outstanding. Kathy Smith contributedan excellentchapteron aquatic therapy that I found truly enlightening. Brent Bernsteinworked closelywith Anne Gawne and provided avery informative chapter on polio foot and ankle problems. DorothyAielloisone of rhe physicaltherapists at mycenter, and sheisincrediblyknowledgeable.Her writing re flectsadeep understanding of polio-related issuesfrom both a researchand clinical xi xii 8estPracticeGuidelinesfroPhysicalTherapy perspective. Pima McConnell wasextremely kind to write a chapter quickly as my deadline loomed. She did agreat job, and I am very thankful for her hard and fast work. Beth Kowallisan occupational therapistwhosework many ofusreferto on a regularbasis.Herchapterisan importantpartofthisvolume. Maria Cole and Laura Ryanarebothoccupationaltherapistsat mycenter. Iam regularlyimpressedbytheir skilland empathy. Claire Kalpakjian, Sunny Roller, and Denise Tate wrote an in sightful chapter on the psychological well-being ofpolio survivors-an often over looked subject. Toallofyou who contributed: thank-you! Iam alsograteful to the many poliosurvivorswho havesupportedmyworkat the International Rehabilitation Center for Polio at Spaulding Rehabilitation Hospital. In particular, I am thankful for the wonderfulsupportofDavid Rubin and hiswife, Arlene. David Rubin isasuccessfulbusinessman who contracted polio at 9 yearsof age.Althoughheisstillveryactive,heissufferingfrom postpoliosyndrome. David is determined to help not only himself, but other polio survivors who are similarly strugglingwith the lateeffectsofthis disease.The Rubin Family Fundfor Polio Re searchhelped to makethis book possible. A number ofpeople at Spauldinghave been extraordinarilyhelpful aswell.Anna Rubin isthe polio outreachand educationcoordinatorat my center and isan amaz ing person to work with. Lori McCrohon isthe clinicalcoordinatorand isagentle, compassionate,and capablewoman. Lynn Fordeexpertlymanagesmycenterand has helped raisethe levelofskillofallofthe clinicians on myteam, includingthosewho contributedto this book. TerryCucuzza and TerryO'Brienareresearchlibrarians at Spauldingwho help meon adailybasiswith finding the importantinformation that Ineed. Ioften wonderifthey know how many livestheir hard work touches. Walter Fronteraisthe Chairman ofmydepartment (PhysicalMedicineand Rehabilitation) atHarvardand hasbeen mybiggestsupporterand finestmentorthroughoutmyaca demic career. He is highly respected for his intelligence and thoughtful analysisof clinical,administrative, and researchissues.DianaBarrett isthe ChairofSpaulding's board, and her energyand enthusiasmareinfectious. Itisan honorand apleasure to watch Diana in action. David Storto has been awonderful leader and visionary for Spaulding. Iappreciate hisinsight and support. This book waspublished at a time when Elsevieracquired Hanley & Belfus,Inc. Therefore, I haveseveraleditors to thankfor their hard work on thisvolume. Ihave worked with BillLamsbackon severalbooks, and I alwaysappreciate his perceptive opinions. TomStringer and CeceliaBayrunshelped mestartand finishthisbook, re spectively.Linda Belfuswasabigsupportbehindthe scenes. And finally,Iamgrateful to mycolleague,Anne Gawne, withoutwhom this book would not exist.Thankyou and mayGod blessyou. Julie K.Silver,MD CHAPTER 1 Diagnosing Postpolio Syndrome: Inclusion and Exclusion Criteria Lauro S. Halstead, MD, MPH Startingaround 1970, reports begantoappear in the medicalliterature that per sonswho had paralytic poliomyelitismany yearsearlierwereexperiencing new health problemsrelatedtotheirprior illness.4,11,35,37,42Initially,oneofthe most common terms to describe these problems was thelateeffectsofpolio.15,34 This term referstoallofthemusculoskeletaland neuromuscularcomplaints that affectthesein dividuals,many ofwhich arenodifferent from thesymptoms experiencedbyanyone who haslivedwith achronicneuromuscularcondition formany decades. However, withinthisbroadcategoryofsymptoms,itsoonbecameclearthattherewasasmaller group ofsymptoms that wasmorespecificand discrete.3,22,31Although nodiagnostic test existsfor thesesymptoms, most expertsagreethat they share acommon patho physiologyprimarily involvingadysfunction ofpolio-affected motor units.t? These symptoms arenowwidelyknown aspostpoliosyndrome(PPS).This chapter describes the natural history ofpolio, the historical background ofPPS, and how PPS iscur rently diagnosed usingboth inclusion and exclusioncriteria. THE NATURAL HISTORYOF POLIO Historically, polio has been divided into three fairlydistinct stages:acute illness, period ofrecovery,and stable disability. Bythe mid-1980s, however,clinicians and researchers began to realize there was a distinct fourth stage characterized by the onset ofnewsymptoms relatedto theoriginal polioattack.13,23,30This stagehasbeen described by various terms, including the late effects ofpolio, postpolio sequelae, postpolioprogressive muscularatrophy, postpolio muscle dysftnction, and postpolio syn drome.18,21.50,56The namespostpolio muscledysftnctionand postpolioprogressive mus cularatrophyemphasize abnormal muscle function. This narrow focus makes these terms more appropriate for research.Bycontrast, PPS isbroadly defined, making it more practical for clinical purposes. In addition, PPS has been widely used in the medicaland layliterature formanyyears.For thesereasons,PPSisusedin thisbook. 2 DiagnosingPostpolioSyndrome:InclusionandExclusionCriteria Figure 1showsthe typicalcoursefor the three traditionalstagesofparalyticpolioas wellasthebeginningofstageIV.Thedataforthesehealthand functional changesare basedon the acute and chronic polio experienceofa group of persons evaluated at The InstituteforRehabilitationand ResearchPost-PolioClinic in Houston,Texas." STAGEI:ACUTEILLNESS The symptoms at the onset of polio aresimilar to many otherviral illnesses: they include mild fever,headache, sore throat, diarrhea or vomiting, and malaise.In the greatmajority ofindividuals, thesesymptoms aregonewithin 2or 3days.For fewer than 5% ofthe peoplewho contract polio, however, the symptoms are more severe, reflecting a viral invasion ofthe central nervous system (CNS).46 Infection ofthe CNS results in a sharp escalation of symptoms with high fever, stiff neck, severe headache,and musclepains.Insomeindividuals,thediseasestopsthereand noweak nessorparalysiseveroccurs.Inothers (approximately 1-2%ofthoseaffected),thein fection continues to spread, producing variable amounts of muscle paralysis or weaknessin thelimbs,trunk, and eventhefaceand neck.Duringthelargeepidemics of the 1940s and 1950s, roughly 12% ofthose who developed acute paralytic po liomyelitisdied from breathingorswallowingcomplications.24,43.65.66 STAGEII:PERIOD OFRECOVERY Recoverybeginsassoon asan individual's temperature returns to normal and the other symptoms subside. This stagecan lastfrom weeksto years,depending on the severityofinvolvement and ageatonset. Personswhocontractpolioaschildren orin fantsand haveextensiveparalysistakethelongesttimeto recover.Duringthisperiod, individuals usually begin an intensive program ofrehabilitation in the hospital or home with the goalofstrengtheningand retraining weakened musclesand learning 100 - ................ o ABC D E F ----25---- -5- -7- -8- --7-- Years Figure1. Natural historyofpolio. Healthandfunctionalchangesshowingthefourstagesof poliofor132IndividualswithPPSatthe Posf-PolloClinicInHouston.Texas.A=birth:B=onsetof acutepolio (averageageof7years):BtoC =periodofrecovery(anaverageof8years);Cto D=maximumrecoveryandperiodofneurologicandfunctionalstability(anaverageof25 years);D= onsetofPPS(8toDan averageof33years);E= time ofclinic evaluation(DtoE.an averageof5years);F= death(EtoF.unknown). Dashedline = projectedcoursewithoutPPS. DiagnosingPostpolioSyndrome: InclusionandExclusionCriteria 3 toregainlostfunction. Forthegroupofpersonsshown inFigure1,theaveragelength ofstageIIwas8years. STAGE11/:STABLEDISABILITY StageIII beginswhen aperson reachesaplateau ofmaximum recoveryofstrength and stamina. Theprecisetimewhen thisstagestarts maybehard to determine, espe ciallyifthe individual isstillgrowing and changingdevelopmentallyor isundergoing reconstructive surgery to enhance strength and function. Despite these difficulties, most people haveageneralideaaboutwhen their recoverywascomplete. STAGEIV:POSTPOLIOSYNDROME Thethirdstageofpolio lastsindefinitelyfor many individuals, perhaps forthe ma joritywho had paralytic polio. For asmany as50% ofindividuals, the stageofstable disability ends andstageIV or PPS beginswith the onset ofnewweakness,which is often accompaniedbyothersymptoms,such asfatigue,pain in the musclesor joints, and decreasedfunction.2,10,49,68 Forthe individuals shown inFigure 1,stageIII lasted an averageof25 years.Stage IV began, on average,33 yearsafter the acute onset of polio. Asimilar interval isfound in otherstudies, butthe rangehasbeen reported to extend from 2 to 8decades.1,27.30 THE HISTORYOF POSTPOLIO SYNDROME Thelateeffectsofpolio havebeenrecognizedformore than acenturywith thefirst descriptionsappearingintheFrench medicalliteraturein 1875.12,17,51Thesecasesin volvedthree young menwho had paralyticpolio ininfancyand developedsignificant newweaknessandatrophyasyoung adults. Thesenewproblems occurred inmuscles previously affected by polio and in musclesthought to be spared.Allofthe subjects had physicallydemanding jobs that required strength and repetitive activities. In a commentaryononeofthecases,thegreat 19th centuryFrench neuropathologistJean MartinCharcotsuggestedseveralhypotheses forthesechanges.U He believedan ini tialdiseaseofthe spinal cord (e.g.,polio) mightleavesome individuals more suscep tibletoasubsequentspinaldisorder. He alsohypothesizedthatthenewweaknesswas caused byoveruseofthe involvedmuscles. His observations aresurprisingly relevant to ourcurrentunderstandingofPPS. After those initial reports, there wasonly sporadic interest in the lateeffectsofpo lioformany decades.In the centuryafterCharcot'sobservations, fewerthan 35 pub lished reports appeared, describing fewerthan 250 cases.67Aswith the firstsubjects, thesereports described newproblems thatincludedweakness,atrophy,and fascicula tions, occurringup to 71 yearsafteran attack ofparalyticpolio. Itisunclear why theseaftereffectsofpolio remained an obscure and largelyunex plored area ofmedicine until recently. Few diseasesare aswidely prevalent in the world or havebeenasintensivelyinvestigated aspolio. Becauseofthe rapid and dra matic onset ofsymptoms, polio wasviewedasaclassicexample of an acute viralin fectiousdisease.Asa result, most ofthe scientificenergyand resourcesweredirected
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