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Dental Hygiene ETDs Electronic Theses and Dissertations

7-2-2011

Reducing Pneumonia Related Death inAmyotrophic Lateral Sclerosis Patients ThroughImproved Oral Health CareSusan Wray Jones

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DEDICATION

Thisthesisisdedicatedto:

Those who have fought the ALS battle and

Those who fought and must go on – their families

My three boys Darrin, Clayton and Colin

Thank you for being my light …there’s never a wish better than this

and

In memory of Michael Allen Correll

“Ihavefoughtthegoodfight,Ihavefinishedtherace,Ihavekeptmyfaith…”2Timothy4:7

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ACKNOWLEDGEMENTS

To my thesis committee: Elaine Sanchez Dils, RDH, MA; Dr. Sarah Youssof,

Medical Director ALS Center University of New Mexico, Christine Nathe, RDH, MS and

Demetra Logethetis, RDH, MS, my sincere gratitude for your assistance and guidance.

To my professor, mentor and most importantly my friend Elaine Sanchez Dils…

It has been said that the success of students can be determined by the instructor’s

dedication to their education. Through your support, you have exemplified how one

positive, resolute individual can affect the outcome of a student. Thank you for your level

of commitment to my education, research and thesis. Truly, at a time in your life when

you needed the support, I thank you for being mine.

To the collaborating ALS Association Certified Centers and ALS Association

Executive Directors, thank you for your assistance with my research. I hope that the light,

shed upon this topic may, in some small way, positively impact the lives of those fighting

the courageous and arduous battle with the monster that is amyotrophic lateral sclerosis.

Tomyparents,brotherandsister,thankyouforalwaysbeingthere.

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REDUCING PNEUMONIA RELATED DEATH IN

AMYOTROPHIC LATERAL SCLEROSIS PATIENTS THROUGH

IMPROVED ORAL HEALTH CARE

By

SUSAN WRAY JONES

B.S., Dental Hygiene, University of Nebraska, 1991

M.S., Dental Hygiene, University of New Mexico, 2011

ABSTRACT

InpatientsdiagnosedwithAmyotrophicLateralSclerosis(ALS),the

primarypathologicalcauseofdeath,asdeterminedbyautopsy,ispneumonia.

Researchassociatespoororalhealthwithanincreasedincidenceofpneumonia.

Theincidenceisfurtherelevatedinmechanicallyventilated,disabledandhigh‐risk

individuals.Aproactiveapproachinoralhealthcarecouldmitigatetheriskof

pneumoniarelateddeathsinpatientswithALS.Thisstudyevaluatedwhetherthe

dentalneedsofALSpatientswerebeingmet.

Overathree‐monthperiod121ALSpatientsweresurveyedregardingtheir

oralhealthstatus.Bothwrittenandonlinesurveyformatswereemployed.ALS

AssociationMultidisciplinaryClinicsandCertifiedCentersassistedinsurvey

promotionanddistribution.Researchresultswereanalyzedutilizingbivariate

Pearsoncorrelationcoefficientstodeterminerelationshipsamongstudyvariables.

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Resultsshowedpatients’difficultyinobtainingdentalhealthcareincreased

by38.7%whentheresponsibilityoftheiroralhealthcaretransitionedfrompatient

tocaregiver.Alsoincreasingthedifficultyinobtainingdentalhealthcarewasthe

lengthoftimeelapsedsincepatientdiagnosis.Anoverwhelming85%ofpatients

respondedthattheyhadnotreceiveddentalhealthcareinformationattheir

multidisciplinaryclinicappointments.

Educatingpatients,theircaregiversandALSmedicalsupportpersonnelon

thesignificanceoforalhealthcareanditsassociationwithpneumoniaand

pneumoniarelateddeathcouldhaveapositiveimpactonALSpatientlife

expectancy.Thisstudysupportstheneedforinclusionofadentalcomponentinto

ALSpatients’multidisciplinaryclinicappointments.

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TABLE OF CONTENTS

CHAPTER 1 INTRODUCTION……………………………………………………….1 Statement of the Problem………………………………………………………..1 Significance of the Problem……………………………………………………..2 Operational Definitions………………………………………………………….3 Assumptions……………………………………………………………………..5 Limitations……………………………………………………………………....5 Methodology…………………………………………………………………….6 CHAPTER 2 LITERATURE REVIEW………………………………………………..7 Amyotrophic Lateral Sclerosis (ALS)…………………………………………..8

History…………………………………………………………………..8 Epidemiology…………………………………………………………....9 Etiology………………………………………………………………….9 Clinical Features………………………………………………………...10 Diagnosis………………………………………………………………..11 Treatment……………………………………………………………….12 Primary Cause of Death………………………………………………...12 Correlation Between Oral Health and Respiratory Disease…………………….13 Mechanical Ventilation and Respiratory Diseases……………………………...14 Reduction of Respiratory Complications Through Oral Health Care Measures.16 CHAPTER 3 METHODS AND MATERIALS………………………………………..17 CHAPTER 4 RESULTS……………………………………………………………….19

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CHAPTER 5 DISCUSSION…………………………………………….……………..26 APPENDIXES APPENDIX A – Actual Qualitative Patient Survey Comments……….……………….33 APPENDIX B - SurveyMonkey Patient Flyer ALS Association Executive Director Participation Invitation Letter…………………………………………………..37 APPENDIX C - SurveyMonkey Patient Flyer………………………………………….38 APPENDIX D - SurveyMonkey Patient Survey………………………………………..39 APPENDIX E ALS - Association Certified Center Medical Director Participation Invitation Letter…………………………………………………………………44 APPENDIX F - ALS Association Certified Center Medical Director Participation Consent………………………………………………………………………….45 APPENDIX G - ALS Patient Survey – Informed Consent Cover Letter………………46 APPENDIX H - Dental Consideration in Patients With Amyotrophic Lateral Sclerosis (ALS) Patient Survey…………………………………………………………...47 APPENDIX I - ALS Association Certified Center Collaborators……………………...48 REFERENCES………………………………………………………………………….49

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Chapter1:Introduction AmyotrophicLateralSclerosis(ALS),alsoknownasLouGehrig’sdisease,isa

degenerative,fatalneuromusculardisease.EachyearintheUnitedStates5600

peoplearenewlydiagnosed.Thereisnoknowncure.Fromdateofdiagnosis

typicallifeexpectancyisthreetofiveyears.Duringthattimethepatientsteadily

deterioratesfromafunctionalstateintoacompletelyparalyzed,fullydependent

lifestyle.Pneumoniainducedrespiratoryfailureistheleadingcauseofdeath.1Itis

speculatedthat,astheirdiseaseadvances,patientswithALSoftenfindmaintaining

oralhealthquicklybecomesachallenge.Recentstudiesassociatepoororalhygiene

andperiodontaldiseasewithcolonizedoropharyngealrespiratorypathogensthat,

whenaspirated,mayinducepneumonia.3Therefore,ALSpatientswithinsufficient

oralhealthandtoanevenhigherdegree,patientsoptingformechanicalventilation

areatanincreasedriskfordevelopinglife‐threateningpneumonia.Researchalso

supportsthehypothesisthat,inthesesusceptiblepatients,oralanddentalhealth

educationcanreducetheincidenceofpneumonia.21Asthediseaseprogressesand

oralhealthcareresponsibilitiestransitiontothecaregiver(s)dentalhygiene

educationandinstructionbecomepivotaltothepatient’swell‐being.

StatementoftheProblem

AsALSpatients’motorskillsdeclinethelevelofdifficultyinperforming

activitiesofdailyliving(ADL)andobtainingmedicalcareincreases.Notonlyisitan

obstacleforthepatientbut,finances,transportationandtreatmentschedulingalso

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complicatethematterforcaregivers.Dentalhealthcareandoralhygiene,initially

maintainedbythepatientnowbecometheresponsibilityofthecaregiver.

Poororalhygieneandperiodontaldiseasehavebeenpositivelylinkedwith

pneumonia,theleadingcauseofdeathinALSpatients.3Studieshavealsoshowna

reductioninpneumoniaassociatedwithpoororalhealthwhenpreventativeoral

hygieneproceduresareperformed.3Therefore,itisimperativethattheALSpatient,

aswellastheircaregivers,understandnotonlythesignificanceoforalhealthbut

arealsotrainedinproperoralhygieneprotocols.Thisresearchstudywasdesigned

todeterminewhetherthedentalhealthcareneedsofALSpatientsarebeingmet.

SignificanceoftheProblem

Thereisnocurrentresearchsupportingthenecessityofdental/oralhealth

treatmentduringtheprogressionofALS.Noristhereresearchthatidentifiesthe

varyinglevelsoforalhealthcarerequiredbyALSpatientsthroughoutthecourseof

thedisease.

AstheALSpatients’motorskillsdeteriorate,theirabilitytomaintainoral

healthcarealsodeclines.Additionally,caregiversmaynotbeawareofthe

significanceoforalhealthinrelationshiptothespecificneedsofthepatient.Oral

hygieneinstructiontailoredtothevariousstagesofthediseaseiseasily

disseminated;andtheALSpatient’sprimarycaregiverisintheoptimalpositionto

meetthoseneeds.

Progressiveweakeningofthemusclesutilizedforeating,breathing,drinking

andswallowingcreatesadditionalobstaclesfortheALSpatient.52Managementof

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thesesymptomsmayincludeventilatorysupport.Non‐invasivebi‐levelpositive

airwaypressuredevices(orBiPAP®)machinesareoftenincorporatedatnightfor

respiratoryrelief.Tracheostomyanddirectmechanicalventilationdevicesaretwo

additionaltreatmentoptions,thoughseldomchosenbyALSpatients.68,70

Regardlessofthedecision,alltheseoptionshaveprofoundeffectsontheoralcavity

andone’sabilitytomaintaindentalhealth.Becausethepatient’sconditionmay

rapidlychange,oralhygieneeducationneedstobeprovidedtoboththepatientand

thecaregiver.

Obtainingdentalcareatadentalfacilityoftenposesitsownsetof

complications.NotalldentalpracticesareequippedtohandleALSpatientsinallof

theirvariantstages.Patientsmayhavetoseekcarefromadifferentprovideror

foregodentaltreatmentaltogether.Whilechanginghealthcareproviderscanbe

stressfultoanyperson,tothepatientwithALS,thisprocessmayprovetobe

overwhelmingandnegativelyinfluencetheirdecisiontoseekcare.

EvaluationofALSpatients’dentalneedsisimperativeinprovidingoptimal

patientcare.Assessingthepatient’scurrentdentalconditionandoralhealth

knowledgemayprovidevaluableinformationregardingwhethertheinclusionofa

dentalcomponentwithinALSAssociationMultidisciplinaryClinicswouldbe

beneficial.

OperationalDefinitions

ActivitiesofDailyLiving(ADL)

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Basicpersonaltasksincaringforoneselfdaily,forexample,dressing,bathingand

eating.

AmyotrophicLateralSclerosis(ALS)

Achronic,progressivediseasemarkedbygradualdegenerationofthenervecellsin

thecentralnervoussystemthatcontrolvoluntarymusclemovement.Thedisorder

causesmuscleweaknessandatrophy.Symptomscommonlyappearinmiddleto

lateadulthood,withdeathinthreetofiveyears.Theetiologyisunknown,andthere

isnoknowncure.

UpperMotorNeuron

Foundinthemotorcortexareaofthebrain,theysendmessagestolowermotor

neuronstocontrolskeletalmusclemovement.Primarilyinvolvedwithmaintaining

muscletoneandinitiationofvoluntarymusclemovement.

LowerMotorNeuron

Alsoknownasanteriorhorncells,theytakemessagesfromuppermotorneuronsto

controlthemusclefiberstheyinnervate.

Bi­levelPositiveAirwayPressure(BiPAP)

Mechanicalventilationdesignedtoassistpatientsinmovingairbothintoandoutof

lungs.

MechanicalVentilation

Machinesusedtoassistorreplacespontaneousbreathing.

Invasive–Artificiallysupportedventilationprovidedthroughacannulaor

breathingtubeplaceddirectlyintopatient’strachea.

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Non­invasive–Ventilatoryassistancedeliveredviaanasal,oral/nasalor

full‐facemaskdesignedtoreplicatepatients’normalrespiration.

Ventilator­AssociatedPneumonia(VAP)

Commoncomplicationcausingsignificantmorbidityandmortalityincriticallyill

patients,broadlydefinedaspneumoniadevelopingmorethan48hoursafter

intubation.4

DurableMedicalEquipment(DME)

Adaptive,reusablemedicalequipmentusedtoimprovepatientcomfortand/or

prolongpatientindependence.Mustbemedicallynecessaryandprescribedbya

physician.

Assumptions

Forthepurposeofthisinvestigationitisassumedthatallpatientssurveyed

havebeenpreviously,positivelydiagnosedwithclinicalevidenceofamyotrophic

lateralsclerosisandhaveprovidedhonest,accurateresponsesregardingtheir

condition.

Itisalsoassumedthatarticlesanddocumentsreferencedinthis

investigationutilizedethicalandaccuratemethodstoobtainandpresentfactual

information.

Limitations

Despitenumerousresourcesregardingamyotrophiclateralsclerosis,there

waslimitedinformationavailableonthenumberofALSpatientsthatelectfor

mechanicalventilation,thetypeandextentofmechanicalventilationusedandthe

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lengthoftimebetweenapatient’sfinalclinicappointmentanddateofdeath.

ResultsindicatingthenumberofALSpatientsthatchoosetodieathomeincontrast

toaskilledcarefacilityandwhethertheroleofprimarycaregiverwasfamilialoran

outsideentityvariedgreatly,dependentuponthegeographicalregionstudied.

Initialsurveyresultssuggestedtimeelapsedsincepatientdiagnosismay

helpindicatetheindividual’scurrentphysicalstate.Collectionofthisinformation

wasincludedintheamendedelectronicsurvey.Clarificationofthepatient’sdegree

ofmobilityandlevelofself‐careatthetimeofsurveymayalsoprovevaluablein

futurestudies.

AlthoughfiveALSCertifiedCentersagreedtoparticipatewithwrittensurvey

distributionandcollection,thedatafromonelocation,BannerGoodSamaritan

MedicalCenterofPhoenixArizona,wasnotreceivedintimetobeincludedin

statisticalanalysis.

Methodology

Aresearchstudywasconductedinwhich121ALSpatientsweresurveyed

duringathree‐monthperiodfromNovember2010throughJanuary2011.Patients

weresurveyedeitherinpaper(written)formatduringroutineALSAssociation

CertifiedCenterappointmentsorasecurewebsite.PreviouslydiagnosedALS

patientswhowere18yearofageorolderwererecruited.SPSSwasemployedfor

statisticalanalysis.TheUniversityofNewMexicoHumanResearchReview

Committee(HRRC),approvedthisstudyonAug16,2010.Participantcompletionof

surveyimpliedconsent.

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Chapter2:ReviewofLiterature

Literaturereviewwasconductedbyresearcherusingmedicalsubjectheadings

(MeSHterminology)inPubMed/MEDLINEdatabasefromSeptember2010–

January2011.Citedreferencesfrompreviouslypublishedarticleswerealsousedto

locateadditionalresources.

MeSHterms:

Pulmonaryinfection/disease

Respiratory

AmyotrophicLateralSclerosis

Periodontaldisease

Oralhealth/hygiene

Intubation

Mechanicalventilation

Pneumonia

Oral/dentalhygieneeducation

Oral/dentalhygieneinstruction

Keywords:

Respiratory,AmyotrophicLateralSclerosis,Periodontal,Oralhygiene,Pneumonia

Inclusioncriteria:

ArticlesthatevaluatedcorrelationsbetweenALS,respiratoryconditions,

pneumonia,ventilation,dental/oralhealthandoralhygieneeducation/instruction

werereviewed.

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Exclusioncriteria:

Searchwaslimitedtohumanstudies.

Thefollowingmeasureswereassessed:

AmyotrophicLateralSclerosis

PrimarycauseofdeathinALSpatients

Correlationbetweenoralhealthandrespiratorydisease

Correlationbetweenmechanicallyventilatedpatientsandrespiratorydisease

Reductionofrespiratorycomplicationsthroughpreventativeoralhealthcare,

educationandinstruction

AmyotrophicLateralSclerosis(ALS)

History

ItwasJeanMartinCharcot,aFrenchphysicianandfounderofmodern

neurologywho,inthe1870’s,firstlinkedthesymptomsofamyotrophiclateral

sclerosistothegroupofnervesspecificallyaffectedbythedisease.55Itisbelieved

CharcotemployedahousemaidwithALSandwasthereforeabletosystematically

observetheclinicalmanifestationsofherdisease.Hismeticulousobservationsand

descriptionsofthediseaseremainaccuratetoday.Throughouttheworldthedisease

isknownbydifferentnames.InpartsofEuropethediseaseisstillknownas

MaladiedeCharcot(Charcot’sdisease).IntheUnitedStatesthediseaseiswidely

recognizedasALSorLouGehrig’sdisease;namedafterNewYorkYankeesbaseball

legendLouGehrig,whoretiredafterbeingdiagnosedin1939.Untilhisdeathin

1941Gehrigusedhiscelebritytopromoteawarenessandeducatethepublicabout

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thedisease.64In1993theSOD1geneonchromosome21wasfoundtoplayarolein

somecasesoffamilialALS.65

Epidemiology

TheannualincidenceofALSisonetotwocasesper100,000people.Itis

estimatedthat,duringanygiventime,30,000peopleintheUnitedStateshavethe

disease.9Eachyearanaverageof5600Americansarenewlydiagnosed,the

equivalentof15peopleperday.Thetypicalageofonsetrangesfrom40‐70yearsof

agewiththemedianagebeing55.Itis20%morecommoninmalesandnot

affectedbyracial,ethnicorsocioeconomicstatus.9Fromthedateofdiagnosismore

than50%willlivethreetofiveyears.Approximately20%willlivemorethanfive

years,10%morethantenyearsand5%willlivelongerthantwentyyears.77

Etiology

AmultitudeoftheorieshavepostulatedtheetiologyofALS.However,todate,

nonehaveprovedstatisticallysignificant.Charcotfirsthypothesizedacorrelation

betweenpoliomyelitisandthedevelopmentofALS.In1907,SAKWilsonquestioned

therolesofheavymetalinvolvement.11Wilson’stheorygainedmomentumthrough

theearly1900’suntilresearchshowedmercuryandlead,theleadingsuspects,were

capableofproducingreversibleALS‐likesymptoms.Sincethen,ahostofprevailing

theoriesincludingdietarydeficiency,environmentalfactors,physicalinjury64,toxic

exposure,vascular,immuneandinflammatorydisordershavebeeninvestigated

withoutsubstantiation.52Currentresearchleanstowardavastmultifactorial

approachencompassinggenetics,viral,autoimmuneandneurotoxichypotheses.61

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ClinicalFeatures

ALSisadisorderofthemotorneuronsresponsibleforcontractingthe

skeletalmuscles.11Inhealth,lowermotorneurons,locatedinthebrainstemand

spinalcord,innervatethemusclefibers.Whenamotorneuronfiberbecomes

diseaseditcannolongercontrolthemuscleitinnervatesanddenervationoccurs.

Consequently,theimpairedmusclefibersshrinkandthemusclebecomesunableto

contract.Ifsurroundingmotorneuronsarehealthytheycanregeneratenerve

fibersandtakeovercontrolfromthedenervatedfibers.Despitetheoverall

reductioninthenumberofmotorneurons,aslongasthisprocessismaintained,

musclestrengthremainsconstant.11

Whentherateofdenervationexceedstherateofreinnervation,muscle

strengthdecreases.InmorethanhalfofALSpatientstheprogressiveweakeningof

musclesareamongthefirstsymptomsnoted.Initiallimb(armorleg)weaknessis

morecommonthanbulbaronset;whichinvolvesmusclescontrollingspeechand

swallowing.52Typicalsignsoflowermotorneurondegenerationincludemuscle

weakness,muscleatrophy,musclecramping,fasciculations,pareticbulbarpalsy

(dysarthria,dysphagia,sialorrhea),hyporeflexiaanddifficultyswallowingwhich

canleadtolife‐threateningaspirationissues.66

Uppermotorneuronsarelocatedinthebrainandbrainstem.Theycarry

informationfrombraincentersthatcontrolthemusclesofthebody.Theseneurons

synapsewiththelowermotorneuronswhichtransmitinformationtothemuscles.12

Therefore,inorderformotorcommandsgeneratedinthecentralnervoussystemto

reachthemusclesofthebody,signalsmustutilizeboththeupperandthelower

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motorneurons.Uppermotorneurondysfunctionprogressesmoreslowlyand

revealsitselfinsymptomssuchaslossofdexterity,musclespasticity,hyperreflexia

andspasticbulbarpalsy.52

Thetypeanddegreeofmotorneurononsetaretwoofthemain

characteristicsutilizedindiagnosingandclassifyingALS.52Anotherpopularbasis

usedinclassificationisthemethodofacquisition.52Ninetytoninety‐fivepercentof

patientshavethesporadicformofALS(SALS);ALSwithnoknownfamilialhistory.

Theremaining5‐10%havefamilialALS(FALS),withacloserelativehavingthe

disease.65Theclinicalfeaturesofthetwoacquisitiontypesareindistinguishable.In

1998theElEscorialcriteriawasdevelopedasthestandardforclassifyingALS

patientsinclinicalresearch.Thesecriteriaarebasedonclinicalevidenceoflower

anduppermotorneuroninvolvementandrepresentthedegreeofdiagnostic

certainty.52

Diagnosis

Currentlythereisnoonehundredpercentaccurate,definitivetestto

diagnoseALS.Diagnosisisbasedonamyriadofsigns,symptoms,neurologicaland

laboratorydatawhilesimultaneouslyeliminatingthediversediagnosticalternatives

thatmimicALS.52TheremultiplegenemutationsassociatedwithfamilialALS,

severalofwhichcanbetestedbycommerciallabs.82TheSOD1geneticmutation

representstheonlyknowncauseinALSdevelopment.65However,subsequent

studiesonAmericanandBritishFALSpatients,haveshownthattheSOD1genetic

mutationonlyaccountsfor20%ofinheritedALS83,84

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Treatment

Predominanttreatmentconsistsofavarietyofphysicaltherapiesand

palliativedrugs.In1995,Rilutek®(riluzole)becamethefirstFDAapprovedALS

drug.10Todate,itremainstheonlyFDAapprovedALSdrugavailableintheUnited

States.Researchersbelieveriluzolehelpsprotectthemotorneuronsfroman

overproductionofglutamate;asubstanceinvolvedinnervoussystemfunction

which,inexcess,provestoxictotheneurons.76Clinicaltrialshavedemonstrateda

modesttwotothreemonthextensioninlifeexpectancy.10,75,76InAmericaALSis

consideredan“orphandisease”inthatitisararedisorderaffectingfewerthan

200,000people.10Consequently,drugdiscoveryremainsachallenge,as

pharmaceuticalcompaniesareunlikelytomakeaprofitfromsuchasmallmarket.

Hencetheydonotdedicateresourcestocostlyresearchanddrugdevelopment.In

1983,CongresspassedtheOrphanDrugActprovidingexclusivemarketingrights

andfinancialincentivestoencourageorphandiseaseresearch.10Despitethese

initiatives,todaymorethan130yearslater,ALSremainsanincurabledisease.

PrimaryCauseofDeath

Clinicaldeathisdefinedasthecessationofthetwoprimarycriteria

necessarytosustainlife:bloodcirculationandbreathing.5Mostresourcesavailable

onamyotrophiclateralsclerosisidentifytheleadingcauseofclinicaldeathas

respiratoryfailure.1,6‐8Thepathologicalcauseofdeathisdiagnosedthrough

laboratoryanalysisofbodilyfluidsand/ortissues.5Inthepostmortemstatethis

processisknownasanautopsy.Todate,verylittleliteratureexistsidentifyingthe

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causeofdeathinALSpatientsthroughpostmortempathologicalanalysis.Itis

estimatedtheautopsyrateamongALSpatientsisapproximatelyfourpercent.6

Withinthebodyofresearchthatdoesexist,theprimarycauseofdeathinmorethan

70percentofthecasesiseitherbroncho‐pneumoniaoraspirationpneumonia.1,6,7,8

Atwenty‐twoyearstudyfollowing100ALSpatientsfoundamajordiscrepancy

betweentheclinicallyassessedandpathologicallydeterminedcauseofdeath.7A

lessthan20%concordancebetweenclinicalandpathologicalconclusions,showed

clinicalassessmentnottobeareliablemarkerofdeath.7

CorrelationBetweenOralHealthandRespiratoryDisease

Recentliteraturesupportstheassociationbetweenrespiratorydiseaseand

oralhealthandconfirmstheoralcavitytobeaharborofinfectiousrespiratory

pathogens.3In2001,followinganineyearstudyof358veterans,dentaldecayand

thepresenceofcariogenicbacteriaandperiodontalpathogenswereshowntobe

significantaspirationpneumoniariskfactors.13Anotherstudyobserved189elderly

personsoverafouryearperiodandconfirmedanassociationbetweenpneumonia

anddecayedteeth.Inthisstudydependenceoncaregiverswasalsolinkedto

pneumonia.14Athirdstudylinkedhigherplaquescoreswithaprevioushistoryof

respiratorytractinfections.15Periodontaldiseasehasbeenmoderatelyassociated

withatherosclerosis,myocardialinfarctionandcardiovasculardisease16andthe

riskofchronicobstructivepulmonarydiseaseisalsoknowntobesignificantly

elevatedwithsevereperiodontalattachmentloss.17

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Pneumoniaisdefinedasaninflammatoryconditionofthelungcausedby

bacterial,viral,fungalorparasiticinfections.5Theriskofpneumoniais,inpart,

determinedbythespecificbacteriainhaledandthebody’sabilitytoeliminatethe

bacteriafromtheairwaymucosa.Toeliminateaspiratedbacteriafromthelower

airway,multipledefensemechanismsmustfunctionproperly.Poororalhygieneand

thepresenceofperiodontaldiseasemayfosteroropharyngealcolonizationof

respiratorypathogenswhichincreasetheprobabilityofaspirationpneumonia,

especiallyinhigh‐riskpatients.3,80Theeffectivenessofthesemechanismsmayalso

befurtherimpairedbyavarietyoflifeconditionssuchasadvancedage,residingina

nursinghomeorhospitalanddebilitatedpersons.3,

MechanicalVentilationandRespiratoryDiseases

InadvancedstagesofALStheprocessbreathing,orvoluntarilyexchanging

oxygenandcarbondioxide,maybecomedifficultandoftenresultsinrespiratory

distress.Symptomsoftenincludeheadachesuponwaking,physicalexhaustion,

laboredbreathing,andtheinabilitytocoughorspeaklongsentences.Decisions

associatedwithventilatoryassistancebecomeintegralinALSdisease

management.24Optionsincludenon‐invasivepositivepressureventilation(NIPPV)

andinvasiveventilation.Bothmodalitiesdemonstrateprolongedsurvivalratesand

arebelievedtoimprovethepatient’soverallqualityoflife.26‐30Bilevelpositive

pressureairwaymachines,otherwiseknownasaBiPAP,areconsideredanon‐

invasiveformofartificialventilation.UnlikeCPAP,whichprovidescontinuous

airwaypressure,theBiPAPunitprovidestwolevelsofairpressure,oneon

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inhalationandanotheronexpiration.Variantnasalandfacialdeliverysystemsare

availabletoassistwithoxygenintakeandcarbondioxideremoval.Thereareother

formsofnon‐invasiveassistedventilationavailable.However,inALSpatients,the

BiPAPsystemismostprevalent.18Noninvasivepositivepressureventilationusein

ALSpatientshasbeenassociatedwithprolongedsurvivalrates.18Inrecentyears

nocturnalNIPPVusehasbecomethetreatmentofchoiceforALSpatientssuffering

chronicrespiratoryinsufficiency.19TheextentofNIPPVuseinamyotrophiclateral

sclerosispatientshasnotbeenwidelystudied.18Onesourceestimatedsuccessful

NIPPVuseamongALSpatientsashighas70%andsuggestedhigherusagewas

likelylimitedbyimproperlyfittedmasks.68

ApproximatelythreetofivepercentofALSpatientselectedtohaveinvasive

ventilation.18,68Theimmediatelyaforementionedsourcealsonotedmorefrequent

selectionofinvasiveventilationassociatedwithculturalorigin.Forexample,ALS

patientsresidinginJapanmorefrequentlyselectinvasiveventilationthanthose

residingintheUnitedStates.68Mechanicalventilatorysupportisprovidedviaa

tracheostomy,asurgicallycreatedopeninginthetracheathroughwhichairis

forcedonanindividualized,timedcycle.20Thisisanirreversibledecisionbutmay

exponentiallyprolongthepatient’slife.Despitethebenefitthecostofinvasive

ventilationisestimatedbetween$153K‐$336Kperyear.Additionally,invasive

ventilationrequires24‐hourskilledcaregiversupport.19

Nosocomialbacterialpneumonia,orventilator‐associatedpneumonia(VAP),

isthemostcommoninfectionreportedbyintensivecareunits(ICU).2VAPis

associatedwithaseventotwenty‐onefoldincreaseintheincidenceofpneumonia2

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andaccountsfor47%ofallICUpatientinfections.32Twentyeightpercentof

mechanicallyventilatedpatientswilldevelopthispneumoniaresultinginan

attributableincreaseinmorbidityandmortality.2Anotherstudyfoundnosocomial

pneumoniaprevalenceinICU’srangedfrom10%–65%,withathirteentofifty‐five

percentfatalityrate.23Studiesconductedinthe1990’ssuggestventilator‐

associatedpneumoniaisavitaloutcomedeterminantincriticallyillpatients.23

ReductionofRespiratoryComplicationsThroughOralHealthCareMeasures

Poororalhealthcanleadtocomplexmedicalanddentalissues.31Ithasalso

beenpositivelylinkedtoanincreasedincidenceofpneumoniainventilated,

debilitatedandotherhigh‐riskpatientgroups.3,13Asignificantamountofresearch

hasbeenconducted,evincingconsistentresults,onthereductionofrespiratory

complicationsthroughoralhygienemeasures.Standardizedoralhygieneprotocols

caneffectivelydecreasethecolonizationofdentalbacteriaassociatedwith

respiratorypathogensthatcausepneumonia.33‐39Mechanicaltoothbrushingand

chlorhexidinemouthrinseswereshowntobethetwomosteffectivemodalities.33‐39

A2009studyfound,afterimplementationofanoralcareprotocol,a46%reduction

inventilator‐associatedpneumoniacasesduringatwelvemonthperiod.36Studies

confirmedtheseresultsnotonlywithICUpatientsbutalsoinnursinghome

residents.34

ThemajorityofALSpatientsthatelecttodiehomeisvariantupon

geographicalregion.InEuropeitapproximately52‐63%ofpatientsdieathomein

contrastto85%electingtodieathomewithintheUnitedStates.40‐42,68,85One

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sourceestimated85%ofALSpatientsdieunderthecareofafamilialcaregiver.68As

thediseaseprogressed,thecaregiver’sburdenbecomesenormous;exponentially

moresowhenmechanicalventilationisinvolved.Manycaregiversresorttohospice

orhireskilledhealthcareworkersforassistancewiththeconstantcarethatis

required.Studiesevaluatingnurses’complianceofsimpleandCDC(Centersfor

DiseaseControlandPrevention)oralhealthcareguidelinesfoundthat,intheICU,

theseprotocolswereneitherconsistentlynoruniformlyimplemented.32,43‐49

Nurseswerefoundtolackknowledgeinrecommendedoralhealthcare

procedures44,45anddiscrepancieswerenotedamongreportedandactualpractices

andpolicies.48Despitesignificantevidenceontherelationshipbetweenoralhealth

careanddiseaseprevention,nursesgenerallyfailtofullyappreciateits

implications.49

Chapter3:MethodsandMaterials

Thisstudy’sconclusionswerebasedontheresultsfrom60papersurveys

distributedtoexistingALSpatientsatALSAssociationCertifiedCenter

appointmentsand61ALSpatientsparticipatinginthesurveyonline.Participant

completionofthesurveyimpliedconsent.Thesurveywasanonymousandno

personalidentifierswerecollected.

ALSACertifiedCenterMedicalDirectorsweresentinitialcontactletters

invitingtheirparticipation.Fiveofthethirty‐fourclinicscontactedparticipated.

CertifiedCenterMedicalDirectorswereaskedtosignandreturnparticipation

commitmentletters.ContactinformationregardingtheirCenter’sdesignated

18

surveycoordinatorwasalsocollected.Participantsthenreceivedasurveypacket

containing27patientsurveyswithattachedinformationcoverletters.

DesignatedindividualswereaskedtodistributesurveystoALSpatients,or

theirprimarycaregiver,overtheageofeighteen.AsallpatientsattendingCertified

Centermultidisciplinaryappointmentshadbeenpreviouslydiagnosedwith

amyotrophiclateralsclerosis;therefore,patientprivacyandsurveyrecipient

selectionwasnotanissue.Patientcoverlettersdescribedthecontentofthesurvey,

investigatorcontactinformation,InstitutionalReviewBoard(IRB)approval,

informedconsentandthepurposeofthestudy.Locationmarkerswerepre‐printed

oneachsurveyandusedonlytoidentifyonlythesitefromwhichthedatawere

collected.Onapre‐determinedenddate,ALSAssociationCertifiedCenter

designatedindividualsreturnedallcompletedsurveys.

FlyerspromotingtheidenticalsurveyinanonlineformatweremailedtoALS

AssociationExecutiveDirectorsinNovember2010.Directorswereaskedto

distributeflyerstoALSpatientsoverage18.ALSAssociationExecutiveDirectors

disseminatedflyerstoALSpatientsthroughouttheirrespectiveregionsofthe

UnitedStatespromotingtheonlinesurvey.Inanattempttopreventduplicate

responses,flyerswerenotsenttoregionsparticipatinginthewrittensurvey.

Directorswerefreetodisseminateflyersinelectronicorpaperformat.

SurveyMonkey,aweb‐basedinterfacespecializinginthecreationandpublishingof

customwebsurveys,wasutilizedfortheonlineeffort.Parametersweresetto

prohibitmultipleresponsesfromauniqueIPaddress.Theonlinedatacollection

periodranfromNovember2010throughJanuary2011.

19

Demographicdataincludinggender,onsetdiagnosis,typeofALS,ageat

diagnosis,historyofmilitaryservice,intubationandventilatorysupportwere

collected.Todeterminewhetherpatientshadanincreaseindentaltreatmentneeds,

patientrootcanaltherapyandamalgamdentalrestorationswereassessedboth

priortoandsincediagnosis.Participantswerealsoquestionedonthefrequency

anddifficultyinobtainingdentalcarepriortoandsincediagnosis.Theprimary

personresponsibleforperformingoralhygienecareaswellastheroutinely

implementeddentalhygieneregimenswasalsoasked.Patientswerequestionedas

towhethertheyhadreceivedoralhygieneeducationorinstructionattheirALS

AssociationMultidisciplinaryCenterappointments.Afinalsectionallowedpatients

toaddqualitativecommentstheyfeltwerepertinenttothestudy.

Chapter4:Results

Thestudysamplewascompromisedof121diagnosedamyotrophiclateral

sclerosispatientsovertheageofeighteen.

Populationdemographics:

GenderGender Male Female DidnotidentifyN= 61 56 4

SurveypopulationagerangeAgerange 18‐29years 30‐39years 40‐49years 50+years Notdisclosed

N= 2 5 25 87 2

Historyofmilitaryservice

Yes21%; No79%

20

ALSOnsetType

75participantsreportedbulbaronset,80participantslimbonset.

16respondentsdidnotidentifyonsettype.

FormofALS

7%(n=8)reportedfamilialformALS(FALS)

73%(n=88)reportedsporadicformALS(SALS)

21%(n=25)didnotidentifyALSform.

CurrentOralHealthCareRegimes

94% toothbrushing

44% flossing

12% swabbing

53% mouthrinse

7% utilizeothermechanisms

DataAnalysis

AstatisticalanalysisofthedatawasformattedintheSPSS11.5statistical

softwareprogramandutilizedstandardbivariatecorrelations,usingPearson

correlationcoefficient,todeterminethelevelofinter‐dependenceamongvariables.

Relevantdatawasanalyzedusingstandardfrequencycounts,crosstabulations

(measuringinter‐variablerelationships)andstepwiseregression.

Atthe95%statisticalconfidencelevel,thebivariatecorrelationanalysis

showedastrongcorrelationbetweenvariablesmeasuringincreaseddifficultyin

obtainingprofessionaldentalcareand1)presenceofafeedingtube,2)patient’s

21

inabilitytoeatfoodthroughmouthand3)caregiver(s)asprimarilyresponsiblefor

dentalhealthcare.

CrossTabulations

Frequencyanalysisofthesamevariablesshowed23%ofrespondentshave

feedingtubes,26%areundertheprimarycareofacaregiver,and20%ofthese

individualsfinditincreasinglydifficulttoobtainprofessionaldentalcare.Strong

existingcorrelationsbetweenthesevariablessuggestthatthemajorityofthese

characteristicsareassociatedwiththesameALSpatients.

22

Yes23%

No77%

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

CurrentlyHaveFeedingTube

Yes20%

No73%

Noreply7%

0%

10%

20%

30%

40%

50%

60%

70%

80%

IncreasedDifVicultyinObtainingDentalCare

23

OfthoseALSpatientsundertheresponsibilityofaprimarycaregiver,38.7%

reportedanincreaseindifficultyobtainingdentalcare,comparedto13.8%of

patientscapableofself‐care.Thisfindingsuggeststhatasthediseaseprogresses

andtheresponsibilityofdentalhealthcaretransitionstoacaregiver,thedifficulty

inobtainingdentalhealthcareincreases.Oralhealthinstructionandeducationmay

bewarrantedamongstbothgroups.

Patient74%

Caregiver26%

0%

10%

20%

30%

40%

50%

60%

70%

80%

PersonResponsibleforPatient'sOralCare

24

IncreasedDifficultyinObtainingDentalCare*PersonResponsibleforDentalCareCrossTabulation

Theincreaseindifficultyinobtainingdentalcarevariablealsoshoweda

directrelationshipwiththelengthoftimesincediagnosisresponse.Asthedisease

ascendstothethree‐yearmark,thelikelihoodofthepatientexperiencingincreased

difficultyaccessingdentalcarealsoincreases;alogicalconclusiongiventheaverage

timeelapsedfromdiagnosistodeathisthreetofiveyears.

IncreasedDifficultyinObtainingDentalCare*LengthofTimeSinceDiagnosis

CrossTabulation

Furtheranalysisofpatientswithfeedingtubes,alsoindicatingincreased

difficultyinobtainingdentalcare,showedatrendinthenumberofsilver/metal,

(amalgam)fillingspresentpriortodiagnosisinrelationtothelikelihoodofhavinga

25

feedingtube.10.5%ofrespondentswith1‐3fillingspriortodiagnosishadafeeding

tube;thatnumberincreasedto21.1%inthosewith4‐6fillingsand52.6%among

thosewith7ormorefillings.Whiletheseresultsindicateapotentialtrend,the

samplesizeofthosewithfeedingtubesandpriorfillings,19respondents,was

statisticallysmall.

NumberofFillings*PresenceofFeedingTube–CrossTabulation

Frequencyanalysesrevealedanoverwhelming85%ofrespondentshavenot

receivedinformationregardingdentalhealthcareattheirALSAssociation

MultidisciplinaryClinicAppointments.Thisfindingstronglyadvocatestheinclusion

ofadentalcomponentinamyotrophiclateralsclerosispatient’smultidisciplinary

appointments.

26

CHAPTER5–Discussion

Thisstudysurveyed121patientsovertheageofeighteendiagnosedwith

ALS.Theoriginalintentwastosurveypatientsatallofthe34ALSAssociation

CertifiedCenters.However,manyoftheALSAssociationCertifiedCenterMedical

DirectorswerereluctanttoparticipatewithoutobtainingInstitutionalReview

Board(IRB)approvalfromtheirrespectiveinstitution.Ofthe34centersinvitedto

participate,fivegaveconsent.DuringatelephoneconversationwithDr.RichardS.

Bedlack,MedicalDirectorofDukeALSClinic,on9/29/201080hesuggested

distributionofaflyerpromotingthesurveyinanonlineformatmightproduce

bettercompliance.Anonlinesurveywascreated;utilizingSurveyMonkey.com,and

amendedHRRC(IRB)approvalwasobtainedfromtheUniversityofNewMexico.

Then,lettersweresentto57regionalALSAssociationChapterExecutiveDirectors.

Thesurveyresultsobtainedfromthismethodofcontactprovednotonlyeasierin

Yes15%

No85%

0%

10%

20%

30%

40%

50%

60%

70%

80%

90%

ReceivedOralHealthCareInformationatMultidisciplinaryClinicAppointments

27

promotionanddistributionbutalsoindatacollection.Thedemographicsofthis

samplepopulationstronglyresembledoverallALSpopulationcharacteristics

regardinggender,age,typeofALSonset,historyofmilitaryserviceanddisease

form.

Inordertoexpandthescopeofthestudyseveraladditionaldentalrelated

parameterswereincluded.Thesequestionsinquiredaboutthetypeandnumberof

dentalrestorationspresentinthepatient’smouthbeforeandsincediagnosis.The

twosurveyformatsdifferedonlyinthattheonlinesurveyincludedanadditional

question,askingpatientsthelengthoftimesincetheirdiagnosis.Seventy‐four

percentoftheoverallsamplepatientpopulationrespondedthattheywerecapable

ofself‐care.InearlierstagesofALSwhenpatientsarelesslikelytobecompromised

withmobilityandswallowingdifficulties,itwasanticipatedpatients’overalllevelof

self‐carewouldnotonlybehigherbut,thattheiroralhealthcareregimenmightalso

includenormalactivitiesofselfcaresuchasbrushingandflossing.Withdisease

progressionitwasanticipatedoralhealthcareresponsibilitieswouldtransitiontoa

caregiverandobtainingprofessionaldentalhealthcarewouldbecomemoredifficult.

Resultsfromtheonlinesurveypopulation,wherelengthoftimesincediagnosiswas

reported,stronglysupportedthesecorrelations.Theresultssuggestedthatoral

healthcareregimensamongpatientswhereagreateramountoftimehadelapsed

sincediagnosiswererepresentedinthe12%thatreportedswabbingintheirdental

care.Whileswabbingalonedoesnotprovideeffectiveoralhealthcare,theaddition

ofanantimicrobialagentsuchaschlorhexidinehasshownsignificantimprovement

inpatientperiodontalpocketdepthsaswellasplaqueandcalculuslevels.78

28

Inclusiveoftheentiresurveypopulation,astrongcorrelationexisted

betweenthepresenceofafeedingtube,theinabilitytoeatfoodthroughthemouth,

andcaregiver(s)havingprimaryresponsibilityofthepatient’soralhealthcare.This

suggestedthatasthediseaseadvancedandnutritionalintakethroughthemouth

becamemorechallenging,patientsweremorelikelytohavefeedingtubesandtheir

dailyoralhealthcareregimendependentonacaregiver(s).Percutaneous

EndoscopicGastrostomy(PEG),orfeedingtubes,areaneffectivesystemusedto

providepatientswithnutritionandhydration.71UsedinALSpatients,PEG’s,have

beenshowntostabilizetheweightlosstypicallyexperienced.71However,itisalso

importanttorecognize,eventhoughthepatientisnotparticipatingintraditional

oralfoodintake,theirneedfordentalhealthcaredoesnotdecrease.Whendental

healthcareisneglected,bacterialpathogenswithintheoralcavitycontinuetothrive.

Ifnotdisruptedthroughoralhealthcaremeasures,thealreadyat‐riskpatient

possessesanevengreaterincreasedriskofdevelopinglife‐threateningpneumonia.3

Almost40%ofrespondentswhosecareisdependentonacaregiverreported

anincreaseddifficultyinobtainingprofessionaldentalhealthcare.Intheearly

stagesofALS,patientsaremorelikelytobecapableofself‐careandmaintaining

routineprofessionaldentalcareappointments.Withintheseappointments,patient

educationbythedentalprofessionalsregardingthesignificanceoforalhealthcare

throughoutthecourseoftheirdiseaseispivotal.Astheoverallhealthstatusofthe

patientdecreasesothercomplicationsinseekingprofessionaldentalcarearise.

Patientmobility,transportation,treatmentscheduling,accessibilitytodentaloffices

withwheelchairaccommodationsaswellassymptomsofdysphagia,dyspnea,

29

variantsalivalevels,musclespasticity,fatigueandotherobstaclesfurtherimpede

accesstotraditionaldentalcare.Ifpatientsareabletoobtainprofessionaldental

carewhileunderthesupervisionofacaregiver,caregiveroralhealtheducationand

instructionattheseappointmentsbecomesevenmorecritical.Researchclearly

indicatesthateventrainedcriticalcarenurses,withspecificoralhealthguidelines,

donotconsistentlycomplywithrecommendedoralhygieneprotocols.43‐46This

supportsthenecessityofunderstandingthesignificanceoforalhealthcareas

essentialtotheALSpatient’swell‐being,notonlybythepatientbutalsothe

caregiver,whomayormaynotbeatrainedskilledcareworker.

Datafromtheonlinesurveyalsosuggestedarelationshipexistedbetween

thelengthoftimesincediagnosisanddifficultyinobtainingdentalcare.The

correlationbetweendifficultyinobtainingdentalcareandALSonsetdiagnosistype

was.034;althoughthisleanedslightlytowardlimbonset,itwasnotastatistically

significantassociation. AsALSpatientsmigrateawayfromindividualizedmedical

appointmentsandtowardmultidisciplinaryclinicappointments,thesignificanceof

oralhealthcareeducationandinstructionisoftenminimizedifrecognizedatall.

Over85%ofthetotalsurveypopulationreportednothavingreceiveddentalhealth

careinformationattheirmultidisciplinaryclinicappointments.Ifthepatientshad

notbeeneducatedatpreviousprofessionaldentalcareappointments,itislikely

theydonotfullyappreciatetheassociationbetweenpoororalhealthandthe

incidenceofpneumoniaaswellasthemeasuresintegralinmitigatingtheserisks.

Significantresearchhasbeenconductedregardingmechanicallyventilated

andotherhigh‐riskpatients;thisresearchadvocatestheuseoforalhygiene

30

protocolstoreducetheoverallincidenceofpneumonia.33,36‐39Oral

decontaminationwith2%chlorhexidinesolutionalonehasbeenstronglylinkedtoa

reductioninbacterialcolonizationandincidenceofventilatorassociated

pneumonia(VAP)amongmechanicallyventilatedpatients.35Limitedresearch

existsontheprevalenceofventilatoruseamongALSpatients,thoughithasbeen

estimatedthatBiPAP®usemaybeashighas70%.68Non‐invasivepositive

pressureventilationpatients(BiPAP®)arealsoconsideredathigh‐riskfor

acquiringpneumoniaduetofluctuatingsalivarylevels,swallowing,mobility

difficultiesandotheroralhealthcareconsiderations.80

Currently,verylittleinformationexistsregardingthesignificanceoforal

healthcareinamyotrophiclateralsclerosispatients.WhileALSAssociation

multidisciplinaryclinicsareinanoptimalpositiontoprovidethesepatientsand

theircaregiverswithcriticaloralhealthcareinformationandeducation,85%of

surveyrespondentsreportedthisaspecthadbeenoverlooked.TheALSAssociation

websitedoesincludeasectionentitled“OralCareforthePatientwithALS:AGuide

fortheCaregiver,”however,itdoesnotaddresstheassociationbetweenpoororal

healthandincreasedriskofpneumonia.Nordoesitaddresstheuseof

antimicrobialagentsinmitigatingthisrisk.79Educationonthesignificanceoforal

healthcareanditsassociationwithpneumoniaandpneumoniarelateddeathcould

beeasilydistributedtopatientsandcaregiversininformativepamphletsatclinic

appointmentsorwebsitelinks.Inlaterdiseasestages,oralswabbingwith

antimicrobialagentssuchaschlorhexidinecouldbeimplementedataminimalcost.

TheALSpatientandtheircaregiversdohavenumerousotheroralhealthcare

31

optionsrangingfrommodifiedmanualandelectrictoothbrushestohighlyeffective

suctiondevicesaswellastheantimicrobialagentsalreadydiscussed.Adental

hygienistcouldprovidevaluableinformationduringeducationanddental

screeningsatALSAssociationchaptermeetings,localsupportgroupsand

multidisciplinarycarevisits.Inmanycitiesmobiledentalunitsareavailableto

assistinbothroutineandcriticaldentalhealthmaintenancevenues.

Someofthemostinterestingandinformativedatawithinthesurveyresults

werethequalitativepatientcomments.Uponreading,itwouldbedifficulttoargue

thatdentalcarehasnotbeenneglected.Patientsandcaregiversalikewere

confusedregardingthetypeofdentaltreatmentneededaswellashowandwhere

toobtainit.Respondentsposedquestionsconcerningtheneedforageneraldentist

versusaspecialistandwhetheranassociationexistedbetweensilver/metal

“amalgam”fillingsandtheacquisitionofALS.Individualresponsesalsoincluded

“difficulttobrush,”“choking,”and“bitinglipandinsidejaw”.Toahealthcare

professional,theseresponsesweredifficulttohear,especiallyknowingwecan

educateandassistthesepatientsduringtheirtimeofgreatestneed.

Ithasbeenestimatedover80%ofamyotrophiclateralsclerosispatientsin

theUnitedStatesdiewithintheirownhomeundertheprimarycareofafamilial

caregiver.1,42,45,68Currentresearchclearlyshows,uponpostmortemexamination,

theleadingcausesofdeathinALSpatientsareaspirationandbroncho‐pneumonia.7

Recognizingthecorrelationbetweenpoororalhealthandpneumonia,andtheeven

greaterriskinthosepatientsutilizingmechanicalventilation,onecanappreciatethe

significanceoforalhealthcareinformationandeducationinpreventionof

32

pneumoniarelateddeath.13,33‐39Studiesevaluatingtheknowledgeandcompliance

levelofhigh‐riskandintensivecareunitnursesuncoversanalarmingdiscrepancy

betweenrecommendedoralhygieneproceduresandactualperformance

protocols.43‐50Duetothesubstantialcostand24‐hourcarerequiredforALS

patients,familiesareoftenforcedtorelyonavarietyofcaregiversratherthana

singleorlimitednumberofskilledcarepersonnel.Ifthesemedicalprofessionals

arenotcognizantofthecorrelationbetweenpoororalhealthandriskofpneumonia,

howcanweexpecttheaverageALSpatientortheircaregiverstounderstandthe

importanceofaneffectiveoralhealthcareregimen?Inconclusion,recognizing85%

ofpatientsreportednothavingreceivedoralordentalhealthcareinformationat

theirALSAssociationmultidisciplinaryclinicappointments,itislogicaltoassume

themajorityofpatientswithamyotrophiclateralsclerosisandtheircaregiversdo

notfullyappreciatethesignificanceofaproperoralhealthcareregimen.This

demonstratesthenecessityforinclusionofadentalhealthcarecomponentintoALS

patientmultidisciplinaryclinicappointments.Fewdocumentedstudieshavebeen

conductedontheoralhealthstatusofALSpatientsthroughoutthecourseoftheir

disease.Thisstudyhasvalidatedthefactthattherearemanyintegralcomponents

withintherelationshipbetweenALSandoveralloralhealth.Futureresearchshould

includeaprospectivestudyevaluatingtheextentandspecifictypesdentalhealth

careinterventionsneeded.

33

APPENDIX A

Actual Qualitative Patient Survey Comments

Please add any additional comments that pertain to your experience

with ALS and Dental Considerations: (Open-Ended Response)

1. During a 30 day stain in 2 different hospitals I found dental hygiene

to the most overlooked aspect of the care I received

2. Lost filling this week, should it be put back in?

3. It is hard to hold the toothbrush as hands are weak and tire quickly.

Fingers getting weaker so much harder to floss, especially in back -

and it hurts to open my mouth wide. Why did you ask about filling

and root canals?

4. Started using a power toothbrush 6 mos ago.

5. choking episodes at dentist

6. I receive all my care including dental care through the VA.

7. when I went for my last cleaning and exam, I asked the hygenist to

keep my head higher than usual and they were happy to comply.

they have another patient with ALS that they are working with to

accomodate his needs

8. Dr. Jeffrey Day of Fredericksburg, Va. accepted me w/o any

hessitation. He is very mindfull of my als

34

9. My wife is totally bedridden. She is unable to even sit in a

wheelchair. A little over a year ago my wife experienced severe

teeth grinding. She broke the bone holding her front bottom six

teeth. They were loose and wobbly and we were afraid that she

would aspirate her teeth. Our dentist came to the house and

removed those teeth. Since then several other teeth have

"disappeared." We used to brush her teeth and suction out the

toothpaste, but we know swab her teeth with 50% peroxide and

water daily.

10. I have PLS.

11. I lost 3/4 of my teeth at age 18, the remainder at age 65

12. My dentist is not especially sympathetic to my needs. I tell him

that I do not want x-rays because I choke on the pieces that you

put in your mouth and his response is something like "well, we

need to take x-rays to spot problems with your teeth." I tell him

that I have bigger problems than my teeth, but it's in one ear and

out the other.

13. handicap accessibility, dentists willingness to deal with the

difficulties of ALS dental care, dental offices ability to accomodate

the power wheel chair in their spaces have all made dental care

difficult for my mother. I have found that county dental clinics are

the most receptive to her with her condition....private practices

35

have an array of reasons why they will not accept her...not always

sure how real those reasons are....suspect they just don`t want to

have to deal with the more complex client.

14. Biting my lip and inside of jaw.

15. none

16. Should we see a Special Dentist? Or Regular one?

17. Professional dental care not practical due to high risk of

aspirating liquids

18. gagging is an issue

19. Have used "Water Pic" both before & after onset.

20. Since I thought I had a limited time left I admit I thought one

thing I could do without was dental cleaning. Wrong! After not

having any cavities as an adult I now have two small ones and I

had a decalcified spot repaired. So it is back to the dentist I am

going. My dental office was marvelous about making things as easy

for me as possible. For instance I did not have to change rooms -

cleaning, exams, repair, x-rays, and payment processing were all

done in same room. And there is a back exit for handicap patients

to come and go.

21. My wife is very concerned as to whether or not amalgam fillings

containing mercury could be causing ALS in people. Is there

enough evidence that the amalgam fillings should be removed?

36

22. Difficult to locate a dentist that can accommodate a power chair.

23. I made my first visit to a dental facility at a VA hospital last

week. I have to wait 6 wks. to get an approval from the VA to get

outside help with my teeth.

24. did not go to the dentist before diagnoses no need. still have

not had a need to see dentist

25. i have noticed breathing difficulties when laying prone in a dental

chair. My dentist has been very accommodating in making my

dental visit as easy and comfortable as possible.

26. Harder to brush

27. No dental coverage

28. Had trouble circling (responses) wife is a dental hygienist

29. Had dentures 20+ years

30. My wife is a hygienist

31. …patient receives cleanings from dentist every three months

(prior to diagnosis, every six months) patient has difficulty opening

mouth wide enough during professional cleanings, making effective

dental care challenging

37

APPENDIXB

SurveyMonkeyPatientFlyerALSAssociationExecutiveDirectorParticipationInvitation

ThefirsttimeImet“Ben”Iwasworkingasadentalhygienistinaprivatepracticedentaloffice.BenwasnewlydiagnosedwithAmyotrophicLateralSclerosis.Wecleanedhisteeth,talkedtohimabouthishomecareandsenthimhomelikeeveryotherpatient.ButasyouknowBenwasnotlikeeveryotherpatient.ItwasnotuntilmyUnclewasdiagnosedwithALSthatIfullyunderstoodthecomplexityofALSandtheobstaclesinobtainingdentalhealthcare.Oralhealthcareisanintegralcomponenttoeveryperson’sphysicalwellbeing,includingthosediagnosedwithAmyotrophicLateralSclerosis(ALS).ItisspeculatedthatasthediseaseadvancespatientswithALSoftenfindmaintainingandreceivingoralhealthcarecanquicklybecomeachallenge.ElaineSanchezDilsandSusanJoneswiththeUniversityofNewMexicoDepartmentofSurgery,DivisionofDentalHygieneareconductingaresearchstudyinvestigatingdentalconsiderationsinALSpatients.WewouldliketoincludethepatientsthatvisittheALSCentersinyourregion.Patients,ortheirdesignatedindividual,wouldbehandedaflyeraskingthemtoparticipateinananonymousonlinesurveyabouttheirdentalexperiencessincediagnosis.Thesurveytakesabout5minutestocompleteandcouldbecompletedatthelocationandtimeoftheirchoice.Researchstudyinformationislistedbelow.Theflyerisattachedforprintinganddistribution.Ifyoupreferhardcopies,pleasecontactusandwewillmailthemdirectlytoyou.TheenddatefordatacollectionisJanuary31,2011.Pleasefeelfreetocontactuswithanyquestions.Thankyouforyourtimeandassistance,Elaine Sanchez Dils, RDH, MA Susan W. Jones, RDH, MSc Associate Professor Master’s Degree Candidate Principal Investigator Research Coordinator and Co-Investigator (505)272-0838 (505)272-4513 EDils@salud.unm.edu SWJones@salud.unm.eduUniversityofNewMexicoIRBApproved8/16/2010(HRRC#10‐200)FederalWideAssuranceNumber:00003255 Survey link:

https://www.surveymonkey.com/s/ALSdentalresearchstudy

38

APPENDIXCSurveyMonkey Patient Flyer

!""#$%&'()!'*+,$"%,-',.'*+/,$0$*+,"'$%'/"-"+/01'-,234''

!

"#$!%&'!(&)*!&!+,--*.*'%*/!!

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B8*,8*=!?=!:?,!,8*!E*:,#5!:**E>!?@!1G)!+#,;*:,>!#=*!H*;:3!F*,I!!!!

!"#$%&'$()*+I!!

!

!

To participate go to: https://www.surveymonkey.com/s/ALSdentalresearchstudy

Survey is anonymous and takes about

5 minutes to complete. !!

!

J5*#>*!+#=,;D;+#,*!H$!4#:2#=$!.%K!/0%%!!

78#:L!$?2!@?=!$?2=!,;F*I! J5*#>*!D?:,#D,!2>!B;,8!#:$!M2*>,;?:>I!!

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! ! ! ! ! ! ! ! YZQ[\!RQQT!]%0(/00I!1++=?<*E!%'(1232>,(/0%0^!

39

APPENDIXD

SurveyMonkeyPatientSurvey(Questionsnumberedinsurveylogicformat)

Den

40

41

42

43

44

APPENDIXE

ALSAssociationCertifiedCenterMedicalDirectorParticipationInvitationLetter

45

APPENDIXF

ALSAssociationCertifiedCenter–MedicalDirectorParticipationConsent (Recipient Name) Director, ALS Association Certified Center (School Name) (Street Address) (City, ST Zip code) University of New Mexico Division of Dental Hygiene 2320 Tucker NE, MSC09 5020 (505) 272-4513 Research Investigators, This letter is to confirm that (Insert Clinic Name) is willing to allow our patients, diagnosed with Amyotrophic Lateral Sclerosis to participate in the “Dental Considerations in Patients with Amyotrophic Lateral Sclerosis” research study. I am aware that by signing this letter I am acknowledging our institution and any of its employees will have the sole responsibility of distributing and collecting the anonymous surveys. It also confirms that our institution and its employees will not have further interaction with the research study. We do ask if the primary point of contact will not be you, the Director of the ALS Association Certified Center, you provide below the name, telephone number, physical address and e-mail address for the individual you would like the investigators to directly contact. I understand that if I have questions regarding the study or this letter, I may contact the Principal Investigator, Elaine Sanchez Dils (505-272-0838 – EDils@salud.unm.edu), Research Coordinator and Co-Investigator, Susan Jones (505-272-4513 – SWJones@salud.unm.edu) or the University of New Mexico Health Sciences Center Human Research Review Committee (505-272-1129). It is the understanding and expectation that the above named research study will only commence upon complete approval from the University of New Mexico Health Sciences Center Human Research Review Committee. Sincerely, (Recipient Name) Director, ALS Association Certified Center (Phone number) (E-mail address) Alternate Contact Person Information Name: Telephone Number: Address: E-mail Address:

46

APPENDIXG

ALSPatientSurvey–InformedConsentCoverLetter

Page 1 of 1

OFFICIAL USE ONLY

APPROVED 8/16/10

The University of New Mexico Human Research Review Committee

University of New Mexico Health Sciences Center

Informed Consent Cover Letter for Anonymous Surveys

STUDY TITLE

Dental Considerations in Patients with Amyotrophic Lateral Sclerosis

Professor Elaine Sanchez Dils RDH, MA and Susan Jones RDH, BS from the Department of Surgery, Division of Dental

Hygiene, are conducting a research study. The purpose of the study is to determine whether or not the dental needs of

Amyotrophic Lateral Sclerosis (ALS) patients are adequately being met. You are being asked to participate in this study

because of your ALS diagnosis.

Your participation will involve completion of a short written survey. The survey should take about 5 minutes to complete.

Your involvement in the study is voluntary, and you may choose not to participate. There are no names or identifying

information associated with this survey. The survey includes questions such as ”Since diagnosis how frequently has

patient received dental care?” You can refuse to answer any of the questions at any time. There are no known risks in

this study, but some individuals may experience discomfort when answering questions. All data will be kept for 5 years

in a locked file in Professor Sanchez Dils’ office and then destroyed.

The findings from this project will provide information on whether or not ALS patients would benefit from the inclusion

of a dental component to their ALS Association Certified Center appointments. If published, results will be presented in

summary form only.

If you have any questions about this research project, please feel free to call Elaine Sanchez Dils at (505)272-0838 or

Susan Jones at (505) 272-4513. If you have questions regarding your legal rights as a research subject, you may call the

UNMHSC Office of Human Research Protections at (505) 272-1129.

By returning this survey in the envelope provided, you will be agreeing to participate in the above described research

study.

Thank you for your consideration.

Sincerely,

Researcher’s Name & Title

Elaine Sanchez Dils, RDH, MA Susan Jones, RDH, BS

Associate Professor Masters Degree Candidate

HRRC#10-200 Version Date: 4/9/10

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APPENDIXH DentalConsiderationsinPatientswithAmyotrophicLateralSclerosis(ALS)

PatientSurvey LocationCode:

GenderofPatient: M FAgeattimeofALSDiagnosis:_____________ALSOnsetDiagnosis: BulbarOnset LimbOnset TypeofALS: Familial SporadicDoesyouhaveahistoryofmilitaryservice? Yes NoPleasecircleanswer:

1. Areyouintubated? Yes No 2. Doyouhaveafeedingtube? Yes No 3. Canyoueatfoodthroughyourmouth? Yes No 4. Priortodiagnosis,didyouhavesilver

(amalgam)toothfillingsinyourmouth? Yes No Notknown

5. Proceedtonextquestionifyouanswered‘NO’or‘NOTKNOWN’toquestion#5.Approximatelyhowmanysilver(amalgam)fillingswereinyourmouthpriortodiagnosis?

1‐3 4‐6 7orgreater

Notknown

6. Priortodiagnosis,didyouhaverootcanaltherapyonanyteethinyourmouth? Yes No Not

known

7. Proceedtonextquestionifyouanswered‘NO’or‘NOTKNOWN’toquestion#6.Approximatelyhowmanyrootcanaltherapiesweredoneinyourmouthpriortodiagnosis?

1‐3 4‐6 7orgreater

Notknown

8. Priortodiagnosis,howfrequentlydidyoureceiveprofessionaldentalcare? Only

withpain Rarely

2ormore

timesperyear

Notknown

9. Sincediagnosis,howfrequentlyhaveyoureceivedprofessionaldentalcare? Only

withpain Rarely

2ormore

timesperyear

Notknown

10. Sincediagnosis,hasthefrequencyofprofessionaldentalcaredecreased? Yes No Not

known

11. Sincediagnosis,haveyouseenanincreaseddifficultyaccessingregulardentalcare? Yes No Not

known

12. Whoisresponsibleforperformingyourdailyhomeoralcare(brushing,flossing,etc)? Patient Caregiver Other

13. Whatdailyhomecareregimesareperformedregularly(circleallthatapply)? Brushing Flossing Swabbing Rinses Other

14. HaveyoureceivedinformationregardingoralhealthordentalcareduringyourALSAssociationCertifiedCenterappointments?

Yes No

*PleaseaddanyadditionalcommentsthatpertaintopatientexperienceswithALSandDentalconsiderations.

48

APPENDIXIStudyCollaborators

MedicalDirector,ZacharySimmons,M.D.

ResearchCoordinator,Helen(Beth)StephensM.A,CCRC

TheALSClinicatPennStateMiltonS.HersheyMedicalCenter

Hershey,Pennsylvania

MedicalDirector,SarahYoussof,MD

ALSCenterUniversityofNewMexico

Albuquerque,NewMexico

MedicalDirector,KevinFelice,D.O.

AssistantResearchDirector,LavanyaRajachandranPh.D

Neuromuscular/ALSClinic

HospitalforSpecialCare

NewBritain,Connecticut

MedicalDirector,EzgiTiryaki,MD

ResearchCoordinator,CindyRhode,RN

HennepinFacultyAssociates,MultispecialtyClinic

MinneapolisMedicalResearchFoundation

Minneapolis,Minnesota

MedicalDirector,ToddLevine,M.D.

BannerGoodSamaritanMedicalCenter

Phoenix,Arizona

49

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