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IdiopathicPulmonaryFibrosis:TakeControlwithKnowledge
Today’sProgram
§ Part1:UnderstandingtheDiagnosis
§ Part2:OptionsforTreatment
§ Part3:PatientandCaregiverSupport
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Part1:UnderstandingtheDiagnosis
IdiopathicPulmonaryFibrosis(IPF)
§ Scarringofthelungsofunknowncause
§ AsIPFprogresses,itprohibitsthelungsfrommovingoxygenintothebloodstream
§ OneofthemanyInterstitialLungDiseases(ILDs)
§ Consideredadiagnosisofexclusion– mustexcludeanumberofotherpossibilitiesbeforediagnosiscanbemade
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https://guide.eu-ipff.org/what-is-idiopathic-pulmonary-fibrosis/
ClinicalCourseofIPF
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Walter N et al. Proc Am Thorac Soc. Vol 3. 330–338, 2006
Whatwasmostchallengingforyou,oryourlovedones,atthetimeofdiagnosis?
A. StrugglingtounderstandandexplainIPF
B. LearningaboutlifeexpectancywithIPF
C. FeelingisolatedandunsupportedtocopewithandmanagethediagnosisofIPF
D. Concernfortheimpactthediseasewouldhaveuponfamily
E. Alloftheabove
F.
Selectoneresponse
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Radiology,PathologyandPhysiologyofIPF
§ChestCT(CATscan)• LookingforpatternofabnormalitiescalledUsualInterstitialPneumonia(UIP)• 4classifications:Definite;Probable;Indeterminate;Alternativediagnosis
§ Lungbiopsy(VATS)• LookingforpatternofabnormalitiescalledUsualInterstitialPneumonia• SimilarclassificationasCT
§PulmonaryFunctionTests(PFTs)• Restrictivedefects(lowlungvolumes),diffusingcapacity
abnormalities(compromisedabilitytoabsorbO2)
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RespiratorySymptoms
§ Shortnessofbreath
§ Cough
§ Chestcongestion
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HowtoCometoaDiagnosisofIPF
§ Diagnosisismadewhenacauseofthefibrosiscannotbeidentified,andthereisnounderlyingillnesswithwhichfibrosiscanbeassociated(auto-immuneconditions)AND§ TheCT,PFTs,biopsyallsupportIPF
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ChallengesatTimeofDiagnosis
§Makingsenseofoftenunheardofdisease
§ Acceptingdiagnosisbeforebeingabletomove
forward
§ Communicatingmedicalissuestoothers
§ Learningtolivewithphysicalconstraints
§ Facingrealitiesofadvancedcareplanning
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GeneticsofIPF
§ Patientsoftenhaveconcernsforfamilymembers
becomingaffected
§Majorityoftestingbeingdoneinresearchsettings
§ RegistryofIPFrelatedgenetictestingoptions:
• https://www.ncbi.nlm.nih.gov/gtr/all/tests/?term=C180
0706&filter=testtype:clinical
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PatientStory
Q:ASKTHEPANELquestionsaboutyourIPFdiagnosis
Part2:OptionsforTreatment
AfterdiagnosisofIPF,whatwasthefirsttreatmentyou,oryourlovedone,received?
A. IPFdrugs(OFEV® orEsbriet®)
B. Pulmonaryrehabilitation
C. Oxygentherapy
D. Enrollmentinaclinicaltrial
E. Haven’t/hasn’tstartedtreatment
F.
Selectoneresponse
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TreatmentOptions
§ Twooralmedications,Esbriet(pirfenidone)andOfev (nintedanib)
§ Bothslowthelossoflungfunctionbyabout50%§ Initialchallengesareidentifyingwhichtochoseandwhentostart
§ Somepatients/physicianschosetostartimmediatelyafterdiagnosismade,otherstakewatchfulwaitingapproach
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TreatmentOptions
§ Strategiestointroducemedications
• Considerslowingdoseescalation,increasedoseonlywhenmedicationisbeingwelltolerated
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TreatmentOptions– pirfenidone (Esbriet)
§ Standarddosingtitrationschedule:• 1capsule/tabthreetimesadayx1weekthen• 2capsules/tabsthreetimesadayx1weekthen• Three267mgcapsules/tabletsthreetimesaday(orone801mgtabthreetimesaday)
§ Symptomdrivenalternativeschedule– onlyincreasedosewhentoleranceachieved• Start1capsule/tabthreetimesaday• Iftolerableafter1-3weeks,increaseto2capsules/tabsthreetimesaday• Iftolerableafter1-3weeks,increaseto3capsules/tabsthreetimesaday
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TreatmentOptions– pirfenidone (Esbriet)
§ Canalsoconsiderincreasingby1capsule/tabperweek,i.e.increasefromatotalof3adayto4,then4adayto5,etc.
§ Ifsideeffectsdevelop,returntothedoseatwhichtherewereminimalornosideeffects– oncesymptomsresolve,startaslowerdoseescalation
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TreatmentOptions– nintedanib (Ofev)
§ Standarddosingtitrationschedule:• Nouptitration,startat1capsuletwicedaily
§ Symptomdrivenalternativeschedule– onlyincreasedosewhentoleranceisachieved,i.e.startwith1capsule,iftolerating,increaseto1capsuletwicedailyafter1-2weeks
§ Ifsideeffectsdevelop,returntodoseatwhichtherewereminimal/nosideeffects– slowdoseescalation
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TreatmentOptions
§ Surveillance• Monthlyappointmentsforthefirst3-6monthswhichincludelabwork– liverfunctiontests(LFTs)
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TreatmentAdherence–ManagingSideEffects:Gastrointestinal
§ Pirfenidone:nausea,upsetstomach,acidreflux,vomiting,lossofappetite,diarrhea• Doseescalationstrategiesasalreadydiscussed• Takingthe3capsules/tabletsatseparatetimesduringameal• PPI(e.g.omeprazole),H2-blocker(e.g.famotidine,ranitidine)• Pro-motilityagents• Anti-nauseamedications
§ Nintedanib:diarrhea,nausea,vomiting,anorexia• Loperamide(Imodium)– canbeusedasneededorscheduledroutinely1-2timesaday(2-16mgdaily)
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TreatmentAdherence–ManagingSideEffects:Rash
Pirfenidone:importanttodeterminewhetherphotosensitivityvs.drugeruption§ Toavoidphotosensitivity– sunscreen,sunprotectiveclothing
§ Drugeruptionnotrelatedtosunexposuretypicallyrequirespermanentdiscontinuationofthemedication
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TreatmentAdherence– ManagingSideEffects:FatigueandLossofEnergy
§Maintaingoodposture§ Avoidunnecessarytasks§Organizeyouractivities§ Reorganizeyourclosetsandshelves§ Keepduplicatesoffrequentlyuseditems
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TreatmentAdherence– ManagingSideEffects:FatigueandLossofEnergy
§ CookonSundayfortheentireweek§ Investinarollingutilitycartorfour-wheeledwalker§ Askforhelp§Getadisabilityplateorplacard
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TreatmentOptions–WhentoChangeMedications
§ SignificantlyelevatedLFTs§ Intolerable/unmanageablesideeffects• Ifthesecondmedicationtriedisworse,canreturntofirstifsafetodoso(ifGIsymptomswerepredominantreasontochangeinitially)
§ Drugrash• Non-photosensitivityrash,orifphotosensitivityrashandsunsimplycannotbeavoided
§ Lackofefficacy– hardtodefine,considerifFVCconsistentlyfalling>200mL/yr whileontreatment
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OtherIllnessesorComplicationsoftheDiseaseThatDeserveAttention
§GERD– acidreflux§ Pulmonaryhypertension§ Cardiovasculardisease§ Lungcancer
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SymptomManagement- StrategiestoManageShortnessofBreath
§ Controlyourbreathing§Maintaingoodposture§ Practicerelaxationtechniques§ Supplementaloxygen§ Pulmonaryrehabilitation
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SymptomManagement–StrategiestoManageCough
§ Commonalternativecausestoconsider:• Post-nasaldripfrominflammation/irritationofnoseand/orsinusescanbeeitherallergicornon-allergicinnature• Asthma/allergies• Esophagealreflux(acidreflux,GERD)• Smoking,COPD– chronicbronchitis• Medications(ACEinhibitors)
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SymptomManagement–StrategiestoManageCough
§Medications:• Overthecounter:
⎻ Dextromethorphan(maxdose120mg/day)• Prescription:
⎻ Opiates- codeine,hydrocodone⎻ Benzonatate⎻ Prednisone(mayrelievecongestion)
• Nebulizedoptions:⎻ Saline⎻ Hypertonicsaline⎻ Ipratropium/albuterol⎻ Lidocaine⎻ Morphine/hydromorphone
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AcuteExacerbations
§ Significantworseningofrespiratorysymptoms,oxygenneeds,CToveracourseofdaystoasmallnumberofweeks,notexplainedfullybyanalternativediagnosis
§ Provoked(triggered)orUnprovoked(idiopathic)§ ProvokedAEstypicallytriggeredbysomestressplaceduponthelungs(pneumonia,mechanicalventilation,infectionelsewherewith/withoutsepsis,drugtoxicity,aspirationevent)
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AcuteExacerbations
§ Prevention:• Maintainvaccinations• Handwashing/avoidsickcontacts• ManageGERDappropriately• Avoidairborneirritants/pollutants• Avoidlungtoxicmedications• Pirfenidone /nintedanib
§ Treatment• HighflowO2>non-invasiveventilation>mechanicalventilation• Steroids,empiricantibiotics,diuresiswhenappropriate
§ Iftransplantisbeingconsidered,maintainlinesofcommunicationwithtransplantteam
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FollowingDiseaseProgression
§ Evolutionofsymptoms§ Pulmonaryfunctiontestand6minutewalktestresults
§Oxygenneeds§ CTfindings
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PatientStory
Q:ASKTHEPANELquestionsaboutyouroptionsfortreatment
Part3:PatientandCaregiverSupport
WhataboutIPFconcernsyoumost?
A. Understandingtreatmentsand/ortrialsavailabletome
B. Theuncertaintyoftheprognosis
C. PreparingforatimewhenIwilllosemyindependence
D. Effectofshortnessofbreathandcoughuponmydailyactivities
E. Decidingonwhethertopursuelungtransplantation
F.
Selectoneresponse
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Non-PharmacologicalTreatment–OxygenTherapy
§ CriteriaforstartingO2§ Pulsedvs.continuousflow§ Conservingdevices§Nasalcannulas- findingtherightfit§Usinganationalorlocalcarrier
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Non-PharmacologicalTreatment–OxygenTherapy
§ ImportanceofUsingSupplementalOxygenforthosewhorequireit• Everyoneneedssufficientoxygentosupportexertionorexercise• Lowoxygenlevelsplacesastrainontheheartthatovertimecanleadtoelevatedpressuresintheheartandlungs.Thismayeventuallycausethepumpactionofthehearttobelessefficient• Lowoxygenlevelcancausefatigueandlimitexercisecapacity• Lowoxygenlevelscanalsodecreaseone’sabilitytothinkclearly
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Non-PharmacologicalTreatment–PulmonaryRehabilitation
§ Exerciseprogramthatoccursinasafe,monitoredenvironmentwhilelearningmoreabouthowtomanageyourcondition
§ Commongoals:• Reverse/minimizetheeffectsofdeconditioning• Improvestrengthandflexibilityofperipheralmuscles(armsandlegs)• Improvestabilityofcoremuscles(trunkandabdominals)• Improveleveloffitness/aerobiccapacity• Learnpacingstrategiesandacclimatetoshortnessofbreath
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Non-PharmacologicalTreatment–PulmonaryRehabilitation
§ Pulmonaryrehabilitationisrunbyateamofphysicaltherapists,respiratorytherapists,nurses,physicians,nutritionists,etc.
§ InitialAssessment:• Establishabaselineleveloffunction• Assessposture,strength,rangeofmotion,flexibility,balance,sensation,andwalkingpattern• 6minutewalktesttoassessaerobiccapacity– repeatedhisattheendofrehabtogaugeimprovement
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Non-PharmacologicalTreatment–PulmonaryRehabilitation
§ StructureoftheProgram:• Frequency:3-5x/week• Intensity:Finda“sustainablechallenge”byreachingintoreservecapacityandworkingatalevelbeyondmostdailyactivities.• Type:needtoutilizeatypeofexercisethatismeaningfultoyourdailyfunction/lifedemands• Time:needtotrytoachieve20minutesormoreofaerobictypeexercise;cumulativeintervalsarefine,keepingrestperiodsshort.Thisisaprogressiveincreaseintime,workingtowardcontinuousexercise,ifpossible
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Non-PharmacologicalTreatment–PulmonaryRehabilitation
§Whathappenswhentheprogramisover?• Duringrehab,anexerciseprogramforhomeisdeveloped,itisessentialtocontinueafterthestructuredprogramends• Gainsstarttodwindlerightawayifyoustopexercising,butallisnotlost….youjusthavetoresumeexerciseandworktowardsbuildingupyourenduranceagain• Ifyoustopexercisingforseveralweeks,youwillhavetostartagainatamuchlowerintensityorperhapsinintervals,andworkyourwaybackup• Somepulmonaryrehabcentersoffermaintenanceprogramsonsite
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TransplantEvaluation– KeyIssues
§ Decidingwhethertopursue§ Timingoftransplant§What’sinvolvedintheevaluationprocess§ Strategiesforstayingfitduringlaterstages§ Preandpost-transplantsupportgroups
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GoalsofCare
§ AlleviatingsymptomslikecoughandSOB§Managingmedications’sideeffects§ Beingactiveandeatinghealthydiet§ Avoidingcoldsandinfections§Utilizingsupplementaloxygenappropriately§ Supportwithadvancedcareplanningandpalliativecareresources
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GoalsofCare
§ AdvancedCarePlanning• LivingWill• EstablishingaSupportNetwork/CareTeam
§ PalliativeCare• Specializedmedicalcarefocusedonpainandsymptomcontrol,communication/coordination,emotionalsupport,family/caregiversupport
• Appropriateatanystageofillness,usedinparallelwithothertherapies
• Goal:Wellness,improvedqualityoflife§Hospice
• Apalliativecareapproachattheendoflife• Focusisattendingtomedical,social,psychologicalandspiritualneedsofpatientsandfamiliesattheendoflife
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JoinaLocal/VirtualSupportGroup
§ Findingpeer-to-peerconnection&support
§Offeringanswersfrompersonalexperiences
§ Learning&sharingaboutlivingwithIPF
§ Advocatingforholisticcare§ Bringinghopetothenextperson
§ Appreciatingeverydayasavictory
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SupportCareResources
§ InterstitialLungDisease(ILD)Collaborative• Apatient-physiciancollaborativeforunderstanding,management,andtreatmentofInterstitialLungDiseases.
• http://www.ildcollaborative.org/about
§ PulmonaryFibrosisFoundation• ConnectwithalocalsupportgroupandmedicalcenterinthetreatmentofpulmonaryfibrosisaroundtheUnitedStates.
• http://www.pulmonaryfibrosis.org/life-with-pf/find-medical-care?show=support
§ AmericanLungAssociation• Offersfreeonlinecommunitieswithpeer-to-peersupportforthoseLivingwithPulmonaryFibrosisandthoseCaringforPulmonaryFibrosis.
• http://www.lung.org/support-and-community/online-support-communities/
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PatientStory
Q:ASKTHEPANELanyquestions,includingresourcesforongoingsupport
Thankyou
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