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