navigating the new era in ipf: coordination of care faculty title affiliation

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NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

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Page 1: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

NAVIGATING the NEW ERA in IPF:

Coordination of CareFACULTY

TitleAffiliation

Page 2: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Learning Objectives

• Formulate a plan for communication with patients and families on all aspects of care

• Identify opportunities for referral as part of the multidisciplinary IPF management plan

Page 3: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Approach to Patient Communication

• Discuss disease course, monitoring tests, therapy options– Literacy, numeracy, language

• Give patients a role in their management– Share ILD checklist– Provide information resources – Specific patient actions (rehab, exercise, risk factor reduction,

oxygen, etc)– Seeking help from family or support group

• Share next steps– ILD referral?– Lung transplantation evaluation– Select appropriate IPF therapy

Page 4: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

King TE Jr, et al. Lancet. 2011;378(9807):1949-1961.

(5-10% of patients per year)

The Disease Course of IPF is Extremely Variable “No one is average.” – David Lederer, MD

Page 5: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Monitoring for Disease Progression

• Every 3 to 6 months:–PFTs –6MWT (distance/nadir saturation)–O2 requirement –Comorbidities–Consider dyspnea questionnaire (UCSD)

• HRCT–Annually or when suspicion for clinical worsening

Page 6: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Disease Severity and Progression in IPF

TESTS/CLINICAL FACTORS PREDICTIVE VALUE

DLCO• Baseline values or degree of change over time

predict survival

6MWT• Desaturation predicts survival• Distance predicts survival• Heart rate recovery after 6MWT predicts survival

FVC • Baseline values or degree of change over time predict survival

Pulmonary hypertension • Associated with higher mortality

Dyspnea score • Correlates with disease progression

Respiratory event • Predicts worse survival

Ley B, et al. Am J Respir Crit Care Med. 2011;183(4):431-440.

Page 7: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Past Performance May Predict Future Returns

• Variable disease trajectory, not attributable to stage at diagnosis (lead-time bias)

• Continuum of phenotypes of longevity• Discussion of prognosis should not be a one-time

occurrence, but rather a dynamic counseling aspect of patient care

Brown AW, et al. Chest. 2012;142(4):1005-10.

Page 8: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

ILD Checklist

• Therapeutic options x• Supplemental oxygen• Age-appropriate vaccinations • Risk factor reduction x• Pulmonary rehabilitation x• Clinical trials x• Lung transplant evaluation x• Patient education • Advocacy group involvement

x• Mental health needs x

ReferralOpportunity?

Page 9: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Two New Therapies Approved October 2014

• Oral drugs slow the decline of FVC in IPF patients with mild/moderate disease–Pirfenidone –Nintedanib

• No head-to-head trials• Choice based on clinical profile, patient

preference, drug/drug interactions, and side effect tolerance

King TE, et al. N Engl J Med. 2014;370(22):2083-2092.Richeldi L, et al. N Engl J Med. 2014;370(22):2071-2082.

Page 10: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Oxygen Therapy

• Goal: Maintain SpO2 > 89%– Rest, activity, sleep

• Give patients control over their disease• Make sure patients are using O2 correctly

• Regular assessment– Nocturnal oximetry

(yearly or with change in status)– Exercise oximetry (q3 months)

Page 11: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Air Travel with Oxygen

• Altitude simulation testing (when available)• Oxygen and arrangements• Airline checklist• Oxygen supplier checklist

Adapted from TSA Web site. http://www.tsa.gov/traveler-information/travelers-disabilities-and-medical-conditionsAccessed July 2014.

Page 12: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Risk Factor Reduction

• Smoking cessation• Weight management• Sleep study • Exercise training/pulmonary rehab• Screen and address co-morbidities

– GERD– OSA– Heart disease (diastolic dysfunction/PH/CAD)– Thromboembolic disease

Page 13: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Recreational Activities

• Normalcy should be maintained as much as possible• Regular activities give rhythm to life• Low intensity activities enhance pleasure and social

contact– Socializing – Cultural activities– Family events– Sexual activity– Exercise

Page 14: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Pulmonary Rehabilitation

• Program originally designed for COPD• Education, exercise, support/counseling • Run by PT/RT• Goals:

– Improve self-management– Reduce symptoms– Optimize functional capacity– Increase social participation

Holland AE, et al. Thorax. 2008;63:549-554.

Page 15: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Author Citation Dyspnea QOL 6MWD Other

Swigris et al. Respir Care 2011 Trend Trend 61.6mFatigue

Anxiety Depression

Salhi et al. Chest 2010 - - 79m -

Ferreira et al. Chest 2009 Better - 56m -

Kozu et al. Respiration 2011 Better Same 16m Force ADL

Holland et al. Thorax 2008 Better Energy 25.1m VO2

Nishiyama et al. Respirology 2008 Same Better 46m -

* Statistically significant

Pulmonary Rehabilitation Studies in IPF

Page 16: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Exercise Training Effect on 6MWD

• 57 subjects (34 with IPF) randomized to 8 weeks of PR or weekly telephone support

• Conclusions– Exercise training improves exercise capacity and symptoms in patients with ILD– Benefits are not sustained at 6 months

Holland AE, et al. Thorax. 2008;63:549-554.

*P < 0.05

100

0

-50

-100

50 *

Baseline 26 Weeks

9 Weeks

Ch

ang

e in

6M

WD

(m

) ControlExercise

Training

Page 17: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Baseline 6MWD Predicts Improvement After Training

• 3 center retrospective study of 113 subjects with ILD (50 IPF)• Multifaceted PR for 2-3 hours 2-3x/week for > 6-8 weeks• Borg score and 6MWD improved after PR (P < 0.0001)• Higher baseline 6MWD predicts a smaller improvement after PR (P < 0.0001)

Ferreira A, et al. Chest. 2009;135(2):442-447.

Ch

ang

e in

6M

WT

Dis

tan

ce(m

)

Baseline 6MWT Distance(m)

Page 18: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Stay Active

Page 19: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Communication is Key

• Use tools appropriate to patient– Whiteboard in office– Printed material– Web links

• Help patients sustain activities of daily life– Occupational therapy consult– Palliative care consultation

• Individualize!– Optimist/pessimist– Rate of information sharing– Amount of detail– Support mechanisms

• Discuss newly approved therapies

Page 20: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Support Groups

• Education– From the facilitator/guest speaker– From others in the group

• Support– Reduces isolation– Builds community– Shared coping practices

• Gets patients out of the house• Not for everyone

Page 21: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Referral to ILD CenterTiming Matters

Page 22: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Why refer early to an ILD Center?

• Diagnostic expertise–Standardized assessment–Confirmation of diagnosis

• Management expertise–Choice of an appropriate therapy–Oxygen prescription–Pulmonary rehabilitation–Attention to obesity and sarcopenia/frailty–Potential enrollment in a clinical trial –Transplant evaluationFlaherty et al. Am J Respir Crit Care Med 2004;170:904-10.

Flaherty et al. Am J Respir Crit Care Med 2007;175:1054-60.Lamas et al. Am J Respir Crit Care Med 2011;184:842-7.

Page 23: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Delayed Care Associated with Higher Mortality

Lamas et al. Am J Respir Crit Care Med. 2011;184:842-847.

P for trend = 0.04

Page 24: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Benefits of Early ILD Center Referral

< 1 1 to 2 2 to 4 > 40.0

1.0

2.0

3.0

4.0Mortality Transplantation

Years From Symptoms Onset to ILD Center Referral

Adj

uste

d H

azar

d Ra

tio

Hazard ratios adjusted for age, FVC%, gender, and socioeconomic status

Lamas et al. Am J Respir Crit Care Med. 2011;184:842-847.

Page 25: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Referral for Lung Transplantation

Page 26: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Lung Transplantation is Increasing

http://www.ishlt.org/registries/slides.asp?slides=heartLungRegistry. Accessed August 2014.

19901991

19921993

19941995

19961997

19981999

20002001

20022003

20042005

20062007

20082009

20102011

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1,500

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Num

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nspl

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IPF

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COPD

Page 27: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Lung Transplantation for IPF:2014 Referral Guidelines

• Histopathologic or radiographic evidence of usual interstitial pneumonitis (UIP)

• Abnormal lung function: FVC < 80% predicted or DLCO < 40% predicted

• Any dyspnea or functional limitation attributable to lung disease

• Any oxygen requirement, even if only during exertion

Weill D, et al. J Heart Lung Transplant.2014 Jun 26. [Epub ahead of print].

Page 28: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

IPF Impairs Quality of Life

Page 29: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

IPF Impairs QOL in Multiple Domains

• Symptoms– Dyspnea a➙ ctivity limitation

• Can’t do X anymore• Can’t do Y as quickly as I’d like• Can’t do Z without having to stop and rest

– Fatigue / exhaustion– Cough

• Dry, Nagging, Never satisfying

• Outlook– Hopeless / Abandoned– Frustrated / Uneducated / Embarrassed

Page 30: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Swigris JJ, et al. Respir Med. 2008;102(12):1675-1680.

Physiologic restriction

and impaired gas exchange

Hypoxemia

Impaired cognition

??

Loss of control

Fatigue

Dyspnea and Tachypnea

Physical inactivity

Anxiety and Fear

Depression

Decreased Social

Participation

DeconditioningDecreased functional capacity

Impaired QOL

Idiopathic Pulmonary

Fibrosis

Page 31: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Summary: Actions for Practitioners

• Educate patients – Refer to reliable sources

• Prescribe O2 – (screen for resting/nocturnal/exertional requirement)

• Prescribe medication• Look for treatable comorbid conditions• Refer

– Pulmonary rehab– ILD center – Lung transplantation evaluation

• Monitor for disease progression

Page 32: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Summary: Actions for Patients

• Get educated• Notify if change in status/new symptoms • Focus on things that he/she can still do

–Stay active–Consider pulmonary rehab program

• Adhere to management plan• Participate in research• Consider a support group

Page 33: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Physician Resources

• PILOT–www.pilotforipf.org

• Pulmonary Fibrosis Foundation–http://www.pulmonaryfibrosis.org/

• Clinical trials–http://www.clinicaltrials.gov/ct2/search

• Patient Counseling Tools–http://www.pilotforipf.org/patient_tools.php

Page 34: NAVIGATING the NEW ERA in IPF: Coordination of Care FACULTY Title Affiliation

Patient Resources

• INSPIRE support groups– https://www.inspire.com/conditions/pulmonary-fibrosis/

• Pulmonary Fibrosis Physician Blogs– Jeff Swigris: www.pulmonaryfibrosisresearch.org/blog– David Lederer: PFDoc.org

• Local support groups• On-line resources

– www.patientslikeme.com– www.coalitionforpf.org– www.pulmonaryfibrosis.org– www.lungsandyou.com– www.knowipfnow.com