navigating the new era in ipf: coordination of care faculty title affiliation
TRANSCRIPT
NAVIGATING the NEW ERA in IPF:
Coordination of CareFACULTY
TitleAffiliation
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
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
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
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
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.
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.
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?
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.
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)
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.
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
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
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.
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
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
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)
Stay Active
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
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
Referral to ILD CenterTiming Matters
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.
Delayed Care Associated with Higher Mortality
Lamas et al. Am J Respir Crit Care Med. 2011;184:842-847.
P for trend = 0.04
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.
Referral for Lung Transplantation
Lung Transplantation is Increasing
http://www.ishlt.org/registries/slides.asp?slides=heartLungRegistry. Accessed August 2014.
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19921993
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19981999
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20042005
20062007
20082009
20102011
0
500
1,000
1,500
2,000
2,500
3,000CF IPF COPD Alpha-1 IPAH Re-Tx
Num
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f Tra
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ants
IPF
CF
COPD
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].
IPF Impairs Quality of Life
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
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
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
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
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
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