Transcript A New Era in IPF: Coordination of Care
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
• • •
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
The Disease Course of IPF is Extremely Variable
“No one is average.” – David Lederer, MD
(5-10% of patients per year)
King TE Jr, et al. Lancet. 2011;378(9807):1949-1961.
Monitoring for Disease Progression
• • Every 3 to 6 months: – PFTs – 6MWT (distance/nadir saturation) – O 2 requirement – Comorbidities – Consider dyspnea questionnaire (UCSD) HRCT – Annually or when suspicion for clinical worsening
Disease Severity and Progression in IPF TESTS/CLINICAL FACTORS
DL CO 6MWT FVC Pulmonary hypertension Dyspnea score Respiratory event
PREDICTIVE VALUE
• Baseline values or degree of change over time predict survival • • • • Desaturation predicts survival Distance predicts survival Heart rate recovery after 6MWT predicts survival Baseline values or degree of change over time predict survival • Associated with higher mortality • Correlates with disease progression • 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 Supplemental oxygen Age-appropriate vaccinations Risk factor reduction Pulmonary rehabilitation Clinical trials Lung transplant evaluation Patient education Advocacy group involvement Mental health needs Referral Opportunity?
x x x x x x x
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 SpO 2 > 89% – Rest, activity, sleep Give patients control over their disease Make sure patients are using O 2 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-conditions Accessed 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.
Pulmonary Rehabilitation Studies in IPF
Author Swigris et al.
Salhi et al.
Ferreira et al.
Kozu et al.
Citation Respir Care 2011 Chest 2010 Chest 2009 Respiration 2011 Holland et al.
Nishiyama et al.
Thorax 2008 Respirology 2008 Dyspnea Trend Better Better Better Same QOL Trend Same Energy Better 6MWD Other 61.6m
79m 56m 16m 25.1m
46m Fatigue Anxiety Depression Force ADL VO 2 * Statistically significant
Exercise Training Effect on 6MWD 100
Control Exercise
50
*
0
• •
-50 -100
Training
Baseline 9 Weeks
*
P
< 0.05
26 Weeks
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.
Baseline 6MWD Predicts Improvement After Training Baseline 6MWT Distance(m)
• • • • 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.
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 Center
Timing 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 evaluation Flaherty
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
P
for trend = 0.04
Lamas et al. Am J Respir Crit Care Med. 2011;184:842-847.
Benefits of Early ILD Center Referral
4,0 3,0 2,0 1,0 0,0 Mortality Transplantation < 1 1 to 2 2 to 4
Years From Symptoms Onset to ILD Center Referral
> 4 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
3 000 CF IPF COPD Alpha-1 IPAH 2 500 Re-Tx 2 000
COPD
1 500 1 000 500
IPF CF
0 http://www.ishlt.org/registries/slides.asp?slides=heartLungRegistry. Accessed August 2014.
Lung Transplantation for IPF: 2014 Referral Guidelines
• • • • Histopathologic or radiographic evidence of usual interstitial pneumonitis (UIP) Abnormal lung function: FVC < 80% predicted or DL CO < 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 ➙ • activity 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
Fatigue Decreased functional capacity Deconditioning Idiopathic Pulmonary Fibrosis Physiologic restriction and impaired gas exchange ?
Hypoxemia ?
Impaired cognition Dyspnea and Tachypnea Anxiety and Fear Physical inactivity Decreased Social Participation Depression Loss of control
Swigris JJ, et al. Respir Med. 2008;102(12):1675-1680.
Impaired QOL
Summary: Actions for Practitioners
• • • • • • Educate patients – Refer to reliable sources Prescribe O – 2 (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 – – Local support groups On-line resources – – – – – Jeff Swigris: www.pulmonaryfibrosisresearch.org/blog David Lederer: PFDoc.org
www.patientslikeme.com
www.coalitionforpf.org
www.pulmonaryfibrosis.org
www.lungsandyou.com
www.knowipfnow.com