Conflicts of interest. Objec5ves / Outline. Pulmonary rehabilita5on. Pulmonary rehabilita5on in COPD. Pulmonary rehabilita5on in COPD 11/26/13

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1 Conflicts of interest UBC I have no conflicts of interest related to this presenta5on Treatment op+ons From a global perspec+ve: Non- pharmaceu+cal treatments Pulmonary Fibrosis Founda5on Summit La Jolla, CA December 7, 2013 Christopher J. Ryerson, MD FRCPC Department of Medicine, UBC Vancouver, Canada I receive research and/or clinical funding from: Intermune Medimmune Actelion Gilead I receive advisory board/consulta5on fees from: Intermune Objec5ves / Outline Review evidence & recommenda5ons for non- pharmaceu5cal treatment op5ons of IPF Pulmonary rehabilita+on Vaccina,on Support groups & educa5on Diet / Nutri5on Lung transplanta5on Pulmonary rehabilita5on A structured exercise and educa5onal program that involves aerobic condi5oning, strength and flexibility training, educa5onal lectures, nutri5onal interven5ons, and psychosocial support Usually 2-3 hours per session, 2-3 sessions per week, total dura5on of 6-9 weeks Followed by a self- supervised home exercise program Nici et al, AJRCCM 2006;173:1390. Pulmonary rehabilita5on in COPD Pulmonary rehabilita5on in COPD Benefits Exercise capacity Intensity of breathlessness Health- related quality of life Anxiety and depression Number of hospitaliza5ons and days in the hospital Recovery a`er hospitaliza5on Arm func5on Effects beyond dura5on of PR Effect of long- ac5ng bronchodilators Survival Respiratory muscle func5on Evidence Weak GOLD recommends PR in COPD pa5ents with breathlessness when walking at their own pace on level ground 1

2 ILD clinicians & researchers generally believe PR works PR works in COPD Many mechanisms of benefit in COPD likely apply to pa5ents with ILD (i.e. cardiovascular performance, peripheral muscle func5on, some educa5on components) Anecdotal clinical experience in pa5ents with ILD Several studies in ILD suggest similar effects to COPD 2 RCTs and several cohort studies Two RCTs of PR in ILD 6- minute walk distance (and several consistent cohort studies) All ILD Nishiyama et al, 2008 Holland et al, 2008 Popula+on IPF: n = 28 ILD: n = 57 (IPF n = 34) Interven+on 2 sessions per week x 10 weeks 2 sessions per week x 8 weeks Prescrip5on for long- term home exercise program Control Not stated Telephone support Outcomes 6MWD, QOL, dyspnea, exercise capacity Post- rehab only 6MWD, QOL, dyspnea, exercise capacity Post- rehab + 6- month follow- up PR improves 6MWD by 39 metres in ILD (95% CI: 15 to 62) No effect at 6 months: 7.4 metres (95% CI: - 36 to 51) (Holland study only) Nishiyama et al, Respirology 2008;13:394; Holland et al, Thorax 2008;63: minute walk distance Quality of life All ILD Baseline IPF only Follow- up PR improves 6MWD by 27 metres in IPF (95% CI: 3 to 50) PR improves QOL, but unclear long- term effect 2

3 Dyspnea Baseline Follow- up The majority of pa5ents with IPF should be treated with pulmonary rehabilita5on (weak recommenda5on, low- quality evidence) IPF Clinical Prac5ce Guideline The panel voted: 19 to 0 in favor of PR in IPF PR transiently improves dyspnea Raghu et al, AJRCCM 2011;183:788. Symptoma5c endpoints Quality of life Dyspnea Depression Func5onal endpoints Exercise capacity Physical ac5vity Short- term Long- term? p=0.15 Who should be treated? RCT & one cohort study suggest greater improvement with earlier PR Higher FVC & nadir SpO 2, lower RVSP à Greater benefit users benefit less Other cohort studies suggest greater improvement in more advanced disease Lower baseline 6MWD à Greater benefit Benefit in hospitalized ILD pa5ents Muscle func5on? p=0.06 Ryerson et al, ATS 2013 abstract; submiped for peer review. Holland et al, RespMed 2012;106:429; Ferreira et al, Chest 2009;135:442; Huppmann et al, ERJ 2013;42:444; Johnson- Warrington et al, JCardiopulmRehabPrev 2013;33:189. Who should be treated? is not a drug (?) Center for Medicare and Medicaid Services considers oxygen to be equipment Selected pa5ents with a severely impaired func5onal capacity can s5ll benefit from rehabilita5on hpp:// therapy.html 3

4 Mul5ple RCTs in COPD, including MRC & NOTT trials that showed improved survival with long- term oxygen therapy in pa5ents with significant hypoxemia PaO 2 60 mmhg Similar improvements in quality of life and other endpoints Cumula5ve survival Con5nuous oxygen Nocturnal oxygen Time from randomiza5on General recommenda5ons PaO 2 55 mmhg (or SpO 2 <88%) PaO 2 59 mmhg (or SpO 2 <89%) + right heart dysfunc5on Con5nuous or nocturnal oxygen therapy in hypoxemic chronic obstruc5ve lung disease: a clinical trial. Nocturnal Therapy Trial Group. Ann Intern Med 1980; 93:391. Long term domiciliary oxygen therapy in chronic hypoxic cor pulmonale complica5ng chronic bronchi5s and emphysema. Report of the Medical Research Council Working Party. Lancet 1981; 1: and- Educa5on/Medicare- Learning- Network- MLN/MLNProducts/downloads/ OxgnThrpy_DocCvg_FactSheet_ICN pdf in ILD/IPF No studies of con5nuous oxygen therapy Benefit is extrapolated from COPD studies (NOTT, MRC) Dyspnea improved when transiently breathing 28% FiO 2 compared to room air in 10 pa5ents with ILD Ambulatory oxygen improves walk distance & dyspnea Weak data from small retrospec5ve series IPF guideline recommenda5on Pa5ents with IPF and clinically significant res5ng hypoxemia should be treated with long- term oxygen therapy (strong recommenda5on, very low- quality evidence) IPF Clinical Prac5ce Guideline Bajwah et al, Thorax 2013;68:867; Swinburn et al, Am Rev Respir Dis 1991;143:913; Visca et al, ERJ 2011;38:987; Frank et al, ERJ 2012;40:269. Raghu et al, AJRCCM 2011;183:788. Two influenza vaccines Inac5vated influenza vaccine (IIV) IM Injec5on This is the typical influenza vaccine recommended annually for all individuals 6 months of age Newer intranasal live apenuated influenza vaccine (LAIV) Causes a very low- grade infec5on in virtually all pa5ents More serious infec5ons possible in severe immunocompromised pa5ents (e.g. bone marrow transplant) Should not be given to pa5ents with chronic lung disease Influenza vaccines are typically trivalent (include 3 strains likely to cause outbreaks in a given year), although newer quadrivalent strains are replacing these Two pneumococcal vaccines Polysaccharide vaccine (Pneumovax; 23 subtypes) Recommended by the US Advisory Commipee on Immuniza5on Prac5ces (ACIP) for all adults with chronic lung disease Provides protec5on from approximately 2/3 of recent pneumococcal infec5ons Conjugate vaccine (Prevnar 13) Recommended in addi,on to Pneumovax (although not simultaneously) in immunocompromised individuals, including chronic cor5costeroid use Carrier protein may increase immunogenicity & mucosal immunity à decreases carriers à herd immunity 4

5 Recommenda5ons for pneumococcal vaccina5on Vaccinate at diagnosis of ILD and again at age 65 if at least 5 years have elapsed since the first vaccina5on Some advocate re- vaccina5on every 5 years in pa5ents with chronic respiratory disease Support groups & educa5on The majority of IPF pa5ents lack knowledge about their condi5on ILD/IPF support groups exist at many ILD centers Individual educa5onal components vary among centers and there is no evidence to guide the design of support groups There are mul5ple educa5onal resources available These are o`en unstudied, unvalidated, and contain incorrect or outdated informa5on Collard HR et al. Respir Med 2007;101:1350. Conclusions There are likely clinically significant benefits to pulmonary rehabilita5on, oxygen therapy, and vaccina5ons This is based on weak evidence, but obtaining addi5onal data may not be feasible given the widespread acceptance of these therapies Selected pa5ents with a severely impaired func5onal capacity can s5ll benefit from rehabilita5on Conclusions Support groups and educa5onal programs are likely beneficial Studies are required to determine the op5mal design and components of these programs 5