1. PFF Teal = 0+160+175 MAIN COLORS PFF Green = 120+162+47 Light Green = 193+216+47 Red = 242+102+73 HIGHLIGHT COLORS Light Grey = 220+220+210 Dark Grey = 100+98+76 Black = 0+0+0 TOWARDS AN IMPROVED QUALITY OF LIFE: STARRING OXYGEN THERAPY AND PULMONARY REHAB ANNE E. HOLLAND, PHD CLINICAL CARE: COMPREHENSIVE CARE – BEYOND PHARMACOLOGICAL THERAPIES NOVEMBER 14, 2015

2. PFF Teal = 0+160+175 MAIN COLORS PFF Green = 120+162+47 Light Green = 193+216+47 Red = 242+102+73 HIGHLIGHT COLORS Light Grey = 220+220+210 Dark Grey = 100+98+76 Black = 0+0+0 DISCLOSURES No relationships to disclose ©2015 Pulmonary Fibrosis Foundation. All rights reserved.2

3. Health-related quality of life (HRQoL) is impaired, across multiple domains De Vries ERJ 2001

4. Dowman et al 2015 Lower FVC associated with worse health-related quality of life r=-0.35, p<0.001 Health-related quality of life is impaired in pulmonary fibrosis

5. Dowman et al 2015 More dyspnoea associated with worse health-related quality of life r =0.75, p<0.001 Health-related quality of life is impaired in pulmonary fibrosis

6. Impact of PF on HRQoL – qualitative studies Swigris 2005, Collard 2007, Schoenheit 2011, Holland 2015, Duck 2015 DyspnoeaCough Poor sleep Fatigue ILD

7. Swigris 2005, Collard 2007, Schoenheit 2011, Holland 2015, Duck 2015 DyspnoeaCough Poor sleep Fatigue Dependence Impact on relationships Negative experience of therapies Protracted diagnostic pathway Lack of information Financial burden ILD Impact of PF on HRQoL – qualitative studies

8. Unmet needs in PF –patient perspectives Collard 2007, Schoenheit 2011, Holland 2015, Duck 2015 Honesty about prognosis and optionsAccess to centres of excellenceFormal support structuresDecreased barriers to oxygen provisionPublic advocacy

9. No statistically or clinically significant difference in HRQoL at 52 weeks Richeldi NEJM 2014 Does treatment improve quality of life?

10. Less people with worsening dyspnoea in pirfenidone group, but not significant King NEJM 2014 Does treatment improve quality of life?

11. Pulmonary rehabilitation “A comprehensive intervention based on a thorough patient assessment followed by patient-tailored therapies that include, but are not limited to, exercise training, education, and behaviour change…” “…designed to improve the physical and psychological condition of people with chronic respiratory disease and to promote the long-term adherence to health-enhancing behaviours” Spruit et al AJRCCM 2013

12. Evidence for rehabilitation in PF Moderate effect size Significant gains in quality of life Data from Dowman et al Cochrane 2014

13. Evidence for rehabilitation in IPF Data from Dowman et al Cochrane 2014 Moderate effect size Significant gains in quality of life

14. Benefits of rehabilitation on HRQoL are not sustained at 6 months Holland et al Thorax 2008

15. Components of rehab for PF Holland et al Respiration 2015 Standard pulmonary rehab format appears effective

16. Non-exercise components of pulmonary rehabilitation for PF Patients –Tell me about the future (honestly) – what to expect? –Topics specific to PF as well as general topics cough, medications and side effects, limiting disease progression, end of life planning –Happy to be in groups with non-PF patients Clinicians –Content tailored to PF –No consensus on whether prognosis and end of life planning should be discussed Holland et al CRD 2015

17. ATS guidelines for IPF: Recommends long term oxygen therapy if clinically significant resting hypoxaemia (SpO 2 <88%) Strong recommendation, very low quality evidence (no survival benefit shown) Uncertainty about the balance of benefits vs costs / inconvenience to patients However strong physiological rationale and concerns about withholding oxygen Oxygen therapy Raghu AJRCCM 2001

18. Oxygen therapy Short term improvements in physiology in randomised crossover trials OutcomeMean difference 95% CIFavoursAuthor Peak work17 Watts10 to 24OxygenHarris-Eze 1994 VO2 peak0.26 L.min -1 0.18 to 0.34OxygenHarris-Eze 1994 Endurance time6 minutes0.64 to 11.16OxygenBye 1982 6MWD13 meters-38 to 64OxygenNishiyama 2013

19. Oxygen therapy Inconsistent short-term effects on dyspnoea 6 crossover studies 5 during exercise, 1 at rest 3 with randomized testing order Rest: mean ↓ in dyspnea 18mm on 100mm VAS Swinburn 1991 Exercise: inconsistent effects across trials 2 favour oxygen, 3 no effect of oxygen Mean reduction in Borg 0.06 points (95% CI -0.24 to 0.13)

20. Little data specific to PF Bigger fall in HRQoL in those who developed the need for oxygen therapy at 12 months compared to those who did not: physical function, general health, vitality, social function, mental health highly confounded Tomioka 2007 Oxygen therapy and quality of life

21. Improved confidence Feel more in control Constant worry about running out of oxygen ‘… with the oxygen on at least I can get around, not as fast as normally, but I can get around....and I don’t seem to cough as much’ [Terry] ‘…I couldn’t sit here this long talking to you without oxygen’ [Frank]’ Patient experiences of oxygen therapy Duck J Adv Nursing 2015

22. Towards improved HRQoL in PF Reduced HRQoL is an important problem in PF Not just about symptoms – also fear of the future, dependence, relationships, treatment experiences, access to information, financial burden and trust in HCP Patients expect that treatment will improve their HRQoL No magic bullet that delivers for everyone Likely to require multi-faceted, individual treatment packages that include supportive care: pulmonary rehabilitation and oxygen therapy

23. PFF Teal = 0+160+175 MAIN COLORS PFF Green = 120+162+47 Light Green = 193+216+47 Red = 242+102+73 HIGHLIGHT COLORS Light Grey = 220+220+210 Dark Grey = 100+98+76 Black = 0+0+0 Thank you.