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Ancillary Management Strategies for Patients with IPF

Ancillary Management Strategies for Patients with IPF. Educational Activity Learning Objective. Engage in effective patient communication and collaborative care practices to improve patient understanding and use of ancillary therapies in IPF care. Exercise and Pulmonary Rehabilitation.

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Ancillary Management Strategies for Patients with IPF

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  1. Ancillary Management Strategies for Patients with IPF

  2. Educational Activity Learning Objective • Engage in effective patient communication and collaborative care practices to improve patient understanding and use of ancillary therapies in IPF care

  3. Exercise and Pulmonary Rehabilitation • Formal pulmonary rehabilitation programs are preferred for initiation of exercise • Cardiovascular disease screening should be performed before initiation • Blood pressure and O2 saturation should be monitored; oxygen should be titrated to attenuate desaturation • Reimbursement emerging issue for IPF • Benefits • Improved dyspnea • Improved endurance (6MWT) • Social interactions • Adherence reinforced Ferreira A, et al. Chest. 2009;135:442-447. Nishiyama O, et al. Respirology. 2008;13:394-399.

  4. IPF: A Cycle Perpetuating Inactivity Swigris JJ, et al. Respir Med. 2008;102:1675-1680.

  5. Pulmonary Rehabilitation Objectives • Decrease respiratory symptoms and complications • Permit a return to work or leisure activities • Increase control over daily functioning • Improve physical conditioning and exercise performance • Improve emotional well-being Pulmonary Rehabilitation. http://www.pilotforipf.org/resources/PtCounseling_07.pdf. Accessed August 2010.

  6. Diet • Goal is proper weight, BMI 18.5–25 • Not too low (danger of malnutrition) • Not too high (danger of metabolic syndrome) • Corticosteroid therapy introduces challenge of weight gain

  7. Survival and BMI Survival (%) • Retrospective study of 197 patients with IPF, 70% men • Survival associated with BMI (HR, 0.93 for each unit increase in BMI, P = 0.002) • Cannot exclude possibility of lead time bias (influence of BMI on diagnosis) • Replicates result of COPD study (Schols 1998) BMI < 25 25–30 30+ Time (Years) Alakhras M, et al. Chest. 2007;131:1448-1453. Schols AM, et al. Am J Respir Crit Care Med. 1998;157(6 Pt 1):1791-1797.

  8. Acute Respiratory Failure in the ICU:Ventilate? • Meta-analysis included 135 ICU patients with IPF • Mechanical ventilation subsequent to Acute Respiratory Failure (ARF) • Mean duration of ventilation: 8.6 days • 3-month mortality rate: 94% • Recommendations • Do not ventilate patient with AE of IPF • Ventilation may be appropriate for other ILD or reversible conditions associated with IPF Mallick S. Respir Med. 2008;102:1355-1359.

  9. Non-Invasive Mechanical Ventilation • Small retrospective study comparing MV to NIV • NIV associated with • Improvement in PaO2 /FiO2 vs spontaneous breathing (134 ± 36 vs. 89 ± 26; P = 0.0004) • No significant effects on PaCO2 and pH values • Significant decrease in RR (26 ± 7 vs. 36 ± 9 breaths/min; P = 0.002) in all individuals • NIV failed to improve PaO2 /FiO2 in 9 individuals • 5 underwent iMV and died after 8.8 ± 5.8 days • 4 died before undergoing ETI • NIV should be considered as alternative to MV Mollica C, et al. Respiration. 2010;79:209-215.

  10. no no yes yes Managing ARF in the ICU Patient in ICU with ARF Does patient have IPF? • Other condition? • Pneumothorax • Infection • Heart failure • PE • Recent surgery Address other condition; ventilation may be appropriate Address if reversible; ventilation may be appropriate If AE of IPF, ventilation is futile Mallick S. Respir Med. 2008;102:1355-1359.

  11. End-of-Life Issues • Advance directives • Will • Living will • Durable power of attorney • Share decisions with significant others in advance End-of-Life Issues. http://www.pilotforipf.org/resources/PtCounseling_03.pdf. Accessed August 2010.

  12. Depression and Nonadherence • Depressed patients are 3 times more likely to be nonadherent with medical treatment • Screen for depression • During the past month: • Have you often been bothered by feeling down, depressed, or hopeless? • Have you often been bothered by having little interest or pleasure in doing things? DiMatteo MR, et al. Arch Intern Med. 2000;160:2101-2107.Ebell MH. Am Fam Physician. 2004;69:2421-2422.

  13. HRQOL Measures for IPF • Do HRQOL measures reflect disease progression? • Retrospective analysis of BUILD-1 data • Baseline and 6 month assessments Swigris JJ, et al. Respir Med. 2010;104:296-304.

  14. SF-36 Correlates with Change in FVC Patients (%) 13 20 53 8 6 Swigris JJ, et al. Respir Med. 2010;104:296-304.

  15. SF-36 of Placebo-Treated BUILD-1 Patients Raghu G, et al. Eur Respir J. 2010;35:118-123.

  16. SF-36 Improvement With Bosentan 0.0 Raghu G, et al. Eur Respir J. 2010;35:118-123.

  17. STEP-IPF Sildenafil Trial • Study Design • IPF patients with moderate pulmonary impairment • 180 patients randomized 1:1:sildenafil:placebo • Duration • 12 weeks RCT • 12 weeks open-label, all receive sildenafil • Endpoints • 1oImprovement of ≥ 20% in 6MWD • 2oChange in 6MWD, dyspnea, and QOL IPF Clinical Research Network. N Engl J Med. 2010;363:620-628.

  18. STEP-IPF Results • No significant change in 6MWD at 12 or 24 weeks • No difference in mortality or acute exacerbations after 12 or 24 weeks • QOL • Improvement with treatment on St. George’s Respiratory Questionnaire (P = 0.01) • No improvement on SF-36 or EQ-5D tests • Dyspnea • Improvement with treatment on SOB Questionnaire (P = 0.006) • No improvement on Borg Dyspnea Index after walk test • Gas exchange at 12 weeks • Improvement in DLCO (P = 0.04) • Improvement in arterial oxygen saturation (P = 0.05) IPF Clinical Research Network. N Engl J Med. 2010;363:620-628.

  19. STEP-IPF Conclusions • Data are insufficient to conclude that sildenafil is effective for patients with advanced IPF • Expert opinion: sildenafil might be considered for managing dyspnea if • DLCO < 35% and • No contraindication (eg, aortic stenosis) • Monitor dyspnea in 12 week test, D/C sildenafil if no improvement IPF Clinical Research Network. N Engl J Med. 2010;363:620-628.

  20. HRQOL Conclusions • HRQOL is important for patients • IPF negatively impacts all domains • Therapy may positively impact some domains (STEP, BUILD-1) • SF-36, SF-12, or SGRQ may be useful • IPF-specific HRQOL tools in development

  21. Sleep • Caregiver should observe patient • Oxygen desaturation common with ILD, independent of obstructive sleep apnea (OSA) • Frequent awakenings • Arrhythmia • MI more common during sleep • GERD: don’t eat just before sleep

  22. Is OSA Common in IPF? • 55 subjects with IPF • Sleep apnea evaluation • Epworth Sleepiness Scale (ESS) • Sleep Apnea Scale of Sleep Disorders (SA-SDQ) • Nocturnal polysomnography (NPSG) • Other measures • Spirometry (FEV1, FVC) • Total lung capacity • DLCO • BMI Lancaster LH, et al. Chest. 2009;136:772-778.

  23. OSA Is Common in IPF No OSA AHI  5/h 12% • Did not correlate with OSA • Spirometry • Lung volume • DLCO • ESS • Did correlate with OSA • SA-SDQ: r = 0.45, P = 0.01 • BMI: r = 0.30, P = 0.05 • Findings consistent with Mermigkis et al • Newly diagnosed IPF (n = 34) • REM AHI correlated with TLC (P = 0.03, r = -0.38) 20% 68% Mild AHI 5–15/h Moderate/Severe AHI > 15 events/h Lancaster LH, et al. Chest. 2009;136:772-778.Mermigkis C, et al. Sleep Breath. 2010 Mar 16. [Epub ahead of print] AHI: apnea-hypopnea index

  24. GERD • Acid GERD prevalent in IPF (87%) • 47% experience classic GERD symptoms • GERD and IPF severities not correlated Raghu G, et al. Eur Respir J. 2006;27:136-142.

  25. 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

  26. Role of Family/Caregiver • Caregiver is a critical partner • Caregiver needs information • Disease course • Special needs of patient • Ways to access other resources (eg, Web sites, local support groups, etc) • Engage in disease management • Attend clinic with patient • Encourage exercise • Monitor symptoms and medications • Help organize medical care • Taking care of the caregiver • Emotional stress • Time management • Independent needs • Advocate for patient

  27. Air Travel with Oxygen • Security • Oxygen and arrangements • Airline checklist • Oxygen supplier checklist • Altitude simulation testing (when available) Adapted from TSA Web site. http://www.tsa.dhs.gov/travelers/airtravel/specialneeds/editorial_1374.shtm#2 Accessed August 2010.

  28. VTE Increases Risk for IIP P interaction Multivariate Adjusted HR for IIP (95% CI) Sode BF, et al. Am J Respir Crit Care Med. 2010;181:1085-1092.

  29. Vascular Disease and IPF • Correlation of IPF and CVD • UK Health Improvement Network database • 920 IPF cases, 3593 controls • Conclusion: People with IPF have an increased risk of vascular disease OR = odds ratio, RR = rate ratio. Hubbard RB, et al. Am J Respir Crit Care Med. 2008;178:1257-1261.

  30. Emphysema Decreases Survival in IPF Survival Months Significant factors for mortality Mejía M, et al. Chest. 2009;136:10-15.

  31. Smoking • Increased risk of respiratory infection • Increased risk for IPF • IPF is risk factor for lung cancer • Management • Counseling • Nicotine replacement • Medication (nicotine, bupropion, varenicline) • Proactive phone calls • Social support GOLD Guidelines 2008. http://www.goldcopd.com/Guidelineitem.asp?l1=2&l2=1&intId=2003. Accessed August 2010. Jorenby DE. Circulation. 2001;104;E51-E52.

  32. Lung Transplantation • Lung Transplantation in IPF • Questions to ask • Are you a candidate? • The procedure • Postsurgical care and rehabilitation • Benefits and risks • Preparation for Transplant • What is a lung transplant? • How does the system work? • What can I expect after my transplant? • How can I prepare for a transplant? • Where do I begin? Lung Transplantation. http://www.pilotforipf.org/resources/PILOTPamphlet3.pdf. http://www.pilotforipf.org/resources/PtCounseling_04.pdf Accessed August 2010.

  33. Clinical Trial Participation • Offer high standard of care • Add to patient empowerment • Burdens • May require travel • Frequent visits to research center • Selected open Phase 3 trials • ARTEMIS-IPF and ARTEMIS-PH (ambrisentan) • PANTHER (prednisone, azathioprine, N-acetylcysteine (NAC)) • Pulmonary hypertension secondary to IPF (sildenafil) • Pulmonary hypertension secondary to IPF (bosentan, phase 4) Clinicaltrials.gov. http://www.clinicaltrials.gov/ct2/results?term=ipf. Accessed August 2010.

  34. Patient Tool:Clinical Trial Participation • What is a clinical trial? • Questions to ask the physician • First steps in participation • Benefits and risks Clinical trials as a treatment option for IPF. http://www.pilotforipf.org/resources/PILOTPamphlet1.pdf. Accessed August 2010.

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