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 PULMONARY FIBROSIS: WHAT AND WHY SESSION LEADERS: JOAO ALBERTO M. DE ANDRADE, MD JOYCE LEE, MD

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 WHAT IS PULMONARY FIBROSIS AND WHY IS IT A PROBLEM? JOYCE S. LEE, MD PULMONARY FIBROSIS: WHAT AND WHY NOVEMBER 13, 2015

4. WHAT IS PULMONARY FIBROSIS?

5. PULMONARY FIBROSIS = INTERSTITIAL LUNG DISEASE = DIFFUSE PARENCHYMAL LUNG DISEASE

6. PULMONARY FIBROSIS = INTERSTITIAL LUNG DISEASE = DIFFUSE PARENCHYMAL LUNG DISEASE

7. PULMONARY FIBROSIS Known Cause Unknown Cause GranulomatousMiscellaneous AutoimmuneIPF EnvironmentalNSIP Drug Sarcoid LAM LCH CEP

8. PULMONARY FIBROSIS UNIFYING FEATURE: ( VARYING AMOUNTS OF ) INFLAMMATION AND FIBROSIS OF THE LUNG

9. Terminology PULMONARY = related to the lung FIBROSIS = thickening and scarring PULMONARY FIBROSIS = thickening and scarring of the lung

10. Lung anatomy 101

11. Take out waste - Alveolar unit

12. http://www.le.ac.uk/pathology/teach/va/anatomy/case2/lunganim.gif Normal conditions:

13. http://www.le.ac.uk/pathology/teach/va/anatomy/case2/lunganim.gif Normal conditions:

14. http://www.le.ac.uk/pathology/teach/va/anatomy/case2/lunganim.gif Normal conditions:

15. In the setting of fibrosis:

16. Consequences Normal: Fibrotic: Oxygen can’t move from the air to the blood. Air Blood O2O2 O2O2 Courtesy of Hal Collard

17. Consequences The lungs can’t breath deeply and bring in oxygen. Normal: Fibrotic: O 2 O2O2 O 2 O2OO2O 2 O2O2 O2O2 Lungs expand well and lots of oxygen enters Lungs expand poorly and less oxygen enters Courtesy of Hal Collard

18. How can my doctor tell if I have pulmonary fibrosis? Signs & Symptoms Lung function ImagingLung biopsy

19. Symptoms and signs Asymptomatic Chronic cough Cold that won’t go away Shortness of breath Fatigue Decreased exercise tolerance Low oxygen saturation at rest or with activity Crackles on exam Clubbing of the fingers

20. How can my doctor tell if I have pulmonary fibrosis? Signs & Symptoms Lung function ImagingLung biopsy

21. Lung function Decreased forced vital capacity (FVC) Decreased diffusing capacity for carbon monoxide (DLCO) Restrictive lung process Trends in FVC and DLCO can tell us how your lungs are doing

22. How can my doctor tell if I have pulmonary fibrosis? Signs & Symptoms Lung function ImagingLung biopsy

23. Imaging: High-resolution CT  Thin slices of your chest and lungs  Air is dark, tissue is grey/white  Allows your MD to determine the cause of pulmonary fibrosis in ~50% of cases.

25. How can my doctor tell if I have pulmonary fibrosis? Signs & Symptoms Lung function ImagingLung biopsy

26. Normal lung tissue Alveolar spaces (air) Interstitium (tissue)

27. With pulmonary fibrosis

28. WHY IS PULMONARYFIBROSIS A PROBLEM?

29. FEEL SURVIVESURVIVE FUNCTION

30. FEEL SURVIVESURVIVE FUNCTION Cough Shortness of breath Fatigue Depression Anxiety Quality of life

31. Pulmonary fibrosis affects how you feel CoughCough "a nagging desire to cough constantly" “never feeling relieved after coughing" DyspneaDyspnea “even brushing my teeth an exertion” breathless “carrying groceries… carrying anything” ExhaustionExhaustion “overwhelming fatigue” “consistent lack of energy” “completely wiped out” Swigris et al Health Qual Life Outcomes 2005

32. FEEL SURVIVESURVIVE FUNCTION Exercise Hobbies & recreation Work Memory Social interaction

33. Pulmonary fibrosis affects how you function ForethoughtForethought need to “analyze every activity” “How far away is the parking lot from the door?" Social Participation fear of “catching something” problems “keeping up with certain relationships” Dependence “The least satisfying aspect of my life is not being as independent” Swigris et al Health Qual Life Outcomes 2005

34. FEEL SURVIVESURVIVE FUNCTION Prognosis Acute exacerbation Lung transplantation

35. Pulmonary fibrosis affects how you survive Bjoraker et al AJRCCM 1998

37. Pulmonary fibrosis is a problem Affects how you feel, function and survive Affects 1 out of 200 adults over the age of 65 in the US Approximately 50,000 new cases each year This number is likely to increase over time 40,000 Americans die from IPF each year

38. But, there is reason to hope In 2014, two treatments were approved for IPF International interest in finding better treatments for this group of conditions Increasing resources from foundations, support groups, and universities Education (patients, providers) Counseling (genetic, end of life care) Support network

39. Thank you.