PFF Teal = MAIN COLORS PFF Green = Light Green = Red = HIGHLIGHT COLORS Light Grey = Dark Grey = Black = PULMONARY FIBROSIS: WHAT AND WHY SESSION LEADERS: JOAO ALBERTO M. DE ANDRADE, MD JOYCE LEE, MD

PFF Teal = MAIN COLORS PFF Green = Light Green = Red = HIGHLIGHT COLORS Light Grey = Dark Grey = Black = WHAT IS PULMONARY FIBROSIS AND WHY IS IT A PROBLEM? JOYCE S. LEE, MD PULMONARY FIBROSIS: WHAT AND WHY NOVEMBER 13, 2015

WHAT IS PULMONARY FIBROSIS?

PULMONARY FIBROSIS = INTERSTITIAL LUNG DISEASE = DIFFUSE PARENCHYMAL LUNG DISEASE

PULMONARY FIBROSIS = INTERSTITIAL LUNG DISEASE = DIFFUSE PARENCHYMAL LUNG DISEASE

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

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

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

Lung anatomy 101

Take out waste - Alveolar unit

Normal conditions:

Normal conditions:

Normal conditions:

In the setting of fibrosis:

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

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

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

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

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

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

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

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.

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

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

With pulmonary fibrosis

WHY IS PULMONARYFIBROSIS A PROBLEM?

FEEL SURVIVESURVIVE FUNCTION

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

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

FEEL SURVIVESURVIVE FUNCTION Exercise Hobbies & recreation Work Memory Social interaction

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

FEEL SURVIVESURVIVE FUNCTION Prognosis Acute exacerbation Lung transplantation

Pulmonary fibrosis affects how you survive Bjoraker et al AJRCCM 1998

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

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

Thank you.