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 DIFFICULT CONVERSATIONS: DISCUSSING PROGNOSIS & GOALS OF CARE SONYE K. DANOFF, MD, PhD PULMONARY FIBROSIS: WHAT AND WHY NOVEMBER 13, 2015

2. No conflict of interest relevant to this presentation

3. Objective of this talk To consider how best to address prognosis and goals of care for yourself or your loved one with PF con·ver·sa·tion noun \ ˌ kän-vər- ˈ sā-shən\ The spoken exchange of thoughts, opinions, and feelings; talk.

4. Prognosis The likely course of a disease or ailment -Oxford English Dictionary Prognosis = Quantity + Quality of Life

5. What do we mean by PF (ILD)? Group of lung disorders, not a single disease Rare: affects about 500,000 in US Likelihood of having PF is similar worldwide Idiopathic Pulmonary Fibrosis (IPF) is one form of PF There are many non-IPF forms of ILD (which often have different treatments) –Autoimmune disease, medication, environmental Average age at diagnosis –IPF: 60 yr old –PF with autoimmune disease: 50 yr old

6. Non-IPF Interstitial Lung Disease Likely equally or more common than IPF Generally, better prognosis More responsive to therapy –Medication –Environmental modification Very heterogeneous patient population Frequently younger population

7. How is prognosis determined? What is the underlying disease? –IPF vs not IPF –Pattern on lung biopsy or chest CT scan Other features of the lung disease –Pulmonary Function Tests –Need for oxygen Other characteristics of the person –Age 7

8. Predictors of Mortality Age Gender Ethnicity Smoking status Dyspnea Clubbing Co-morbidity –emphysema –PH, CAD CT consistent with UIP Lung Bx of UIP Decline in PFTs or 6MWD # of fibroblastic foci Biomarkers –KL-6 –SP-A, SP-D –CCL-18 –fibrocytes Ley et al. AJRCCM 183; 431-440

9. Models for Predicting Mortality GAP –Gender –Age –PFTs CPI –PFTs 9 Age Likelihood of Dying

10. Median Survival for IPF AgeMedian Survival ( years) <656.8 65-694.5 70-743.2 75-792.6 80-842.0 >851.6 Navaratnam, et al. Thorax, April 2011

11. Death and Dying

12. Goals of Care What do you want for yourself over the course of your illness? –Length of life –Quality of life –Independence –Comfort –Control 12

13. Goals of Care is Not a Recipe or a Prescription It is a conversation –With your doctor –With your family –With your support system –With yourself Allows you, your loved ones and your doctor to advocate for what matters most to you 13

14. What Stands in the Way of Goals of Care Conversations “I don’t want to worry my wife/husband.” “I am not sure what I want.” “I don’t want my patient to feel that I have given up.” “It is too depressing to think about.” 14

15. Think about it this way… 15 In this world nothing can be said to be certain, except death and taxes. -Benjamin Franklin There isn’t much chance you will have a say in taxes, but this is the opportunity to have a say in the terms of your life and death. Goals of care don’t prevent death, but they put you in control of deciding what matters to you.

16. Palliation The commitment to allow each person to live out his/her life in comfort and dignity Oxygen Mobility (Wheelchair, Scooter, Handicap Tags) Control of dyspnea and cough Support for patient and care providers Advanced directives/goals of care

17. Hospice Less than 6 months to live Mobilize increased resources Focus on comfort Controlling symptoms Caregiver respite Addressing issues of grief/loss

18. To the Lisa Sandler Spaeth and Cecilia Fisher-Rudman Funds for Pulmonary Fibrosis at Johns Hopkins & To our patients who keep us focused on what really matters With Gratitude