An International Case Study of Lung Transplantation

1. An International Case Study of Lung Transplantation Selim M. Arcasoy, M.D. Professor of Medicine Medical Director, Lung Transplantation Program Director, Interstitial Lung Disease Program New York-Presbyterian Hospital Columbia University Medical Center

2. The Impact of Lung Disease • Chronic, progressive and disabling symptoms • Breathlessness, cough, sputum production • Decline in functional status and quality of life • Greater than 35 million Americans have lung disease • Financial cost to society (>$150 billion per year) • Decreased work days and productivity, disability, costs of medical care • Premature mortality • Number 3 killer in the U.S. behind heart disease & cancer • 400,000 Americans die of lung disease every year • Responsible for one in six deaths

3. Four Major Lung Diseases Treatable with Lung Transplantation • Chronic obstructive pulmonary disease (COPD) • Emphysema/chronic bronchitis due to tobacco smoking or alpha1-antitrypsin deficiency (genetic) • 12 million have COPD, 12 million are under-diagnosed • Pulmonary fibrosis (scarring of the lungs) • End result of more than 200 diseases • Affects >500,000 Americans • Cystic fibrosis (CF): A genetic disease • ~30,000 Americans have CF with 1,000 new cases/yr • Pulmonary hypertension

4. Cystic FibrosisPatient 1 • 9-year old girl • Diagnosed with CF at age 4 months • Recurrent respiratory infections and low body weight • Developed pulmonary MRSA infection 2 years prior • Nine hospitalizations for intravenous antibiotics • Lung function at 30% of predicted for her age and needs to use 3 LPM of supplemental oxygen • Family makes contact with our Lung Txp Program • Required tests ordered and performed in Turkey • Tests reviewed and family moves to U.S. for evaluation

5. Cystic FibrosisPatient 1 • First visit on April 6, 2007 • Further testing and consultations • Multidisciplinary team discussion • Placed on the active lung transplant list • Recurrent infections while awaiting transplant • Lung transplantation on November 25, 2009

7. Cystic FibrosisPatient 2. A Case of Good Fortune • Older sister of our first patient • Diagnosed at age 7 years • Much healthier than her younger sister • Attending school in the U.S. as her family moved for her sister’s transplant • Becomes sicker in 2010 • Lung function drops from 51% to 22% • Hospitalized with fever and respiratory failure • Urgent lung transplant evaluation and listing • Lung transplantation performed on Sep 17, 2010

9. End-stage Lung

11. And, here they are….

12. Stages of Respiratory Disease and Failure

13. June 11, 1963 JD Hardy, MD History of Lung Transplantation • 36 patients underwent lung transplantation between 1963 and 1975 with no long-term survivors • Introduction of cyclosporine in 1978 • First successful heart-lung Tx performed in 1981, single lung Tx in 1983 and double lung Tx in 1986 • 1954 - First successful kidney transplant • 1967 - First successful liver transplant • 1968 - First successful heart transplant

14. Number of Lung Transplants in the Last 3 Decades 2013 JHLT. 2013 Oct; 32(10): 965-978

15. YES Advanced lung disease with limited lifespan and severe functional limitation Lack of alternative therapies Demonstrated compliance with therapy and follow-up Strong family support NO Active or recent cancer Advanced dysfunction of a major organ system Untreatable infection Substance addiction Documented noncompliance and lack of family support Who is a Candidate for Lung Transplant?General Guidelines A complex decision that requires extensive evaluation and multidisciplinary team discussion

16. Lung Transplantation on a ScaleRisk-Benefit Balance Benefits Survival Function Quality of Life Risks Mortality Morbidity Complexity Patient Expectations Patient characteristics Type of lung disease Other medical illnesses Prognosis without transplant

17. Steps From Initial Evaluation to Transplantation • First visit to meet the transplant team • Focus on initial patient screening, assessment of patient/family motivation, and education • Extensive testing and consultations • Multidisciplinary team meeting for a decision • Potential outcomes: • Immediate active listing • Accepted as a potential future candidate, but needs to meet certain goals or get sicker • Denial because of contraindications

19. Transplant Listing and The Call • Patients are placed on the active national list • All listed patients are assigned a Lung Allocation Score (LAS) based on their clinical characteristics • Age, disease, lung, heart and kidney function, labs • Score ranging from 0 (less ill) to 100 (gravely ill) • Waiting period with significant regional variation • Donor and recipient matched by blood type and size • Transplant has to occur within 6 to 8 hours after removal of lungs from the donor

21. Ex Vivo Lung Perfusion“Lung In A Box”

23. Extracorporeal Membrane Oxygenation (ECMO)

24. Case 3

25. Before And After

28. NewYork-Presbyterian Hospital Lung Transplant Volume Per Year 1996-2012 New Program

29. NewYork-Presbyterian HospitalLung Transplant Survival7/01/2001 - 12/31/2012 (n = 559) Survival NYPH vs UNOS1-year: 91% vs 84% 5-year: 68% vs 53% 10-year: 47% vs 28%

30. Functional Status Before and After Lung Transplantation

31. Employment Status Before and After Lung Transplantation