1. LUNG TRANSPLANTATION and PULMONARY FIBROSIS Maria L. Padilla, MD Associate Prof. of Medicine Director of ILD/IPF and Advanced Lung Disease Program MSSM/MSMC

2. Organ Transplantation

3. Lung Transplantation and IPF Important Questions: –What is it? –Why? –For Whom? –When? –How? –What are the results?

4. Lung Transplantation and IPF What is Lung Transplantation? The operation that replaces diseased, failing lungs with a functional organ.

5. Lung Transplantation and IPF Thoracic Transplantation –Heart-Lung Transplantation –Single Lung Transplantation –Bilateral Lung Transplantation –Lobar Transplantation

6. Lung Transplantation and IPF WHY? It offers the opportunity to return to a better functional capacity when all medical therapies have been ineffective. Ultimate form of treatment

7. Lung Transplantation Process: –Referral –Evaluation –Listing with UNOS –Waiting time –Transplantation –Post transplantation care

8. Lung Transplantation and IPF For whom? –Candidate Selection Age less than 65y Absence of other organ dysfunction Non-smoker, non-drinker, no drug abuse Appropriate weight Good support system Stable psychosocial and emotional status

9. Lung Transplantation CANDIDATE EVALUATION –Blood type, biochemical panel, serologies –Renal function determination –Skin testing and sputum cultures –PFT’s, ABG’s, 6-min walk, +/-exercise tests –Imaging: CXR, HRCT, V/Q, bone densitometry –Cardiac tests: echo; stress tests; RHC with hemodynamics and LHC where indicated

10. Lung Transplantation and IPF When? When patient is: medically physically psychologically/emotionally, READY! and lungs become available

11. Lung Transplantation LESS THAN 20% OF DONORS ARE SUITABLE LUNG DONORS: –AGE AND SMOKING HX EXCLUDE SOME –LUNGS ARE FRAGILE--EDEMA, INFXN, VENTILATOR COMPLICATIONS –ALLOGRAFT INTOLERANCE TO PROLONGED ISCHEMIA

12. Lung Transplantation and IPF When lungs become available, they are offered on the basis of: –Time on the waiting list No consideration for severity of illness or urgency –Exception: 90 d credit when IPF patient listed –Blood type –Lung size –Other factors (?)

13. Lung Transplantation and IPF While on Waiting List: –Adhere to medical treatment –Participate in pulmonary rehabilitation –Maintain good nutrition and acceptable body weight –Attend support groups –Keep a positive attitude and visualize a brighter tomorrow

14. Lung Transplantation Registry: –United Network for Organ Sharing and the Organ Procurement and Transplantation Network (UNOS/OPTN) –1988-2000 –7764 lung transplants (7625 C, 139 LD) –719 heart-lung transplants Data as of Nov. 2000

15. Lung Transplantation

16. Lung Transplantation and ILD Activity (SLT and BLT)- 1988-1999 –IPF (LD 5)972 –SARCOIDOSIS148 –PF (OTHER) 82 –LAM 47 –OB (non-retransplant) 46 –OCCUP. LUNG DIS. 11 –RHEUMATOID DIS. 5 From UNOS/OPTN data as of 2/17/2001

17. Lung Transplantation and IPF Idiopathic Pulmonary Fibrosis –Waiting time too long for some patients –At risk for developing 2* PHN –Prior Thoracic surgeries (OLB, Ptx) –High frequency of osteoporosis, obesity –Documented survival benefit –H-L, 2.9%; BLT, 7.1%; SL, 19.5% (ISHLT)

18. Lung Transplantation-IPF

20. Lung Transplantation Issues and complicating factors –Need for chronic immunosuppression –Acute and chronic rejection –Infection –Side effects of medicines –Cost of procedure and follow up care

21. Lung Transplantation and ILD IMPROVED FUNCTIONAL STATUS IMPROVED PHYSIOLOGY(pulm+ cv) SURVIVAL BENEFIT (IPF) COMPARABLE INCIDENCE OF AR, CR INFECTION IMPROVED QUALITY OF LIFE

22. Lung Transplantation

25. Lung Transplantation and IPF/ILD LT is a therapeutic modality of great value Efforts to overcome LT limitations needed: –Increase donor pool--review criteria Living donors---Lobar transplantation –Prevent CR-improve treatment Earlier listing of candidates with ILD/IPF Explore new therapies as bridge to LT –New IPF paradigm