LUNG TRANSPLANTATION and PULMONARY FIBROSIS Maria L. Padilla, MD Associate Prof. of Medicine Director of ILD/IPF and Advanced Lung Disease Program MSSM/MSMC Organ Transplantation Lung Transplantation and IPF • Important Questions: – What is it? – Why? – For Whom? – When? – How? – What are the results? Lung Transplantation and IPF • What is Lung Transplantation? The operation that replaces diseased, failing lungs with a functional organ. Lung Transplantation and IPF • Thoracic Transplantation – Heart-Lung Transplantation – Single Lung Transplantation – Bilateral Lung Transplantation – Lobar Transplantation 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 Lung Transplantation • Process: – Referral – Evaluation – Listing with UNOS – Waiting time – Transplantation – Post transplantation care 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 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 Lung Transplantation and IPF • When? When patient is: medically physically psychologically/emotionally, READY! and lungs become available 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 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 (?) 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 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 Lung Transplantation Lung Transplantation and ILD • Activity (SLT and BLT)- 1988-1999 – IPF (LD 5) – SARCOIDOSIS – PF (OTHER) – LAM – OB (non-retransplant) – OCCUP. LUNG DIS. – RHEUMATOID DIS. 972 148 82 47 46 11 5 • From UNOS/OPTN data as of 2/17/2001 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) Lung Transplantation-IPF Lung Transplantation-IPF 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 Lung Transplantation and ILD • • • • IMPROVED FUNCTIONAL STATUS IMPROVED PHYSIOLOGY(pulm+ cv) SURVIVAL BENEFIT (IPF) COMPARABLE INCIDENCE OF AR, CR INFECTION • IMPROVED QUALITY OF LIFE Lung Transplantation 100% 80% 60% 40% 20% No Activity Limitations Performs with Assistance Total Assistance 0% 1 Year 3 Years 5 Years Lung Transplantation Lung Transplantation 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