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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?
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LUNG TRANSPLANTATION and PULMONARY FIBROSIS Maria L. Padilla, MD Associate Prof. of Medicine Director of ILD/IPF and Advanced Lung Disease Program MSSM/MSMC
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 and ILD • Activity (SLT and BLT)- 1988-1999 • IPF (LD 5) 972 • SARCOIDOSIS 148 • 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
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 • 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 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