Speaker's Highlight

  • Don Sin University of British Columbia, St. Paul Hospital (Canada)
    Kenneth R. Chapman Toronto General Hospital Research Institute (Canada)
  • Parameswaran Nair McMaster University (Canada)
    Carolyn Calfee UCSF (U.S.A.)
  • Gregory P. Downey University of Colorado School of Medicine (U.S.A.)
    David A. Schwartz University of Colorado School of Medicine (U.S.A.)
  • Neil Schluger Tuberculosis Control Branch, California Department of Public Health (U.S.A.)
    Nick Kim Critical Care & Sleep Medicine, University of California San Diego (U.S.A.)
  • Nicola Hananiah Baylor College of Medicine (U.S.A.)
    Jae-Joon Yim Seoul National University College of Medicine (Republic of Korea)
  • Koichiro Asano Tokai University School of Medicine (Japan)
    Diahn-Warng Perng Taipei Veterans General Hospital (Taiwan)
  • Konstantinos Kostikas University of Ioannina (Greece)
    Karin Klooster University Medical Center Groningen (Kingdom of the Netherlands)
  • Lung transplantation for pulmonary fibrosis: candidate selection

    Idiopathic pulmonary fibrosis remains the most common indication for lung transplantation in Australia, accounting for roughly one in three procedures performed at the country's three adult units in Melbourne, Brisbane and Sydney. As antifibrotic therapy has matured and become subsidised through the Pharmaceutical Benefits Scheme, more patients now live longer with progressive disease, making the question of when to list them more pressing than ever. Data presented at the APSR 2022 congress emphasised that candidate selection is no longer a simple matter of physiological decline but a multidisciplinary judgement about resilience, social support and the capacity to survive a demanding postoperative course.

    For clinicians in regional Queensland, Western Australia or Tasmania, the pathway is shaped by geography as much as by disease severity. Patients from Cairns, Broome or Launceston must travel hundreds of kilometres to reach a transplant unit, and that reality alone shapes referral timing. Choosing the right recipient therefore means weighing disease trajectory against the practical capacity of the individual to undergo assessment, relocation and lifelong follow-up within a highly specialised national service.

    Understanding pulmonary fibrosis and its progression

    The phrase "pulmonary fibrosis" covers a spectrum of interstitial lung diseases, but idiopathic pulmonary fibrosis is the prototype driving transplant referrals. It typically presents in the seventh decade with progressive dyspnoea and dry cough, and median survival without transplant still hovers around three to five years from diagnosis. Other fibrosing conditions, including hypersensitivity pneumonitis, connective tissue disease-related ILD and progressive fibrosing sarcoidosis, follow similar trajectories once lung function falls below a critical threshold.

    Antifibrotic agents have changed the slope of that curve. Nintedanib and pirfenidone, both subsidised by the PBS for IPF since 2017, slow forced vital capacity decline by roughly half. They are not curative, however, and a meaningful proportion of patients still progress to a point where transplantation offers the best chance of meaningful survival. Recognising this transition, ideally before continuous home oxygen is needed at rest, is the foundation of sound candidate selection.

    Multidisciplinary evaluation of transplant candidates

    Every potential recipient at an Australian transplant unit is reviewed by a multidisciplinary committee that includes a transplant pulmonologist, cardiothoracic surgeon, transplant coordinator, infectious diseases physician, psychiatrist or clinical psychologist, and social worker. The intent is to characterise the disease, screen for occult comorbidity and confirm that the patient has the personal and logistical resources to adhere to lifelong immunosuppression, monitoring and infection avoidance.

    The Australian and New Zealand Lung Transplant Review Group has long argued that this team-based approach reduces post-transplant mortality and improves organ utility. Patients referred from outside the metropolitan catchment of The Prince Charles Hospital, the Alfred or St Vincent's often attend a week-long outpatient work-up that includes right heart catheterisation, coronary assessment, lung allocation scoring and an extensive psychosocial interview. Each component produces a small piece of evidence that, taken together, supports a binary decision: list now, list later, or do not list.

    Key eligibility criteria in Australian practice

    Although no two centres apply identical cut-offs, several criteria are widely accepted. Age is rarely an absolute barrier, with most units listing patients up to 70 and considering selected candidates beyond that. Body mass index must usually fall between 17 and 32, with values outside that range associated with worse survival. Active infection, recent malignancy within five years, and uncontrolled coronary disease are generally considered temporary or permanent contraindications.

    Equally important is assessment of adherence. Patients report that the daily discipline of medications, clinic visits and exercise is more demanding than the operation itself. Those with a documented history of missed appointments, untreated mental illness or active substance use face a higher threshold. Conversely, well-managed depression, a stable marriage or strong kinship care, and a track record of engaging with physiotherapy are positive signals the committee weighs deliberately.

    Timing the referral and listing

    The biggest avoidable mistake is referring too late. Australian data consistently show that patients listed from the intensive care unit or while on mechanical ventilation have markedly worse survival than those listed while still ambulatory. International guidelines suggest referral as soon as forced vital capacity falls below 80 per cent predicted with progressive decline, or earlier if there is symptomatic fibrosis, hospitalisation for exacerbation or a need for ambulatory oxygen.

    For those living in remote parts of the Northern Territory or the wheat belt of Western Australia, timing is complicated by the need to relocate close to the transplant unit for the often prolonged waiting period. Some centres arrange temporary accommodation in Melbourne or Brisbane through partnerships with not-for-profit lodges, but the burden on carers and the loss of income during relocation are real and should be discussed openly before listing.

    Preparing patients and families for the journey

    Once a patient is accepted, prehabilitation begins in earnest. Pulmonary rehabilitation, nutritional optimisation, treatment of reflux and management of osteoporosis are all addressed before the operation. Carer education is equally central, and most centres require at least one identified support person who can commit to months of bedside presence and ongoing assistance after discharge.

    Financial and logistical preparation matter too. Medicare covers the operation and most inpatient care, but out-of-pocket costs for accommodation, interstate travel and some medications can be substantial. Patients can sometimes access early release of superannuation on compassionate grounds, and the Patient Assisted Travel Schemes in Queensland, Western Australia and the Northern Territory provide modest subsidies. Discussing these realities early avoids surprises that derail recovery.

    Guidance for clinicians managing pulmonary fibrosis referrals

    • Refer when forced vital capacity falls below 80 per cent predicted and the disease is symptomatic, not when the patient is bed-bound.
    • Treat gastro-oesophageal reflux and obstructive sleep apnoea before listing, as both affect post-transplant outcomes.
    • Confirm antifibrotic therapy has been optimised and tolerated, but do not delay referral if it has not.
    • Engage an identified primary carer early and assess their capacity honestly.
    • Screen for coronary disease with CT or invasive angiography in everyone over 50, given the high prevalence in the Australian IPF population.
    • Discuss travel, accommodation and superannuation options before listing, not after the donor call.
    • Reassess eligibility at each follow-up, because medical and social circumstances evolve.

    Selecting the right candidate is rarely a single decision made at a single moment. It is a sequence of judgements made over months or years, ideally long before the patient reaches a tertiary centre in respiratory failure. The patient who is listed while still walking, has a committed family, lives within reach of specialist care, and has had every reversible comorbidity addressed is the one who will derive the most benefit. That triad of timing, support and preparation is what Australian transplant programmes are designed to protect, and it is the standard the referring physician carries from the moment they first suspect the trajectory has become irreversible.

    Richard Russell Nuffield Department of Clinical Medicine, University of Oxford (United Kingdom)
  • Mona Bafadhel King’s College London (United Kingdom)
    David Jackson Guy’s and St Thomas’ Hospital, King’s College London (United Kingdom)
  • James Chalmers University of Dundee (United Kingdom)
    David Price University of Aberdeen (United Kingdom)

Copyright ⓒ APSR2022 all rights reserved

APSR Congress Privacy Policy