Launch of Position Statement and Patient Registry for Pulmonary Fibrosis

Irish Thoracic Society Calls for the Appropriate Staffing and Resourcing of Centres of Expertise, and Implementation of Standard Pathway of Care for all Patients with Lung Fibrosis in Ireland

Call comes at launch of patient registry and position statement on management of lung fibrosis in Ireland

The Irish Thoracic Society has today called for the centres of expertise in lung fibrosis across Ireland to receive the required resources and staffing so that all patients with idiopathic pulmonary fibrosis (IPF) – more commonly known as lung fibrosis – receive the same high standards of care. The society is also calling for a clear and specific National Clinical Programme to facilitate a standard pathway of care and to ensure the disease receives the critical focus it requires at all levels of the health service.

IPF is a chronic, fatal disease characterised by progressive scarring of the lungs – approximately 1,000 people in Ireland are living with it, with around 400 new cases diagnosed each year – a similar frequency to that of stomach, brain, and testicular cancers. It is more common in men than women, with a death rate worse than many forms of cancer. Evidence suggests that death rates for IPF in Ireland are above the European Union average.

The call came at the launch of a new national patient registry for lung fibrosis by Catherine Byrne TD, the Minister of State for Health Promotion, taking place in the Royal College of Physicians of Ireland. The registry, alongside the publication of a position statement on the management of lung fibrosis, are intended to increase knowledge of the disease and improve the care of patients in Ireland. Both are initiatives of the Irish Thoracic Society – the national organisation of healthcare professionals caring for people with lung disease – and are the culmination of intense multi-disciplinary work by the respiratory community in Ireland.

The Irish Thoracic Society is urging that the specialist centres of expertise in lung fibrosis be resourced with multi-disciplinary teams (MDTs) to ensure early diagnosis and optimal care for patients. These centres should be structured to ensure fair and equitable access for all patients no matter where they live and to ensure that patients with suspected lung fibrosis are diagnosed and treated within 16 weeks. Centres currently have significant deficits in terms of resources and the staffing of multi-disciplinary teams. A specialised multi-disciplinary team should include:

  • A respiratory consultant with a special interest in lung fibrosis
  • Lung fibrosis specialist nurse (circa. 1 per 50 patients)
  • Specialist respiratory physiotherapist with an interest in lung fibrosis
  • Access to thoracic surgery, rheumatology, palliative care, medical social worker, psychological and nutritional supports

Professor Anthony O’Regan, Consultant Respiratory Physician, believes that the development of new therapies means a much more hopeful outlook for patients that must be capitalised upon:

“Until relatively recently the lack of effective diagnosis and treatment options for lung fibrosis, meant it was the ‘Cinderella’ of respiratory diseases. However, the world of treating lung fibrosis has changed dramatically over the past decade thanks to novel therapies and an increase in the rate of successful lung transplants. These developments have brought real hope to patients, making early diagnosis and rapid referral to treatment more crucial than ever. Unfortunately, as lung fibrosis progresses, patients’ needs become more complex and multi-faceted.

“Consequently, we must be able to respond quickly with access to appropriate medical, physiotherapy, nutritional, psychological, social and palliative supports, as well as information on accessing lung transplantation where appropriate. It is incumbent on the health service to act urgently to resource all centres of expertise in lung fibrosis to the required standards. This should be achieved in tandem with the development of a National Clinical Programme on lung fibrosis to reflect how seriously we take this disease. Only then will patients reap the benefits of a standard pathway of care and access to novel treatments in a way that is fair, equitable and timely.”

David Crosby, who lives with IPF (lung fibrosis) and was the recipient of a double lung transplant in 2016, knows the importance of optimum care:
“As someone who has travelled the road from a devastating diagnosis through to a life-saving double lung transplant, I know what it means to have the opportunity to have a second chance at life. I have experienced first-hand the benefits of being under the care of a team of people with specialist expertise. I had a nurse specialist who was my main point of contact and guiding light, as well as access to a physiotherapist, nutritionist and counsellor who helped me to manage my condition both physically and mentally. Of course, that’s not to mention my consultant and the transplant team who gave me a new lease of life. But I was one of the lucky ones. Everyone with a diagnosis of IPF must have access to these supports, regardless of where they live.”

Professor Ross Morgan, President of the Irish Thoracic Society, acknowledged the contribution of many experts in the development of the initiatives:

“The commitment of medical and healthcare professionals to optimising the care of patients with lung fibrosis is illustrated by the involvement of a wide range of disciplines in this work – including physicians, surgeons, pathologists, radiologists, nurses and physiotherapists. Patients had a particularly important role to play, and were represented by the Irish Lung Fibrosis Association. This has been a truly multi-disciplinary process and one that fully reflects the complexity of the disease and the needs of patients. The Irish Thoracic Society has been very proud to facilitate this work.”