News and Upcoming Events 

 

 

Would You Like To Become More Involved With ILFA?

Perhaps you could do a once-off fundraising event such as a coffee morning or maybe you would be interested in setting up a local ILFA support group where you live.

You can decide what level of involvement you would like to have. If you are interested in getting more involved, ILFA would like to hear from you.

You can contact us via post @ PO Box 10456, Blackrock, County Dublin; via email @ info@ilfa.ie or by calling 086 871 5264

The 2018 Fergus Goodbody Memorial Lecture

ILFA was set up in memory of Fergus Goodbody in 2002 to facilitate research, education and support for Idiopathic Pulmonary Fibrosis (IPF). Every two years since 2010, ILFA has invited an international leader in IPF to deliver an educational lecture to healthcare professionals in Ireland. The 2018 Fergus Goodbody Memorial Lecture on IPF took place on 14th March in collaboration with the Royal College of Physicians of Ireland (RCPI). 170 healthcare professionals registered to attend, making this our largest educational event to date. Dr Diarmuid O'Shea, Registrar of the RCPI, welcomed the speakers and audience to the Masterclass in Respiratory Medicine and was especially delighted to welcome Nicky Goodbody to the event.

Professor Toby Maher from the Royal Brompton Hospital, London spoke on 'Developments in IPF' and discussed the incidence, pathology, and treatment of IPF, and research highlights. The incidence of IPF is increasing and in the UK approximately 1 in 100 deaths are due to IPF, therefore the disease is not as rare as previously thought. The lungs are constantly exposed to substances such as smoke, dust and pollutants that cause repeated micro-injury at a cellular level. Scarring (fibrosis) is part of the normal wound healing process but with IPF, fibrosis goes out of control and is not switched off in susceptible individuals. In 2013, two successful clinical trials paved the way for the anti-fibrotic medications Pirfenidone and Nintedanib, to be prescribed as IPF treatments. The search for new drug discoveries is ongoing and currently over 20 different compounds are being studied as potential IPF treatments.

Prof Maher spoke about the potential of biomarkers (any test used to measure health, disease or response to treatment; for example, blood tests, spirometry measurements, scans etc.) to help characterise IPF patients. The PROFILE Study followed 600 newly diagnosed patients over 3-years and showed that IPF patients had a distinct blood protein signature compared to healthy individuals. And patients with more progressive IPF appear to have different protein profiles to those with stable disease. These results could help identify patients who may respond to different therapies. Studies investigating the delivery of drugs to the lung by inhalation are also in progress. This is an exciting time for research and evidence is accumulating for compounds that may be effective IPF therapies. At least 3 such compounds are likely to progress to clinical trials in the next year.

Prof Maher's presentation can be viewed here.    

 

Anne Marie Russell, Clinical Doctoral Research Fellow at the National Heart and Lung Institute at Imperial College London spoke on 'Palliative care and end of life management for IPF patients.’ The World Health Organisation defines palliative care as “an approach that improves the quality of life of patients and their families facing the problems associated with life-threatening illness, through the prevention and relief of suffering by means of early identification and impeccable assessment and treatment of pain, and other problems, physical, psychosocial, and spiritual.” Much of the learning on palliative care has originated from the cancer-care model.

The disease course with IPF is unpredictable and therefore patients should be informed that palliative care services are available if needed. The assessment of patients’ palliative care needs is a dynamic and iterative process and an integrated approach is needed to care for patients holistically and stream-line their care.

In 2017 a major international collaboration produced a review article entitled ‘Palliative care in interstitial lung disease: living well’ that was published in The Lancet medical journal. The article explained that palliative care focuses on optimising quality of life for IPF patients and managing symptoms effectively. Unfortunately, there are common misperceptions that palliative care is only for end-of-life care however palliative care has an important role in the early course of IPF to improve quality of life. Patients should have access to palliative care and be sign-posted to these services early on following their diagnosis.

Anne-Marie described some of the treatments to manage breathlessness, psychological distress and cough. Unfortunately, the current evidence base is weak, and more research is needed to determine the efficacy of treatments.

If patients are not informed about services available to them, they can feel lost and abandoned especially if they only have a yearly hospital review. Clinicians in the community have a duty to recognise when patients are deteriorating, to refer patients back to specialist centres for re-assessment, and review patients regularly to ensure appropriate treatment. Symptoms should be measured, managed and adjusted.

Anne-Marie highlighted work called ‘The Five Wishes’ that helps people consider what is important at the end of life. These questions address;

  1. The person I want to make care decisions for me when I can’t
  2. The type of medical treatment I want / don’t want
  3. How comfortable I want to be
  4. How I want people to treat me
  5. What I want my loved ones to know

 Anne-Marie's presentation can be viewed here.   


Professor Edward McKone
, St Vincent's University Hospital, spoke about Cystic Fibrosis, and Professor Anthony O'Regan, University College Hospital Galway, gave a lecture entitled 'Alphabet soup' and summarised different types of Interstitial Lung Disease. 


Prof O'Regan's presentation can be viewed here.   

 

The presentations are available to watch on the ILFA website and YouTube.

ILFA is grateful to the RCPI for their collaboration and to Boehringer Ingelheim Ltd. for supporting this educational event.

 

Photo: Eddie Cassidy, Nicky Goodbody, Prof Toby Maher, Anne-Marie Russell, Prof Anthony O’Regan, Prof Jim Egan
and Prof Edward McKone.