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Advancing in IPF Research

The 6th Adavancing in IPF (AIR) meeting took place in Vienna, Austria on 18th and 19th of November and brought together almost 300 respiratory healthcare professionals from across Europe. This unique meeting focussed on recent developments in interstitial lung disease (ILD) and idiopathic pulmonary fibrosis (IPF) and some of the world’s leading experts in this field shared their insights. The meeting was chaired by Professor Athol Wells from the United Kingdom and Professor Carlo Vancheri from Italy.

The meeting got off to a great start as Professor Bruno Crestani from France discussed recent developments in our understanding of what factors contribute to IPF at a cellular level. The cells in the lungs are particularly susceptible to damage due to contaminated air, chemicals, particles, cigarette smoke, bacteria and viruses that we breathe in. Lung fibrosis starts when the normal repair mechanism in the lungs goes out of control and scar tissue forms. Recent innovative research has looked at the increase of certain biological molecules when experiments that physically ‘stretch’ lung cells are measured. Going forward, some of these biological molecules may be selected as potential research targets.

 

Professor Wym Wyts from Belgium addressed the audience on recent developments in oxygen treatment, pulmonary rehabilitation and anti-reflux therapies. The research available reinforces that these elements are crucial for helping patients maintain their quality of life and can help alleviate symptoms. He concluded his presentation by announcing that hope is on the horizon as more studies on patient reported outcome measures (PROMS) are being conducted to help assess, improve and maintain quality of life.

 

Professor Carlo Alberra from Italy described the latest understandings in relation to the benefits of the anti-fibrotic medicines, Perfinidone and Nintedanib, for patients with IPF. Both medicines can preserve lung function although they work by different mechanisms. He also discussed some of the new drugs currently under investigation. FG3019 is a new molecule that has a good safety and tolerability in very preliminary studies. There are currently 8 drug trials underway for IPF and hopefully there will be positive results for a few of these entities in the coming years.

Professor Martin Kolb from Canada discussed the latest advances in diagnostic techniques. A biomarker is any biological measurement that can enhance our understanding of a medical condition, for example, lung function tests, CT scans, and measurements of biological molecules. In order to be useful, a good biomarker should be involved in the disease mechanism, reflect disease severity (in order to be a good indicator of prognosis), and the measurements must be reproducible, sensitive and specific. Prof Kolb spoke about some of the genetic biomarkers that are currently under investigation but stressed that biomarkers are not yet ready for use in the treatment or prognosis of IPF.

 

Professor Sara Tomasetti from Italy spoke about cyro-biopsy as an important and emerging technique for taking lung biopsies. This technique is fast, easy to use and involves freezing samples of lung tissue to minus 80 degrees Celsius. The lung tissue that is retrieved can subsequently be prepared for examination under a microscope. Major drawbacks to this technology include the lack of a standard protocol for the technique and the number and size of biopsies needed to give a clear picture of the lung architecture, and the possibility of developing complications for example, bleeding or subsequent risk of infection. This technique should only be performed by trained experts.

 

Dr Simon Walsh from the United Kingdom spoke about the challenges facing radiologists (doctors who read x-rays and CT scans). Radiologists assess images of the lung tissue to identify areas of honeycombing – a distinct pattern, similar to the inside of a ‘crunchie’ bar, and subtle changes called ‘ground glass opacities’ that are associated with IPF. New technology including computer analysis of CT scans is coming and will help the multi-disciplinary team diagnosing IPF, however this technology will not replace the specific expertise and experience of trained respiratory radiologists.

Professor Vincent Cottin from France spoke about the difficulties of accurately diagnosing IPF. A correct diagnosis is vitally important as it impacts on the patients’ treatment, the use of anti-fibrotic medicine, and the disease prognosis. For a significant proportion of patients, a ‘working diagnosis of IPF’ is given as it is very difficult to classify the disease fully without all the results (for example, if a patient does not have a lung biopsy).

 

Professor Athol Wells talked about the different stages of IPF – early disease and advanced disease. He highlighted the need to incorporate a measurement of exercise to help patients as they can struggle to understand the severity of their disease in medical terms. He encouraged the audience to have in-dept discussions with their patients to decide on the best treatment for them. There is no ‘one-size-fits-all’ approach for IPF.

Professor Jim Egan from the Mater Misericordiae University Hospital in Dublin gave a state of the art lecture on Lung Transplantation. He described the low level of lung transplants across Europe as an unmet patient need that needs to be addressed. The question he is asked most frequently concerns a patients age and if they are eligible for a lung transplant. Prof Egan believes that every patient should be considered for a transplant provided that they have good general health (apart from their IPF). Patients should have a strong heart, good kidneys. an acceptable body weight, and a willingness to take part in exercise and pulmonary rehabilitation classes to maximise their exercise tolerance. He challenged the audience to speak with their local lung transplant centres in their countries and to engage with the transplant surgeons on behalf of their patients. Internationally, 40% of all lung transplants should be performed in IPF patients but some surgeons are nervous about operating on older adults (those aged over 65 years). This mindset needs to change. Lung transplantation is completely life-saving for patients with IPF and the survival outcomes are improving all the time.

 

Professor Marlies Wijsenbeek from the Netherlands spoke about managing patients diagnosed with IPF using the “A-B-C-D-E” approach.

A = assess the patient’s needs

B= back up the patient and give them support

C= comfort care – this term refers to managing patients’ symptoms. For example, the treatment of cough was discussed. Cough can be a very debilitating symptom and one study showed that patients cough on average 60 times every hour. More research on cough is needed.

D = disease modifying drugs for example using the medicine Perfinidone and Nintedanib (anti-fibrosis drugs) to slow down disease progression

E = end of life discussions

Professor Wijsenbeek told that audience that well informed patients can make realistic choices regarding their care and speaking candidly with patients will help them make decisions that reflect their values.

 

Dr Toby Maher from the United Kingdom described a study where 50 patients used simple spirometry devices at home to see if this technology could help predict disease progression. Patients received training in how to use the device and they were asked to blow into the machine once a day at the same time every day and record the result in a diary. Patients were asked to contact the hospital if the result had decreased by 10% over 3 days. The results between the home devices and the hospital equipment showed similar patterns although the readings did not match. Some patients could not complete the study due to the development of a problematic cough when using the machine. Overall, the study showed that home monitoring is helpful, however larger studies are needed to verify the findings.

Dr Maria Molina-Molina from Spain described the treatment of familial IPF - when two or more members of the same family have an interstitial lung disease. She also stressed the importance for doctors and nurses to take a very detailed medical from patients and to ask about the health other family members and relatives. It is important to find ask about the entire history of other family members on a few different occasions in case new information comes to light. Genetic testing to find defective genes is not at a clinically useful stage at the moment but research is ongoing. Current advice for patients includes stop smoking if they are smokers, lead a healthy lifestyle, and minimise the risk of a lung injury by avoiding exposures to pollution, chemicals, dusts, and bird droppings.

Gunther Wanke is an IPF patient from Austria and he was invited to address the audience and share his perspectives on being diagnosed with IPF. Gunther spoke in German and his talk was translated so the audience could understand. He described his symptoms, seeking medical help and being diagnosed with IPF. He also described how the health system in Austria supports patients by funding specialist nurses who visit patients in their home. He discussed how he manages to live well and maintain his health. Gunther spoke very well and emphasised that “Life is different but it’s a life worth living”.

Nicola Cassidy (ILFA) was invited to deliver a presentation entitled “What matters to patients and carers”. Nicola discussed ILFA’s involvement in an international project organised by the European Respiratory Society’s Taskforce on IPF and the European Lung Foundation. Patient organisations across Europe were invited to take part in this major project and patients and carers from Belgium, Italy and the United Kingdom also participated. Focus groups of patients and carers were asked for feedback on a number of topics and the patient priorities that emerged included the need for good communication, quality of life, exercise and end of life care. Nicola highlighted some of the communication difficulties and experiences of poor communication reported by patients. To overcome bad communication, healthcare professionals need to learn continually. It is important to use plain language, speak slowly, get to know that patient’s values and understand what’s important to them and their family, and also encourage the patient and carer to ask questions.

Anne Marie Russell from the United Kingdom spoke about patient communication strategies. Anne Marie offered advice to healthcare professionals on how to structure appointments and consultations with patients, how to start difficult conversations and how to be self-aware. It is important for healthcare professionals to establish good rapport with patients and help them discuss and understand the treatment options and support them in the decision making process.Anne-Marie also described how different communication styles may be needed at different points of the disease course. The latter part of her presentation described patient's use of the Internet as a source of information on IPF. A recent study showed that there is no reliable method for patients to identify websites that provide appropriate information on IPF and this can result in patients relying on websites that provide incorrect, inaccurate or false information. Anne-Marie finished by describing new ways of engaging with patients via technological advances including the patientMpower app that is being developed here in Ireland in collaboration with ILFA.

The AIR Vienna Conference was a very special meeting - it was fast paced, highly educational and superbly organised. Sincere thanks to the Co-chair, Professor Athol Wells, for championing the patients and carers perspectives at the AIR Meetings and inviting ILFA’s participation over the last 4 years.