A call to action for idiopathic pulmonary fibrosis

A call to action for idiopathic pulmonary fibrosis
© iStock/gremlin

The EU-IPFF sets out a series of recommendations to improve the quality of life and treatment outcomes of people living with idiopathic pulmonary fibrosis (IPF).

In November 2018, the European Federation of Idiopathic Pulmonary Fibrosis and Related Disorders (EU-IPFF) Benchmarking Report on Access to IPF Care in Europe was launched and reflected yet another important accomplishment for the IPF community. The comparison of care standards identifies best practices to be encouraged and gaps to be filled, proposing concrete solutions to improve patients’ quality of life. The resulting conclusions will propel policy asks to ensure a high level of quality care across Member States.

Idiopathic pulmonary fibrosis is a rare, chronic, progressive disease that affects the lungs. Due to its rarity, most people have never heard of it. Nevertheless, for those affected, the symptoms (among which are shortness of breath and dry cough) are debilitating: a gradual decline in lung function leads to reduced oxygenation of the blood, brain and other vital organs. Without treatment, many patients only survive two to five years after diagnosis.

Pharmaceutical investment in this area has been a game-changer. With the development of two drugs, treatment is widely available throughout Europe. However, there are various unmet needs that may cause a delay in receiving treatment, which can increase the progression of the disease and in some cases, due to the severity, treatment cannot be provided, leading to a fatal ending.

The EU-IPFF was established in 2016 to improve the care and the quality of life of IPF patients across the EU. As a federation, they have developed the first European IPF Patient Charter and successfully encouraged the adoption of a Written Declaration by the European Parliament. In addition, they launched the first IPF Benchmarking Report in November 2018.

The Benchmarking Report

With a view to driving evidence-based policies, the EU-IPFF commissioned the Benchmarking Report to measure how those European countries represented by its members are performing on different areas of IPF care and management. The report highlights best practices and identifies gaps where urgent policy action is needed.
The Benchmarking Report aimed to collect quality information about IPF patient care in EU-IPFF member countries in order to:

  • Compare the situation of patients between countries
  • Identify gaps in IPF care across EU-IPFF member countries
  • Identify best practices in IPF care across Europe
  • Make recommendations for solutions that could improve IPF care and the overall quality of life of IPF patients.

Key findings of the Benchmarking Report

The Benchmarking Report outlines the current state of IPF care and management in Europe, identifying best-performing countries along with challenges that demand greater political attention and an immediate response.

In terms of international guidelines on idiopathic pulmonary fibrosis,1 it was found that the majority of them are validated by relevant international respiratory medical societies, including the European Respiratory Society. Nevertheless, only a handful of countries have developed or are developing national guidelines, or are on track to implement such guidelines.2 This means that on a national level there are still various discrepancies when it comes to the care and management of IPF.

In addition, the Benchmarking Report showed that IPF patients lack access to reliable information. In most countries, information and self-management materials are either limited or not fully integrated into the care process. Newly diagnosed IPF patients do not always receive supporting information from specialised centres; in the majority of the cases, it is up to patient support groups to provide these. Additionally, self-management education tools are not always available for patients.

One of the largest challenges that IPF patients still face today is diagnosis. Across all countries, respondents reported that prompt diagnosis is challenging. Patient representatives noted slow referral periods, with extended gaps between the initial suspicion of idiopathic pulmonary fibrosis and a confirmed diagnosis. Delayed diagnosis or misdiagnosis due to scarce awareness of symptoms or delayed recognition are major obstacles within the diagnostic process. As treatment is necessary for increased survival time, a delay or misdiagnosis can be fatal for patients. With a median survival time of three years it is of great importance that patients receive the necessary care as soon as possible.

Despite IPF care being fragmented across Europe, the Benchmarking Report revealed that EMA-approved pharmacological treatments for IPF are available and reimbursed with no out-of-pocket costs for patients in all 13 countries surveyed. However, prompt referral to specialised centres, a prerequisite for receiving a timely prescription for treatment, remains a major challenge. Many respondents deemed the ‘watch-and-wait’ approach to treatment to be a barrier. In addition to pharmacological treatment there are also non-pharmacological options available for patients suffering from IPF. This includes oxygen therapy, pulmonary rehabilitation and lung transplantation. A number of interviewees also reported access to non-pharmacological treatment as being either restrictively available or, to some extent, unavailable. When available, it is not always fully covered or reimbursed, resulting in out-of-pocket expenses for patients. This can be challenging for IPF patients as non-pharmacological options can help reduce the symptoms and increase quality of life.

Lastly, access to palliative care and end-of-life care is extremely fragmented throughout Europe. Some countries have established palliative care centres and teams, while others are only beginning to provide this kind of support. Of course, this is a sensitive topic but should remain a priority for implementation.

The IPF call to action

Considering the unmet needs that the Benchmarking Report shed light on, EU-IPFF has consolidated a list of ten recommendations for European and national policymakers:

  1. Rare diseases should remain a priority in the future European Commission agenda
  2. Funding is earmarked for rare diseases in the future Horizon Europe framework, in order to reach the ambitious target of developing 1,000 new therapies for rare diseases by 2027, including a cure for idiopathic pulmonary fibrosis3
  3. To increase support for the integration and establishment of European Reference Networks for screening at-risk groups, expediting diagnosis and encouraging standardisation of rare disease care (including access to non-pharmacological treatment)
  4. To increase opportunities for rare disease patient groups to collaborate and capacity-build, encouraged through future funding programmes, such as the European Social Fund Plus and the European Joint Programme on Rare Diseases
  5. To increase awareness raising of IPF amongst healthcare professionals and primary care practitioners to improve the recognition of early signs and symptoms, and ensure the implementation of IPF guidelines within the country
  6. To support the collaboration with relevant European Reference Networks and networking excellence at national level, to better connect primary, secondary and tertiary care, and foster early diagnosis and timely access to treatment
  7. To promote multidisciplinary care and recognition of specialised nurses at national level
  8. To recognise the burden and financial impact of idiopathic pulmonary fibrosis on patients and their families, and ensure access to non-pharmacological treatment, especially oxygen therapy
  9. To provide access to palliative care to all IPF patients
  10. To encourage the development of local support groups, given their role in providing patients with information and peer support.

The Benchmarking Report in the European Parliament

On 20 November 2018, the Group of Socialists and Democrats and EU-IPFF hosted an event at the European Parliament. The event, titled ‘A forward-looking agenda for rare diseases: bringing Europe closer to its people’, reviewed what has been achieved on the topic of rare diseases in the past four years and what remains to be done.

Against the backdrop of the current mandate of the European Parliament and European Commission drawing to a close, the event brought together stakeholders from across the health sector, as well as EU policymakers – notably MEP Elena Gentile (S&D Group, IT), who discussed the role of the European Parliament in keeping rare diseases at the top of the policy agenda. Annika Nowak, member of the cabinet of Commissioner for Health and Food Safety, Vytenis Andriukaitis, spoke about how the Commission will continue to ensure that rare diseases remain a political priority for the new legislature. In addition, Franz Schaefer, chair of the ERNs Coordinators Group, discussed how to scale up successful initiatives within the European Reference Networks.

During the event, the achievements of the past few years were celebrated, including the establishment of the ERNs, a network of highly specialised European healthcare professionals working on rare diseases. However, it was also acknowledged that gaps remain within and across EU countries when it comes to delivering care and treatment for complex, chronic conditions such as idiopathic pulmonary fibrosis. These unmet needs were discussed using the Benchmarking Report as a focal data point. As Francesco Bonella, chairman of the EU-IPFF’s Scientific Advisory Board, says: “Besides the improvement of services and access to care for patients, implementing and integrating research in the clinical setting would bring real, positive change for people with rare diseases.”

Speaking about the importance of EU support for rare disease patients, EU-IPFF secretary Liam Galvin said: “It is amazing to see how EU-IPFF has made such an impact since the foundation year in 2016. The involvement of the European Institutions has fostered our hope and that of many patients, doctors and researchers. It is now up to EU-IPFF, healthcare professionals and policymakers at European and national level to turn our recommendations into concrete actions.”

While healthcare delivery remains a Member State competence, there is a role for the EU to play in sharing best practices and pooling resources to benefit all European IPF patients. The upcoming 2019-2024 European parliamentary term represents an opportunity for future EU leaders to promote radical policy change that can improve healthcare services and outcomes.

Ultimately, this Benchmarking Report will inform decision making at every level to eliminate the inequalities in access to specialised care that people with idiopathic pulmonary fibrosis still experience today.

For more information on the Benchmarking Report, please visit www.eu-ipff.org.


  1. Available at https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5450933/
  2. The following countries have developed national guidelines: Germany, United Kingdom, Ireland. The following countries are in the process of developing national guidelines: Poland, Austria.
  3. As set by the International Rare Diseases Research Consortium (IRDiRC) – http://www.irdirc.org/about-us/vision-goals/

Professor Francesco Bonella
Ruhrlandklinik University Hospital, Essen
Chairman, EU-IPFF Scientific Advisory Board
Liam Galvin,
Secretary, EU-IPFF
Director, Irish Lung Fibrosis Association
Tweet @EU_IPFF

This article will appear in issue 8 of Health Europa Quarterly, which will be published in February 2019.

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