The Guest Blog

Guest blog post by Carlos Lines Millán, President of the European Idiopathic Pulmonary Fibrosis & Related Disorders Federation (EU-IPFF).

Idiopathic Pulmonary Fibrosis (IPF) is a rare but devastating, irreversible and chronic disease affecting around 100,000 Europeans. The causes are unknown, no universal cure exists, it is only ever manageable and those seeking a lung transplant are playing a lottery. The combination of rarity, lack of diagnosis and limited access to IPF treatments can be a debilitating nightmare for patients. To improve patient lives, the European Commission must work together with member states to ensure timely access for IPF patients to orphan drugs approved by European Medicines Agency (EMA) and address inequalities in access to lung transplantation and non-pharmacological treatment.

Who are the people suffering from IPF? They may still be working or recently retired after many years’ work, looking forward to enjoying life. Long walks and a round of golf or two perhaps but then they begin to feel out of breath. The visit to the doctor sheds no light and their condition can go misdiagnosed for months or even years while more common respiratory diseases like asthma are suspected. Only after the simplest of tasks like climbing the stairs become akin to ascending mountains, are they finally referred to see a specialist.

What is known about IPF is that it affects the healing process of the lungs and over time they become thick and scarred causing a condition called fibrosis. This leads to a progressive decline in lung function. As an insidious disease it can remain stable for years and then your health can deteriorate suddenly from an initial shortness of breath and chronic cough, to respiratory failure and death.

Patients feel left behind and ignored by a society that is currently paying very little attention to this disease as it is so uncommon. The nature of orphan diseases such as IPF complicates matters further as the causality of symptoms can be difficult to determine. As a consequence patients like John in the UK and the IPF patient community as a whole often face desperate circumstances when looking for answers.

The poor provision of treatments to manage the disease and reduce patient discomfort also requires immediate action. Medications are available but how they are prescribed varies from one medical practitioner to another and a standardized approach across Europe is clearly needed. Drug approvals also need to be expedited at national levels and a European solidarity fund should be set up to allow access to drugs that have received European Medicines Agency (EMA) approval.

But there are various treatments available from the support and reassurance that Pulmonary Rehabilitation can provide early on through to Oxygen Therapy which helps patients remain active in the later stages of the condition.

These people deserve to be heard and treatments should be both made available and prescribed based on what the patient feels is working best for them.

Ultimately, as it stands the only successful treatment for IPF is a lung transplant. However, as the disease predominantly affects those aged between 50 and 70 years of age, this chance can be taken away by eligibility criteria and age limitations within some member states. It is a basic human right to have access to equal treatment irrespective of a patient’s stage in life or where they reside and all should receive a fair and realistic opportunity to undergo transplant procedures. The Commission can help make this reality by urging member states to address inequalities in access to lung transplantation and non-pharmacological treatment.

Furthermore, to advance the hope for IPF treatment, the European Commission must provide more funding and opportunities for patients to participate in IPF research by mainstreaming it into national agendas and the calls for European-funded projects under the European research framework Horizon 2020.

The brave people fighting IPF are our parents, our grandparents, our family and possibly ourselves in the future. It is necessary that the Commission and member states stand up to the challenge of IPF by funding research and increasing access to treatments.

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