lungsTo coincide with Rare Disease Day 2016, pharmaceutical company Boehringer Ingelheim has announced two new initiatives to support people living with idiopathic pulmonary fibrosis (IPF).

In the UK, more than 15,000 people have IPF, which is a fatal condition characterised by permanent scarring of the lungs and loss of lung function over a period of time. It can take up to 2 years to confirm diagnosis from the onset of symptoms, and most patients won’t live more than 3 years from diagnosis.

The first of the new initiatives launched to address the lack of understanding around IPF is Inspiration magazine, which is a collaboration between patients, patient groups and leading clinicians to put IPF in the spotlight and calls for greater awareness and understanding of this devastating condition.

The second initiative was an international study, which has provided an insight into living with IPF. The study looked at the experiences of more than 500 people living with IPF in more than 20 countries worldwide, and revealed the real-life emotions faced by those with the condition. The results are launched on Rare Disease Day 2016 to coincide with this year’s theme ‘Patient Voice’, recognising the crucial role that patients play in voicing their needs.

When asked ‘what do you think of when you think of IPF?’, the thought most frequently expressed by respondents was breathlessness (33%), one of the most common symptoms of IPF. As the condition causes the lung tissue to become thickened, stiff and scarred over time, the reality is that shortness of breath makes it difficult for people affected by IPF to carry out everyday tasks such as showering or getting dressed.

The research also revealed that the progressive and unpredictable nature of IPF places an emotional burden on those affected by the condition. Almost a quarter (23%) of respondents said they fear not being able to do the things they used to do before having IPF and 1 in 3 people (29%) said they were “frustrated” by the condition.

Professor Luca Richeldi from the University of Southampton, said:  “I am impressed by the efficacy and easy reading of the whole report. It really catches the attention on the important points and is something that so far has not been available to the community.

“With the number of people being diagnosed with IPF in the UK steadily increasing year on year, government and policymakers need to prioritise this condition as though it were a cancer. This report will go a long way towards achieving this aim.”

Dr Toby Maher, Consultant Respiratory Physician at the Royal Brompton Hospital in London, added: “This thought-provoking research echoes what my patients talk to me about when we discuss life with IPF. Thankfully our understanding of the treatment of IPF has moved forward a lot in the last few years. As physicians we can help address the negative emotions many patients experience at diagnosis and improve how they see their future with the disease so they feel positive about starting effective treatment.”

Patient advocacy

Patient advocacy groups also play an important role in making it easier to live with the condition; 42% of respondents said that patient groups made them feel less isolated and provide access to important information.

Mike Bray, Chairman of Action for Pulmonary Fibrosis, said: “Living with IPF can be distressing and too few people appreciate just how isolating living with a rare disease like IPF can be. This research and the launch of the collaborative magazine Inspiration are important ways for those of us who are affected to be heard, so that understanding and support for the IPF patient community continues to improve."