An international survey of over 500 people living with idiopathic pulmonary fibrosis (IPF) in more than 20 countries worldwide reveals the real-life emotions faced by those with the devastating disease. IPF affects approximately 3 million people worldwide, yet the reality of the condition remains little understood. 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.
Diagnosing IPF can be difficult, and around half of patients are initially misdiagnosed. The average time from first symptoms to diagnosis of IPF is between one and two years, and patients are then faced with the reality that they have an incurable disease. The emotional turmoil people with IPF experience is clearly reflected in the answers; many respondents said they felt ‘worried’ (49%), ‘afraid’ (45%), and ‘confused’ (34%) when they finally received a diagnosis.
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 reveals that the progressive and unpredictable nature of this fatal lung disease places an emotional burden on those affected by the condition; almost a quarter (23%) of respondents stated that they feared not being able to do the things they used to do before having IPF and one in three people said they felt “frustrated” by the condition (29%). The emotions felt by people affected by IPF vary following diagnosis and after receiving medical care; 31% of respondents felt that emotions become more positive and the outlook brighter at that time whilst 28% felt that emotions become more negative and the outlook less bright.
Dr Toby Maher, Consultant Respiratory Physician at the Royal Brompton Hospital in London, United Kingdom commented:
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.
Early and accurate diagnosis of IPF is important, as this enables the early initiation of pharmacological treatment and other disease management options.
Patient advocacy groups also play an extremely 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.
Robert Davidson, President, Canadian Pulmonary Fibrosis Foundation (CPFF) said:
Living with IPF can be frightening and too few people appreciate just how isolating living with a rare disease like IPF can be. This research is an important way for those of us who are affected to be heard, so that understanding and support for the IPF patient community continues to improve.
While a future with IPF is at times a daunting prospect, many people affected by the disease expressed a positive view of the year ahead. When asked what they or someone they know with IPF would like to spend time doing over the next year, 39% said they would enjoy time with family and one in five (21%) said they would like to travel or go on holiday.