Coinciding with the Rare Disease Day 2016 theme, “Patient Voice — Join us in making the voice of rare diseases heard,” an international survey’s results were released, giving voice to more than 500 idiopathic pulmonary fibrosis (IPF) patients across 20 countries, and detailing the tumult of emotions they experience living with a rare and deadly disease.
IPF is indeed a daunting illness, the survey’s responses highlighted, with many patients reporting reactions to their diagnosis that ranged from “worry” (49 percent) and “fear” (45 percent), to “confusion” (34 percent). When asked, “What do you think of when you think of IPF?” the most frequent response was “breathlessness” (33 percent), which is also one of the more common symptoms of the disease. Patients also spoke of being “fearful of not being able” to do things they did before becoming ill (23 percent), and of being “frustrated by the disease” (29 percent).
Because IPF is difficult disease to diagnose and is often misdiagnosed, people wait on average one to two years between the start of their symptoms and the start of proper medical treatment. Those under IPF medical care, perhaps for this reason, had varied responses, with 31 percent saying their emotions became more positive and their outlook brighter after diagnosis and treatment, and 28 percent expressing more negative feelings and views. Importantly, the results revealed the extreme importance of patient advocacy groups, with 42 percent of respondents stating that patient groups made them feel less isolated and provided them with access to important information.
Dr. Toby Maher, consultant respiratory physician at Royal Brompton Hospital in London, said in a press release,“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.”
Despite the debilitating nature of the disease, patients spoke positivity of their plans for the coming year, which included enjoying time with family (39 percent), and traveling and vacations (21 percent).
IPF is a chronic interstitial lung disease affecting the tissue that supports the alveoli in the lungs. It is characterized by inflammation and the accumulation of scar tissue (fibrosis), leading to progressive loss of elasticity, failure of proper lung activity, and insufficient oxygen to the blood and organs. The disease is of unknown origin, with no cure. IPF clinical course and progression varies among patients, and the trajectory and rate of decline are difficult to predict at diagnosis. According to the National Organization for Rare Diseases (NORD), the exact incidence of IPF is unknown, but estimates point to 2 to 29 affected people for every 100,000 in the general population.
The Rare Disease Day is celebrated each year on the last day of February, which this year fell on Feb. 29.