AB017. Interstitial lung disease patient diagnostic journey (intensity)

Background: Interstitial lung disease (ILD) is complicated to diagnose because its signs and symptoms mimic a wide range of common medical conditions. The objective of the INTENSITY survey was to advance the understanding of respondents’ diagnostic experiences with ILDs, including idiopathic pulmonary fibrosis (IPF). The survey was designed to trace the path that respondents took to diagnosis, identify obstacles they faced, and gauge the emotional impact of their journey. Results from the INTENSITY survey will help us more fully understand respondents’ diagnostic experiences so we can further our efforts to improve timely diagnosis and treatment of ILDs.

Methods: We administered a 25-minute quantitative online survey from August 14–26, 2015. Of the 1,152 respondents screened to ensure they were US residents, and had been diagnosed with an interstitial lung disease, 600 met criteria and completed the survey. Recruitment was accomplished through two channels: email invitations sent to 16,000 Pulmonary Fibrosis Foundation (PFF) members and an open invitation to participate through the pulmonary fibrosis community website

Results: Shortness of breath (77%) and cough (53%) were the most common early symptoms in ILD patients. Twenty-five percent of respondents saw their primary care physician once before being referred to a specialist, however 61% of respondents saw their primary care physician >2 times before referral to a pulmonologist. Misdiagnosis occurred in 55% of respondents. The median time from symptom onset to correct diagnosis was 11 months, however 49% of respondents carried an incorrect diagnosis for 1 to 10 years. Thirty-eight percent were misdiagnosed more than twice, most frequently with asthma, pneumonia, bronchitis, and allergies. An accurate diagnosis was achieved after a median of 6 months and a median of 3 physician visits. The majority underwent an invasive diagnostic procedure. Fourteen percent of respondents saw more than 6 physicians before receiving a correct diagnosis. For 28% of respondents the diagnostic process took over 2 years. Sixty-one percent of patients underwent an invasive diagnostic procedure (bronchoscopy or surgical lung biopsy). Forty-five percent underwent a surgical lung biopsy and 21% of patients underwent both procedures. Sixty-four percent of respondents acknowledged experiencing stress due to a lack of understanding about their disease, with 51% believing that their symptoms were associated with aging. Seventy-three percent of respondents felt isolated because friends and family were not familiar with ILD or IPF. This was compounded by time away from families, friends and personal life related to the diagnostic odyssey experienced by 84% of respondents.

Conclusions: During their diagnostic evaluation, adults with ILD endure misdiagnoses, long delays in achieving a correct diagnosis, and emotional hardships. The barriers to a timely, accurate ILD and IPF diagnosis might be overcome with a more focused effort dedicated to disease and symptom awareness as well as tools to improve diagnostic accuracy.

Keywords: Interstitial lung disease (ILD); diagnostic experiences; intensity survey