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Article Summary
Patient Experiences With Pulmonary Fibrosis

Collard HR, Tino G, Noble PW, Shreve MA, Michaels M, Carlson B, Schwarz MI. Respir Med. 2006, doi:10.1016/j.rmed.2006.10.002.

Introduction:

• Accurate diagnosis of pulmonary fibrosis (PF) remains a challenge, although important advances have been made in defining the clinical, radiologic, and histopathologic criteria^1,2.
• This study addresses how advances in research have impacted community practice and the information patients are receiving from their physicians.
• The attitudes and experiences of pulmonary fibrosis patients were explored, with regard to diagnosis and management of their disease.

Study Design:

• The Coalition for Pulmonary Fibrosis (CPF)³ developed a 52-question survey in conjunction with clinicians to collect information from its members regarding patient experiences with the management of IPF and other forms of pulmonary fibrosis. The questionnaire was distributed between July 2003 and December 2004.
• Information requested: demographics, family history, potential environmental exposures, diagnostic testing performed, treatments prescribed, and patient attitudes regarding the adequacy of information received from health care providers.
• The questionnaire was distributed to more than 2000 CPF member patients and caregivers.
• Completed questionnaires were compiled by Michaels Opinion Research, Inc., an independent research firm.

Results:

• 1448 current patients (n=1251) or caregivers of current patients (n=197) comprised the study group.
• Education:
• 63.5% agreed that there was a clear lack of information and resources about PF available at the time of diagnosis, and only 51.2% reported being informed about treatment options.
• Diagnosis:
• 55% of respondents reported a delay of > 1 year between the earliest indications of a potential breathing problem and the diagnosis of pulmonary fibrosis.
• Initial incorrect diagnosis of PF was reported frequently: bronchitis (18.5%), asthma (14.1%), COPD (7.5%), emphysema (6.6%), and heart disease (5.0%).
• 50.2% reported having a surgical lung biopsy and 21.5% reported having undergone a bronchoscopy.
• Treatment:
• Overall, 74.7% reported current pharmacotherapy for PF.
• 49.8% reported currently taking prednisone and another 20.9% had been prescribed prednisone in the past.
• Some patients reported receiving cyclophosphamide (9%), azathioprine (18.9%), or colchicine (12.5%), indicating that fewer than half are treated with the ATS/ERS-recommended prednisone plus cytotoxic agent.
• 28.5% reported use of interferon gamma 1-b.
• 24.4% reported use of herbs or nutritional supplements.
• Percent of respondents reporting receiving no pharmacotherapy has been increasing (see Figure 1, **P<0.01, *P<0.05).

Implications/Recommendations:

• Efforts should be increased to educate PF patients about diagnosis and disease management.
• There is a trend towards not treating PF, and off-label drug use is common.
• Limitations of study:
• Data are self-reported by CPF members; accuracy of facts such as medication or biopsy method not subject to verification.
• 50% response rate is good for surveys, but may be source of sampling bias.
• No account taken of QOL.

Expert Opinion:

Collard and colleagues, in their large survey of patient experiences and perceptions of pulmonary fibrosis, found that at diagnosis half of the patients did not have a clear understanding of the disease and the available treatment options. What are the reasons for this lack of patient information? Most IPF patients are cared for by primary care physicians and community-based pulmonologists, who may not have the same depth of experience with IPF as academic pulmonologists or specialized nurses. Pulmonary fibrosis is often misdiagnosed as CHF, asthma or COPD. Early diagnosis permits referral to specialized centers and optimal disease management, which may entail pulmonary rehabilitation, proper use of oxygen, cardiovascular fitness, weight reduction, enrollment in clinical trials and lung transplantation.

What can be done to rectify the situation? There are a number of programs available to the practicing pulmonologist that provide informative updates on diagnosis and management of IPF. Additionally, education of primary care physicians, medical students, residents and fellows will be critical as effective therapies are developed.

References:

1. American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med. 2000;161:646–664.
2. Flaherty KR, King Jr TE, Raghu G, et al. Idiopathic interstitial pneumonia: what is the effect of a multidisciplinary approach to diagnosis? Am J Respir Crit Care Med. 2004;170(8):904–910.
3. Coalition for Pulmonary Fibrosis Web site. Available at: http://www.coalitionforpf.org/. Accessed February 6, 2007.

Article link:

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