Think of healthy lungs like a wet sponge. A wet sponge is easy to squeeze, then quickly expands back to full size - much like easily taking a breath. Now, imagine a dry sponge. A dry sponge is like scarred and stiff lung tissue.1 It has very little flexibility and makes it difficult to breathe normally.
This is similar to what people living with progressive fibrosing interstitial lung disease (PF-ILD) experience every day. PF-ILD is a rare condition and a subset of interstitial lung disease (ILD). A patient develops a ‘progressive fibrosing phenotype’,2 leading to irreversible3,4,5 and progressive damage2,3,4 to both lung structure and function3,4,5,6,7. This means the lungs become progressively scarred, thickened and stiff. Symptoms can include shortness of breath, chronic cough, tiredness and gradual, unintended weight loss.8,9,10
No time to waste
Physicians estimate that patients experience significant delays in the diagnosis of ILD and the detection of progressive fibrosis.11 Progressive fibrosing ILDs have a significant impact on patients, including worsening lung function and lessening quality of life.12
“PF-ILD patients do not have time to waste in ensuring that they have proper access to care in Canada,” says Sharon Lee, executive director of the Canadian Pulmonary Fibrosis Foundation. “Delays in diagnosis leads to delays in starting the care they need, and the impacts of this is devastating to these individuals and their families.”
Recently, the Institute for Health Economics (IHE) conducted a roundtable on PF-ILD, and the policy recommendations from the diverse stakeholder group provided some guidance in areas to focus on. It was clear from those discussions that management of PF-ILD requires improvement. The condition requires access to a range of services and navigation through the system is challenging, particularly for those with less independence or functional loss from the disease.13
Enhancing patient care
Key actions identified at the roundtable included: increased surveillance for early identification of those needing services; accelerated implementation of multi-disciplinary guidance; investment in patient navigators to support patients and families; and implementing hub-and-spoke models of care (with centralized hubs linked to well-trained primary care teams).14
PF-ILD patients do not have time to waste in ensuring that they have proper access to care in Canada,” says Sharon Lee, executive director of the Canadian Pulmonary Fibrosis Foundation.
Several other initiatives are underway, uniting healthcare professionals, researchers, pharmaceutical companies, and patient organizations to collaborate on ways to enhance care for PF-ILD patients, such as PATHWAYS - Indigenous Health Collaborations, and in organizations such as members from the Canadian consortium MEDTEQ+ in Quebec and the University Hospital Foundation in Alberta.
“Progressive fibrosing ILDs have a significant impact on patients, including worsening lung function and lessening quality of life,” says Dr. Martin Kolb, a respirologist at St. Joseph’s Healthcare Hamilton and director of Respirology at McMaster University. “Enhancing care for patients will help improve outcomes in ILD patients across the spectrum.”
To learn more about interstitial lung diseases, visit the Canadian Pulmonary Fibrosis Foundation website at www.cpff.ca.
1 Mayo Clinic. Pneumonitis. https://www.mayoclinic.org/diseases-conditions/pneumonitis/symptoms-causes/syc-20352623
2 Byrne A, Maher T, Lloyd C. Pulmonary Macrophages: A new therapeutic pathway in fibrosing lung disease? Trends Mol Med 2016;22:303–316.
3 Leach H, et al. Endothelial cells recruit macrophages and contribute to a fibrotic milieu in bleomycin lung injury. Am J Respir Cell Mol Biol 2013;49(6):1093–1101.
4 Alunno A, et al. Clinical, epidemiological, and histopathological features of respiratory involvement in rheumatoid arthritis. Biomed Res Int 2017;7915340.
5 Wynn T. Integrating mechanisms of pulmonary fibrosis. J Exp Med 2011;208:1339–1350.
6 Murtha L, et al. The processes and mechanisms of cardiac and pulmonary fibrosis. Front Pysiol 2017;8:777.
7 Zeisberg M, Kalluri R. Cellular mechanisms of tissue fibrosis. 1. Common and organ-specific mechanisms associated with tissue fibrosis. Am J Physiol Cell Physiol 2013;304:C216–C225.
8 Canadian Pulmonary Fibrosis Foundation. Idiopathic Pulmonary Fibrosis Patient Information Guide. Available at: https://cpff.ca/wp-content/uploads/2016/05/IPF_Guide_2012_EN_Rev_Feb_2106_ForWeb.pdf [Accessed January 2021].
9 British Lung Foundation. What are the symptoms of pulmonary fibrosis? Available at https://www.blf.org.uk/support-for-you/pulmonary-fibrosis/symptoms [Accessed September 2019].
10 Holtze C, Flaherty K, Kreuter M, et al. Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150):180078.
11 Wijsenbeek M, Kreuter M, Olson A, et al. Progressive fibrosing interstitial lung diseases: current practice in diagnosis and management. Curr Med Res Opin. 2019;35(11):2015-2024. doi:10.1080/03007995.2019.1647040
12 Cottin V, Wollin L, Fischer A, et al. Fibrosing interstitial lung diseases: knowns and unknowns. Eur Respir Rev 2019; 28: 180100 [https://doi.org/10.1183/1600617.0100-2018].
13 Husereau D, Sproule J. Optimizing care for patients with Progressive Fibrosing Interstitial Lung Disease (PF-ILD). The Institute for Health Economics. 2021. Available at: https://www.ihe.ca/advanced-search/optimizing-care-for-patients-with-progressive-fibrosing-interstitial-lung-disease-pf-ild-
14 Husereau D, Sproule J. Optimizing care for patients with Progressive Fibrosing Interstitial Lung Disease (PF-ILD). The Institute for Health Economics. 2021. Available at: https://www.ihe.ca/advanced-search/optimizing-care-for-patients-with-progressive-fibrosing-interstitial-lung-disease-pf-ild-
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