In this webinar, our mixed panel of health professionals and consumers provide expert insights into what Progressive Pulmonary Fibrosis (PPF) is, how to manage symptoms like cough and breathlessness, strategies to help improve quality of life, building up your exercise tolerance, understanding the benefits of palliative care, and more.
Interstitial lung diseases (ILDs) are chronic conditions typically causing inflammation or fibrosis in the lungs. In Australia and New Zealand, the most common ILD is Idiopathic Pulmonary Fibrosis (IPF), a condition where the cause is unknown. However, there are many other ILDs that lead to pulmonary fibrosis, such as hypersensitivity pneumonitis (e.g. bird fancier’s lung), connective tissue disease-related ILD, sarcoidosis, and pneumoconiosis (e.g. coal miners’ lung).
Pulmonary fibrosis involves the thickening, inflammation, and scarring of lung tissue, impairing oxygen transfer from the lungs to the bloodstream. This can be visualized through CT scans, which show changes in lung structure. Progressive Pulmonary Fibrosis (PPF) is a term used to describe ILDs that worsen over time, as evidenced by deteriorating CT scans, lung function tests, or symptoms.
PPF encompasses various ILDs, including sarcoidosis, scleroderma, and hypersensitivity pneumonitis. While some forms of pulmonary fibrosis remain stable, others progress, leading to worsening symptoms and lung function. IPF is distinct from PPF as it is invariably progressive and not caused by other conditions.
Patients with PPF often present with breathlessness, reduced exercise capacity, weight loss, cough, and low energy. Diagnosis involves lung function tests, CT scans, and other pathology tests. Treatment includes vaccinations, staying active, pulmonary rehabilitation, oxygen therapy, medications (e.g., antifibrotics like nintedanib), and peer support groups. Palliative care can also enhance quality of life for patients with advanced illness.
Future directions for PPF include precision diagnosis, gene-based markers, artificial intelligence in CT scan analysis, new medications, and support networks. Mobile applications are being developed to help patients track their lung fibrosis progress and provide support, especially in regional areas.
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