Expanding the Role for Non-Invasive Ventilation in Cystic Fibrosis (CF)

Funded Activity Summary

Many patients with Cystic Fibrosis (CF) have disturbed breathing during sleep and poor sleep quality. Our preliminary data shows the problems may be worse during flare ups of the disease, with patients having less dreaming (REM) sleep causing reduced daytime attention and concentration. This could have adverse consequences for exam performance, work ability and driving skills. Also, even with maximum treatment, flare ups can result in permanent loss of lung capacity and quality of life. We propose a novel approach to treat the flare ups with short-term portable assisted ventilation (nBVS) as well as standard treatment as we strongly believe that the portable ventilator will improve gas exchange with better matching of ventilation and blood flow and will hasten recovery. We believe that long term nBVS will be superior to oxygen therapy alone in delaying the onset of respiratory failure and improving survival. We have convincing evidence to suggest that nBVS improves ventilation perfusion distributions rapidly and that this benefit increases over 6 months of regular use. The likely explanation is that it keeps the airways open and reduces mucus plugging. In short, nBVS is a treatment in its own right for abnormal gas exchange in CF. The ideal time for nBVS is during sleep when it can be used for a long time period when patients are most at risk of falls in oxygen saturation. In summary, we believe that nBVS is likely to have a major impact in delaying the progession of disease and improving patient outcomes. Almost all patients with CF have evidence of sinus disease and many have nasal polyps. We have convincing questionnaire data showing an association between sinusitis, nasal obstruction and poor sleep quality. Our preliminary sleep study data in patients with active sinusitis confirm multiple arousals and sleep fragmentation. We plan to examine the impact of sinus surgery in improving sleep disordered breathing and quality of life in patients with CF.

Funded Activity Details

Start Date: 01-01-2003

End Date: 01-01-2005

Funding Scheme: NHMRC Project Grants

Funding Amount: $315,375.00

Funder: National Health and Medical Research Council

Research Topics

ANZSRC Field of Research (FoR)

Respiratory Diseases

ANZSRC Socio-Economic Objective (SEO)

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Other Keywords

Bilevel Ventilatory Support | Clinical Science | Cystic Fibrosis | Nasosinus Disease | Neurocognitive Assessment | Respiratory Medicine | Sleep Architecture Abnormalities and Sleep Fragmentation | Sleep Breathing Disorders | Ventilation Perfusion Inequality

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