Clinical Trial: Chest Physiotherapy and Lung Function in Primary Ciliary Dyskinesia
Study Status: Not yet recruiting
Recruit Status: Unknown status
Study Type: Interventional
Official Title: The Influence of Chest Physiotherapy on Lung Function Parameters in Primary Ciliary Dyskinesia
Brief Summary: Primary ciliary dyskinesia (PCD) is a rare disease, caused by impairment of the motile cilia. Patients present with chronic upper and lower respiratory tract infections. The therapy is mainly supportive and based on that of cystic fibrosis. Chest physiotherapy is one of the cornerstones of the therapy, however the influence of chest physiotherapy on lung function (short term and long term) is not clear. For interpretation of longitudinal lung function data it is important to examine the short time effect of chest physiotherapy. We hypothesize that a session of chest physiotherapy improves lung function and that thus lung function tests must be performed in a standardized way.
Detailed Summary:
Primary ciliary dyskinesia (PCD) is a rare disease, caused by congenital dysfunction of the motile cilia, located in the upper and lower respiratory tract, in the reproductive system and in the embryonal node. Ineffective ciliary beating results in disturbed mucociliary clearance, which is an important defense mechanism in the respiratory tract. It causes recurrent and chronic upper and lower respiratory tract infections, leading to reversible (mucus plugging) and irreversible lung damage (bronchiectasis, atelectasis, mucus plugging). Just like cystic fibrosis, it is characterized by obstructive lung disease, measured by spirometry.
Using Multiple Breath Washout measurements, it has been shown that peripheral airways disease is present in PCD. Probably, MBW parameters are already abnormal before forced expiratory volume in one second is abnormal.
The therapy for patients with PCD is mainly supportive: regular oral or intravenous antibiotics to treat airway infections and chest physiotherapy to actively increase mucociliary clearance. Chest physiotherapy has the objective to clear mucus from the lungs. Guidelines support the treatment of patients with PCD with chest physiotherapy. It is known that in patients with PCD, exercise has a more bronchodilating effect than the administration of salbutamol. However, no objective data describe the acute effect of chest physiotherapy on spirometry and MBW parameters. Moreover, short term effects of intervention on spirometry and MBW parameters can influence the interpretation of long-term evolution of these parameters.
In cystic fibrosis, one study has assessed the short-term influence of chest physiotherapy on lung function, measured by spirometry and MBW before and after chest physiotherapy. The authors found no significant influenc
Sponsor: Universitaire Ziekenhuizen Leuven
Current Primary Outcome: Difference in FEV1 before and after treatment [ Time Frame: 30 minutes ]
Original Primary Outcome: Same as current
Current Secondary Outcome: Difference in LCI before and after chest physiotherapy [ Time Frame: 30 minutes ]
Original Secondary Outcome: Difference in LCI befor and after chest physiotherapy [ Time Frame: 30 minutes ]
Information By: Universitaire Ziekenhuizen Leuven
Dates:
Date Received: August 22, 2013
Date Started: October 2013
Date Completion: October 2014
Last Updated: August 27, 2013
Last Verified: August 2013
