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Trial record 8 of 21 for:    primary ciliary dyskinesia

Chest Physiotherapy and Lung Function in Primary Ciliary Dyskinesia

This study is not yet open for participant recruitment. (see Contacts and Locations)
Verified August 2013 by Universitaire Ziekenhuizen Leuven
Sponsor:
Information provided by (Responsible Party):
Universitaire Ziekenhuizen Leuven
ClinicalTrials.gov Identifier:
NCT01929356
First received: August 22, 2013
Last updated: August 27, 2013
Last verified: August 2013
  Purpose

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.


Condition Intervention
Primary Ciliary Dyskinesia
Procedure: Chest physiotherapy

Study Type: Interventional
Study Design: Endpoint Classification: Efficacy Study
Intervention Model: Single Group Assignment
Masking: Open Label
Primary Purpose: Treatment
Official Title: The Influence of Chest Physiotherapy on Lung Function Parameters in Primary Ciliary Dyskinesia

Resource links provided by NLM:


Further study details as provided by Universitaire Ziekenhuizen Leuven:

Primary Outcome Measures:
  • Difference in FEV1 before and after treatment [ Time Frame: 30 minutes ] [ Designated as safety issue: No ]
    Lung function will be performed before a session of chest physiotherapy, and repeated 30 minutes after a chest physiotherapy session of 20 minutes with airway clearance techniques and use of PEP mask


Secondary Outcome Measures:
  • Difference in LCI before and after chest physiotherapy [ Time Frame: 30 minutes ] [ Designated as safety issue: No ]
    Multiple Breath Washout (MBW) will be performed before and 30 min after a session of chest physiotherapy. The difference in LCI (lung clearance index) will be used as secondary outcome.


Estimated Enrollment: 20
Study Start Date: October 2013
Estimated Study Completion Date: October 2014
Estimated Primary Completion Date: August 2014 (Final data collection date for primary outcome measure)
Arms Assigned Interventions
Experimental: chest physiotherapy
session of 20 minutes chest physiotherapy with physiotherapist, use of airway clearance techniques, PEP (positive expiratory pressure) device
Procedure: Chest physiotherapy
20 minutes of chest physiotherapy by physiotherapist

Detailed Description:

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 influence of chest physiotherapy on spirometry parameters, nor on MBW parameters. Therefore, longitudinal interpretation can be performed irrespective of the timing of the lung function measurements. However, cystic fibrosis and PCD have a different pathophysiological mechanism with a more 'mechanical' disturbance of the mucociliary clearance in PCD, compared to a more 'viscous' disturbance of mucociliary clearance in CF. Therefore, the results in CF are not applicable to PCD. Even more, we hypothesize that chest physiotherapy will have a significant effect on lung function parameters (spirometry and MBW) in patients with PCD due to its external mechanical effect on mucus clearance.

  Eligibility

Ages Eligible for Study:   6 Years to 50 Years
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Criteria

Inclusion Criteria:

  • Primary ciliary dyskinesia
  • able to perform spirometry and MBW (>6 years)

Exclusion Criteria:

  • acute exacerbation
  Contacts and Locations
Choosing to participate in a study is an important personal decision. Talk with your doctor and family members or friends about deciding to join a study. To learn more about this study, you or your doctor may contact the study research staff using the Contacts provided below. For general information, see Learn About Clinical Studies.

Please refer to this study by its ClinicalTrials.gov identifier: NCT01929356

Contacts
Contact: Mieke Boon, MD +3216342589 mieke.boon@uzleuven.be

Locations
Belgium
University Hospital Gasthuisberg Leuven Not yet recruiting
Leuven, Belgium, 3000
Contact: Mieke Boon, MD    +3216342589    mieke.boon@uzleuven.be   
Contact: Christiane De Boeck, MD, Phd    +3216343820    christiane.deboeck@uzleuven.Be   
Principal Investigator: Mieke Boon, MD         
Sponsors and Collaborators
Universitaire Ziekenhuizen Leuven
Investigators
Principal Investigator: Mieke Boon, MD research fellow
  More Information

No publications provided

Responsible Party: Universitaire Ziekenhuizen Leuven
ClinicalTrials.gov Identifier: NCT01929356     History of Changes
Other Study ID Numbers: UZLMB001
Study First Received: August 22, 2013
Last Updated: August 27, 2013
Health Authority: Belgium: Federeal Agency for drugs and health care products

Keywords provided by Universitaire Ziekenhuizen Leuven:
lung function tests
chest physiotherapy

Additional relevant MeSH terms:
Ciliary Motility Disorders
Dyskinesias
Kartagener Syndrome
Bronchial Diseases
Bronchiectasis
Cardiovascular Abnormalities
Cardiovascular Diseases
Central Nervous System Diseases
Congenital Abnormalities
Dextrocardia
Genetic Diseases, Inborn
Heart Defects, Congenital
Heart Diseases
Movement Disorders
Nervous System Diseases
Neurologic Manifestations
Otorhinolaryngologic Diseases
Respiratory System Abnormalities
Respiratory Tract Diseases
Signs and Symptoms
Situs Inversus

ClinicalTrials.gov processed this record on November 27, 2014