Cough Efficiency in Cystic Fibrosis

Cystic Fibrosis Clinical Trial

Official title:

Cough Efficiency in Cystic Fibrosis

Clinical Trial Details — Status: Completed

Administrative data

• NCT number: NCT01636219
• Other study ID #: SHEBA-11-8709-OE-SMC
• Status: Completed
• Phase: N/A
• First received: July 5, 2012
• Last updated: February 6, 2014
• Start date: September 2012
• Est. completion date: August 2013

Study information

• Verified date: February 2014
• Source: Sheba Medical Center
• Contact: n/a
• Is FDA regulated: No
• Health authority: Israel: Ministry of Health
• Study type: Observational

Clinical Trial Summary

The major causes of morbidity and mortality in Cystic Fibrosis (CF) are linked to the process of chronic inflammatory of the airway, leading to the progressive damage of the small bronchioles and subsequently to the proximal bronchi. A connection between weaknesses of respiratory muscles in CF and deficits of CFTR in the muscle has been established. Insufficient cough in CF patients may advance re-current respiratory infections. A voluntary cough flow volume (C-FVC) profile incorporates the characteristics of the forced expiratory flow volume curve (FE-VC). The study aims to explore the correspondence of voluntary cough-flow-volume and maximum expiratory flow-volume maneuvers in relation to disease complications in CF patients.

Description:

Cystic fibrosis (CF) is the most common lethal life shortening genetic disease caused by mutations of the trans-membrane conductance regulator (CFTR) gene. The major causes of morbidity and mortality in CF are linked to the process of chronic inflammatory of the airway, leading to the progressive damage of the small bronchioles and subsequently to the proximal bronchi. Cough is a back-up mechanism for mucus clearance which comes into effect in health during emergency situations, such as following the inhalation of a foreign body, and in lung disease where often the primary host defense clearance mechanism, namely mucociliany clearance, is compromised

Several studies have showed a connection between weaknesses of respiratory muscles in CF and deficits of CFTR in the muscle; sustain infection of pseudomonas; lower than normal tension time index and low fat free mass [3-6]. Weakness of the respiratory muscle may insinuate insufficient cough in CF patients.

Effective cough is initiated in several mechanical stages: a) inhaling a variable amount of air, b) closure of the glottis, c) contraction of respiratory muscles, and d) forced expiration to residual volume [7-13] A voluntary cough flow volume (C-FVC) profile therefore, incorporates the characteristics of the forced expiratory flow volume curve (FEVC) in that the first "spike" represents the peak cough flow, and the volume exhaled by the cough corresponds with the vital capacity. The descending portion of the C-FVC including secondary spikes decrease in a linear fashion as lung volume goes down from total lung capacity to residual volume.10 similar to the FEVC flow decay. Any disturbance in either of the cough stages may impair its efficiency.

The aim of this study is to explore the information that can be gained on cough ability in a group of CF patients, by the performance of voluntary cough-flow-volume maneuver and in relation to the characteristics of a maximum expiratory flow-volume curve.

Study plan How does this advance the field? In this study we wish to evaluate for the first time the cough ability derived from the voluntary cough flow volume curve for detection of insufficient cough in patients with CF. We hope to show that the cough flow volume curve corresponds with changes in cough ability in these patients in relation to lung function deterioration.

What are the clinical implications? An objective following-up of cough ability deterioration may allow the opportunity to introduce special respiratory therapy for strengthening cough and ease secretion flow in these patients.

Recruitment information / eligibility

• Status: Completed
• Enrollment: 100
• Est. completion date: August 2013
• Est. primary completion date: June 2013
• Accepts healthy volunteers: No
• Gender: Both
• Age group: 8 Years to 50 Years

Eligibility

Inclusion Criteria:

• Cooperation with spirometry

Exclusion Criteria:

• exacerbation, patients younger than 8 years.

Study Design

Observational Model: Cohort, Time Perspective: Cross-Sectional

Related Conditions & MeSH terms

• Cystic Fibrosis
• Fibrosis

Locations

Country	Name	City	State
n/a

Sponsors (1)

Lead Sponsor	Collaborator
Sheba Medical Center

References & Publications (9)

Beardsmore CS, Wimpress SP, Thomson AH, Patel HR, Goodenough P, Simpson H. Maximum voluntary cough: an indication of airway function. Bull Eur Physiopathol Respir. 1987 Sep-Oct;23(5):465-72. — View Citation

Bianchi C, Baiardi P. Cough peak flows: standard values for children and adolescents. Am J Phys Med Rehabil. 2008 Jun;87(6):461-7. doi: 10.1097/PHM.0b013e318174e4c7. — View Citation

Divangahi M, Balghi H, Danialou G, Comtois AS, Demoule A, Ernest S, Haston C, Robert R, Hanrahan JW, Radzioch D, Petrof BJ. Lack of CFTR in skeletal muscle predisposes to muscle wasting and diaphragm muscle pump failure in cystic fibrosis mice. PLoS Genet. 2009 Jul;5(7):e1000586. doi: 10.1371/journal.pgen.1000586. Epub 2009 Jul 31. — View Citation

Divangahi M, Matecki S, Dudley RW, Tuck SA, Bao W, Radzioch D, Comtois AS, Petrof BJ. Preferential diaphragmatic weakness during sustained Pseudomonas aeruginosa lung infection. Am J Respir Crit Care Med. 2004 Mar 15;169(6):679-86. Epub 2003 Dec 11. — View Citation

Enright S, Chatham K, Ionescu AA, Unnithan VB, Shale DJ. The influence of body composition on respiratory muscle, lung function and diaphragm thickness in adults with cystic fibrosis. J Cyst Fibros. 2007 Nov 30;6(6):384-90. Epub 2007 Apr 2. — View Citation

Hayot M, Guillaumont S, Ramonatxo M, Voisin M, Préfaut C. Determinants of the tension-time index of inspiratory muscles in children with cystic fibrosis. Pediatr Pulmonol. 1997 May;23(5):336-43. — View Citation

Langlands J. The dynamics of cough in health and in chronic bronchitis. Thorax. 1967 Jan;22(1):88-96. — View Citation

McCool FD, Leith DE. Pathophysiology of cough. Clin Chest Med. 1987 Jun;8(2):189-95. — View Citation

Pedersen OF, Lyager S, Ingram RH Jr. Airway dynamics in transition between peak and maximal expiratory flow. J Appl Physiol (1985). 1985 Dec;59(6):1733-46. — View Citation

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Cough flow volume indices compaired to healthy known data	The indices includs peak cough flow, Inspiratory capacity, number of cough spikes and cough vital capacity.	2 years	No
Secondary	forced vital capacity menuver	the cough flow volume curve is comapred to the forced vital capacity maneuver.	2 years	No