Inspiratory muscle strength relative to disease severity in adults with stable cystic fibrosis

Robert L Dekerlegand; Denis Hadjiliadis; Anne K Swisher; J Scott Parrott; Albert J Heuer; Mary Jane Myslinski

doi:10.1016/j.jcf.2015.05.003

Inspiratory muscle strength relative to disease severity in adults with stable cystic fibrosis

J Cyst Fibros. 2015 Sep;14(5):639-45. doi: 10.1016/j.jcf.2015.05.003. Epub 2015 May 23.

Authors

Robert L Dekerlegand¹, Denis Hadjiliadis², Anne K Swisher³, J Scott Parrott⁴, Albert J Heuer⁵, Mary Jane Myslinski⁶

Affiliations

¹ Rutgers, The State University of New Jersey, School of Health Related Professions, Doctor of Physical Therapy Program - South, 40 East Laurel Road, Stratford, NJ 08084, USA. Electronic address: dekerlro@shrp.rutgers.edu.
² University of Pennsylvania, Adult Cystic Fibrosis Program, Perelman Center for Advanced Medicine, 3400 Civic Center Blvd, 1st Floor West, Philadelphia, PA 19104, USA. Electronic address: denis.hadjiliadis@uphs.upenn.edu.
³ West Virginia University, Division of Physical Therapy, Room 8314, PO Box 9226, Morgantown, WV 26506, USA. Electronic address: aswisher@hsc.wvu.edu.
⁴ Rutgers, The State University of New Jersey, School of Health Related Professions, Interdisciplinary Studies, 65 Bergen Street, Rm. 353A, Newark, NJ 07101, USA; Rutgers, The State University of New Jersey, School of Public Health, Quantitative Methods, 65 Bergen Street, Rm. 353A, Newark, NJ 07101, USA. Electronic address: parrotja@shrp.rutgers.edu.
⁵ Rutgers, The State University of New Jersey, School of Health Related Professions, Respiratory Therapy, 65 Bergen Street, Rm. 358, Newark, NJ 07101, USA. Electronic address: heueraj@shrp.rutgers.edu.
⁶ Rutgers, The State University of New Jersey, School of Health Related Professions, Doctor of Physical Therapy Program - North, 65 Bergen Street, Rm. 721, Newark, NJ 07101, USA. Electronic address: myslinsk@shrp.rutgers.edu.

PMID: 26005006
DOI: 10.1016/j.jcf.2015.05.003

Abstract

Background: Due to heterogeneity in pulmonary disease, current literature may misrepresent inspiratory muscle involvement in cystic fibrosis (CF). This study investigated inspiratory muscle strength (IMS) relative to disease severity in adults with CF.

Methods: Maximal inspiratory pressure (MIP) was assessed in 58 adults with stable CF grouped by disease severity (20 mild, 20 moderate, 18 severe) and compared to 20 controls. Relationships between MIP, lung function, dyspnea and anthropometrics were evaluated using multivariable linear models.

Results: MIP in cmH2O and %-predicted was decreased in advanced CF lung disease as compared to mild disease and healthy controls (p<0.05). Disease severity accounted for 24% of the variance in IMS after controlling for confounding variables (p<0.001).

Conclusions: IMS is decreased in some adults with stable CF with moderate and severe pulmonary disease, and is related to dyspnea. Future studies should determine if decreased IMS contributes inefficient breathing patterns, respiratory pump dysfunction, and/or exercise intolerance in advanced CF.

Keywords: Adult; Cystic fibrosis; Inspiratory muscle strength; Maximal inspiratory pressure; Respiratory muscle.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Adult
Cross-Sectional Studies
Cystic Fibrosis / diagnosis
Cystic Fibrosis / physiopathology*
Female
Forced Expiratory Flow Rates
Humans
Inhalation / physiology*
Male
Middle Aged
Muscle Strength / physiology*
Prognosis
Respiratory Function Tests
Respiratory Muscles / physiopathology*
Severity of Illness Index
Vital Capacity
Young Adult