Teresa Pitts1, Rachel Bordelon2, Alyssa Huff3, Barry J Byrne4, Barbara K Smith5. 1. Kentucky Spinal Cord Injury Research Center, University of Louisville, Louisville, KY, USA. 2. Department of Osteopathic Medicine, A.T. Still University, Kirksville, MO, USA. 3. Department of Physiology and Biophysics, University of Louisville, Louisville, KY, USA. 4. Department of Pediatrics, University of Florida, Gainesville, FL, USA. 5. Departments of Physical Therapy and Pediatrics, University of Florida, P.O. Box 100154, Gainesville, FL, 32610-0154, USA. bksmith@phhp.ufl.edu.
Abstract
PURPOSE: While factors leading to hypoventilation have been well studied in Pompe disease, cough effectiveness and airway clearance practices are less understood. We aimed to identify significant factors that influence peak cough flow (PCF) in Pompe, and to detect whether pulmonary hygiene practices were reflective of reduced PCF. METHODS: This is a prospective observational study of 20 subjects with Pompe disease (infantile-onset: 7, juvenile-onset: 6, adult-onset: 14). Subjects performed spirometry, maximal respiratory pressures, and cough (voluntary: n = 24, spontaneous: n = 3). Subjects or their parents reported airway clearance and secretion management practices. Relationships between disease variables, pulmonary function, and cough parameters as well as group differences in cough parameters were evaluated. RESULTS: Subjects with infantile-onset disease had significantly lower PCF (p < 0.05) and tended to require more external ventilatory support (p = 0.07). In juvenile- and adult-onset disease, PCF differed according to external ventilatory requirement [daytime: 83.6 L/min (95% CI 41.2-126.0); nighttime: 224.6 L/min (95% CI 139.1-310.2); none: 340.2 L/min (95% CI 193.3-487.6), p < 0.005]. Cough inspiratory volume also differed significantly by ventilatory requirement [daytime: 5.5 mL/kg (95% CI 3.0-8.0); nighttime: 16.0 mL/kg (95% CI 11.8-20.2); none: 26.8 mL/kg (95% CI 11.9-41.7), p < 0.001]. However, routine airway clearance or secretion management practices were only consistently reported among patients with infantile-onset disease (infantile: 86%, juvenile: 0%, adult: 14%, p < 0.005). CONCLUSIONS: Cough weakness was detected in the majority of patients with Pompe disease and was influenced by both inspiratory and expiratory muscle function. Patients at risk for problems or with ineffective PCF should be urged to complete routine pulmonary hygiene.
PURPOSE: While factors leading to hypoventilation have been well studied in Pompe disease, cough effectiveness and airway clearance practices are less understood. We aimed to identify significant factors that influence peak cough flow (PCF) in Pompe, and to detect whether pulmonary hygiene practices were reflective of reduced PCF. METHODS: This is a prospective observational study of 20 subjects with Pompe disease (infantile-onset: 7, juvenile-onset: 6, adult-onset: 14). Subjects performed spirometry, maximal respiratory pressures, and cough (voluntary: n = 24, spontaneous: n = 3). Subjects or their parents reported airway clearance and secretion management practices. Relationships between disease variables, pulmonary function, and cough parameters as well as group differences in cough parameters were evaluated. RESULTS: Subjects with infantile-onset disease had significantly lower PCF (p < 0.05) and tended to require more external ventilatory support (p = 0.07). In juvenile- and adult-onset disease, PCF differed according to external ventilatory requirement [daytime: 83.6 L/min (95% CI 41.2-126.0); nighttime: 224.6 L/min (95% CI 139.1-310.2); none: 340.2 L/min (95% CI 193.3-487.6), p < 0.005]. Cough inspiratory volume also differed significantly by ventilatory requirement [daytime: 5.5 mL/kg (95% CI 3.0-8.0); nighttime: 16.0 mL/kg (95% CI 11.8-20.2); none: 26.8 mL/kg (95% CI 11.9-41.7), p < 0.001]. However, routine airway clearance or secretion management practices were only consistently reported among patients with infantile-onset disease (infantile: 86%, juvenile: 0%, adult: 14%, p < 0.005). CONCLUSIONS:Cough weakness was detected in the majority of patients with Pompe disease and was influenced by both inspiratory and expiratory muscle function. Patients at risk for problems or with ineffective PCF should be urged to complete routine pulmonary hygiene.
Authors: Antonio Sarmento; Armèle F Dornelas de Andrade; Íllia Nadinne Df Lima; Andrea Aliverti; Guilherme Augusto de Freitas Fregonezi; Vanessa R Resqueti Journal: Respir Care Date: 2017-02-14 Impact factor: 2.258
Authors: Barbara K Smith; Manuela Corti; A Daniel Martin; David D Fuller; Barry J Byrne Journal: Respir Physiol Neurobiol Date: 2015-11-28 Impact factor: 1.931
Authors: Matthias Boentert; Hélène Prigent; Katalin Várdi; Harrison N Jones; Uwe Mellies; Anita K Simonds; Stephan Wenninger; Emilia Barrot Cortés; Marco Confalonieri Journal: Int J Mol Sci Date: 2016-10-17 Impact factor: 5.923
Authors: Harrison N Jones; Maragatha Kuchibhatla; Kelly D Crisp; Lisa D Hobson-Webb; Laura Case; Milisa T Batten; Jill A Marcus; Richard M Kravitz; Priya S Kishnani Journal: Neuromuscul Disord Date: 2020-09-28 Impact factor: 4.296
Authors: Lauren Tabor Gray; Kasey L McElheny; Terrie Vasilopoulos; James Wymer; Barbara K Smith; Emily K Plowman Journal: Dysphagia Date: 2022-08-05 Impact factor: 2.733