Cough Effectiveness and Pulmonary Hygiene Practices in Patients with Pompe Disease

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.

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Abbreviations

FVC:

Forced vital capacity

GAA:

Acid alpha-glucosidase

IQR:

Interquartile range

MIP:

Maximal inspiratory pressure

MEP:

Maximal expiratory pressure

MV:

Mechanical ventilation

PCF:

Peak cough flow

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Acknowledgements

Some data were collected as part of NCT02354651, supported by R21-HD090752 to BKS, through the National Institute of Child Health and Human Development.

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Correspondence to Barbara K. Smith.

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Pitts, T., Bordelon, R., Huff, A. et al. Cough Effectiveness and Pulmonary Hygiene Practices in Patients with Pompe Disease. Lung 197, 1–8 (2019). https://doi.org/10.1007/s00408-018-0171-1

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Keywords

  • Cough
  • Pulmonary hygiene
  • Pompe disease
  • Respiratory muscles