The effect of inspiratory muscle training in PCD and CF patients: A pilot study

BackgroundEffective work of breathing and bronchial hygiene requires synergy of inspiratory and expiratory muscles. Inspiratory muscle training (IMT) is a part of pulmonary rehabilitation in chronic obstructive pulmonary disease (COPD). There is some evidence of its efficacy in cystic fibrosis (CF) and, recently, in long COVID-19. We are not aware of studies on IMT in primary ciliary dyskinesia (PCD). Our aim was to assess the effect of IMT on respiratory muscle strength and pulmonary function in PCD and CF patients.MethodsA single center pilot study. Spirometry, lung clearance index (LCI), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP) were measured at baseline (visit 1), after a month of IMT with & REG;POWERbreathe (visit 2), and at follow-up (visit 3).ResultsThe cohort included 27 patients (19 PCD, 8 CF); mean age 18.4 & PLUSMN; 9.8 years. After a month of IMT, there was a significant increase in MIP and MIP% (6.19-7.44, p = .015; and 81.85%-100.41%, p = .046, respectively), which was sustained at visit 3. Compliance & GE;90% led to higher improvement in MIP. In sub-group analysis, improvement in MIP and MIP% remained significant for PCD patients (p = .026 and p = .049, respectively). No significant changes were found in spirometry, MEP or LCI.ConclusionsIMT was well-tolerated and led to improved inspiratory muscle strength in PCD patients. The clinical implication of improved MIP should be further investigated. Larger, long-term studies are needed to evaluate long-term effects of IMT on pulmonary function, respiratory muscle strength, pulmonary exacerbations, and quality of life.

Automated summary

This study aimed to assess the effect of inspiratory muscle training (IMT) on respiratory muscle strength and pulmonary function in primary ciliary dyskinesia (PCD) patients. The results showed that IMT significantly increased the maximal inspiratory pressure (MIP) in PCD patients, indicating improved respiratory muscle strength. However, larger, long-term studies are needed to evaluate the long-term effects of IMT on pulmonary function and respiratory muscle strength.