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Physiotherapy in Primary Ciliary Dyskinesia—What Is the Evidence?: A Narrative Review and Personal Experience

Pediatric Pulmonology·August 16Open Access
Respiratory SystemLimited evidenceBronchiectasisPrimary Ciliary DyskinesiaNarrative ReviewAirway Clearance TherapyExercise InterventionMucolytic TherapyMixedDornase AlfaHypertonic Saline

Summary

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What was studied

This narrative review evaluates the evidence for physiotherapy—especially airway clearance therapy (ACT)—in managing Primary Ciliary Dyskinesia (PCD), covering techniques, mucolytic agents, exercise, and age-specific considerations across the lifespan.

Key findings

No ACT technique (PEP, ACBT, autogenic drainage, IPV, HFCWO, etc.) has shown clear superiority over others; evidence for mucolytics such as hypertonic saline or rhDNase is weak or controversial; exercise may improve ventilatory capacity and quality of life, but data are scarce.

Study limitations

Current recommendations rely heavily on expert opinion and extrapolation from cystic fibrosis and non-CF bronchiectasis, rather than PCD-specific RCT data; randomized trials are ethically difficult when ACT is already considered standard care; practice varies widely with no standardized international guidelines.

Clinical implications

Start ACT early in PCD, adapt the technique to the patient's developmental stage, and prioritize adherence strategies—but recognize that the choice of specific ACT method should be individualized, as no single approach is proven superior. Mucolytics should be used cautiously given limited supporting evidence in PCD.

Related Questions

Explore related topics

Which airway clearance techniques are most effective for children with primary ciliary dyskinesia?Is hypertonic saline beneficial in primary ciliary dyskinesia compared to cystic fibrosis?How does exercise therapy impact lung function and quality of life in primary ciliary dyskinesia?

Publication Details

Year
2026
Journal
Pediatric Pulmonology
Source
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