Bench To Bedside: New Findings in Primary Ciliary Dyskinesia

By Joseph H. Sisson, MD; and Johnny L. Carson, PhD

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Treatment of PCD

The treatment of PCD, while largely symptomatic, is often delayed because of delayed diagnosis. Diagnosis of PCD is often delayed because respiratory infections are common in infants and because clinicians may not be familiar with the rare syndrome of PCD. For example, in a pediatric PCD referral center, the average age at diagnosis for PCD was 4.4 years despite evidence of neonatal distress in the majority of those individuals.⁶¹ As is true with all types of bronchiectasis, early diagnosis and aggressive treatment likely improves outcome in PCD patients.

The treatment of PCD is similar to the treatment of other forms of bronchiectasis, focusing largely on antibiotics and methods to enhance cough and sputum expectoration. Antibiotics clearly have a role in controlling bacterial colonization in PCD patients, who are typically colonized with Haemophilus influenzae, Pseudomonas, and coliforms. Although cyclic antibiotics are commonly used as prophylaxis for clinical exacerbations, there are no trials demonstrating an advantage for any specific antibiotic regimens. As with other individuals with bronchiectasis, the failure of a PCD patient to respond to empiric oral antibiotics should prompt the clinician to culture the sputum to exclude a multidrug-resistant or unusual pathogen. Although not proven in clinical trials, macrolide antibiotics may also have a role in the treatment of PCD independent of their antibiotic effects. Macrolides can stimulate ciliary motility in vitro^62,63 and, in one case report, appeared to improve clearance of secretions when given in combination with an inhaled b-agonist.⁶⁴ Because PCD patients tend to have some degree of airway obstruction during exacerbations, inhaled b-agonists, steroids, and theophylline have all been used as supportive treatments. One study suggests that when PCD patients are treated aggressively with daily chest physiotherapy, monthly spirometric monitoring, and sputum cultures with specific antibiotic treatment, they have stable lung function and fewer complications.⁶⁵ Exercise has also been used as a treatment for PCD patients and was found to increase peak flow rates better than treatment with inhaled b-agonists.⁶⁶ One explanation for this result is that exercise probably promoted vigorous coughing, and cough is the main way that airway secretions are cleared in individuals with PCD.

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