From the Research
For a patient with Primary Ciliary Dyskinesia (PCD) on a ventilator requiring endotracheal suctioning every 3 hours, I recommend a comprehensive nebulizer and chest physiotherapy schedule coordinated with the suctioning routine. The patient should receive nebulized hypertonic saline 7% (6-7 mL) followed by nebulized bronchodilator such as albuterol 2.5 mg every 6 hours, with the first daily treatment administered early morning, as supported by the most recent study on the management of PCD 1. Dornase alfa (Pulmozyme) 2.5 mg should be given once daily to help break down DNA in thick secretions. Chest physiotherapy should be performed 30 minutes after each nebulizer treatment, including postural drainage, percussion, and vibration for 20-30 minutes per session, focusing on all lung segments but emphasizing areas with greatest secretion burden as identified on assessment. Key aspects of the treatment include:
- Regular airway clearance
- Routine microbiological surveillance
- Antibiotic treatment for pulmonary exacerbation
- Health vaccinations, as outlined in treatment recommendations for PCD 2. Endotracheal suctioning should follow each chest physiotherapy session, with additional suctioning as needed based on the established 3-hour schedule. This regimen addresses the fundamental issue in PCD—impaired mucociliary clearance—by thinning secretions with hypertonic saline, improving bronchodilation with albuterol, breaking down DNA in secretions with Dornase alfa, and using mechanical techniques to mobilize secretions from peripheral to central airways for removal via suctioning. Although a recent study found little evidence to support the hypothesis that hypertonic saline improves quality of life in PCD patients 3, the use of hypertonic saline is still recommended as part of a comprehensive treatment plan, given its potential benefits in reducing airway mucous viscosity. The schedule should be adjusted based on the patient's response, with increased frequency during respiratory infections and continuous reassessment of effectiveness through monitoring oxygen saturation, ventilator parameters, and secretion characteristics, in line with the consensus recommendations for the diagnosis, monitoring, and treatment of PCD 4.