A 10-year-old girl with a known diagnosis of cystic fibrosis is admitted to the pediatric ward for an acute pulmonary exacerbation. She presents with an increased productive cough, decreased exercise tolerance, and a 12% drop in her forced expiratory volume in 1 second () from baseline. She is started on intravenous antipseudomonal antibiotics. The respiratory therapist is preparing her daily airway clearance regimen, which involves multiple inhaled medications and physical modalities.
Arrange the following therapies in the correct physiological sequence they should be administered during a single treatment session to maximize airway clearance and drug deposition.
- 1Administration of inhaled albuterol
- 2Administration of inhaled 7% hypertonic saline
- 3Administration of inhaled dornase alfa
- 4High-frequency chest wall oscillation
- 5Administration of inhaled tobramycin
Answer
The correct physiological sequence for cystic fibrosis airway clearance therapy is: (1) Inhaled albuterol, (2) Inhaled hypertonic saline, (3) Inhaled dornase alfa, (4) High-frequency chest wall oscillation, and (5) Inhaled tobramycin.
The sequence of cystic fibrosis pulmonary therapies is designed to sequentially prepare the airway, thin the secretions, mobilize the mucus, and finally treat the underlying infection. Bronchodilators (albuterol) must go first to maximize airway diameter and prevent bronchospasm. Next, mucolytics and hydrating agents (hypertonic saline and dornase alfa) are given to loosen and thin the characteristic inspissated secretions. With the mucus prepared, mechanical clearance (chest wall oscillation) physically mobilizes the sputum for expectoration. Finally, with the airways as clear as possible, inhaled antibiotics (tobramycin) are administered to achieve maximum penetration and deposition into the lung parenchyma.
Step-by-Step Solution
Key Concept
Cystic Fibrosis Airway Clearance Therapy Sequence