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Case Study 1 A 22-month-old male with spinal muscular atrophy (SMA) type 1 presented to the pulmonary clinic in moderate to severe respiratory distress. His parent stated that he typically uses bilevel noninvasive ventilation (NIV) at night and during naps only. However, for the past 2 days he had “needed his BiPAP 24/7 and is unable to come off of it” due to respiratory distress. He was also receiving albuterol via small volume nebulizer and CPT every 4 hours. The child was admitted directly to the pediatric intensive care unit. Upon admission, a chest radiograph was ordered that revealed right upper lobe atelectasis and a right middle lobe opacification. The left lung was clear. It also showed a bell-shaped configuration of the thorax and ribs, which is typical for SMA 1. The inspiratory and expiratory pressures and breath rate for the NIV were increased. IPV was started with albuterol and hypertonic saline every 4 hours in addition to cough assist every 2 hours for airway secretion clearance. After 3 days, the child’s X-ray showed mild improvement, but he still had atelectasis on the right. He continued to receive albuterol and cough assist treatments every 2 hours while alternating IPV and CPT every 4 hours. After 3 more days, the chest X-ray showed improvement in the atelectasis; he was able to be weaned from the increased NIV settings, and twice a day sprints were started. When he tolerated NIV settings close to his home regimen, albuterol and cough assist treatments were reduced to every 4 hours, and IPV and hypertonic saline were discontinued. On day 12 the patient was discharged home. 1. Why is IPV indicated for this child? 2. Why were the NIV settings increased? 3. Why do most children with SMA need cough assistance therapy as part of their daily airway clearance regimen? CHAPTER 25CASE STUDIES Case Study 2This patient is a 13-year-old, Caucasian, 8th-grade female with cystic fibrosis (CF). She was diagnosed with CF at 2 months of age, after newborn screening and genetic testing results confirmed the mutation. She has mild to moderate severity of disease, with a forced ex-piratory volume in 1 second (FEV1) at 85% of predicted. She has been hospitalized 5 times in the last 10 years for pulmonary infections with Pseudomonas aeruginosa. She has not tested positive for methicillin-resistant Staphylococcus aureus or Burkholderia cepacia. She has missed approximately 6 school days this year due to CF. Her daily routine on school days includes waking up at 6 a.m., first taking her nebulized medications, which include a bronchodilator and a mucolytic agent. This is followed by her vest therapy, which takes ap-proximately 45 minutes to adequately percuss every lung segment. This is usually interrupted by some coughing episodes, which may be prolonged, and she ends with a huff maneuver. The morning routine continues with eating breakfast and taking multiple medications that include a prophylactic antibiotic, nonsteroidal anti-inflammatory drug (NSAID), antihistamine agent, pancreatic enzymes, and vitamins. After school, her mother performs manual CPT with postural drainage. This therapy takes 45 minutes to 1 hour, during which time she may have several coughing episodes as well. She then relaxes and spends time with her family and friends. Before eating dinner she takes more medications, including NSAID, antibiotic, and pancreatic enzymes. Before bedtime she takes another nebulized bronchodilator treatment and follows that with the vest therapy for another 45 minutes while watching television. 1. What is vest therapy? 2. Is there an alternative airway clearance device that could be used when the vest is not available? 3. What does postural drainage achieve for patients with cystic

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