Neuromuscular respiratory failure and airway clearance — formative SAQs

SAQ 1 — The boy with Duchenne muscular dystrophy who sleeps badly (10 marks, 15 minutes)

A 13-year-old boy with Duchenne muscular dystrophy, now using a wheelchair full time, is brought to clinic with morning headaches that ease through the day, daytime sleepiness and falling school performance. He has had three chest infections this winter and his parents say his cough sounds weak. His chest is clear today. [1]

a) Explain the two separate ways neuromuscular weakness causes respiratory failure, and match each of this boy's problems to one of them. (4 marks) [1] [6]

b) What bedside and sleep investigations would you use to confirm each problem, and what is the key measurement for detecting nocturnal hypoventilation? (3 marks) [1] [7]

c) Outline the two parallel treatments this boy needs and why oxygen alone is not the answer. (3 marks) [2] [6]

SAQ 2 — The infant with spinal muscular atrophy and a cold (10 marks, 15 minutes)

A 9-month-old infant with spinal muscular atrophy type 1 presents with two days of coryza, increased work of breathing and a weak cough. She is tachypnoeic with reduced air entry at the right base and looks tired. Her parents already have a cough assist machine at home. [4]

a) Why is a minor viral illness a respiratory emergency in this infant, and what is the governing rule of acute management? (3 marks) [1] [4]

b) Describe your immediate management of secretions and ventilation, and the role and risk of supplemental oxygen. (4 marks) [1] [6]

c) Outline the anticipatory home plan that should be in place to reduce the impact of the next infection. (3 marks) [4] [6]