Considerations for chest clearance and cough augmentation in severe bulbar dysfunction: a case study

This case study describes a 21-year-old male with congenital myotonic dystrophy referred to respiratory physiotherapy with a weak cough and upper respiratory tract secretions. Mechanical insufflation-exsufflation (MI-E) was prescribed. Post initiation, the patient described a worsening of secretions and increased attendances to hospital with suspected chest infection. He also described difficulties with speaking after use of MI-E. Multidisciplinary assessment of cough as well as bulbar and swallow function resulted in a primary diagnosis of oro-pharyngeal dysphagia as well as weak cough. An alternative prophylactic therapy programme including active cycle of breathing, chest wall percussions, and manually assisted cough, was prescribed to facilitate clearance of upper airway secretions and patient comfort. The case highlights some of the risks associated with cough augmentation techniques derived from single-discipline intervention in the neuromuscular patient population. Comprehensive multidisciplinary assessment and management were key to redefining this patient's diagnosis, allowing effective and individualised treatment.