Case study: Use of the high frequency chest wall oscillation (HFCWO) to support airway clearance in a child with SMA 1 (spinal muscular atrophy)

Abstract

Introduction/Aim: SMA 1 is a neuromuscular disease(NMD) that leads to significant respiratory failure in infancy. A 5yr old girl with SMA 1 was admitted to PICU with probable aspiration having progressed from overnight nasal non-invasive ventilation 16/4 to continuous via full facemask 18/4. She had right middle/lower lobe collapse/consolidation, an oxygen requirement and showed minimal improvement despite antibiotics and intensive physiotherapy 6 x day. The aim of physiotherapy was to find a child and family centred intervention that was safe, effective yet sustainable. Method: Physiotherapy included manual techniques, assisted cough with cough assist device (Nippy Clearway) and suction. Day 5 she rapidly declined and oxygen demand escalated from Fi02 0.5 to 1.0, heart rate(HR) 160, respiratory rate(RR) 60, Sp02 >88%, PcC02 34mmHg. A decision was made to trial HFCWO (The Vest®) in an attempt to avoid intubation. HFCWO uses an inflatable jacket that compresses the chest wall producing oscillations to facilitate secretion movement proximally. It was also postulated it could improve expiratory flow rates by mimicking chest wall strapping. To prevent airway collapse during HFCWO, IPAP was increased during treatment and combined with cough assist device and suction. Results: The treatment with HFCWO was well tolerated with a reduction in reported treatment associated pain, significant section clearance demonstrated, including a large mucus plug. Following treatment Fi02 reduced to 0.4, HR 140 and RR 28. Over the following days and right sided CXR changes resolved and NIV weaned to home setting in air prior to discharge to Post Acute Care Physiotherapy service. Conclusion: Due to the successful outcomes with The Vest® the family have continued to use it as part of her physiotherapy management at home. The patient has had no unplanned admissions to hospital in the year since discharge home with a Respiratory Physio Action plan including HFCWO.L6269401362019-04-02 <br />