Use of non-invasive ventilation in cerebral palsy

Patients with cerebral palsy (CP), especially those at the severe end of the spectrum (Gross Motor Function Classification System levels IV-V equivalent), frequently suffer from sleep disturbance and sleep-disordered breathing (SDB). Non-invasive ventilation (NIV) is increasingly used in this patient group, albeit with little published evidence of its effectiveness in CP. This article aims to review the current evidence in the use of NIV in children with CP, highlighting areas of uncertainties, as well as the balance of potential risks, challenges and benefits. We would also share our experience and practical considerations in using NIV to manage SDB in this group of patients. Between January 2010 and December 2016, 21 patients (median age 11.1 (range 1.7-16.1) years) with CP were initiated on NIV at Great Ormond Street Hospital for Children following diagnosis of moderate to severe SDB on cardiorespiratory polygraphy. Over half of our patients with CP (n=11) failed to establish on NIV either due to intolerance of mask and/or ventilation pressure at the initial trial in hospital, or poor adherence during follow-up, in contrast to the overall failure rate of 8.7% among total patient population commenced on NIV. Children with CP constitute a relatively small but challenging group of patients. Decision to undertake NIV should be individualised, based on benefit outweighing the risk and burden of the treatment.