Long Term Non-Invasive Ventilation in Children: Impact on Survival and Transition to Adult Care

Background The number of children receiving domiciliary ventilatory support has grown over the last few decades driven largely by the introduction and widening applications of non-invasive ventilation. Ventilatory support may be used with the intention of increasing survival, or to facilitate discharge home and/or to palliate symptoms. However, the outcome of this intervention and the number of children transitioning to adult care as a consequence of longer survival is not yet clear. Methods In this retrospective cohort study, we analysed the outcome in children (< 17 years) started on home NIV at Royal Brompton Hospital over an 18 year period 1993-2011. The aim was to establish for different diagnostic groups: survival rate, likelihood of early death depending on diagnosis or discontinuation of ventilation, and the proportion transitioning to adult care. Results 496 children were commenced on home non invasive ventilation; follow-up data were available in 449 (91%). Fifty six per cent (n = 254) had neuromuscular disease. Ventilation was started at a median age (IQR) 10 (3-15) years. Thirteen percent (n = 59) were less than 1 year old. Forty percent (n = 181) have transitioned to adult care. Twenty four percent (n = 109) of patients have died, and nine percent (n = 42) were able to discontinue ventilatory support. Conclusion Long term ventilation is associated with an increase in survival in a range of conditions leading to ventilatory failure in children, resulting in increasing numbers surviving to adulthood. This has significant implications for planning transition and adult care facilities.