Paediatric home ventilatory support: Changing milieu, proactive solutions

In 1989, an editorial in the Medical Journal of Australia called for better support for adults requiring long-term mechanical ventilation. The sentiments were echoed in an article 10 years later, calling for a national register of all adults and children requiring ventilatory support, reflecting that not just ‘Superman, but Everyman, should be able to live at home on a ventilator’. In 2011, despite an escalating number of patients, we see little change. Many differences exist between the needs of children who require home ventilation compared with adults and, as paediatricians, we have chosen to write this paper to highlight the issues faced by a growing number of children in Australia and New Zealand who are medically stable but require ventilatory support for all or part of every day. We use the following definitions in this article: • Ventilatory support – assistance to respiration provided by mechanical means • Ventilator dependency – where an individual requires a mechanical aid for ventilation for any period of the day in order to maintain optimal health • Long-term ventilation (LTV) – failure to wean from ventilatory support once medically stable, 3 months after the institution of ventilation • Invasive ventilation – ventilatory support delivered via an endotracheal tube or a tracheotomy • Non-invasive ventilation (NIV) – ventilatory support delivered via a nasal mask or similar non-invasive interface. • Home – may be the child’s home, a foster home or a groupliving environment Factors driving the growth in paediatric home ventilation are a change in society’s expectations of quality of life in the presence of long-term disability, the pressure to reduce the duration of hospital stay, and recognition that children with a broad range of medical conditions derive substantial benefits from long-term respiratory support in the home. Improvements in equipment design and technical support have broadened access to equipment for children who require respiratory support at home. Children who would previously have died or been managed long term in hospital with ventilatory support via a tracheotomy are increasingly managed with less invasive forms of ventilatory support (NIV or continuous positive airway pressure (CPAP) via a mask). Earlier reviews of this topic were ambivalent regarding the benefits of long-term home ventilation. In the last 10 years, better information has become available on the long-term outcomes of children on home ventilation. While provision of a high-quality domiciliary service is expensive, a proportion of children only need short-term support, while it is estimated that another 40% eventually outgrow their need for treatment. This is an important contrast to adults, among whom only a small percentage (3%) improves sufficiently to discontinue therapy. In addition, it is now clear that, once a child is established on ventilatory support at home, non-elective readmission to hospital is unusual. Home-based care is often feasible and may be less expensive than in-hospital care, and the increasing demand has highlighted issues of funding for children requiring complex care outside specialised hospital settings. Cost savings and other benefits have been best studied in the use of NIV for children with neuromuscular disease, where treatment of chronic respiratory failure is associated with decreased hospital admissions, reduced hospital days, reduced intensive-care days and improved quality of life. For this group of patients, chronic respiratory failure is a medical complication that can be effectively managed by ventilation at home. The aim of this article is to raise awareness of the increasing medical burden together with the gaps in resource provision for children on home ventilatory support throughout Australia and New Zealand and provide a consensus opinion on proactive solutions.