Long term non-invasive ventilation in children: impact on survival and transition to adult care

Abstract

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.

Fig 1. Consort Diagram.

Fig 1 shows a CONSORT-style diagram for the patients who were identified, analysed and subsequent outcome.

Fig 2. Patients initiated on ventilatory support by year and diagnosis.

Fig 2 shows the number of patient’s initiated on ventilator support at our centre by year of initiation. It also shows the diagnosis group of the patient’s initiated each year. Note that over time the group diagnosis proportions change from predominantly neuromuscular disease (NMD) to include other such as chronic lung disease (CLD) and syndromes which include Trisomy 21. Central apnoea (CA), upper airway (UA) and chest wall disease (CWD).

Fig 3. Survival probabilities for patients with neuromuscular disease who start non invasive ventilation.

Fig 3A shows survival probabilities for; congenital muscular dystrophy (CMD); congenital myopathy; spinal muscular atrophy type II (SMA II); Duchenne muscular dystrophy (DMD) commenced on NIV < 17 years old; DMD commenced on NIV > 17 years old. Fig 3B shows survival probabilities for; air way abnormalities post cardiac surgery (cardiac); central apnoea; chronic lung disease; chest wall disease; obesity hypoventilation syndrome; other diseases not classified in any other group; syndrome and upper airway.