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.[3, 4] 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 group-living 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.[5-8] In the last 10 years, better information has become available on the long-term outcomes of children on home ventilation.[9-18] While provision of a high-quality domiciliary service is expensive,[19, 20] a proportion of children only need short-term support, while it is estimated that another 40% eventually outgrow their need for treatment.[9, 10] 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[18, 23] and may be less expensive than in-hospital care,[18, 20] 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,[25-28] where treatment of chronic respiratory failure is associated with decreased hospital admissions, reduced hospital days, reduced intensive-care days[25, 29] and improved quality of life.[30-33] 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.
Do we really know the size of the problem?
There has been only one previous national review of children cared for at home on ventilatory support in Australia. This was a report undertaken in 1993 by the National Health and Medical Research Council, which identified 19 children and 39 young adults receiving home ventilation, 6 via tracheotomy. The largest paediatric Australian state review (from Victoria) was recently published by Tibballs et al. The service based at the Royal Children's Hospital in Melbourne was established in 1985, with funding from the State Health Department and involves a multidisciplinary approach to hospital discharge, with training of parents and laypersons as care givers in most cases. The review describes the 168 children who went through the program until the end of 2008 and demonstrates a considerable increase in patient numbers over the years the program has been operating. An equivalent 12-year retrospective review in New Zealand was undertaken a few years previously (up to 2004), which also documented an increasing trend in children on home ventilatory support managed through one centre in Auckland. That study showed home CPAP, NIV or IVS was initiated in 160 children (median age 6 years) over the 12 years, and the numbers and complexity of support rose in the study period. Respiratory support was discontinued in 57% of cases, after a median of 12.5 months (range 3–52 months); in 38%, support was no longer required due to an improvement in the medical condition, 7% of children died and 2% transitioned to adult care. In contrast, the Victorian review stated only 16% recovered, 22% died and 17% were transferred. The differences in outcome between these two reviews highlight the heterogeneity of each centre's population and the difficulty in extrapolating detailed information from one centre to the whole region.
In the absence of a formal register or resources to keep local databases, it is difficult to obtain accurate information on the total number of children on home ventilation in Australia and New Zealand. In 2008, following the publication of the Australasian guideline, we undertook the first email survey of all paediatric respiratory and sleep medicine specialists in Australia and New Zealand to obtain an estimate of patient numbers on ventilatory support. In Australia, nine specialist paediatric centres in six states responded, and in New Zealand, Starship Children's Hospital in Auckland compiled data from five paediatric departments throughout the country known to have children on ventilatory support. Respondents outlined major increases in the numbers of children on all types of ventilatory support in all geographic areas. Tables 1 and 2 summarise the centres' responses regarding the number of children actively managed now compared with 10 years ago. While the numbers of children ventilated at home via tracheotomy remained small, there was a threefold rise in Australia to 1.2/100 000 and a twofold rise in New Zealand to 0.5/100 000 (Table 1). In 2008, the numbers of children per 100 000 in New Zealand and Australia supported at home on NIV and CPAP were strikingly similar (NIV ∼5.1/100 000; CPAP ∼8.5/100 000) (Table 2). While no specialist paediatric respiratory and sleep medicine centre in Australasia was omitted from the survey results, it is acknowledged that some cases of CPAP, in particular, not under tertiary care, may have been missed. In addition, in Europe, there is a large group of children whose need for ventilatory support is currently unrecognised due to a lack of diagnostic and treatment facilities, and the same may be true in Australia and New Zealand.
|Victoria||3 (0.3)||9 (0.9)|
|Queensland||3 (0.4)||3 (0.4)|
|New South Wales (includes ACT)||7 (0.6)||26 (2.0)|
|Western Australia||–||6 (1.5)|
|South Australia||1 (0.4)||2 (0.7)|
|Total Australia||14 (0.4)||46 (1.2)|
|Total New Zealand||2 (0.2)||5 (0.5)|
|Respiratory support||NIV||CPAP||Total NIV|
|Victoria||2 (0.2)||21 (2.2)||10 (1.1)||69 (7.3)||12 (1.3)||90 (9.5)|
|Queensland||15 (2.2)||35 (4.3)||42 (6.1)||46 (5.7)||57 (8.2)||81 (10.0)|
|New South Wales (includes ACT)||7 (0.6)||81 (6.1)||20 (1.6)||163 (12.2)||27 (2.2)||244 (18.3)|
|Western Australia||10 (2.7)||28 (7.1)||5 (1.4)||33 (8.3)||15 (4.1)||61 (15.4)|
|South Australia||5 (1.8)||37 (13.2)||4 (1.4)||25 (8.9)||9 (3.2)||62 (22.1)|
|Total Australia||39 (1.1)||202 (5.1)||81 (2.2)||336 (8.5)||120 (3.3)||538 (13.7)|
|Total New Zealand||3 (0.4)||48 (5.2)||5 (0.6)||80 (8.6)||8 (1.0)||128 (13.7)|
In other centres around the world, the numbers of children supported with ventilation via tracheotomy appears to have remained relatively static over the past 10 years[9, 36] or even, as suggested in a recent UK point in time census, reduced. Gowans et al. found that the prevalence of children (<16 years) requiring invasive mechanical ventilation at home in the US state of Utah had changed little over an 8-year period (1996–2004) – 5 to 6.3/100 000. Our Australasian estimates of children on invasive ventilator support are much lower: New Zealand 0.5/100 000 (<15 years of age) and Australia 1.2/100 000 (<15 years of age). These Australasian figures are comparable with that reported in the UK in 1999 at 0.5/100 000 (children only <16 years). However, according to recent data from the UK LTV working group, 206 children were maintained on tracheotomy LTV, which using UK paediatric (≤16 years) population data from mid-2008 (according to the Office for National Statistics) equates to 1.8/100 000.
Direct comparisons of population rates for other types of paediatric home ventilation is difficult, as surveys have been undertaken at different points in time (e.g. Fauroux et al. 2002 reported 102 children in France on CPAP and NIV, which equates to 0.08/100 000 – much lower than our Australasian survey), and data are often combined with adults (e.g. Eurovent study 2005 reported 6.6/100 000 adults and children). However, if recent numbers from the UK LTV working group are adjusted using population data (according to the Office for National Statistics), total LTV support of any mode (n = 933) was used by 8.1 children/100 000 in the UK, in comparison with New Zealand and Australia at 14.3 and 14.8/100 000, respectively. While we acknowledge both sources may underestimate true numbers, this comparison would suggest higher numbers in Australasia. If we focus only on CPAP and NIV in the UK (n = 704 or 6.1/100 000), the burden appears to be twice as high in Australia and New Zealand (13.7/100 000, respectively).
Variability in patient numbers and, therefore, population estimates across different geographic areas could be contributed to by a variety of factors – patient selection, paediatric population density, prevalence of chronic disorders and physician issues pertaining to number, knowledge and expertise in sleep medicine and LTV. It seems likely that there is also growing awareness of the impact of sleep-related disorders on a variety of paediatric conditions in primary and secondary health care. Also of importance are the state and federal funding lines and whether there are existing home ventilation programs.
Do similar care programs exist across Australia and New Zealand?
Our 2008 survey also requested information on resources and funding care programs in each state of Australia and in New Zealand. Our comments in the following about current services in each area are based on the responses to that survey.
Funding options for children requiring invasive ventilatory support were relatively consistent, with all areas having identified funding pathways for payment of carers. Equipment was often paid for from hospital budgets, with only some states having specific funding avenues for this. Co-ordination of care was variable however, usually without specific infrastructure and carried out on an ad hoc basis. Availability of out-of-hospital locations for respite care was also very variable, meaning that respite care often happened in hospital wards or intensive-care units.
For non-invasive ventilatory support, initial treatment and primary care is often undertaken by tertiary centre respiratory paediatricians and intensivists. Some centres employ clinical nurse consultants (CNCs) or case workers to co-ordinate patient care. A minority of services utilise service providers outside public hospitals such as those provided by some device manufacturers. Contrary to the finding with invasive ventilation, there is no consistency in funding for equipment, servicing or the supply of consumable items for NIV, with the costs being borne by insurance companies or families themselves in some areas, while others have government programs (Queensland). The responsibility for the co-ordination of care usually falls to hospital Respiratory Services, with some centres having a CNC assigned to this role, but others rely on staff in sleep medicine services.
Authors from several countries have described their experience with home-based ventilation programmes for children.[9, 18, 24, 36-46] In Australia and New Zealand, guidelines for home ventilation in children were recently published, adding to those published in the United Kingdom (1998) and in the USA (1990). These existing reports highlight substantial differences in practices, care packages and financial support. As exemplified by our 2008 survey, such disparity also exists in Australia and New Zealand. No formal guidelines exist for the management of children highly dependent on nightly NIV, apart from a document specific to central congenital hypoventilation syndrome (CCHS) from the United States.
What are the key areas that need addressing?
Funding models vary among states in Australia and with New Zealand. Completing applications for funding is frequently the responsibility of medical and nursing staff and can be extremely time consuming if it involves multiple departments and service providers. If families relocate between states or countries, new applications for local funding are generally required. New Zealand services do not have centralised funding or co-ordination, so significant inequities also exist within New Zealand. There are further inequalities, such as in the state of Victoria, which has a dedicated respiratory support service (equipment and staffing) for adults but not for children.
We attest that appropriate and equitable funding packages for home care should be available to all children when they meet criteria of stability for discharge.[4, 49] NIV does not constitute lesser care needs for children who are obligatorily dependent on nightly NIV (such as those with CCHS). Packages need to provide the same level of care for these children as for those on tracheotomy ventilation. The fact that ventilatory abnormalities in children may improve or deteriorate with age means that flexibility in packages is required over time. The major deficiencies in current funding are for diagnostic services, equipment and long-term management of NIV and CPAP. Clear pathways should be accessible for channelling funding applications.
Carers and care packages
Three broad models for care exist:
- The host hospital provides equipment and supplies (e.g. UK and New Zealand)
- Private sector management with hospital only follow up (e.g. USA)
- Specialised community-based organisation management agencies (e.g. France)
Within Australia, a mixture of these models is in operation.
Trained carers are often required to support and provide respite for parents caring for a child on ventilatory support at home. Financial assistance to provide these carers varies considerably across Australasia, particularly when patients are on NIV. Carers to support children on tracheotomy ventilation may be provided from 8 to 24 h/day and from 5 nights a week despite recommendations for carers during all sleep times 7 days a week. The level of training varies from trained lay people to registered nurses. Carer support also varies in some areas – if carers take a sick or holiday leave, some patients are forced into respite care or hospital. Agencies specialising in the provision of carers can expedite discharge and address some of these issues, but not all states have access to these resources.
The training of carers for patients on tracheotomy ventilation is provided by a variety of service providers, host hospital departments or community agencies. For NIV, there are some programs, but most are reliant on the local tertiary respiratory services or sleep medicine units. The UK survey highlighted staffing obstacles: failure to recruit qualified nursing staff or trained carers, along with delay in obtaining funding. In Australia and New Zealand, although the patient group is similar, community support services for children requiring long-term NIV differ significantly from those in the UK.
We acknowledge that the provision of adequate carers is a more generic issue and important to many paediatric and adult groups with significant disability. The resources and, particularly, carer support required to manage someone on ventilation in the community are greatly influenced by the degree of physical disability and ventilator dependence. An American paper has suggested 16-h/day carer input, but it can be argued that those who are ventilator dependent have an obligatory requirement for 24-h/day supervision, making trained carer support particularly important. The Australian guidelines outlined a best practice level of service provision for children requiring ventilatory support at different levels of dependency. What is usually available almost always falls short of this mark, and the gap in care is met by family and friends.
Our survey demonstrated that there is a marked discrepancy between centres in the availability of suitable respite care for children on home ventilation. The scarcity of carers and limited resources for suitable paediatric respite facilities can result in inappropriate hospital ward or intensive-care admission.
The current structure of centralised specialist services poses access challenges for many patients. Compounding this, contracts with different suppliers mean that different makes and models of machines with various types of disposable equipment are used in different locations. Equipment support for children on invasive ventilation ranges from hospital intensive-care-based services at a host hospital to community/district hospitals close to the child's residence. Maintenance of NIV equipment is predominantly the responsibility of biomedical engineers from the host health service provider or the company from which the machine is purchased. Families of children utilising NIV are most often required to pay for disposables if these are not provided by their host hospital or health board.
Co-ordination of services
Forward planning is needed to ensure provision of appropriate medical services to all children, including those living far from tertiary centres, with access to lung function, paediatric sleep medicine and experience in home ventilation. In Australia and New Zealand, services have evolved to perform this role without concurrent growth of suitable infrastructure, meaning that high patient numbers are likely to impact on the maintenance and quality-of-service provision. Dedicated databases and co-ordination of staff are needed to ensure the highest quality of treatment, initiation and follow-up.