organizing home ventilation

SYMPOSIUM: RESPIRATORY MEDICINE

Organizing home ventilationHayley Smith

Tom Hilliard

AbstractThe number of children and young people receiving long term ventilation

continues to rise, with increasing survival from intensive care, improve-

ments in equipment and changing attitudes towards providing respiratory

support. These children are also now largely at home rather than in

hospital. However for those who have complex problems and are most

dependent on ventilation, discharge can be a slow, difficult and costly

process. There are a number of barriers to discharge for this group of chil-

dren, including professional attitudes, problems with commissioning and

funding, and establishment of care packages, in addition to complex

social issues and difficulties with housing. Good discharge planning starts

at the outset of establishing a child on long term ventilation, and aims to

overcome these barriers, facilitating discharge in a safe and timely

manner. A full assessment of a child and family’s needs, having

a discharge coordinator and working in partnership with the family and

the agencies involved, are all key to the success of this process. Ongoing

care after discharge must also allow for changes in a child’s needs and

support for the family, including transition to adult services and end of

life care planning where appropriate.

Keywords child; discharge planning; home care services; mechanical

ventilation; tracheostomy

Introduction

The number of children on long term ventilation (LTV) continues to

increase. A survey in the United Kingdom in 1990 identified 24

children on LTV, of whom only nine were at home. A subsequent

survey in 1991 found 141 children receiving LTV, with 93 of them

being cared for at home. The most recent estimate in 1998 was 944

children, with 91% at home. Our own service has grown from

supporting 10 children in 2000 to over 80 in 2010, of whom all but

one are currently at home. LTV for children has therefore increased

dramatically over the last twodecades, and their location of care has

largely moved from intensive care to the home. Involvement with

a child or young person on LTV is now relatively common formany

paediatricians.

There are a variety of reasons for this increase. Equipment

technology has improved, particularly for that related to non-

invasive ventilation, and there has been a major expansion in the

Hayley Smith Dip HE in Nursing Studies is a Clinical Nurse Specialist in Long

Term Ventilation in the Department of Paediatric Respiratory Medicine,

Bristol Children’s Hospital, Bristol, UK. Conflicts of interest: none.

Tom Hilliard MD FRCPCH is a Consultant in Paediatric Respiratory

Medicine in the Department of Paediatric Respiratory Medicine, Bristol

Children’s Hospital, Bristol, UK. Conflicts of interest: none.

PAEDIATRICS AND CHILD HEALTH 21:5 224

use of respiratory support in children and young people with

neuromuscular disease. There have also been changes in attitudes

towards the appropriateness of providing ventilation to children

with some conditions, and to supporting them in their home. The

survival of critically ill children from paediatric or neonatal

intensive care has also improved, but often with children needing

long term respiratory support. However despite the fact that being

cared for at home rather than in hospital is to the advantage of the

young person and their family, there are a number of clear barriers

to discharge. Discharge from hospital to home for these children

remains a lengthy, costly and complex process; one series

describing the discharge of children on tracheostomy ventilation

had an average of 9.6 months in hospital awaiting discharge.

Children will obviously vary in their complexity and needs, and

this article provides a practical framework on which to plan

a child’s discharge and beyond.

Underlying conditions and types of ventilation

The underlying reasons for LTV generally fall into four groups:

(i) neuromuscular disease, (ii) airway and/or pulmonary

abnormalities, (iii) abnormalities in control of breathing and (iv)

spinal cord injury. Neuromuscular disease is the most common.

LTV can be divided into invasive (via a tracheostomy) and non-

invasive ventilation (NIV). Within NIV, there is a further division

into continuous positive airway pressure (CPAP) and bi-level

positive airways pressure. Ventilation may also be needed either

continuously or partially, usually when asleep. It may be critical

for survival, e.g. in congenital central hypoventilation syndrome

(CCHS) or patients may be able to be without their ventilator for

a period of time. To a large extent the underlying condition and

these factors will dictate the type of ventilation used. An infant

with CCHS who has severe sleep related hypoventilation or

a major airway anomaly is likely to need tracheostomy ventila-

tion, whereas a young person with neuromuscular disease can

have nocturnal NIV. Overall, NIV is the commonest form of LTV.

The type of ventilation and/or the interface used for an indi-

vidual may also change over time depending on their needs and

the progress of their underlying problems.

The decision to start long term ventilation

The decision to embark on LTV is the most crucial step in the

whole process. A full and frank discussion should take place with

the family, and when appropriate the young person, and involved

professionals. Sometimes the decision to start LTV is a clear and

relatively easy one. However there are also circumstances when

deciding to start a process of LTV is extremely difficult, when it is

not clear whether it will be in the best interests of the child.

Attitudes to providing respiratory support in some very severe

progressive conditions have changed over the last decade; type 1

spinal muscular atrophy (SMA) is an example. While there are

very significant doubts about the appropriateness of tracheostomy

ventilation for infants with severe type 1 SMA, there is increasing

use of NIV in infants with SMA in the UK. In other conditions, the

decision around starting ventilation may be around when to start

respiratory support. This may involve repeated physiological

recordings during sleep, for example when determining onset of

sleep hypoventilation in a progressive neuromuscular disease

such as Duchenne muscular dystrophy. Regular and open

� 2010 Published by Elsevier Ltd.

http://dx.doi.org/10.1016/j.paed.2010.10.008


discussions with young people and their families in this situation

will likely make initiation of LTV at the correct time easier.

LTV may have to be started at short notice and at a time of

a critical illness, or theremay be time to plan a package of initiation

and ongoing care. This will largely be dictated by the underlying

condition and the type of ventilation needed. It is important at the

outset to clearly discuss the possible outcomes with families, and

the likely time frame over which ventilation is likely to be needed.

This may range from the relatively short term over months, to long

term to adult life, as well as palliative support in a young child who

may only survive another few months. Plans for how to cope with

an escalation of needs should be made early on in the process.

Starting LTV in the acute situation will usually take place in an

intensive care setting, with usually a child progressing to a less

intensive area before discharge. Children may also move from

a regional centre to their local hospital when they are more stable,

and to then facilitate a local discharge process. In this instance close

liaison between the hospitals is needed, and there should be

a transfer planning process with meetings between key staff. Our

service also initiates non-invasive LTV for some of our children and

young people at home, and this is ideal when there is time to allow

for habituation to the mask and gradual increases in ventilation

pressures. Initiation at home may also decrease the complexity of

a discharge package, but still involves careful assessment and

planning around a child and family’s needs.

Equipment

A ventilator for LTV must be appropriate for the type of ventilation

needed and be suitable for home use. For invasive ventilation, the

ventilator must be suitable for use with a tracheostomy. The leak

around the tracheostomy must be appropriate, and for young

children a tracheostomy tube with an extended flange, or

a customized tracheostomy, may be useful. A non-invasive venti-

lator must be compatible with its interface, for example a vented or

non-ventedmaskmay be needed. There are a large variety ofmasks

available including nasal (covering the nose only), full face

(covering the mouth and nose) and other variations. Mask fit is the

most important factor in NIV, and ensuring the right comfort and fit

while minimizing leak are vital to ensure adequate ventilation and

increase compliance. There are a number of different ventilator

modes available (e.g. ventilation determined by pressure limits or

the volume delivered, or a combination). Less experienced units

should seek advice from their regional centre on ventilator and

interface issues, including the settings required. These may also

change over a relatively short time frame for children transferred

from their regional centre.

Ventilators for home use will have a number of other factors

that need to be considered, e.g. portability, alarms and battery life.

Alarms need to be set appropriately (and staff understand their

function) to detect disconnection, or tracheostomy tube blockage.

A child who is critically dependent on ventilation will require

a second back-up ventilator. Tracheostomy ventilation needs

a humidified (or “wet”) circuit, with a dry circuit only for short

term portable use, and carers need to be familiar with these and

the requirement for changing them. Many non-invasive ventilators

now have humidifiers built-in. There may be a significant amount

of other equipment necessary (e.g. monitors, suction machine,

oxygen; see Figure 1) and this needs to be carefully listed and


purchased for home use. These may take up significant space at

home, and they may also need to be made portable. For young

children a buggy may need adaptation and getting advice,

including from other parents, may be very useful. There is also

a need for a sustainable system for funding, ordering, delivering

and disposal of consumables. Equipment will need regular

servicing with supply of back-up equipment available when this is

being carried out.

Training

Teaching and training for the family should begin as soon as

possible. Encouraging an active involvement through training

will reassert a degree of control to parents and as well as a feeling

of progress, moving towards the end goal of discharge. Training

should encompass the use and maintenance of equipment,

procedures and emergency care. However, it is important not to

overload parents with too much information at once; each parent

will learn at their own pace and should be given adequate time

for instruction, questions and gaining of competence. Grand-

parents or other family members may also wish to be involved in

this process. Training programmes should be based around

specific competencies, supported by formal local documentation.

Staff may also need specific training, particularly if they are not

experienced in LTV, or a particular piece of equipment. The

regional centre may have nurse specialists who can facilitate this,

potentially also with the help of company representatives.

Training of unqualified carers who are employed in a care

package should proceed along the same lines. Specific arrange-

ments may need to be made (e.g. honorary contracts) to allow

carers to train within the hospital. It may take time for carers to

develop their confidence and competence, and this should be

discussed with the family so that they understand this.

Planning discharge

Discharge planning should start once a child is established on LTV

in hospital. Although there will be a need for a period of time to

assess clinical stability, planning for discharge should continue

rather than awaiting stability. Several studies have identified

significant barriers to discharge while children on LTV have

remained in hospital for many months, and these are listed in

Table 1. Good discharge planning attempts to overcome these

barriers, so that discharge can be achieved in a safe and timely

manner with good cooperation between the agencies and profes-

sionals involved.

Noyes and Lewis have written a care pathway (From hospital

to home) that illustrates the optimal management of a child from

initiation of LTV through discharge, care at home and then

transition to adult services, and forms part of the National

Service Framework for Children, Young People and Maternity

Services. They use the example of a child with CCHS, who has

tracheostomy ventilation while she is asleep, but this pathway

can be used with modification for other children who may have

different underlying problems and ventilatory needs. It gives

detailed practical advice on discharge planning, and is extremely

useful reading for anyone involved in the discharge process. The

discharge planning process can be divided into the following

main areas, with a basic checklist for the process given in

Table 2.



Figure 1 Equipment required for an infant dependent on invasive ventilation to be outside the home.


Assessment

A full multi-disciplinary and multi-agency assessment of the

child’s and family’s needs is a fundamental early part of the

discharge process and will direct the planning of how to support

them in due course. There are a number of general and specialist

assessment tools available and the whole family’s needs will

need to be taken into account. Parents may also benefit from

talking to other parents who have children at home on LTV so

they can assess their own needs.

Care package

Identifying an appropriate model of care will depend on the needs

assessment together with local factors such as expertise, commis-

sioning policy and service capacity, including voluntary and private

sector organizations. Professionals inexperienced in deciding on

care packages should seek advice from those experienced in

commissioning, so that an appropriate model of care is proposed

Barriers to discharge

C Attitudes of professionals

C Commissioning & funding of services

C Poor management by professionals

C Difficulty in recruiting carers

C Complex social issues

C Housing problems

Table 1


from the outset. Important factors will include the child’s depen-

dence on ventilation, the type of ventilation, the child’s other

medical issues, the family’s ability to cope and the other demands

on their time. A child dependent on tracheostomy ventilation will

usually need a care package involving at least several nights of carer

support,whereas familieswith childrenonnon-invasive ventilation

may not need any specific night carers. There also needs to be

a balance between what is ideal and what is deliverable, as well

some flexibility to provide extra support when necessary.

Carers do not generally need to have professional training or

qualifications, but are trained to care for specific children following

set procedures and protocols. They are trained to know their limits,

so that they seek help when necessary according to care plans, and

do not make independent clinical decisions. However recruiting

carers to form a care package is often one of the major delays to

discharge. Strategies to aid recruitment (and retention) of carers are

outlined in the From hospital to home document. Using teams of

carers can give more flexibility, and our local Lifetime Service

(based in Bath and Bristol) has used this approach successfully. In

addition to the initial programme of training, carers need ongoing

supervision, mentorship and support.

Funding

This care package should then be proposed to the local commis-

sioning body, with a full costing of what is required. Discussions

regarding funding should take place at an early stage, with ideally

an agreement in principle for funding pending a full costing in due

course, so that discharge planning should continue while funding

is being sought. Parents need to be kept informed about the



Checklist of processes for discharge planning

Equipment Establish child on appropriate

ventilator and interface

Purchase other equipment required

Ensure availability of supply of consumables

Arrange regular servicing

Lead coordinator Identify key worker to coordinate discharge

Stability Identify medical stability of child and

appropriateness of discharge

Assessment Perform full multi-disciplinary and multi-

agency assessment of child and family’s needs

Use available assessment tools to aid this

process

Package Identify care package based on needs

assessment

Establishment of personnel necessary for

package

Funding Early discussion to seek approval in principle

Submit proposal of care package

to funding body

Training Training for parents, hospital staff and carers

based around competencies with clear

documentation

Housing Establish whether adaptation or alternative

housing required

Meetings Planning of meetings at strategic points

Invitation to key personnel

Appropriate management of meetings and

outcomes with dissemination of information

Risk assessment Analysis of risks and action to minimize risk

Action plans Planning for escalation of needs

End of life care planning

Support Support for parents throughout process

www.longtermventilation.nhs.uk

www.breatheon.org.uk

www.cafamily.org.uk

Final discharge Trial periods at home and staged discharge

Discharge documentation

Table 2

Key personnel during discharge planning process

C Family and child

C Respiratory consultant

C Other consultants involved (may be general, specialist or

community paediatricians)

C Clinical nurse specialist in LTV

C Ward nursing staff

C Community nursing staff

C Commissioning organization

C Health visitor

C General practitioner

C Social worker

C Dietician

C Speech and language therapist

C Physiotherapist (hospital and/or community)

C Occupational therapist


funding process, but should not be directly involved in discus-

sions. In our experience it is unusual for funding to be refused if

adequate information is provided to the funding body.

Care packages for those children with the most complex needs,

particularly on invasive ventilation, can be extremely costly; the

illustrative package in the care pathway (2005 prices) was over

£97 000 for the first year including equipment, with ongoing staff

costs of £53 000 each year. However caring for a child in intensive

care (£603 000 per year) or a high dependency unit (£301 000 per

year) is much more expensive. There should therefore be a strong

financial incentive to prevent delay in discharge.
C Voluntary sector support worker
C School staff
Working together
Table 3

A multitude of professionals will be involved from a variety of

disciplines and agencies, and getting everyone to work together


towards a common aim can be challenging. An important part of

facilitating working together is identifying a key worker or lead

coordinator for a child’s discharge and ongoing care. A discharge

coordinator for children on LTV has been shown to shorten time

to discharge, and increase professionals’ satisfaction with the

discharge process. Throughout the whole process it is essential to

continue to work in partnership with the child’s parents, keeping

them actively involved in the process while providing them with

appropriate support. Being in contact with other parents of

children on LTV can be helpful, and there are number of other

support networks (see Table 2).

Housing

Issues with housing are a major barrier to discharge, with one-

third of families living in unsuitable housing in one series. An

appraisal of the family home will be an important part of the

initial needs assessment. A child on LTV should ideally have

their own bedroom, with sufficient space and power supply for

equipment and storage of consumables. If there are additional

carers, they need to have appropriate space and potentially their

own facilities. The involvement of community occupational

therapists can help in assessment of the home and necessary

adaptations, while social workers can support applications for

funding where appropriate and help negotiation with local

authorities and housing associations.

Meetings

Proper management of discharge planning meetings is an essential

part of moving forward the discharge process. These should be

scheduled at strategic points and organized by the lead coordinator.

Key attendees are those who have a stake in the discharge and

ongoing care of the child, can actively contribute to themeeting and

can make decisions when necessary. We have provided a list of

suggested invitees to plan meetings in Table 3. Families should be

actively involved in meetings, with adequate preparation of


http://www.longtermventilation.nhs.uk

http://www.breatheon.org.uk

http://www.cafamily.org.uk



anticipated outcomes. Meetings should follow an agenda and be

appropriately chaired, with the agreement of action points and

those responsible for outcomes within agreed time frames.Minutes

should be circulated following the meeting and these can update

non-attendees or other professionals.

Risk management

Healthcare organizations are obliged to assess, register and

manage risk, and risk management is central to the discharge

process. Risk assessments should be performed using that orga-

nization’s template, with analysis of identified risks and action to

reduce them to an acceptable level. It is an ongoing process and

will need to be reviewed when significant changes occur with the

child or their environment. This process should be seen as being

supportive to the family rather than obstructive.

Final discharge

Discharge can be a staged process, and this is very helpful for

children with complex needs who have had a prolonged stay.

Parents can spend increasing amounts of time with their child

outside the hospital and at home, with carers also spending time at

the family home. Approaching discharge a trial run of a day, night

and then longer can be a valuable time for addressing unexpected

issues. A final discharge date then needs to be carefully chosen,

making sure it is a time when there is appropriate backup support

available.

Ongoing care

Outcome

A recent description of an Australian home ventilation programme

of 168 children over a 20-year period, with a variety of underlying

conditions and types of ventilation, had 36 deaths, but only two of

these were unexpected. 16% of children improved sufficiently to

come off ventilation. These outcomes are generally positive, and

their service used similar packages of care to those in the UK. This

contrasts with an American cohort of 228 children on tracheos-

tomy ventilation in a home program; over 22 years, 47 died, but

49% of these deaths were deemed to be unexpected. Avoidable

causes of deaths included mucus plugging and tracheostomy

misplacement, highlighting the increased vulnerability of tra-

cheotomized children, particularly those on LTV.

Quality of life

There is a growing body of literature on the quality of life for chil-

dren and young people on LTV and their families. Stayingwithin an

intensive care unit has a profoundly negative impact on young

people, exposing them to other children dying, lack of parental

attachment, an abnormal home boundary and problems with

communication. Children say that their ventilator makes them feel

better, but they can have low self-esteem, lowhealth-related quality

of life scores, and medicalization of their childhood. Health

professionals must consider their own prejudices when trying to

estimate the quality of life in young people with physical impair-

ments; a study from Switzerland showed that quality of life in

young men with Duchenne muscular dystrophy did not differ from

controls (except for physical function), and was similar between

those on LTV and those yet to go on to LTV. Clear themes for

families experiences are being the “lifeline” for their child, over-

whelming parental responsibility, stress, a new perception of


normal since LTV began (including for siblings), becoming experts,

isolation, and living with uncertainty and loss. Giving support and

facilitating respite care is an important part of providing ongoing

care.

Adverse effects

One of the most common side effects of non-invasive mask

ventilation is a facial skin injury. Transient or prolonged

erythema, and in some cases, skin necrosis, were found in almost

half of children on NIV in a French study. Damage to the mask

and prolonged use each day were associated with skin injury,

whereas changing to a custom-made mask lessened injuries.

Paying close attention to the mask fit, potentially changing

masks, and decreasing the use of NIV and potentially pressures

when appropriate can help to avoid injury. Facial flattening is

also a significant risk in children with growing facial structures.

Clinical review

When a child is discharged home it is essential to define the

clinical responsibilities between professionals in the community

and the hospital. Families need clear lines of communication for

problems in normal working hours and outside these times.

There should be clear guidelines on what to do when there are

clinical queries or equipment problems. There should be easy

access for re-admission to hospital, including intensive care

when this is appropriate. Plans on short-term alterations in

ventilator settings and the use of additional oxygen with acute

intercurrent illnesses need to be set out in advance. A child’s LTV

needs may also change over time, so that some children may

need to have a plan for weaning ventilation, while some will

have increased ventilatory needs, largely related to the progress

of their underlying condition. When this is predictable, this

should be openly discussed in advance with the family and when

relevant, young person. Increasing time on ventilation may

prompt the use of additional forms of ventilation (e.g. daytime

mouth piece ventilation) or a discussion around changing the

overall mode of ventilation. Children with difficulties with

sputum clearance may benefit from a mechanical insufflation/

exsufflation device and if they have not tried this already,

considering its use after ventilation has started may be useful.

We would recommend early end of life care planning for

children with life limiting conditions, and our service uses

a nationally recognized advanced care plan framework called the

Wishes Document. This acts as a template for the discussion and

recording of a family’s wishes for when a child becomes unwell,

has a life threatening event and after death.

Transition

Young people dependent on long term ventilation often face

a number of challenges as they approach their adult years and

need to have clear plans for their transition and transfer into adult

services. Services for transitioned adult patients continue to

develop, and there may be different expectations and resources in

adult support services. Hospital admission to an adult medicine

service may also be quite different to a young person’s paediatric

experience, and this will need to be discussed in advance. We hold

joint clinic reviews during the process of transition, similar to our

transfer of young people with other chronic conditions. Advance

planning is key to success in this process.



Practice points

C Discharge planning should start at the outset of initiating long

term ventilation.

C Perform a full needs assessment of the child and family, using

available assessment tools.

C Appoint a discharge coordinator to drive the steps in the

process and facilitate partnership between the family and the

professionals involved.

C Plan ongoing care following discharge, including the changing

needs of the child.


Summary

The number of children requiring long term respiratory support

continues to increase, and for those most dependent on ventila-

tion, discharge home can be a lengthy and complex process.

Successful discharge requires a thorough assessment of the

child’s needs, meticulous planning and coordination, and

working in partnership with the agencies involved. Professionals

also need to plan for the ongoing care of the child and family

after discharge, including how their evolving needs should be

met over time. A

FURTHER READING

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Edwards JD, Kun SS, Keens TG. Outcomes and causes of death in children

on home mechanical ventilation via tracheostomy: an institutional and

literature review. J Paediatr 2010 (published online).

Fraser J, Harris N, Berringer AJ, Prescott H, Finlay F. Advanced care plan-

ning in children with life-limiting conditions e the wishes document.

Arch Dis Child 2010; 95: 79e82.

Jardine E, O’Toole M, Paton JY, Wallis C. Current status of long term

ventilation of children in the United Kingdom: questionnaire survey.

BMJ 1999; 318: 295e9.

Kohler M, Clarenbach CF, Boni L, Brack T, Russi EW, Bloch KE. Quality of

life, physical disability, and respiratory impairment in Duchenne

muscular dystrophy. Am J Respir Crit Care Med 2005; 172: 1032e6.

Lewis M, Noyes J. Risk management and clinical governance for complex

home-based health care. Paediatr Nurs 2007; 19: 23e8.

Mah JK, Thannhauser JE, McNeil DA, Dewey D. Being the lifeline: the

parent experience of caring for a child with neuromuscular disease

on home mechanical ventilation. Neuromuscul Disord 2008; 18:

983e8.

Noyes J. ‘Ventilator-dependent’ children who spend prolonged periods of

time in intensive care units when they no longer have a medical need

or want to be there. J Clin Nurs 2000; 9: 774e83.


Noyes J. Barriers that delay children and young people who are dependent

on mechanical ventilators from being discharged from hospital. J Clin

Nurs 2002; 11: 2e11.

Noyes J, Lewis M. From hospital to home. Guidance on discharge

management and community support for children using long-term

ventilation. Barnardo’s, http://www.barnardos.org.uk/from_hospital_

to_home.pdf; 2005.

Noyes J, Lewis M. Care pathway for the discharge and support of children

requiring long term ventilation in the community. National Service

Framework for Children, Young People and Maternity Services,

Department of Health, http://www.dh.gov.uk/en/Publicationsand

statistics/Publications/PublicationsPolicyAndGuidance/DH_4115098;

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Noyes J. Health and quality of life of ventilator-dependent children. J Adv

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Noyes J. Comparison of ventilator-dependent child reports of health-

related quality of life with parent reports and normative populations.

J Adv Nurs 2007; 58: 1e10.

Tearl DK, Cox TJ, Hertzog JH. Hospital discharge of respiratory-technology-

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http://www.barnardos.org.uk/from_hospital_to_home.pdf

http://www.barnardos.org.uk/from_hospital_to_home.pdf

http://www.dh.gov.uk/en/Publicationsandstatistics/Publications/PublicationsPolicyAndGuidance/DH_4115098

http://www.dh.gov.uk/en/Publicationsandstatistics/Publications/PublicationsPolicyAndGuidance/DH_4115098


organizing home ventilation

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