Clinical Nutrition ESPEN xxx (2016) e1ee5

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Clinical Nutrition ESPEN

journal homepage: http://www.clinicalnutritionespen.com

Original article

Current practices and experience of transition of young people on long

term home parenteral nutrition (PN) to adult services e A perspective
from specialist centres
E. Kyrana a, S.V. Beath b, S. Gabe c, M. Small c, S. Hill a, *, on behalf of the members BAPEN,
and of the
a
b
c

Paediatric Gastroenterology Department, Great Ormond Street Hospital, UK

The Liver Unit (Including Small Bowel Transplantation), Birmingham Children's 14 Hospital, West Midlands B4 6NH, UK
The Intestinal Failure Unit, St Mark's Hospital, Northwick Park, Watford Road, Harrow, Middlesex HA1 3UJ, UK

a r t i c l e i n f o

s u m m a r y

Article history:
Received 3 December 2015
Accepted 2 April 2016

Background: There has been an estimated vefold increase in the number of children receiving parenteral nutrition (PN) at home in the past 10 years with some children approaching the age when they
should be referred to adult services whilst still on treatment. Models of care for moving young people
onto adult providers of PN at home are not yet well developed, and transition is a potentially dangerous
time for young people with complex health needs.
Methods: A questionnaire to ascertain current experience and practices of transition in the context of
home PN services was dispatched to 170 consultant gastroenterologists who were members of the British
Association of Parenteral and Enteral Nutrition (BAPEN) and also to all 40 members of the Nutrition and
IF working group of the British Society of Paediatric Gastroenterology and Nutrition (BSPGHAN). Anonymised returns were received from 12 adult and 18 paediatric centres.
Results: We estimate about 50% paediatric IF centres, and the three largest adult IF centres responded to
the survey. We identied 14 young adults already transitioned and 43 currently in transition. The
practices and processes of transition reported were highly variable. Time taken to achieve transition
ranged from under 6 months up to 2 years. The most frequent concerns to be identied were confusion
around care routines and psychological problems at the time of transition (in one third of respondents).
Conclusions: We conclude that a transition pathway and service standards for adolescents on home PN
should be developed, consideration should be given to checklists for practical aspects (e.g. pumps), key
worker and psychology input to enhance emotional resilience of the young people and carers.
2016 Published by Elsevier Ltd on behalf of European Society for Clinical Nutrition and Metabolism.

Keywords:
Parenteral nutrition (PN)
Home parenteral nutrition
Adolescent transition

1. Introduction
The availability of parenteral nutrition (PN) over the last two
decades and improvements in managing patients on PN have
dramatically increased the survival of children with intestinal failure (IF). The number of children discharged home on PN appears to
have increased vefold in the last 20 years [1]. Although the majority develop intestinal autonomy with increasing age, a group of
these children are approaching the age when they should be
referred to adult services. Transition describes the process by which

* Corresponding author. Paediatric Gastroenterology Department, Great Ormond

10 Street Hospital, Great Ormond Street, London WC1N 3JH, UK.
E-mail address: susan.hill@gosh.nhs.uk (S. Hill).

medical care for adolescents with chronic disorders is handed over

from the paediatric to the adult team. Home PN services for children in the UK are organised regionally with 15 expert centres
(dened as a centre with 4 or more patients on home PN) providing
initial training, referral to home care teams and long term medical
supervision. Depending on the patient's geographical proximity to
the specialist centre, shared care arrangements with local hospitals
are undertaken.
Typically during childhood PN at home is supplied by a commercial homecare company. The PN is administered by parents/
carers who have undergone a formal training programme. The
parents/carers are also responsible for administering other medications, for attending medical appointments and for over-seeing
the activities and well-being of their child. Whereas patients on

http://dx.doi.org/10.1016/j.clnesp.2016.04.003
2405-4577/ 2016 Published by Elsevier Ltd on behalf of European Society for Clinical Nutrition and Metabolism.

Please cite this article in press as: Kyrana E, et al., Current practices and experience of transition of young people on long term home parenteral
nutrition (PN) to adult services e A perspective from specialist centres, Clinical Nutrition ESPEN (2016), http://dx.doi.org/10.1016/
j.clnesp.2016.04.003

e2

E. Kyrana et al. / Clinical Nutrition ESPEN xxx (2016) e1ee5

Abbreviations
BAPEN

British Association for Parenteral and Enteral

Nutrition
BSPGHAN British Society of Paediatric Gastroenterology,
Hepatology and Nutrition
BIFA
British Intestinal Failure Alliance
IF
intestinal failure
PN
parenteral nutrition
SBS
short bowel syndrome

30 were the specialist centre providing the PN at home and ve

shared care. Seventeen used a single home care company to
formulate and deliver the PN to the home whereas 12 used more
than one company.1 Seven of the respondents replied that they
share care home PN patients with specialist units and 11 replied
that they share care home PN patients with non-specialist units.
Thirteen of the 18 paediatric respondents had referred patients
to an adult specialist unit. Twenty-two of the 30 adult and/or
paediatric had referred patients to an intestinal transplant
centre.
3.2. Home PN patient demographics

home PN are aware of their condition and do grow up with various

restrictions to their activities, they can develop a level of resilience,
maintain a positive outlook and cope well with illness related demands [2]. Children attend school and participate in usual childhood activities other than contact sports. Nevertheless adulthood
poses certain challenges for them as they take on the responsibility
of administering the PN themselves. They will need to be trained in
the administration of home PN and to clearly comprehend what
poses a risk to their health and well-being. At the same time the
young person is having to deal with the usual challenges of taking
on responsibilities and might be moving away from their family to
work/attend university.
For the above reasons, transition is a potentially dangerous time
for young people with complex health needs and can be associated
with excess mortality [3]. This survey was undertaken jointly by
BAPEN and BSPGHAN. The aim was to ascertain current experience
and practices of transition in the context of home PN services and
gauge future demand for transition services. A secondary aim was
to raise awareness in the adult and paediatric sectors of the
growing number of young people likely to need home PN support
from adult services in the near future.
2. Methods
A questionnaire was designed using the electronic Survey
Monkey Format and dispatched via e-mail in April 2013 to 170
British Intestinal Failure Alliance (BIFA) members (a special interest
group within BAPEN of gastroenterologists with expertise in
managing intestinal failure (IF)), and to all 40 BSPGHAN (British
Society of Gastroenterology, Hepatology and Nutrition) members of
the Nutrition and IF working group. Please see Table 1 for the details of the questionnaire. It was composed of ten questions and the
respondents had space to add in free text comments for each
question. Replies were received anonymously.
Ethical approval for the survey was not required as no patient
identiable data were collected.
3. Results
3.1. Characteristics of home PN providers and referring patterns
Responses were received from 30 different centres, 12 adult and
18 paediatric. The adult centre respondents were either gastroenterology consultants (n8) or nurse specialists (n4). The paediatric
replies were from paediatric consultants (n12), paediatric nurse
specialists (n4) and dietitians (n2). None of the respondents were
pharmacists.
The second question looked at the interaction of the respondent's unit with other care providers. For all 30 respondents
their unit provided PN and home PN services. Twenty-ve of the

Question 3 and 4 asked about the age distribution of the patients and aetiology of IF. The centres were asked to report on the
size of the centre and the age range of patients cared for in one
year, rather than precise gures (i.e. 0e4, 5e9, 10e20, 21e50,
>50 pt) e (see Table 2). The bold numbers in the cells represent
numbers of centres, e.g. 12 centres report they have 1e4 patients
each (12e48 in total) within the age bracket of 11e19 years, 1
centre reports 5e9 patients within the same age bracket and
another one centre reports 10e20 patients. We would estimate
that a minimum of 27 patients to a maximum of 77 are at the age
of 11e19 years and are therefore soon to require or are undergoing transition. The main underlying cause for IF was short
bowel syndrome (n 378, 61.8%); enteropathy (n 96, 15.7%);
Motility disorder (n 84, 13.7%) and other (n 54, 8.8%) e total
patients with IF reported on was 612.
3.3. Centres' experience of transition
Sixteen paediatric professionals (89%) reported that they had
initiated transition for 43 patients in the last 5 years and the 12
adult professionals had received 14 referrals from paediatric services to continue PN at home. Twenty-eight respondents (both
adult and paediatric) reported that 41 patients had completed the
process of transition in the last 5 years.
Twenty-one respondents reported on the average length of the
transition process for their centre. For four (19%) it was less than 6
months, for 7 (33.3%) it was 6 months to 1 year, for 7 (33.3%) it was
1e2 years, for 1 (4.8%) it was more than 2 years and for 2 (9.5%) it
was something other than the above time frames.
Question 7 addressed the issues of complications occurring
during the transition process, based on their experience with patients that had been transitioned. The respondents had to decide if
the listed complications happened hardly ever, sometimes,
more than expected or frequently. The seventeen responses to
this question are shown in Table 3. Psychological problems and
confusion about catheter care and administration of PN between
the adult and paediatric centre (4 respondents) were considered
frequent (2/16 and 4/17 respondents respectively). The most common problems to occur more than might be expected were hospital
admissions and psychological problems (3/17 and 4/16 respondents
respectively). Two respondents thought academic performance had
deteriorated more than might be expected and 2 respondents
thought there was more than confusion about changes in management of care than would be expected. Frequency of catheter
related blood stream infections was not greater than expected by

1
By company we mean a homecare company that manufactures and distributes
PN to patients on treatment at home. The manufacturing unit can obtain stability
for 21e28 days on certain individually formulated/bespoke prescriptions and
usually delivers the PN with the necessary ancillary equipment to the home on a 2
weekly basis.

Please cite this article in press as: Kyrana E, et al., Current practices and experience of transition of young people on long term home parenteral
nutrition (PN) to adult services e A perspective from specialist centres, Clinical Nutrition ESPEN (2016), http://dx.doi.org/10.1016/
j.clnesp.2016.04.003

E. Kyrana et al. / Clinical Nutrition ESPEN xxx (2016) e1ee5

e3

Table 1
The questionnaire.
Domain

Questions

a) characteristics of HPN providers and

referring patterns
b) Home PN patient demographics

1. Where do you work and at what grade?

2. How does your HPN service interact with other care providers?
3. As of January 2012 until December 2012 please estimate how many of your patients on long term home PN (i.e. anticipated
requirement for HPN for more than 12 months) fell into the following age categories: 0e10 years, 1119 years, 20e30 years or
older than 30 years.
4. How many of your patients on long term HPN January 2012 to December 2012 fell into the following aetiological categories (if
more than one choose the main/dominant problem causing intestinal failure)
Short bowel syndrome
Enteropathy
Motility disorder
Other?
5. In the past ve years (since January 2008) how many patients have you begun or completed transition to adult services for
long term HPN?
6. On average how long do you nd transition takes from rst discussions with the patient to completion?
7. In your experience, on average do the following complications occur during the period of transition from paediatric to adult
services: catheter related blood stream infections, hospital admissions, psychological problems and/or poor emotional
adjustment, deterioration in academic performance and confusion about differences in catheter care and administration of
PN?
8. Do you use any of the following transition tools?
E.g. key worker contact pre clinic; designated clinic; hand held record
9. Do you have any observations about your experience for patients on long term HPN?
Things that worked well
Things that went wrong
Developments planned in future
Transition tools which you used not previously mentioned
Did the experience of transition have a positive or negative impact on your patients?
10. For adult gastroenterologists do you insist that the patient has to:
Stay with the same home care company and same coordinator
Change home care company
Adopt new procedures Change their pump?

c) Centre's experience of Transition

Table 2
Centre size and age ranges of patients in follow up (as of April 2013).
Very small size centresa Small size centresb Medium size centresc Large size centresd Total

Age range
Paediatric HPN providers 0e10yrs
min e max
11e19yrs
min e max
Adult HPN providers
20e30yrs
min e max
>30yrs
min e max
a
b
c
d
e
f

5 centres
no. of patients in follow up (5e20)
12 centres
no. of patients in follow up (12e48)
9 centres
no. of patients in follow up (9e36)
0
no. of patients in follow up

4 centres
(20e36)
1 centre
(5e9)
1 centre
(5e9)
2 centres
(10e18)

4 centres
(40e80)
1 centre
(10e20)
1 centre
(10e20)
5 centres
(50e100)

2 centres
(>40)
0e
e
0e
e
6f centres
(>120)

15
(85e176)
14
(27e77)
11
(24e65)
11
(180e318)

Centre size: 1e4 patients on HPN.

5e9 patients on HPN.
10e20 patients on HPN.
More than 20 patients.
Three centres skipped this question on numbers of patients according to age range.
One centre reported on >200 patients.

Table 3
Reported complications.

Hospital admissions
Catheter related blood stream infections
Psychological problems
Deterioration in academic performance
Confusion about care outcomes

Hardly ever

Sometimes

More than expected

Frequently

12.5%
53%
13%
47%
29%

50%
47%
50%
40%
35%

25%
0%
25%
14%
12%

12.5%
0%
12.5%
0%
24%

any respondent with 9/17 or 53% reporting that infections were

hardly happening at all.
Question 8 looked more specically into transition practices
and 20 of the 30 respondents reported on their practices
(Fig. 1). Ten reported that a transition nurse/key worker
attended clinic; 9 had a designated clinic attended by the
paediatric and adult physician; eleven reported there was preclinic contact between the transition key worker and the patient; ve said there was post-clinic contact by the transition
worker and the patient. Two have given the young person their
own hand held record.

Question 9 asked about the professionals' experiences of what

did and did not work well, what potential future developments
were planned and if they thought the overall transition process had
a positive or negative impact on their patient. Practices that were
reported to work well were enabling easy access to both teams for
the young person, remaining with the same home care company,
using the same pump. Good transition also included organising
meetings with adult physicians outside the clinic environment and
an orientation visit for the young person to the adult ward and
clinic. Joint clinics at both the paediatric and adult settings also
worked well.

Please cite this article in press as: Kyrana E, et al., Current practices and experience of transition of young people on long term home parenteral
nutrition (PN) to adult services e A perspective from specialist centres, Clinical Nutrition ESPEN (2016), http://dx.doi.org/10.1016/
j.clnesp.2016.04.003

e4

E. Kyrana et al. / Clinical Nutrition ESPEN xxx (2016) e1ee5

Fig. 1. Current practices in transition. OPD: outpatient department, YP: young person.

Adverse events included emergency admissions to the new unit

prior to completion of the transition process, when the young
person and/or family had unrealistic expectations from the adult
service. Uncertainty as to who was responsible for each decision at
a given time point was a problem and one family felt unwelcome
when they arrived at a deserted outpatients department.
Question 10 was aimed at adult physicians, and answered by six
respondents. Four reported that patients stayed with the same
home care company and care coordinator during transition and
none insisted they change home care company, although one
insisted they adopt new procedures. No centre insisted that the
infusion pump be changed even though 2 reported this sometimes
became necessary.
4. Discussion
We estimate that about 50% of paediatric IF centres, and the
largest adult IF centres responded to the survey. This low response
rate probably reects the fact that while the vast majority of BIFA
members provide a wide range of nutritional services and treatments, the provision of adolescent home PN is concentrated in a
relatively small group of larger centres. Since the aim of this survey
was to ascertain how centres which provide home PN services were
managing transition of young people to adult services and to capture information on pitfalls and examples of good practice, the good
response from the larger centres compensated for the lack of responses from the small units not actively participating in transition.
The number of teenagers approaching transition is currently
around 52 with a similar number of young adults (44) aged
20e30yrs who are currently receiving PN. The numbers are
approximate, because the centres were asked to report on average
numbers for the year, but they do illustrate the fact that paediatric
centres tend to be small or medium in size (only 2/18 had more
than 20 patients on PN at home) and the adult centres which
responded were mainly medium or large in size. This point is
important because the experience of moving to a large, possibly
more impersonal, centre can be very daunting for patients after
being accustomed to receiving personalised care in a family centred
paediatric unit. Not surprisingly the most common reason for the IF
was short bowel syndrome (SBS). The relative frequency of SBS has
increased strikingly in children since 1993 [1].
The reported practices and processes of transition were highly
variable. The time taken over transition ranged from 6 to 24
months. The timing of the transition process is also an important

issue. It has been previously highlighted that the focus of transition

should be the achievement of competency in self-care by the young
person and should not be driven by government or hospital driven
age based policies [4]. Ideally transition should be completed after
the young person's growth potential is achieved. Growth and pubertal delay are common in children on articial nutrition and can
be delayed until after the age of 18 years in some conditions in this
particular group of children.
One third of respondents identied confusion around care
routines and psychological problems at the time of transition. Clear
and repeated communication seems to be the obvious key to
addressing issues of confusion [5]. Explaining the transition process
to the young person and their family and dening goals at the start
can help manage their expectations and facilitate the transition
process. It is usual for these families to be heavily involved in the
care of their loved one and this change in dynamics can be a major
source of stress. Checklists of the desired competencies may ensure
that objectives are met and can help track progress. Joint meetings
with the paediatric and adult teams is another effective way of
minimising communication gaps and visits to the adult settings can
help minimise anxiety. Written information is of paramount
importance. Care plans with the names and contact details of the
relevant professionals with corresponding responsibilities can help
guide the young person and their family at times of crisis. Where
there has been a change in equipment written information on their
uses and troubleshooting guides can also be valuable. Hand held
health records could also prove invaluable, but from our survey it
became apparent that this is not a common practice amongst respondents. Having a keyworker as a point of contact for the young
person can facilitate good communication [5]. The key-worker, who
would usually be a nurse specialist, can be involved in coordinating and organising the transition process from a practical
point of view, can attend clinics with the young person, can contact
the young person before and after clinics and can be the link between the young person and the new services taking over the
young adult's care.
Another aspect of transition involves educating the young person about their illness. Many of these patients have been unwell
from a young age and some may not remember the early stages of
their condition when they may have been more unwell. They may
have a limited understanding of their condition and its management. This could mean that they may not fully understand the
serious implications of non-compliance and this could jeopardize
their well-being. There have been reports were even a one hour
education session led by a nurse or key worker can help the young
person more efciently through the transition process [6].
The need for psychology input was also highlighted by the
survey. The transition process generates a lot of anxiety for the
young person and their family. Every case is different and the needs
vary, but a mechanism for access to psychology input should exist.
Whereas there is no indication that young people with IF have more
or different psychological issues to their peers in adolescence,
when they do have problems the added burden of accepting and
managing a chronic illness may hinder their coping mechanisms.
This survey had some limitations. It was not designed to record
the patient's experience of transition. A survey looking into the
patient experience would be helpful in adding insight that would
help plan for better, more efcient transition services in the future.
In addition its design meant that duplicate responses could not be
ruled out, although the individual replies were sufciently distinct
so as to make this an unlikely event. Also it did not allow for some
additional information to be reported e.g. the specic diagnosis of
the teenagers in transition or the reason of the hospital admissions
in the complications section. The survey design was purposefully
kept simple to allow for better engagement with widely different

Please cite this article in press as: Kyrana E, et al., Current practices and experience of transition of young people on long term home parenteral
nutrition (PN) to adult services e A perspective from specialist centres, Clinical Nutrition ESPEN (2016), http://dx.doi.org/10.1016/
j.clnesp.2016.04.003

E. Kyrana et al. / Clinical Nutrition ESPEN xxx (2016) e1ee5

services caring for young adults on PN at home e even so, three

centres skipped the question about numbers of patients stratied
according to their age. Prior to this survey there has been limited
awareness and understanding of the extent and the importance of
the problems of transition on PN treatment. One of the strengths of
the survey was that it has raised awareness of the need to make
provision for transition of young adults with IF to adult services.
In summary, better service standards relating to timing of initial
stages of transition and employment of a key worker for each case
should be developed. This would help centres from a practical point
of view to develop a pathway of care for transition, especially those
smaller centres who engage in transition relatively infrequently e
12/18 paediatric respondents reported that they looked after 4 or
less young people aged 11e19yrs. A step by step process would
include check lists for the practical aspects (e.g. pumps, handheld
health records, who's who lists) as well as psychology input to
enhance emotional resilience of the young people and support for
carers. A NICE report on Transition from children's to adult services is due to be published in 2016. Although this will be a generic
document, it will provide a framework from which to work on
development of service standards for young people with IF on
home PN. It is anticipated that the NICE report will include evidence around young people's experiences of transition since this is
mentioned in their scope consultation table. As previously stated,
the use of PN has radically changed outcomes for children with IF, to
such an extent that increasingly they are reaching the age for
transition to the adult services and can expect to have a good
quality of life and adult life expectancy. It is within the responsibilities of their physicians and carers to help them make this
transition from dependent children to responsible, informed,
autonomous adults.
In conclusion we would like to highlight ve major points,
1. transition can take as long as two years and is greatly facilitated
by the appointment of an identied key worker for the young
person
2. psychological issues need to be addressed prior to transition
3. written information can ensure clarity about all aspects of care
4. communication between the paediatric and adult centre is
facilitated with at least one patient consultation where a professional from each centre is present
5. aim to keep the same infusion pump after transition

e5

Statement of authorship
EK wrote the original manuscript and has presented this work in
3 conferences. SB, SG, MS and SH were involved in the design and
undertaking of the survey and have reviewed and edited the
manuscript.
Conict of interest
The authors have no conict of interest to declare. SH has
participated in a Calea advisory board.
Funding
No funding was obtained.
Acknowledgements
We would like to acknowledge the help from Dr Janet Baxter
and Professor Jeremy Powel- Tuck for providing access to the BIFA
email list and distribution, also Dr Sue Protheroe and Dr Mark
Beattie for advice and support from BSPGHAN, and from Sarah
MacDonald for advice from the allied health professional group. We
would also like to thank the following colleagues: D Basude, M
Cosgrove, S Cunningham, I Davies, A Fagbemi, D Flynn, C Holden, J
Koglmeier, S Naik, and J Puntis who made returns and gave feedback and many others who participated anonymously.
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Please cite this article in press as: Kyrana E, et al., Current practices and experience of transition of young people on long term home parenteral
nutrition (PN) to adult services e A perspective from specialist centres, Clinical Nutrition ESPEN (2016), http://dx.doi.org/10.1016/
j.clnesp.2016.04.003