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Patient safety vulnerabilities for children with intellectual
disability: A systematic review and narrative synthesis
Journal: BMJ Paediatrics Open
Manuscript ID bmjpo-2017-000201
Article Type: Original article
Date Submitted by the Author: 30-Aug-2017
Complete List of Authors: Mimmo, Laurel; Sydney Children's Hospital Randwick, Clinical Governance Harrison, Reema; University of New South Wales - Randwick Campus, School of Public Health and Community Medicine, Faculty of Health Hinchcliff, Reece; University of Technology Sydney Faculty of Health, Centre for Health Services Management
Keywords: Health Service, Patient perspective, General Paediatrics, Multidisciplinary
team-care, Children's Rights
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TITLE PAGE
Manuscript Title
Patient safety vulnerabilities for children with intellectual disability: A systematic review and
narrative synthesis
Corresponding author
Laurel Mimmo
Bright Alliance Building Level 9
Sydney Children’s Hospital
High Street, RANDWICK NSW Australia 2031
Email: [email protected]
Telephone: +61 2 93825660
Names and affiliations of contributing authors
Laurel Mimmo
Clinical Governance Unit, Sydney Children’s Hospitals Network, Sydney, Australia
Reema Harrison
School of Public Health and Community Medicine, Faculty of Health, University of New
South Wales, Sydney, Australia
Reece Hinchcliff
University of Technology Sydney, Centre for Health Services Research, Ultimo NSW 2007,
Australia
Word count for the text of the manuscript
3325
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PATIENT SAFETY VULNERABILITIES FOR CHILDREN WITH INTELLECTUAL DISABILITY: A
SYSTEMATIC REVIEW AND NARRATIVE SYNTHESIS
ABSTRACT
Purpose: Adults and children with intellectual disability (ID) are vulnerable to preventable
morbidity and mortality due to poor quality healthcare. Whilst poor quality care has been
commonly identified amongst children with ID, evidence of the patient safety outcomes for
this group is lacking and therefore explored in this review.
Data sources: Systematic searches of six electronic bibliographic research databases were
undertaken from January 2000 to July 2016, in addition to hand searching.
Study selection: Keywords, subject headings and MeSH terms relating to the experience of
iatrogenic harm during hospitalisation for children with ID were used. Potentially relevant
articles were screened against the eligibility criteria.
Data extraction: Data regarding: author(s), publication year, country, sample, health service
setting, study design, primary focus, and main findings related to measures of quality and
safety performance were extracted.
Results of data synthesis: Sixteen studies met the inclusion criteria, with three themes
emerging: the impact of the assumptions of health care workers (HCWs) about the child
with ID on care quality and associated safety outcomes; reliance on parental presence
during hospitalisation as a protective factor; and the need for HCWs to possess
comprehensive understanding of the IDs experienced by children in their care, to
scientifically deduce how hospitalisation may compromise their safety, care quality, and
treatment outcomes.
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Conclusion: When HCWs understand and are responsive to children’s individual needs and
their ID, they are better placed to adjust care delivery processes to improve care quality and
safety during hospitalisation for children with ID.
Keywords: Intellectual disability, Health service; Patient perspective; General Paediatrics;
Multidisciplinary team-care; Children’s Rights
What is already known about this subject?
• Approximately 1% to 3% of all children globally have Intellectual Disability (ID).
• Adverse events during hospitalisation are common, occurring in approximately 9.2%
to 36.7% of children.
• People with ID are admitted to hospital more frequently than their peers, but rates
and antecedent factors of adverse events amongst this population are unknown.
What this study hopes to add?
• To minimise adverse events amongst children with ID, and promote greater equity in
care, it is necessary to understand the specific risks associated with care for this
population.
• The synthesised literature presented shows that when healthcare workers
understand the child with ID and how hospitalisation can increase their vulnerability
to iatrogenic harm, disparate treatment outcomes can be avoided.
• Healthcare workers can improve care quality and optimise treatment outcomes for
these children during hospitalisation by engaging with parents/carers when planning
care.
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INTRODUCTION
The provision of safe, effective and high quality health care is an unalienable human right
(1). Poor quality care during hospitalisation is associated with iatrogenic harm and adverse
events (AEs) (2, 3) making it a key global concern (4). The estimated rate of AEs in
Organisation for Economic Co-operation and Development countries is 10 percent of
hospitalised adults, which is thought to be conservative (2, 5) . In the paediatric context,
reported rates of AEs for children during hospitalisation vary internationally from 9.2% in
Canada (6), 14.2% in the United Kingdom (UK) (7), and 36.7% in the United States (US) (8).
Children are vulnerable to AEs due to unique iatrogenic risks; for example in children, drug
dosage calculations are weight-based, commonly resulting in medication errors (3).
Greater vulnerability to poor quality care during hospitalisation is evident in particular
populations (4). People with Intellectual Disability (ID) have higher rates of preventable,
premature mortality than the general population (9, 10) and experience poorer quality care
during hospitalisation (11, 12). This represents a clear health inequity for people with ID (9,
13), exacerbated by the challenges in identifying and reporting patient safety issues
amongst this population (12), which make it difficult to quantify their incidence of AE.
Children with complex medical needs are reported to experience higher rates of AEs and
prolonged admissions during hospitalisation (6, 14). For children with ID, the possibility of
experiencing iatrogenic harm is compounded by the unique vulnerabilities of children to AEs
during hospitalisation (3, 14), and the poor care quality experienced by patients with ID (9,
11). It is imperative to determine and address the features of hospitalisation that increase
the likelihood of children with ID experiencing poor quality care, which manifest as
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iatrogenic harm and AEs. The vulnerability of children with ID to experience poor quality and
less safe care is an ethically- and economically-significant issue, yet one that is under-
examined.
This study aimed to provide a narrative synthesise of published evidence concerning the
experience of iatrogenic harm during hospitalisation for children with ID to discern the
evidence base and knowledge gaps. The review findings highlight opportunities for policy
changes and quality improvement (QI) interventions that could enhance the quality of care
delivered to children with ID internationally.
METHODS
The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)
statement is an evidence-based approach for reporting in systematic reviews and meta-
analyses that was used to guide the reporting of this review (15).
Eligibility criteria
Inclusion criteria
Since the 1999 publication of the pivotal work, To Err is Human, patient safety research has
intensified in magnitude and scope (2). As such, we searched for publications in English
published since 2000 that met the criteria listed below.
Participants: Children (<18 years of age) in hospital as inpatients, aligning with the United
Nations definition of child (16). Included publications were required to reference children
with ID. This could include either a specific condition known to include ID, such as Down
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Syndrome (DS), or terms that are synonymous with ID such as cognitive impairment,
learning disability or developmental disability (13).
Intervention: AEs, which could be described using any of the following terms: adverse event,
medical error, near miss, adverse incident, unsafe care, patient safety incident, or problems
with quality of care.
Comparator: Clinical outcomes for children without ID of a care process known to require
hospitalisation.
Outcomes: Either clinical outcomes for children with ID of a procedure known to require
hospitalisation, self- or family/carer-reported outcomes of such care experiences, or any
other terms referring to objective or subjective measures of the quality and safety of
inpatient health care.
Exclusion criteria
Publications were excluded if they were published prior to 2000, were not available in
English, did not focus on patient safety or quality in children who were inpatients and under
18, or did not include reference to children with ID in the abstract. Publications of the
following issues were excluded:
• primary care settings;
• inpatient mental health settings;
• risks of hospitalisation due to pre-admission patient factors;
• hospital admissions of less than 24 hours;
• effectiveness of surgery without a non-ID comparator;
• errors in diagnosis of ID or medical negligence;
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• patient/parent satisfaction with healthcare services, which did not focus on adverse
safety events;
• case reviews;
• or rates of hospitalisation and/or healthcare utilisation by children with ID.
Publications that did not include analysis of impacts on care quality and patient safety were
excluded. The final exclusion criteria ensured that included publications focused on
inpatient care, as short stay contexts present other Q&S concerns that warrant separate
research (2).
Study identification
A range of text words, synonyms and subject headings for the major foci of this study were
used to undertake a systematic search of six electronic databases from January 2000 to
August 2016. An initial search was conducted in Medline to refine the search terms (see
Appendix 1 for search terms). The remaining searches were conducted from August 5th
to
August 25th
, 2016, involving: MEDLINE, EMBASE, CINAHL, PsychInfo, Scopus, and Web of
Science. Hand searching of reference lists of included publications was also undertaken to
ensure that all relevant sources were included for data extraction and synthesis.
Study selection and data extraction
Titles and abstracts were screened, and a copy of the full paper was obtained for relevant
articles. The following data were extracted from eligible publications: author(s), publication
year, country, sample, health service setting, study design, and main focus. Where available,
data extraction included main findings related to measures of Q&S performance, such as
length of stay, and morbidity and mortality outcomes, as well as any self-reported
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experiences of adverse events or iatrogenic harm, unsafe care or concerns with quality of
care.
Assessment of the quality of the studies
The quality of included publications were assessed by the authors using the Quality
Assessment Tool for Studies with Diverse Designs (17). Using this tool the authors assessed
the study quality of the included publications using a four-point scale (0-3) against 16
criteria to indicate the quality of each publication and the overall body of evidence. One
reviewer (LM) individually assessed all publications and a second reviewer independently
assessed a subset of the publications; disagreements were resolved through discussion
resulting in substantial agreement (κ= 0.75) between reviewers on a random sample of 25%
of the papers. Because there were few publications, we did not exclude publications based
on the quality assessment. Quality assessment data was used to explore the strength of the
available evidence.
Narrative synthesis of key themes of included publications
The findings of included publications were analysed using a narrative synthesis approach,
based on the study objectives, to elicit key themes articulated in the literature (18). A
narrative approach was necessary to synthesise the heterogeneous qualitative and
quantitative findings (19). Overarching descriptions of each publication were tabulated
(Table 1 supplementary), then patterns in the data were explored to identify consistent
themes across the publications. Regular discussions amongst the study team helped refine
the analysis and enhance study validity.
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RESULTS
Title and abstract screening resulted in the identification of 23 publications that fulfilled the
inclusion criteria. From these, 11 publications were eligible based on the full text review,
with a further five identified from reference list searches. A total of 16 publications were
included in the review (see Figure 1).
Characteristics of included studies
A list of included studies, with titles, aims, methodology, participants and main findings with
key themes, is displayed in Table 1. Eleven publications were published since 2010. All were
set in specialist paediatric units, and all but one, in high income countries. Seven
publications were from the US, five from the UK, two from The Netherlands, and one each
from Canada, India and Japan.
Twelve publications used quantitative methodologies, though no publication specified rates
of iatrogenic harm or AEs amongst children with ID. In eight publications, data was
extracted from a single centre, three publications used data from a national database, and
one was a multicentre study. Four publications used qualitative methods to explore the
experience of hospitalisation for children with ID within a specific health service, without
comparator populations.
While the study population of interest was all children with ID, 10 publications were specific
to children with DS, with the remaining six inclusive of any ID condition. Ten publications
compared treatment outcomes during hospitalisation between children with and without
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DS, and two publications compared outcomes between children with different types of ID or
special needs.
Study quality
Diverse quality was seen across publications when assessed against the quality criteria (17)
(see Table 2). Most provided theoretical background to the study, and descriptions of the
methods for data collection and analysis. All but one publication relied on opportunistic
sampling through a single centre, or retrospective data from existing databases.
Subsequently, sample sizes were wide-ranging, though study populations were generally
well described. Discussion of limitations varied; most gave concise critiques, though two
publications lacked any critique of limitations.
Table 2: Criteria study quality assessed against
Quality Criteria
Explicit theoretical framework
Statement of aims/objective in body of report
Clear description of research setting
Evidence of sample size considered in terms of analysis
Representative sample of reasonable size
Description of procedure for data collection
Rationale for choice of data collection tool
Detailed recruitment data (no. approached, declined etc.)
Statistical assessment of reliability & validity of measurement tools
(quantitative)
Fit between study objectives & method of data collection
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Fit between study objectives & content of data collection tool
Fit between study objectives and method of analysis
Good justification for method of analysis
Assessment of reliability of analytic process (qualitative)
Evidence of user involvement in design (e.g. pilot work)
Strengths & limitations critically discussed
Reproduced with permission
Key themes
Three main themes were identified: the impact of HCWs assumptions about the child with
ID on care quality and associated safety outcomes; reliance on parental presence during
hospitalisation as a protective factor from poor care quality and safety outcomes; and the
need for HCWs to understand the child and the ID to know how hospitalisation may
compromise their safety, care quality, and treatment outcomes. Each theme is described
below.
The impact of HCWs assumptions about the child with ID on care quality and associated
safety outcomes
Labels, assumptions and stereotyping were described in three publications, which had
positive and negative impacts on care provision (20-22). Having a diagnosis or label of an ID
can be beneficial for parents to negotiate their child’s care needs with HCWs (21) and for
early diagnosis to maximise treatment outcomes of comorbidities (23). Assumptions
however, made by HCWs regarding the child’s behaviour, cognitive ability, or experience of
pain may mean they do not understand the unique needs of each child (20-22, 24).
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Generalisations regarding analgesic requirements or behaviour led to experiences of poor
quality care and adverse outcomes during hospitalisation for the child with ID (21, 25).
The influence of subtle assumptions and stereotyping of children with ID highlighted in pain
assessment (26). Children with ID had lower pain scores and reduced administration of
analgesia than children without ID (24, 25, 27, 28), and two publications suggest this may be
due to HCW’s preconceptions (24, 25). Several authors commented their findings challenged
long held beliefs regarding the benefit of surgical interventions to treat comorbidities in
children with DS (29-32), or effective assessment and management of pain in children with
ID (24, 27, 28).
Several publications found that parents identified ready access to healthcare, effective
communication between patients and HCWs, and positive treatment outcomes, as being
dependent on the assumptions, stereotypes and judgements of HCWs (20-22).
Parents/carers consistently reported that assumptions, stereotypes and judgements about
their child led to poor care experiences and safety concerns (20, 21), compounded by a lack
of effective communication (21, 22). Four publications found, to some extent, that
parents/carers felt HCW judgements about them or their child with ID influenced the quality
of care their child received (20-22, 33).
Reliance on parental presence during hospitalisation as a protective factor from poor care
quality
HCWs reliance on parental presence to supervise, protect and advocate for the care of their
child was described in four publications (20-22, 34); and to assist in the assessment of pain
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in two publications (25, 27). HCWs reliance on parents also manifest as an expectation for
parents/carers to manage their child’s medical needs and behaviour during hospitalisation
(22, 34). Parents felt this reliance was frustrating when negotiating their child’s care needs
with HCWs (20, 21). Aston et al. (2014) identified this tension; HCW’s relied on parental
presence during hospitalisation, only to judge the parent when they provide a lot of detail
(21). As one parent said, “I found as soon as I started to talk and say what James’ meds
were, what his seizure activity was, that he was non-verbal, I didn’t stop […] and it was just
like all of a sudden “well she’s a know-it-all” and that’s not my intent, my intent was so that
people knew James.” (21, p.301).
To overcome this reliance, parents/carers felt it was important that HCWs listened to them
and viewed them as the expert of their child (20, 22, 33). In knowing more about the child
and their ID label by listening to parents, several publications found HCWs can adapt the
hospital environment, such as bed location, and negotiate the child’s care needs, to
minimise poor care experiences during hospitalisation (20-22). When HCWs understood the
complexities of their child’s medical needs in the context of the current hospitalisation (20,
22, 34) reliance on parental presence could be lessened.
Need for HCWs to understand the child and the ID to know how hospitalisation may
compromise their safety, care quality, and treatment outcomes
Having specific knowledge about a child’s ID can mean HCWs identify responses or
physiological susceptibilities to the hospital environment that impact on treatment
outcomes (24, 26, 27, 35), and may have a protective value against poor morbidity and
mortality related to treatment (23, 27, 29). When HCWs apply knowledge of the child with
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ID and make adjustments to care delivery and the hospital environment during
hospitalisation, it may reduce poor quality care and clinical complications (22). For example,
understanding of how known immunity issues in DS contributes to post-operative infections
was identified in four publications (23, 26, 31, 36), though mitigation strategies were not
reported. One publication noted that this susceptibility may have contributed to additional
intensive care support in children with DS (23). Another publication suggested that
knowledge of DS could be protective, finding children with DS “are predisposed to
complications and thus warrant more cautious management” (31, p.181).
Parents generally perceived ID labels as important for HCWs to know, as the hospital and
HCWs then knew something about the needs of their child to ensure quality care (21, 22).
Two publications reported HCWs must understand the child with ID and their needs during
care planning to optimise the care quality during hospitalisation (20, 22). Aston et al. (22)
comment that reducing the vulnerability of children with ID to poor quality care goes
beyond educating HCWs, necessitating broader social and hospital system change (21).
DISCUSSION
The aim of this research was to synthesise published evidence regarding the experience of
iatrogenic harm during hospitalisation for children with ID. Sixteen publications, comprising
seven from the US, five from the UK, two from The Netherlands, and one each from Canada,
India and Japan, met the inclusion criteria. There is evidence that HCWs stereotype
behaviours (20-22) and overlook factors associated with ID that predispose the child to poor
quality care and harm (21, 36).
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Behaviours of the child with ID during hospitalisation may be interpreted or stereotyped as
normal, when they are in fact an expression of pain or discomfort (25, 27). Assumptions
around the experience of pain or behaviour may result in unnecessary respiratory
compromise, admission to intensive care, and prolonged hospital admission for the child
with ID (24, 26). This demonstrates there are specific aspects of hospitalisation that expose
children with ID to harms that are preventable, avoidable, and not experienced to the same
extent by children without ID.
Implications
An awareness of the child and their ID, or having a label of ID, was seen to be beneficial (21,
22), and may even be protective against poor outcomes (23, 27, 29, 31). Several
publications noted that children with DS are understood to have compromised immunity
(23, 26, 31, 36), which can increase their susceptibility to healthcare associated infections,
compared to children without DS. This is assumed to be the reason children with DS are
shown to have more non-cardiac complications post operatively, such as respiratory illness
and surgical site infections (23, 31, 36). Exploration into care quality factors of
hospitalisation that increase infection risk for children with DS would add to the knowledge
base for this population.
Inadequate or insufficient measures of pain and pain management were also identified,
resulting in respiratory complications or prolonged hospital admission (24, 27). While pain
management was identified as a particular challenge for this group, only four publications
explored this, suggesting evidence to understand the impact of inadequate pain
management on treatment outcomes in children with ID is warranted.
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Broad assumptions on the utility of safe and equitable care for children with ID were also
found (25, 29-31). This suggests HCWs infrequently recognise that children with ID have
unique care needs during hospitalisation to ensure safe quality of care (20, 22). There is
evidence of reliance on parental presence to care for, advocate and speak for their child
(20-22, 34); and parents were often relied on to assess their child’s pain (25, 27). This
reliance infers that parents and hospital staff share anxieties about the child with ID being
left alone, unprotected, in the hospital environment (20). Exploration into how beliefs and
values held by HCW and parents around healthcare Q&S for children with ID influence the
experience of care quality is another area of potential future study.
The paucity of research into the hospital experience of iatrogenic harms poses a challenge
to reducing health inequities for children with ID. Many papers excluded from this review
reported on the frequency of hospitalisations, healthcare utilisation, length of stay and/or
complexity of healthcare needs for the child with ID (37-44), but did not report on the
influence of healthcare Q&S on these factors. The experience of iatrogenic harms for
children with ID in different contexts, such as hospitalisation for Mental Health treatment,
primary care and/or outpatient treatment, and day only treatment, are particularly lacking.
While individual teams and disciplines have devised various interventions to mitigate the
believed perils of hospitalisation, QI interventions and policy changes that are not evidence-
informed may ultimately fail to improve, or even possibly worsen, the problems they are
designed to address (45). Without a clear picture and scale of the problem, policy makers
and healthcare leaders are ill-equipped to mandate change.
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Globally, partnerships with consumers to optimise healthcare Q&S, improve patient
outcomes, and reduce healthcare costs are increasing (2, 4), strengthened by international
recommendations for HCW training in patient engagement (46). The study findings
complement this shift, indicating that training HCWs to partner with families of children
with ID in planning and implementing their healthcare can have a positive impact on the
care quality experience (22). This could be integrated into formal clinical training programs
for health professionals, and into standard healthcare organisational training, guidelines and
policies. Healthcare organisations can also support Q&S research and interventions that
apply principles of Experience-based Co-design to facilitate HCW partnerships with
consumers (47).
Limitations
While the study population of interest was all children with ID, nine of the 12 publications
were specific to children with DS. While findings were consistent across the DS and non-DS
publications, the emphasis on DS may reduce the generalisability of the findings for all ID
conditions. The search strategy may have omitted some relevant publications, particularly
due to variations in the definition of ID. For example, publications that referred to children
with complex medical conditions but not explicitly ID were excluded from this review.
CONCLUSION
The published literature indicates that the vulnerability of children with ID increases their
likelihood of experiencing poor quality care and iatrogenic harm during hospitalisation,
impacting treatment outcomes. By listening to parents to learn about the child and their ID,
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HCWs and organisations can tailor healthcare delivery and the hospital environment to
reduce or mitigate iatrogenic harm and AEs, optimising treatment outcomes. Ensuring
HCWs are knowledgeable about the children they care for and their ID is critical to achieving
greater equity for this group. Partnering with families of children with ID in planning and
implementing their care needs during hospitalisation is therefore important. Embedding
patient engagement principles across clinical training programs, healthcare policy and
practice may enable and upskill HCWs to ensure a positive care quality experience for all
children during hospitalisation.
Acknowledgements
The authors gratefully acknowledge Professor Joanne Travaglia for the suggestion to
conduct this review.
Contributors LM and RHi conceived and designed the study. LM searched the literature,
screened abstracts and full-text, extracted the data. All named authors contributed to the
thematic data analysis, drafting manuscripts and have approved the final manuscript.
This research received no specific grant from any funding agency in the public, commercial
or not-for-profit sectors.
Competing interests All authors have completed the ICMJE uniform disclosure form at
www.icmje.org/coi_disclosure.pdf and declare: no support from any organisation for the
submitted work; no financial relationships with any organisations that might have an
interest in the submitted work in the previous three years; no other relationships or
activities that could appear to have influenced the submitted work.
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Table 1: Included studies, with authors, country, methodology, participants, purpose of study, and main findings/themes
Authors/year Country
Quantitative
methodology
Qualitative
methodology Participants Purpose of study Themes
Aston, Breau and
MacLeod; 2014 (22)
Canada Feminist post-
structuralist; semi-
structured interviews;
thematic analysis
37 in total:
8 children with IDs;
17 parents;
12 nurses
To better understand how children with
intellectual disability, their parents and
nurses experience care during
hospitalisation.
• Impact of HCW's
assumptions on
care quality.
• Reliance on
parental
presence.
• Importance of
understanding
the child and the
ID.
Atz et al; 2011 (35) USA Multicentre
prospective and
retrospective cohort
study
120 children;
96 with Down syndrome
24 without Down
syndrome
Outcomes after specific cardiac surgery,
comparing children with and without
Down syndrome.
• Benefit of labels.
• Importance of
understanding
the child and the
ID.
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Brown and Guvenir;
2008 (21)
United
Kingdom
Semi-structured
interviews; thematic
analysis
28 participants:
13 carers of children with
learning disability
13 nursing staff
2 children with learning
disability
Describe the experiences of children,
their families and staff during
hospitalisation.
• Reliance on
parental
presence.
• Listening to
parents.
• Importance of
understanding
the child and the
ID.
Croot; 2012 (34) United
Kingdom
Constructivist;
interview; thematic
analysis
12 in total from 9 families:
11 parents; 1 grandparent
Provide evidence of the care needs of
Pakistani parents with a child with
learning disabilities.
• impact of HCW's
assumptions on
care quality.
• Listening to
parents.
• Importance of
understanding
the child and the
ID
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Desai et al; 2014 (24) United
Kingdom
Single centre;
Retrospective cohort
study
107 children;
67 with Down Syndrome
40 without Down
syndrome
Determine if Down syndrome is a risk
factor in the early postoperative period
following specific cardiac surgery.
• Benefit of labels.
• Importance of
understanding
the child and the
ID.
Fudge et al; 2010 (30) USA National database;
Retrospective cohort
study.
45 579 children;
4350 with Down syndrome
41229 without Down
syndrome
Describe the postoperative morbidity and
mortality rates for children with Down
syndrome undergoing cardiac surgery.
• Importance of
understanding
the child and the
ID.
Furukawa; 2013 (36) Japan Single centre;
Retrospective cohort
study
235 children;
8 with Down syndrome
227 without Down
syndrome
Evaluate mortality and postoperative
outcomes after cardiac surgery in
children with Down syndrome.
• Impact of HCW's
assumptions on
care quality.
• Importance of
understanding
the child and the
ID.
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Graham et al; 2009
(27)
USA Single centre;
Retrospective cohort
study
3437 anaesthetic events in
children;
479 had complex special
health care needs
2958 did not have special
health care needs.
To determine the utilization of
anaesthesia resources by children with
complex special health care needs.
Included children with developmental
disorders such as Down syndrome.
• Impact of HCW's
assumptions on
care quality.
• Reliance on
parental
presence.
• Listening to
parents.
Gupta et al; 2014 (31) USA National database;
Retrospective cohort
study.
2815 children;
121 with Down syndrome
2694 without Down
syndrome.
Evaluate morbidity and mortality
associated with ECMO in children with
Down syndrome and heart disease.
• Impact of HCW's
assumptions on
care quality.
• Importance of
understanding
the child and the
ID.
Lal et al; 2013 (33) India National database;
Retrospective case-
control study.
64 children;
32 with Down syndrome;
32 matched controls.
Investigate outcomes from specified
cardiac surgery in children with Down
syndrome and compared to children
without Down syndrome.
• Impact of HCW’s
assumptions on
care quality.
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Malviya et al; 2001
(28)
USA Single centre;
Retrospective cohort
study
Random sample of
children who had spinal
fusion
19 had cognitive
impairment
23 did not have cognitive
impairment
To compare pain assessment and
management practices in children with
and without cognitive impairment.
• Impact of HCW's
assumptions on
care quality.
• Importance of
understanding
the child and the
ID.
Morabito et al; 2006
(32)
United
Kingdom
Single centre;
Retrospective cohort
study
173 children with
Hirschsprung's Disease;
17 with Down syndrome
156 without Down
syndrome
Compare the outcomes of surgery for
Hirschspung's Disease in children with
Down syndrome to those without Down
syndrome.
• Impact of HCW's
assumptions on
care quality.
• Importance of
understanding
the child and the
ID
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Pratt, Baird and
Gringras; 2012 (23)
United
Kingdom
QI audit and
evaluation
QI audit and interviews Experience of 52 children
with neurodevelopmental
problems;
Pre change interviews:
20 staff and 4 families.
Post change interviews:
20 staff
8 parents
Evaluate the effectiveness of a quality
improvement programme to improve the
care experience during hospitalisation for
children with learning difficulties, autism
and challenging behaviour.
• impact of HCW's
assumptions on
care quality.
• Listening to
parents.
• Importance of
understanding
the child and the
ID
Travassos, van
Herwaarden-
Lindeboom and van
der Zee; 2011 (26)
The
Netherlands
Single centre;
Retrospective cohort
study
149 children with
Hirschsprung's disease;
20 with Down syndrome;
129 without Down
syndrome
Compare the outcome of surgical
treatment for Hirschsprung’s disease in
children with and without Down
syndrome.
• Impact of HCW's
assumptions on
care quality.
• Importance of
understanding
the child and the
ID
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Valkenburg et al; 2011
(29)
The
Netherlands
Single centre;
Retrospective cohort
study
45 neonates having same
surgical procedure
15 with Down syndrome;
30 without Down
syndrome.
To compare postoperative analgesic pain
scores and management between
neonates with and without Down's
syndrome.
• Impact of HCW's
assumptions on
care quality.
• Importance of
understanding
the child and the
ID
Van Driest et al; 2013
(25)
USA Single centre;
Retrospective cohort
study
121 children having
specific cardiac surgery;
44 with Down syndrome;
77 without Down
syndrome.
Compare opioid administration after
cardiac surgery in children with and
without Down syndrome.
• Impact of HCW's
assumptions on
care quality.
• Importance of
understanding
the child and the
ID
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Figure 1: Study selection process
542 of records identified through
database searching, 94 retained 0 of additional records
identified through other sources
82 records after duplicates removed
82 records screened 59 records excluded
23 full-text articles
assessed for eligibility
11 studies included
7 of full-text articles
excluded, with reasons
16 studies included in
synthesis
5 studies from reference
lists included
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Example of Search Strategy
Search for: from 15 [limit 14 to (yr="2000 -Current" and "all child (0 to 18 years)" and
english)] keep 124,135,196
Results: 3
Database: Ovid MEDLINE(R) 1946 to Present with Daily Update
Search Strategy:
--------------------------------------------------------------------------------
1 iatrogenic disease/ or cross infection/ (64476)
2 iatrogenic.mp. (29618)
3 medical errors/ or exp medication errors/ or near miss, healthcare/ (27176)
4 patient harm/ or patient safety/ (10331)
5 hospitalization/ or "length of stay"/ (144572)
6 adolescent, hospitalized/ or child, hospitalized/ (6385)
7 patient experience.mp. (2128)
8 1 or 2 or 3 or 4 or 5 or 6 or 7 (261935)
9 exp Intellectual Disability/ (87028)
10 exp Cognition Disorders/ (74569)
11 exp Learning Disorders/ (20281)
12 Developmental Disabilities/ (17107)
13 9 or 10 or 11 or 12 (191796)
14 8 and 13 (2140)
15 limit 14 to (yr="2000 -Current" and "all child (0 to 18 years)" and english) (320)
16 from 15 keep 124,135,196 (3)
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Patient safety vulnerabilities for children with intellectual
disability in hospital: A systematic review and narrative
synthesis
Journal: BMJ Paediatrics Open
Manuscript ID bmjpo-2017-000201.R1
Article Type: Original article
Date Submitted by the Author: 20-Nov-2017
Complete List of Authors: Mimmo, Laurel; Sydney Children's Hospital Randwick, Clinical Governance
Harrison, Reema; University of New South Wales - Randwick Campus, School of Public Health and Community Medicine, Faculty of Health Hinchcliff, Reece; University of Technology Sydney Faculty of Health, Centre for Health Services Management
Keywords: Health Service, Patient perspective, General Paediatrics, Multidisciplinary team-care, Children's Rights
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TITLE PAGE
Manuscript Title
Patient safety vulnerabilities for children with intellectual disability in hospital: A systematic
review and narrative synthesis
Corresponding author
Laurel Mimmo
Bright Alliance Building Level 9
Sydney Children’s Hospital
High Street, RANDWICK NSW Australia 2031
Email: [email protected]
Telephone: +61 2 93825660
Names and affiliations of contributing authors
Laurel Mimmo
Clinical Governance Unit, Sydney Children’s Hospitals Network, Sydney, Australia
Reema Harrison
School of Public Health and Community Medicine, Faculty of Health, University of New
South Wales, Sydney, Australia
Reece Hinchcliff
University of Technology Sydney, Centre for Health Services Research, Ultimo NSW 2007,
Australia
Word count for the text of the manuscript
3540
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PATIENT SAFETY VULNERABILITIES FOR CHILDREN WITH INTELLECTUAL DISABILITY IN
HOSPITAL: A SYSTEMATIC REVIEW AND NARRATIVE SYNTHESIS
ABSTRACT
Purpose: Adults and children with intellectual disability (ID) are vulnerable to preventable
morbidity and mortality due to poor quality healthcare. Whilst poor quality care has been
commonly identified amongst children with ID, evidence of the patient safety outcomes for
this group is lacking and therefore explored in this review.
Data sources: Systematic searches of six electronic bibliographic research databases were
undertaken from January 2000 to October 2017, in addition to hand searching.
Study selection: Keywords, subject headings and MeSH terms relating to the experience of
iatrogenic harm during hospitalisation for children with ID were used. Potentially relevant
articles were screened against the eligibility criteria. Non-English language papers were
excluded.
Data extraction: Data regarding: author(s), publication year, country, sample, health service
setting, study design, primary focus, and main findings related to measures of quality and
safety performance were extracted.
Results of data synthesis: Sixteen studies met the inclusion criteria, with three themes
emerging: the impact of the assumptions of health care workers (HCWs) about the child
with ID on care quality and associated safety outcomes; reliance on parental presence
during hospitalisation as a protective factor; and the need for HCWs to possess
comprehensive understanding of the IDs experienced by children in their care, to
scientifically deduce how hospitalisation may compromise their safety, care quality, and
treatment outcomes.
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Conclusion: When HCWs understand and are responsive to children’s individual needs and
their ID, they are better placed to adjust care delivery processes to improve care quality and
safety during hospitalisation for children with ID.
Keywords: Intellectual disability, Health service; Patient perspective; General Paediatrics;
Multidisciplinary team-care; Children’s Rights
What is already known about this subject?
• Approximately 1% to 3% of all children globally have Intellectual Disability (ID).
• Adverse events during hospitalisation are common, occurring in approximately 9.2%
to 36.7% of children.
• People with ID are admitted to hospital more frequently than their peers, but rates
and antecedent factors of adverse events amongst this population are unknown.
What this study hopes to add?
• To minimise adverse events amongst children with ID, and promote greater equity in
care, it is necessary to understand the specific risks associated with care for this
population.
• The synthesised literature presented shows that when healthcare workers
understand the child with ID and how hospitalisation can increase their vulnerability
to iatrogenic harm, disparate treatment outcomes can be avoided.
• Healthcare workers can improve care quality and optimise treatment outcomes for
these children during hospitalisation by engaging with parents/carers when planning
care.
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INTRODUCTION
The provision of safe, effective and high quality health care is an unalienable human right
(1). Poor quality care during hospitalisation is associated with iatrogenic harm and adverse
events (AEs) (2, 3) making it a key global concern (4). The estimated rate of AEs in
Organisation for Economic Co-operation and Development countries is 10 percent of
hospitalised adults, which is thought to be conservative (2, 5) . In the paediatric context,
reported rates of AEs for children during hospitalisation vary internationally from 9.2% in
Canada (6), 14.2% in the United Kingdom (UK) (7), and 36.7% in the United States (US) (8).
Children are vulnerable to AEs due to unique iatrogenic risks; for example in children, drug
dosage calculations are weight-based, commonly resulting in medication errors (3).
Greater vulnerability to poor quality care during hospitalisation is evident in particular
populations (4). People with Intellectual Disability (ID) have higher rates of preventable,
premature mortality than the general population (9, 10) and experience poorer quality care
during hospitalisation (11, 12). This represents a clear health inequity for people with ID (9,
13), exacerbated by the challenges in identifying and reporting patient safety issues
amongst this population (12), which make it difficult to quantify their incidence of AE.
Children with complex medical needs are reported to experience higher rates of AEs and
prolonged admissions during hospitalisation (6, 14). For children with ID, the possibility of
experiencing iatrogenic harm is compounded by the unique vulnerabilities of children to AEs
during hospitalisation (3, 14), and the poor care quality experienced by patients with ID (9,
11). It is imperative to determine and address the features of hospitalisation that increase
the likelihood of children with ID experiencing poor quality care, which manifest as
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iatrogenic harm and AEs. The vulnerability of children with ID to experience poor quality and
less safe care is an ethically- and economically-significant issue, yet one that is under-
examined.
This study aimed to provide a narrative synthesise of published evidence concerning the
experience of iatrogenic harm during hospitalisation for children with ID to discern the
evidence base and knowledge gaps. The review findings highlight opportunities for policy
changes and quality improvement (QI) interventions that could enhance the quality of care
delivered to children with ID internationally.
METHODS
The Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)
statement is an evidence-based approach for reporting in systematic reviews and meta-
analyses that was used to guide the reporting of this review (15).
Eligibility criteria
Inclusion criteria
Since the 1999 publication of the pivotal work, To Err is Human, patient safety research has
intensified in magnitude and scope (2). As such, we searched for publications in English
published since 2000 that met the criteria listed below.
Participants: Children (<18 years of age) in hospital as inpatients, aligning with the United
Nations definition of child (16). Included publications were required to reference children
with ID. This could include either a specific condition known to include ID, such as Down
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Syndrome (DS), or terms that are synonymous with ID such as cognitive impairment,
learning disability or developmental disability (13).
Intervention: AEs, which could be described using any of the following terms: adverse event,
medical error, near miss, adverse incident, unsafe care, patient safety incident, or problems
with quality of care.
Comparator: Clinical outcomes for children without ID of a care process known to require
hospitalisation.
Outcomes: Either clinical outcomes for children with ID of a procedure known to require
hospitalisation, self- or family/carer-reported outcomes of such care experiences, or any
other terms referring to objective or subjective measures of the quality and safety of
inpatient health care.
Exclusion criteria
Publications were excluded if they were published prior to 2000, were not available in
English, did not focus on patient safety or quality in children who were inpatients and under
18, or did not include reference to children with ID in the abstract. Publications of the
following issues were excluded:
• primary care settings;
• inpatient mental health settings;
• risks of hospitalisation due to pre-admission patient factors;
• hospital admissions of less than 24 hours;
• effectiveness of surgery without a non-ID comparator;
• errors in diagnosis of ID or medical negligence;
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• patient/parent satisfaction with healthcare services, which did not focus on adverse
safety events;
• case reviews;
• or rates of hospitalisation and/or healthcare utilisation by children with ID.
Publications that did not include analysis of impacts on care quality and patient safety were
excluded. The final exclusion criteria ensured that included publications focused on
inpatient care, as short stay contexts present other Q&S concerns that warrant separate
research (2).
Study identification
A range of text words, synonyms and subject headings for the major foci of this study were
used to undertake a systematic search of six electronic databases from January 2000 to
October 2017. An initial search was conducted in Medline to refine the search terms (see
Appendix 1 for search terms). The remaining searches were conducted from November 3
rd
to November 11th
, 2017, involving: MEDLINE, EMBASE, CINAHL, PsychInfo, Scopus, and Web
of Science. Hand searching of reference lists of included publications was also undertaken to
ensure that all relevant sources were included for data extraction and synthesis.
Study selection and data extraction
Titles and abstracts were screened, and a copy of the full paper was obtained for relevant
articles. The following data were extracted from eligible publications: author(s), publication
year, country, sample, health service setting, study design, and main focus. Where available,
data extraction included main findings related to measures of Q&S performance, such as
length of stay, and morbidity and mortality outcomes, as well as any self-reported
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experiences of adverse events or iatrogenic harm, unsafe care or concerns with quality of
care.
Assessment of the quality of the studies
The quality of included publications were assessed by the authors using the Quality
Assessment Tool for Studies with Diverse Designs (17). Using this tool the authors assessed
the study quality of the included publications using a four-point scale (0-3) against 16
criteria to indicate the quality of each publication and the overall body of evidence. One
reviewer (LM) individually assessed all publications and a second reviewer independently
assessed a subset of the publications; disagreements were resolved through discussion
resulting in substantial agreement (κ= 0.75) between reviewers on a random sample of 25%
of the papers. Because there were few publications, we did not exclude publications based
on the quality assessment. Quality assessment data was used to explore the strength of the
available evidence.
Narrative synthesis of key themes of included publications
The findings of included publications were analysed using a narrative synthesis approach,
based on the study objectives, to elicit key themes articulated in the literature (18). A
narrative approach was necessary to synthesise the heterogeneous qualitative and
quantitative findings (19). Overarching descriptions of each publication were tabulated (see
Table S1), then patterns in the data were explored to identify consistent themes across the
publications. Regular discussions amongst the study team helped refine the analysis and
enhance study validity.
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RESULTS
Title and abstract screening resulted in the identification of 30 publications that fulfilled the
inclusion criteria. From these, 11 publications were eligible based on the full text review,
with a further five identified from reference list searches. A total of 16 publications were
included in the review (see Figure 1).
Characteristics of included studies
A list of included studies, with titles, aims, methodology, participants and main findings with
key themes, is displayed in Table S1. Eleven publications were published since 2010. All
were set in specialist paediatric units, and all but one, in high income countries. Seven
publications were from the US, five from the UK, two from The Netherlands, and one each
from Canada, India and Japan.
Twelve publications used quantitative methodologies, though no publication specified rates
of iatrogenic harm or AEs amongst children with ID. In eight publications, data was
extracted from a single centre, three publications used data from a national database, and
one was a multicentre study. Four publications used qualitative methods to explore the
experience of hospitalisation for children with ID within a specific health service, without
comparator populations.
While the study population of interest was all children with ID, 10 publications were specific
to children with DS, with the remaining six inclusive of any ID condition. The post-operative
experience for children with Down Syndrome (DS) compared to children without DS was
described in 10 studies. Of these, six studies related to specifically to cardiac surgery
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outcomes (20-25), and two considered anaesthetics and/or pain management after surgery
(26, 27). The remaining two studies looked at surgical intervention for Hirschsprung’s
disease, comparing outcomes in children with and without DS (28, 29).
Two papers reviewed the comparative hospital experience for children with non-specific ID
to children without ID. One of these described the post general anaesthetic (GA) experience
for children with complex special healthcare needs, referencing developmental disorders
and DS in the abstract as a factor of interest (26). The final paper investigated the
assessment and management of pain in children with and without ID after spinal surgery
(30).
Study quality
Diverse quality was seen across publications when assessed against the quality criteria (17)
(see Table S2). Most provided theoretical background to the study, and descriptions of the
methods for data collection and analysis. All but one publication relied on opportunistic
sampling through a single centre, or retrospective data from existing databases.
Subsequently, sample sizes were wide-ranging, though study populations were generally
well described. Discussion of limitations varied; most gave concise critiques, though two
publications lacked any critique of limitations.
Key themes
Three main themes were identified: the impact of HCWs assumptions about the child with
ID on care quality and associated safety outcomes; reliance on parental presence during
hospitalisation as a protective factor from poor care quality and safety outcomes; and the
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need for HCWs to understand the child and the ID to know how hospitalisation may
compromise their safety, care quality, and treatment outcomes. Each theme is described
below.
The impact of HCWs assumptions about the child with ID on care quality and associated
safety outcomes
Labels, assumptions and stereotyping were described in three publications, which had
positive and negative impacts on care provision (31-33). Having a diagnosis or label of an ID
can be beneficial for parents to negotiate their child’s care needs with HCWs (32) and for
early diagnosis to maximise treatment outcomes of comorbidities (21). Assumptions
however, made by HCWs regarding the child’s behaviour, cognitive ability, or experience of
pain may mean they do not understand the unique needs of each child (31-34).
Generalisations regarding analgesic requirements or behaviour led to experiences of poor
quality care and adverse outcomes during hospitalisation for the child with ID (26, 32).
The influence of subtle assumptions and stereotyping of children with ID highlighted in pain
assessment (29). Children with ID had lower pain scores and reduced administration of
analgesia than children without ID (26, 27, 30, 34), and two publications suggest this may be
due to HCW’s preconceptions (26, 34). Several authors commented their findings challenged
long held beliefs regarding the benefit of surgical interventions to treat comorbidities in
children with DS (23-25, 28), or effective assessment and management of pain in children
with ID (27, 30, 34).
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Several publications found that parents identified ready access to healthcare, effective
communication between patients and HCWs, and positive treatment outcomes, as being
dependent on the assumptions, stereotypes and judgements of HCWs (31-33).
Parents/carers consistently reported that assumptions, stereotypes and judgements about
their child led to poor care experiences and safety concerns (31, 32), compounded by a lack
of effective communication (32, 33). Four publications found, to some extent, that
parents/carers felt HCW judgements about them or their child with ID influenced the quality
of care their child received (31-33, 35).
Reliance on parental presence during hospitalisation as a protective factor from poor care
quality
HCWs reliance on parental presence to supervise, protect and advocate for the care of their
child was described in four publications (31-33, 35); and to assist in the assessment of pain
in two publications (26, 30). HCWs reliance on parents also manifest as an expectation for
parents/carers to manage their child’s medical needs and behaviour during hospitalisation
(33, 35). Parents felt this reliance was frustrating when negotiating their child’s care needs
with HCWs (31, 32). Aston et al. (2014) identified this tension; HCW’s relied on parental
presence during hospitalisation, only to judge the parent when they provide a lot of detail
(32). As one parent said, “I found as soon as I started to talk and say what James’ meds
were, what his seizure activity was, that he was non-verbal, I didn’t stop […] and it was just
like all of a sudden “well she’s a know-it-all” and that’s not my intent, my intent was so that
people knew James.” (32, p.301).
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To overcome this reliance, parents/carers felt it was important that HCWs listened to them
and viewed them as the expert of their child (31, 33, 35). In knowing more about the child
and their ID label by listening to parents, several publications found HCWs can adapt the
hospital environment, such as bed location, and negotiate the child’s care needs, to
minimise poor care experiences during hospitalisation (31-33). When HCWs understood the
complexities of their child’s medical needs in the context of the current hospitalisation (31,
33, 35) reliance on parental presence could be lessened.
Need for HCWs to understand the child and the ID to know how hospitalisation may
compromise their safety, care quality, and treatment outcomes
Having specific knowledge about a child’s ID can mean HCWs identify responses or
physiological susceptibilities to the hospital environment that impact on treatment
outcomes (20, 29, 30, 34), and may have a protective value against poor morbidity and
mortality related to treatment (21, 23, 30). When HCWs apply knowledge of the child with
ID and make adjustments to care delivery and the hospital environment during
hospitalisation, it may reduce poor quality care and clinical complications (33). For example,
understanding of how known immunity issues in DS contributes to post-operative infections
was identified in four publications (21, 22, 28, 29), though mitigation strategies were not
reported. One publication noted that this susceptibility may have contributed to additional
intensive care support in children with DS (21). Another publication suggested that
knowledge of DS could be protective, finding children with DS “are predisposed to
complications and thus warrant more cautious management” (28, p.181).
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Parents generally perceived ID labels as important for HCWs to know, as the hospital and
HCWs then knew something about the needs of their child to ensure quality care (32, 33).
Two publications reported HCWs must understand the child with ID and their needs during
care planning to optimise the care quality during hospitalisation (31, 33). Aston et al. (22)
comment that reducing the vulnerability of children with ID to poor quality care goes
beyond educating HCWs, necessitating broader social and hospital system change (32).
DISCUSSION
The aim of this research was to synthesise published evidence regarding the experience of
iatrogenic harm during hospitalisation for children with ID. Sixteen publications, comprising
seven from the US, five from the UK, two from The Netherlands, and one each from Canada,
India and Japan, met the inclusion criteria. There is evidence that HCWs stereotype
behaviours (31-33) and overlook factors associated with ID that predispose the child to poor
quality care and harm (22, 32).
Behaviours of the child with ID during hospitalisation may be interpreted or stereotyped as
normal, when they are in fact an expression of pain or discomfort (26, 30). Assumptions
around the experience of pain or behaviour may result in unnecessary respiratory
compromise, admission to intensive care, and prolonged hospital admission for the child
with ID (29, 34). This demonstrates there are specific aspects of hospitalisation that expose
children with ID to harms that are preventable, avoidable, and not experienced to the same
extent by children without ID.
Implications
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An awareness of the child and their ID, or having a label of ID, was seen to be beneficial (32,
33), and may even be protective against poor outcomes (21, 23, 28, 30). Several
publications noted that children with DS are understood to have compromised immunity
(21, 22, 28, 29), which can increase their susceptibility to healthcare associated infections,
compared to children without DS. This is assumed to be the reason children with DS are
shown to have more non-cardiac complications post operatively, such as respiratory illness
and surgical site infections (21, 22, 28). Exploration into care quality factors of
hospitalisation that increase infection risk for children with DS would add to the knowledge
base for this population.
Inadequate or insufficient measures of pain and pain management were also identified,
resulting in respiratory complications or prolonged hospital admission (30, 34). While pain
management was identified as a particular challenge for this group, only four publications
explored this, suggesting evidence to understand the impact of inadequate pain
management on treatment outcomes in children with ID is warranted.
Broad assumptions on the utility of safe and equitable care for children with ID were also
found (23, 24, 26, 28). This suggests HCWs infrequently recognise that children with ID have
unique care needs during hospitalisation to ensure safe quality of care (31, 33). There is
evidence of reliance on parental presence to care for, advocate and speak for their child
(31-33, 35); and parents were often relied on to assess their child’s pain (26, 30). This
reliance infers that parents and hospital staff share anxieties about the child with ID being
left alone, unprotected, in the hospital environment (31). Exploration into how beliefs and
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values held by HCW and parents around healthcare Q&S for children with ID influence the
experience of care quality is another area of potential future study.
The paucity of research into the hospital experience of iatrogenic harms poses a challenge
to reducing health inequities for children with ID. Many papers excluded from this review
reported on the frequency of hospitalisations, healthcare utilisation, length of stay and/or
complexity of healthcare needs for the child with ID (36-43), but did not report on the
influence of healthcare Q&S on these factors. The experience of iatrogenic harms for
children with ID in different contexts, such as hospitalisation for Mental Health treatment,
primary care and/or outpatient treatment, and day only treatment, are particularly lacking.
Furthermore, how the experience of hospitalisation may vary according to severity of ID is
not explored in any of the included papers and is an emerging area of interest (44).
While individual teams and disciplines have devised various interventions to mitigate the
believed perils of hospitalisation, QI interventions and policy changes that are not evidence-
informed may ultimately fail to improve, or even possibly worsen, the problems they are
designed to address (45). Without a clear picture and scale of the problem, policy makers
and healthcare leaders are ill-equipped to mandate change.
Globally, partnerships with consumers to optimise healthcare Q&S, improve patient
outcomes, and reduce healthcare costs are increasing (2, 4), strengthened by international
recommendations for HCW training in patient engagement (46). The study findings
complement this shift, indicating that training HCWs to partner with families of children
with ID in planning and implementing their healthcare can have a positive impact on the
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care quality experience (33). This could be integrated into formal clinical training programs
for health professionals, and into standard healthcare organisational training, guidelines and
policies. Healthcare organisations can also support Q&S research and interventions that
apply principles of Experience-based Co-design to facilitate HCW partnerships with
consumers (47).
Limitations
While the study population of interest was all children with ID, nine of the 12 publications
were specific to children with DS. While findings were consistent across the DS and non-DS
publications, the emphasis on DS may reduce the generalisability of the findings for all ID
conditions. The search strategy may have omitted some relevant publications, particularly
due to variations in the definition of ID. For example, publications that referred to children
with complex medical conditions but not explicitly ID were excluded from this review.
Future reviews in this area could consider search strategies that specify terms for common
conditions or syndromes.
A limitation of this review is the exclusion of non-English language studies. However, studies
from six countries were included, traversing North America, the United Kingdom, Asia and
Continental Europe. The identification of consistent themes across studies from these
regions indicate that the findings are likely to be generalisable to the experiences of children
with ID in hospital across a broad range of contexts.
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CONCLUSION
The published literature indicates that the vulnerability of children with ID increases their
likelihood of experiencing poor quality care and iatrogenic harm during hospitalisation,
impacting treatment outcomes. By listening to parents to learn about the child and their ID,
HCWs and organisations can tailor healthcare delivery and the hospital environment to
reduce or mitigate iatrogenic harm and AEs, optimising treatment outcomes. Ensuring
HCWs are knowledgeable about the children they care for and their ID is critical to achieving
greater equity for this group. Partnering with families of children with ID in planning and
implementing their care needs during hospitalisation is therefore important. Embedding
patient engagement principles across clinical training programs, healthcare policy and
practice may enable and upskill HCWs to ensure a positive care quality experience for all
children during hospitalisation.
Acknowledgements
The authors gratefully acknowledge Professor Joanne Travaglia for the suggestion to
conduct this review.
Contributors LM and RHi conceived and designed the study. LM searched the literature,
screened abstracts and full-text, extracted the data. All named authors contributed to the
thematic data analysis, drafting manuscripts and have approved the final manuscript.
This research received no specific grant from any funding agency in the public, commercial
or not-for-profit sectors.
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Competing interests All authors have completed the ICMJE uniform disclosure form at
www.icmje.org/coi_disclosure.pdf and declare: no support from any organisation for the
submitted work; no financial relationships with any organisations that might have an
interest in the submitted work in the previous three years; no other relationships or
activities that could appear to have influenced the submitted work.
Attached as supplementary files
Appendix 1: Example of search strategy from Medline
Table S1: Summary of included studies
Table S2: Criteria study quality assessed against
Figure 1: Study selection flowchart
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Example of Search Strategy
Search for: from 15 [limit 14 to (yr="2000 -Current" and "all child (0 to 18 years)" and
english)]
Database: Ovid MEDLINE(R) 1946 to Present with Daily Update
Search Strategy:
--------------------------------------------------------------------------------
1 iatrogenic disease/ or cross infection/ (70658)
2 iatrogenic.mp. (33568)
3 medical errors/ or exp medication errors/ or near miss, healthcare/ (30938)
4 patient harm/ or patient safety/ (14645)
5 hospitalization/ or "length of stay"/ (168954)
6 adolescent, hospitalized/ or child, hospitalized/ (6783)
7 patient experience.mp. (3005)
8 1 or 2 or 3 or 4 or 5 or 6 or 7 (302463)
9 exp Intellectual Disability/ (95805)
10 exp Cognition Disorders/ (85267)
11 exp Learning Disorders/ (24037)
12 Developmental Disabilities/ (19261)
13 9 or 10 or 11 or 12 (216241)
14 8 and 13 (2354)
15 limit 14 to (yr="2000 -Current" and "all child (0 to 18 years)" and english) (391)
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Table 1: Included studies, with authors, country, methodology, participants, purpose of study, and main findings/themes
Authors/year Country
Quantitative
methodology
Qualitative
methodology Participants Purpose of study Themes
Aston, Breau and
MacLeod; 2014 (32)
Canada Feminist post-
structuralist; semi-
structured interviews;
thematic analysis
37 in total:
8 children with IDs;
17 parents;
12 nurses
To better understand how children with
intellectual disability, their parents and
nurses experience care during
hospitalisation.
Impact of HCW's
assumptions on
care quality.
Reliance on
parental
presence.
Importance of
understanding
the child and the
ID.
Atz et al; 2011 (20) USA Multicentre
prospective and
retrospective cohort
study
120 children;
96 with Down syndrome
24 without Down
syndrome
Outcomes after specific cardiac surgery,
comparing children with and without
Down syndrome.
Benefit of labels.
Importance of
understanding
the child and the
ID.
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Brown and
Guvenir; 2008 (31)
United
Kingdom
Semi-structured
interviews; thematic
analysis
28 participants:
13 carers of children with
learning disability
13 nursing staff
2 children with learning
disability
Describe the experiences of children,
their families and staff during
hospitalisation.
Reliance on
parental
presence.
Listening to
parents.
Importance of
understanding
the child and the
ID.
Croot; 2012 (35) United
Kingdom
Constructivist;
interview; thematic
analysis
12 in total from 9 families:
11 parents; 1 grandparent
Provide evidence of the care needs of
Pakistani parents with a child with
learning disabilities.
impact of HCW's
assumptions on
care quality.
Listening to
parents.
Importance of
understanding
the child and the
ID
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Desai et al; 2014 (21) United
Kingdom
Single centre;
Retrospective cohort
study
107 children;
67 with Down Syndrome
40 without Down
syndrome
Determine if Down syndrome is a risk
factor in the early postoperative period
following specific cardiac surgery.
Benefit of labels.
Importance of
understanding
the child and the
ID.
Fudge et al; 2010 (23) USA National database;
Retrospective cohort
study.
45 579 children;
4350 with Down syndrome
41229 without Down
syndrome
Describe the postoperative morbidity and
mortality rates for children with Down
syndrome undergoing cardiac surgery.
Importance of
understanding
the child and the
ID.
Furukawa et al;
2013 (22)
Japan Single centre;
Retrospective cohort
study
235 children;
8 with Down syndrome
227 without Down
syndrome
Evaluate mortality and postoperative
outcomes after cardiac surgery in
children with Down syndrome.
Impact of HCW's
assumptions on
care quality.
Importance of
understanding
the child and the
ID.
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Graham et al;
2009 (26)
USA Single centre;
Retrospective cohort
study
3437 anaesthetic events in
children;
479 had complex special
health care needs
2958 did not have special
health care needs.
To determine the utilization of
anaesthesia resources by children with
complex special health care needs.
Included children with developmental
disorders such as Down syndrome.
Impact of HCW's
assumptions on
care quality.
Reliance on
parental
presence.
Listening to
parents.
Gupta et al; 2014 (24) USA National database;
Retrospective cohort
study.
2815 children;
121 with Down syndrome
2694 without Down
syndrome.
Evaluate morbidity and mortality
associated with ECMO in children with
Down syndrome and heart disease.
Impact of HCW's
assumptions on
care quality.
Importance of
understanding
the child and the
ID.
Lal et al; 2013 (25) India National database;
Retrospective case-
control study.
64 children;
32 with Down syndrome;
32 matched controls.
Investigate outcomes from specified
cardiac surgery in children with Down
syndrome and compared to children
without Down syndrome.
Impact of HCW’s
assumptions on
care quality.
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Malviya et al;
2001 (30)
USA Single centre;
Retrospective cohort
study
Random sample of
children who had spinal
fusion
19 had cognitive
impairment
23 did not have cognitive
impairment
To compare pain assessment and
management practices in children with
and without cognitive impairment.
Impact of HCW's
assumptions on
care quality.
Importance of
understanding
the child and the
ID.
Morabito et al;
2006 (28)
United
Kingdom
Single centre;
Retrospective cohort
study
173 children with
Hirschsprung's Disease;
17 with Down syndrome
156 without Down
syndrome
Compare the outcomes of surgery for
Hirschspung's Disease in children with
Down syndrome to those without Down
syndrome.
Impact of HCW's
assumptions on
care quality.
Importance of
understanding
the child and the
ID
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Pratt, Baird and
Gringras; 2012 (33)
United
Kingdom
QI audit and
evaluation
QI audit and interviews Experience of 52 children
with neurodevelopmental
problems;
Pre change interviews:
20 staff and 4 families.
Post change interviews:
20 staff
8 parents
Evaluate the effectiveness of a quality
improvement programme to improve the
care experience during hospitalisation for
children with learning difficulties, autism
and challenging behaviour.
Impact of HCW's
assumptions on
care quality.
Listening to
parents.
Importance of
understanding
the child and the
ID
Travassos, van
Herwaarden-
Lindeboom and
van der Zee; 2011
(29)
The
Netherlands
Single centre;
Retrospective cohort
study
149 children with
Hirschsprung's disease;
20 with Down syndrome;
129 without Down
syndrome
Compare the outcome of surgical
treatment for Hirschsprung’s disease in
children with and without Down
syndrome.
Impact of HCW's
assumptions on
care quality.
Importance of
understanding
the child and the
ID
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Valkenburg et al;
2011 (27)
The
Netherlands
Single centre;
Retrospective cohort
study
45 neonates having same
surgical procedure
15 with Down syndrome;
30 without Down
syndrome.
To compare postoperative analgesic pain
scores and management between
neonates with and without Down's
syndrome.
Impact of HCW's
assumptions on
care quality.
Importance of
understanding
the child and the
ID
Van Driest et al;
2013 (34)
USA Single centre;
Retrospective cohort
study
121 children having
specific cardiac surgery;
44 with Down syndrome;
77 without Down
syndrome.
Compare opioid administration after
cardiac surgery in children with and
without Down syndrome.
Impact of HCW's
assumptions on
care quality.
Importance of
understanding
the child and the
ID
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Confidential: For Review OnlyTable 2: Criteria study quality assessed against
Quality Criteria
Explicit theoretical framework
Statement of aims/objective in body of report
Clear description of research setting
Evidence of sample size considered in terms of analysis
Representative sample of reasonable size
Description of procedure for data collection
Rationale for choice of data collection tool
Detailed recruitment data (no. approached, declined etc.)
Statistical assessment of reliability & validity of measurement tools (quantitative)
Fit between study objectives & method of data collection
Fit between study objectives & content of data collection tool
Fit between study objectives and method of analysis
Good justification for method of analysis
Assessment of reliability of analytic process (qualitative)
Evidence of user involvement in design (e.g. pilot work)
Strengths & limitations critically discussed
Reproduced with permission
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