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Confidential: For Review Only

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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REFERENCES

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2. National Patient Safety Foundation. Free from Harm: Accelerating Patient Safety

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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;


study.

45 579 children;


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;


study

235 children;


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;


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;


study.

2815 children;


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;


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;


study

173 children with

Hirschsprung's Disease;


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;


study

149 children with

Hirschsprung's disease;


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;


study

45 neonates having same

surgical procedure


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;


study

121 children having

specific cardiac surgery;


77 without Down

syndrome.

Compare opioid administration after

cardiac surgery in children with and


• 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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Confidential: For Review OnlyAppendix 1

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


BMJ Paediatrics Open on A

ugust 1, 2020 by guest. Protected by copyright.

http://bmjpaedsopen.bm

j.com/

bmjpo: first published as 10.1136/bm

jpo-2017-000201 on 29 January 2018. Dow

nloaded from



1

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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2

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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3

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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4

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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Confidential: For Review OnlyAppendix 1

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;


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;


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;


study.

45 579 children;


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;


study

235 children;


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;


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;


study.

2815 children;


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 matched controls.

Investigate outcomes from specified

cardiac surgery in children with Down

syndrome and compared to children


Impact of HCW’s

assumptions on

care quality.

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Malviya et al;

2001 (30)

USA Single centre;


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;


study

173 children with

Hirschsprung's Disease;


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;


study

149 children with

Hirschsprung's disease;


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;


study

45 neonates having same

surgical procedure


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;


study

121 children having

specific cardiac surgery;


77 without Down

syndrome.

Compare opioid administration after

cardiac surgery in children with and


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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