Family involvement in rehabilitation programmes for children and adolescents with acquired brain injury: A scoping literature review.

Paediatric acquired brain injury (ABI) can adversely impact families, and it is widely accepted that families should be involved in the rehabilitation of children/adolescents with ABI. However, there is limited guidance about how to best involve families in paediatric ABI rehabilitation. Several programmes involving the families of children/adolescents with ABI have been developed, but there are no published reviews outlining their characteristics. This scoping literature review aimed to synthesize information about these programmes and develop an understanding of how families are involved in them. Four databases were systematically searched to identify sources of evidence that described programmes in paediatric ABI rehabilitation that involve family members. One hundred and eight sources of evidence describing 42 programmes were included. Programmes were categorized as: service coordination (n = 11), psychosocial (n = 17), support groups (n = 4), training/instruction (n = 9), and education (n = 1). Families' involvement in these programmes varied across programme development, delivery, and evaluation stages. The findings of this scoping literature review outline how families can be involved in paediatric ABI rehabilitation. While this review outlines many approaches to supporting families, it also highlights the need for models of family-centred care to better articulate how clinicians and services can involve families in paediatric ABI rehabilitation.

Engaging children and adolescents with acquired brain injury and their families in goal setting: The family perspective.

This study explored the experiences of goal setting in paediatric rehabilitation from the perspectives of children and adolescents with acquired brain injury (ABI) and their parents in paediatric rehabilitation. Using a qualitative research design, 15 semi-structured interviews were conducted with 13 parents and 8 young people with ABI aged between 9 and 18 years who were engaged in outpatient rehabilitation. Interview transcripts were analysed using constructivist grounded theory methods. Two main themes and several sub-themes emerged: Experiences of goal setting: The role of professionals; The role of the young person; and The role of the parents. Working as a team: Understanding each other and building trust; Communicating, sharing knowledge and different perspectives; and Being flexible. These themes reflect parent's and young people's experience of goal setting during paediatric rehabilitation for ABI and suggest clinicians play an important role in educating young people and their families about goal setting in the outpatient rehabilitation context. Young people and their parents also perceive the focus of outpatient rehabilitation as working collaboratively with clinicians to gain knowledge to manage the consequences of ABI. Our findings emphasize the importance of the therapeutic consumer-clinician relationship and the need to actively engage young people in goal setting.

Rehabilitation models of care for children and youth with traumatic brain and/or spinal cord injuries: A focus on service structure, service organization, and the barriers and facilitators of rehabilitation service provision.

To understand the systems underlying current rehabilitation models of care used with children and youth (0-21years) who sustain traumatic brain and/or spinal cord injuries. This study gathered qualitative data on service structures, service organization, and the barriers and facilitators of service provision in selected medical rehabilitation service(s) (MRS) and community-based rehabilitation service(s) (CBRS). Informants from 11 rehabilitation services were interviewed using a semi-structured interview guide. Interviews were analysed in NVivo using content analysis method. Experiences shared by the service representatives indicated that most services supported children and youth with brain injury, with a limited number also specializing in spinal cord injuries. MRS often delivered care in inpatient or outpatient settings, while CBRS offered home/community-based services. Care planning often started either prior to or shortly after admission from acute care settings, using either multidisciplinary or interdisciplinary teamwork models. Strengths of the services included innovation and provision of family-centred care; while challenges experienced included difficulty translating evidence into practice and poor team communication. Models of care were similar across services, with a focus on providing family-centred care. Several shared challenges were described, and service representatives expressed interest in forming partnerships and collaborations to address these challenges through innovative initiatives.

Engaging children and adolescents with acquired brain injury and their families in goal setting: The clinician perspective.

This paper explored clinicians' experiences of the goal setting process with children and adolescents with acquired brain injuries (ABI) and their families in paediatric neurorehabilitation. Semi-structured interviews were conducted with 13 clinicians, all members of an interdisciplinary paediatric rehabilitation service, who work with children and adolescents with ABI and their families. Interview transcripts and additional data were analysed using constructivist grounded theory methods. Three main themes and sub-themes were developed: (1) Seeing the bigger picture: Goals change over time; Families set bigger picture goals; Need-to-dos: Goals that the child/adolescent needs to achieve; and Want-to-dos: Goals that the child/adolescent wants to achieve; (2) Collaborating as a team: Everyone needs to be on the same page; Hearing the child's/adolescent's voice; and Parents as advocates; and (3) Recognizing and navigating challenges: Child-/adolescent- and family-related challenges and Time as a service-related challenge. Participants perceived the clinician's role during goal setting as that of an active collaborator, enabling children and adolescents with ABI and their families to generate meaningful goals. These findings demonstrate insights into goal setting in paediatric ABI neurorehabilitation from clinicians' perspectives, and highlight the importance of collaboration, flexibility and anticipation of challenges in facilitating children's, adolescents' and families' involvement in this process.

Rehabilitation models of care for children and youth living with traumatic brain and/or spinal cord injuries: A focus on family-centred care, psychosocial wellbeing, and transitions.

To describe current rehabilitation models of care and programmes, in Australia and internationally, related to family-centred care, psychosocial support, and transitions used for children and youth who have sustained major traumatic brain and/or spinal cord injuries. Fourteen services were interviewed, including eight medical rehabilitation services, three community-based rehabilitation services, two insurance services, and one state-wide education service provider. Semi-structured interviews were transcribed and analysed using qualitative content analysis in NVivo. Compared to the number of services supporting children and youth with traumatic brain injury, a very limited number supported those with spinal cord injury. Although valued and often included in the model of care, family-centred care was rarely systematically evaluated by the services. Most services provided psychosocial and transition support to children and youth, and their families in the short-term post-injury, but not in the long-term. Several challenges also hindered the smooth delivery of these support services, including poor communication between service providers. These findings show that services aimed to provide the best possible care to these children and youth, and their families; guided by rehabilitation models of care. However, challenges persist with regards to delivering family-centred care, coordinating return to school, and providing long-term support for psychosocial problems and transition.

Quality of Life Outcomes in Primary Caregivers of Children with Esophageal Atresia.

OBJECTIVE: To investigate the quality of life (QoL) impact on primary caregivers of children with esophageal atresia. STUDY DESIGN: We used a prospective cohort study design, inviting primary caregivers of children with esophageal atresia to complete the following questionnaires: Parent Experience of Child Illness (PECI), Patient-Reported Outcomes Measurement Information System (PROMIS) Anxiety, PROMIS Depression, 12-Item Short Form Survey (SF-12), and Pediatric Quality of Life Inventory (PedsQL). The PECI, PROMIS Anxiety and Depression, and SF-12 assessed caregiver QoL, and the PedsQL assessed patient QoL. Patients with Gross type E esophageal atresia served as controls. RESULTS: The primary caregivers of 100 patients (64 males, 36 females; median age, 4.6 years; range, 3.5 months to 19.0 years) completed questionnaires. The majority (76 of 100) of patients had Gross type C esophageal atresia. A VACTERL (vertebral anomalies, anorectal malformation, cardiac anomalies, tracheoesophageal fistula, renal anomalies, limb anomalies) association was found in 30, ≥1 esophageal dilatation was performed in 57, and fundoplication was performed in 11/100. When stratified by esophageal atresia types, significant differences were found in 2 PECI subscales (unresolved sorrow/anger, P = .02; uncertainty, P = .02), in PROMIS Anxiety (P = .02), and in SF-12 mental health (P = .02) and mental component summary scores (P = .02). No significant differences were found for VACTERL association, nor esophageal dilatation. Requirement for fundoplication resulted in lower SF-12 general health score, and lower PedsQL social and physical functioning scores. CONCLUSIONS: We have demonstrated that caring for a child with esophageal atresia and a previous requirement for fundoplication impacts caregiver QoL.

Quality of life in parents of seriously Ill/injured children: a prospective longitudinal study.

PURPOSE: Parents of children with serious childhood illness or injury (SCII) are at risk of experiencing poor quality of life (QoL). This study investigated the nature of parent QoL at the time of child diagnosis and seven months post-diagnosis, the change in parent QoL over time, and early factors influencing short-term and longer-term parent QoL. METHODS: The sample was drawn from a prospective longitudinal cohort study conducted within a paediatric hospital setting. Participants comprised 223 parents of 167 children diagnosed with a life-threatening illness and hospitalised in the cardiology, oncology, or intensive care departments. Examined data included QoL ratings completed by parents within four weeks of diagnosis and seven months post-diagnosis, and demographic, illness-related, and psychosocial predictor measures collected within four weeks of diagnosis, or four months post-diagnosis. RESULTS: Generalised Estimating Equations were utilised to analyse data. Results indicated poor parent QoL at diagnosis, and normalised parent QoL at seven months. Improvement occurred most noticeably in the psychosocial domain. Reduced acute stress symptomatology and increased psychological flexibility were associated with higher parent QoL at diagnosis. Increased perceived emotional resources predicted enhanced parent QoL at seven months. CONCLUSION: Paediatric medical care teams should consider the challenges to QoL experienced by parents of children with SCII. Parents reporting acute stress symptoms during the acute-illness phase should be prioritised for intervention. Further, parent-dyads presenting at post-acute care settings reporting poor emotional resources would benefit from psychosocial and educative support.

Exploring health state utility values of parents of children with a serious illness.

PURPOSE: In cost-utility analysis, outcomes are usually measured in terms of quality-adjusted life years, where length of life is adjusted by levels of health-related quality of life (HRQoL) using a single value, known as a health state utility value (HSUV). This study explores the HSUVs of parents of children with a serious illness admitted within three hospital departments (cardiology, oncology and paediatric intensive care) across four time points, compares HSUVs with Australian population norms and examines predictors of parental HSUVs. METHODS: Data were obtained from the Take a Breath study, where parental HSUVs were measured using the Assessment of Quality of Life-8 Dimensions (AQoL-8D). Descriptive statistics and t-tests compared the AQoL-8D scores with population norms, while repeated measures ANOVA was used to compare parental HSUVs of children treated across illness groups over time. Regression analysis was performed to determine predictors of parental HSUVs. RESULTS: Parental HSUVs were not significantly different from the general Australian population norms (p = 0.939), as measured by the AQoL-8D. However, statistically significant differences were observed in the psychosocial super-dimension (p < 0.01) and in all eight AQoL-8D dimensions, except for senses and self-worth. No significant differences were observed across illness groups. Parental HSUVs were associated with psychosocial risk factors of trait anxiety (p < 0.001), depression (p < 0.001) and family beliefs (p = 0.024). CONCLUSION: Having a child with a severe illness has an impact on the psychosocial aspects of parental HRQoL regardless of the type of illness. Psychosocial health factors of trait anxiety, depression and family beliefs were important predictors of parental HSUV.

Early Emotional, Behavioural and Social Development of Infants and Young Children with Congenital Heart Disease: A Systematic Review.

The experiences of diagnosis of congenital heart disease (CHD), cardiac surgery and hospitalisation(s) are distressing and represent a significant stressor for a child and family, which may impact psychosocial development. This systematic review provides a synthesis of psychosocial outcomes of infants and young children with congenital heart disease who had cardiac surgery early in life. Twenty-eight studies related to infant and young children's psychosocial development, specifically emotional, social and behavioural functioning were identified. Variability was related to methodological factors including differences in study design, varying measurement tools and heterogeneous samples. Despite these limitations, the majority of studies were of high quality. The most common finding was a high prevalence of low-severity emotional and behavioural dysregulation. Young children with severe CHD or comorbid conditions experienced greater impairment, with higher rates of externalising behaviour problems, although internalising behaviour problems were also evident. This review integrates findings from literature in the past 28 years on the psychosocial well-being of infants and young children with CHD and demonstrates a risk for emotional, social and behavioural development difficulty, and, importantly, that symptoms of psychosocial impairment are detectable very early in infancy. We advocate for assessment and monitoring of emotional and behavioural regulation and social development to be routinely conducted from infancy to enable prevention and early intervention.

Psychological trajectories of mothers and fathers following their child's diagnosis of a life-threatening illness or injury: A longitudinal investigation.

OBJECTIVE: Explore the mental health trajectories of parents following their child's life-threatening illness/injury. METHODS: Participants were 217 parents (mean age: 34.9-40.0; 66 fathers) of 165 children who presented to a tertiary hospital with a life-threatening illness/injury. Parents completed questionnaires about their mental health and psychosocial stressors within 4 weeks of the child's illness/injury (T1), and 4 months (T2), 7 months (T3), and 19 months (T4) postdiagnosis. RESULTS: For both mothers and fathers, mental health symptoms were elevated at diagnosis declining to normal levels by T3, with a pattern of increase at T4. Fathers demonstrated a faster decline in symptoms between T1 and T2, and fathers, but not mothers, experienced a relapse in depressive symptoms at T4. Fathers reported higher rates of work changes. CONCLUSIONS: These findings have important implications for the design and timing of parental interventions to support families of children with life-threatening disease/injury.

Featured Article: Trajectories of Posttraumatic Stress Symptoms in Parents of Children With a Serious Childhood Illness or Injury.

Objective: Serious childhood illness is associated with significant parent psychological distress. This study aimed to (a) document acute and posttraumatic stress symptoms (PTSS) in parents of children with various life-threatening illnesses; (b) identify trajectory patterns of parental PTSS and recovery over 18 months; (c) determine psychosocial, demographic, and illness factors associated with trajectory group membership. Methods: In total, 159 parents (115 mothers, 44 fathers) from 122 families participated in a prospective, longitudinal study that assessed parent psychological responses across four time points-at diagnosis, and 3, 6, and 18 months later. Children were admitted to the Cardiology, Oncology, and Pediatric Intensive Care Departments in a tertiary pediatric hospital. The primary outcome was parent PTSS. Results: Three distinct parent recovery profiles were identified-"Resilient," "Recovery," and "Chronic." The "Resilient" class (33%) showed low distress responses across the trajectory period, whereas the "Recovery" class (52%) showed significantly higher levels of distress at the time of diagnosis that gradually declined over the first months following their child's illness. Both of these classes nevertheless remained within the normative range throughout. In contrast, the "Chronic" class (13%) was consistently high in severity, remaining within the clinical range across the entire period. Psychosocial factors such as mood, anxiety, and emotional responses predicted group membership, whereas demographic and illness factors did not. Conclusions: Parents show considerable resilience in the face of children's life-threatening illnesses. Early assessment of parent psychosocial factors may aid identification of those who would benefit from early intervention.

Psychosocial, Demographic, and Illness-Related Factors Associated With Acute Traumatic Stress Responses in Parents of Children With a Serious Illness or Injury.

This study investigated factors associated with acute stress symptoms in parents of seriously ill children across a range of illnesses and treatment settings within a pediatric hospital setting. It was hypothesized that psychosocial variables would be more strongly associated with acute stress responses than demographic and child illness variables. Participants were 115 mothers and 56 fathers of children treated within the oncology, cardiology, and intensive care departments of a pediatric hospital. Acute stress, psychosocial, demographic, and medical data were collected within the first 4 weeks of the child's hospital admission. A robust hierarchical regression model revealed that psychosocial factors significantly explained 36.8% of the variance in parent acute stress responses (p < .001); demographic variables significantly added a further 4.5% (p = .022), but illness-related factors did not contribute to the model. Findings support the implementation of a general psychosocial screening approach for parents across the wider hospital system, and that psychosocial risk factors may be targeted in interventions across different illnesses and treatment settings to improve parent outcomes.

Family Psychosocial Risk Screening in Infants and Older Children in the Acute Pediatric Hospital Setting Using the Psychosocial Assessment Tool.

OBJECTIVE: To examine the validity of the Psychosocial Assessment Tool (PAT) with families of infants (<2 years) and children admitted to hospital with acute life-threatening illnesses. METHODS: A total of 235 parents of 177 children admitted to oncology, cardiology, or pediatric intensive care completed the PAT and measures of acute stress, trait anxiety, family functioning, and quality of life, a mean 3.7 weeks following diagnosis. A modified PAT was used for families of infants, rendering two forms, PAT (<2) and PAT (2+). RESULTS: Psychometrics for PAT (<2) and PAT (2+) were acceptable. PAT Total and Subscale scores for each version were significantly correlated with validation measures. Internal consistency for PAT subscales was variable. Receiver Operating Characteristics provided some support for PAT cutoffs. PAT scores across illness groups were comparable. CONCLUSIONS: This study provides promising support for the PAT as a psychosocial screener for families of infants and older children across illness conditions.

Take A Breath: study protocol for a randomized controlled trial of an online group intervention to reduce traumatic stress in parents of children with a life threatening illness or injury.

BACKGROUND: A substantial proportion of parents whose child is diagnosed with a life-threatening illness, experience high levels of distress that can lead to long-term difficulties in mental health, family functioning and child adjustment. This study evaluates the efficacy of an Acceptance Commitment Therapy-based group intervention designed to reduce distress symptoms in these parents. The program is delivered using videoconferencing to overcome factors that prevent participation in traditional face-to-face therapy. METHOD/DESIGN: The study is a randomized control trial of the Take A Breath group intervention for parents demonstrating elevated symptoms of acute stress, delivered via videoconferencing in six 90 min group sessions. Participants are the primary caregivers of children aged 0 to 18 years admitted for a life threatening illness or injury to the Oncology, Cardiology, Neurology or Intensive Care Departments of a tertiary pediatric hospital. Parents will be randomized to intervention or waitlist control 4-10 months after their child's diagnosis. Measures will be collected prior to and immediately post intervention for intervention and waitlist parents to assess program efficacy. Intervention parents will be followed up at 6 months to assess the maintenance of program effects. We predict that intervention parents will show fewer symptoms post intervention than waitlist parents (primary outcomes: traumatic stress, depression, anxiety, stress symptoms), reflecting improvements in the psychological skills addressed in the intervention (mediating factors). It is anticipated that reductions in mental health difficulties for intervention parents will be maintained up to 6 months post-intervention and will be associated with broader improvements in parents' adjustment, child adjustment and child wellbeing (secondary outcomes). DISCUSSION: This study is unique in evaluating a group intervention delivered to parents of children affected by of a diverse range life-threatening illness or injury. Online communication technology is employed to reduce participation barriers. If proven efficacious, this trans-diagnostic approach offers the potential for broad use as part of the suite of psychosocial services provided to families through tertiary pediatric settings. TRIAL REGISTRATION: ACTRN12611000090910 . Trial Registration Date: 14/09/2011 Protocol Date/version: September 2015, version M Study Status: Ongoing.

Early Traumatic Stress Responses in Parents Following a Serious Illness in Their Child: A Systematic Review.

A systematic review of the literature investigating the early traumatic stress responses in parents of children diagnosed with a serious illness/injury. A literature review was conducted (September 2013) using Medline, PsycINFO, and CINAHL databases. Twenty-four studies related to parents of children hospitalized due to diagnosis of cancer, type 1 diabetes, meningococcal disease, trauma or serious injury, preterm birth and other serious illnesses requiring admission to intensive care were included. Parents were assessed for early traumatic stress symptoms within 3 months of their child's diagnosis/hospitalization. Prevalence rates of acute stress disorder in parents ranged from 12 to 63%. Prevalence of posttraumatic stress disorder ranged from 8 to 68%. Variability was related to methodological factors including differences in study design, timing of assessments, measurement tools, and scoring protocols. Psychosocial factors rather than medical factors predicted parent distress. This review integrates and compares early traumatic reactions in parents with children suffering a range of serious illnesses. Findings suggest a high prevalence of acute and posttraumatic stress symptoms in parents. Methodological inconsistencies made comparison of early traumatic stress prevalence rates difficult. Risk factors associated with traumatic stress symptoms were identified.

Cognitive deficits and posttraumatic stress disorder in children: A diagnostic dilemma illustrated through a case study.

Studies investigating the neuropsychological functioning of children who experience trauma have predominantly focused on maltreated populations. This article presents a case study that details the longitudinal outcome of a girl who experienced a motor vehicle accident at 5 years of age. It highlights the clinical relevance of research investigating the neuropsychological impact of single-incident trauma on children. It illustrates difficulties clinicians face in discriminating between the effects of developmental delay, traumatic brain injury, attention-deficit/hyperactivity disorder, trauma, and posttraumatic stress symptoms or posttraumatic stress disorder, especially in children with compensable injuries. The state of the current literature is discussed, and directions for future research are provided.

Parent distress reactions following a serious illness or injury in their child: a protocol paper for the take a Breath Cohort Study.

BACKGROUND: Diagnosis of life threatening childhood illness or injury can lead to significant distress reactions in parents, with many experiencing clinically significant levels of post-traumatic stress symptoms. These symptoms can have long-term adverse impacts on parent mental health, family functioning, and the adjustment of the ill child. Independent studies have found such reactions in several different illness groups. However, very little research has systematically compared the prevalence, impact and trajectories over time of post-traumatic stress symptoms in parents across different childhood illness groups with an acute life threat. The current study seeks to map the course of post-traumatic stress reactions in parents of children with various life threatening illnesses over an 18 month period, and identify factors that predict successful adaptation in families. METHOD/DESIGN: The current study described is of a prospective, longitudinal design. The sample included parents of children admitted to four major hospital departments at the Royal Children's Hospital, Melbourne, Australia, for a life threatening illness or injury. Eligible parents were those who were caregivers of children aged 0-to 18-years admitted to the Oncology, Cardiology, Neurology and Pediatric Intensive Care Unit. Parents were recruited acutely, and completed self-report questionnaires at four time-points: within the first 4 weeks (T1:); then at 4 months (T2); 7 months (T3); and 19 months (T4) after admission. Questionnaires assessed parent and child mental health and wellbeing, and a number of risk and reliance factors such child illness factors, parent demographic factors, and psychosocial factors. DISCUSSION: This study is one of the first to document the trajectory of post-traumatic stress responses in parents of very ill children, across illness groups. Given that it will also identify risk and resilience factors, and map the course of parent outcomes over an 18 monthperiod, it has the potential to inform novel strategies for intervention.

Neurodevelopmental outcome following open heart surgery in infancy: 6-year follow-up.

BACKGROUND: Children undergoing open heart surgery are at risk of neurological injury. A cohort of 35 patients, who had undergone cardiac surgery during infancy, had a significant reduction in Bayley Scale of Infant Development scores at a 12-month assessment. This cohort has now reached an appropriate age to reassess developmental progress. METHODS: Detailed psychometric testing was conducted on 20 children from the original cohort using the Weschler Preschool and Primary Scale of Intelligence, the Wide Range Assessment of Memory and Learning, and the Wechsler Individual Achievement Test. Parents completed the Connor's Rating Scale, the Behaviour Rating Scale of Executive Functioning, and the Child Behaviour Checklist. RESULTS: The mean age of the cohort at assessment was 6.6 (standard deviation 0.4) years. Mean scores on all tests of intelligence, memory, academic achievement, and executive function fell within the average range. Of the children, 20-35% were found to have significant difficulties across these areas. Mean scores in the areas of social, emotional, behavioural, and psychological functioning also fell within the average range. Of the children studied, 35% had clinically significant problems in these areas. There was only a weak association between the 12-month scores and the Full-Scale Intelligence Quotient at 6 years. CONCLUSION: Detailed psychometric testing of these children suggests that they generally function in the average range; however, a significant proportion falls below age expectations in all the areas assessed. This highlights the importance of long-term follow-up with routine developmental screening to allow identification of a subgroup that may benefit from early educational and behavioural intervention.

Psychosocial risk in families of infants undergoing surgery for a serious congenital heart disease.

OBJECTIVE: The aim of this study was to explore the acute psychosocial risk in families with infants undergoing surgery for a congenital heart disease and, secondarily, to explore the psychosocial impact of antenatal versus post-natal diagnoses. METHOD: The study sample comprised 39 caregivers (28 mothers) of 29 children diagnosed with a congenital heart disease and requiring surgery within the first 4 weeks of life. Psychosocial risk was measured using the Psychosocial Assessment Tool, which was adapted to include four novel items examining infant risk factors, namely, sleeping, feeding, crying, and bonding difficulties. Parents' psychosocial risk was measured within 4 weeks after their child's surgery and stratified into a three-tiered framework: Universal, Targeted, and Clinical risk. RESULTS: Of the total sample, 61.5% of parents were classified as Universal, that is, at lowest risk; 35.9% as Targeted, and 2.6% as Clinical. The within-family parent total Psychosocial Assessment Tool score correlations were non-significant, and there were no differences between families of infants who received post-natal versus antenatal diagnosis or single ventricle versus biventricular repair. Linear regression found that a higher parent education significantly predicted a lower total Psychosocial Assessment Tool score. CONCLUSIONS: Findings indicate that, although the majority of parents adapt to the acute stress of surgery for a serious cardiac illness in their infant, the remaining 38.5% report an increased psychosocial risk associated with higher rates of emotional distress, which may impact on the parental quality of life and capacity for optimal parenting. The distribution of psychosocial risk in parents of children undergoing surgery for a congenital heart disease is consistent with that described for parents of children with other serious paediatric diagnoses.

Psychosocial Outcomes of Parents of Children with Hirschsprung Disease Beyond Early Childhood.

PURPOSE: The lifelong impact of Hirschsprung disease (HD) upon children and their families is increasingly well recognized. Parental psychosocial wellbeing and family functioning are determinants of psychological and health-related outcomes in children with chronic conditions. We performed a cross-sectional cohort study to evaluate the psychosocial functioning of parents/caregivers of children with HD, beyond early childhood. METHODS: Parents/caregivers of children with HD, aged 4-14 years, managed at a tertiary pediatric surgical center were surveyed. Parent psychosocial outcomes, including adjustment to illness and family response, were assessed using four validated measures: Family Management Measure (FaMM); Parent Experience of Child Illness (PECI); Patient Reported Outcomes Measurement Information System (PROMISR) anxiety; and PROMISR depression. The Pediatric Quality of Life Inventory (PedsQL) was administered to assess child quality of life (proxy-report). RESULTS: Forty parents (mean age 38.7 ± 5.6 years) of children with HD (mean age 8.0 ± 2.5) participated. Parents expressed greater long-term uncertainty (PECI) and poorer perceived condition management ability (FaMM) than comparator chronic disease cohorts. Other scores for parental adjustment to their child's condition (PECI) and family response (FaMM) were comparable to reference cohorts. Symptoms of anxiety and depression were prevalent in our cohort (52.5 % and 42.5 % respectively); however, the proportion with moderate - severe PROMISR anxiety (χ2 = 2.50, p = 0.114) and depression (χ2 = 0.156, p = 0.693) scores did not significantly differ from the expected population distribution. Proxy-reported child quality of life (PedsQL) was significantly reduced relative to healthy children (p = 0.0003), but comparable to those with physical health problems with special healthcare needs (p = 0.624). CONCLUSIONS: Parents of children with HD experience long-term uncertainty and have poorer perceived condition management ability than parents of children with other chronic childhood illnesses. This work highlights the importance of targeted parental education and support beyond primary surgical management, and provides a benchmark for this cohort, against which subsequent intervention-based studies may be assessed. LEVEL OF EVIDENCE: II.