Comprehensive care programmes for children with medical complexity.

BACKGROUND: Children with medical complexity (CMC) represent a small, but growing, proportion of all children. Regardless of their underlying diagnosis, by definition, all CMC have similar functional limitations and high healthcare needs. It has been suggested that improving aspects of healthcare delivery for CMC improves health- and quality of life-related outcomes for children and their families and reduces healthcare-related expenditure. As a result, dedicated comprehensive care programmes have been established at many hospitals to meet the needs of CMC; however, it is unclear if such programmes are effective. OBJECTIVES: Our main objective was to assess the effectiveness of comprehensive care programmes that aim to improve care coordination and other aspects of health care for CMC and to assess whether the effectiveness of such programmes differs according to the programme setting and structure. We aimed to assess their effectiveness in relation to child and parent health, functioning, and quality of life, quality of care, number of healthcare encounters, unmet healthcare needs, and total healthcare-related costs. SEARCH METHODS: We searched CENTRAL, MEDLINE, Embase, and CINAHL in May 2023. We also searched reference lists, trial registries, and the grey literature. SELECTION CRITERIA: Randomised and non-randomised trials, controlled before-after studies, and interrupted time series studies were included. Studies that compared enrolment in a comprehensive care programme with non-enrolment in such a programme/treatment as usual were included. Participants were children that met the criteria for the definition of CMC, which is: having (i) a chronic condition, (ii) functional limitations, (iii) increased health and other service needs, and (iv) increased healthcare costs. Studies that included the following types of outcomes were included: health; quality of care; utilisation, coverage and access; resource use and costs; equity; and adverse outcomes. DATA COLLECTION AND ANALYSIS: Two review authors independently extracted data, assessed the risk of bias in each included study, and evaluated the certainty of evidence according to GRADE criteria. Where possible, data were represented in forest plots and pooled. We were unable to undertake a meta-analysis for comparisons and outcomes, so we used a structured synthesis approach. MAIN RESULTS: We included four studies with a total of 912 CMC as participants. All included studies were randomised controlled trials conducted in hospitals in the USA or Canada. Participants varied across the included studies; however, all four studies included children with complex and chronic illness and high healthcare needs. While the primary aim of the intervention was similar across all four studies, the components of the interventions differed: in the four studies, the intervention involved some element of care coordination; in two of the studies, it involved the child receiving care from a multidisciplinary team, while in one study, the intervention was primarily centred on access to an advanced practice nurse care coordinator and another study involved nurse a practitioner-paediatrician dyad partnering with families. The risk of bias in the four studies varied across domains, with issues primarily relating to the lack of blinding of participants, personnel, and outcome assessors, inadequate allocation concealment, and incomplete outcome data. Comprehensive care for CMC compared to usual care may make little to no difference to child health, functioning, and quality of life at 12 or 24 months (three studies with 404 participants) and we assessed the evidence for the outcomes in this category (child health-related quality of life and functional status) as being of low certainty. For CMC, comprehensive care probably makes little or no difference to parent health, functioning, and quality of life compared to usual care at 12 months (one study with 117 participants) and we assessed the evidence for this outcome as being of moderate certainty. Comprehensive care for CMC compared to usual care may slightly improve child and family satisfaction with, and perceptions of, care and service delivery at 12 months (three studies with 453 participants); however, we assessed the evidence for these outcomes as being of low certainty. For CMC, comprehensive care probably makes little or no difference to the number of healthcare encounters (emergency department visits) and the number of hospitalised days (hospital admissions) compared to usual care at 12 months (three studies with 668 participants), and we assessed the evidence for these outcomes as being of moderate certainty. Three of the included studies (668 participants) reported cost outcomes and had conflicting results, with one study reporting significantly lower healthcare costs at 12 months in the intervention group compared to the control group, one reporting no differences between groups, and the other study reporting a greater increase in total healthcare costs in the intervention group compared to the control group. Overall, comprehensive care may make little or no difference to overall healthcare costs in CMC; however, the methods used to measure total healthcare costs varied across studies and the certainty of the evidence relating to this outcome is low. No studies assessed the costs to the family. AUTHORS' CONCLUSIONS: The findings of this review should be treated with caution due to the limited amount and quality of the published research that was available to be included. Overall, the certainty of the evidence for the effectiveness of comprehensive care for CMC ranged from low to moderate across outcomes and there is currently insufficient evidence on which to draw strong conclusions. There is a need for more high-quality randomised trials with consistency of the target population and intervention components, methods of reporting outcomes, and follow-up periods, as well as full cost analyses, taking into account both costs to the family and costs to the healthcare system.

Audit of enuresis referrals on the waiting list for a tertiary hospital outpatient clinic.

AIM: Enuresis, defined as intermittent incontinence occurring exclusively during sleep, affects 4-19% of children, but can be effectively treated using education and alarm-bell therapies. However, delays in treatment are likely to impact upon the quality of life of the child, parents and carers. Poor quality and incomplete referrals are thought to be a major driver of inefficiencies. The aim of this study was to explore characteristics of enuresis referrals on the waiting list for a general medicine clinic at a tertiary paediatric hospital. METHODS: An audit was conducted to examine all enuresis referrals on the general medicine outpatient clinic waiting list in February 2019 at The Royal Children's Hospital, Melbourne. Enuresis referrals with an organic cause and those for children less than 5 years of age were excluded. RESULTS: Of the 2613 referrals on the general medicine waiting list, 486 of 2613 (19%) were related to enuresis. The median age of patients on the waiting list was 8 years and 65% (315/486) were male. Sufficient detail was provided to determine temporal and disease stratification in 45% (218/486) of referrals; primary versus secondary enuresis, and monosymptomatic versus non-monosymptomatic enuresis. The mean number of days on the waiting list calculated at the time of data extraction (13 February 2019) was 226 (±179) days. CONCLUSIONS: The findings from this study suggest that there are long waiting times for enuresis services and referrals often do not contain complete information.

Polyethylene Glycol Dosing for Constipation in Children Younger Than 24 Months: A Systematic Review.

OBJECTIVES: Evaluate safety and effectiveness of Polyethylene glycol (PEG) for chronic constipation in children aged younger than 24 months. Identify the optimum dose of PEG to manage chronic constipation in children aged younger than 24 months. METHODS: In this systematic review, Embase, Medline Ovid, Pubmed, and the Cochrane Library were searched between January 1, 2000 and February 1, 2019. Studies investigating functional constipation, in which patients younger than 24 months of age were treated with PEG, were considered as potentially eligible for review. Two authors screened the studies against inclusion/exclusion criteria. Study quality was assessed with the PEDro quality assessment, Cochrane risk of bias tool, and/or the Newcastle-Ottawa Scale. RESULTS: Five studies (2 randomized controlled trials, 3 retrospective chart reviews) satisfied selection criteria (n = 459). All studies employed different dosage categories: mean effective maintenance dose, mean initial dose, mean short-term and long-term dose, and mean daily dose. Dosage regimens were variable, with 0.45 to 1.1 g ·â€Škg ·â€Šday for PEG3350 and 0.48 to 0.65 g ·â€Škg ·â€Šday for PEG4000. Adverse effects were transient across all studies for all types of PEG; these included diarrhea and abdominal pain. CONCLUSIONS: This systematic review provided evidence for a lack of reported side effects from PEG for children aged younger than 24 months. Evidence to establish appropriate dosage regimens does not exist.An infographic accompanying this article can be found at http://links.lww.com/MPG/B839.

Paediatric bladder dysfunction: A single centre experience of public hospital wait times.

AIM: Paediatric bladder dysfunction, including daytime urinary incontinence and enuresis, is a common and distressing condition. Unfortunately, children with these symptoms are often on waitlists for several months. This treatment delay may significantly impact upon the child and family unit. This study aimed to quantify waiting times for children who had attended hospital outpatient clinics for symptoms of wetting. METHODS: A retrospective review was undertaken for patients who had been referred to The Royal Children's Hospital, Melbourne outpatient clinics for symptoms of wetting (with/without bowel symptoms). Data regarding the referral and triage pathway, up to the time of the first clinic appointment, were collected. These data were compared to a previous audit conducted in the same setting. RESULTS: A total of 101 clinic attendances were included in this study. The overall waiting time, from receipt of referral to the patient's first clinic attendance, was a median of 181 days (n = 94 valid responses; range 7-695). Wait times for patients with isolated symptoms of wetting were similar to patients with mixed bowel and bladder dysfunction (187 and 171.5 days, respectively). Most patients were triaged to the continence clinic (n = 68), whilst smaller proportions of patients were seen in the encopresis (n = 14), urology (n = 13), general medicine (n = 2), gastroenterology (n = 1) and nurse-led enuresis clinic (n = 3). CONCLUSIONS: The waiting times for patients with wetting generally exceeded 5 months. Alternative pathways for triage need to be explored to manage demand and improve wait times.

Retrospective audit of referral and triage pathways of paediatric patients with constipation and soiling.

AIM: This study aimed to explore referral and triage pathways for paediatric patients referred to an Australian hospital with bowel dysfunction (isolated or mixed bowel and bladder). METHODS: We conducted a retrospective audit of patients who attended their first clinic appointment during April to June 2014. Patients were included if they: (i) were a new patient referred for symptoms of constipation, soiling, daytime urinary incontinence or enuresis; and (ii) attended the encopresis, general medicine, continence, gastroenterology, paediatric surgery, urology, renal or Child and Adolescent Mental Health clinic. Patients with an organic cause (e.g. Hirschsprung disease) for their dysfunction were excluded. RESULTS: Of 1485 new patients seen at our targeted clinics, 281 (18.9%) had symptoms of bowel and/or bladder dysfunction. After excluding patients aged younger than 3 years (n = 43) and those with isolated bladder dysfunction (n = 130), 56 were referred for isolated bowel dysfunction and 52 for mixed bowel and bladder dysfunction. The median wait time from referral to first appointment was 3.8 months. Median wait times varied across symptom groups (isolated bowel, 4.6 months; mixed 3.4 months) and clinics (encopresis, 7.7 months; general medicine, 2.5 months). Over a 12-month period, patients attended an average of 3.5 appointments (isolated bowel, mean 3 appointments; mixed, mean 4 appointments). CONCLUSION: Paediatric patients with symptoms of bowel and bladder dysfunction wait several months to be seen in a public tertiary referral hospital. Alternative pathways for care, such as community-based primary care, need to be explored to improve timely management.

Cerebral hypomyelination associated with biallelic variants of FIG4.

The lipid phosphatase gene FIG4 is responsible for Yunis-Varón syndrome and Charcot-Marie-Tooth disease Type 4J, a peripheral neuropathy. We now describe four families with FIG4 variants and prominent abnormalities of central nervous system (CNS) white matter (leukoencephalopathy), with onset in early childhood, ranging from severe hypomyelination to mild undermyelination, in addition to peripheral neuropathy. Affected individuals inherited biallelic FIG4 variants from heterozygous parents. Cultured fibroblasts exhibit enlarged vacuoles characteristic of FIG4 dysfunction. Two unrelated families segregate the same G > A variant in the +1 position of intron 21 in the homozygous state in one family and compound heterozygous in the other. This mutation in the splice donor site of exon 21 results in read-through from exon 20 into intron 20 and truncation of the final 115 C-terminal amino acids of FIG4, with retention of partial function. The observed CNS white matter disorder in these families is consistent with the myelination defects in the FIG4 null mouse and the known role of FIG4 in oligodendrocyte maturation. The families described here the expanded clinical spectrum of FIG4 deficiency to include leukoencephalopathy.

Children who soil: A review of the assessment and management of faecal incontinence.

Soiling is a common and distressing condition affecting children. In the vast majority of patients, it is associated with constipation. Most constipation is functional and is best thought of as difficulty achieving adequate bowel emptying. In a small minority of patients, there is no associated constipation, so-called non-retentive faecal incontinence. The aetiology of this latter condition in children remains unclear. The mainstay of management in all cases is a regular toileting programme, together with laxatives as required. This must be individualised considering the diagnosis, the age of the child and the psychosocial factors affecting the child and family. The diagnosis is made with a thorough history and examination, supplemented in some cases with targeted investigations. Engaging the child and family in a long-term treatment programme (at least 6 months to 2 years) is essential for treatment success. The following clinical practice guideline and algorithm for the assessment and management of children who soil represents consensus opinion using available evidence.

Diagnostic scores, questionnaires, quality of life, and outcome measures in pediatric continence: A review of available tools from the International Children's Continence Society.

PURPOSE: We reviewed and collated information concerning the available tools for the measurement of symptoms and outcomes in pediatric continence. MATERIALS AND METHODS: MEDLINE, EMBASE, and CINHAL databases were searched for relevant articles published prior to December 2016 and independently screened by two researchers. Expert opinion was also widely sought through consultation with the ICCS Board membership and their professional networks and the multidisciplinary authorship group. The most relevant materials were then selected for analysis and inclusion and resulted in a document available on the ICCS website for all members to review. Insights and feedback were considered with consensus and agreement reached to modify the document. RESULTS AND CONCLUSIONS: A variety of useful tools for the assessment and measurement of bladder and bowel dysfunction and quality of life and behavioral comorbidities are presented together with their indications and potential pitfalls. ICCS cannot recommend one over another as the most useful for each clinician will vary depending on the clinical setting, available time, and patient population. We provide a framework for choosing those that are most appropriate based on our findings.

Childhood constipation.

BACKGROUND: Constipation is best defined as difficulty passing stools that may be infrequent (≤2 per week), painful and associated with stool retention. Childhood constipation is common, with a prevalence of 3-30% worldwide. Most constipation in children is functional and related to behavioural withholding after an unpleasant stool event. Successful diagnosis and management can occur in primary care, and specialist referral is only needed for refractory cases or concerns regarding organic pathology. OBJECTIVE: This article aims to provide a structure for evaluating, diagnosing and managing childhood functional constipation in general practice. DISCUSSION: Structured history and examination can screen for organic pathology or red flags that require specialist referral. Investigations such as abdominal X-ray are not routine. Early management of childhood constipation provides improved quality of life for patients and their families. Management, including disimpaction and maintenance, should maintain continuity of care and provide long-term support for families.

Transabdominal electrical stimulation increases colonic propagating pressure waves in paediatric slow transit constipation.

BACKGROUND AND AIMS: In slow-transit constipation (STC) pancolonic manometry shows significantly reduced antegrade propagating sequences (PS) and no response to physiological stimuli. This study aimed to determine whether transcutaneous electrical stimulation using interferential current (IFC) applied to the abdomen increased colonic PS in STC children. METHODS: Eight children (8-18 years) with confirmed STC had 24-h colonic manometry using a water-perfused, 8-channel catheter with 7.5 cm sidehole distance introduced via appendix stomas. They then received 12 sessions (20 min/3× per week) of IFC stimulation (2 paraspinal and 2 abdominal electrodes), applied at a comfortable intensity (<40 mA, carrier frequency 4 kHz, varying beat frequency 80-150 Hz). Colonic manometry was repeated 2 (n=6) and 7 (n=2) months after IFC. RESULTS: IFC significantly increased frequency of total PS/24h (mean ± SEM, pre 78 ± 34 vs post 210 ± 62, p=0.008, n=7), antegrade PS/24h (43 ± 16 vs 112 ± 20, p=0.01) and high amplitude PS (HAPS/24h, 5 ± 2:10 ± 3, p=0.04), with amplitude, velocity, or propagating distance unchanged. There was increased activity on waking and 4/8 ceased using antegrade continence enemas. CONCLUSIONS AND INFERENCES: Transcutaneous IFC increased colonic PS frequency in STC children with effects lasting 2-7 months. IFC may provide a treatment for children with treatment-resistant STC.

Slow-transit constipation with concurrent upper gastrointestinal dysmotility and its response to transcutaneous electrical stimulation.

PURPOSE: Transcutaneous electrical stimulation (TES) speeds up colonic transit in children with slow-transit constipation (STC). This study examined if concurrent upper gastrointestinal dysmotility (UGD) affected response to TES. METHODS: Radio-nuclear transit studies (NTS) were performed before and after TES treatment of STC as part of a larger randomised controlled trial. UGD was defined as delayed gastric emptying and/or slow small bowel transit. Improvement was defined as increase of ≥1 Geometric Centre (median radiotracer position at each time [small bowel = 1, toilet = 6]). RESULTS: Forty-six subjects completed the trial, 34 had NTS after stimulation (21 M, 8-17 years, mean 11.3 years; symptoms >9 years). Active stimulation increased transit in >50% versus only 25% with sham (p = 0.04). Seventeen children also had UGD. In children with STC and either normal upper GI motility (NUGM) and UGD, NTS improved slightly after 1 month (57 vs. 60%; p = 0.9) and more after 2 months (88 vs. 40%; p = 0.07). However, mean transit rate significantly increased with NUGM, but not UGD (5.0 ± 0.2: 3.6 ± 0.6, p < 0.01). CONCLUSION: Transcutaneous electrical stimulation was beneficial for STC, with response weakly associated with UGD. As measured by NTS, STC children with NUGM responded slightly more, but with significantly greater increased transit compared to those with UGD. Higher numbers are needed to determine if the difference is important.

Trunk strength and mobility changes in children with slow transit constipation.

BACKGROUND AND AIM: It appears that there are no published reports on childhood slow transit constipation (STC) that have considered the state of the musculoskeletal components of the trunk in these children. The present study aimed to determine whether children with STC have different trunk musculoskeletal characteristics that might be related to their defecation difficulties, compared to controls. METHODS: With the aid of computer-analyzed photographs and clinical testing, 41 children with STC and 41 age-matched controls were examined for Marfanoid features, sitting posture, spinal joint mobility and trunk muscle strength. The latter was assessed by measuring maximum voluntary abdominal bulging and retraction in sitting, and active trunk extension in prone-lying. Levels of general exercise and sedentary activities were evaluated by questionnaire. RESULTS: STC subjects were more slumped in relaxed sitting (P < or = 0.001), less able to bulge (P < or = 0.03) and less able to actively extend the trunk (P = 0.02) compared to controls. All subjects sat more erect during abdominal bulging (P < or = 0.03). CONCLUSION: The results show that STC children have reduced trunk control and posture, which indicates that clinicians should include training of trunk muscles and correction of sitting posture. There was no evidence that children with STC exercised less than the controls.

Postnatal support for drug users: evaluation of a specialist health visiting service.

Women who misuse drugs require a high level of support during their pregnancy and in the postnatal period. A service to provide additional support to such women in the postnatal period was developed in Scotland, through the integration of a specialist health visitor (SHV) working within the existing multi-agency drug support team (DST). This paper reports on a study to identify views and experiences of both the women who use the service, and the health and social care professionals working with the SHV service, of its effects. Results revealed that specific aspects of the SHV service were viewed positively by women and professionals. However, potential for confusion over lines of accountability between professionals could exist. Also, meeting the complex needs of these women in a sustained way in the community may remain problematic.

What psychosocial well-being in the postnatal period means to midwives.

OBJECTIVE: to explore midwives' views of psychosocial well-being in the postnatal period. DESIGN: qualitative study using focus-group interviews conducted in 1999. SETTING: two community health centres and a school of nursing and midwifery in Scotland. PARTICIPANTS: a convenience sample of community and student midwives. ANALYSIS: thematic analysis was undertaken through the identification of codes, categories and themes. FINDINGS: the categories were generated from the interview questions: 'the meaning midwives give to women's psychosocial well-being', 'midwives' assessment of women's well-being', and 'midwives views of worrying behaviours' displayed by women. From the first two categories, themes of 'coping', 'expectations', 'observation and communication skills', 'labour debriefing', and 'previous contact with women' emerged. Midwives assessed coping and unmet expectations through a range of communication and observational skills, including the use of a form of labour debriefing. Midwives who knew women during their pregnancy thought that they were able to assess coping and expectations better in the postnatal period. The midwives tended to describe women using stereotypical categories. From the third category, 'worrying behaviours', three themes emerged; 'extreme or obsessive behaviours about self, the baby or house' 'wanting to detain you' and 'quiet women'. CONCLUSIONS: the meaning midwives give to psychosocial well-being includes a complex interplay between midwives' views of psychosocial well-being and their assessment of it. The importance midwives give to knowing women in pregnancy has implications for the ongoing debate about the provision of continuity of carer. Midwives used a range of techniques to elicit accurate information, to confirm problems or be reassured that all was well. Views based on stereotypical generalisations should be challenged.