Parents' Trigger Tool for Children with Medical Complexity - PAT-CMC: Development of a recognition tool for clinical deterioration at home.

AIM: To develop a trigger tool for parents and lay caregivers of children with medical complexity (CMC) at home and to validate its content. DESIGN: This was a multi-method study, using qualitative data, a Delphi method and a concept mapping approach. METHODS: A three-round electronic Delphi was performed from December 2021 to April 2022 with a panel of 23 expert parents and 30 healthcare providers, supplemented by a preliminary qualitative exploration of children's signs of deterioration and three consensus meetings to develop the PArents' Trigger Tool for Children with Medical Complexity (PAT-CMC). Cognitive interviews with parents were performed to assess the comprehensiveness and comprehensibility of the tool. The COREQ checklist, the COSMIN guidelines and the CREDES guidelines guided the reporting respectively of the qualitative study, the development and content validity of the trigger tool and the Delphi study. RESULTS: The PAT-CMC was developed and its content validated to recognize clinical deterioration at home. The tool consists of 7 main clusters of items: Breathing, Heart, Devices, Behaviour, Neuro-Muscular, Nutrition/Hydration and Other Concerns. A total of 23 triggers of deterioration were included and related to two recommendations for escalation of care, using a traffic light coding system. CONCLUSION: Priority indicators of clinical deterioration of CMC were identified and integrated into a validated trigger tool designed for parents or other lay caregivers at home, to recognize signs of acute severe illness and initiate healthcare interventions. IMPACT: The PAT-CMC was developed to guide families in recognizing signs of deterioration in CMC and has potential for initiating an early escalation of care. This tool may also be useful to support education provided by healthcare providers to families before hospital discharge. PATIENT OR PUBLIC CONTRIBUTION: Parents of CMC were directly involved in the selection of relevant indicators of children's clinical deterioration and the development of the trigger tool. They were not involved in the design, conducting, reporting or dissemination plans of this research.

Pain Assessment in People with Intellectual Disability: Improving Skills of Health and Educational Personnel at Istituto Serafico of Assisi, Italy.

BACKGROUND: Assessment of pain in people with intellectual disability (PWID) is a difficult clinical task. Poor knowledge and confidence in assessing pain in PWID result in underestimation and undertreatment. Available resources for healthcare personnel and caregivers on pain assessment in PWID are still very limited. The aim of the study was to measure the level of knowledge and confidence in assessing pain in PWID of health and education personnel at Istituto Serafico, before and after training. SUBJECTS AND METHODS: The Istituto Serafico is a neuro-rehabilitation center caring for people with complex disabilities. Nurses, rehabilitation therapists, social health workers (SHW) and educators were invited to participate in a 4-hours theoretical and practical training. Participants were assessed through a knowledge and confidence questionnaire on pain assessment in PWID, administered before and after the training. RESULTS: 123 participants attended both the theoretical and practical sessions. Median age was 43 years (range 23-67); 89 were females and 34 males. They were 10 (8%) nurses, 9 (7%) rehabilitation therapists, 77 (63%) SHW, 27 (22%) educators. Only 7 (6%) participants (5 nurses and 2 SHW) declared to have previously received formation on pain. Participants who felt "quite confident" in assessing pain increased from 28% to 73% after the training. The median score to the 24 knowledge questions raised from 15/24 correct answers (range 6-22, 62.5%) in the pre-test to 21/24 (range 11-24, 87.5%) in the post-tests (p=0.001) Conclusions: The study highlights the great need of education programs for health and educational personnel working with PWID on pain assessment and the potential to improve knowledge and confidence through theoretical and practical training. A greater awareness of pain causes, clinical manifestations and consequences of untreated pain, could improve patient care, quality of life and rehabilitation goals.

Self-care in children and young people with complex chronic conditions: a qualitative study using Emotional Text Mining.

Objectives: To explore: (1) self-care behaviors in children and young people (range: 6 months-24 years) with complex chronic conditions, characterized by the diagnosis of a severe chronic condition, substantial family-identified needs, functional limitations associated with technology dependence, and intensive use of healthcare services; (2) the contribution to self-care of family members and other persons involved in the child's health and daily life context (e.g., health professionals and teachers), and (3) the principal factors that might have influenced the self-care process associated with developmental age. Methods: A qualitative descriptive study was conducted in an Italian academic tertiary pediatric hospital between September 2020 and May 2021. Overall, 25 focus groups and 7 online interviews were conducted via videoconferencing. Textual data were analyzed using Emotional Text Mining to identify three levels of communication: the factors, the main themes (clusters), and the sub-themes. Results: A total of 104 participants were enrolled, including 27 patients with complex chronic conditions (12 males, mean age = 11.1 ± 4.40), 33 parents, 6 siblings, 33 health professionals, and 5 teachers. Participants described the process of self-care through four main factors: "self-care", "external settings", "family", and "management". Five clusters (themes) were identified: (1) Self-care management (device; consulting); (2) Shift of agency (influencing factors; parents; school); (3) Self-care support (normal life and personal development; multidisciplinary support); (4) Daily self-care maintenance/monitoring; (5) Treatment adherence. Self-care management was mostly relevant for parents of children aged between 6 months and 3 years. Conclusion: The self-care process varies according to the needs related to the specific developmental age and the evolution of the clinical condition over time. The contribution of the family, health professionals, and social networks is fundamental for adequate self-care. To help families manage the unstable condition of their children at home, it is necessary to strengthen support networks implement home care, and ensure continuity of care.

The development of the International Classification of Functioning, Disability and Health for Child and Youth (ICF-CY) Core Sets: a systematic review.

PURPOSE: The aim of this systematic review is to verify the development of the International Classification of Functioning, Disability and Health for Child and Youth (ICF-CY), investigating methodology and how many core sets have been created. METHODS: Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) guidelines were used to carry out the systematic review. Six bibliographic databases were searched: MEDLINE, SCOPUS, Web of Sciences, CINHAL, PEDro, and OT Seeker. Papers included in the study have the following characteristics: (a) pediatric population with different health conditions, (b) assessment of ICF domains, (c) development of ICF-CY core set in different health conditions, and (d) recommendation for clinical uses. RESULTS: Search strategies allowed to identify 270 research papers. After the elimination of duplicates, 154 articles were analyzed. Finally, 28 records were included for qualitative synthesis. Twelve different ICF-CY Core Sets were identified. Autism spectrum disorder, attention-deficit/hyperactivity disorder, and cerebral palsy were the main health conditions studied at international level. Most of the studies involved international experts using Cieza' methodology to inform ICF-CY Core Set. CONCLUSIONS: After 15 years since the adoption of ICF-CY, it still finds some barriers to use. Concrete actions should be taken to develop further core sets following a rigorous methodology and to contribute implementing the ICF framework.Implication for rehabilitationIn 15 years since the implementation of International Classification of Functioning, Disability and Health for Child and Youth (ICF-CY), only 12 core sets have been developed.To develop ICF-CY Core Set, health professionals should follow methodology described by Cieza et al.Strong collaboration between low- and middle-income countries and high-income countries are recommended.

Instruments Measuring Self-Care in Children and Young Adults With Chronic Conditions: A Systematic Review.

Children and young adults (CYAs) with chronic conditions need to engage in self-care to improve their quality of life. This study aimed to retrieve the literature on instruments to assess self-care in CYAs living with chronic conditions and evaluate the psychometric proprieties of the instruments retrieved. A systematic literature review was conducted on six databases to identify peer-reviewed papers that described or used an evaluation instrument of self-care in CYAs with chronic conditions. Twenty-three articles describing 11 instruments of self-care were identified. Five instruments (45.45%) were developed for specific diseases, while six (54.54%) for various chronic illnesses. Most of the instruments were focused on treatment adherence within self-care maintenance (i.e., behaviors to maintain illness stability), excluding the monitoring of clinical parameters or the management of exacerbations. This review provides an overview of available instruments that measure self-care in CYAs with chronic conditions, which health professionals could use for patient education.

Self-Care in Pediatric Patients with Chronic Conditions: A Systematic Review of Theoretical Models.

BACKGROUND: To improve outcomes in children and young adults (CYAs) with chronic conditions, it is important to promote self-care through education and support. AIMS: (1) to retrieve the literature describing theories or conceptual models of self-care in CYAs with chronic conditions and (2) to develop a comprehensive framework. METHODS: A systematic literature search was conducted on nine databases, according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. All peer-reviewed papers describing a theory or a conceptual model of self-care in CYAs (0-24 years) with chronic conditions were included. RESULTS: Of 2674 records, 17 met the inclusion criteria. Six papers included a theory or a model of self-care, self-management, or a similar concept. Six papers developed or revised pre-existing models or theories, while five papers did not directly focus on a specific model or a theory. Patients were CYAs, mainly with type 1 diabetes mellitus and asthma. Some relevant findings about self-care in CYAs with neurocognitive impairment and in those living with cancer may have been missed. CONCLUSIONS: By aggregating the key elements of the 13 self-care conceptual models identified in the review, we developed a new overarching model emphasizing the shift of self-care agency from family to patients as main actors of their self-management process. The model describes influencing factors, self-care behaviors, and outcomes; the more patients engaged in self-care behaviors, the more the outcomes were favorable.

Prenatal Features of Bladder Agenesis in a Female Fetus with Genital Transposition.

Bladder agenesis is a rare condition, mostly affecting females, where diagnosis is usually made in infancy when investigating urinary incontinence. Neonatal cases are uncommon, and none have been reported antenatally. The few male patients with this condition rarely survive: among associated anomalies, different degrees of penoscrotal transposition are the most evident feature. The association of genital transposition in a female infant with prenatal description of bladder agenesis has not been previously reported. Early diagnosis is important, enabling planning of surgical reconstruction early in life and appropriate parental counseling.

Pain therapy, pediatric palliative care and end-of-life care: training, experience, and reactions of pediatric residents in Italy.

UNLABELLED: Pediatric palliative care represents the ideal response to life-limiting and life-threatening diseases and requires a specific and multidisciplinary training. This study aims at evaluating in Italy the training programs offered in pain therapy and pediatric palliative care, the exposure, and the personal experience concerning end-of-life care management. The data have been obtained through a survey addressed to all the residents specializing in pediatrics in Italy. Three hundred forty-eight of 1,200 residents from 33 of the 41 schools of pediatrics existing in Italy responded to the questionnaire. One hundred seventy-four of them (50 %) declared they received training in end-of-life care at least once: 146 during their graduation course of medicine, 84 during the pediatric residency, and 54 in both occasions. Sixty percent of respondents were present at one death at least, with an increasing percentage in the last years of residency (91.5 % in the fifth year) but only 12 % were directly involved in the management (36.2 % in the fifth year); 5.7 % managed at least one communication of death; 12.6 % followed sedo-analgesia protocols. Only 11 % of residents felt ready to face end-of-life care management. CONCLUSION: The training in end-of-life care in Italy is not currently satisfactory. Further efforts are therefore required to create a comprehensive and multidisciplinary training.

Markers of activated inflammatory cells correlate with severity of liver damage in children with nonalcoholic fatty liver disease.

Concomitantly to the obesity epidemic, nonalcoholic fatty liver disease (NAFLD) has become the leading cause of liver disease in children. NAFLD encompasses a spectrum of histological damage ranging from simple steatosis to nonalcoholic steatohepatitis (NASH), with possible progression to cirrhosis. There is growing evidence that the immune system plays a pivotal role in the initiation and progression to NASH but the cellular nature of the hepatic inflammation is still unknown. The present study includes 34 children with biopsy-proven NAFLD. Liver damage was evaluated by the NAFLD activity score (NAS), and the inflammatory infiltrate was characterized by immunohistochemistry for CD45, CD3 and CD163 which are markers of leukocytes, T cells and activated Kupffer cells/macrophages, respectively. Our results have shown that CD45+ (P<0.0001) and CD163+ (P<0.0001) cells were markedly increased in children with severe histological activity (NAS≥5) compared to children with lower activity (NAS<5), whereas CD3+ cells were significantly lower (P<0.01) in children with severe histological activity. There was a significant association between the numbers of CD45+, CD3+ and CD163+ cells, regarding both the portal tract and liver lobule, and the severity of steatosis, ballooning and fibrosis (P<0.01). These data suggest that the severity and composition of the inflammatory infiltrate correlate with steatosis and the severity of disease in children with NAFLD. Moreover, a decrease in CD3+ cells may be involved in the pathogenesis of liver damage. Future studies should evaluate whether it can predict the progression of liver disease independently of established histological scores.

An unusual case of anisocoria by vegetal intoxication: a case report.

A 12 year old boy presented with an acute onset of anisocoria and blurred vision. Ocular motility was normal but his right pupil was dilated, round but sluggishly reactive to light. There was no history of trauma, eye drops' instillation, nebulised drugs or local ointments. His past medical history was negative.A third nerve palsy was considered but the performed cerebral MRI was normal.On further anamnestic investigation the boy revealed that he had spent the morning doing gardening, and especially working on a "trumpet plant". Datura and Brugmansia are well known toxic plant; all Datura and Brugmasia plants contain, primarily in their seeds and flowers, tropane alkaloids such as scopolamine, hyoscyamine and atropine. Systemic and local intoxications have already been described.The day after anisocoria was much less evident and completely resolved in three days.We present this case of an unusual cause of mydriasis to underline once more the importance of a well and deeply conducted medical history.