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Marfan syndrome (MFS) is a multisystemic, autosomal dominant connective tissue disorder that occurs de novo in 25%. In many families, parent and child(ren) are affected, which may increase distress in parents. To assess distress, 42 mothers (29% MFS) and 25 fathers (60% MFS) of 43 affected children, completed the validated screening‐questionnaire Distress thermometer for parents of a chronically ill child, including questions on overall distress (score 0–10; ≥4 denoting “clinical distress”) and everyday problems (score 0–36). Data were compared to 1,134 control‐group‐parents of healthy children. Mothers reported significantly less overall distress (2, 1–4 vs. 3, 1–6; p = .049; r = −.07) and total everyday problems (3, 0–6 vs. 4, 1–8; p = .03; r = −.08) compared to control‐group‐mothers. Mothers without MFS reported significantly less overall distress compared to mothers with MFS, both of a child with MFS (1, 0–4 vs. 3.5, 2–5; p = .039; r = −.17). No significant differences were found between the father‐groups, nor between the group of healthy parents of an affected child living together with an affected partner compared to control‐group‐parents. No differences in percentages of clinical distress were reported between mothers and control‐group‐mothers (33 vs. 42%); fathers and control‐group‐fathers (28 vs. 32%); nor between the other groups. Distress was not associated with the children's MFS characteristics. Concluding, parents of a child with MFS did not show more clinical distress compared to parents of healthy children. However, clinical distress was reported in approximately one‐third and may increase in case of acute medical complications. We advise monitoring distress in parents of a child with MFS to provide targeted support.

Background Interest in patient involvement in research is growing. Research should rather be ‘with’ or ‘by’ patients, and not only be ‘about’ or ‘for’ patients. Patients’ active involvement in research is not self-evident and special efforts have to be made. If we make efforts towards patient involvement, it could contribute to even more relevant projects with an even greater impact. In this paper we describe the process of development of a tool to support patient involvement in research projects. Methods The tool development was done in a co-creation of experience experts (patients and their parents/relatives) together with researchers. We used a participatory method in an iterative process comprising three consecutive stages. First, the purpose for the tool was explored, using focus groups. Second, the main ingredients and conceptualization for the tool were determined, using a narrative review. Third, the so-called Involvement Matrix was formalized and finalized using various expert panels. Results A conversation tool was developed, through which researchers and patients could discuss and explain their roles of involvement in a research project. This tool was formalized and visualized as a ‘matrix’. The so-called Involvement Matrix describes five roles (i.e., Listener, Co-thinker, Advisor, Partner, and Decision-maker) and three phases (i.e., Preparation, Execution, and Implementation) and includes a user’s guide. Conclusion The Involvement Matrix can be used prospectively to discuss about possible roles of patients in different phases of projects, and retrospectively to discuss whether roles were carried out satisfactorily. Sharing experiences with the Involvement Matrix and evaluating its impact are the next steps in supporting patient involvement in research.

Although essential for providing optimal adolescent patient support, knowledge of the impact of Marfan syndrome in adolescence is limited. To explore adolescents’ perceived impact of Marfan syndrome on (physical) functioning (activities, participation), disability (limitations, restrictions), contextual factors and support needs, we interviewed 19 adolescents with Marfan syndrome. Audio-recordings were transcribed, coded and analysed using thematic analysis. Identified themes were “difficulties in keeping up with peers” and “being and feeling different from peers”. Furthermore, an adolescent Marfan syndrome-specific International Classification of Functioning, Disability and Health for Children and Youth (ICF-CY) model derived from the data describing the adolescent perceived impact of Marfan syndrome on functioning, disability and its contextual factors. Adolescents perceived problems in keeping up with peers in school, sports, leisure and friendships/relationships, and they could not meet work requirements. Moreover, participants perceived to differ from peers due to their appearance and disability. Contextual factors: coping with Marfan syndrome, self-esteem/image, knowledge about Marfan syndrome, support from family/friends/teachers, ability to express needs and peer-group acceptation acted individually as barrier or facilitator for identified themes. Conclusion: Adolescents with Marfan syndrome perceived limitations and restrictions in (physical) functioning. They perceived problems in keeping up with peers and perceived to differ from peers due to their appearance and disability. This warrants awareness and tailored physical, psychosocial, educational and environmental support programmes to improve (physical) functioning and empowerment of adolescents with Marfan syndrome.What is known:• Marfan syndrome is a hereditary connective tissue disorder.• Marfan syndrome affects multiple systems.What is new:• Adolescents with Marfan syndrome perceive (1) problems in keeping up with peers in school, sports, leisure, friendships/relationships and work (2) to differ from peers due to their appearance and disability.• An adolescent Marfan syndrome-specific International Classification of Functioning, Disability and Health for Children and Youth model derived from the data describing the adolescent perceived impact of Marfan syndrome on functioning, disability and contextual factors.

Supplemental Digital Content is available in the text., Objectives: To conduct a scoping review to 1) describe findings and determinants of physical functioning in children during and/or after PICU stay, 2) identify which domains of physical functioning are measured, 3) and synthesize the clinical and research knowledge gaps. Data Sources: A systematic search was conducted in PubMed, Embase, Cumulative Index to Nursing and Allied Health Literature, and Cochrane Library databases following the Preferred Reporting Items for Systematic Reviews and Meta-analyses extension for Scoping Reviews guidelines. Study Selection: Two investigators independently screened and included studies against predetermined criteria. Data Extraction: One investigator extracted data with review by a second investigator. A narrative analyses approach was used. Data Synthesis: A total of 2,610 articles were identified, leaving 68 studies for inclusion. Post-PICU/hospital discharge scores show that PICU survivors report difficulties in physical functioning during and years after PICU stay. Although sustained improvements in the long-term have been reported, most of the reported levels were lower compared with the reference and baseline values. Decreased physical functioning was associated with longer hospital stay and presence of comorbidities. A diversity of instruments was used in which mobility and self-care were mostly addressed. CONCLUSIONS: The results show that children perceive moderate to severe difficulties in physical functioning during and years after PICU stay. Longitudinal assessments during and after PICU stay should be incorporated, especially for children with a higher risk for poor functional outcomes. There is need for consensus on the most suitable methods to assess physical functioning in children admitted to the PICU.

Background To explore experiences of parents of children with disabilities using the WWW, roadmap, a tool to support them in exploring needs, finding information, and asking questions of professionals and to explore differences between parents who had used the WWW‐roadmap to prepare for consultation with their rehabilitation physician and parents who had not. Methods In a sequential cohort study, we included 128 parents; 54 used the WWW‐roadmap prior to consultation and 74 received care‐as‐usual. Both groups completed questionnaires after consultation, assessing empowerment, self‐efficacy, parent and physician satisfaction, family centredness of care, and experiences using the tool. Additionally, 13 parents were interviewed. Results Parents who used the WWW‐roadmap looked up more information on the Internet. No other differences between parents and physicians were found. In the interviews, parents said that the WWW‐roadmap was a useful tool for looking up information, exploring and asking questions, and maintaining a comprehensive picture. Conclusion Using the WWW‐roadmap prior to consultation did not improve self‐efficacy, satisfaction, or family centredness of care. Findings suggest positive experiences regarding factors determining empowerment, creating conditions for a more equal parent–physician relationship. The WWW‐roadmap is useful for parents to explore their needs and find information, but more is needed to support empowerment in consultations.

SummaryBackground A family-centred approach to services of children with disabilities is widely accepted as the foundational approach to service delivery in paediatric health care. The 56 items of the Measure of Processes of Care questionnaire (MPOC-56) all reflect elements of family-centred service. In this study, we investigated which elements of family-centred service are rated important by parents of children with cerebral palsy by adding a question on importance to each item of the MPOC-56 (MPOC-56-I). Methods In total, 175 parents of children with cerebral palsy completed the MPOC-56-I. For each MPOC item, parents were asked to rate the importance on a 5-point scale ranging from 0 (not important at all) up to and including 4 (very important). We used Spearman's rank correlation coefficient to further explore the variation in parents' importance ratings. Results Parents' importance ratings of the MPOC-56 items varied. The percentage of parents rating an item important (importance rating 3 or 4) varied between 43.8% and 96.8%. The percentage of parents rating an item unimportant (rating 0 or 1) varied between 0.0% and 20.3%, and the percentage of parents rating an item neutral (rating 2) varied between 3.0% and 36.0%. Most diverse importance ratings were found for five items concerning the provision of general information. Three correlations between these items and child and parent characteristics were found. Six items were rated important by almost all (≥95%) parents. These items concern elements of specific information about the child, co-ordinated and comprehensive care for child and family and enabling and partnership. Conclusions Parents rate the importance of family-centred services for their situation in various ways. These findings endorse that family-centred services should recognize the uniqueness of families and should be tailored to what parents find important.

Aim: To describe the course of parents' perceptions of the family centredness of rehabilitation services provided to their children with cerebral palsy (CP) before and after the transition from preschool to school-based services. Background: Parents of 59 children with CP aged 2.5 to 4.5 years filled in the 56-item Measure of Processes of Care (MPOC-56) on three occasions pre (2) and post (1) transition to school-based services. Friedman tests were used to describe changes in parents' perceptions over time. Mann–Whitney U tests were used to describe differences in course of parents' perceptions between regular school and special school or day care. Results: Parents' perceptions of preschool services were stable between the ages of 2.5 and 3.5 years, with a decline after transition on four of the five domains of the MPOC (P

Background: Valid tools to assess family needs for children with physical disabilities are needed to help tune paediatric rehabilitation care processes to individual needs of these families. To create such a family needs inventory, needs of families of children with a physical disability (age 0-18 years) were identified. We examined differences in the number and type of needs listed by families when asked for by means of an interview compared with using an inventory. Methods: Forty-nine families of children with a wide variety of physical disabilities (mean age 7.7 years; SD 4.6) participated in semi-structured interviews, focusing on family needs. They also checked an inventory of 99 items (based on a previously conducted literature review), regarding their family needs. In addition, individual interviews with healthcare professionals, and panel meetings with healthcare professionals and parents were held to further identify relevant family needs for the inventory. Results: The individual parent and healthcare professional interviews raised 41 needs that were not included in the original inventory of 99 items. Moreover, the panel meetings raised a further 49 needs. After restructuring and reformulating several items, a 187-item Family Needs Inventory - Paediatric Rehabilitation (FNI-PR) was created. The parent interviews revealed significantly less family needs (mean number of needs = 10.8; SD = 6.0) compared with using the inventory (mean number of needs = 31.7; SD = 19.7) (P < 0.0001). Most expressed family needs were related to both general and specific information concerning the child's development and treatment, aids and information about legislation and to rules relating to compensation of costs. Conclusion: Based on responses of parents and healthcare professionals the FNI-PR has been developed, a comprehensive inventory for family needs that can be used in paediatric rehabilitation. An inventory checked by parents resulted in more family needs than a single open-ended question. The inventory may facilitate the implementation of family-centred care.

The purpose of this paper is to describe the course of the health-related quality of life (HR-QoL) of children with cerebral palsy (CP) between the ages of 2.5 and 4.5 years, at both group and individual level. We also examined whether CP characteristics are helpful in understanding which children show a decrease in HR-QoL. HR-QoL of 72 children with CP was measured using the TNO-AZL Preschool children Quality of Life (TAPQOL) questionnaire at the ages of 2.5, 3.5 and 4.5 years. The course of HR-QoL was compared between groups with different CP characteristics. Median scores for 10 of the 12 domains of the TAPQOL were found to be stable between ages 2.5 and 4.5 years. However, individual children showed great changes in HR-QoL at these ages, for all domains. A larger proportion of children with less severe CP showed a decrease in HR-QoL for the behaviour problems domain (p = 0.02), and a larger proportion of unilaterally affected children showed a decrease in HR-QoL regarding the anxiety (p

A family-centred approach to services of children with disabilities is widely accepted as the foundational approach to service delivery in paediatric health care. The 56 items of the Measure of Processes of Care questionnaire (MPOC-56) all reflect elements of family-centred service. In this study, we investigated which elements of family-centred service are rated important by parents of children with cerebral palsy by adding a question on importance to each item of the MPOC-56 (MPOC-56-I).In total, 175 parents of children with cerebral palsy completed the MPOC-56-I. For each MPOC item, parents were asked to rate the importance on a 5-point scale ranging from 0 (not important at all) up to and including 4 (very important). We used Spearman's rank correlation coefficient to further explore the variation in parents' importance ratings.Parents' importance ratings of the MPOC-56 items varied. The percentage of parents rating an item important (importance rating 3 or 4) varied between 43.8% and 96.8%. The percentage of parents rating an item unimportant (rating 0 or 1) varied between 0.0% and 20.3%, and the percentage of parents rating an item neutral (rating 2) varied between 3.0% and 36.0%. Most diverse importance ratings were found for five items concerning the provision of general information. Three correlations between these items and child and parent characteristics were found. Six items were rated important by almost all (≥95%) parents. These items concern elements of specific information about the child, co-ordinated and comprehensive care for child and family and enabling and partnership.Parents rate the importance of family-centred services for their situation in various ways. These findings endorse that family-centred services should recognize the uniqueness of families and should be tailored to what parents find important.

To describe the course of parents' perceptions of the family centredness of rehabilitation services provided to their children with cerebral palsy (CP) before and after the transition from preschool to school-based services.Parents of 59 children with CP aged 2.5 to 4.5 years filled in the 56-item Measure of Processes of Care (MPOC-56) on three occasions pre (2) and post (1) transition to school-based services. Friedman tests were used to describe changes in parents' perceptions over time. Mann-Whitney U tests were used to describe differences in course of parents' perceptions between regular school and special school or day care.Parents' perceptions of preschool services were stable between the ages of 2.5 and 3.5 years, with a decline after transition on four of the five domains of the MPOC (P 0.05). The domain providing general information was scored lowest (median at baseline 3.56, IQR 2.39) compared with the four other MPOC domains, but remained stable over time. No differences in course of parental perceptions were found for school type.The transition from preschool to school-based services for children with CP is associated with a decrease in parents' perception of family centredness independent of the type of school. The transition in services has a negative impact on perceived family-centred practices.

Valid tools to assess family needs for children with physical disabilities are needed to help tune paediatric rehabilitation care processes to individual needs of these families. To create such a family needs inventory, needs of families of children with a physical disability (age 0-18 years) were identified. We examined differences in the number and type of needs listed by families when asked for by means of an interview compared with using an inventory.Forty-nine families of children with a wide variety of physical disabilities (mean age 7.7 years; SD 4.6) participated in semi-structured interviews, focusing on family needs. They also checked an inventory of 99 items (based on a previously conducted literature review), regarding their family needs. In addition, individual interviews with healthcare professionals, and panel meetings with healthcare professionals and parents were held to further identify relevant family needs for the inventory.The individual parent and healthcare professional interviews raised 41 needs that were not included in the original inventory of 99 items. Moreover, the panel meetings raised a further 49 needs. After restructuring and reformulating several items, a 187-item Family Needs Inventory - Paediatric Rehabilitation (FNI-PR) was created. The parent interviews revealed significantly less family needs (mean number of needs = 10.8; SD = 6.0) compared with using the inventory (mean number of needs = 31.7; SD = 19.7) (P0.0001). Most expressed family needs were related to both general and specific information concerning the child's development and treatment, aids and information about legislation and to rules relating to compensation of costs.Based on responses of parents and healthcare professionals the FNI-PR has been developed, a comprehensive inventory for family needs that can be used in paediatric rehabilitation. An inventory checked by parents resulted in more family needs than a single open-ended question. The inventory may facilitate the implementation of family-centred care.

Objective. The study objective is to investigate the Health Related Quality of Life (HRQoL) of children with cerebral palsy at the age of 2.5 years in comparison with their healthy peers and to investigate the correlation of gross motor functioning and HRQoL. Design. At 89 children included in the PERRIN CP 0-5 study various characteristics of development were assessed. HRQoL was measured using the TAPQOL, and gross motor functioning was classified using the GMFCS. HRQoL scores of the group of children with cerebral palsy were compared to the scores of their healthy peers. Also the correlation between HRQoL and GMFCS was investigated. Results. A statistical significant difference in HRQoL was found between the two groups of children at six of twelve scales of the TAPQOL (motor functioning, social functioning, communication, liveliness, problem behaviour, and lung problems). On the domain problem behavior, the group of children with CP had a better HRQoL, on the other domains the healthy peers scored better. Of these domains, all except motor functioning were statistically significant related to GMFCS level (range |rs| 0.26-0.44). Conclusion. Children with cerebral palsy have a worse Health-Related Quality of Life compared to their healthy peers. Level of motor functioning as classified using the GMFCS is related to HRQoL. In general, children with a lower level of motor functioning have a worse Health Related Quality of Life.