Publications by authors named "Lotte Haverman"

50 Publications

Pediatric patients report lower Health-Related Quality of Life in daily clinical practice compared to new normative PedsQL data.

Acta Paediatr 2021 Apr 10. Epub 2021 Apr 10.

Amsterdam UMC, University of Amsterdam, Emma Children's Hospital, Psychosocial Department, Meibergdreef 9, Amsterdam, The Netherlands.

Aim: To compare Health-Related Quality of Life (HRQOL) of pediatric patients with newly collected HRQOL data of the general Dutch population, explore responses to individual items and investigate variables associated with HRQOL.

Methods: Children (8-12y) and adolescents (13-17y) from the general population (N=966) and from a pediatric population (N=1209), completed the Pediatric Quality of Life Inventory (PedsQL ) online via the KLIK Patient Reported Outcome Measures portal. PedsQL scale scores were compared between groups with independent t-tests, by age-group and gender. Responses to PedsQL items were explored using descriptive analyses. Linear regression analyses were performed to determine which variables were associated with HRQOL.

Results: Pediatric patients reported worse HRQOL than the general population on all PedsQL scales (p≤.001, d=0.20-1.03), except social functioning, and a high proportion reported problems on PedsQL items, e.g., 'I have trouble sleeping'. Younger age, female gender and school absence were negatively associated with HRQOL (β=-0.37-0.10, p≤.008).

Conclusion: Pediatric patients reported lower HRQOL than the general population, and school absence, female gender and younger age was associated with lower HRQOL. The results underline the importance to structurally monitor pediatric patients' HRQOL in clinical practice to detect problems and offer the right help on time.
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http://dx.doi.org/10.1111/apa.15872DOI Listing
April 2021

Preconception expanded carrier screening: a focus group study with relatives of mucopolysaccharidosis type III patients and the general population.

J Community Genet 2021 Mar 22. Epub 2021 Mar 22.

Amsterdam UMC, Department of Clinical Genetics, Amsterdam Reproduction and Development Research Institute, Vrije Universiteit Amsterdam, PO Box 7057, 1007, MB, Amsterdam, the Netherlands.

Preconception expanded carrier screening (ECS) enables prospective parents to assess their risk of having a child with an autosomal recessive disorder. Knowledge on motivations, feelings, and considerations people have towards the offer and use of ECS is limited. To enrich the public and professional discussion on ECS implementation, this study explored the perspectives towards various aspects of ECS in seven focus groups compromising first- and second-degree relatives of MPS III patients (N=9, N=4, N=5, N=5) and members of the general Dutch population (N=6, N=7, N=5). The focus groups were audio recorded and the transcripts were qualitatively analyzed to identify themes. Both relatives of MPS III patients and participants from the general population supported offering ECS, in particular for severe, childhood-onset disorders. Important barriers identified for ECS were a lack of genetic knowledge and a perceived lack of personal relevance and awareness, as well as out-of-pocket costs of testing. The majority of participants would prefer full disclosure of individual test results instead of couple-based test results. Moreover, offering people a choice for the way of reporting was proposed. All participants agreed that more efforts, for example by governmental campaigns, should be made to increase awareness on the availability, potentials, and limitations of ECS. Educating prospective parents about ECS is essential for increasing awareness and informed decision making. This study provides valuable insights that can be used by governments and public health authorities when considering implementation of preconception ECS.
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http://dx.doi.org/10.1007/s12687-021-00519-2DOI Listing
March 2021

How to use the patient-reported outcomes measurement information system (PROMIS) in international orthopaedic trauma research: A practical approach: Letter to the Editor regarding "Is PROMIS the new standard for patient-reported outcomes measures in orthopaedic trauma research?" by Nathan N. O'Hara et al. Injury May 2020.

Injury 2021 Mar 12. Epub 2021 Mar 12.

Department of Child and Adolescent Psychiatry and Psychosocial Care, Amsterdam Reproduction and Development, Amsterdam Public Health, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, The Netherlands; Department of Strategy and Policy, Amsterdam UMC, Vrije Universiteit Amsterdam, Amsterdam, The Netherlands. Electronic address:

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http://dx.doi.org/10.1016/j.injury.2021.03.021DOI Listing
March 2021

Reducing posttraumatic stress in parents of patients with a rare inherited metabolic disorder using eye movement desensitization and reprocessing therapy: a case study.

Orphanet J Rare Dis 2021 Mar 10;16(1):126. Epub 2021 Mar 10.

Department of Child and Adolescent Psychiatry, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Parents of children with severe inborn errors of metabolism frequently face stressful events related to the disease of their child and are consequently at high risk for developing parental posttraumatic stress disorder (PTSD). Assessment and subsequent treatment of PTSD in these parents is however not common in clinical practice. PTSD can be effectively treated by Eye Movement Desensitization and Reprocessing (EMDR), however no studies have been conducted yet regarding the effect of EMDR for parental PTSD. EMDR is generally offered in multiple weekly sessions which may preclude participation of parents as they are generally overburdened by the ongoing and often intensive care for their child. Therefore, we offered time-limited EMDR with a maximum of four sessions over two subsequent days to two parents of mucopolysaccharidosis type III (MPS III) patients to explore its potential effects. Both qualitative and quantitative outcomes were used to evaluate treatment effects. Both parents felt more resilient and competent to face future difficulties related to the disease of their child, and no adverse effects were reported. Quantitative outcomes showed a clinically significant decrease in post traumatic stress symptoms and comorbid psychological distress from pre- to post treatment, and these beneficial effects were maintained at follow-up. In conclusion, time-limited EMDR may be a highly relevant treatment for traumatized parents of children with MPS III, and probably also for parents of children with other rare progressive disorders. Further research is needed to validate the efficacy of EMDR in this specific population.
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http://dx.doi.org/10.1186/s13023-021-01768-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7948366PMC
March 2021

Psychometric properties of the Patient-Reported Outcomes Measurement Information System (PROMIS®) pediatric item bank peer relationships in the Dutch general population.

Qual Life Res 2021 Feb 19. Epub 2021 Feb 19.

Child and Adolescent Psychiatry & Psychosocial Care, Amsterdam Reproduction and Development, Amsterdam Public Health, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Meibergdreef 9, Postbus 22660, 1100 AD, Amsterdam, The Netherlands.

Purpose: This study aimed to validate the PROMIS Pediatric item bank v2.0 Peer Relationships and compare reliability of the full item bank to its short form, computerized adaptive test (CAT) and the social functioning (SF) subscale of the Pediatric Quality of Life Inventory (PedsQL™).

Methods: Children aged 8-18 (n = 1327), representative of the Dutch population completed the Peer Relationships item bank. A graded response model (GRM) was fit to the data. Structural validity was assessed by checking item-fit statistics (S-X, p < 0.001 = misfit). For construct validity, a moderately strong correlation (> 0.50) was expected between Peer Relationships and the PedsQL SF subscale. Cross-cultural DIF between U.S. and NL was assessed using logistic regression, where an item with McFadden's pseudo R > 0.02 was considered to have DIF. Percentage of participants reliably measured was assessed using the standard error of measurement (SEM) < 0.32 as a criterion (reliability of 0.90). Relative efficiency ((1-SEM)/n) was calculated to compare how well the instruments performed relative to the amount of items administered.

Results: In total, 527 (response rate: 39.7%) children completed the PROMIS v2.0 Peer Relationships item bank (n = 15) and the PedsQL™ (n = 23). Structural validity of the Peer Relationships item bank was sufficient, but one item displayed misfit in the GRM model (S-X < 0.001); 5152R1r ("I played alone and kept to myself"). The item 733R1r ("I was a good friend") was the only item that displayed cross-cultural DIF (R = 0.0253). The item bank correlated moderately high (r = 0.61) with the PedsQL SF subscale Reliable measurements were obtained at the population mean and > 2SD in the clinically relevant direction. CAT outperformed all other measures in efficiency. Mean T-score of the Dutch general population was 46.9(SD 9.5).

Conclusion: The pediatric PROMIS Peer Relationships item bank was successfully validated for use within the Dutch population and reference data are now available.
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http://dx.doi.org/10.1007/s11136-021-02781-wDOI Listing
February 2021

Patient-reported outcome measures in pediatric solid organ transplantation: Exploring stakeholder perspectives on clinical implementation through qualitative description.

Qual Life Res 2021 Jan 14. Epub 2021 Jan 14.

Canadian Donation and Transplantation Research Program, Edmonton, Canada.

Purpose: Patient-reported outcome measures (PROMs) are standardized instruments used to collect data about the subjective assessment of medical care from the patient perspective. Implementing PROMs within pediatric clinical settings has gained increasing importance as health services prioritize patient-centred pediatric care. This study explores the perspectives of pediatric solid organ transplant patients, caregivers, and healthcare practitioners (HCPs) on implementing PROMs into clinical practice.

Methods: Qualitative description methods were used to elicit stakeholder perspectives. Semi-structured interviews were conducted across five Canadian transplant centres. Purposive sampling was used to obtain maximum variation across age, gender, and transplant program for all participants, as well as discipline for HCPs.

Results: The study included a total of 63 participants [patients (n = 20), caregivers (n = 22) and HCPs (n = 21)]. Nearly all participants endorsed the implementation of PROMs to enhance pediatric transplant clinical care. Three primary roles for PROMs emerged: (1) to bring a transplant patient's overall well-being into the clinical care conversation; (2) to improve patient communication and engagement; and, (3) to inform the practice of clinical pediatric transplant care. Insights for effective implementation included completing electronic PROMs remotely and prior to clinical appointments by patients who are eight to 10 years of age or older.

Conclusions: This study contributes to current research that supports the use of PROMs in clinical pediatric care and guides their effective implementation into practice. Future directions include the development, usability testing, and evaluation of a proposed electronic PROM platform that will inform future research initiatives.
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http://dx.doi.org/10.1007/s11136-020-02743-8DOI Listing
January 2021

Managing women-specific bleeding in inherited bleeding disorders: A multidisciplinary approach.

Haemophilia 2020 Dec 13. Epub 2020 Dec 13.

Van Creveldkliniek, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands.

Introduction: Multidisciplinary management of women-specific bleeding is important to preserve quality of life, healthy reproduction and social participation of women and girls with bleeding disorders (WBD).

Aim: To support appropriate multidisciplinary care for WBD in haemophilia treatment centres.

Methods: Two case examples are presented and management issues discussed from different health care perspectives, including the nurse, patient, psychologist, gynaecologist, geneticist, psychosexual therapist and haematologist.

Results: Woman with bleeding disorders may experience heavy menstruation from menarche onwards. This has a physical and psychosocial impact requiring a multidisciplinary approach. If a woman with an inherited bleeding disorder desires to become pregnant, preconception counselling is essential, to discuss genetic diagnosis, state of the art treatment options for the bleeding disorder in question and possible choices to prevent having an affected child, as well as maternal bleeding risks during conception, delivery and the post-partum period.

Conclusion: Adequate management and good education of WBD requires a patient-centred multidisciplinary approach with experienced specialists in a haemophilia treatment centre.
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http://dx.doi.org/10.1111/hae.14221DOI Listing
December 2020

Parenting a child with Marfan syndrome: Distress and everyday problems.

Am J Med Genet A 2021 01 9;185(1):50-59. Epub 2020 Oct 9.

Department of Pediatrics, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands.

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.
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http://dx.doi.org/10.1002/ajmg.a.61906DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7756496PMC
January 2021

Preconception expanded carrier screening: Impact of information presented by text or video on genetic knowledge and attitudes.

J Genet Couns 2021 Apr 17;30(2):457-469. Epub 2020 Sep 17.

Psychosocial department, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, Netherlands.

Preconception expanded carrier screening (ECS) aims to identify couples with an increased risk of having a child with an autosomal recessive (AR) disorder before pregnancy, thereby enabling reproductive choices. Genetic knowledge and experiential knowledge both influence the uptake of ECS. As people in the general public often lack such knowledge, it is essential to provide appropriate and understandable information when offering ECS. This study investigated the effect of an educational video, compared to an educational text, on the knowledge and attitudes toward preconception ECS in the general population. Both the text and video consisted of a brief educational summary on AR inheritance and on the type of disorders included in ECS, with the progressive neurodegenerative condition mucopolysaccharidosis type III (MPS III) as an example. Participants in the reproductive age were invited in collaboration with a research agency. Respondents (N = 789) were offered an educational video prior to completing an online questionnaire that examined genetic knowledge, the perceived severity of MPS III, perceived risk, and attitudes toward ECS. Outcomes were compared to reference data collected previously in which respondents had been offered an educational text (N = 781). We first again studied the attitudes toward ECS in a smaller educational text group (N = 266) in order to assess whether attitudes had changed over time due to increased media coverage on ECS, which did not reveal any significant changes. Respondents who were offered the video had a better genetic knowledge, perceived MPS III as more severe, perceived their risks higher and were more likely to participate in ECS compared to those who were offered text. Online video may well be used as supportive tool to the genetic counseling process, creating more knowledge on ECS and severe genetic disorders included in preconception screening panels.
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http://dx.doi.org/10.1002/jgc4.1332DOI Listing
April 2021

Patient-Reported Outcome Measures in Routine Pediatric Clinical Care: A Systematic Review.

Front Pediatr 2020 28;8:364. Epub 2020 Jul 28.

Department of Community Health Sciences, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada.

Integration of patient-reported outcome measures (PROMs) in routine clinical care is growing but lacks consolidated evidence around its impact on pediatric care. This systematic review aims to evaluate the impact of integrating PROMs in routine pediatric clinical care on various outcomes in pediatric clinical care. MEDLINE, Embase, CINAHL, PsycINFO, and Cochrane Library. Web of Science database was searched selectively to ensure extended coverage. We included longitudinal studies reporting on the integration of PROMs in routine pediatric clinical care of chronic diseases. Studies in languages other than English, published prior to the year 2000, and reporting on secondary data were excluded. Two reviewers independently extracted data from included studies. Extracted data included citation of each study, type of healthcare setting, location of the study, characteristics of patient population, type of chronic disease, name and type of PROM, mode of administration, and reported outcomes. Out of 6,869 articles, titles and abstracts of 5,416 articles and full text of 23 articles were screened in duplicate. Seven articles reporting results from six studies met eligibility criteria. Integration of PROMs increased the identification and discussion around health-related quality of life (HRQOL), especially in psychosocial and emotional domains, but showed mixed results with the impact on quality of care. No studies assessed the impact of integrating PROMs on healthcare utilization. Due to significant heterogeneity in the studies, a meta-analysis was not conducted. Integrating PROMs could have a positive impact on HRQOL; however, further studies are required to determine the impact of PROMs in routine pediatric clinical care.
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http://dx.doi.org/10.3389/fped.2020.00364DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7399166PMC
July 2020

Illness cognitions associated with health-related quality of life in young adult men with haemophilia.

Haemophilia 2020 Sep 25;26(5):793-799. Epub 2020 Aug 25.

Psychosocial Department, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Introduction And Aim: Knowledge on patterns of beliefs about the illness (illness cognitions) can provide insight into individual differences in adjustment to haemophilia. The current study aimed to identify (a) which sociodemographic and disease characteristics were associated with illness cognitions and (b) which illness cognitions were associated with health-related quality of life (HRQOL) in young adult men with haemophilia, besides sociodemographic and disease characteristics.

Methods: Young adult men (18-30 years) with haemophilia in the Netherlands participated in an online multicentre cross-sectional study. Participants completed the Pediatric Quality of Life Inventory Young Adult version (PedsQL_YA). Potential sociodemographic determinants were assessed with the Course of Life Questionnaire (CoLQ) and illness cognitions with the Illness Cognition Questionnaire (ICQ). Multiple linear regression analyses were performed to assess potential determinants of illness cognitions and HRQOL.

Results: Seventy young adult men with haemophilia (mean age 24.7 years, SD 3.5) participated. Born outside the Netherlands (β -0.24) and >1 bleed past 6 months (β -0.32) were associated with less acceptance of the disease. More acceptance was associated with better HRQOL in all domains: β 0.23-0.39. More helplessness was associated with worse total (β -0.30) and physical (β -0.42) HRQOL. Disease benefits, sociodemographic and disease characteristics were not associated with HRQOL.

Conclusion: Illness cognitions are associated with HRQOL in young adult men with haemophilia. Early recognition and identification of illness cognitions are important to facilitate support and psychosocial treatment to optimize young adults' well-being. Extra attention is needed for young adult men with frequent bleeds because they are at risk of lowered levels of acceptance of the disease.
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http://dx.doi.org/10.1111/hae.14120DOI Listing
September 2020

A retrospective assessment of the KLIK PROM portal implementation using the Consolidated Framework for Implementation Research (CFIR).

Qual Life Res 2020 Aug 15. Epub 2020 Aug 15.

Psychosocial Department, G8-136, Emma Children's Hospital/Amsterdam UMC, University of Amsterdam, Meibergdreef 9, Postbox 2266, 1100 DD, Amsterdam, The Netherlands.

Purpose: The KLIK Patient-Reported Outcome Measure (PROM) portal is an evidence-based intervention implemented in clinical practice in > 25 Dutch hospitals for patients (children and adults) who regularly visit the outpatient clinic. Implementation science frameworks can be used to understand why implementation succeeded or failed, to structure barriers and enablers, and to develop implementation strategies to overcome barriers. This paper aimed to (A) retrospectively describe determinants of successful KLIK PROM implementation using the Consolidated Framework for Implementation Research (CFIR), and (B) identify current barriers and match implementation strategies.

Methods: (A) The KLIK implementation process was described retrospectively based on literature and experience, using the 39 CFIR constructs organized in five general domains: intervention characteristics, outer setting, inner setting, characteristics of individuals, and implementation process. (B) The CFIR-Expert Recommendations for Implementing Change (ERIC) Implementation Strategy Matching tool identified current barriers in the KLIK implementation and matched implementation strategies that addressed the identified barriers.

Results: (A) The most prominent determinants of successful KLIK PROM implementation lie in the following CFIR domains: intervention characteristics (e.g., easy to use), characteristics of individuals (e.g., motivation), and process of implementation (e.g., support). (B) 13 CFIR constructs were identified as current barriers for implementing the KLIK PROM portal. The highest overall advised ERIC strategy for the specific KLIK barriers was to identify and prepare champions.

Conclusion: Using an implementation science framework, e.g., CFIR, is recommended for groups starting to use PROMs in clinical care as it offers a structured approach and provides insight into possible enablers and barriers.
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http://dx.doi.org/10.1007/s11136-020-02586-3DOI Listing
August 2020

Using an implementation science approach to implement and evaluate patient-reported outcome measures (PROM) initiatives in routine care settings.

Qual Life Res 2020 Jul 10. Epub 2020 Jul 10.

Nuffield Department of Population Health, University of Oxford, Oxford, UK.

Purpose: Patient-reported outcome and experience measures (PROMs/PREMs) are well established in research for many health conditions, but barriers persist for implementing them in routine care. Implementation science (IS) offers a potential way forward, but its application has been limited for PROMs/PREMs.

Methods: We compare similarities and differences for widely used IS frameworks and their applicability for implementing PROMs/PREMs through case studies. Three case studies implemented PROMs: (1) pain clinics in Canada; (2) oncology clinics in Australia; and (3) pediatric/adult clinics for chronic conditions in the Netherlands. The fourth case study is planning PREMs implementation in Canadian primary care clinics. We compare case studies on barriers, enablers, implementation strategies, and evaluation.

Results: Case studies used IS frameworks to systematize barriers, to develop implementation strategies for clinics, and to evaluate implementation effectiveness. Across case studies, consistent PROM/PREM implementation barriers were technology, uncertainty about how or why to use PROMs/PREMs, and competing demands from established clinical workflows. Enabling factors in clinics were context specific. Implementation support strategies changed during pre-implementation, implementation, and post-implementation stages. Evaluation approaches were inconsistent across case studies, and thus, we present example evaluation metrics specific to PROMs/PREMs.

Conclusion: Multilevel IS frameworks are necessary for PROM/PREM implementation given the complexity. In cross-study comparisons, barriers to PROM/PREM implementation were consistent across patient populations and care settings, but enablers were context specific, suggesting the need for tailored implementation strategies based on clinic resources. Theoretically guided studies are needed to clarify how, why, and in what circumstances IS principles lead to successful PROM/PREM integration and sustainability.
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http://dx.doi.org/10.1007/s11136-020-02564-9DOI Listing
July 2020

Matching Psychosocial Support Needs of Parents of a Child with a Chronic Illness to a Feasible Intervention.

Matern Child Health J 2020 Oct;24(10):1238-1247

Psychosocial Department (G8-136), Emma Children's Hospital, Amsterdam University Medical Centers, Meibergdreef 9, 1105 AZ, Amsterdam, the Netherlands.

Objectives: Parents of children with a chronic illness (CI) are at risk for psychosocial problems. The aim of this study was to refine an existing face-to-face intervention into an online psychosocial group intervention for parents by (1) exploring which themes are important, (2) determine what type of intervention parents would like and (3) assess parents' practical preferences.

Methods: Parents of children with a CI (0-18 years) were invited to complete an online questionnaire. To acquire more in-depth information, focus groups and telephone interviews were conducted. Descriptive statistics were used.

Results: 272 parents (mean age = 43.1 years, 85% female) participated. Three focus groups (15 parents) and seven telephone interviews were conducted. Most important themes were: the CI of the child, family functioning, taking care of yourself, relationships with others and practical support. Parents preferred a group with parents of children in the same age category. At first, parents preferred face-to-face contact. After an explanation and demonstration of an online intervention, parents became more positive about online support, mostly because they could participate from home.

Conclusions For Practice: Parents have a need for psychosocial support focusing on different themes. Professionals should explain and demonstrate an online intervention to parents. Based on these results, Op Koers Online for parents was developed. An RCT to assess feasibility and effectiveness of the intervention is currently running.
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http://dx.doi.org/10.1007/s10995-020-02925-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7476963PMC
October 2020

Attitudes of relatives of mucopolysaccharidosis type III patients toward preconception expanded carrier screening.

Eur J Hum Genet 2020 10 1;28(10):1331-1340. Epub 2020 Jun 1.

Amsterdam UMC, Psychosocial Department, Emma Children's Hospital, University of Amsterdam, Meibergdreef 9, Amsterdam, The Netherlands.

Preconception expanded carrier screening (ECS) aims to detect carrier couples of autosomal recessive (AR) disorders before pregnancy in order to increase reproductive autonomy of prospective parents. Genetic knowledge and knowledge gained from experience influence decision making on participation in genetic testing and understanding carrier test results. In this study we assessed whether parents and relatives of patients with the severe AR condition mucopolysaccharidosis type III (MPS III), who are expected to have genetic and experiential knowledge, have more positive attitudes toward ECS than the Dutch reference group. Parents of all MPS III patients known to the Dutch expert center were invited to participate and asked to invite first and second degree relatives. The online questionnaire started with an educational text, and assessed attitudes toward ECS, genetic knowledge and perceived MPS III severity. Results were compared with the Dutch population. Parents and relatives of MPS III patients (n = 159) scored higher on the genetic knowledge test and perceived MPS III as more severe compared with the general Dutch population (n = 781). Parents and relatives reported to be more likely to participate in ECS (84.3% and 62.5%, respectively) compared with the public (31%) (p < 0.001). Being a relative of a MPS III patient was the strongest variable in the regression analyses for intended ECS participation. Our results show that genetic knowledge influences ECS decision making. Therefore, appropriate information on ECS and genetic counseling is needed to enable prospective parents from the general population, including relatives of patients with severe hereditary disorders, to make informed decisions.
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http://dx.doi.org/10.1038/s41431-020-0648-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7609293PMC
October 2020

Clinicians' perspective on the implemented KLIK PROM portal in clinical practice.

Qual Life Res 2020 May 28. Epub 2020 May 28.

Psychosocial Department, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, G8-136, Meibergdreef 9, Postbox 22660, 1100 DD, Amsterdam, The Netherlands.

Purpose: Since 2011, the evidence-based KLIK Patient Reported Outcome Measure (PROM) portal has been implemented in clinical practice in > 20 Dutch hospitals. Patients and/or parents complete PROMs on Health Related Quality of Life, symptoms and psychosocial functioning before their outpatient consultation. Answers are converted into an ePROfile and discussed by clinicians during consultation to monitor well-being over time and detect problems early. This study aims to get insight into the KLIK implementation from the clinician's perspective.

Methods: As part of the KLIK implementation process, annual meetings were held with multidisciplinary teams to evaluate the use of KLIK. An online questionnaire was sent regarding (1) overall satisfaction, (2) feeling competent to discuss PROMs, (3) use of KLIK during the consultation, (4) influence of KLIK on the consultation, (5) usability of the KLIK PROM portal, (6) satisfaction with PROMs and feedback, and (7) support of the KLIK expert team. Open questions about (dis)advantages were included. Descriptive analyses were used.

Results: One hundred and forty-eight clinicians (response-rate 61%) from 14 hospitals in the Netherlands participated. Results show that: (1) clinicians report an overall satisfaction of median = 69/100 (visual analogue scale), (2) 85.8% feel competent discussing the ePROfile, (3) 70.3% (almost) always discuss the ePROfile, (4) 70.3% think that KLIK improves consultation, (5) 71.6% think KLIK is easy to use, (6) 80.4% are satisfied with the feedback of the overall KLIK ePROfile, (7) 71.6% experience sufficient support of the KLIK team.

Conclusion: Participating clinicians are generally satisfied with KLIK. Improvements to the KLIK PROM portal are now realized based on the mentioned disadvantages (e.g., shorten PROM completion by use of PROMIS and integrating KLIK with Electronic Health Records).
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http://dx.doi.org/10.1007/s11136-020-02522-5DOI Listing
May 2020

Psychometric Properties of the Pediatric Patient-Reported Outcomes Measurement Information System Item Banks in a Dutch Clinical Sample of Children With Juvenile Idiopathic Arthritis.

Arthritis Care Res (Hoboken) 2020 12;72(12):1780-1789

Emma Children's Hospital, Amsterdam University Medical Center, University of Amsterdam, Amsterdam, The Netherlands.

Objective: To assess the psychometric properties of 8 pediatric Patient-Reported Outcomes Measurement Information System (PROMIS) item banks in a clinical sample of children with juvenile idiopathic arthritis (JIA).

Methods: A total of 154 Dutch children (mean ± SD age 14.4 ± 3.0 years; range 8-18 years) with JIA completed 8 pediatric version 1.0 PROMIS item banks (anger, anxiety, depressive symptoms, fatigue, pain interference, peer relationships, physical function mobility, physical function upper extremity) twice and the Pediatric Quality of Life Inventory (PedsQL) and the Childhood Health Assessment Questionnaire (C-HAQ) once. Structural validity of the item banks was assessed by fitting a graded response model (GRM) and inspecting GRM fit (comparative fit index [CFI], Tucker-Lewis index [TLI], and root mean square error of approximation [RMSEA]) and item fit (S-X statistic). Convergent validity (with PedsQL/C-HAQ subdomains) and discriminative validity (active/inactive disease) were assessed. Reliability of the item banks, short forms, and computerized adaptive testing (CAT) was expressed as the SE of theta (SE[θ]). Test-retest reliability was assessed using intraclass correlation coefficients (ICCs) and smallest detectable change.

Results: All item banks had sufficient overall GRM fit (CFI >0.95, TLI >0.95, RMSEA <0.08) and no item misfit (all S-X P > 0.001). High correlations (>0.70) were found between most PROMIS T scores and hypothesized PedsQL/C-HAQ (sub)domains. Mobility, pain interference, and upper extremity item banks were able to discriminate between patients with active and inactive disease. Regarding reliability, PROMIS item banks outperformed legacy instruments. Post hoc CAT simulations outperformed short forms. Test-retest reliability was strong (ICC >0.70) for all full-length item banks and short forms, except for the peer relationships item bank.

Conclusion: The pediatric PROMIS item banks displayed sufficient psychometric properties for Dutch children with JIA. PROMIS item banks are ready for use in clinical research and practice for children with JIA.
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http://dx.doi.org/10.1002/acr.24094DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7756261PMC
December 2020

Prevention of relapses with levamisole as adjuvant therapy in children with a first episode of idiopathic nephrotic syndrome: study protocol for a double blind, randomised placebo-controlled trial (the LEARNS study).

BMJ Open 2019 08 1;9(8):e027011. Epub 2019 Aug 1.

Paediatric Nephrology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Introduction: Idiopathic nephrotic syndrome (INS) is characterised by a high relapse rate up to 80% after initial response to standard therapy with corticosteroids. Steroid toxicity is common and causes a great burden of disease that negatively influences the health-related quality of life (HRQoL). Recently, studies have shown that levamisole, an anthelminthic drug, significantly improves relapse-free survival in children with frequent relapses or steroid dependency. Compared with other steroid-sparing drugs, levamisole has relatively few side effects. We hypothesise that adding levamisole to standard therapy with corticosteroids in children with a first episode of INS will prevent relapses, decrease cumulative dosage of steroids used and improve HRQoL. This paper presents the study protocol for the LEARNS study (LEvamisole as Adjuvant therapy to Reduce relapses of Nephrotic Syndrome).

Methods And Analysis: An international, double-blind, placebo-controlled randomised trial will be conducted in 20 participating hospitals in the Netherlands and Belgium. Participants (n=92) with a first episode of INS, aged 2-16 years, who achieve remission after 4 weeks of oral prednisolone will be randomly assigned (1:1) to receive either levamisole 2.5 mg/kg alternate day or placebo added to prednisolone (18-week tapering schedule) for a total of 24 weeks. Follow-up will be until 2 years after first presentation. Additionally, parents and/or children will fill out five HRQoL questionnaires. Primary outcome of the LEARNS study is occurrence of relapses within 12 months after first presentation. Secondary outcomes include time to first relapse, cumulative steroid dose after 2 years, safety parameters and quality of life scores.

Ethics And Dissemination: The trial was approved by the Medical Ethical Committee. Results of the study will be published in a peer-reviewed journal.

Trial Registration Number: NL6826, 2017-001025-41.
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http://dx.doi.org/10.1136/bmjopen-2018-027011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6688689PMC
August 2019

Pediatric Perceived Cognitive Functioning: Psychometric Properties and Normative Data of the Dutch Item Bank and Short Form.

J Int Neuropsychol Soc 2019 09 10;25(8):845-856. Epub 2019 Jun 10.

Pediatric Psychosocial Department, Emma Children's Hospital, Amsterdam UMC University Medical Centers, 1105AZ Amsterdam, The Netherlands.

Objective: With increasing numbers of children growing up with conditions that are associated with acquired brain injury, efficient neuropsychological screening for cognitive deficits is pivotal. Brief self-report measures concerning daily complaints can play an important role in such screening. We translated and adapted the pediatric perceived cognitive functioning (PedsPCF) self- and parent-report item bank to Dutch. This study presents (1) psychometric properties, (2) a new short form, and (3) normative data for the short form.

Methods: A general population sample of children and parents was recruited. Dimensionality of the PedsPCF was assessed using confirmatory factor analyses and exploratory bifactor analyses. Item response theory (IRT) modeling was used to evaluate model fit of the PedsPCF, to identify differential item functioning (DIF), and to select items for the short form. To select short-form items, we also considered the neuropsychological content of items.

Results: In 1441 families, a parent and/or child participated (response rate 66% at family level). Assessed psychometric properties were satisfactory and the predominantly unidimensional factor structure of the PedsPCF allowed for IRT modeling using the graded response model. One item showed meaningful DIF. For the short form, 10 items were selected.

Conclusions: In this first study of the PedsPCF outside the United States, studied psychometric properties of the translated PedsPCF were satisfactory, and allowed for IRT modeling. Based on the IRT analyses and the content of items, we proposed a new 10-item short form. Further research should determine the relation of PedsPCF outcomes with neurocognitive measures and its ability to facilitate neuropsychological screening in clinical practice.
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http://dx.doi.org/10.1017/S1355617719000572DOI Listing
September 2019

Options for the Interpretation of and Recommendations for Acting on Different PROMs in Daily Clinical Practice Using KLIK.

Med Care 2019 05;57 Suppl 5 Suppl 1:S52-S58

Psychosocial Department, Amsterdam UMC, University of Amsterdam, Emma Children's Hospital, Amsterdam.

Introduction: This paper describes the different ways in which information is being fed back to health care providers (HCPs) using the Dutch evidence-based KLIK Patient-Reported Outcome Measures (PROMs) portal (www.hetklikt.nu). KLIK was initially developed for children with a chronic illness and their parents, and recently expanded for use in adult health care. Patients complete online PROMs at home about their health-related quality of life (HRQOL), symptoms and/or psychosocial functioning before an outpatient consultation with their HCP. PROMs are subsequently converted into an individual KLIK electronic (e)PROfile and discussed during the consultation to facilitate systematic attention for HRQOL.

Methods For Pro Score Interpretation: KLIK includes a variety of different PROMs. The KLIK ePROfile initially consisted of literal representations of the individual PROM items in the European traffic light colors (red, orange, green) and only one graph. Over the years, the KLIK ePROfile evolved into a broader spectrum of feedback options; (1) literal representation of individual items, (2) summary scores, and (3) graphic representations (4 options).

Developing Recommendations For Acting On Pro Results: The primary goal of KLIK is to longitudinally monitor patient and parent self-reported or proxy-reported health outcomes, which means that the focus of KLIK is not mainly on recommending actions for PRO results. However, there are several aids that help interpretation of the KLIK ePROfile and recommendations are given for actions based on PROM scores. The main aid is the KLIK training, which includes a theoretical and a practical part. In the training, 2 tools-a decision tree and a summary of information about the KLIK ePROfile-are given to the HCPs to assist them in using KLIK.

Discussion: Customization of a PROM portal is needed for each different patient group and clinical setting. Because the KLIK website is flexible, every PROM (with good psychometric qualities and permission of the publisher) can be built in. However, implementing and feeding back PROMs in pediatric health care encompasses different challenges, because different versions of the same PROM are needed for children of different ages, as well as proxy and self-report versions.
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http://dx.doi.org/10.1097/MLR.0000000000001061DOI Listing
May 2019

Impact of using patient-reported outcome measures in routine clinical care of paediatric patients with chronic conditions: a systematic review protocol.

BMJ Open 2019 03 30;9(3):e027354. Epub 2019 Mar 30.

Department of Community Health Sciences, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada.

Introduction: Chronic diseases among children are associated with lower health-related quality of life (HRQOL) and higher utilisation of healthcare services. Integrating Patient-Reported Outcomes Measures (PROMs) in routine clinical care has been shown to reduce utilisation of healthcare services while improving patient outcomes. The objectives of our study are to: (1) identify previously implemented and evaluated PROMs for chronic conditions in paediatric settings; (2) consolidate the evidence to evaluate the impact of using PROMs on HRQOL, healthcare utilisation, patient outcomes (eg, symptoms control) and quality of care among paediatric patients with chronic conditions. The findings from this review will inform the future integration of PROMs in paediatric clinical practice.

Methods And Analysis: We will systematically search the following electronic databases: MEDLINE, EMBASE, CINAHL, PsychINFO and Cochrane library. Reference lists of included studies will also be searched in Web of Science (Thomson Reuters) database to ensure more complete coverage. Two reviewers will independently screen the studies and abstract the data using standardised form. Extracted data will be analysed and synthesised. Finally, a narrative synthesis of summarised data will be presented.

Ethics And Dissemination: Ethical approval is not required, as the proposed systematic review will use data from published research articles. The results of this study will be disseminated through publication in peer-reviewed journals, scientific conferences and meetings, and the lead author's doctoral dissertation.

Prospero Registration Number: CRD42018109035.
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http://dx.doi.org/10.1136/bmjopen-2018-027354DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6475156PMC
March 2019

Professional functioning of young adults with congenital coagulation disorders in the Netherlands.

Haemophilia 2019 May 12;25(3):e138-e145. Epub 2019 Mar 12.

Department of Pediatric-Hematology and Hemophilia Comprehensive Care Treatment Center, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Introduction And Aim: Suboptimal health-related quality of life and lowered employment rates found in a previous study in young adults (YA) with congenital coagulation disorders (CCD) in the Netherlands underline the need for more insight into professional functioning of YA with CCD and into determinants of professional functioning.

Methods: Young adults (18-30 years) with CCD participated in a cross-sectional study. Professional functioning was assessed with the Work Productivity and Activity Impairment questionnaire (WPAI). Potential determinants were assessed with the Course of Life Questionnaire (CoLQ), Pediatric Quality of Life Inventory Young Adult version (PedsQL_YA), Illness Cognition Questionnaire (ICQ) and Haemophilia Activities List (HAL). Logistic regression analyses were performed in the complete sample of YA with CCD, and in YA men with haemophilia separately, to examine determinants of WPAI outcomes.

Results: Ninety-four YA (77 men; mean age 24.1 years, SD 3.5 and 17 women; mean age 24.5 years, SD 3.8) with CCD (74% haemophilia A/B) participated. 74.5% of YA were paid employed for on average 30 hours per week. Of these, more than a quarter reported work impairment. Older age and a non-severe type of haemophilia (in the sample of YA men with haemophilia) were associated with successful (paid) employment. No variables were associated with professional functioning (expressed as Presenteeism and Overall work impairment) in patients with CCD or haemophilia.

Conclusion: Three-quarters of YA with CCD were successful in finding paid employment. Though absenteeism was low, YA with paid employment needs attention as a considerable part experienced work impairment.
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http://dx.doi.org/10.1111/hae.13698DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6850762PMC
May 2019

Health related quality of life of infants and children with avoidant restrictive food intake disorder.

Int J Eat Disord 2019 04 7;52(4):410-418. Epub 2019 Feb 7.

Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Emma Children's Hospital, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands.

Objective: The aim of this study was to compare health related quality of life (HRQOL) in infants and children with avoidant restrictive food intake disorder (ARFID) to healthy and chronically ill controls.

Method: A cross-sectional study was conducted in children who meet ARFID criteria at our tertiary care pediatric feeding clinic (September 2014 to July 2016). Before consultation, parents of patients (n = 100) were asked to complete questionnaires to determine HRQOL: the TNO-AZL Preschool Children Quality of Life (0-5 years), and "Pediatric Quality of Life Inventory" (6-7 and 8-10 years). HRQOL of ARFID patients was compared to both healthy (0-5 years n = 241; 6-7 years n = 61; 8-10 years n = 192) and chronically ill (0-5 years n = 79; 6-7 years n = 11; 8-10 years n = 26) controls.

Results: The prevalence of ARFID was 64%. HRQOL of ARFID patients aged 0-5 years (n = 37) was significantly lower on 6/12 scales (appetite, lungs, stomach, motor functioning, positive mood and liveliness) compared to healthy controls (P < .01), and on 4/12 scales (appetite, stomach, motor functioning, and liveliness) compared to chronically ill controls (P < .01). The ARFID patients scored significantly better on the problem behavior scale compared to healthy and chronically ill controls (P < .01). ARFID patients aged 6-7 (n = 9) had significantly lower scores in 3/6 scales (total score, psychosocial health, and school functioning) (P < .01), and aged 8-10 (n = 2) had a significantly lower school functioning scale (P < .01) compared to healthy controls.

Conclusion: HRQOL of children with ARFID is decreased on multiple scales. The effect on HRQOL should be incorporated in clinical practice, and clinical studies should add HRQOL as an outcome measure.
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http://dx.doi.org/10.1002/eat.23037DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6594067PMC
April 2019

Attitudes of the general population towards preconception expanded carrier screening for autosomal recessive disorders including inborn errors of metabolism.

Mol Genet Metab 2019 01 10;126(1):14-22. Epub 2018 Dec 10.

Amsterdam UMC, University of Amsterdam, Psychosocial Department, Emma Children's Hospital, Meibergdreef 9, Amsterdam, Netherlands. Electronic address:

Background: A substantial number of severely debilitating and often ultimately fatal inborn errors of metabolism (IEMs) still lack an effective disease-modifying treatment. Informing couples before a pregnancy about an increased risk of having a child with an inherited disorder is now feasible by preconception expanded carrier screening (ECS). While knowledge about carrier status enhances reproductive autonomy, it may also result in ethical dilemmas. The purpose of this study was to assess the attitudes of the general Dutch population towards preconception ECS and to investigate which factors influence these attitudes.

Methods: Data collection was carried out in collaboration with a market research agency. In total, 1188 Dutch individuals of reproductive age (18-45 years) were invited by email to complete an online ECS questionnaire in 2016. Prior to the start of the questionnaire, a written explanation of the concepts of autosomal recessive (AR) inheritance, carrier status and ECS was presented.

Results: The questionnaire was completed by 781 individuals (65.7%), of whom 31% indicated they would take an ECS test themselves. In addition, 55% agreed that ECS should be offered to all prospective parents. The most frequently selected argument in favor of ECS (47.2%) was that participants want to spare a child from a life with a severe hereditary disorder. The reason most often mentioned not to participate in ECS (48%) was that participants reported not having a hereditary disorder in the family. The majority preferred receiving individual test results above a couple-based disclosure method in which participants receive the carrier status results only when they are a carrier couple of the same disorder. Participants with religious beliefs were less likely to participate in ECS, whereas participants who were considering a (future) pregnancy were more likely to participate.

Conclusion: Our study demonstrates an overall positive attitude among participants of reproductive age in the general Dutch population towards preconception ECS. A striking misconception is that many of the participants believe that ECS is of interest only for those with a positive family history of one of the hereditary disorders. This finding emphasizes the importance of providing understandable, balanced information and education to the general public regarding the concepts of inheritance when presenting the option of carrier screening. Our results provide valuable insights that can be used in the debate about the responsible implementation of preconception ECS for AR disorders, including IEMs.
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http://dx.doi.org/10.1016/j.ymgme.2018.12.004DOI Listing
January 2019

Health-related quality of life, anxiety, depression and distress of mothers and fathers of children on Home parenteral nutrition.

Clin Nutr 2019 08 2;38(4):1905-1912. Epub 2018 Jul 2.

Department of Pediatric Gastroenterology and Nutrition, Emma Children's Hospital AMC, Amsterdam, The Netherlands. Electronic address:

Background & Aims: Parents of children with intestinal failure, dependent on Home Parenteral Nutrition (HPN), may experience psychosocial problems due to the illness and intensive treatment of their child. Literature concerning psychosocial problems is scarce. Therefore, we aimed to investigate Health-Related Quality of Life (HRQOL), levels of anxiety, depression, distress and everyday problems of these mothers and fathers.

Methods: A multicenter study was conducted among 37 mothers and 25 fathers of 37 children on HPN (response-rate 37/49 = 76%, mean age children = 5.1 years, SD = 4.6). Parents completed three questionnaires to measure different outcomes on the KLIK website (www.hetklikt.nu): the TNO-AZL QOL Questionnaire (TAAQOL) to measure HRQOL, the Hospital Anxiety and Depression Scale (HADS) to measure anxiety and depression, and the Distress Thermometer for Parents (DT-P) to measure distress. Scores were compared to Dutch reference mothers and fathers using Mann-Whitney U-tests.

Results: No differences were found in HRQOL, measured by the TAAQOL, between HPN parents compared to the reference groups, except for the subscale 'depressive emotions' for mothers (p = .01) and 'daily activities' for fathers (p = .04). HPN mothers reported higher levels of depression compared to reference mothers (p = .001). In addition, HPN mothers and fathers reported higher levels of distress than reference mothers (p = .001) and fathers (p = .03). HPN mothers reported significantly more problems in the practical, emotional, cognitive and parenting domains, fathers in the social, emotional and parenting domains.

Conclusions: On HRQOL, anxiety and depression, HPN parents generally did not show much differences compared to reference parents. However, when asked about parental distress and everyday problems, HPN treatment of their child seems highly stressful for some parents and influences daily functioning. Therefore, structural screening for parental psychosocial problems in clinical practice, e.g. using the DT-P, is necessary in order to improve the well-being of both these parents and their children dependent on HPN.
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http://dx.doi.org/10.1016/j.clnu.2018.06.981DOI Listing
August 2019

Psychosocial Functioning in Parents of MPS III Patients.

JIMD Rep 2019 7;44:33-41. Epub 2018 Jul 7.

Psychosocial Department, Emma Children's Hospital, Academic Medical Center, Amsterdam, The Netherlands.

Background: Mucopolysaccharidosis type III (MPS III or Sanfilippo syndrome) is a lysosomal storage disease resulting in progressive neurocognitive decline during childhood and early demise. Its diagnosis may have a great impact on parents, potentially leading to psychosocial problems such as anxiety, depression, parental distress, and posttraumatic stress.

Methods: Twenty-six mothers and 19 fathers of 34 Dutch MPS III patients completed the "Hospital Anxiety and Depression Scale" (HADS), the "Distress Thermometer for Parents" (DT-P), and the "Self-Rating Scale for Posttraumatic Stress Disorders" (SRS-PTSD). Independent-sample T-tests and chi-square tests were used to assess differences between parents of MPS III patients and reference groups regarding anxiety and depression (HADS), distress (DT-P), and posttraumatic stress (SRS-PTSD).

Results: Mothers met the criteria for clinically relevant anxiety (50%) and depression (34.6%) more frequently compared to reference mothers (p = 0.001). Fathers more often met the criteria for clinically relevant depression (36.8%) compared to reference fathers (p = 0.022). Clinically relevant distress was highly prevalent in mothers (84.6%) and fathers (68.4%) of MPS III patients compared to reference parents (p < 0.01). Finally, the prevalence of PTSD was strikingly higher in both mothers (26.9%) and fathers (15%) than reported in the general Dutch population (respectively, p < 0.001 and p < 0.05).

Conclusions: We report a clinically relevant impact of parenting an MPS III patient on psychosocial functioning, which is demonstrated by high levels of anxiety, depression, distress, and a remarkably high prevalence of PTSD. Structural monitoring of the psychosocial functioning of MPS III parents is therefore essential and may be beneficial for the whole family.
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http://dx.doi.org/10.1007/8904_2018_119DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6323010PMC
July 2018

Children on dialysis as well as renal transplanted children report severely impaired health-related quality of life.

Qual Life Res 2018 06 27;27(6):1445-1454. Epub 2018 Jan 27.

Department of Pediatric Nephrology, Emma Children's Hospital, Amsterdam, The Netherlands.

Objectives: To assess health-related quality of life (HRQoL) across three renal replacement therapy modalities (preemptive transplant, non-preemptive transplant, and dialysis) in comparison with the healthy norm and other chronic health conditions, and to explore related patient factors.

Study Design: All prevalent end-stage renal disease (ESRD) patients aged 8-18 years who spent at least 6 months on their current treatment modality in the Netherlands, Belgium, and part of Germany were approached to complete the Pediatric Quality of Life Inventory 4.0 (PedsQL™) questionnaire. We determined the differences between groups on PedsQL™ mean scores, the proportion of children with an impaired HRQoL (≥ 1 SD lower than the healthy norm), the proportion of problems on individual items of the PedsQL™, and the effect of time on current treatment. Linear regression models were used to explore determinants of HRQoL.

Results: 192 out of 278 patients (20% preemptive transplant, 58% non-preemptive transplant, 22% dialysis) filled in the PedsQL™ (response rate 69%). Independent of treatment modality, patients had significantly lower mean scores and consequently higher proportions of impaired HRQoL on almost all domains compared to the healthy norm and other chronic health conditions. Patients with a preemptive transplant only reported higher scores on physical health compared to the other treatment modalities. Having comorbidities was the most important determinant associated with lower HRQoL scores.

Conclusion: Dialysis and renal transplantation both have a severe impact on the HRQoL of children with ESRD. Physicians should be aware of this continuous burden. Furthermore, to develop tailored interventions for children with ESRD, qualitative studies are needed to gain more insight in the determinants of HRQoL in the different treatment modalities.
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http://dx.doi.org/10.1007/s11136-018-1789-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5951873PMC
June 2018

Parental Distress and Quality of Life in Pediatric Inflammatory Bowel Disease: Implications for the Outpatient Clinic.

J Pediatr Gastroenterol Nutr 2018 04;66(4):630-636

Department of Pediatric Gastroenterology.

Objectives: The interrelation between the course of inflammatory bowel disease (IBD) in children and parent's distress, and the subsequent impact this may have on health-related quality of life (HRQOL) of the child is unclear. Therefore, we investigated patient's HRQOL and parental distress and the association between the course of IBD, parental distress, and HRQOL of pediatric patients with IBD.

Methods: Pediatric patients with IBD (8-18 years) and parents were invited. Patients completed the Pediatric Quality of Life Inventory, and parents the Distress Thermometer for Parents, simultaneously. Disease course was expressed as current clinical disease activity or months since last IBD flare. Patient's HRQOL and parental distress were compared to healthy controls.

Results: In total, 87 patients (71% response rate, 59% boys, median age 15.2 years) and parents were included. Patients had an impaired total HRQOL (β = 0.125, P = 0.010), driven by lower physical (0.196, P = 0.001) and school (β = 0.232, P < 0.001) functioning. Parents of children with IBD exhibited comparable levels of distress to parents of healthy children on the total problem and most subdomain problem scores (practical, social, emotional, physical, and cognitive), yet experienced more frequent parenting problems (P = 0.025). More severe disease course (months since last IBD flare) was indirectly associated, through parental distress, with decreased HRQOL of patients.

Conclusions: Worse disease course is directly associated with increased distress of parents and indirectly with lower HRQOL of children and adolescents with IBD. Distress of parents may be considered in management of pediatric IBD to improve HRQOL of children.
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http://dx.doi.org/10.1097/MPG.0000000000001756DOI Listing
April 2018

Parental distress 6 months after a pediatric cancer diagnosis in relation to family psychosocial risk at diagnosis.

Cancer 2018 01 13;124(2):381-390. Epub 2017 Sep 13.

Psychosocial Department, Emma Children's Hospital, Academic Medical Center, Amsterdam, the Netherlands.

Background: This study was aimed at assessing fathers' and mothers' distress 6 months after a pediatric cancer diagnosis and at determining whether this is related to the level of family psychosocial risk 1 month after the diagnosis.

Methods: A sample of 192 families completed the electronic Psychosocial Assessment Tool (ePAT) 1 month after the diagnosis. At 6 months after the diagnosis, 119 mothers and 98 fathers completed the Distress Thermometer for Parents (DT-P; of which n=132 had also completed the ePAT at baseline). The DT-P consists of a thermometer score ranging from 0 to 10 (with a score ≥ 4 indicating clinical distress), problem domains (total, practical, social, emotional, physical, cognitive, and parenting for children < 2 years old and for children ≥ 2 years old), and a desire for a referral. The DT-P scores of mothers and fathers were compared with the scores of a reference group of 671 mothers and 463 fathers with healthy children. Within the pediatric cancer group, the DT-P scores of families with elevated total ePAT-scores were compared with the DT-P scores of parents with universal ePAT scores.

Results: Parents of children with cancer more often reported clinical distress on the DT-P than parents of healthy children (fathers, 59.2% vs 32.3%; P < .001; mothers, 63% vs 42.3%; P < .001) and reported more problems on all DT-P domains (P < .001 to P = .042) except for the parenting domain for children < 2 years old. Furthermore, the ePAT predicted parental distress 6 months after the diagnosis because parents with elevated ePAT scores reported more problems than parents with universal scores on the DT-P thermometer and most of the DT-P domains (P < .001 to P = 1.00).

Conclusions: Initial ePAT risk scores at diagnosis are predictive of future mean levels of parental distress. Cancer 2018;124:381-90. © 2017 American Cancer Society.
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http://dx.doi.org/10.1002/cncr.31023DOI Listing
January 2018

Distress and everyday problems in Dutch mothers and fathers of young adolescents with Down syndrome.

Res Dev Disabil 2017 Aug 12;67:19-27. Epub 2017 Jun 12.

Academic Medical Center, University of Amsterdam, Psychosocial Department, Emma Children's Hospital, Amsterdam Public Health research institute, Post Box 22660, 1100 DD, Amsterdam, The Netherlands. Electronic address:

Background: To provide targeted support to parents of children with DS, knowledge of their distress and everyday problems is crucial. For this purpose, psychosocial screening instruments can be a valuable addition to routine clinical practice.

Aims: To determine differences on a psychosocial screener concerning distress and everyday problems in parents of young adolescents (YAs) with DS versus control parents and in mothers of YAs with DS versus fathers.

Methods And Procedures: We compared outcomes of the Distress Thermometer for Parents in 76 mothers and 44 fathers of 11-13-year-olds with DS versus 64 mothers and 52 fathers of age-matched children without DS (comparing mothers and fathers separately). Additionally, we compared mothers and fathers within 34 parent couples of YAs with DS.

Outcomes And Results: Clinical distress was not more frequent than in control parents. Mothers further did not report more everyday problems and only differed from their controls on one problem domain and some problem items. Fathers, however, reported more problems than their controls across most domains and wished to talk to a professional about their situation more frequently. Outcomes in mothers and fathers within parent couples did not differ significantly.

Conclusions And Implications: This is one of few studies to report on the use of psychosocial screening instruments in parents of children with DS. Our results suggested that attention for fathers of YAs with DS is required. Psychosocial screening instruments that inquire about specific problems and the wish for referral can play an important role in achieving this.
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http://dx.doi.org/10.1016/j.ridd.2017.05.005DOI Listing
August 2017