Rare Diseases
Conditions
Keywords
rare diseases, pediatric surgery, children and adolescent, quality of life, psychological distress
Brief summary
Families of children with rare diseases (i.e., not more than 5 out of 10.000 people are affected) are often highly burdened with fears, insecurities and concerns regarding the affected child and his/her siblings. The aim of the present research project is to examine the psychosocial burden of the children with rare (congenital) pediatric surgical diseases and their family in order to draw attention to a possible psychosocial care gap in this population.
Detailed description
The central objective of the cross-sectional study is to show the psychosocial supply gap for families with children and adolescents affected by rare diseases in the field of pediatric surgery. Among the rare diseases that are included are diaphragmatic hernia, anorectal malformations, esophageal atresia, Hirschsprung's disease and biliary atresia. In order to have a comparative sample, additional data of a matched control group are collected. Central standardized psychosocial outcomes will be assessed from the perspectives of the parents, the affected child and the siblings.
Interventions
None listed
Sponsors
Universitätsklinikum Hamburg-Eppendorf
Study design
Observational model
COHORT
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
1 Days to 21 Years
Healthy volunteers
Yes
Inclusion criteria
(families of rare disease): * Family with at least one child between 0 and 21 years with a rare pediatric surgical disease. * Consent to participate in the study. * Sufficient knowledge of the German language of parents and children.
Exclusion criteria
(families of rare disease): * Severe acute physical, mental and/or cognitive impairment of the child, so that the questionnaire survey does not appear possible and/or unreasonable at this stage. Inclusion Criteria (control group): * Family with at least one child between 0 and 21 years who have undergone a surgical procedure in the first 3 years of life that does not cause chronic complaints * Consent to participate in the study. * Sufficient knowledge of the German language of parents and children.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Quality of life of the parents (ULQIE) | 4 minutes | Quality of life (QoL) of the parents, assessed from the perspective of the parents by the Ulmer Lebensqualitätsinventar für Eltern chronisch kranker Kinder (ULQIE; Goldbeck & Storck, 2002). The instrument consists of 29 items, which are answered on a five-point Likert scale (0 to 4). Higher scores indicate greater QoL. |
| Mental health of the parents (BSI) | 5 minutes | Mental health of the parents, assessed from the perspective of the parents by the Brief Symptom Inventory (BSI; Franke, 2000). The instrument consists of 53 items, which are answered on a five-point Likert scale (0 to 4). Higher BSI scores indicate greater psychological distress. |
| Health-related quality of life of the chronically-ill children/adolescents (Peds-QL 4.0) | 4 minutes | Health-related quality of life of the chronically-ill children/adolescents, assessed from the perspective of the child/adolescent (from 10 years of age) and from the perspective of the parents by the Pediatric Quality of Life Inventory 4.0 (Peds-QL 4.0; (Varni, Seid, & Kurtin, 2001). Items will be linearly transformed to a scale of 0 to 100, with higher scores indicating better HRQoL. |
| Psychiatric disorders of the chronically-ill children/adolescents and the siblings (SDQ) | 4 minutes | Psychiatric disorders of the chronically-ill children/adolescents and the siblings assessed perspective of the child/adolescent (from 10 years of age) and from the perspective of the parents by the Strengths and Difficulties Questionnaire (SDQ; Klasen, Woerner, Rothenberger, & Goodman, 2003). Items are rated on a three-point Likert scale (0 to 2). Higher scores represent greater psychopathology or greater prosocial behavior. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Relationships between siblings (SRQ) | 3 minutes | Sibling relationship, assessed from the perspective of the siblings (from 10 years of age) by the Sibling Relationship Questionnaire (Bojanowski, Riestock, Nisslein, Weschenfelder-Stachwitz, & Lehmkuhl, 2015). Each item is scored on a 5-point Likert scale (1 to 5). |
| Family relationships, family dynamics and functionality (FB-A) | 3 minutes | Family relationships, family dynamics and functionality, assessed from the perspective of the parents, of the chronically-ill children/adolescents and from the sibling, respectively by Familienbögen (FB-A; Cierpka & Frevert, 1994). Items are rated on a four-point Likert scale (0 to 3) |
| Coping of the parents (CHIP-D) | 3 minutes | Coping of the parents, assessed from the perspective of the parents by the German version of the Coping Health Inventory for Parents (CHIP-D; McCubbin, McCubbin, Cauble & Goldbeck, 2001). Items are rated on a four-point Likert scale (0 to 3). Higher scores represent greater use of the respective coping pattern. |
| Familial predispositions (FaBel) | 3 minutes | familial predispositions assessed from the perspective of the parents by the Familien-Belastungs-Fragebogen (FaBel; Ravens-Sieberer, 2001). Items are rated on a four-point Likert scale ranging from 1 (is not right at all) to 4 (is completely right). |
| Interpersonal problems (IIP-32) | 3 minutes | Interpersonal problems assessed from the perspective of the parents by the Inventory of Interpersonal Problems-32 (IIP-32; Thomas, Brähler, & Strauß, 2011). Items are rated on a four-point Likert scale from 0 (not at all) to 4 (extremely). It provides an overall score and eight subscale scores. |
| Coping of the chronically-ill children/adolescents and the siblings (Kidcope) | 3 minutes | Coping of the chronically-ill children/adolescents and the siblings, assessed from the perspective of the children/adolescents (from 10 years of age) by the Kidcope Checklist (Kidcope; Spirito, Stark & Williams, 1988). Items related to frequency of the coping strategies are rated on a 4 point scale (0 = Not at all to 3 = Almost all the time), and items related to efficacy are rated on a 5 point scale (0 = Not at all to 4 = Very much). |
| Social support of the parents, of the chronically-ill children/adolescents and of the siblings (OSSS) | 3 minutes | Social support of the parents, of the chronically-ill children/adolescents and of the siblings, assessed from the perspective of the parents, of the chronically-ill children/adolescents and from the sibling, respectively, by the Oslo Social Support Scale (OSSS; Dalgard, 2006). The sum score ranges from 3 to 14. The higher the sum score, the stronger the social support. |
| Sociodemographic information of the parents | 2 minutes | Sociodemographic information of the parents, assessed from the perspective of the parents by ad-hoc items. |
Countries
Germany
Outcome results
None listed