A web-based psychological support program for caregivers of children with rare chronic diseases: a randomized controlled trial.

BACKGROUND: Approximately 50% of rare diseases have symptom onset during childhood. A high level of nursing care and an often uncertain prognosis put caregivers of the affected children at high risk for psychological distress. At the same time, their caregivers have limited access to appropriate psychological care. The aim of this study was to evaluate a web-based psychological support program for caregivers of children with chronic rare diseases (WEP-CARE). METHODS: German-speaking parents (recruited between May 2016 and March 2018) caring for children aged 0-25 years with a rare disease showing clinically relevant anxiety symptoms, were assigned to either the WEP-CARE (n = 38) or treatment as usual (n = 36) condition within a randomized controlled trial. The primary outcome measure was parental anxiety, assessed with the Generalized Anxiety Disorder Questionnaire (GAD-7). Secondary outcomes were fear of disease progression, depression, coping, quality of life and user satisfaction. The group differences were tested through repeated-measures analyses of variance. The WEP-CARE group was additionally followed up three months after the treatment. RESULTS: A significant time-group interaction was found for anxiety (F (1,35) = 6.13, p = .016), fear of disease progression (F (1,331) = 18.23, p < .001), depression (F (1,74) = 10.79, p = .002) and coping (F (1,233) = 7.02, p = .010), suggesting superiority of the WEP-CARE group. Sustainability of the treatment gains regarding anxiety, fear of disease progression and coping was confirmed at the 6-month follow-up assessment (p < .01). A significant interaction effect could not be found for quality of life (F(1,2) = 0.016; p = .899). Both participating parents and therapists were satisfied with WEP-CARE. CONCLUSIONS: Our results underline the efficacy and feasibility of WEP-CARE for parents of children with various rare diseases.

Prolonged Grief, Posttraumatic Stress, and Depression Among Bereaved Parents: Prevalence and Response to an Intervention Program.

Bereaved parents may experience diverse psychological symptoms. Possible interventions are not yet well established. In this study, the psychological symptoms of 323 bereaved parents (mean age = 39.97, SD = 7.21, 52.0% female), referred to a 4-week family-oriented rehabilitation (FOR) program, were assessed. The baseline assessments indicated that 160 (49.5%) parents showed symptoms of prolonged grief disorder (PGD). Complicated grief was indicated in 272 (84.2%), depression in 191 (59.1%), and posttraumatic stress disorder in 242 (74.9%) parents. Mothers were at higher risk of complicated grief (p ≤ .001), depression (p = .029), and posttraumatic stress disorder (p = .004), compared to fathers. Significant remissions of symptoms between admission and discharge from the program are presented as symptoms of complicated grief, depression, and posttraumatic stress. The effect sizes ranged between d = 0.68 and 1.22. In addition, significantly fewer parents fulfilled PGD criteria on discharge from the FOR program (p ≤ .001). The special FOR program appears promising with regard to improving the bereaved parents' mental health.

Postinfectious Bronchiolitis Obliterans in Children: Diagnostic Workup and Therapeutic Options: A Workshop Report.

Bronchiolitis obliterans (BO) is a rare, chronic form of obstructive lung disease, often initiated with injury of the bronchiolar epithelium followed by an inflammatory response and progressive fibrosis of small airways resulting in nonuniform luminal obliteration or narrowing. The term BO comprises a group of diseases with different underlying etiologies, courses, and characteristics. Among the better recognized inciting stimuli leading to BO are airway pathogens such as adenovirus and mycoplasma, which, in a small percentage of infected children, will result in progressive fixed airflow obstruction, an entity referred to as postinfectious bronchiolitis obliterans (PIBO). The present knowledge on BO in general is reasonably well developed, in part because of the relatively high incidence in patients who have undergone lung transplantation or bone marrow transplant recipients who have had graft-versus-host disease in the posttransplant period. The cellular and molecular pathways involved in PIBO, while assumed to be similar, have not been adequately elucidated. Since 2016, an international consortium of experts with an interest in PIBO assembles on a regular basis in Geisenheim, Germany, to discuss key areas in PIBO which include diagnostic workup, treatment strategies, and research fields.

[Quality criteria of internet-based cognitive-behavioral interventions for children and adolescents and their parents - A systematic review]. / Qualitätskriterien internetbasierter kognitiv-behavioraler Interventionen für Kinder und Jugendliche sowie deren Eltern ­ Ein systematisches Review.

Quality criteria of internet-based cognitive-behavioral interventions for children and adolescents and their parents - A systematic review Abstract. Introduction: This study provides an overview of existing internet-based evidence-based cognitive behavioral interventions (iCBT) for children/adolescents and their parents. The studies were evaluated based on preliminary defined quality criteria regarding their usability in the healthcare system. Methods: The review was performed according to PRISMA guidelines and evaluated the scientific literature concerning to iCBT. We conducted a comprehensive literature search of PubMed, PsychINFO, PsychARTICLES, PsychNDEX and Science Direct using relevant keywords. Studies were rated according to 10 previously predefined quality measures. Results: A total of 4,479 articles were screened, 62 of which were selected for further analysis. Thereof, 24 international iCBT programs with 50 randomized controlled trials (RCTs) were evaluated and met the quality criteria (M = 7.25, SD = 1.07). Further 13 publications showed preliminary efficacy for another 11 international iCBT programs and met the quality criteria (M = 7.45, SD = .82). There was no significant difference between programs evaluated within and without RCTs t(33) = -.56, p = .58. Conclusions: Our results indicate that multiple international iCBT programs exist. Because in Germany the waiting time for psychotherapeutic or psychiatric treatment is still about five months, iCBT programs have the potential to reduce this gap.

Patient education for children with interstitial lung diseases and their caregivers: A pilot study.

OBJECTIVE: Patient education in children with rare chronic diseases like children's interstitial lung disease (chILD) remains a challenge. AIMS: To develop and evaluate a component-based educational program for individual counselling and to improve patients' and caregivers' self-efficacy and treatment satisfaction. Furthermore, to create chILD-specific educational material and assess physicians' satisfaction with the intervention as well as patients' health-related quality of life (HrQoL). METHODS: The study was conducted in two German centers for pediatric pulmonology, as a single-group intervention with pre-post-follow-up design. RESULTS: Participants (N = 107, age: M = 7.67, SD = 5.90) showed significant improvement of self-efficacy (self-report: t = 2.89, p < 0.01; proxy-report: t = 3.03, p < 0.01), and satisfaction (patients: t = 3.56, p = 0.001; parents t = 6.38, p < 0.001) with the medical consultations. There were no pre-post differences in HrQoL. Participants were highly satisfied with the material and the physicians with the program. CONCLUSIONS: The chILD education-program is a promising strategy to improve patients' and their parents' self-efficacy and treatment-satisfaction. Specific effects of the intervention need to be determined in a randomized controlled trial. PRACTICE IMPLICATION: Healthcare providers managing pediatric patients with chILD, may choose to use a patient education-program specifically tailored to the needs of chILD patients and their families, such as the program described here, which is the first of its kind.

Development and validation of a health-related quality of life questionnaire for pediatric patients with interstitial lung disease.

PURPOSE: Children's interstitial lung disease (chILD) is often associated with multiple burdens and chronic impairment of health-related quality of life. Patient reported outcomes describe the patients' perspective on medical conditions and their treatments. We aimed to develop and evaluate the psychometric properties of a chILD-specific PRO (chILD-QoL) as an instrument for monitoring the patients' health status. METHODS: Items were generated through focus groups with parents, patients, and interviews with pediatric pulmonologists. After a pretest of the German pilot version, the questionnaire was refined and translated into four European languages. Psychometric properties of the questionnaire were analyzed within a multi-center collaborative throughout Europe involving 180 parents of children with an interstitial lung disease and 65 pediatric patients. RESULTS: The final instrument is available in different developmentally adapted versions from infancy to adolescence, comprising between 5 and 11 items. The scales showed high internal consistency (Cronbachs'α between 0.85 and 0.94). Convergent validity was indicated by moderate to high correlations (r = 0.43-0.91) with the Pediatric Quality of Life Questionnaire (PedsQL™ 4.0 Core module). Lower scores were significantly associated with dyspnoea (tproxy = 3.18, P = 0.002), tachypnoea (tproxy = 2.95, P = 0.002), and with worse clinical course of lung disease (tself = 3.96, P < 0.05) as reported by the physicians. CONCLUSIONS: The results indicate the reliability and validity of the chILD-QoL for pediatric patients with interstitial lung diseases. It can be used for screening and monitoring subjective health status as perceived by the patients and/or their caregivers, as well as for evaluation of health-related quality of life in clinical trials and intervention research.

Outcomes of an enhancement study with additional psychoeducational sessions for healthy siblings of a child with cancer during inpatient family-oriented rehabilitation.

OBJECTIVE: Chronic illness of a child puts healthy children of the family at risk of distress. Previous studies have demonstrated that healthy children's psychological symptoms can be reduced when the child knows more about the disease. So far, there is limited evidence of the effectiveness of psychoeducational interventions for healthy children. AIMS: To compare the effectiveness of an inpatient family-oriented rehabilitation program with vs without additional psychoeducational sessions for healthy children of families with children with cancer. PATIENTS AND METHODS: We performed a controlled study in 4 German family-oriented rehabilitation clinics. The outcomes of n = 73 healthy children (mean age: M = 9.55; SD = 3.14; range: 4-18), who participated in 5 additional psychoeducational sessions, were compared with the outcomes of n = 111 healthy children (mean age: M = 8.85; SD = 3.28; range: 4-17), who underwent the usual inpatient rehabilitation program. Primary outcomes were the healthy children's cancer-specific knowledge and their emotional symptoms. Secondary outcomes were family satisfaction and quality of life. RESULTS: Intention-to-treat analyses showed that both groups improved significantly from preintervention to postintervention. Improvements comprised knowledge about cancer (F(1,174) = 11.03, p < 0.001), self-reported emotional symptoms (F(1,135) = 31.68, p < 0.001), and parent-proxy-reported emotional symptoms (F(1,179) = 37.07, p < 0.001). The additional psycho-educational program did not significantly enhance the outcomes. The same pattern of significant improvement in both conditions emerged for all secondary outcomes. The immediate effects of the intervention persisted until 2 months after discharge from the rehabilitation program. CONCLUSIONS: Inpatient family-oriented rehabilitation is effective in improving multiple psychosocial outcomes of healthy children in families which have a child with cancer. Additional psycho-educational sessions did not show any substantial additional improvement.

[Psychometric Properties of the German Version of the Sibling Perception Questionnaire (SPQ)]. / Psychometrische Eigenschaften der deutschen Version des Sibling Perception Questionnaire (SPQ).

The Sibling Perception Questionnaire (SPQ) measures the impact a cancer disease of a child has on a sibling's interpersonal relations in the family, communication, intrapersonal perceptions and fear of the disease. Aim of this study was the psychometric evaluation of the German version of the SPQ. For this purpose, data of 216 healthy siblings including 164 self-reports and 212 parent reports were gathered. For further analyses the 4 subscales "interpersonal difficulties", "intrapersonal difficulties", "open communication" and "fear of the disease" with overall 23 items were evaluated. Sufficient internal consistencies in the parent report (Cronbach's α between 0.73 and 0.87) and in the self-report version (α between 0.59 and 0.80) were found for all subscales except the subscale fear of the disease (α=0.35 and 0.16). Regarding external validity, significant correlations emerged between scales of the SPQ and scales of the Strengths and Difficulties Questionnaire (SDQ) and the Quality of Life Inventory for Children (LQ-KID) (r=- 0.37; r=0.63; p≤0.001). The German version of the SPQ can be regarded as a reliable and valid instrument for the assessment of emotional responses of healthy children on their sibling's cancer disease. Further investigations on different samples are recommended.

Predictors of health-related quality of life in children with chronic heart disease.

OBJECTIVE: Chronic paediatric heart disease is often associated with residual symptoms, persisting functional restrictions, and late sequelae for psychosocial development. It is, therefore, increasingly important to evaluate the health-related quality of life of children and adolescents with chronic heart disease. The aim of this study was to determine medical and socio-demographic variables affecting health-related quality of life in school-aged children and adolescents with chronic heart disease. Patients and methods The Pediatric Cardiac Quality of Life Inventory was administered to 375 children and adolescents and 386 parental caregivers. Medical information was obtained from the charts. The socio-demographic information was provided by the patients and caregivers. RESULTS: Greater disease severity, low school attendance, current cardiac medication, current parental employment, uncertain or limited prognosis, history of connection to a heart-lung machine, number of nights spent in a hospital, and need for treatment in a paediatric aftercare clinic independently contributed to lower health-related quality of life (self-report: R2=0.41; proxy-report: R2=0.46). High correlations between self-reports and parent-proxy reports indicated concordance regarding the evaluation of a child's health-related quality of life. CONCLUSIONS: Beyond medical treatment, integration into school is important to increase health-related quality of life in children and adolescents surviving with chronic heart disease. Regular screening of health-related quality of life is recommended to identify patients with special needs.