Assessing the reliability and validity of a health-related quality of life instrument, CPADULT, in a Dutch sample of adults with severe disabilities who are non-ambulatory.

BACKGROUND: A measure to provide insight regarding health-related quality of life of adults with severe motor and intellectual disabilities was lacking. For this reason, the CPADULT was developed. This measure includes domains relating to an individual's physical, mental, and social functioning. The purpose of this study was to assess the psychometric characteristics of the CPADULT. METHOD: Caregivers (n = 47; 77% female, 23% male) of individuals with severe disabilities who are non-ambulatory completed the questionnaire. Internal consistency, test-retest reliability and construct validity were analysed. RESULTS: Internal consistency was adequate with Cronbach's alpha values from 0.75 to 0.95. Test-retest reliability was good, as intraclass correlation coefficient of the total score was 0.84 (domains: 0.61-0.89). Construct validity was confirmed with significant differences between subgroups of motor or intellectual abilities. CONCLUSION: The CPADULT has sufficient reliability and validity as a proxy measure of health-related quality of life for adults with severe disabilities who are non-ambulatory.

Development and sensibility assessment of a health-related quality of life instrument for adults with severe disabilities who are non-ambulatory.

BACKGROUND: Insight in health-related quality of life (HRQoL) of adults with severe disabilities who are non-ambulatory is important, but a measure is lacking. The aim was to develop a HRQoL measure for this group. METHOD: The developmental process consisted of the adaptation process of a proxy HRQoL measure for children with severe disabilities who are non-ambulatory and the assessment of the sensibility of the developed instrument. A three-step process was used: focus groups, e-survey and interviews. RESULTS: In total, 72% of the items remained unchanged. Three new items and one element to an existing item were added. In ten items, the formulation of the items was adapted to the target group. Concerning the sensibility, respondents suggested minor changes to the instruction and the output scales. CONCLUSIONS: This study has yielded a proxy HRQoL measure for adults with severe disabilities who are non-ambulatory, the CPADULT, with good sensibility.

Longitudinal validation of the Caregiver Priorities and Child Health Index of Life with Disabilities in a Dutch sample of nonambulatory children with severe disabilities.

BACKGROUND: The purpose of this study was to evaluate the longitudinal validity of the Dutch version of the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD), a health-related quality of life instrument for nonambulatory children with severe motor disabilities and accompanying disorders. METHODS: The effect of two interventions, Botox injections in the hip region and percutaneous endoscopic gastrostomy, was followed over time. Caregivers (n = 38) of nonambulatory children (26 boys, 12 girls; mean age: 9 years, 5 months [4 years, 9 months]) with severe disabilities completed the questionnaire prior to the intervention, at 3 months and 6 months follow up. Seven a priori hypotheses were formulated. Longitudinal validity was analysed by a paired t test of the pre-post scores and correlation analysis between the change-scores and two external criteria: a caregivers' perceived change in health-related quality of life of the child questionnaire and a general health-related quality of life instrument. RESULTS: The results reported here follow completely the pattern we hypothesized for four analyses and partially in the remaining three. In the Botox group, the mean change-score at 3 months was 6.9 points (p < 0.05), which exceeds the minimal clinically important difference of 5.8 points. At 6 months, the effect was diminished to 4.5 points, in line with the temporary effect of Botox. There were moderate positive correlations between the change-scores and an external criterion (Spearman's rho: 0.46-0.58). CONCLUSIONS: This study indicates that the CPCHILD Dutch version has sufficient longitudinal validity statistically and clinically in nonambulatory children with severe disabilities.

Motor and non-motor determinants of health-related quality of life in young dystonia patients.

OBJECTIVES: To systematically investigate the relationship between motor and non-motor symptoms, and health-related quality of life (HR-QoL) in children and young adults with dystonia. METHODS: In this prospective observational cross-sectional study, 60 patients (6-25 years) with childhood-onset dystonia underwent a multidisciplinary assessment of dystonia severity (Burke-Fahn-Marsden Dystonia Rating Scale, Global Clinical Impression), motor function (Gross Motor Function Measure, Melbourne Assessment of Unilateral Upper Limb Function), pain (visual analogue scale), intelligence (Wechsler Intelligence Scale), executive functioning (Behavior Rating Inventory of Executive Function) and anxiety/depression (Child/Adult Behavior Checklist). Measures were analyzed using a principal component analysis and subsequent multiple regression to evaluate which components were associated with HR-QoL (Pediatric Quality of life Inventory) for total group, and non-lesional (primary) and lesional (secondary) subgroups. RESULTS: Patients (29 non-lesional, 31 lesional dystonia) had a mean age of 13.6 ±â€¯5.9 years. The principal component analysis revealed three components: 1) motor symptoms; 2) psychiatric and behavioral symptoms; and 3) pain. HR-QoL was associated with motor symptoms and psychiatric and behavioral symptoms (R2 = 0.66) for the total sample and lesional dystonia, but in the non-lesional dystonia subgroup only with psychiatric and behavioral symptoms (R2 = 0.51). CONCLUSIONS: Non-motor symptoms are important for HR-QoL in childhood-onset dystonia. We suggest a multidisciplinary assessment of motor and non-motor symptoms to optimize individual patient management.

Pain and hospital admissions are important factors associated with quality of life in nonambulatory children.

AIM: This was the first study to investigate the factors associated with health-related quality of life (HRQoL) in nonambulatory children with cerebral palsy (CP), based on a HRQoL measure specifically developed for this population. METHODS: The Dutch version of the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD-DV) was used to measure HRQoL. It was completed by 66 parents of 47 boys and 19 girls with nonambulatory CP aged between five and 18 years with gross motor function classification system (GMFCS) levels of IV and V. Factors measured were the child's motor and cognitive impairments, comorbidities, pain, parents' education and occupations and family structure. Multiple linear regression analyses were used to determine the significant factors and the relative contribution of these factors to the CPCHILD-DV scores. RESULTS: The most important factors associated with poorer HRQoL scores were pain and hospital admissions in the previous six months. Other factors were as follows: increased GMFCS level, feeding by gastrostomy tube, inability to communicate verbally, cognitive impairment, poor seizure control and higher parents' educational qualifications. CONCLUSION: Pain and hospital admissions were the most important factors that were negatively associated with HRQoL in nonambulatory children with CP between five to 18 years.

Validation of the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) in a sample of Dutch non-ambulatory children with cerebral palsy.

PURPOSE: To assess the reliability and validity of the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD)-Dutch Version, a proxy measure of health status and well-being of non-ambulatory children with cerebral palsy (CP). METHODS: Parents (n = 66) of 47 boys/19 girls between 5 and 18 years with CP (GMFCS IV-V) participated. To assess the reliability each domain and the total measure was tested for internal consistency, test-retest and inter-rater reliability. Known-groups validity of the CPCHILD-DV was assessed by comparing mean scores of clinically distinct subgroups and convergent validity by correlating the CPCHILD-DV with the TNO-AZL Preschool Children Quality of Life (TAPQOL). RESULTS: The mean CPCHILD-DV total score was 52.0 (SD11.5). Test-retest reliability of the total score as assessed by intraclass correlations (ICC) was 0.73 (domains: 0.55-0.80). For the inter-rater reliability the ICC was 0.64 (domains: 0.58-0.90); the Cronbach's alpha's ranged from 0.60 to 0.95. The CPCHILD score could differentiate between GMFCS levels and between subgroups of cognitive level in the domain "communication and social interaction". There were moderate significant correlations (range r(s): 0.31-0.50) between sections of CPCHILD-DV and TAPQOL. CONCLUSIONS: The CPCHILD-DV has sufficient reliability and validity as a proxy measure of health status and well-being of non-ambulatory children with CP.