Health-related quality of life in adolescents with cerebral palsy; a cross-sectional and longitudinal population-based study.

AIMS: The aims of this population-based cross-sectional and longitudinal study were to investigate different aspects of health-related quality of life (HRQoL) in adolescents with cerebral palsy (CP), to define possible changes in HRQoL from childhood to adolescence and to identify factors associated with low HRQoL in adolescence. METHODS: Proxy-reports of 64 adolescents, aged 12-17 years, with bilateral CP in GMFCS levels III-V participating in a surveillance programme, included five of the six domains from the HRQoL instrument Caregiver Priorities & Child Health Index of Life with Disabilities (CPCHILD): (1) Activities of Daily Living and Personal Care, (2) Positioning, Transfer and Mobility, (3) Comfort and Emotions, (5) General Health and (6) Overall Quality of Life, and the two questions on pain from the Child Health Questionnaire (CHQ). Fifty-eight participants comprised the longitudinal sample. RESULTS: From childhood to adolescence, the mean CPCHILD domain scores decreased slightly in General Health and remained unchanged in the other four domains. In the domain General Health, the number of medications increased, which was the reason for the score decrease. Pain severity increased significantly. Severe motor impairment was associated with low scores in domains 1, 2, 3 and 5, and more severe pain with low scores in domains 2, 3, 5 and 6. A low domain score in childhood was associated with a low score in each corresponding domain in adolescence. INTERPRETATION: An assessment of HRQoL should be included in CP surveillance programmes because this could identify needs for interventions in individuals with severe CP. This study indicates the importance of improved pain management in both children and adolescents with severe CP.

Pain burden in children with cerebral palsy (CPPain) survey: Study protocol.

Pain is a significant health concern for children living with cerebral palsy (CP). There are no population-level or large-scale multi-national datasets using common measures characterizing pain experience and interference (ie, pain burden) and management practices for children with CP. The aim of the CPPain survey is to generate a comprehensive understanding of pain burden and current management of pain to change clinical practice in CP. The CPPain survey is a comprehensive cross-sectional study. Researchers plan to recruit approximately 1400 children with CP (primary participants) across several countries over 6-12 months using multimodal recruitment strategies. Data will be collected from parents or guardians of children with CP (0-17 years) and from children with CP (8-17 years) who are able to self-report. Siblings (12-17 years) will be invited to participate as controls. The CPPain survey consists of previously validated and study-specific questionnaires addressing demographic and diagnostic information, pain experience, pain management, pain interference, pain coping, activity and participation in everyday life, nutritional status, mental health, health-related quality of life, and the effect of the COVID-19 pandemic on pain and access to pain care. The survey will be distributed primarily online. Data will be analyzed using appropriate statistical methods for comparing groups. Stratification will be used to investigate subgroups, and analyses will be adjusted for appropriate sociodemographic variables. The Norwegian Regional Committee for Medical and Health Research Ethics and the Research Ethics Board at the University of Minnesota in USA have approved the study. Ethics approval in Canada, Sweden, and Finland is pending. In addition to dissemination through peer-reviewed journals and conferences, findings will be communicated through the CPPain Web site (www.sthf.no/cppain), Web sites directed toward users or clinicians, social media, special interest groups, stakeholder engagement activities, articles in user organization journals, and presentations in public media.

Recurrent pain in adolescents with cerebral palsy: a longitudinal population-based study.

AIM: To investigate the pain characteristics, pain interference with activities of daily living, and use of analgesics in adolescents with cerebral palsy (CP) and compare the results with previous findings. METHOD: Sixty-seven adolescents (median age 14y 4mo, range 12y 2mo-17y, 28 females, 39 males) classified in Gross Motor Function Classification System (GMFCS) levels III to V, who participated in a CP surveillance programme, were assessed on pain measures twice, 5 years apart. Primary caregivers marked recurrent pain sites and graded pain interference with activities of daily living and sleep. Information on pain severity was obtained through two questions from the Child Health Questionnaire (CHQ) and were transformed into a pain score scaled from 0 to 100, where 100 represented no pain. The use of short-acting analgesics was recorded. RESULTS: Over 5 years, the prevalence of recurrent pain, number of pain sites, pain intensity, and pain frequency all increased significantly. The most frequent pain sites were the hip/thigh in GMFCS level V and knee in GMFCS level III. The median CHQ pain score decreased from 60 to 40 (p<0.001). Pain interference with activities of daily living increased (p=0.011) but not for sleep. Twenty-eight of 54 participants with moderate or severe pain (CHQ pain score ≤60) received no short-acting analgesics. INTERPRETATION: In adolescents with CP, pain increased over 5 years despite follow-up in a surveillance programme. For enhanced management of pain, we propose that an algorithm on pain should be included in surveillance programmes.

Flaunting our assets. Making the most of the Nordic registry goldmine: Cerebral palsy as an example.

Aims:To describe the early experiences of a Nordic multidisciplinary cerebral palsy (CP) registry research program combining data from national medical quality registries, follow-up programs and cohort data, in addition to data from other national registries; to explore the scientific and practical uses of such research, and provide recommendations for facilitating similar work in the future. Methods: The work was divided into three themes: medical outcomes, social and public health outcomes, and health economics; and three cross-cutting teams: a reference team, a challenge team, and a communication and dissemination team. Initially each country will perform domestic research, and in the second stage data will be merged across all Nordic countries. Data from national registries with vital statistics, education and work, social benefits, and healthcare will be used. Comparisons will be matched for both the individuals with CP and their parents. Results: Initial work has been done on agreeing which variables to request from the respective agencies and planning the correct procedures and steps required to acquire the data. As of 2018, Sweden, Norway, and Finland have received approved ethics board applications. Iceland and Denmark are waiting for their approvals. A webpage and a platform for internal communication have been created. Conclusions: Nordic register research has great potential. Linking national CP quality registries and follow-up programs with other large national registries holds particular promise because problems identified through research can be applied at a population level. It is imperative that ethical clearance and data delivery processes are streamlined and transparent, and that data variables are measured the same way in the different countries.

Is more frequent physical therapy associated with increased gross motor improvement in children with cerebral palsy? A national prospective cohort study.

Purpose: To investigate the association between physical therapy frequency and gross motor improvement in children with cerebral palsy (CP).Materials and methods: This is a prospective cohort study of 442 children aged 2-12 years, Gross Motor Function Classification System levels I-V, from the Cerebral Palsy Follow-up Program and the Cerebral Palsy Register of Norway. Outcome was change in reference percentiles for the Gross Motor Function Measure (GMFM-66) between two subsequent assessments (N = 1056) analyzed in a linear mixed model.Results: It was a dose response association between physical therapy frequency and gross motor improvement. Mean change was 4.2 (95% CI: 1.4-7.1) percentiles larger for physical therapy 1-2 times per week and 7.1 (95% CI: 2.6-11.6) percentiles larger for physical therapy >2 times per week, compared to less frequent physical therapy when analyzed in a multivariable model including multiple child and intervention factors. The only statistically significant confounder was number of contractures which was negatively associated with gross motor improvement.Conclusions: When gross motor improvement is a goal for children with CP, more frequent physical therapy should be considered.Implications for rehabilitationIn general, the gross motor development of Norwegian children with cerebral palsy was as expected according to the reference percentiles for the GMFM-66.When gross motor improvement is a goal for children with cerebral palsy, high-frequency physical therapy should be considered.Contractures should be addressed in order to optimize gross motor improvement for children with cerebral palsy.

Factors Associated with Enhanced Gross Motor Progress in Children with Cerebral Palsy: A Register-Based Study.

AIM: To examine associations between interventions and child characteristics; and enhanced gross motor progress in children with cerebral palsy (CP). METHODS: Prospective cohort study based on 2048 assessments of 442 children (256 boys, 186 girls) aged 2-12 years registered in the Cerebral Palsy Follow-up Program and the Cerebral Palsy Register of Norway. Gross motor progress estimates were based on repeated measures of reference percentiles for the Gross Motor Function Measure (GMFM-66) in a linear mixed model. Mean follow-up time: 2.9 years. RESULTS: Intensive training was the only intervention factor associated with enhanced gross motor progress (mean 3.3 percentiles, 95% CI: 1.0, 5.5 per period of ≥3 sessions per week and/or participation in an intensive program). Gross motor function was on average 24.2 percentiles (95% CI: 15.2, 33.2) lower in children with intellectual disability compared with others. Except for eating problems (-10.5 percentiles 95% CI: -18.5, -2.4) and ankle contractures by age (-1.9 percentiles 95% CI: -3.6, -0.2) no other factors examined were associated with long-term gross motor progress. CONCLUSIONS: Intensive training was associated with enhanced gross motor progress over an average of 2.9 years in children with CP. Intellectual disability was a strong negative prognostic factor. Preventing ankle contractures appears important for gross motor progress.

Hand Function in a Population-Based Sample of Young Children with Unilateral or Bilateral Cerebral Palsy.

AIM: To describe aspects of hand function in a population-based sample of young children with clinical signs of unilateral or bilateral cerebral palsy (CP). METHOD: A cross-sectional study with data from national CP registers in Norway. Manual ability was classified with the Manual Ability Classification System (MACS) or Mini-MACS. Hand use in bimanual activities was measured with the Assisting Hand Assessment (AHA) for unilateral CP or the newly developed Both Hands Assessment (BoHA) for bilateral CP. RESULTS: From 202 children, 128 (57 females) were included (Mini-MACS/MACS levels I-V, mean age 30.4 months; SD = 12.1). Manual abilities were distributed across levels I-III in unilateral CP and levels I-V in bilateral CP. Variations in AHA and BoHA units were large. One-way ANOVA revealed associations between higher AHA or BoHA units and Mini-MACS/MACS levels of higher ability (p < 0.01) and higher age (p < 0.04). CONCLUSIONS: Compared with young children with unilateral CP, children with bilateral CP showed greater variation in Mini-MACS/MACS levels, and both sub-groups showed large variations in AHA or BoHA units. The classifications and assessments used in this study are useful to differentiate young children's ability levels. Such information is important to tailor upper limb interventions to the specific needs of children with CP.

Severe hip displacement reduces health-related quality of life in children with cerebral palsy.

Background and purpose - Hip displacement is common in children with severe cerebral palsy (CP) and can cause problems such as pain, contractures, and nursing difficulties. Caregiver priorities and child health index of life with disabilities (CPCHILD) is a recently developed measure of health-related quality of life (HRQL) in children with severe CP. The associations between CPCHILD scores and hip displacement have not been investigated. We explored the effect of hip displacement on HRQL. Patients and methods - 67 children were recruited from the population-based Norwegian CP register. Mean age was 9 (7-12) years. There were 40 boys. Gross motor function classification system (GMFCS) distribution was 12 level III, 17 level IV, and 38 level V. Hip displacement was assessed by radiographic migration percentage (MP). The criterion for hip displacement was MP of the worst hip of ≥40%. Primary caregivers responded to 5 of the 6 domains of the CPCHILD questionnaire. Results - Hip displacement was found in 18 children and it was significantly associated with lower scores on the CPCHILD domains 3 (Comfort and Emotions) and 5 (Health), but not with domains 1 (Activities of Daily Living/Personal Care), 2 (Positioning, Transfer, and Mobility), and 6 (Overall Quality of Life). GMFCS level V was a significant predictor of low scores in all the domains. Interpretation - For the assessment of HRQL in children with severe CP and hip problems, we propose a modified and simplified version of the CPCHILD consisting of 14 of 37 questions. This would reduce the responders' burden and probably increase the response rate in clinical studies without losing important information.

Growth during infancy and early childhood in children with cerebral palsy: a population-based study.

AIM: To describe growth in infancy and early childhood in children with cerebral palsy (CP). METHOD: One hundred and four children with CP born at minimum 36 weeks' gestation in 2002 to 2010 were included. Prospectively collected growth data were requested from public health clinics. We calculated standard deviation (SD) scores (z-scores) for weight and height for 12 set age points for each child from birth to 5 years, and for head circumference from birth to 12 months. RESULTS: Children with CP had normal growth in weight and height if they were born non-small for gestational age (non-SGA) or had mild motor impairments (i.e. Gross Motor Function Classification System [GMFCS] I-II), whereas children born SGA or with severe motor impairments (GMFCS III-V) had reduced growth (p<0.001). Children with feeding difficulties in infancy had reduced growth in weight and height throughout early childhood, while children without feeding difficulties had normal growth. Head circumference growth decreased most severely among children born SGA, who had mean z-scores of -3.0 (95% confidence interval [CI] -3.7 to -2.2) at 1 year. INTERPRETATION: Children with mild CP had normal growth in weight and height until 5 years, and in head circumference during infancy. Feeding difficulties in infancy and being born SGA were strongly associated with reduced growth.

Physical activity and motor function in children and adolescents with neuromuscular disorders.

PURPOSE: To determine physical function and activity level in children and adolescents with neuromuscular disorders. METHODS: Seventeen children and adolescents aged 10 to 18 years with neuromuscular disorders who were ambulatory participated. Physical function was measured using the Hammersmith Motor Ability Scale and the Six-Minute Walk Test. Physical activity (PA) level was measured using the SenseWear Armband activity monitor. Activities were recorded using a questionnaire. Results were compared with previous data published for peers who are healthy and public recommendations. RESULTS: Hammersmith Motor Ability Scale scores were lower than normal values. Results of the Six-Minute Walk Test were considerably lower than results for peers who are healthy. Time in PA and the number of steps were considerably lower on holidays and slightly lower on weekdays compared with the values for peers who are healthy. No vigorous activity was registered. CONCLUSIONS: The participants had lower physical function and PA levels than peers who are healthy. The small, heterogeneous sample makes generalizations challenging and multicenter studies are recommended.