Meaningful outcomes for children and their caregivers attending a paediatric brain centre.

AIM: To identify meaningful outcomes of children and their caregivers attending a paediatric brain centre. METHOD: We compiled a long list of outcomes of health and functioning of children with brain-related disorders such as cerebral palsy, spina bifida, (genetic) neurodevelopmental disorders, and acquired brain injury. We incorporated three perspectives: patients, health care professionals, and published outcome sets. An aggregated list was categorized using the International Classification of Functioning, Disability, and Health: Children and Youth version in a patient validation survey for children and parent-caregivers to prioritize outcomes. Outcomes were considered meaningful when ranked 'very important' by 70% or more of the participants. RESULTS: We identified 104 outcomes from the three perspectives. After categorizing, 59 outcomes were included in the survey. Thirty-three surveys were completed by children (n = 4), caregivers (n = 24), and parent-caregivers together with their child (n = 5). Respondents prioritized 27 meaningful outcomes covering various aspects of health and functioning: emotional well-being, quality of life, mental and sensory functions, pain, physical health, and activities (communication, mobility, self-care, interpersonal relationships). Parent-caregiver concerns and environmental factors were newly identified outcomes. INTERPRETATION: Children and parent-caregivers identified meaningful outcomes covering various aspects of health and functioning, including caregiver concerns and environmental factors. We propose including those in future outcome sets for children with neurodisability. WHAT THIS PAPER ADDS: Outcomes that children with brain-related disorders and their parent-caregivers consider to be the most meaningful cover a wide range of aspects of functioning. Involving these children and their parent-caregivers resulted in the identification of important outcomes that were not covered by professionals and the literature. Parent-caregiver-related factors (coping, burden of care) and environmental factors (support, attitudes, and [health care] services) were identified as meaningful.

ICF Core Sets for the assessment of functioning of adults with cerebral palsy.

AIM: To report on the results of the online international consensus process to develop the comprehensive and brief International Classification of Functioning, Disability and Health (ICF) Core Sets for adults with cerebral palsy (CP). METHOD: An online iterative decision-making and consensus process involved 25 experts, including clinicians and researchers working with adults with CP, an adult with CP, and the parents of adults with CP from all six regions of the World Health Organization. The most relevant categories were selected from a list of 154 unique second-level candidate categories to develop the ICF Core Sets for adults with CP. This list resulted from evidence gathered during four preparatory studies, that is, a systematic literature review, a qualitative study, an expert survey, and an empirical study. RESULTS: The consensus process resulted in the comprehensive ICF Core Set containing 120 second-level ICF categories: 33 body functions; eight body structures; 50 activities and participation; and 29 environmental factors, from which the most essential categories, 33 in total, were selected for the brief ICF Core Set. For body functions, most of the categories were mental functions and neuromusculoskeletal and movement-related functions. Body structures were mostly related to movement. All the chapters of the activities and participation component were represented, with mobility and self-care as the most frequently covered chapters. For environmental factors, most of the categories addressed products and technology and services, systems, and policies. INTERPRETATION: The comprehensive and brief ICF Core Sets for adults with CP were created using a new online version of an established ICF Core Set consensus process. These Core Sets complement the age-specific ICF Core Sets for children and young people with CP and will promote standardized data collection worldwide.

A core outcome set for multimorbidity risk in individuals with cerebral palsy.

AIM: To: (1) investigate the importance of outcome measurement instruments (OMIs) within a core outcome set (COS) for multimorbidity (at least two chronic health conditions) risk in individuals with cerebral palsy (CP); (2) investigate the feasibility of OMIs within the COS in international clinical research settings in adolescents and adults with CP; and (3) describe the associations between the COS data and Gross Motor Function Classification System (GMFCS) levels. METHOD: Eighty-three individuals with CP completed a survey on health outcomes: physical behaviour, nutrition, sleep, endurance, body composition, blood pressure, blood lipids, and glucose. A cross-sectional study assessed the feasibility of the COS in 67 adolescents and adults with CP (mean age 30y, SD 15y 1mo, min-max: 14-68y, 52.2% male) at four centres. Prevalence of multimorbidity risk and associations with GMFCS levels are described. RESULTS: Most participants rated physical behaviour, nutrition, sleep, and endurance as very important. Body composition, blood pressure, nutrition, and sleep were highly feasible since data were collected in 88% or more participants who consented to having the assessments. Physical behaviour, cardiorespiratory endurance, and blood draws were collected in less than 60% of participants. Total time sedentary (ρ=0.53, p<0.01) and endurance (ρ=-0.46, p<0.01) were significantly associated with GMFCS level. INTERPRETATION: The COS identified that most participants had poor sleep quality and endurance, did not have healthy diets, and showed increased sedentary behaviour. Individuals with CP valued these outcomes as most important, suggesting a need to assess these modifiable behaviours in this population. Objective measures of physical behaviour and cardiorespiratory endurance in the COS required additional personnel, time, and participant burden. We recommend that healthcare providers should perform a simpler first screen using questionnaire-based assessments and then focus the use of the remainder of the COS if required for the patient.

UM DESFECHO CENTRAL DEFINIDO PARA RISCO DE MULTIMORBIDADE EM INDIVÍDUOS COM PARALISIA CEREBRAL: OBJETIVO: (1) Investigar a importância dos instrumentos de medição de desfechos (IMD) dentro de um conjunto de desfechos principais (CDS) para risco de multimorbidade (pelo menos duas condições crônicas de saúde) em indivíduos com paralisia cerebral (PC); (2) Investigar a viabilidade de IMD dentro do CDS em cenários internacionais de pesquisa clínica em adolescentes e adultos com PC; e (3) Descrever as associações entre os dados do CDS e os níveis do Sistema de Classificação da Função Motora Grossa (GMFCS). MÉTODO: Oitenta e três indivíduos com PC completaram uma pesquisa sobre desfechos em saúde: comportamento físico, nutrição, sono, resistência, composição corporal, pressão arterial, lipídios no sangue e glicose. Um estudo transversal avaliou a viabilidade do COS em 67 adolescentes e adultos com PC (idade média de 30 anos, desvio padrão de 15 anos e 1 mês, min-max: 14-68 anos, 52,2% do sexo masculino) em quatro centros. São descritas a prevalência do risco de multimorbidade e as associações com os níveis de GMFCS. RESULTADOS: A maioria dos participantes classificou o comportamento físico, nutrição, sono e resistência como muito importantes. Composição corporal, pressão arterial, nutrição e sono foram altamente viáveis, uma vez que os dados foram coletados em 88% ou mais dos participantes que consentiram em realizar as avaliações. Comportamento físico, resistência cardiorrespiratória e coleta de sangue foram coletados em menos de 60% dos participantes. O tempo total de sedentarismo (ρ = 0,53, p < 0,01) e resistência (ρ = −0,46, p < 0,01) foram significativamente associados ao nível de GMFCS. INTERPRETAÇÃO: O CDS identificou que a maioria dos participantes tinha má qualidade e resistência do sono, não tinha dietas saudáveis e apresentava um comportamento sedentário aumentado. Indivíduos com PC valorizaram esses desfechos como mais importantes, sugerindo a necessidade de avaliar esses comportamentos modificáveis nessa população. Medidas objetivas de comportamento físico e resistência cardiorrespiratória no CDS exigiram pessoal adicional, tempo e sobrecarga do participante. Recomendamos que os profissionais de saúde realizem uma primeira triagem mais simples usando avaliações baseadas em questionários e, em seguida, concentrem o uso do restante do CDS, se necessário para o paciente.

Development of an ICF Core Set for adults with cerebral palsy: capturing their perspective on functioning.

AIM: To examine the most relevant aspects of functioning of adults with cerebral palsy (CP) from their perspective, in order to develop an International Classification of Functioning, Disability and Health (ICF) Core Set for adults with CP. METHOD: We conducted six focus group discussions with adults with CP without intellectual disability and seven interviews with adults with CP with intellectual disability and caregivers, addressing all ICF components. Meaningful concepts were identified from verbatim transcripts and linked to ICF categories by two independent researchers. RESULTS: In total, 31 adults with CP without intellectual disability (mean [SD] age 46y 1mo [14y 1mo]; 20 females, 11 males; Gross Motor Function Classification System [GMFCS] levels I-IV) and seven adults with CP and intellectual disability (mean [SD] age 25y 8mo [6y 8mo]; four females, three males; GMFCS levels III-V) participated. We identified 132 unique second-level categories: 47 body functions, seven body structures, 43 activities and participation, and 35 environmental factors. The most frequently mentioned categories were emotional function, pain, muscle tone function, support of family, products and technology, and health services. INTERPRETATION: Adults with CP experienced problems in a broad range of body functions and activities and indicated the importance of environmental factors for functioning. The identified categories will be added to the list of candidate items to reach consensus on an ICF Core Set for adults with CP. What this paper adds Including the lived experience is crucial for fully understanding functioning of adults with cerebral palsy (CP). Adults with CP perceive environmental factors as essential elements for everyday functioning. Adults with intellectual disability should be considered as a group with specific problems.

Autonomy in participation in cerebral palsy from childhood to adulthood.

AIM: To determine the long-term development of autonomy in participation of individuals with cerebral palsy (CP) without intellectual disability. METHOD: Individuals with CP (n=189, 117 males, 72 females; mean age [SD] 21y 11mo [4y 11mo], range 12-34y); were assessed cross-sectionally (46%) or up to four times (54%), between the ages of 12 and 34 years. Autonomy in participation was classified using phase 3 of the Rotterdam Transition Profile. A logistic generalized estimating equation regression model was used to analyse autonomy in six domains (independent variables: age, Gross Motor Function Classification System [GMFCS] level, and interaction between age and GMFCS level). Proportions of autonomy were compared to references using binomial tests (p<0.05). RESULTS: In most domains, over 90% of participants (n=189, 400 observations, 80% in GMFCS levels I and II) reached autonomy in participation in their late twenties, except for intimate/sexual relationships. Those in GMFCS levels III to V compared to those in GMFCS levels I and II had less favourable development of autonomy in the transportation, intimate relationships, employment, and housing domains, and more favourable development in the finances domain. Compared to references, fewer individuals with CP were autonomous in participation. INTERPRETATION: This knowledge of autonomy may guide the expectations of young people with CP and their caregivers. Furthermore, rehabilitation professionals should address autonomy development in intimate relationships, employment, and housing, especially in individuals with lower gross motor function. WHAT THIS PAPER ADDS: Individuals with cerebral palsy without intellectual disability achieved autonomy in most participation domains. Regarding intimate relationships, they continued to have less experience compared to age-matched references. Development of autonomy was less favourable for individuals in Gross Motor Function Classification System levels III to V.

AUTONOMÍA EN LA PARTICIPACIÓN EN LA PARÁLISIS CEREBRAL DESDE LA INFANCIA A LA EDAD ADULTA: OBJETIVO: Determinar el desarrollo a largo plazo de la autonomía en la participación de individuos con parálisis cerebral (PC) sin discapacidad intelectual. MÉTODO: Individuos con PC (n=189, 117 varones, 72 mujeres; edad media [DE] 21 años y 11 meses [4 años y 11meses], rango 12-34 años); fueron evaluados de forma transversal (46%) o hasta cuatro veces (54%), entre las edades comprendidas entre los 12 y los 34 años. La autonomía en la participación fue clasificada usando la fase 3 del perfil de transición de Rotterdam (Rotterdam Transition Profile). Se utilizó un modelo de regresión logístico generalizado estimando la ecuación para analizar la autonomía en 6 dominios (variables independientes: edad, nivel del Sistema de Clasificación de la Función Motora Gruesa, [GMFCS], y la interacción entre la edad y el nivel GMFCS). Las proporciones de la autonomía se compararon con las referencias mediante pruebas binomiales (p<0,05). RESULTADOS: En la mayoría de los dominios, más del 90% de los participantes (n=189.400 observaciones, 80% en los niveles I y II de la GMFCS) alcanzaron la autonomía en la participación bien entrados los 20 años, excepto para las relaciones íntimas/sexuales. Aquellos en los niveles III y V de la GMFCS en comparación con los niveles I y II de la GMFCS tuvieron un desarrollo de la autonomía menos favorable en el transporte, relaciones íntimas, empleo y en el dominio de la vivienda, y un desarrollo más favorable en el dominio de las finanzas. En comparación con las referencias, menos individuos con PC fueron autónomos en la participación. INTERPRETACIÓN: Este conocimiento de la autonomía puede guiar las expectativas de los jóvenes con PC y de sus cuidadores. Además, los profesionales de la rehabilitación deberían abordar el desarrollo de la autonomía en las relaciones íntimas, el empleo y la vivienda, especialmente en aquellos individuos con una función motora gruesa más baja.

AUTONOMIA NA PARTICIPAÇÃO EM PARALISIA CEREBRAL DA INFÂNCIA À VIDA ADULTA: OBJETIVO: Determinar o desenvolvimento em longo prazo da autonomia na participação de indivíduos com paralisia cerebral (PC) sem deficiência intelectual. MÉTODO: Indivíduos com PC (n=189, 117 do sexo masculino, 72 do sexo feminino; média de idade [DP] 21a 11m [4a 11m], variação 12-34a); foram avaliados transversalmente (46%) ou até quatro vezes (54%), entre as idades de 12 e 34 anos. A autonomia na participação foi classificada usando a fase 3 do Perfil de Transição de Rotterdam. Um modelo de regressão logística com generalizada com equações estimadas foi usado para analisar a autonomia em seis domínios (variáveis independentes: idade, Nível segundo o Sistema de Classificação da Função Motora Grossa [GMFCS], e interação entre idade e nível GMFCS). As proporções de autonomia foram comparadas com as referências usando testes binomiais (p<0,05). RESULTADOS: Na maior parte dos domínios, cerca de 90% dos participantes (n=189.400 observações, 80% nos níveis GMFCS I e II) atingiram autonomia na participação no final da terceira década de vida, exceto para relações íntimas/sexuais. Aqueles nos níveis GMFCS III a V comparados com os níveis I e II tiveram desenvolvimento menos favorável nos domínios da autonomia no transporte, relações íntimas, emprego, e domicílio, e mais desenvolvimento mais favorável no domínio das finanças. Em comparação com as referências, menos indivíduos com PC foram autônomos na participação. INTERPRETAÇÃO: Este conhecimento sobre a autonomia pode guiar as expectativas de jovens com PC e seus cuidadores. Além disso, profissionais da reabilitação devem abordar o desenvolvimento da autonomia nas relações íntimas, emprego e domicílio, especialmente nos indivíduos com menor função motora grossa.

Development curves of communication and social interaction in individuals with cerebral palsy.

AIM: To determine development curves of communication and social interaction from childhood into adulthood for individuals with cerebral palsy (CP). METHOD: This Pediatric Rehabilitation Research in the Netherlands (PERRIN)-DECADE study longitudinally assessed 421 individuals with CP, aged from 1 to 20 years at baseline, after 13 years (n=121 at follow-up). Communication and social interactions were assessed using the Vineland Adaptive Behavior Scales. We estimated the average maximum performance limit (level) and age at which 90% of the limit was reached (age90 ) using nonlinear mixed-effects modeling. RESULTS: One-hundred individuals without intellectual disability were aged 21 to 34 years at follow-up (39 females, 61 males) (mean age [SD] 28y 5mo [3y 11mo]). Limits of individuals without intellectual disability, regardless of Gross Motor Function Classification System (GMFCS) level, approached the maximum score and were significantly higher than those of individuals with intellectual disability. Ages90 ranged between 3 and 4 years for receptive communication, 6 and 7 years for expressive communication and interrelationships, 12 and 16 years for written communication, 13 and 16 years for play and leisure, and 14 and 16 years for coping. Twenty-one individuals with intellectual disability were between 21 and 27 years at follow-up (8 females, 13 males) (mean age [SD] 24y 7mo [1y 8mo]). Individuals with intellectual disability in GMFCS level V showed the least favourable development, but variation between individuals with intellectual disability was large. INTERPRETATION: Individuals with CP and without intellectual disability show developmental curves of communication and social interactions similar to typically developing individuals, regardless of their level of motor function. Those with intellectual disability reach lower performance levels and vary largely in individual development. WHAT THIS PAPER ADDS: Communication and social interactions in individuals with cerebral palsy without intellectual disability develop similarly to typically developing individuals. Communication and social interactions of individuals with intellectual disability develop less favourably and show large variation.

CURVAS DE DESARROLLO DE COMUNICACIÓN E INTERACCIÓN SOCIAL EN NIÑOS CON PARÁLISIS CEREBRAL: OBJETIVO: Determinar las curvas de desarrollo de la comunicación y la interacción social desde la infancia hasta la edad adulta para las personas con parálisis cerebral (PC). MÉTODO: Esta Investigación de Rehabilitación Pediátrica en el Estudio de los Países Bajos (PERRIN)-DECADE evaluó longitudinalmente 421 individuos con PC, de 1 a 20 años en el inicio, después de 13 años (n=121 en el seguimiento). La comunicación y las interacciones sociales se evaluaron utilizando la Escala de comportamiento adaptativo de Vineland. Estimamos el límite promedio de rendimiento máximo (nivel) y la edad a la que se alcanzó el 90% del límite (edad90 ) utilizando un modelo no lineal de efectos mixtos. RESULTADOS: Cien individuos sin discapacidad intelectual tenían entre 21 y 34 años en el seguimiento (39 mujeres, 61 varones; edad media [DS] 28 y 5 meses [3 años y 11meses]). Los límites de las personas sin discapacidad intelectual, independientemente del nivel del Sistema de Clasificación de la Función Motora Gruesa (GMFCS), se acercaron a la puntuación máxima y fueron significativamente superiores a los de personas con discapacidad intelectual. Edad 90s entre 3 y 4 años para la comunicación receptiva, 6 y 7 años para la comunicación expresiva y las interrelaciones, 12 y 16 años para la comunicación escrita, 13 y 16 años por juego y ocio, y 14 y 16 años por sobrellevarlo. Veintiún individuos con discapacidad intelectual tenían entre 21 y 27 años en seguimiento (8 mujeres, 13 hombres; edad media [DS] 24 años y 7 meses [1 año y 8 meses]). Las personas con discapacidad intelectual en el nivel V de GMFCS mostraron el desarrollo menos favorable, pero la variación entre las personas con discapacidad intelectual fue grande. INTERPRETACIÓN: Las personas con PC sin discapacidad intelectual muestran curvas de desarrollo de comunicación e interacciones sociales similares a las personas con desarrollo típico, no considerando su nivel de función motora. Las personas con PC y discapacidad intelectual alcanzan niveles de rendimiento más bajos y varían en gran medida en el desarrollo individual.

CURVAS DE DESENVOLVIMENTO DA COMUNICAÇÃO E INTERAÇÃO SOCIAL EM CRIANÇAS COM PARALISIA CEREBRAL: OBJETIVO: Determinar as curvas de desenvolvimento e interação social da infância para a adolescência para indivíduos com paralisia cerebral (PC). MÉTODO: Este estudo Europeu de Reabilitação Pediátrica na Holanda (PERRIN)-DECADE avaliou longitudinalmente 421 indivíduos com PC, com idades de 1 a 20 anos na linha de base, após 13 anos, (n=121 no acompanhamento). A comunicação e interação social foram avaliadas usando as Escalas Vineland de Compartamento Adaptativo. Estimamos o limite máximo de desempenho médio (nível) e idade em que 90% do limite foi atingido (idade90 ) usando modelos não-lineares de efeitos mistos. RESULTADOS: Cem indivíduos sem deficiência intelectual com idades entre 21 e 34 anos no acompanhamento (39 do sexo feminino, 61 do sexo masculino; média de idade [DP] 28a 5m [3a 11m]). Os limites de indivíuduos sem deficiência intelectual, independente do nível do Sistema de Classificação da Função Motora Grossa (GMFCS), se aproximou da pontuação maxima e foram significativamente maiores do que os valores de indivíduos com deficiência intelectual. A idade 90s variou entre 3 e 4 anos para comunicação receptiva, 6 e 7 anos para comunicação expressiva e intercomunicações, 12 e 16 anos para comunicação escrita, 12 e 16 years para brincadeiras e lazer, e 14 e 16 anos para adaptabilidade. Vinte e um indivíduos com deficiência intelectual estavam entre 21 e 27 anos no acompanhamento (8 do sexo feminino, 13 do sexo masculino; média de idade [DP] 24a 7m [1a 8m]). Indivíduos com deficiência intelectual no nível GMFCS V mostraram o desenvolvimento menos favorável, mas a variação entre indivíduos com deficiência intelectual foi grande. INTERPRETAÇÃO: Indivíduos com PC com e sem deficiência intelectual mostram curvas desenvolvimentais de comunicação e interação social similares a indivíduos com desempenho típico, independente do nível de função motora. Aqueles com deficiência intelectual tiveram menores níveis de performance e variaram amplamente no desenvolvimento individual.

Epidemiology of Cerebral Palsy in Adulthood: A Systematic Review and Meta-analysis of the Most Frequently Studied Outcomes.

OBJECTIVE: To describe the epidemiology of health status, impairments, activities and participation in adults with cerebral palsy (CP). DATA SOURCES: Embase, MEDLINE, Web of Science, PsycINFO, Cumulative Index to Nursing and Allied Health, Cochrane, and Google Scholar were searched for 3 themes ("cerebral palsy," "adult," and "outcome assessment") in literature published between January 2000 and December 2018. STUDY SELECTION: Full-article peer-reviewed English journal articles on descriptive, observational, or experimental studies describing the most studied outcomes in adults with CP (n≥25, age≥18y) were included. Studies were included in the analyses if frequently studied outcomes were described in at least 3 studies using similar methods of assessment. DATA EXTRACTION: Data were extracted independently by 2 authors from 65 articles (total N=28,429) using a standardized score sheet. DATA SYNTHESIS: Meta-analyses revealed that overall, on average 65.1% (95% confidence interval [CI], 55.1-74.5) of adults with CP experienced pain, 57.9% (95% CI, 51.1-64.6) were ambulant, 65.5% (95% CI, 61.2-69.7) had little or no limitation in manual ability, 18.2% (95% CI, 10.6-27.2) had tertiary education, 39.2% (95% CI, 31.5;47.1) were employed, and 29.3% (95% CI, 9.0-55.3) lived independently. In adults without intellectual disability, proportions of individuals who were ambulant (72.6% [95% CI, 58.8-84.5]) and lived independently (90.0% [95% CI, 83.8-94.9]) were higher (P=.014 and P<.01, respectively). The Fatigue Severity Scale score was 4.1 (95% CI, 3.8-4.4). Epilepsy (28.8% [95% CI, 20.1-38.4]) and asthma (28.3% [95% CI, 18.7-38.9]) were especially prevalent comorbidities. CONCLUSIONS: The present systematic review and meta-analysis on the epidemiology of adults with CP provided state-of-the-art knowledge on the most frequently studied outcomes. On average, adults with CP are fatigued, and a majority experience pain, are ambulant, and have little or no difficulty with manual ability. On average, 40% are employed and 30% live independently. More uniformity in assessment and reports is advised to improve knowledge on epidemiology and gain insight in more outcomes.

Long-term course of difficulty in participation of individuals with cerebral palsy aged 16 to 34 years: a prospective cohort study.

AIM: To determine the long-term course of difficulty in participation of individuals with cerebral palsy (CP) without intellectual disability between 16 years and 34 years of age. METHOD: One hundred and fifty-one individuals with CP aged 16 to 20 years were included (63% male, 37% female; Gross Motor Function Classification System [GMFCS] levels I-IV; without intellectual disability). The Assessment of Life Habits questionnaire 3.0 general short form was used up to three times biennially and at 13-year follow-up (13-year follow-up: n=98). Scores (range 0-10) reflect difficulty and assistance in participation in housing, education and employment, interpersonal relationships, recreation, community life, and responsibilities. Multilevel models were used to determine the course of difficulty in participation by GMFCS level. RESULTS: Despite high average participation levels, 41% to 95% of adolescents and young adults with CP experienced difficulty. Difficulty in participation in housing and interpersonal relationships increased from age 16 years onwards and in most other life areas in the mid- and late 20s. In adolescents in GMFCS levels III and IV, participation in recreation and community life improved up to age 23 years. INTERPRETATION: Individuals with CP experience increasing difficulties in participation in their mid- and late 20s. Clinicians should systematically check for participation difficulties in young adults with CP and offer timely personalized treatment. WHAT THIS PAPER ADDS: Many individuals with cerebral palsy (CP) aged 16 to 34 years experience difficulty in participation. Difficulty in participation increases in the mid- and late 20s for individuals with CP. Participation in recreation/community life improves before age 23 years for those in Gross Motor Function Classification System levels III and IV.

Outcomes in adults with cerebral palsy: systematic review using the International Classification of Functioning, Disability and Health.

AIM: In the context of the development of an International Classification of Functioning, Disability and Health (ICF) Core Set for adults with cerebral palsy (CP), this systematic review sought to identify the outcome measures used in studies on adults with CP, to examine their content using the ICF as a reference, and to demonstrate the most studied areas in this population. METHOD: Embase, MEDLINE, Web of Science, PsycINFO, CINAHL, Cochrane, and Google Scholar were searched for studies on adults with CP published between 2000 and 2017. Meaningful concepts of commonly used outcome measures were linked to the ICF, and frequencies of resultant ICF categories were explored. RESULTS: In 274 included articles, 332 outcome measures were identified of which 155 were commonly used. In total, 4409 meaningful concepts were linked to the ICF. The component 'Activities and participation' included the most frequent categories, followed by 'Body functions'. The most frequent categories were b280 'Sensation of pain' (37.6%), d450 'Walking' (33.3%), and d850 'Remunerative employment' (27.5%). INTERPRETATION: The broad range of ICF categories identified in this systematic review emphasizes the heterogeneity of functioning and disability in adults with CP. The current results specifically reflect the researchers' perspective and will serve as candidate categories to consider in the development of an ICF Core Set for adults with CP. WHAT THIS PAPER ADDS: Outcomes studied in adults with cerebral palsy are captured in varying International Classification of Functioning, Disability and Health (ICF) categories. Activities and participation were studied more frequently than body functions and structures. Mobility, employment, and self-care were most frequent within ICF's 'Activities and participation' component. Pain and neuromusculoskeletal and movement-related functions were most frequent within 'Body functions'.

Focus on Risk Factors for Cardiometabolic Disease in Cerebral Palsy: Toward a Core Set of Outcome Measurement Instruments.

OBJECTIVE: To identify existing outcome measurement instruments (OMIs) assessing risk factors for cardiometabolic disease in adolescents and adults with cerebral palsy (CP) reported on in the literature or used in the field. DATA SOURCES: The COnsensus-based Standards for the selection of health Measurement Instruments database of systematic reviews and 4 electronic databases (Embase, MEDLINE/Ovid, MEDLINE/Pubmed, PsychINFO) were searched up to June 19, 2017, that yielded 2594 articles. Experts in the field were consulted to identify any additional OMIs. STUDY SELECTION: Two reviewers independently applied inclusion criteria to select eligible studies using or evaluating measurement properties of OMIs assessing 1 of 8 outcomes: cardiorespiratory endurance, body size, body composition, physical behavior, sleep, nutrition, blood pressure, and blood lipids and glucose. Studies with an experimental or observational design including ≥10 adolescents or adults with CP were included. DATA EXTRACTION: One reviewer extracted data that were summarized for study and sample characteristics, outcomes, OMIs used, and if applicable data on measurement properties. Two reviewers rated the methodological quality and the quality of the OMIs. Feasibility for clinical practice and research was rated by experts in the field. DATA SYNTHESIS: Ninety OMIs were identified from 56 included articles and by the experts. Seventy OMIs pertained to cardiorespiratory endurance, body size, body composition, and physical behavior, whereas only 5 were identified for sleep and nutrition. Overall synthesis revealed that there is moderate to poor evidence for good quality of OMIs in this population. Based on feasibility for clinical practice, experts agreed on a single OMI per outcome (and 2 for cardiorespiratory endurance) to be included in a core set. CONCLUSION: Despite the range of available OMIs to assess risk factors for cardiometabolic disease in adolescents and adults with CP, evidence of good quality is often lacking. Nonetheless, a preliminary core set of 9 OMIs was systematically developed.

Course of employment in adults with cerebral palsy over a 14-year period.

AIM: To explore the course of employment in adults with cerebral palsy (CP) over 14 years, and to identify subgroups at risk for unemployment. METHOD: Sixty-five adults with CP (33 males, 32 females; baseline age 25y 8mo, standard deviation [SD] 3y 2mo; intellectual impairment 25%; bilateral CP 65%) participated in a prospective cohort study. Self-reports of employment and work hours per week in 1996, 2000, and 2010 were documented. The course of employment (including sheltered work) and work hours per week were analysed, using generalized estimating equations (GEE). RESULTS: Overall, employment rate was stable over time (38-45%, p=0.413), but lower than in the general population (75-86%, p<0.001). Employment rates were specifically low in adults with intellectual impairment, bilateral CP, and in adults with Gross Motor Function Classification System (GMFCS) levels IV and V. Work hours per week declined (35.0 [SD 7.9] to 31.2 [SD 10.3], p=0.033), especially among females (32.3 [SD 6.4] to 23.4 [SD 7.4], p<0.001). Similar to the general population, females often worked part-time. INTERPRETATION: Employment was low compared with the general population, but remained stable in the long term; however, work hours per week decreased. Adults with intellectual impairment, bilateral CP, and GMFCS levels IV and V are subgroups at risk for unemployment.

Long-Term Deterioration of Perceived Health and Functioning in Adults With Cerebral Palsy.

OBJECTIVE: To describe longitudinal change in perceived health, presence of health issues, and functional level in adults with cerebral palsy (CP). DESIGN: Prospective cohort study. SETTING: Participants' daily environment. PARTICIPANTS: Adults (N=49) with CP (age range, 35-45y; 27 [55%] men; 36 [75%] spastic) formerly known in pediatric rehabilitation care participated. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Postal questionnaires were completed by the adults or their proxies (n=9). Health outcomes included perceived health (adapted from the 36-Item Short Form Health Survey) and presence of health issues such as pain, severe fatigue (dichotomized), and functional level (Barthel Index; walking performance). RESULTS: Over a 10-year period, the percentage of adults with CP worrying about their health increased (29%-54%; P=.008) and those indicating that health problems limit their activities increased (19%-45%; P=.002). In the same period, most adults continued to report good general health (93%-86%; P=.148). Presence of some health issues increased over time, such as pain; severe fatigue was a common health issue at follow-up (32%). Over a 14-year period, mobility and self-care deteriorated (Barthel Index, 17.1±4.8 to 16.3±5.6; P=.007). Walking performance, specifically indoors, declined (83%-71%; P=.010). CONCLUSIONS: Adults with CP experienced deterioration in health outcomes in the long term. Most notably, perceived health and functional level decreased. Pain and severe fatigue were the most common health issues in adult CP. More research is required to explore the mechanisms at work in the process of aging in persons with CP. Systematic follow-up of adults with CP appears necessary to timely detect and intervene in health problems and functional decline.

Prevalence of fatigue, pain, and affective disorders in adults with duchenne muscular dystrophy and their associations with quality of life.

OBJECTIVES: To assess the prevalence of fatigue, pain, anxiety, and depression in adults with Duchenne muscular dystrophy (DMD), and to analyze their relationship with health-related quality of life. DESIGN: Cross-sectional study. SETTING: Home of participants. PARTICIPANTS: Adults (N=80) with DMD. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Fatigue was assessed with the Fatigue Severity Scale; pain with 1 item of the Medical Outcomes Study 36-Item Short-Form Health Survey and by interview; and anxiety and depression by using the Hospital Anxiety and Depression Scale. Health-related quality of life was assessed using the World Health Organization Quality of Life Scale-Brief Version. Associations between these conditions and quality of life were assessed by means of univariate and multivariate logistic regression analyses. RESULTS: Symptoms of fatigue (40.5%), pain (73.4%), anxiety (24%), and depression (19%) were frequently found. Individuals often had multiple conditions. Fatigue was related to overall quality of life and to the quality-of-life domains of physical health and environment; anxiety was related to the psychological domain. CONCLUSIONS: Fatigue, pain, anxiety, and depression, potentially treatable symptoms, occur frequently in adults with DMD and significantly influence health-related quality of life.

Developmental trajectories of social participation in individuals with cerebral palsy: a multicentre longitudinal study.

AIM: This study aimed to determine the developmental trajectories of social participation, by level of gross motor function and intellectual disability, in a Dutch population of individuals with cerebral palsy (CP) aged 1 to 24 years. METHOD: As part of the Pediatric Rehabilitation Research in the Netherlands (PERRIN+), 424 individuals with CP (261 males, 163 females; mean age [SD] 9y 6mo [6y 2mo]; Gross Motor Function Classification [GMFCS] levels I-V [50% level I]; 87% with spastic CP; 26% with intellectual disability) were longitudinally followed for up to 4 years between 2002 and 2007. Social participation was assessed with the Vineland Adaptive Behavior Scales survey. Effects of age, GMFCS level and intellectual disability were analysed using multilevel modelling. RESULTS: The developmental trajectories for individuals in GMFCS levels I to IV did not significantly differ from each other. For individuals without intellectual disability, the degree of social participation increased with age and stabilized at about 18 years. These individuals reached social participation levels similar to typically developing individuals. The trajectories were significantly less favourable for individuals in GMFCS level V and individuals with intellectual disability. INTERPRETATION: Intellectual disability is more distinctive for the development of social participation than GMFCS level. The developmental trajectories will support individuals with CP and their families in setting realistic goals and professionals in optimizing the choice of interventions at an early age.

Developmental trajectories of receptive and expressive communication in children and young adults with cerebral palsy.

AIM: The aim of this study was to determine the developmental trajectories of expressive (speech) and receptive (spoken and written language) communication by type of motor disorder and intellectual disability in individuals with cerebral palsy (CP). METHOD: The development of 418 participants (261 males, 157 females; mean age 9y 6mo [SD 6y 2mo], range 1-24y; Gross Motor Function Classification System (GMFCS) level I [n=206], II [n=57], III [n=59], IV [n=54], V [n=42]) was followed for 2 to 4 years in a longitudinal study. Communication performance was measured using the Vineland Adaptive Behavior Scales. The type of motor disorder was differentiated by type of CP as unilateral spastic (USCP, n=161), bilateral spastic (BSCP, n=202), and non-spastic (NSCP, n=55), while intellectual disability was determined by IQ or school type (regular or special). A multilevel analysis was then used to model the developmental trajectories. RESULTS: The most favourable development of expressive communication was seen in USCP (vs BSCP ß [SE]-2.74 [1.06], NSCP ß [SE]-2.67 [1.44]). The difference between the development trajectory levels of children with and without intellectual disability was smaller for children with USCP than for those with BSCP and NSCP. For receptive communication, the most favourable development was found for all children with USCP and for BSCP or NSCP without intellectual disability (vs intellectual disability ß [SE]-4.00 [1.16]). Development of written language was most favourable for children without intellectual disability (vs intellectual disability ß [SE]-23.11 [2.85]). INTERPRETATION: The development of expressive communication was found to be most closely related to type of motor disorder, whereas the development of receptive communication was found to be most closely related to intellectual disability.

Long-term trajectories of health-related quality of life in individuals with cerebral palsy: a multicenter longitudinal study.

OBJECTIVES: To (1) determine the long-term trajectory of health-related quality of life (HRQOL) for the dimensions of physical complaints and motor, psychological, and social functioning for groups of individuals with cerebral palsy (CP) aged 1 to 24 years; (2) assess the variability in HRQOL within individuals with CP over time; (3) assess the variability in HRQOL between individuals with CP; and (4) compare the HRQOL in individuals with CP to reference data of typically developing individuals. DESIGN: Multicenter prospective longitudinal study. SETTING: Rehabilitation departments of 3 university medical centers and various rehabilitation centers in The Netherlands. PARTICIPANTS: Dutch individuals with CP (N=424; age, 1-24y). INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: The HRQOL dimensions of physical complaints and motor, psychological, and social functioning. Each individual visited the rehabilitation department for 3 or 4 measurements. The time between measurements was 1 or 2 years. RESULTS: Individuals with CP experience an HRQOL that, on average, remains fairly stable over time. Variability in HRQOL within individuals with CP was similar to that within typically developing individuals. Variability between individuals with CP could be explained by type of CP (motor functioning), Gross Motor Function Classification System level (physical complaints and motor and social functioning), and intellectual disability (physical complaints and social functioning). Finally, individuals with CP experienced a lower HRQOL than did typically developing individuals, especially for the dimensions of motor and social functioning. CONCLUSIONS: Many changes take place in the psychosocial development of the individual with CP, which accordingly change their expectations and those of their caregivers, peers, and professionals. As a result, perceived physical complaints and motor, psychological, and social functioning remain fairly stable over many years.

Reducing bottlenecks: professionals' and adolescents' experiences with transitional care delivery.

BACKGROUND: The purpose of this study was to describe the interventions implemented in a quality improvement programme to improve transitional care and evaluate its effectiveness in reducing bottlenecks as perceived by professionals and improving chronically ill adolescents' experiences with care delivery. METHODS: This longitudinal study was undertaken with adolescents and professionals who participated in the Dutch 'On Your Own Feet Ahead!' quality improvement programme. This programme followed the Breakthrough Series improvement and implementation strategy.A total of 102/128 (79.7%) professionals from 21 hospital teams filled out a questionnaire at the start of the programme (T0), and 79/123 (64.2%; five respondents had changed jobs) professionals completed the same questionnaire 1 year later (T1). Seventy-two (58.5%) professionals from 21 teams returned questionnaires at both time points. Of 389 and 430 participating adolescents, 36% and 41% returned questionnaires at T0 and T1, respectively. We used descriptive statistics and two-tailed, paired t-tests to investigate improvements in bottlenecks in transitional care (perceived by professionals) and care delivery (perceived by adolescents). RESULTS: Professionals observed improvement in all bottlenecks at T1 (vs. T0; p < 0.05), especially in the organisation of care, such as the presence of a joint mission between paediatric and adult care, coordination of care, and availability of more resources for joint care services. Within a 1-year period, the transition programme improved some aspects of patients' experiences with care delivery, such as the provision of opportunities for adolescents to visit the clinic alone (p < 0.001) and to decide who should be present during consultations (p < 0.05). CONCLUSIONS: This study demonstrated that transitional care interventions may improve the organisation and coordination of transitional care and better prepare adolescents for the transition to adult care within a 1-year period. By setting specific goals based on experiences with bottlenecks, the breakthrough approach helped to improve transitional care delivery for adolescents with chronic conditions.

Inactive and sedentary lifestyles amongst ambulatory adolescents and young adults with cerebral palsy.

BACKGROUND: To assess physical behaviour, including physical activity and sedentary behaviour, of ambulatory adolescents and young adults with cerebral palsy (CP). We compared participant physical behaviour to that of able-bodied persons and assessed differences related to Gross Motor Functioning Classification System (GMFCS) level and CP distribution (unilateral/bilateral). METHODS: In 48 ambulatory persons aged 16 to 24 years with spastic CP and in 32 able-bodied controls, physical behaviour was objectively determined with an accelerometer-based activity monitor. Total duration, intensity and type of physical activity were assessed and sedentary time was determined (lying and sitting). Furthermore, distribution of walking bouts and sitting bouts was specified. RESULTS: Adolescents and young adults with CP spent 8.6% of 24 hours physically active and 79.5% sedentary, corresponding with respectively 123 minutes and 1147 minutes per 24 hours. Compared to able-bodied controls, persons with CP participated 48 minutes less in physical activities (p < 0.01) and spent 80 minutes more sedentary per 24 hours (p < 0.01). Physical behaviour was not different between persons with GMFCS level I and II and only number of short sitting bouts were significantly more prevalent in persons with bilateral CP compared to unilateral CP (p < 0.05). CONCLUSIONS: Ambulatory adolescents and young adults with CP are less physically active and spend more time sedentary compared to able-bodied persons, suggesting that this group may be at increased risk for health problems related to less favourable physical behaviour. TRIAL REGISTRATION: Nederlands trial register: NTR1785.

Focus on fatigue amongst young adults with spastic cerebral palsy.

BACKGROUND: This study aimed to assess fatigue amongst young adults with spastic cerebral palsy (CP), to determine subgroups at risk for fatigue and to explore the relationship between fatigue and cardiopulmonary fitness and daily physical activity level. PARTICIPANTS: Young adults with spastic CP, Gross Motor Function Classification System (GMFCS) levels I to III, aged 16 to 24 years. METHODS: Fatigue (Fatigue Severity Scale) and self-reported daily physical activity (Physical Activity Scale for Individuals with Physical Disabilities) were assessed for 56 participants using questionnaires. Daily physical activity was objectively measured using accelerometry (Vitamove system) over 72 hours. Progressive maximal aerobic cycling was used to measure cardiopulmonary fitness. RESULTS: The mean Fatigue Severity Scale (FSS) score was 3.7 (SD 1.4). Forty percent of participants were fatigued, including 12.5% who were severely fatigued. Participants with bilateral CP (FSS = 4.2 (SD 1.4)) were more fatigued compared to those with unilateral CP (FSS = 3.1 (SD 1.3)) (p < 0.01). Levels of cardiopulmonary fitness (2.4 L/min (SD 0.8)) and daily physical activity (8.5% (SD 3.0)) were not significantly related to fatigue (respectively p = 0.10 and p = 0.55), although for cardiopulmonary fitness a trend was found. CONCLUSIONS: Fatigue is already present at a relatively young age amongst adults with CP, and CP subtype is a determinant of fatigue. We did not find significant evidence for a cross-sectional relation of fatigue with cardiopulmonary fitness or daily physical activity. TRIAL REGISTRATION: Nederland's trial register: NTR1785.

Improved occupational performance of young adults with a physical disability after a vocational rehabilitation intervention.

PURPOSE: The purpose of this study was to investigate into more detail how occupational performance of participants of a 1-year multidisciplinary vocational rehabilitation intervention changed over time, using a broad focus on three areas of occupational performance, addressing work, as well as self-care and leisure. In addition, we explored differences between employed and unemployed persons. METHODS: In a pre-post-intervention design, changes in occupational performance, addressing work, self-care and leisure, were evaluated using the Canadian Occupational Performance Measure (COPM) and the Occupational Performance History Interview (OPHI-II). RESULTS: Eleven young adults (median 22 years) with physical disabilities participated. Post-intervention, participants experienced fewer problems and showed improved occupational performance in work, as well as self-care and leisure, and improved satisfaction with performance. Participants also showed improved occupational identity and occupational competence, and total scores on OPHI-II. Participants who did not achieve employment did not differ in demographic characteristics. They experienced problems in all three areas of occupational performance at pre-intervention, and more difficulty in interacting in occupational settings (environment). Post-intervention, their levels of occupational identity, competence and settings were similar to those of employed persons. CONCLUSIONS: Participants showed improved occupational performance after the intervention. The goal of employment and the broad integrated approach of the intervention seemed to motivate participants to resolve problems in work, as well as self-care and leisure. Unemployed persons faced problems in all three areas of occupational performance at start. Although they seemed to catch up during the intervention, they did not achieve employment within 1 year.