Development of a Cerebral Palsy Follow-up Registry in Jordan (CPUP-Jordan).

AIMS: This study aims to describe the development of a Cerebral Palsy Follow-up Registry in Jordan (CPUP-Jordan) and to provide a baseline child and parent demographic information, birth history of the child participants, and distribution of the participants based on topographical distribution of cerebral palsy (CP) and functional classification systems. METHODS: The CPUP-Jordan was developed using a similar framework of a follow-up surveillance programme for persons with CP in Sweden (CPUP). Standard assessment forms were utilized to collect data related to child and family demographics, child birth history, and functional classifications and physiotherapy and occupational therapy assessments and interventions. Research assistants were trained to conduct the assessments. A secured web-based system was developed to store data and disseminate knowledge maintained in the registry. Children with CP were included in the registry if they have confirmed diagnosis of CP. The ascertainment age of inclusion and the minimum age of survival required are 4 years. RESULTS: One hundred sixty-seven children were registered between 2013 and 2015 (mean age is 3.6 ± 3.0 years). Forty-two percent were born premature, and 48% were less than the normal birthweight. Perinatal causes were reported for 54% of the participants. The most common type of CP based on tone disturbance was spastic type, and the most common topographical distributions of motor dysfunction were quadriplegia followed by diplegia. Fifty-six percent of the participants had severe limitation in ambulation; 48% had restricted manual abilities, and 47% had limited communication abilities even with familiar family members and partners. CONCLUSIONS: The development of CPUP-Jordan registry for children with CP proved to be both feasible and informative. The registry baseline descriptive data were similar to those reported in previous research in Jordan supporting validity of the data. The implementation of CPUP-Jordan at national level is expected to have a positive impact on children with CP, clinicians, policymakers, and researchers.

Parents' Experiences of Health and Needs When Supporting Their Adolescents With Cerebral Palsy During Transition to Adulthood.

AIMS: Parents are the primary support providers for adolescents with disabilities, their health and wellbeing is therefore of great importance when planning for youths' transition into adulthood. The aim of this study was to gain a deeper understanding of how parents of adolescents with cerebral palsy (CP) experience their own health and wellbeing and their needs for support during the adolescent's transition to adulthood. METHODS: An inductive qualitative approach was used, including interviews with 15 mothers and fathers to 10 adolescents with CP aged 17-18 years. Latent content analysis was used for analyzing the data. RESULTS: The main theme "Friction blisters chafing and healing during transition" illustrates the parents' experiences. Five sub-themes formed the parents' experiences of concerns along with sorrow and stress in life, worries about what was to come, their need for support, strategies for coping, and experiences of cohesion. CONCLUSIONS: Knowledge of parents' experiences of their health, wellbeing, and needs provide valuable information for the planning of transition for adolescents with disabilities. Help with parents' sorrow, stress, and worry in daily life might be facilitated and parental health safeguarded by a navigator who can both guide and give hands-on support.

Living in transition - experiences of health and well-being and the needs of adolescents with cerebral palsy.

BACKGROUND: Transition to adulthood for adolescents in general is a multifaceted process, and for adolescents with cerebral palsy (CP) it also involves transition from child- to adult-oriented support. CP entails a variety and combination of disabilities, which in association with external factors may make the transition to adult health services difficult. The aim of this study was to gain a deeper understanding of how adolescents with CP experience their own health, well-being and need of support during their transition to adulthood. METHODS: An inductive qualitative approach was used based on interviews with 12 adolescents with CP aged 17-18 years and living in Sweden. Manifest and latent content analysis was used for the analysis of data. RESULTS: The results are described in the main theme 'Living in transition and looking forward to being an adult, but not feeling ready yet and being in need of further support'. Five subthemes highlight the adolescents' experiences of belonging to a family, of the importance of friends and love, of managing daily activities, being surrounded by support and having hopes for the future. CONCLUSIONS: Interviews with adolescents with CP provide valuable information for the planning of transition programmes and for the support of adolescents with disabilities who are in the transition to adult living. According to the adolescents in this study, the support should be flexible and not be fixed to biological age. Personal, individualized information and support was desired by the adolescents in order to be able to manage their own transition. One option to facilitate transition is the stepping-stone of being close to parents or staff members during the first phase, after leaving the parental home.

Prevention of dislocation of the hip in children with cerebral palsy. The first ten years of a population-based prevention programme.

In 1994, a register for cerebral palsy and a health-care programme were started in southern Sweden with the aim of preventing dislocation of the hip in children with cerebral palsy. It involved all children with cerebral palsy born in 1992 or later. None of the 206 affected children born between 1992 and 1997 has developed a dislocation following the introduction of the prevention programme. Another 48 children moved into the area and none developed any further dislocation. Of the 251 children with cerebral palsy, aged between five and 11 years, living in the area on January 1, 2003, only two had a dislocated hip. One boy had moved into the area at age of nine with a dislocation and a girl whose parents chose not to participate in the programme developed bilateral dislocation. One boy, whose condition was considered to be too poor for preventative surgery, developed a painful dislocation of the hip at the age of five years and died three years later. Eight of 103 children in a control group, consisting of all children with cerebral palsy living in the area between 1994 and 2002, and born between 1990 and 1991, developed a dislocation of the hip before the age of six years. The decreased incidence of dislocation after the introduction of the prevention programme was significant (p < 0.001). Dislocation of the hip in cerebral palsy remains a serious problem, and prevention is important. Our screening programme and early intervention when lateral displacement of the femoral head was detected appear to be successful.

Cerebral palsy in southern Sweden I. Prevalence and clinical features.

UNLABELLED: The prevalence, clinical features and gross motor function of children with cerebral palsy in southern Sweden were investigated. The study covered the birth year period 1990-1993, during which 65,514 livebirths were recorded in the area. On the census date (1 January 1998), 68366 children born in 1990-1993 lived in the area. The study comprised 167 children, 145 of them born in Sweden and 22 born abroad. The livebirth prevalence was 2.2 per 1,000, and the prevalence including children born abroad was 2.4 per 1,000. The distribution according to gestational age, birthweight and subdiagnoses was similar to that in earlier Swedish studies, except for a higher rate of dyskinetic syndromes in this study. CONCLUSION: The point prevalence of cerebral palsy was 2.4 and the livebirth prevalence was 2.2. Children born abroad had a higher prevalence and were more often severely disabled. Severe disability was often combined with associated impairments such as mental retardation, epilepsy and visual impairment.

Cerebral palsy in southern Sweden II. Gross motor function and disabilities.

UNLABELLED: The gross motor function and disabilities in children with cerebral palsy in southern Sweden were investigated and related to clinical features. The study covered the birth year period 1990-1993 and comprised 167 children, 145 of them born in Sweden and 22 born abroad. The clinical features and gross motor function were analysed at a mean age of 6.8 y. Clinical features were obtained from a continuing healthcare follow-up programme. Gross motor function was classified according to the Gross Motor Function Classification System (GMFCS). Walking independently was possible for 86% of the hemiplegic, 63% of the pure ataxic, 61% of the diplegic and 21% of the dyskinetic children. None of the tetraplegic children was able to walk. The classification of gross motor function revealed that 59% of the children were categorized into levels I and II (mildly disabled), 14% into level III (moderately disabled) and 27% into levels IV and V (severely disabled). Children born abroad were more severely disabled. CONCLUSION: The standardized age-related classification system GMFCS enabled a specific description of gross motor function in relation to clinical features. Significant differences between GMFCS levels and subgroups of diagnosis, aetiology. intellectual capacity, epilepsy and visual impairment were found.

Comparison of the Gross Motor Function Measure and Paediatric Evaluation of Disability Inventory in assessing motor function in children undergoing selective dorsal rhizotomy.

This study was designed to compare assessment with the functional outcome measures Gross Motor Function Measure (GMFM) and Pediatric Evaluation of Disability Inventory (PEDI) over time, in children with cerebral palsy (CP) undergoing selective dorsal rhizotomy combined with individualised physiotherapeutic interventions. Using the Gross Motor Function Classification System (GMFCS), 18 children with spastic diplegia were divided into two groups according to age-related severity of motor function impairment. Data were collected preoperatively, and at 6 and 12 months postoperatively. Both instruments were sensitive to changes in function over time in the series as a whole and in the group with milder impairment, although the PEDI detected significant changes earlier. In the group with more severe impairment, changes in function were detected only with the PEDI, not with the GMFM. Thus, the instruments are to be considered complementary tests, because they measure different aspects of function.

The American Paediatric Evaluation of Disability Inventory (PEDI). Applicability of PEDI in Sweden for children aged 2.0-6.9 years.

The American Paediatric Evaluation of Disability Inventory (PEDI) is a new instrument for evaluating functional performance in disabled children aged 6 months to 7.5 years. It was developed to determine a child's functional capacity and performance in three domains, self-care, mobility and social function, as reflected in scores on three scales: (i) functional skills (current capability in specific tasks), (ii) caregiver assistance (i.e. provided to facilitate the child's performance), and (iii) modifications (i.e. environmental or technical modifications needed to facilitate the child's function). The present study was designed to compare results obtained using the PEDI in a Swedish sample with the American normative data, and to analyse the content and relevance of PEDI items for use in Sweden. The PEDI was administered as a questionnaire in structured interview form to the parents of 52 non-disabled Swedish children aged 2.0-6.9 years, divided into ten age groups. Correlation analysis (Pearson's r) showed scores for the Swedish sample to manifest strong correlation with the respective American normative data, both for the functional skills (r = 0.90-0.98) and caregiver assistance (r = 0.93-0.99) scales, respectively. Scores for the modification scale were not compared. Thus, the results suggest the American normative data to be appropriate for reference purposes in Sweden.

Reliability of the gross motor function measure in cerebral palsy.

The Gross Motor Function Measure (GMFM), an instrument comprising five dimensions devised by Russell and co-workers (7) to measure gross motor function in children with cerebral palsy (CP) or brain damage, enables changes in performance status to be evaluated after therapy or when monitored over time. We analysed its inter-rater and intra-rater reliability on the three most difficult dimensions. A video-recording of three children with CP performing test tasks was assessed on two occasions at an interval of six months by each of the 15 physiotherapists using the GMFM manual but without previous experience or training in the use of the instrument. Mean percentage scores were similar at the first and second assessments. Both inter- and intra-rater reliabilities were good, inter-rater reliability being 0.77 and 0.88 at the first and second assessments, respectively, and intra-rater reliability 0.68 at the second assessment. The findings suggest the GMFM to be a useful and reliable instrument for assessing motor function and treatment outcome in CP.

Long-term effects of physical exercise on working capacity and pulmonary function in cystic fibrosis.

Seven patients with cystic fibrosis aged 6 to 20 were enrolled for 30 months in a daily exercise program. After 12 months conventional chest physiotherapy was withdrawn. Patients with low initial Shwachman scores improved as regards maximal working capacity. Spirometric data and volume of trapped gas indicated opening of closed airways. We suggest that physical exercise in general should be the basis of pulmonary therapy in cystic fibrosis. Other forms of physiotherapy are advisable when hard physical exercise is not feasible.