Parents' Assessment of Disability in Their Children With Down Syndrome.

AIM: To describe a population of children with Down syndrome and evaluate their parents' assessment of disability. METHODS: Medical records of a population of 80 children with Down syndrome aged 5 to 17 years were analyzed for genetic background and associated diagnoses. And 27 parents to their children agreed to assess disability by employing a set of 26 International Classification of Functioning, Disability and Health body function (b) codes and activity and participation (d) codes. Clinical data were gathered and analysis of parents' assessment of disability using psychometric and Rasch analysis was performed. RESULTS: Clinical data on 27 children assessed by their parents and 53 children not assessed had identical associated diagnoses. The 26 International Classification of Functioning, Disability and Health codes and qualifiers had a mean score of 2.67 (range 1.26-4.11) and corrected code-total correlations mean of 0.55 (range -1.17 to 0.82). Rasch analysis showed proper code MNSQ infit and outfit values with mean 1.03 and 1.06. CONCLUSION: Clinical data on 27 children assessed were similar to 53 children that were not evaluated. Parents' assessment of the 27 children showed good psychometric and Rasch analysis properties. Similar results might be expected in the total population of 80 children.

Parents' Expressions of Concerns and Hopes for the Future and Their Concomitant Assessments of Disability in Their Children.

AIM: To assess parents' ability to express their concerns and hopes for the future in their children with disability and assess their children's disability as well as to analyse these data for consistency. METHOD: Parents of 162 children with spina bifida, spinal muscular atrophy, muscular disorders, cerebral palsy, visual impairment, hearing impairment, mental disability, or disability following brain tumours were asked to freely express their concerns and hopes for the future and to assess disability in their own children by employing a set of 26 International Classification of Functioning, Disability and Health, Children and Youth Version (ICF-CY) body function (b) codes and activity and participation (d) codes. A grounded theory approach was employed to systematize parents' expressions of concerns and hopes; then, parents scored qualifiers on a 5-step qualitative Likert scale. Parents assessed their children's disability in the same way using the ICF-CY 5-step qualifier scale. RESULTS: Altogether, 119 parents freely expressed their concerns and hopes, and 101 of them also assessed their children's disability using the 26 ICF-CY codes. A total of 475 expressions of concern and hopes (issues) were expressed and categorized into 34 areas of concern and hopes (subsections). The most frequently mentioned issues were education; understanding, goodwill, and communication between parents; and community support. Qualitative data on both 5-step qualifier scales showed good reliability. Rasch analysis maps on concerns and hopes for children as well as on the ICF-CY assessment demonstrated good alignment and a clinically relevant progression from the least to the most disabled children. CONCLUSION: Parents can express valid and reliable data on their concerns and hopes for the future and can reliably assess disability in their own children.

Parents' Assessments of Disability in Their Children Using World Health Organization International Classification of Functioning, Disability and Health, Child and Youth Version Joined Body Functions and Activity Codes Related to Everyday Life.

AIM: To help parents assess disability in their own children using World Health Organization (WHO) International Classification of Functioning, Disability and Health, Child and Youth Version (ICF-CY) code qualifier scoring and to assess the validity and reliability of the data sets obtained. METHOD: Parents of 162 children with spina bifida, spinal muscular atrophy, muscular disorders, cerebral palsy, visual impairment, hearing impairment, mental disability, or disability following brain tumours performed scoring for 26 body functions qualifiers (b codes) and activities and participation qualifiers (d codes). Scoring was repeated after 6 months. Psychometric and Rasch data analysis was undertaken. RESULTS: The initial and repeated data had Cronbach α of 0.96 and 0.97, respectively. Inter-code correlation was 0.54 (range: 0.23-0.91) and 0.76 (range: 0.20-0.92). The corrected code-total correlations were 0.72 (range: 0.49-0.83) and 0.75 (range: 0.50-0.87). When repeated, the ICF-CY code qualifier scoring showed a correlation R of 0.90. Rasch analysis of the selected ICF-CY code data demonstrated a mean measure of 0.00 and 0.00, respectively. Code qualifier infit mean square (MNSQ) had a mean of 1.01 and 1.00. The mean corresponding outfit MNSQ was 1.05 and 1.01. The ICF-CY code τ thresholds and category measures were continuous when assessed and reassessed by parents. Participating children had a mean of 56 codes scores (range: 26-130) before and a mean of 55.9 scores (range: 25-125) after repeat. Corresponding measures were -1.10 (range: -5.31 to 5.25) and -1.11 (range: -5.42 to 5.36), respectively. Based on measures obtained at the 2 occasions, the correlation coefficient R was 0.84. The child code map showed coherence of ICF-CY codes at each level. There was continuity in covering the range across disabilities. And, first and foremost, the distribution of codes reflexed a true continuity in disability with codes for motor functions activated first, then codes for cognitive functions, and, finally, codes for more complex functions. CONCLUSIONS: Parents can assess their own children in a valid and reliable way, and if the WHO ICF-CY second-level code data set is functioning in a clinically sound way, it can be employed as a tool for identifying the severity of disabilities and for monitoring changes in those disabilities over time. The ICF-CY codes selected in this study might be one cornerstone in forming a national or even international generic set of ICF-CY codes for the benefit of children with disabilities, their parents, and caregivers and for the whole community supporting with children with disabilities on a daily and perpetual basis.

Environmental needs in childhood disability analysed by the WHO ICF, Child and Youth Version.

INTRODUCTION: The WHO has launched a common classification for disabilities in children, the International Classification of Functioning, Disability and Health, Child and Youth Version (ICF-CY). We wanted to determine whether cat-egories of the environmental (e) and the body functions (b) components of the classification could address environmental needs in children with different disorders and various disability severities. METHODS: A set of 16 e categories and 47 b categories were selected and worded to best enable parents to describe children's everyday support needs and environmental influences through interviews in their own homes. RESULTS: Of the 367 invited parents, 332 (90.5%) participated, providing data on children with spina bifida, spinal muscular atrophy, muscular disorders, cerebral palsy, visual impairments, hearing impairments, mental disability and disabilities following brain tumour treatment. The mean age of children across disabilities was 9.4 years (range: 1.0-15.9). The mean e code score was 35.7 (range: 4.0-64.0), and the mean b code score was 32.2 (range: 0.0-159.0). The most urgent needs as detected by qualifier 4 environmental categories scores were common among children with complex disorders and issues related to health professionals, legal services and health services. CONCLUSIONS: Parents understand the environmental and body function components in a meaningful manner and the codes seem to be valid. Special emphasis should be given to environmental issues for children with more complex disabilities. There was no correlation between the severity of a disability and environmental issues, indicating that each child's needs were basically met, irrespective of disability severity. FUNDING: partnership project § 16, 21, 31 administered by the Danish Health Authority. TRIAL REGISTRATION: not relevant.

Positive experience with intrathecal baclofen treatment in children with severe cerebral palsy.

INTRODUCTION: Treatment of severe spasticity and dystonia with intrathecal baclofen (ITB) in children has been shown to be effective and has therefore been employed in the Region of Southern Denmark. The aim of this retrospective study was to analyse the efficacy and adverse events since ITB was introduced in 2003. METHODS: A total of 46 children who had a baclofen pump from April 2003 to January 2013 were included. The children's medical records were reviewed and clinical characteristics, efficacy and adverse events were registered. The efficacy of treatment experienced by parents was ascertained by telephone interviews, and data were rated on a Likert scale ranging from one to five, where one was no effect and five was marked improvement. RESULTS: After ITB, spasticity was reduced from a median of four to two in the upper extremities and from a median of four to one in the lower extremities. Baclofen infusion was 105.1-2,000 micrograms/day (mean 494.9 micrograms/day). Oral baclofen was reduced from 27.3 to 17.7 mg/day after ITB (p < 0.01). The parents' assessment of improvement in well-being, function and ease of care of their child had a mean score of 3.7, 2.2 and 3.4, respectively. 87.1% of parents stated that ITB had been worthwhile, and 90.3% would recommend it to other parents. Most infectious and mechanical adverse events were experienced during the first 200 days after pump implantation. The total complication rate was 0.40 per pump year. CONCLUSION: ITB resulted in reduced spasticity in children with severe spasticity and dystonia, and ITB could be considered safe. Parents' satisfaction with ITB was rated as good and most parents would recommend ITB to others. FUNDING: not relevant. TRIAL REGISTRATION: not relevant.

Assessing Children With Disabilities Using WHO International Classification of Functioning, Disability and Health Child and Youth Version Activities and Participation D Codes.

AIM: Evaluation of the International Classification of Functioning, Disability and Health child and youth version (ICF-CY) activities and participation d code functions in clinical practice with children across diagnoses, disabilities, ages, and genders. METHODS: A set of 57 codes were selected and worded to describe children's support needs in everyday life. Parents of children aged 1 to 15 years participated in interviews to discuss and rate their child's disability. RESULTS: Of 367 invited parents, 332 (90.5%) participated. The mean age of their children with disability was 9.4 years. The mean code scores were 50.67, the corrected code-total correlations were .76, intercode correlations had the mean of 0.61, and Cronbach's α was .98. As a result of Rasch analysis, graphical data for disability measures paralleled clinical expectations across the total population of 332 children. CONCLUSION: The World Health Organization International Classification of Functioning, Disability and Health child and youth version d code data can provide a coherent measure of severity of disability in children across various diagnoses, ages, and genders.

The Nordic Five to Fifteen questionnaire could provide the basis for a common neurological disability variable.

AIMS: Assessing disabilities in children is essential and Danish parents provide increasingly important feedback on how their child's disability affects daily living. The Nordic Five to Fifteen (FTF) parent questionnaire is widely used in Nordic countries to detect atypical or delayed development in children. Our study evaluated its internal validity and whether it could be used to generate a common disability variable across childhood neurological disorders and severities. METHODS: The 28-statement FTF questionnaire was completed by the parents of children with spina bifida, muscular disorders, spinal atrophy, cerebral palsy, blindness, deafness, mental retardation and disability, who received treatment for brain tumours. Psychometric analysis and Rasch analysis of the five FTF code qualifier level data were carried out. RESULTS: A total of 227 of 332 (68.4%) parents participated. The mean qualifier score was 3.06 (standard deviation 0.89, range 2.31-4.26), and the variances mean was 1.57 (range 0.87-2.38). The corrected code-total correlation was 0.65, and reliability was 0.96. The Rasch analysis demonstrated good fit alignment of codes. CONCLUSION: The FTF questionnaire can be used with children with neurological disabilities, and the Rasch scale analysis results indicate that it could form the analytical basis for developing a common disability variable.

Symptoms and time to diagnosis in children with brain tumours.

INTRODUCTION: Clinical symptoms in brain tumours in children are variable at onset and diagnosis is often delayed. Symptoms were investigated with regard to brain tumour localisation, prediagnostic symptomatic intervals and malignancy. MATERIAL AND METHODS: Clinical data from children aged 0-17 years from Southern Denmark were analysed retrospectively and the results were correlated with data on prehospital symptoms obtained from interviews with parents and general practitioners. RESULTS: A total of 55 children diagnosed during a period of five years were indentified and 31 interviews were obtained. A total of 19 (41%) of the tumours were supratentorial hemispheric and midline and 27 (59%) were infratentorial. At supratentorial localisations, 42% experienced vomiting as their first symptom followed by seizures in 37% and headache in 31%. At infratentorial localisations, headache occurred in 62%, vomiting in 55% and ataxia in 48% of the cases. The prediagnostic symptomatic interval had a median duration of 30 days with vomiting (range 3-330 days), a median of 75 days with headache (5-730 days) and a median of 75 days with ataxia (1-730 days). CONCLUSION: Diagnosis is often late in relation to the presenting symptoms. An earlier diagnosis may be achieved if a brain tumour is considered as soon as any child presents with the relevant symptoms. FUNDING: Not relevant. TRIAL REGISTRATION: ISRCTN88306789.

[Rhizotomy for children with severe spastic cerebral palsy]. / Rhizotomibehandling af børn med svaer spastisk cerebral parese.

INTRODUCTION: Severe spasticity is a limiting factor for motor development in children with spastic cerebral palsy. Botulinum toxin, intrathecal baclofen and peroral baclofen all reduce spasticity but might also limit improvements in functional development over time. In the selective dorsal or posterior rhizotomy (SDR) approach, afferent sensory nerve fibers are cut while efferent motor fibers are preserved. In this way spasticity is reduced and motor functions can improve. SDR is an established treatment method, and the first Danish study is reported. MATERIAL AND METHODS: Twenty Danish children with severe spastic cerebral palsy were evaluated, operated on and trained over a 10-year period from 1992 to 2002. Those on whom operation was performed ranged from 4 to 16 years of age (median 8 years), and training and follow-up took place during the ensuing 60 months. At time of operation, 20-40% of 100-120 dorsal root filaments were cut, corresponding to the II-V lumbar and I-II sacral nerve roots. RESULTS: Spasticity in the lower extremities measured before SDR showed an average Asworth score of 2.0-4.2 (median 3.1). Eighteen months after SDR, scores were 0.8-1.8 (median 1.0), and at 60 months 0.8-1.8 (median 1.0). Both post-operative values were significant (t-test, p < 0.001). Mobility improved over a longer period of time: the Illinois St. Louis scale values before SDR were 1-9 (median 6), while at 18 months post-operative they were 1-9 (median 5) and at 60 months post-operative 1-9 (median 4). At 18 months, scores were non-significant (t test, p > 0.05), but at 60 months they were significant (t < 0.05). According to the Montgomery scale, 4 children had worse post-operative scores and 12 children had better scores. When comparing age at operation with outcome, we observed a certain degree of concordance between relatively younger age and better post-operative muscular function (Pearson's r = 0.8). CONCLUSION: SDR resulted in early and lasting reduction in spasticity in all 20 children operated upon. Improved muscular function, however, required training and time. Not until 60 months after operation were functional measures significantly better than the preoperative values.

[Intrathecal baclofen in the treatment of severe spastic tetraplegia and dystonia in children and adolescents]. / Intratekal baclofenbehandling ved svaer spastisk tetraplegi og dystoni hos børn og unge.

INTRODUCTION: Continuous intrathecal baclofen has been used over the past years especially in adult patients with spasticity of spinal origin. Children and young adults with severe spasticity and dystonia of cerebral origin are difficult to treat in spite of optimal systemic antispasmotic therapy with baclofen, tizanidine, dantrolene and/or diazepam. Intrathecal baclofen has therefore been applied in a group of young patients. MATERIAL AND METHODS: Eight children and young adults from East Denmark with spasticity and 12 with dystonia aged 3-18 years (median 10.9 years) were tested, operated and treated with continuous intrathecal baclofen for a period of 2-64 months (median 22.2 months). Registration of efficacy, fillings, adjustments of baclofen and other therapies were performed in an out patient setting since 1995. RESULTS: Spasticity in lower extremities was reduced from Ashworth score 3.5-4.5 (median 4.2) to Ashworth score 2.5-4.0 (median 2.9; p < 0.001) during infusion with baclofen 5-33 micrograms/kg/24 hours (median 19 micrograms/kg/24 hours). The infusion catheter tip was placed at levels Th1-Th12 (median Th7.5). Peroral baclofen was reduced from an average of 5.0 to 0.44 mg/kg/24 hours, tizanidine from 0.4 to 0.1 mg/kg/24 hours, and dantrolene from 4.0 to 0.4 mg/kg/24 hours. After initial adjustments successively increased dosages of average 0.46 microgram/kg/month were needed to maintain the same level of efficacy. In questionnaires parents or guardians rated less spasticity in lower extremities in 15 out of 19 patients, and less pain in 13 out of 19 patients. CONCLUSION: Continuous intrathecal baclofen was effective in treating severe spasticity and dystonia of cerebral origin with major effect on muscles of the lower extremities, pelvis, and back and in particular opisthotonus was relieved. Efficacy on upper extremities was far less pronounced.