The need for additional care in patients with classical galactosaemia.

Purpose: Classical galactosaemia is an inborn error of galactose metabolism which may lead to impairments in body functions and accordingly, need for additional care. The primary aim of this study was to establish the type and intensity of this additional care. Materials and methods: Patients with classical galactosaemia aged ≥2 years were evaluated with the Capacity Profile, a standardised method to classify additional care needs according to type and intensity. Based on a semi-structured interview, current impairments in five domains of body functions were determined. The intensity of additional care was assessed (from 0, usual care, to 5, total dependence). Results: Forty-four patients with classical galactosaemia, 18 males and 26 females (median age 15 years, range 2-49 years), were included. There was a wide spectrum of impairments in mental functions. Motor function impairments were present in four patients, and mild speech impairments in eight patients. Additional care for sensory functions was uncommon. All patients needed a diet, which care is scored in the physical health domain. Conclusions: Apart from the diet all patients need, classical galactosaemia leads to the need for additional care mainly in the domains of mental functions and speech and voice functions. Implications for rehabilitation The Capacity Profile is a useful tool to demonstrate additional care needs in classical galactosaemia. In classical galactosaemia additional care is mostly indicated by mental impairments and speech and voice functions. One-fifth of patients have impairment of speech and voice functions at time of the study, and half of all patients had received speech therapy in childhood. Over 70% of patients need additional care/help due to impairment of mental functions, ranging from coaching due to social vulnerability to full day care.

Interrater reliability of the capacity profile in children with neurodevelopmental disabilities.

OBJECTIVE: To investigate the interrater reliability of the Capacity Profile (CAP) in children with neurodevelopmental disabilities. DESIGN: Cross-sectional study. SETTING: Six rehabilitation centers in the Netherlands. PARTICIPANTS: Children (N=70) with permanent, nonprogressive neurodevelopmental disabilities. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: The CAP is a method to classify additional care needs of children with nonprogressive neurodevelopmental disabilities in 5 domains of body functions: physical health, motor functions, sensory functions, mental functions, and voice/speech functions. The CAP was scored independently by 2 trained physiatrists during an outpatient visit. Interrater reliability was evaluated using an intraclass correlation coefficient (ICC). RESULTS: Interrater reliability of the CAP is as follows: physical functions, ICC=.74; motor functions, ICC=.85; sensory functions, ICC=.61; mental functions, ICC=.85; and voice/speech functions, ICC=.79. CONCLUSIONS: These findings support the interrater reliability of the CAP, when scored during a visit to the rehabilitation center.

Severe fatigue and reduced quality of life in children with hereditary motor and sensory neuropathy 1A.

Severe fatigue and low quality of life are reported by a majority of adult patients with hereditary motor and sensory neuropathy 1A. In children with hereditary motor and sensory neuropathy 1A, the prevalence and impact of fatigue have not been studied yet. In this questionnaire survey, 55 Dutch children (response rate 77%) with genetically confirmed hereditary motor and sensory neuropathy 1A participated (mean age 15 years [standard deviation 2.1]). Prevalence of severe fatigue (based on a cut-off score of the Checklist Individual Strength) was 24%, in contrast to 14% in a Dutch school-based population (P < .05). Almost all quality-of-life scores (measured with the Child Health Questionnaire-Child Form 87) were significantly worse than population norms (P < .05). Fatigue severity was associated significantly (P < .01) with all quality-of-life scores (-0.4 < r < -0.7). In conclusion, severe fatigue and diminished quality of life are more frequent among children with hereditary motor and sensory neuropathy 1A compared to healthy peers. The strong association between fatigue severity and quality of life suggests a negative impact of fatigue on quality of life in these children.

Participation outcomes for children with acquired brain injury: a narrative review.

AIM: To review the literature on participation outcomes used in children and adolescents with acquired brain injury (ABI) and to synthesize the available evidence on recovery trajectories in participation after ABI. METHOD: This study searched electronic databases (Medline, Cinahl, Embase and PsychInfo) from March 2011 back to the earliest available time (1966) using the following terms with brain injury (brain tumours excluded) and children: social or community or school and outcome assessment or participation. Retrieved articles were rated for methodological quality using Oxford Centre for Evidence Based Medicine criteria (CEBM). RESULTS: Sixteen articles were included for analysis. The methodological characteristics and quality of these studies varied considerably. Three studies used an explicit participation measure, nine studies featured an implicit participation measure and four used tailored participation measures. There is level 1c evidence that children and adolescents with ABI have participation restrictions at home, at school and in the community 18 months (SD = 14) after discharge. CONCLUSIONS: The available literature indicates that children and adolescents with ABI are at risk for participation restrictions. Research on recovery trajectories in participation after ABI in children is lacking. Longitudinal studies using explicit participation measures and higher quality research methodologies (quantitative, qualitative and mixed methods) are recommended.

Immediate effect of a wrist and thumb brace on bimanual activities in children with hemiplegic cerebral palsy.

AIM: The aim of this study was to determine the immediate effect of wearing a wrist and thumb brace on the performance of bimanual activities in children with spastic hemiplegic cerebral palsy. METHOD: In a pre- and post-test cohort study of 25 children (age range 4-11y; mean age 8y 4mo [SD 2y 2mo]; 16 males, 9 females) with spastic hemiplegic cerebral palsy with a Zancolli classification hand score of I, IIA, or IIB (mild and moderate hand dysfunction; children with a Zancolli classification of III - severe hand dysfunction - were excluded from this study), performance of bimanual activities was evaluated with the Assisting Hand Assessment (AHA) on three occasions: one assessment with a static wrist and thumb brace placed on the affected hand and two other assessments without a brace. The differences between AHA scores obtained at the three assessments were evaluated using the repeated measures analysis of variance. RESULTS: Performance of bimanual activities while wearing the brace improved significantly compared to performance without the brace (p<0.001). With the brace, the mean AHA score increased by 3.2 (95% confidence interval 2.1-4.3) from 59.1 to 62.3. The scores of the two assessments without the brace did not differ significantly. INTERPRETATION: In children with spastic hemiplegic cerebral palsy, bracing of the wrist and thumb immediately improves spontaneous use of the affected upper limb in bimanual activities, possibly because bracing permits a more functional hand position.

Construct validity of the Capacity Profile in adolescents with cerebral palsy.

OBJECTIVE: To establish construct validity of the Capacity Profile, a method to comprehensively classify additional care needs in five domains of body functions (physical health, motor, sensory, mental, voice and speech functions), in adolescents with non-progressive, permanent conditions such as cerebral palsy. DESIGN: Cross-sectional study. SUBJECTS: Ninety-four adolescents with cerebral palsy: 60 boys, 34 girls, median age 14.3, range 12-16 years, unilateral (n = 37), bilateral (n = 57), spastic (n = 76), ataxic (n = 4), dyskinetic (n = 5), mixed (dyskinetic and spastic, n =9), Gross Motor Function Classification System: level I (n = 50), level II (n = 6), level III (n = 10), level IV (n = 8), level V (n = 20). METHODS: Associations were calculated between Capacity Profile domains and Vineland Adaptive Behavior Scales (communication, daily activities, social and motor skills) and Gross Motor Function Classification System using Spearman's rho. Furthermore, we explored the independent contribution of the Capacity Profile domains to activities and participation measured with the Vineland Adaptive Behavior Scales. RESULTS: All Capacity Profile domains were significantly associated with all domains of the Vineland Adaptive Behavior Scales and the Gross Motor Function Classification System (P<0.05). Multiple regression analysis showed that the Capacity Profile contributed 87% to variance in communication (Capacity Profile-voice 78%, mental 8% and physical 1%), 85% to daily activities (Capacity Profile-mental 75%, motor 8% and voice 2%), 60% to social skills (Capacity Profile-voice 56% and mental 4%), and 91% to motor skills (Capacity Profile-motor 87%, mental 3% and sensory 1%). CONCLUSION: These findings support the construct validity of the Capacity Profile in adolescents with cerebral palsy. Construct validity in other medical conditions should be further investigated.

Construct validity of the Capacity Profile in preschool children with cerebral palsy.

The Capacity Profile (CAP) classifies additional care needs, subdivided into five domains of body functions (physical health, motor, sensory, mental, and voice/speech) of children with stable conditions. Construct validity of the CAP was established in 72 children (56 males, 16 females) with cerebral palsy (CP); median age 2 years 7 months, range 2 years 6 months to 3 years; 34 unilateral and 37 bilateral spastic-type CP, one dyskinetic-type CP. Gross Motor Function Classification System (GMFCS) classification was 24 in level I, eight in level II, 18 in level III, 14 in level IV, and eight in level V. All CAP domains were significantly associated (p<0.001) with the Functional Skills (rho=-0.42 to -0.85) and Caregiver Assistance scales (rho=-0.42 to -0.82) of the Dutch Paediatric Evaluation of Disability Inventory. The CAP-motor domain and GMFCS were strongly correlated (rho=0.91, p<0.001). Stepwise regression analysis demonstrated that the CAP domains contributed 74% to mobility (CAP-motor 66%, mental 6%, voice 2%); 75% to self-care (CAP-voice 61%, mental 12%, physical 2%); and 70% to social functionality (CAP-mental 68%, voice 2%). CAP demonstrated good construct validity in young children with CP. The independent contribution of CAP domains to daily function underscores the importance of comprehensive assessment with regard to all domains of body functions in heterogeneous conditions like CP.

How well do care providers know the children with developmental disabilities they care for?

AIM: To assess the knowledge from memory of caregivers about the most significant impairments contributing to additional care needs in children with developmental disabilities in therapeutic toddler groups. METHODS: Children's needs for additional care due to impairments of physical health, motor, sensory, mental and voice and speech functions were separately classified using the capacity profile (CAP). Twenty-three therapists and teachers of toddler groups in two regional centres for paediatric rehabilitation assessed the CAP individually, unprepared and without consulting their notes or the clinical record. These CAP scores (150 CAPs of 44 children) were compared with those based on the clinical record using weighted kappa statistics. RESULTS: Weighted kappa values for the two sets of CAP scores ranged from 0.22-0.74 (median 0.53), with the lowest scores for the sensory domain (median 0.32, range 0.22-0.52) and the highest scores for the motor domain (median 0.62, range 0.56-0.74). CONCLUSION: Team members in general had only moderate remembered knowledge of the current impairments determining need of additional care. Remembered knowledge was the poorest for domains not easily observable, such as sensory functions. As this knowledge is essential for optimizing the child's daily environment, improvement of this type of knowledge should be facilitated.

The Capacity Profile: a method to classify additional care needs in children with neurodevelopmental disabilities.

The aim of this study was to determine the interrater reliability and stability over time of the Capacity Profile (CAP). The CAP is a standardized method for classifying additional care needs indicated by current impairments in five domains of body functions: physical health, neuromusculoskeletal and movement-related, sensory, mental, and voice and speech, in children from 3 to 18 years of age. The intensity of care in each domain is defined from 0 (no need for additional care) to 5 (needs help with every activity). The intensity of additional care in each of the five separate domains indicates the CAP for the individual child. We developed the CAP to inform the parents and other caregivers of children with non-progressive, permanent neurodevelopmental disabilities, such as cerebral palsy and myelomeningocele, about the consequences of these conditions. To determine interrater agreement and stability over time, the CAPs of 67 children (39 males, 28 females) with a neurodevelopmental disability (mean age 18y [SD 1.2y]; range 14-22y) were assessed based on a semi-structured interview. In addition, the CAPs of the same individuals at the age of 3 years were determined based on a chart review. Interrater agreement of the CAP at the age of 3 was good to very good (weighted kappa 0.64-0.92). Agreement between the CAP at the age of 18 and the CAP at the age of 3 (providing evidence for stability over time) was also good (weighted kappa 0.68-0.77), except for the domain 'physical health functions', about which agreement was relatively poor (0.47). We conclude that the CAP is a reliable instrument for classifying the additional needs of a child with a non-progressive, permanent neurodevelopmental disability. The preliminary evidence for the stability over time of such needs according to the CAP should be validated in a prospective study.

The reproducibility and validity of the Canadian Occupational Performance Measure in parents of children with disabilities.

OBJECTIVE: To study the reproducibility (inter-rater agreement), the construct and criterion validity of the Canadian Occupational Performance Measure (COPM) in the parents of children with disabilities. DESIGN: The COPM was administered twice by two different occupational therapists. The inter-rater agreement of the content of the prioritized problems was explored. Data analysis of the reproducibility of the scores was based on the Bland and Altman method. Measures used: The construct validity was studied by comparing the results of the COPM with the Pediatric Evaluation of Disability Inventory, and a quality of life questionnaire. The criterion validity was verified with an open-ended question. SETTING: Occupational therapy departments of a university hospital and three rehabilitation institutes. SUBJECTS: One hundred and twenty-nine consecutive parents of children referred for occupational therapy. RESULTS: Data were obtained for 80 children with a mean age of 3.7 years (range 1-7.5). Of the prioritized problems identified in the first interview, 80% were also prioritized in the second interview. The limits of agreement were - 2.4 to +2.3 for the mean performance score and - 2.3 to + 2.6 for the mean satisfaction score. Assumptions about the construct and criterion validity were confirmed. CONCLUSIONS: The inter-rater agreement of the prioritized problems is good enough for client-centred occupational therapy. The reproducibility of the performance and satisfaction scores is moderate. The results support the construct and criterion validity. The COPM identifies many child-unique problems that are not assessed with existing standardized measurement instruments or with a simple open-ended question.

Predicting additional care in young children with neurodevelopmental disability: a systematic literature review.

Children with developmental disabilities often show a variety of associated impairments that lead to a lifelong need for additional care. Careful assessment of these impairments is required not only for diagnostic purposes but also to inform the parents about the expected additional care needs in the future. We present a systematic review of the literature to identify instruments that classify the type and amount of this care for the individual child. A literature search was performed in the Medline database (January 1966 - June 2005) on instruments that classify the type and amount of expected additional care needs in the future. Seven standardized measurement instruments describing current additional care needs were identified, but none of these instruments was developed to provide information about the expected need for additional care in the future. For parents of young children with non-progressive developmental disorders it is essential to be informed on the expectations of required additional care in the future. However, comprehensive instruments providing such information are currently lacking and, thus, need to be developed.

The effect of botulinum toxin type A treatment of the lower extremity on the level of functional abilities in children with cerebral palsy: evaluation with goal attainment scaling.

OBJECTIVE: To measure the effect of botulinum toxin type A (BTX-A) treatment in children with cerebral palsy with regard to individual goals concerning functional abilities, using goal attainment scaling. DESIGN: A single-blind randomized multiple baseline/treatment phase design across subjects. SETTING: The paediatric department of a rehabilitation centre. SUBJECTS: Eleven children with cerebral palsy participated. INTERVENTION: BTX-A treatment of the lower extremity. MAIN MEASURES: A six-point goal attainment scaling of three individual treatment goals at the level of functional abilities. Standardized video-tapes of each goal were recorded weekly for a period of 14 weeks. Rating on the predetermined goal attainment scaling was blinded. RESULTS: Nine of the 11 subjects showed significant improvement in 18 out of 33 goals. Seven subjects showed clinically relevant improvement (at least 2 points on the goal attainment scaling) in 11 goals. Testing the difference between all medians of baseline measurements (after correction for improvement during baseline) and the medians of the treatment phase measurements for all goal attainment scaling scores (n = 33) resulted in significant improvement (p < 0.001). Tested at subject level (medians of the three goal attainment scaling scores per assessment, n = 11), a significant improvement was also found (p = 0.005). The change in goal attainment scaling score was related to the moment of treatment with BTX-A. CONCLUSION: Clinically relevant improvement in individual rehabilitation goals at ability level, achieved with the treatment of BTX-A in children with cerebral palsy, were demonstrated using the goal attainment scaling method.