Cognitive control in adolescents with neurofibromatosis type 1.

Neurofibromatosis Type 1 (NF1) is a genetic disorder characterized by partial loss of growth control that affects the central nervous system. NF1 has been consistently associated with cognitive dysfunction, although there is no consensus on the cognitive profile in NF1 or on brain-cognition relationships. To clarify the pattern of cognitive dysfunction, performance of 16 NF1 patients and 16 age- and sex-matched controls (mean age = 14.5 years, SD = 1.3) was compared on computerized tasks measuring perception, executive functioning (inhibitory control, cognitive flexibility, and working memory), and motor control. A further aim of this study was to contrast performance on tasks or task parts requiring varying levels of cognitive control to find out whether this could explain potential difficulties experienced by this population in different cognitive domains or at different stages of information processing. Repeated measures analyses of variance showed that group differences, indicating poorer performance of NF1 patients, varied as a function of the level of cognitive control required. Evidence was also found for more basic motor skill problems in NF1 patients. Furthermore, NF1 patients were generally slower than controls. Results are discussed in the context of what is known about brain-cognition relationships in NF1.

Experiences of siblings of children with physical disabilities: an empirical investigation.

PURPOSE: This study aimed to investigate the experiences of Dutch siblings of children with physical disabilities (n=43). METHOD: Interviews were conducted to investigate experiences of siblings in 3 areas: the sibling relationship, the relationship with parents, and the relationship with others. The siblings also completed a coping response inventory. RESULTS: The siblings reported difficulties in undertaking activities and in communicating with their brother/sister with a disability. Most siblings worried about the future and the health of their brother/sister with a disability. The siblings reported open communication and trust as the main characteristics of the relationship with their parents. They acknowledged their parents' attempts to treat all children equally, although parents were not always successful in doing so. Having a sibling with a disability did not cause problems in the relationship with friends. The awkward reactions of strangers caused the siblings much annoyance and distress. Siblings in this sample used more approach coping strategies than avoidance strategies, t(34) = 2.37, p < 0.05. CONCLUSION: The brothers and sisters generally did not experience many significant problems, however, a minority of the children did experience problems for which they would like help. In addition, they reported joys as well as problems.

Stress and family satisfaction in parents of children with facial port-wine stains.

A cross-sectional survey was employed to assess parenting stress, family satisfaction, and parental concerns and to determine predictors of stress in parents of children with port-wine stains (PWSs). The participants were 46 parents of 24 children receiving treatment with pulsed dye laser photocoagulation for facial PWS at an outpatient dermatology clinic based at a university medical center. Outcome measures used were self-report instruments assessing psychosocial adjustment (Parenting Stress Index, Family Satisfaction Scale, and Parental Concerns Questionnaire). As a group, parents scored in the average range on the stress and family satisfaction measures when compared with a normative sample; five parents (11%) scored in the clinical range for stress. Forty-nine percent of the variance in parenting stress was accounted for by four variables: the child's age (beta = 0.34; p = 0.031), the parents' degree of family satisfaction (beta = -0.27; p = 0.077), the level of parental concern regarding the child's facial PWS (beta = 0.45; p = 0.005), and the parents' satisfaction with staff communication (beta = -0. 51; p = 0.002). The data suggest that while, as a group, parents of children with a facial PWS report to be in the average range for psychological stress, some do not fare as well as others. Factors associated with lower stress include younger children, more family cohesion and adaptation, fewer parental concerns, and greater satisfaction with parent-staff communication. The potential for the development of medical complications and psychological problems over time suggests the need for treatment of the PWS at an early age. Health care providers should be prepared to screen for clinical levels of distress and to refer parents for psychological intervention when needed.

Team approaches to treating children with disabilities: a comparison.

OBJECTIVE: To investigate differences in team functioning between the multidisciplinary and transdisciplinary models when treating children with disabilities. DESIGN: A crossover trial. SETTING: An outpatient educational and rehabilitation program in a rehabilitation institute based at a university medical center. PARTICIPANTS: A population-based sample of 19 rehabilitation specialists and educators. INTERVENTION: Participants attended four team meetings using the multidisciplinary approach and then attended four team meetings using the transdisciplinary approach. OUTCOME MEASURES: Behavioral ratings of team participation (Transdisciplinary Team Rating Scale) and self-report instruments of team development (Team Assessment Questionnaire) and treatment planning and goal development (Staff Perception Questionnaire). RESULTS: Results of t tests confirmed the hypothesis that there was more team member participation during transdisciplinary meetings than during multidisciplinary meetings (p=.027). There were no differences in levels of team development (p=.329); however, staff members favored the transdisciplinary model for treatment planning and goal development (p < .001). CONCLUSION: This study provides evidence of the effectiveness of the transdisciplinary model. Further research is now needed to investigate outcome variables such as rate of success in attaining treatment goals when using this model.