Children with neurofibromatosis type 1: Functioning in the classroom

Neurofibromatosis Type 1 (NF1) is a multisystem disorder, and cognitiveimpairment is its most common complication in childhood (Hyman, Gill, Shores,Steinberg, Joy, Gibikote, & North, 2003). Although patients with NF1 are at risk forsignificant clinical illnesses, most patients are only mildly affected and live healthy andproductive lives (North, 2000). Most research in NF1 to date has been focused ondefining the physical and cognitive deficits based on standardized testing, with littleemphasis on their functional correlates (Gilboa, Rosenblum, Fattal-Valevski, & Josman,2010b).Evidently, student engagement in the classroom reflects, in a large part, selfregulation skills which are influenced by temperament and higher order cognitiveexecutive function processes. In particular, sustained attention, which comprises bothtask-oriented attention and low impulsivity, predicts academic and behavioral adjustment(Pagani, Fitzpatrick, & Parent, 2012).The purpose of the present chapter is to identify predictors associated with schoolparticipation in a sample of children with NF1. A unique feature of this work is theexamination of children's participation across different classroom activity componentssuch as handwriting and cognitive skills, such as attention and executive function. Eachof these classroom activities may require a unique set of functional skills to meet specific demands. Findings and insights presented in this chapter may help clarify the importantfactors supporting effective engagement by children with NF1 in school programs.The chapter describes the functional problems of children with NF1 in the classroomaccording to the International Classification of Functioning, Disability and Health-Children and Youth version (ICF-CY) (WHO, 2007). The chapter includes currentliterature on NF1, using the framework provided by the ICF-CY in highlighting theholistic evaluation and interpretation of disabilities. In the first part it presents the ICFCY(WHO, 2007) conceptual model, defines the term 'ecological validity ' and detailsregarding two ecologically valid assessment tools (Virtual Classroom and functionalquestionnaire).The chapter subsequently describes the functional academic profile of children withNF1 from three points of view: attention profile, academic skills in relation to executiveprofile and handwriting performance. The set of tasks composing each activity provides auseful indication of the factors that may underlie functional participation of children withNF1 in the classroom context. The chapter illustrates the functional profile with a casestudy and we conclude the chapter with suggestions for further research.