JNCL & education

Juvenile Neuronal Ceroid-Lipofuscinosis (Batten disease) and Education Research

Between 2014 and 2018 WESC Foundation were involved in a collaborative Erasmus+ funded project coordinated by Statped Heimdal (Norway) to investigate education from a lifelong perspective for students with Juvenile Neuronal Ceroid-Lipofuscinosis (JNCL). JNCL is one of a group of medical conditions that have been collectively referred to as Batten Disease. It is a genetic life-limiting neurodegenerative condition that is characterised by a progressive loss of vision, cognition, and movement control. Due to the relative rarity of the condition most health, social care, and education professionals do not have experience in supporting young people with JNCL and their families. The aim of the project was to draw on the specialist knowledge and experience of organisations and families across Europe to compose a non-medical textbook with a focus on JNCL and education.

About this project

The project was divided into three phases. The first phase involved researching previous studies and their findings. The second phase involved collecting data from professionals and families using comprehensive surveys and interviews. The third phase involved compiling the results from the previous studies and from the surveys into a textbook and a series of educational forms that could be used for assessment, planning, and intervention. The textbook contained author contributions from twenty organisations and seven countries (England, Scotland, USA, Norway, Denmark, Finland, and Germany) representing the educational, health, and university sectors, as well as national peer organisations.

Outcomes

One of the main outcomes of this study was the observation that individuals with JNCL experience the impact of their condition very differently. While there are average ages at which declines might be expected, they do vary greatly from individual to individual. Decline in visual ability was observed in the project sample between the ages of 6 and 9, and typically lead to blindness in the preteen years or adolescence. Interviews with family members indicated that individuals benefited from learning braille (or Moon type) early, before major declines in cognitive ability had occurred. Approximately half of the project sample began to have difficulty with speech by the age of 13, and over half had major difficulties with speech or no understandable speech by the age of 19. In contrast, language comprehension was often maintained for a comparatively longer period indicating that training in Alternative and Augmentative Communication methods could ameliorate communication as speech declines.

Looking ahead to the future

It is not currently possible to stop the progression of JNCL but it is possible to compensate for the effects of some declines with pedagogical interventions. Targeted interventions based on windows of opportunity for learning could improve an individual’s quality of life and social participation as the condition progresses.