Management-new

Educational, Social, & Family Support

CLN2-specific management strategies can be implemented to improve the quality of life of the child and the family

Supporting education and socialization1

Maintaining regular school attendance is extremely valuable to sustain a sense of routine for children and their families and ensure continued integration into the community.

Experts note that children are often able to attend school fairly far into the course of disease with appropriate adaptation, allowing for continued socialization and interaction with peers.

Consider adapting school and home environments to accommodate cognitive and behavioral impairments.

  • Communication between the school and medical professionals is encouraged to ensure they have an appropriate knowledge of CLN2 disease and any management needs the child may have in the classroom

Behavioral strategies may be helpful to both families and children with CLN2 disease.

  • Children with CLN2 disease experience sleep disturbances, which greatly impairs quality of life for them and their families
  • Sleep disturbances are difficult to treat, but certain behavioral strategies are recommended for sleep management and include eliminating caffeine or food with stimulant properties, establishing bedtime with a consistent routine, or use of various sleep aids

Communication support is recommended to be initiated early and assessed frequently to address loss of speech.

  • Developing and maintaining alternative communication methods like symbols and gestures can help prolong interaction and underscores the importance of early diagnosis before cognitive loss occurs

Family support is a key component of CLN2 disease care1

Close interactions between the multidisciplinary team and the family are beneficial to the ongoing care of the child. Resources are available for families to help find comfort during difficult times. Families are encouraged to become active in the CLN2 community and connect to:

Disease education

  • Set expectations for symptom management, disease progression, and the need for adaptive devices as motor function begins to decline

Psychologists/counselors

  • Play an important role in helping families deal with the challenges of disease progression and cope with the loss of function

Social workers

  • Offer support for siblings and grandparents, help inform decision-making by parents to be the best advocates for their child, and coordinate services

Genetic counselors

  • Aid in a deeper understanding of the inheritance of the disease and provide a connection to genetic testing for informed family planning
    • In some areas, prenatal testing for siblings of a confirmed CLN2 child is available

Advocacy groups

  • Provide a platform for patient and family interaction, support, and education

Reference: 1. Williams RE, Adams HR, Blohm M, et al. Expert opinion on the management of CLN2 disease. Poster session presented at: The 12th Annual WORLD Symposium; February – March 2016; San Diego, CA.