Batten disease refers to a group of childhood epilepsy disorders called neuronal ceroid lipofuscinoses (NCLs), which are caused by a buildup of fat and protein waste in nerve cells. This results in convulsions, muscle tremors, and developmental delays or regression.

Children with Batten disease often have difficulty learning to walk and talk. Once the symptoms of the disease have developed, many children will regress (lose) developmental progress such as speech or movement. Moreover, many children with Batten disease have difficulty swallowing, especially in the late stages of the disease. Speech therapy is one of the treatments that can be useful in developing and maintaining these skills and children’s quality of life.

What is speech therapy?

Speech therapy involves working with a therapist to develop the muscles of the face and tongue to support speech, language, social and cognitive communication, and swallowing.

For children, speech therapy occurs in the context of play. Therapists work with children during age-appropriate play activities to encourage language development and teach them games or exercises to develop the muscles needed for both speech and swallowing.

What parents and caregivers can do to help

It is important for parents and caregivers to reinforce the lessons and exercises learned during therapy sessions. Parents, teachers, and therapists collaborate as a team to both assess the child, write goals, and implement interventions. Implementation of these exercises and goals should be embedded within the child’s natural daily activities.

Music and speech therapy

A three-year study that began in 2016 and is run by Chiltern Music Therapy and the University of Roehampton in the United Kingdom, is working with 12 youngsters age 3-18 with Batten disease.The participants are receiving weekly music therapy to find out exactly how music can be used to support communication, language, and expression of thoughts and feelings for these children.

The study also is measuring how music can be used to maintain or connect the children with short- and long-term memories, as well as helping them orient to time and place, as confusion inevitably sets in as the disease progresses. Most importantly, the study will find out how music can help maintain a sense of well-being throughout the course of the disease.

The study is being conducted in collaboration with the Baily Thomas Foundation (a charity that aids research into learning disabilities), Amber Trust (a music charity for blind children), the Batten Disease Family Association (BDFA), and the U.K. National Health Service.

Early results showed a promising improvement in language skills and responsiveness in children, and researchers are planning to continue the study incorporating musical elements such as rhythmic speaking, singing, and rhyme to speech therapy in order to encourage consistent language production.

Results of this study will be used to develop handbooks and resources for families caring for children with Batten disease.

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