- Post date:
- Friday, 3 November 2017
Rebecca Atkinson, a neurologic music therapist and researcher, discusses the latest study that’s aiming to answer this question.
Also known as childhood dementia, Batten disease
is a rare genetic disorder that is characterised by the degeneration of the nervous system and results in early death in children and young people. It currently affects around 100 to 150 children and young people in the UK and there is no known cure.
Like other progressive diseases, young patients’ neurological impairments worsen over time. Symptoms include:
- loss of vision, hallucinations and eventual blindness
- severe epileptic seizures
- the decline of speech, language and swallowing abilities, and
- the deterioration of fine and gross motor skills that eventually result in complete loss of mobility.
Since the disease was identified formally in 1903, there have been over 400 mutations which have resulted in 13 variants of the condition, each one presenting differences in terms of the progression for the children and young people affected.
Neurologic music therapy
One of the most distressing elements of Batten disease and other similar conditions is the emotional anguish that people affected suffer. The most important and effective support for this is through palliative care and therapy – in particular, the use of music therapy. Recent evidence suggests that just the simple exercise of playing the patient’s favourite music may help regulate emotions and offer relief at times of psychological crisis (Bills, et al., 1998).
Neurologic music therapy (NMT) is the application of music to cognitive, sensory and motor dysfunctions, which creates positive changes in brain function and behaviour (Thaut, 1999).
Currently, there has been no record of research in this specialised area that clinically measures the effects of music on memory, speech and emotional wellbeing for children with Batten disease. However, numerous studies into the effects of music and neurological conditions have illustrated successful results (Zatorre, 2013; Magee, 2017), and accounts from parents, teachers and therapists, suggest that music may have a crucial role in the lives of people with the disease (Atkinson and Ockelford, 2017).
From a recent Europe-wide study (Elmerskog, [in press]), parents and staff gave their feedback about what was important in the lives of their children affected by Batten disease. Remarkably, music was said to have significant importance, with nine out of 10 parents saying that music and music therapy had (or once had) a high or very high impact on their child’s life. Parents and staff also illustrated how music could:
- aid communication when words alone had been lost
- enable memories to be accessed, and
- help children maintain a sense of wellbeing.
Music in Neurodegeneration study
A three-year study run by Chiltern Music Therapy
and the University of Roehampton
began in 2016, working with 12 children and young people with Batten disease in the UK (aged between three and 18 years old). Named the Music in Neurodegeneration study (MIND), the participants are receiving weekly music therapy to find out exactly how music can be used for supporting communication, language and expression of thoughts and feelings for these children.
The study is also measuring how music can be used to maintain or connect the children with long and short-term memories, as well as helping them to orient to time and place, as confusion inevitably sets in. More importantly, as the children become more confused and experience hallucinations, the study will find out how music can help maintain a sense of wellbeing as the disease progresses.
Primary results from the first 12 months show that the participants displayed higher levels of reactive engagement (whereby the child responds to music interaction initiated by someone else) compared with proactive engagement (whereby the child initiates musical interactions independently). This is in line with our predictions that as children lose the ability to move and speak, their ability to initiate engagement decreases.
However, even when speech has declined or been lost altogether, singing remains remarkably intact. Even more astounding is the ability for these young people to remember words from songs in their entirety, even in cases when their memory has severely deteriorated.
It is not known for certain why this affect occurs, but previous research into the effect of music for adult dementia have highlighted unique changes in brain responsivity when engaging in music-based activity (Brotons and Koger, 2000; Woods et al., 2007).
The study has also discovered existing strategies which are being used to promote memories and aspects of a child’s identity. For example, by creating tactile memory books that are multisensory in nature, children can explore and revisit objects, braille and sound bites associated with their favourite music, holidays or outings, encouraging maintenance of memory for longer.
Sound symbols are also used in schools and education settings to encourage children to decipher their environment using sound-making objects. This involves using the sounds of instruments (i.e. chimes or drums) or excerpts of favourite songs to encourage choice making in music sessions, or more abstract sounds that represent routines, choices or activities (i.e. a football whistle to represent outdoor play).
Activities will be introduced over the next two years, including rhythmic speaking, singing and rhyme, in order to encourage consistent language production. For younger children in the study, a bank of “micro songs” will be taught to help them remember key phrases and build a language framework over time using melody and rhythm.
Other music techniques, technology devices and activities will also be introduced over the coming months to gauge the effectiveness of these on areas of:
- cognition, awareness and choice-making
- communication, speech, and the ability to indicate preferences, and
- overall wellbeing.
Findings from the research will inform the creation of handbooks and online music resources, which will be disseminated throughout schools, children’s centres and homes in the UK. We hope that these will provide practical day-to-day solutions to families and improve the quality of life of children affected by Batten disease both now and in the future.
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