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Sharon travelled to Sweden to attend an Educational conference for Battens Disease. Sharon was partly supported by funds granted to LDNZ by Lottery Ministers Discretionary Fund. Her report follows.

Report for LDNZ by Sharon Noble

There were a lot of positives things coming out of this conference, especially in terms of the information gained and networking with other Batten families. It was a bonus to attend a conference that focussed solely on Batten Disease.

The main objective of the conference was to examine educational approaches for bringing optimal independence to students with Batten Disease. It addressed the need of consensus in the understanding of the educational needs and methods used in working with children and adolescents (and in some cases Adults) with Batten Disease.

There were also good opportunities to exchange ideas and experiences for development in the field of education

A standing ovation was given to Hjordis Gustafson after her presentation “The history of the education for children and adolescents with Batten Disease in Sweden”. The dedication and passion she has had over the many years of caring for the children and their families was more than obvious. She had many wise recommendations and experiences to share. The photo power point she showed at the end of the presentation was heart felt and had the room in tears. She is a valuable resource for all the educational centres, medical professionals and parents.

The presentations of research and use of technical solutions for use of children with various disabilities was interesting. Especially the talking cane and the lap top computers with shell program by SRRIDA Communication Centre.

Jonathan Cooper, Sarah Mole, and Paul Uvebrant gave clear descriptions of research being done in the area of Batten Disease. Gene therapy and enzyme therapy, although useful possibilities are being explored for other Lysosomal storage disorders, these do not appear to be useful in the treatment of Juvenile Batten disease.

Real progress towards a therapy for Juvenile Batten disease is frustratingly slow, but they have learnt some important lessons about where and when the brain is affected. There is some thought that the brain of Juvenile Batten Disease patients is leaking and that there may be a problem with the auto antibodies. Jonathan thought by combining this type of information with an understanding of precisely how brain cells are impacted by the disease will be the key for understanding how Juvenile Batten Disease may finally be treated successfully. It’s just a matter of time. Unfortunately for most of our children time is not a friend!

A number of parents spoke on the affect the disease had on their child and what treatments or strategies had been best suited to the child and how they have coped with living with the disease. Moving towards dependence rather than independence. As expected, because each child, their disease progress, and family was unique to them, ideas of exactly what was done and when they did it varied, but one thing was very clear at some point all the child began to follow the same pattern and path.

Useful contacts had been made with Heather House, Jyvaskylan and Bartimeus representatives. These are special homes or units set up for the education and care for children and young adults with Juvenile Batten disease. They offer support to the child, family, peers and professionals during the total duration of the decline. The key points they stressed were:

• Continuity of care and support
• Protection, security and consistency
• A multidisciplinary approach
• Comfort – bed, toileting, feeding/fluid intake, pain etc
• Need for participation, involvement and a sense of belonging
• Understanding of the disease (pain, eating difficulties, infection, loss of mobility, loss of vision, loss of control, seizures, hallucinations, psychosis and parkinsonian symptoms etc)
• Understanding of who this child is and was (talk to people who know the child, memory books/boxes)
• Company and companionship – social interaction, memory books
• Communication (looking for subtle gesture in the later stages) and honesty
• Aspirations – have some goals – no matter how small. Thinking forward and planning
• Supporting the changes to preserve the self-esteem of the individual
• And always remember “what does this person want and need?”

The ideas shared were practical and identified the need for a person centred approach to bring about empowerment to the individual.

I would like to thank LDNZ and the Ministers Discretionary fund for their financial support towards the cost of travelling to this very special conference.

I would also like to thank Denise Laraman for the tremendous effort she went to, to organise the Comedy Night as a fund raising event and for both Mike King and Andrew Clay for the generous donation of their time and talent. I will provide the LDNZ with the resource booklets I have collected from the conference. This should be of value to other Batten’s families and for contacts for LDNZ to use when helping these families. I will also send relevant information to RFBNZ, the Vision parent support group, Parent to Parent, Parent Resource centre and the Wilson Centre in Auckland.

Thank you once again,

Sharon Noble.
(Parent of a Juvenile Batten Disease child – Antoniya Noble)

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