The Batten Disease Association is an organisation dedicated to improving the well-being of patients and families suffering from Batten disease, a severe and progressive inherited neurological disorder that primarily affects children and young persons. It is one of a group of about 50 disorders collectively known as lysosomal storage disorders. Batten disease primarily affects brain function, leading to the loss of learned skills such as walking, talking and feeding, behavioral problems such as aggression, and severe sleep deprivation. Families with a person affected by Batten Disease therefore require significant support……
· The Australian Chapter of the BDSRA was formed in 1995 by parents of children affected by Batten Disease. Seeing the tremendous need for unified entity that would work toward important goals and recognising, as well, the need for support for family, friends and carers;
· This Association is principally operated by Volunteers as no Government funding is received;
· The Association is an essential resource for the Batten community, and due to the scope and complexity of the work it undertakes, steps were taken to employ a part-time coordinator to meet the equally essential administrative requirements of a National based Charity;
· At this time, all persons enduring this disease in Australia are represented by an Australian Register.
The Association is largely made up of families with a affected children, teenagers and some adults (in some cases may have two). Its members therefore have a first-hand understanding of the despair and sense of loss that parents and families face in coming to terms and living with such a serious diagnosis, knowing what practical information and ongoing support families require, providing assistance to access support through a maze of patient management issues, and providing the gentle guidance that is essential at a very difficult time.
The families braving their child or young person’s degeneration and the ones whom have lost their loved one(s):
v Battens Disease is all consuming. The care needed by our affected children as they slowly lose their just-acquired life, skills and abilities and the love and attention that naturally arises, uses up so much of the energy of a Battens family.
v Helping families to overcome their fears and uncertainties; to hopefully find an acceptance and ultimately to be grateful for the positives. Sometimes it is knowledge that is needed, sometimes emotional support but most often it is simply that families know that there is someone here, someone who has been through all the rollercoaster experiences of caring for an affected family member.
v This degree of support requires 24 hour available support by the Co-ordinator and a team of volunteers.
v Parents of family members with Batten Disease are faced with many difficult decisions during the degeneration of their child/young person due to the fatal nature of the disease. Many changes occur from diagnosis to their final passing, during which a high degree of emotional and physical strain is placed on the main care providers.
v A lack of knowledge and assistance prevails in areas such as pain, behavioural challenges and loss of abilities such as vision and mobility. Every parent and its community endures isolation and breakdowns in existing relationships.
Overview of Activities: The Association has gained interest from families and communities around Australia to support its mission and initiatives for support have been carried out such as new publications in the area of schooling and physiotherapy for these children. As Batten disease is a relatively rare disorder in the community, it is essential that an Association such as this exists for affected families in all States to provide the network of understanding, care and practical help that families need to be able to physically, emotionally and financially cope with the demands of an affected child [in some cases, families have more than one affected child]. The Association performs a critical activity for the Batten disease communities throughout Australia and provides the following programs within its membership:
o Equipment Alert program;
o Publications Library;
o Newly Bereaved Information;
o Newsletter production;
o Website and Email updates - To date, research has not led to a cure for the brain deterioration in children with Batten disease. In the interim, parents and families are keen to know and learn of the latest scientific developments in the field;
o State-wide Information resource and supply;
o Newly Diagnosed Family information packages;
o Planning for State Family Meetings. An educational opportunity rarely awarded to families affected by this disease.
o 24 hour support: Ongoing phone and email contact, not only with the family but their extended family, friends and even their child’s teacher – in remote towns to anywhere in Australia.
Allocation of Funds:
v Funds are represented by donations, fundraiser proceeds, merchandise sales, interest, memberships and tin collections;
v Funds that are received by the BDSRA are distributed to:
o Support programs mentioned above. This also extends to the families’ specialists such as medical practitioners, teachers, physiotherapists and relatives;
o Administrative and running costs, includes a Manager and support person who is on call to meet needs and ensure the fulfillment of legislative requirements and awareness;
o Pledged monies to support the work of scientists on various research projects as approved by the BDSRA (USA) Scientific Board and the Australian Chapter.
V L Anderson.
Date: 1st March 2013