“Each person I have met with Batten has given me an invaluable gift, the gift of knowledge of something so rare which I can use to help others”

SeeAbility nurse, Sarah Kenrick, tells the story of her life’s work caring for people with Batten disease.

My first experience of Batten was during my interview for a Junior Sister post at The Children’s Trust, Tadworth.

During the walkabout I was introduced to a young man and was told he had juvenile Batten disease. In the interview I was asked if I had any experience with Batten. “No” was my reply, “but if I am successful, I will do my best to learn and support the children and their families as best I can.” At that point I can honestly say, I really wanted that job.

Welcomed into Anthony’s life

In April 1990 I started working on Cedar Ward, and within a few weeks one child was pivotal in shaping my career. With permission from Kristina Portelli I will share some memories of Anthony.

Anthony had late infantile Batten, CLN2. He was nine when I first started working with him. He came for respite every other week. As a learning disability nurse, I was not familiar with caring for children with such complex needs, but the other nurses I worked with, and his wonderful mum Kristina, soon had me licked into shape.

Back then we were lucky enough to be able to provide outreach care, which was a new concept, so I was able to visit the family home and go on holidays with them. Over four years we developed a strong rapport, and when Anthony became very ill I was able to go home with the family and help provide care and support at the end of his life.

I feel immensely privileged to have been welcomed into Anthony’s life. Kristina taught me so much, not only about Anthony’s care needs, but also, through her gentle, brave and loving approach she helped me understand how important it is to be inventive, build memories and enjoy every day.

Through my work with Anthony I was able to speak at conferences in Spain, Jersey and New Zealand on the topic of the rights of children with complex disabilities.

Heather House

Over the years I have worked predominantly with older children and adults with juvenile Batten CLN3, and in December 1998 went to work with SeeAbility as manager of Heather House, a new service specifically designed for adults with CLN3.

Fast forward 21 years and, following a move to Aberdeenshire, I am now six months into my post as SeeAbility’s outreach liaison nurse for CLN3. I have worked with over 60 children and adults with Batten, and have been able to support many of them through to the end of their lives.

I have worked with some truly inspirational doctors, nurses, therapists, carers and families, and I have been fortunate to get to know some of the scientists working to understand and find treatment pathways for the disease.

At Heather House, our aims were, and still are, quite simple; we listen to each person, support and respect their needs for their own routines, enable each person to feel safe and valued and help each person to take as much control as they can each day.

Understanding, knowledge and helping others

I have learned that for any care giver, we have to understand the disease, what it does to the cell, to the brain and to the body, how it manifests and changes. We also have to understand and respect the person, the personality, and the family.

At Heather House, we have collected data around epilepsy, medications and basic weekly observations of temperature, respiratory rate and pulse. From this we have been able to develop therapeutic support programmes to enable people to have a more gentle progression through the disease process. I have also developed knowledge around specific behaviours that are seen as part of the disease and how to support the person to manage and stay in control, this aspect forms much of the work I do now.

I feel indebted to the people I have supported, and their families. Each person I have met with Batten has given me a gift that is invaluable, the gift of knowledge about something so rare which I can use to help others, I think of this as my tool box for Batten which I carry with me always.