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Quality of Life in Families of Children with Batten Disease

  • Research type

    Research Study

  • Full title

    An Exploration of Perceived Quality of Life in Families and Carers of Children with Batten Disease at Different Stages of the Condition.



  • Contact name

    Ruth Williams

  • Contact email

  • Sponsor organisation

    Guy's & St Thomas' Foundation NHS Trust, R&D Department

  • Duration of Study in the UK

    0 years, 7 months, 31 days

  • Research summary

    Batten disease or The Neuronal ceroid lipofuscinoses(NCLs)are a group of neurodegenerative diseases which occur mainly in childhood. There is no cure, and patient care is focused on symptom management, which is often challenging. Therefore, Health Related Quality of Life (HR-QoL) considerations and measures are essential in revealing the factors that are important to families and carers with an affected child. There is a very limited body of research looking at what aspects of life are perceived as most valued to families and carers of children living with Batten disease.

    This project aims to explore perceived quality of life (QoL) in families and carers of children with a diagnosis of CLN3, Juvenile Batten disease, at different stages of the condition.

    Participants will be parents, adult siblings or carers of children with a diagnosis of CLN3 Juvenile Batten disease. They will be recruited from the Evelina London Children's Hospital Batten disease clinic and via the Batten Disease Family Association (BDFA) Charity.

    Participants will take part in a focus group to explore their perceptions of QoL in relation to living with a child with Batten Disease.

    It is hoped that such an investigation will allow for the development of a descriptive understanding of what QoL means to families and carers over the degenerative course of the condition. This will also provide a valuable foundation for healthcare professionals working with such families in guiding clinical conversations around palliative care considerations as well as helping them to appreciate the developing challenges and values of families and carers at different stages of the disease. A secondary outcome will be the development of a draft proposal for further research looking at the development of a population-specific, dynamic measure of QoL.

  • REC name

    London - Camden & Kings Cross Research Ethics Committee

  • REC reference


  • Date of REC Opinion

    11 Jul 2016

  • REC opinion

    Favourable Opinion