Oral Presentation 5th International Symposium on Phaeochromocytoma and Paraganglioma 2017

A storybook to help young children understand being at risk of hereditary phaeochromocytoma and paraganglioma syndrome.       (#40)

Georgina M Schlub 1 , Ashely Crook 2 , Jane Fleming 1 , Kristine Barlow-Stewart 1 , Rosie O'Shea 1 , Kathy Tucker 3 , Sian Greening 4
  1. Genetic Medicine, Sydney Medical School - Northern, University of Sydney, St Leonards, NSW, Australia
  2. Department of Clinical Genetics, Royal North Shore Hospital, St Leonards, NSW, Australia
  3. Hereditary Cancer Clinic, Prince of Wales Hospital, Randwick, NSW, Australia
  4. Hereditary Cancer Clinic, Illawara Cancer Care Centre, Wollongong, NSW, Australia

Communication with children about hereditary conditions is a challenging process for which parents often feel ill equipped. Yet, good communication strategies and psycho-education are a leading determinant of adaptation and resilience in children. In the case of hereditary phaeochromocytoma and paraganglioma syndrome (HPPS), which results in rare neuroendocrine tumors that have a high degree of genetic determinism, malignancy and an early age of onset (>5 years), genetic testing and subsequent surveillance in at-risk young children is recommended. Given the exceptionally young age at which this is done, great effort is needed to provide at-risk children and their parents with appropriate genetic counselling, support and communication strategies. Our aim has been to develop and evaluate a psycho-educational resource for young children at risk of HPPS for which no current resource exists despite the high demand. Using the practice of bibliotherapy – where stories are delivered prescriptively, we have created a storybook, for children aged 5-10 years who are at risk of HPPS. It has been developed based on what is currently known about children’s developmental comprehension of heredity and disease transmission and aspires to bring them to a place of insight and comfort about what it means to be at risk of HPPS. Semi-structured qualitative interviews will be conducted and analysed thematically using a general inductive approach to ascertain the experience of ~15 parents reading this book to their children, including parents’ recommendations for its improvement. We anticipate that by incorporating parents’ experience, this resource, which is not only cost effective and easy to deploy, will have pyscho-educational merit that may assist parents in the process of communication with their children about HPPS.