1. The ADSID project A clinical research database of dementia in older adults with Down Syndrome and Intellectual Disabilities Dr Amanda Sinai, Clinical Research Associate, UCL Dr Andre Strydom, Senior Lecturer, UCL Dr Ken Courtenay, Consultant Psychiatrist, BEH MHT

2. The ADSID project Background Objectives Hypothesis Methods Preliminary Results Discussion

3. The ADSID project - Background Dementia is more common in older adults with intellectual disabilities Alzheimer’s disease may affect up to 50% of adults with Down syndrome over the age of 50 Little is known about the course of dementia in these populations except that it is a strong predictor of mortality in adults with Down syndrome Many aspects of the management of dementia in adults with intellectual disabilities remain uncertain

4. The ADSID project - Objectives A dementia special interest group of interested health practitioners in London was established in 2009 It has led to a research collaboration to form a clinical registry which is aimed at: –studying the natural history and care needs associated with dementia in people with Down syndrome and others with intellectual disabilities –studying the outcome of treatment in routine clinical settings

5. The ADSID project - Methods We developed a common dataset, database, and security protocols We addressed data protection issues with support from the National Information Governance Board (NIGB) The initial focus has been on retrospective data from clinical memory assessments in people with Down syndrome Our preliminary analyses are focused on basic demographics and survival

6. The ADSID project - Hypothesis We hypothesised that prognosis of Alzheimer’s disease in people with Down syndrome will be poor when compared to the general population

7. The ADSID project – Preliminary Results The registry now contains data on approximately 300 people with Down syndrome Approximately 90 people have been diagnosed with dementia 54.8 years is the average age of diagnosis 33% of those with dementia have since died The average age of death for those with dementia is 59.8 years (i.e. survival is 5 years)

8. Preliminary Results – Survival of adults with Down syndrome from point of diagnosis with dementia 0.00 0.25 0.50 0.75 1.00 051015 analysis time (years) Kaplan-Meier survival estimate

9. 0.00 0.25 0.50 0.75 1.00 051015 analysis time (years) gender = malegender = female Kaplan-Meier survival estimates Preliminary Results – Survival of adults with Down syndrome from point of diagnosis with dementia, gender

10. Preliminary Results – Survival of older adults with Down syndrome and dementia Preliminary analyses show that: Age was significantly associated with survival (p=0.007). There is approximately 9% increase in the hazard of dying for each 1 year increase in age. The confidence interval for age was (1.02, 1.15), which means that the increase in the hazard of dying could be as low as 2% or as high as 15%. These results are based on the univariate analysis. We have not found any significant difference in survival for gender, level of learning disability, severity of dementia at diagnosis or premorbid epilepsy

11. The ADSID project – Discussion In the general population, survival from time of diagnosis may vary from 3 to 10 years 1 Shortened survival in the general population is associated with increasing age, male gender and disease severity 2 Our data reflects some of the findings seen in the general population, although the age at diagnosis and age at death are much lower in people with Down syndrome and dementia At present, our data may have limitations related to power – we will continue to collect and analyse data 1.Zanetti O, Solerte SB, Cantoni F. Life expectancy in Alzheimer’s Disease (AD) (2009) 2.Paradise M, Walker Z, Cooper C et al. Prediction of survival in Alzheimer’s Disease – The LASER-AD longitudinal study (2009)

12. The ADSID project – Discussion We hope that the registry will be a valuable resource for both exploratory and explanatory research in the future We plan to further analyse: –survival and associated factors –medication use Once we have suitable power, we plan to make more direct comparisons with survival rates in the general population We also plan to explore diagnostic issues, for example effect of different diagnostic criteria

13. Acknowledgements This study is funded by the Baily Thomas Charitable Fund. Many thanks also to: Camino Fernandez, Sharon Jakobowitz and Hannah Boardman, Research Assistants, UCL Khadija Rantell, Research Associate, Biostatistics Group, UCL Members of the London Dementia in Intellectual Disabilities special interest group Involved staff at the various community learning disabilities teams in London and nationwide

14. Any questions? a.strydom@ucl.ac.uk