Rare Disease Day 2016 Conference March 9 - 10: Day 1

1. Public Health and Surveillance of
Rare Childhood Conditions
C.O.R.D. - Rare Disease Day Conference 2016 | Ottawa, ON | March 9, 2016
Jay Onysko, Manager
Maternal, Child and Youth Health Unit, Surveillance and Epidemiology Division
Centre for Chronic Disease Prevention
Public Health Agency of Canada

2. Outline
• Public Health Surveillance at the Agency
• Examples of Surveillance of Rare Childhood Conditions:
• Canadian Congenital Anomalies Surveillance System (CCASS)
• Cancer in young people in Canada surveillance system (CYP-C)
• Canadian Paediatric Surveillance Program (CPSP)
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3. • Public health surveillance – a core public health function
• Based on a Population Health Approach
• Public health a shared responsibility among the federal government
and provincial/territorial governments
• Other federal organization (CIHI, CIHR, Statistics Canada, etc…) have
distinct but complementary roles in health research and monitoring
• Agency leadership and coordination role in performing national public
health surveillance activities
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Public Health Surveillance at the Agency

4. The role of public health surveillance:
• Monitor trends in health events (by time, place, person)
• Help estimate current and future impacts
• Support creation of policy and programs
• Facilitate planning
• Evaluate prevention and control measures
• Identify cases for further study
….in order to take action
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Overview of the Agency and Public Health Surveillance

5. Opportunities for public health action:
a life-course approach
Social, Genetic, and Environmental Determinants of Health
Risk Factors (physical activity, diet, smoking, alcohol)
Pre-Disease (hypertension, obesity, pre-diabetes, etc)
Disease (type 2 diabetes, CVD, cancer)
Complications & Disability
Death
Health promotion Primary
prevention
Secondary
prevention
Tertiary
prevention
Prenatal Birth Death

6. Data Analysis
and
Interpretation
Communication
of information
for Action
Data
Collection
Surveillance
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Surveillance Cycle

7. Surveillance Data Sources
• Health administration data - Electronic medical records; hospital
records; physician billing data
• Surveys – Canadian Community Health Survey; Canadian Health
Measures Survey; Canadian Health Survey of Children and Youth (in
development)
• Registries - Systems specifically designed for surveillance
• Other secondary sources (e.g., school administration databases)

8. Canadian Chronic Disease Surveillance System (CCDSS)
Adult Cancer Surveillance Program
Arthritis and Osteoporosis Surveillance Program
Cardiovascular Disease Surveillance Program
Chronic Respiratory Diseases Surveillance Program
Diabetes Surveillance Program
Mental Health -Illness Surveillance Program
Neurological Conditions Surveillance Program
Childhood Obesity Surveillance Program
Cancer in Young People in Canada
Surveillance Program (CYP-C)
Canadian Congenital Anomalies Surveillance
System (CPSS)
Canadian Paediatric Surveillance Program (CPSP)
Child Maltreatment Surveillance
National Autism Spectrum Disorder Surveillance
Program
Injury Surveillance
Centre for
Chronic Disease
Prevention
(CCDP)
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Surveillance Systems/Programs within the
Centre for Chronic Disease Prevention

9. Canadian Congenital Anomalies Surveillance System
(CCASS)
• Congenital anomalies are a leading cause of infant mortality, morbidity and life-long
disability in Canada.
• CCASS reports prevalence of key congenital anomalies, time trends, national and
international comparisons
• Federal / Provincial / Territorial Collaboration:
– Hospital Administrative Data & enhanced systems
• Monitors key categories: (e.g., Down syndrome, neural tube defects, congenital heart
defects, orofacial clefts, limb deficiency defects and gastroschisis)
• Approximately 1 in 25 infants is diagnosed yearly with one or more congenital anomalies
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10. Canadian Congenital Anomalies Surveillance System (CCASS) –
National trends
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11. Canadian Congenital Anomalies Surveillance System (CCASS) –
Policy Links
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KNOWLEDGE MOBILIZATION PUBLIC HEALTH ACTIONS
Monitoring of neural tube defects trends
following folic acid fortification
• Public health strategies, such as folic acid food
fortification and supplementation to prevent neural tube
defects, have proven successful in Canada
• The Agency's Healthy Pregnancy Guide provides
guidance for optimal pregnancy outcomes
Monitoring of microcephaly • Trend analysis has supported Agency direction in
responding to current international Zika virus –
microcephaly trends
• Starting point for enhanced microcephaly surveillance

12. Cancer in Young People in Canada (CYP-C)
Surveillance Program
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• A partnership between the Agency and the C17 Council
• Aims to fill gaps in knowledge and ultimately reduce the burden of childhood
cancer in Canada
• National population-based registry includes all children (age <15) diagnosed
with cancer in Canada from 2001 onward
• Collects detailed information on diagnosis, treatment, outcomes for 5 years
after diagnosis

13. Cancer in Young People in Canada (CYP-C): Data Elements
Demographics Diagnostic Details Time to Treatment Treatment Outcomes
Sex Date of diagnosis
Diagnosis
First health care
professional
contacted
Treatment plan and
start date
Organ transplant
Date of birth ICDO-M, ICDO-T
and ICCC codes
Date first health care
professional
contacted
Treatment completion
details
Complications
Age at diagnosis Stage at diagnosis
Risk
Dates first seen by:
oncologist, surgeon,
and/or specialist
Chemotherapy
and dose
Hospitalizations
Province
postal code
Grade
Chromosomal testing
Metastases and site(s)
Surgery details Relapse
Ethnicity Radiation (intent, type,
site)
Vital status
Hematopoietic stem
cell transplantation
Height and weight

14. Incidence rates for all cancers combined,
by sex, ages 0-14, 1992-2010
ASIR(per1000000)
- Rates are age-standardized to the 1991 Canadian population.
- Analysis by: Surveillance and Epidemiology Division, CCDP, Public Health Agency of Canada
- Data sources: Canadian Vital Statistics Death database at Statistics Canada

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Mortality rates for all cancers combined,
by sex, ages 0-14, 1992-2010
ASMR(per1000000)
- Rates are age-standardized to the 1991 Canadian population.
- Analysis by: Surveillance and Epidemiology Division, CCDP, Public Health Agency of Canada
- Data sources: Canadian Vital Statistics Death database at Statistics Canada

16. Canadian Paediatric Surveillance Program (CPSP)
• Joint project of the Canadian Paediatrics Society (CPS), the Agency and Health Canada
• National collaborative epidemiological research network by and for practitioners
• Survey-based: Gathers data directly from over 2,500 paediatricians and paediatric
subspecialists
• Rare chronic/infectious diseases/conditions and adverse drug reactions (<500 cases per
year)
• Able to collect data in near real-time; can capture relatively detailed case histories
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17. Examples of national surveillance studies currently underway:
• Acute flaccid paralysis (Open / 1996 – 2016)
• Adverse drug reactions ‒ serious and life-threatening (Open / 2004 – 2016)
• Lyme disease (Open / 2014 – 2017)
• Tuberculosis (Open / 2013 – 2016)
• Hypoglycemia in low-risk term newborns (Open / 2014 – 2016)
• Listeria in the newborn and early infancy (Open / 2015 – 2017)
• Sudden unexpected death in epilepsy (Open / 2014 – 2015)
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Canadian Paediatric Surveillance Program (CPSP)

18. KNOWLEDGE MOBILIZATION PUBLIC HEALTH ACTIONS
Vitamin D-deficiency / rickets among
children in Canada (2007)
• Confirmed the importance of reinforcing CPS recommendation
that exclusively breast-fed infants and children receive vitamin
D supplementation.
Necrotizing fasciitis (2011) • Provided evidence supporting guidelines for universal
childhood varicella immunization program.
Non-type 1 diabetes mellitus (2006 &
2016)
• Identified obesity/overweight as predisposing factors in nearly
all cases of type 2 diabetes in children and youth.
• Evidence continues to be used by health promotion and
disease prevention programs in the Agency and by concerned
partners including the CPS and its members
Exposure to detergent packet injuries
survey (2014)
• Attention brought to detergent packets poisonings contributed
to industry changes to product packaging and marketing
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Canadian Paediatric Surveillance Program (CPSP) –
Policy Links

19. Thank you
• http://www.phac-aspc.gc.ca/publicat/cd-mc/mc-ec/index-eng.php
• http://www.cpsp.cps.ca/
• http://infobase.phac-aspc.gc.ca/
jay.onysko@phac-aspc.gc.ca
cypc-ccjc@phac-aspc.gc.ca
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20. Chronic Disease Infobase Data Cubes
• http://infobase.phac-aspc.gc.ca/
• Data Cubes are interactive databases that quickly allow
users to create tables and graphs in their web browser.
‘Open data’ – Infobase Data Cubes