State and Metropolitan-Area Based Amyotrophic Lateral Sclerosis (ALS) Surveillance

1. State and Metropolitan-Area Based Amyotrophic Lateral Sclerosis (ALS) Surveillance Paul English Susan Paulukonis Jhaqueline Valle March 21, 2012

2. Partnership with ATSDR & McKing Consulting Corporation • ATSDR funded • Implement a metropolitan area-based ALS surveillance project in Los Angeles and the San Francisco Bay Area(18month project)

3. History of ALS

4. What is ALS? • Amyotrophic Lateral Sclerosis (ALS), often called ‘Lou Gehrig’s disease.’ • Motor neuron disease, progressive loss of physical functioning • First identified in 1869 by French neurologist Jean-Martin Charcot • Cause not known in most cases, • Small proportion (10%) of cases are genetically linked • Environmental and occupational associations are being investigated

5. What is ALS? • Incidence: 1 - 3 per 100,000 person-years in industrialized nations (sources differ) • Typical age of onset: 40 to 60 years • Average life expectancy from time of diagnosis: median is 24 months. 90% of cases die within 5 to 8 years of diagnosis

6. Why do in context of tracking? • Building surveillance capacity/experience with registry methodologies • Opportunity to track historic addresses from participants with new methods • Will be able to keep data; use for future case/control studies • Aligns with national funding goals for Tracking and ALS surveillance

7. Only 5-10% of ALS due to inherited genetic mutations Trauma Increased risk in Italian soccer players Occupational Exposure to Pesticides (RR=3.6, 95% C.I. 1.2, 10.5) Bonvicini 2010 ALS and Environment

8. ALS and Environment • Guam: 100 X frequency ALS • BMAA amino acid found in seeds of the cycad Cycasmicronesica in food chain (fruit bat) • BMAA is found in brain tissue of ALS victims • BMAA produced by Cyanobacteria • BMAA found in contaminated shellfish, drinking water • Cyanobacterial blooms (blue-green algae) are increasing due to climate change

9. National ALS Surveillance/Registry

10. Early ALS Surveillance • Agency for Toxic Substances and Disease Registry (ATSDR) funded IL, MA, MO, OH, OR, TX • determine the prevalence of ALS in defined geographic areas (2002-2006) • Inpatient data, outpatient data, self-referrals, nursing home admissions and death certificates • Case ascertainment relatively low • Medical records not always accessible • potential source of bias

11. ALS Registry • Conclusion: create a national ALS registry to more accurately determine incidence/prevalence • National ALS Registry mandated by Congress. • Registry identifies cases from two data sources: • administrative data (CMS, VHA, and VBA) • self-registration through a secure web portal • Non-traditional approach to be validated with more conventional surveillance technique • case reports to health departments

12. State and Metropolitan-Area Surveillance Projects • Objectives: • Use project data to evaluate the completeness of ATSDR’s National ALS Registry • Obtain reliable and timely information on the incidence and prevalence of ALS and better describe the demographic characteristics (age, race, sex, and geographic location)

13. Participating Sites

14. Site Selection • Sites selected to over-represent ethnic and racial minorities • Sites of sufficient size to contribute >100 cases each to the surveillance project • Los Angeles County expected cases: 785 • San Francisco Bay Area expected cases: 360

15. National ALS Registry vs. State/Metro ALS Surveillance

16. Los Angeles & San Francisco Bay Area ALS Surveillance Project

17. Project Implementation • California counties searched for cases: • Los Angeles and • San Francisco Bay Area • Alameda • Contra Costa • San Francisco • San Mateo • Solano

18. Project Implementation • Outreach • Compensation/Training • Data Collection • Case Ascertainment • Case Re-abstraction • Case Verification • Missing Cases • Data Analysis/Final Report

19. Project Implementation Outreach • Approach all neurologists in target counties and surrounding areas. • Mailings, Calls, Office Visits, Professional Meetings, etc.. • Ongoing Compensation/Training • Participating neurology practices are compensated per case • Training provided to staff at participating practices as needed

20. Public Health Surveillance vs. Research • Doctors can release private health information with identifiers without obtaining permission from the person with ALS • Public health surveillance is a permitted but not required release under HIPAA • A signed consent form and a signed HIPAA authorization form are not needed for this project

21. Project Implementation Data Collection-Case Ascertainment • Collection of data on diagnosed/treated cases of ALS, 2009-2011 • Office staff abstract medical records to provide data Data Collection - Re-Abstraction • Subset of records submitted are reviewed/validated for quality check Data Collection - Validation • Validated records are reviewed by project neurologist to assure correct diagnosis Missing Cases • Final records are compared with hospital discharge and vital records (death certificate) data to find missed cases

22. Project Implementation Analysis • Descriptive analysis of cases (age, sex, race/ethnicity, birthplace, etc.) Final Report • Methods used • Problems/Solutions • Summary of data analysis

23. Timeline • January, 2012: Project period begins • May, 2012: Begin data collection • April, 2013: Data collection complete • June, 2013: Analysis of data • July, 2013: Final report/End of project

24. Organizational Endorsement Current Endorsements • ATSDR • ALS Association (ALSA) National • ALSA Golden West Chapter Approaching for Endorsement: • American Academy of Neurology • CA Neurological Association • San Francisco Neurological Society • Los Angeles Neurological Society :

25. Questions for TIAG • Thoughts on use of Metro Area ALS Surveillance data? Funding for additional work? • Contacts in Los Angeles and/or SF Bay Area neurology communities?