The Case for a Mandatory ALS Registry

There are a lot of scientific questions that we do not have the answers to yet; answers that would allow scientists and healthcare professionals to make prevention recommendations and tailor treatments.  Why do some people get symptom onset in their 30s and others in their 50s and 60s?  Why are some people with ALS such as Stephen Hawking able to live with the disease for over 20 years when most will live only 2 to 5 years after diagnosis?  Why do some people get bulbar onset ALS, which holds a poorer prognosis, and others get limb onset ALS?  Is ALS purely genetic or could there possibly be environmental triggers such as insufficient Vitamin D intake or inability to properly metabolize Vitamin D, psychological stress, viruses and bacteria, or chemicals such as hormone disruptors?  Experts believe there are likely multiple pathways to developing ALS.

Incomplete Information in Existing ALS Registry

The only way to really answer these questions is through lab-based research, observational clinical studies and epidemiological investigations.  Money from the Ice Bucket Challenge is being poured into lab-based research, but will take several months to years to see the results.  Ongoing genetic studies based on blood, spinal fluid, and tissues are identifying and investigating the role of familial linked and other genes.  However, there is no mandatory ALS registry like there is for cancer and infectious diseases in the United States, with the exception of the State of Massachusetts.   

In 2008, Congress enacted the ALS Registry Act (PL 110-373) which charged the Centers for Disease Control and Prevention (CDC) with developing and managing the registry.  It consists of existing administrative data maintained by Medicare, Medicaid, the Veterans Health Administration and the Veterans Benefits Administration, coupled with self-reported data collected via a secure, web portal that was launched on October 19, 2010, to identify additional people who would not be captured by the administrative data.  People registering on the web portal are asked screening questions to verify their ALS diagnosis.  They are also asked information on possible risk factors such as age, race, education, income, work and military history, physical activity, alcohol and tobacco use, and family history of neurodegenerative diseases.  In addition, the Agency for Toxic Substances and Disease Registry, a branch of CDC, funds state and metropolitan registries to obtain more reliable and timely data, including demographic information such as age, sex and race and compares this data with the national registry to determine if certain groups are likely underrepresented in the U.S. statistics.  Areas that have received funding include Florida, New Jersey, and Texas State, and the metropolitan areas of Atlanta, Chicago, Baltimore, Detroit, Las Vegas, Los Angeles, Philadelphia and San Francisco. 

The current ALS Registry is a fairly rigorous registry, but certainly does not capture everyone with possible, probable, or diagnosed ALS because it is not a Census.  That is, with the exception of the State of Massachusetts (Section 26 of Chapter 140 of the Massachusetts Acts of 2003), ALS is not a mandatory notifiable disease.  Secondly, the registry cannot estimate ALS incidence (all new cases of ALS) due to the fact that 68% of the cases in the large administrative data do not have a date of diagnosis.  Third, the online ALS Registry is self-report data, which means anyone could register him or herself as having ALS.  A list of screener questions are used to try to weed out those with suspected or perceived ALS from those who actually have ALS.  However there is no good method for accurately verifying someone in this dataset has actually received a doctor’s diagnosis.    

Diseases that are designated as law binding notifiable diseases such as many infectious diseases require doctors and other healthcare providers and labs to report to the authoritative state agency any case that meets the definition for that disease ensuring almost complete capture of everyone diagnosed with ALS.  This would benefit the ALS Community because it would allow public health agencies to more accurately assess incidence (all new cases of ALS) and prevalence (overall the number of people with ALS at any given point in time).  The current registry likely underestimates the number of people with ALS, which means under times of financial strain they may not receive full priority for research dollars and resources.  It also means that agencies will not be able to detect ALS clusters and possible causal links or priority areas to focus limited resources.        
 
If you want ALS to be a national priority, tell your legislators that you want ALS to be a nationally notifiable disease. 

Reference

Mehta et al (2014).  Prevalence of Amyotrophic Lateral Sclerosis-United States, 2010-2011.  CDC MMWR Surveillance Summary, 63(SS07): 1-14.  http://www.cdc.gov/mmwr/preview/mmwrhtml/ss6307a1.htm