Friday, January 8, 2010

Eight states still do not mandate reporting of CJD cases to public health authorities

Neuropathologists are obligated to keep generally up to date on Creutzfeldt-Jakob disease (CJD) research as they are called upon to perform autopsies on patients whose dementia may have been caused by a prion disease. The most overlooked players in the CJD research arena are patient advocates. Many patient advocates are fiercely motivated, often having been inspired to act by the CJD-related death of a loved one. One such advocate is Theresa Matthews (pictured on a 2006 lobbying visit to Washington with her teenage daughter Mary and Illinois Senator Durbin and then Senator Obama). Patient advocates often raise worthy questions about issues surrounding CJD research. One such question is whether adequate epidemiological data on CJD is being collected in every state. Ms. Matthews informs me that currently there are eight states that do not require physicians to report CJD cases. She testified about this issue at a June 2009 meeting of the FDA's Transmissible Spongiform Encephalopathies Advisory Committee. Here's an excerpt from her testimony:

"Accurate disease reporting is a basic and fundamental step of epidemiology... This cannot possibly be done if you are not even counting the cases. The current state of CJD epidemiology in this country is a joke and it is no laughing matter."

Strong words. But Ms. Matthews makes a good point. Mandated reporting is the first step in reliable epidemiological research. Every state should mandate reporting of CJD cases, whether discovered pre-mortem or only at autopsy. Here are the states that fail to mandate reporting: Washington, Nevada, New Mexico, Iowa, Indiana, Alabama, Kentucky, and West Virginia. If you are a resident of one of these states, contact your governor or state legislator to get this situation corrected!


Cure CJD said...

Dr. Moore - thanks for a great article! Ms. Matthews and I have become friends of sorts on-line and she has been very supportive of my family's efforts in fundraising and CJD awareness here in West Virginia. I lost my mom to CJD in August 2007. At that time I contacted the WV State Dept. of Epidemiology to inquire as to why CJD was not a reportable disease. I was not given much of an answer - just that there were no plans to do so. On January 11, 2009, another victim was lost to CJD (from the same area as my mother, less than 20 miles) and there is a third case in a county not far from here. This case has not yet been confirmed but CJD is in the differential. I wonder how the department will feel about this? It is still too rare to qualify as a reportable disease? This disease is relatively ignored in this state. My family hosted an event in support of International CJD Awareness Day. This was our second annual event. And for the second time we were completely ignored by the local media and medical community, despite reaching out to both. Thanks again for all you do - this is such a near and dear cause to my heart. I truly appreciate the work that you do!


Nikki Bland, Bridgeport, WV

Joshua said...

Hi Brain - I agree that we should have stronger reporting laws. However, with sufficient physician education you can still have a pretty well functioning survailance system even in the absence of tough laws. The Neuropathology Division at the University of Washington functions as the statewide Prion Survellance Center under a grant from the National Prion Survellance Center. We beleive that we are capturing the majority of cases based on population incidence statistics from other contries and states with reporting laws at about 1 case per 1,000,000 person years (Allen, et al. Ann Neurol. 2007 Apr;61:371-2).

Josh Sonnen, Seattle, WA

Brian E. Moore, MD said...

Thanks for the comment, Joshua. That's reassuring in the case of Washington. I hope other states have similar set-ups.

Theresa Matthews said...

Dear Dr.Moore,
The IDPH website shows 3 cases of CJD reported in Illinois in 2009. This number is far less than the conservative estimate of 1:1,000,000 cases anticipated to occur in the U.S. and the actual amount expected to be reported in Illinois.
Actually, I think Illinois had more scrapie infected flocks reported in 2009 and I'm quite sure they had more CWD cases reported.
So,are we catching and reporting all cases of CJD? In Illinois, only those cases that receive neuropathological confirmation are required to be reported. Joshua from the University of Washington suggests that sufficient physician education is the key to a well functioning surveillance system. If you look at Illinois data reported prior to the identification of the first case of vCJD in the U.S., the numbers of CJD cases reported in Illinois were much, much higher. What has happened? Are Illinois residents just amazingly resistant to sporadic CJD now or are biopsy and autopsy rates on the decline and more patients receiving a clinical diagnosis instead? Perhaps Joshua wouldn't mind giving his insight on the marked declining number of CJD cases in the nation's fifth largest state. I realize this post might be taken offensively. It is not meant to be. I really do think asking and answering this question is important for all U.S. practitioners, not just those from Illinois. What is the purpose of the NPDPSC if we are not going to use it? Thanks for considering posting my questions.

Brian E. Moore, MD said...

Thanks for the comment, Theresa. I'll contact the IDPH and find out why the reporting in Illinois is so low. Keep an eye out for future posts. I'll also forward your comment to Dr. Josh Sonnen to see if he has anything to say on the topic.

Joshua A Sonnen said...

Hi Brain and Theresa. Cases occur relatively infrequently and a single year is not a lot of time. I would be hesitant to draw inferences about the overall rate by looking at the total number of cases in any one year. The numbers may fluctuate year-to-year due to random chance alone. I'd look at a rolling average over 5 years to see if there was any change in trend. In somewhat comforting news, although there have been over 2000 cases of confirmed prion disease in the US in the last couple of decades, the vast majority (~84%) have been sporadic or genetic/familial (~14%). 5 cases have been iatrogenic (caused by contaminated transplant material) and 4 cases are variant CJD and were likely acquired from eating contaminated beef. Of interest, all 4 cases acquired the disease while living outside of the US. No cases have been acquired from US beef (

There also appears to be little evidence that either scrapie or CWD are transmitted to humans through the direct consumption of sheep or game. There have been anecdotal cases where sufferers of prion disease have had histories of eating game, but Dr. Gambetti at the NPDPSC felt that they didn't have the neuropathologic or biochemical features of transmitted prion disease. (Kong, et al. J Neurosci. August 31, 2005, 25:7944-9) Washington now is included in the list of states that are required by the department of health to report prion disease. I believe this is progress, but I really think that identifying cases comes down to health practitioner education.

I hope this is helpful.

Josh Sonnen, MD
University of Washington Prion Surveillance Center, Seattle

Anonymous said...

I would like information on the CJD Awareness Day. My mother passed away on Sept. 22 2010. I have recently found out she was the possible 3rd case in Butler County PA, population less than 100,000. We are expecting the dna testing part of the test, but she was conformed with CJD. I am aware that she has been reported, but for the others they didn't know what CJD was, and never were tested but had the exact same progression of symptoms, etc. The doctors had no idea what was wrong with her. I found out a name through my own research, and the doctors said I was nuts because it is so rare. Is this what happens? Thank God I am a loud mouth who gets her way or we never would have gotten what we needed. I want to be the one to use their mouth for other victims

Brian E. Moore, MD said...

To Anonymous:
I googled the topic, and apparently International CJD Awareness day is November 12th. But if you have more info on when it is and what happens during that day, I'd be glad to do a blog post on it.

Theresa Matthews said...

Dr. Moore,

A couple of days ago the first forensic pathologist for Maui county in Hawaii, 58 year old Dr. Manoukian, died from CJD. A member of our group contacted Hawaii's DOH about their reporting criteria. It was surprisingly discovered that Hawaii does not mandate reporting!

I have attached a link to the article because it was a nice write up.

Thanks for allowing me to update you all.

Anonymous said...

Hi, my name is Lasamha On September 4,2011 my mom 39 years of age died from CJD whom lived and Chicago was diagnosed with this disease which the doctors at NorthWestern Hospital diagnosed her with the disease a month before she passed away after several of tests and brain biopsy was performed.