Why so young? (This is a long one...)

The posting below was found on the CJD Voice Guestbook.

Wednesday 11/07/2007 3:02:25pm
Name: Lindsay Smith
E-Mail: lsmith12981@yahoo.com
Referred By: Search Engine
Location: Birmingham, AL
Comments: My best friend who is 26 years old was diagnosed with CJD in June. Her name is Megan and she lives in Atlanta, and she has been battling the symptoms since March, and we are told that she is the second youngest person in the US to ever have CJD...this is such a bizarre mystery and truly devastating!!!! Please keep her and her family in your prayers!

Doesn't variant CJD, the form linked to Mad Cow's Disease, have a history of striking younger aged people?

vCJD Differs from Classic CJD
This variant form of CJD should not be confused with the classic form of CJD that is endemic throughout the world, including the United States. There are several important differences between these two forms of the disease. The median age at death of patients with classic CJD in the United States, for example, is 68 years, and very few cases occur in persons under 30 years of age. In contrast, the median age at death of patients with vCJD in the United Kingdom is 28 years.

The incubation period for vCJD is unknown because it is a new disease. However, it is likely that ultimately this incubation period will be measured in terms of many years or decades. In other words, whenever a person develops vCJD from consuming a BSE-contaminated product, he or she likely would have consumed that product many years or a decade or more earlier.

The BSE epidemic in the United Kingdom reached its peak incidence in January 1993 at almost 1,000 new cases per week. The outbreak may have resulted from the feeding of scrapie-containing sheep meat-and-bone meal to cattle. There is strong evidence and general agreement that the outbreak was amplified by feeding rendered bovine meat-and-bone meal to young calves.

Background
Since variant CJD was first reported in 1996, a total of 208 patients with this disease from 11 countries have been identified. As of June 2008, variant CJD cases have been reported from the following countries: 167 from the United Kingdom, 23 from France, 4 from Ireland, 3 from the United States, 3 from Spain, 2 in the Netherlands, 2 in Portugal, and one each from Canada, Italy, Japan, and Saudi Arabia. Two of the three U.S. cases, two of the four cases from Ireland and the single cases from Canada and Japan were likely exposed to the BSE agent while residing in the United Kingdom. One of the 23 French cases may also have been infected in the United Kingdom.

There has never been a case of vCJD that did not have a history of exposure within a country where this cattle disease, BSE, was occurring.

It is believed that the persons who have developed vCJD became infected through their consumption of cattle products contaminated with the agent of BSE or in three cases, each reported from the United Kingdom, through receipt of blood from an asymptomatic, infected donor. There is no known treatment of vCJD and it is invariably fatal.

U.S. Surveillance for variant CJD
The possibility that BSE can spread to humans has focused increased attention on the desirability of enhancing national surveillance for Creutzfeldt-Jakob disease (CJD) in the United States in order to detect variant CJD. Improving methods to detect classic CJD, such as increasing the number of autopsies on patients with suspected prion disease, enhances the ability to identify cases of variant CJD.

The Centers for Disease Control and Prevention (CDC) monitors the trends and current incidence of classic CJD in the United States through several surveillance mechanisms. The oldest and most systematic method includes analyzing death certificate information from U.S. multiple cause-of-death data, compiled by the National Center for Health Statistics, CDC. During 1979-2003 the average annual age adjusted death rates of classic CJD have remained relatively stable. Moreover, deaths from variant CJD in persons aged <30 years in the United States remain extremely rare (<5 cases per 1 billion per year). In contrast, in the United Kingdom, over half of the patients who died with vCJD were in this young age group.

In addition, CDC collects, reviews and when indicated, actively investigates reports by health care personnel or institutions of possible variant CJD cases. Finally and very importantly, in 1996-97, CDC established, in collaboration with the American Association of Neuropathologists, the National Prion Disease Pathology Surveillance Center at Case Western Reserve University, which performs special diagnostic tests for prion diseases, including post-mortem tests that can detect vCJD.

vCJD Cases Reported in the US
Three cases of vCJD have been reported from the United States. By convention, variant CJD cases are ascribed to the country of initial symptom onset, regardless of where the exposure occurred. There is strong evidence that suggests that two of the three cases were exposed to the BSE agent in the United Kingdom and that the third was exposed while living in Saudi Arabia.

The first patient was born in the United Kingdom in the late 1970's and lived there until a move to Florida in 1992. The patient had onset of symptoms in November 2001 and died in June of 2004. The patient never donated or received blood, plasma, or organs, never received human growth hormone, nor did the patient ever have major surgery other than having wisdom teeth extracted in 2001. Additionally, there was no family history of CJD.

The second patient resided in Texas during 2001-2005. Symptoms began in early 2005 while the patient was in Texas. He then returned to the United Kingdom, where his illness progressed, and a diagnosis of variant CJD was made. The diagnosis was confirmed neuropathologically at the time of the patient's death. While living in the United States, the patient had no history of hospitalization, of having invasive medical procedures, or of donation or receipt of blood and blood products. The patient almost certainly acquired the disease in the United Kingdom. He was born in the United Kingdom and lived there throughout the defined period of risk (1980-1996) for human exposure to the agent of bovine spongiform encephalopathy (BSE, commonly known as "mad cow" disease). His stay in the United States was too brief relative to what is known about the incubation period for variant CJD.

The third patient was born and raised in Saudi Arabia and has lived in the United States since late 2005. The patient occasionally stayed in the United States for up to 3 months at a time since 2001 and there was a shorter visit in 1989. The patient's onset of symptoms occurred in Spring 2006. In late November 2006, the Clinical Prion Research Team at the University of California San Francisco Memory and Aging Center confirmed the vCJD clinical diagnosis by pathologic study of adenoid and brain biopsy tissues. The patient has no history of receipt of blood, a past neurosurgical procedure, or residing in or visiting countries of Europe. Based on the patient's history, the occurrence of a previously reported Saudi case of vCJD attributed to likely consumption of BSE-contaminated cattle products in Saudi Arabia, and the expected greater than 7 year incubation period for food-related vCJD, this U.S. case-patient was most likely infected from contaminated cattle products consumed as a child when living in Saudi Arabia (1). The patient has no history of donating blood and the public health investigation has identified no known risk of transmission to U.S. residents from this patient.

Prevention Measures against BSE Spread
To prevent BSE from entering the United States, severe restrictions were placed on the importation of live ruminants, such as cattle, sheep, and goats, and certain ruminant products from countries where BSE was known to exist. These restrictions were later extended to include importation of ruminants and certain ruminant products from all European countries.

Because the use of ruminant tissue in ruminant feed was probably a necessary factor responsible for the BSE outbreak in the United Kingdom and because of the current evidence for possible transmission of BSE to humans, the U.S. Food and Drug Administration instituted a ruminant feed ban in June 1997 that became fully effective as of October 1997.

In late 2001, the Harvard Center for Risk Assessment study of various scenarios involving BSE in the United States concluded that the FDA ruminant feed rule provides a major defense against this disease.

BSE/TSE Action Plan of the Department of Health and Human Services (DHHS)
On August 23, 2001, the Department of Health and Human Services (HHS) issued a department-wide action plan outlining steps to improve scientific understanding of BSE and other transmissible spongiform encephalopathies (TSEs). The action plan has four major components:

Surveillance for human disease is primarily the responsibility of CDC.

Protection is primarily the responsibility of the Food and Drug Administration (FDA).

Research is primarily the responsibility of the National Institutes of Health (NIH).

Oversight is primarily the responsibility of the Office of the Secretary of DHHS.

Source: http://www.cdc.gov/ncidod/dvrd/vcjd/factsheet_nvcjd.htm

I would like to expand on some of the sections above that are bolded. In response to the measures that the US takes for surveillance of CJD through death certificate information, I would have to say I'm doubtful of their accurate findings. My dad's death certificate was printed way before his autopsy results were even given to us. The death certificate doesn't mention anything about which of the 3 forms it was; all that it says is cause of death "Creutzfeldt-Jakob Disease". Also, my step-mom sent me the report from the National Prion Disease Pathology Surveillance Center and I just received it today. I was under the impression that this report was my dad's autopsy so I was thinking it would be fairly detailed in length. However, all that I received is one sheet of paper. It has a big stamp on it that says "Mailed 3/21", but for some reason our family didn't receive it until 4 months later. Even with the serious of this disease, the surveillance center is sure taking their time to perform autopsies. Also, using a bunch of technical terms it basically says that "the results of the tests preformed confim the diagnosis of prion disease with the charactersics of sporadic Creutzfeldt-Jakob disease". It also goes on to say "The PrP gene sequencing rules out the presence of a pathogenic mutation so the prion disease in this case is not familial". However, nothing is mentioned about any test done to rule out vCJD. Also, the last part of the letter basically uses lots of medical mumbo-jumbo to cover their backs and says, "This method does not consitute a definitive diagnostic test of a precise test for prion associated diseases. Possibe sources of diagnositc error include sample mix-up and genotyping errors...." So basically what I get from all of this is that even after an autopsy, diagnosis findings are not 100% positive. I think all of this has a lot to say about the quality of the CDC's surveillance measures for CJD. Also, the FDA is supposed to be protecting us but I'd have to say that I'm doubtful of the quality of their work as well. Like I said in a prior post, there have been drastic cuts in the amount of Food Inspectors so there is more that goes undetected as more food comes into the US and more facilities arise. Obviously two main areas, surveillance and protection, of the BSE/TSE Action Plan of the Department of Health and Human Services are not measuring up. This puts us all at risk! I'm going to contact "Lindsay Smith" to see if there has been any follow up with her best friend and her CJD diagnosis. I'll let all of you know what I find out. Please keep on spreading the word. I hope this information above helps you to see that there is reason for us to need to know more.