Eye from Albany
May 2004
Prion diseases; is the lid about to come off?
by Paul M. Bray
One of government's most valuable functions is public health
including the
prevention, monitoring and response planning for diseases like
interspecies lyme
disease and West Nile Virus and health threats like bio-terrorism.
Yet, for
decades policy and political attention has been monopolized on
modes and costs
of patient treatment. This could, in part, be the effect of the
conservative
notion that government can do no good and everything should be
left to the
market. Whatever the cause, we had better wake up to make sure
our public health
assets are fully engaged to address the growing health threats
ranging from
obesity to instantaneous threats like smallpox. Let me share
a public health
issue that may be raising its ugly presence.
Last fall a colleague of mine from the middle of the State
died. When I
mentioned his death to another colleague, a non-alarmist type,
who lived near the
deceased, he replied, "Yes, it is a very tragic death, he
died of mad cow". I
was speechless, as the thought that anyone in this nation was
vulnerable to mad
cow just didn't seem possible.
Shortly after that episode, I read about prions, a type of
protein, that is
the cause of a number of brain related diseases, most notably
Creutzfeldt-Jacob
disease (CJD), bovine spongiform encephalopathy (human mad cow
disease),
scrapie in sheep and chronic wasting disease in deer. CJD is
generally considered
to be caused by genetic mutation, proteins losing their shape
over time, while
mad cow is caused by infection of prions from outside sources.
CJD is
reported to cause about 250 to 300 hundred relatively swift deaths
each year in the
USA.
Complicating matters is the lack of rapid testing capacity.
Case Western
Reserve has the only facility that can test for human mad cow
disease. The
decreasing number of autopsies performed also limits the ability
of health
authorities to track cases of CJD or other prion related diseases.
In addition, Alzheimer's disease has many common symptoms
that doctor who are
not specialists may not distinguish from CJD and human mad cow
disease.
Italian researchers have recently found a second strain of mad
cow disease
resembling CJD.
A feature of prion biology is that a single protein is able
to generate
different infectious strains each with its own specific characteristics.
Prion
pathology is an emerging science and scientists are just finding
that prions have
the ability to jump the species barrier and infect other mammals.
While the science is emerging, there is a question whether
we also have an
emerging public health crisis affecting our food supply.
So far, the signs of impending crisis are small, but growing
in attention.
You may have noticed an article, "The Case of the Cherry
Hill Cluster by D.T.
Max in the NY Times Magazine on March 28th of this year, about
how a cluster of
CJD deaths in New Jersey may really have been deaths caused by
mad cow. The
writer notes, "Many public health advocates and some scientists
say they suspect
that mad cow does in fact exist in America".
A gadfly, Janet Skarbek, has picked up on the New Jersey cluster
and has been
peppering the NJ Department of Health and Centers for Disease
Control and
Prevention with her theory contaminated meat caused the cluster
of deaths and
that "There has to be a strain of mad cow disease in America
that is rare enough
or different enough to have avoided detection".
Skarbek "sees herself unraveling a conspiracy of government
and business
interests (of food suppliers) to hide an epidemic".
On the west coast, the Seattle Post-Intelligencer reported
"Now largely
because of that made cow case (the diseased cow found in Yakima,
Washington), more
people are wondering whether their elderly relative might have
suffered from
CJD-a degenerative disease of the nervous system-or its variant,
the human form
of mad cow disease".
The fear and debate surrounding these circumstances present
a serious
challenge to our public health authorities. Can surveillance
and testing develop
rapidly enough to contain a real mad cow "epidemic"
and/or the hysteria over the
unknown and mysteries surrounding CJD and other prion related,
interspecies
brain diseases? Today, for example, not enough autopsies are
being performed to
adequately monitor prion pathology.
In New York State, we are fortunate to have the world class
State Wadsworth
public health labs in Albany and other research facilities like
one in Staten
Island where a fast test for chronic wasting disease was recently
developed.
This may be a time and an issue when the premium will be on trust
in the
capacity of our public health authorities to protect us. Yet,
one can only wonder if
we have invested enough in public health resources to adequately
have them
there to meet new and increasing complex threats to our health.
Paul M. Bray is President of P.M.Bray LLC, a planning and
environmental law
firm in Albany, New York. His e-mail is pmbray@aol.com.