Porphyria Educational Services

PORPHYRIA EDUCATIONAL SERVICES BULLETIN
Vol. 1 No. 42 ~ October 10, 1999
FOCUS: Environmental Factors and the Porphyrias

Environmental factors are a trigger for some types of porphyria.
No doubt about it anymore. More and More is being learned and studies are
now showing that a large number of porphyric patients have an exacerbation
of symptoms due to environmental causes.

In an abstract published in "Nature and Genetics" [1996 12:195-199] by
Dr. Grandchamp of Paris, France and a world reknown porphyria
expert, he states: "It is now known that attacks of acute intermittent
porphyria are often precipatated by environmenta; factors.  For this
reason, early detection of gene carriers is important in the prevention
of acute attacks, because affected patients, even though asymptomatic,
can be instructed to avoid precipating factors."

So it is necessary to detect who the gene carriers are in our families and begin
preventative majors as to avoid carriers going from asymptomatic to acute
active porphyrics.  It can be done, and we must move in this direction.  We also
need to move to more and more preventative treatment rather than waiting for acute
attacks to cause us unneccesary and costly hospitalization.

In a paper titled "Redefinition of Abnormal Susceptibility to Environmental
Chemicals: given by the respected and knowledgeable Dr. William E. Morton
MD, a porphyria diagnostian,  before an International gatherings some years back
in Atlanta, Georgia, Dr. Morton recognizes that many doctors are missing the
correct diagnosis.

Dr. Morton states: "For some time we have known of persons
who manifest extreme intolerance for small exposures to environmental
chemicals." He goes on to say that "During the last several years, Mayo
Laboratory's introduction of several new blood-cell porphyrin enzyme
tests has permitted recognition of a broader spectrum of porphyria
cases than previously. Many of these milder and more chronic porphyria
cases have characteristics of MCSS, which appear after the porphyrin
enzyme deficiency has converted from latency to metabolic
activity by porphyrogenic substance exposure."

In talking about the symptomology of porphyria Dr. Morton sted
"Porphyria symptoms can be quite variable, ranging from severe acute attacks
to continuing low-grade smoldering symptoms to asymptomatic latency.
The porphyrias used to be considered very rare conditions
[under 200,000 known cases], discoverable only during an acute
attack when porphyrin excretion was markedly excessive.

Happily today as Dr. Morton points out, " The newly available and
much more sensitive blood-cell enzyme tests have naturally resulted in
greatly increased population frequency estimates (4%- 9%, up from 0.01-0.1%)
in agreement with estimates made more than a century ago."

So what are the environmental factors that cause this increase
of acute porphyrics? Environmental porphyrogenic substances include paints
and pain fumes, expoxies, metal dusts and fumes,alcohols [especially
ethanol], freons, arsenic, biphenyls [PCBs], DDT, chlordane, glycols, glycol ethers and
derivatives, vinyl chloride fumes and dust, , polychlorinated formaldehyde,
other aldehydes, freons, dioxins, hexachlorobenzene chlorophenoxy acetic
acids,  heavy vehuicle exhaust fumes, perfumes and other fragrances
,tetracloro dibenzodioxins [TCBCs]  xhlorine, chlorinated cleaning
agents,and many others that continue to be identified by medical researchers
around the world.

In respect for the environmental factors porphyrics and those
who are highly suspect for porphyria because of familial histories of
porphyria should seek testing and then take all precautions known for
avoiding exposure to these environmental factors. This can come through
identification of index and related latent cases allowing for the control of
symptoms by preventive avoidance of porphyrogenic materials.