Porphyria

Greek for "Purple Urine"
Copyright (C) 1995-2003, JVV


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Diseases characterized by problems in the heme synthesis biochemical pathways. (See chart below.) This is often the base cause of Multiple Chemical Sensitivities (MCS), light sensitivity, some forms of skin problems, and wine colored or purple urine; but is not the only condition that can cause these problems.


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Often, problems in the Cytochrome P-450 heme synthesis and detoxification pathways show up as porphyria type problems.

It has been reported that many persons with Multiple Chemical Sensitivity have heme related problems. Some forms are hereditary, yet a growing number of cases are being reported involving chemical exposures and drug interactions (e.g. Prozac, Zoloft, antacid medications and others.) The P450 system will break these drugs down, using up the P450 feedstocks which would otherwise be used to produce red blood. It is said that about one fifth of our pharmaceuticals affect the P450 pathways.

As P450 feedstocks are diverted from heme synthesis to deal with these drugs and other environmental toxins, other materials (called porphyrinogens,) in the heme synthesis pathways build up and become oxidized in the blood stream to form porphyrins, which absorb light more strongly, even through the skin, and can cause porphyria type symptoms. If the problem is related to short term chemical exposures, the porphyria may be intermittent, making it harder to get a clear diagnosis. (Most hospital urine tests only test for the rarer forms.) It is one of the complaints of many Gulf War Veterans.

Diagnosis can be difficult. This is particularly true when the patient has not been recently exposed, and may no longer be exhibiting strong symptoms or biochemical abnormalities; yet P450 levels may remain low enough to allow transient porphyrias on exposure to common chemicals.

In addition to chemical exposure, exposure to ultraviolet and sometimes even blue light can affect many forms of porphyria. Urine is sometimes discolored during or before an attack: red, orange, purple, even green have been seen at times. But sometimes the porphyrins are excreted via the gall bladder, and never show in the urine.

(One simple test for porphyria is to expose a cup of urine to sunlight for the better part of a day. If the urine becomes wine colored or purple, porphyria is probable. However, lack of color changes will NOT indicate that prophyria is unlikely.)

Some of the more severe symptoms may include skin lesions, mental instability, and neuropathy. Dextrose 10% IV may help in a crisis, as may IV Heme if the dextrose is not enough.

Another problem may involve Subclinical Hypothermia , or Wilson's Syndrome. This is sometimes mistreated as "Thyroid Problems", using Synthroid, when a more balanced T3/T4 treatment may be more beneficial. (However, before considering glandular based therapies, read Mad Cow.) Every degree F below normal can reduce the efficiency of some enzymes by up to 20%. As enzymatic efficiency falls with temperature, symptoms mimicking those of genetic enzyme deficiencies (e.g. porphyria,) may manifest themselves and result in diagnostic inaccuracies. With reduced heme production, reduced oxygen transport may reduce metabolism, thus reducing body temperature, further interfering with P450 and other enzymatic systems. Low body temperature can have behavioral effects as well as interfering with a wide array of repair mechanisms, thus producing many seemingly unrelated symptoms in the same patient.

Porphyrin / Heme Pathways

5-amino-levulinate
      |
      + heme, potassium as catalyst
      |
Porphobilinogen
      +-------------+ multiple pathways.
Polypyrrylmethane * | (In bacteria, to B12 synthesis)
      +-------------+
Uroporphyrinogen III *
      |
Coproporphyrinogen III *
      |
      +  Fe++ as catalyst.
      |
Protoporphyrinogen IX *
      |  
Protoporphyrin IX
      |
      +-(- Ferritin (---  Vitamin C  
      |                   + Fe+3 (--- transferin
      |  
  Proto-Heme
      |
      V 
  Cytochrome "a" and "c" groups     
  Hemoglobin
  Myoglobin

  (In plants, with magnesium instead 
   of Ferritin/Iron, Chlorophyll)
Loss of hydrogens from the porphyrinogens (noted with "*",) generally turns them into porphyrins, which are the cause of the porphyrias. Hydrogen or electron donors, such as vitamin C, are believed to inhibit the transformation of porphyrinogens to porphyrins. Also, vitamin C will facilitate the conversion of the products to cytochromes and hemoglobin, reducing the buildup of intermediate products in the above pathway.

Some forms of porphyria are believed to involve the mitochondria, reducing energy conversion, and promoting a kind of chronic fatigue. It is interesting to note that porphyria patients sometimes talk about losing salt or craving salt. Salt is conserved in the kidneys by the exchange of the sodium ions for hydrogen ions. One may speculate that as sodium concentrations fall, additional stress is placed on the hydrogen ion sources, increasing the generation of porphyrins in susceptible individuals. One such stress may be the need to generate more cytochromes during exposure to environmental toxins or therapy with inappropriate pharmaceuticals. One fifth of all pharmaceuticals are detoxified by the cytochromes, and will thus place additional burdens upon the porphyrin related pathways. Thus individuals with porphyria may be seriously harmed by doctors without specific training and information related to porphyria.

Not all porphyria victims report benefit from high doses of vitamin C, but there are often other issues involved, such as yeast infections, which need to be addressed before benefits of high dose (bowel tolerance) vitamin C therapy can become evident. Yeasts are a particular problem, as high carbohydrate diets are beneficial to many porphyria patients, yet can encourage yeast overgrowth. Sometimes various C Salts are useful. If one listens to what one's body is saying, one stands a better chance of doing the right thing.

Porphyria patients often report that doctors, particularly those in HMO's, are not well versed, and sometimes appear unwilling to run all the proper tests to confirm a diagnosis of porphyria. Some will comment that this may be related to the expense burden that good treatment of porphyria (multiple IV's of Dextrose, Heme, and use of other non-generic medicines,) can create for an HMO. Even in private care, misdiagnosis and improper treatments are not uncommon. Indeed, many victims say improper and often dangerous treatments are the rule, not the exception, when dealing with doctors who have no special training for dealing with porphyria.

A related detoxification pathway involves Paraoxonase and the related oxonases.


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Overview

Porphyria. (General pamphlet)
http://www.ciris.net/~brenjohn/ A porphyria victim's research links. Includes safe and unsafe drugs, and a lot of other information.
The American Porphyria Foundation
American Porphyria Foundation - Hematin

Causes, Influences

Intoxication Porphyria and Hexa Chloro Benzene
Environmental / "Intoxication" Porphyria, Symptoms
Lead Discover Mag, Jan 1996, A detective story.
Testing for Toxic Metal- and Chemical-Induced Porphyrinuria
Drug Interactions
Dave Flockhart's Drug Table
P450 Inhibited by SSRI's, can cause transient porphyria
Problems with SSRI's
Drugs considered unsafe and safe in acute porphyrias
Light Sensitivity: Query on short wavelength light
MEDIC: CLINICAL HANDBOOK
Porphyria cutanea tarda And Hepatoerythropoietic porphyria
Hahnemann Edu - Types
Porphyrias -- Two Types
Examples of Porphyria

Technical

Technical Overview //vetpath.afip.mil/general/friedlander.porphyri.txt
Neuromuscular Home Page
Porphyria Types, Genetics, Which medicines unsafe.
Mitochondrial Differential Diagnosis
Motor Syndromes
http://darwin.clas.virginia.edu/~rjh9u/aip.html
Research
Genetics, Research, etc. With Refs.
Research trials With Refs.

Related Conditions

Iron Overload Diseases Association, Inc.
Gulf War Veterans Org.

Unclassified:
  • http://vetpath.afip.mil/general/friedlander.porphyri.txt
  • http://www.enterprise.net/apf/
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    Note that Subclinical Hypothermia, or Wilson's Syndrome, low body temperature 98.2 or below, may also be present with immune system and other effects in many who have MCS and Porphyria like symptoms.

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