Secondary Porphyria

Secondary Porphyria

Porphyria is an accumulation in the body of the precursors to heme production, the part of the red blood cell that contains iron and binds oxygen. It occurs due to decreased activity of the enzymes that produce each step in the process (different enzyme deficiencies lead to different types of porphyria), and it’s generally thought of as a rare genetic disease.

But it can also be acquired, due to various toxic exposures.

Symptoms of Porphyria

The type of porpyria associated with toxic exposure is primarily Acute Intermittent Porphyria (AIP) and it leads to accumulation of porphobilinogen (PBG) and δ-aminolevulinic acid (ALA)—both of which can be tested for in urine. (More on this in the testing section.)

Symptoms of AIP are quite varied, but can be consolidated as “The 5 P’s” (one of those med student tricks for memorization):

  • Painful abdomen: can be just intense pain, or cramping, constipation, diarrhea, nausea, and/or vomiting.
  • Polyneuropathy: this means pain, weakness, or numbness in multiple nerves. Strange or unusual nerve symptoms are common. Other nervous system disturbances might include dizziness, or intolerance to stimuli (such as light, sound, temperature, smells, and EMF fields.)
  • Psychological Disturbances: These might include anxiety, depression, panic attacks, or insomnia.
  • Purple urine: the heme precursors in the urine contain iron—and oxidized iron is what gives blood its red color. Therefore, the urine tends to look red or purple, though this is not always the case. Sometimes the urine color is normal.
  • Precipitated by triggers: A drug or toxic substance increases production of heme. This overwhelms the enzyme, and then (like a bottleneck), the precursors accumulate.

Drugs That Can Cause Secondary Porphyria

Drugs that tend to trigger porphyria are those that speed up enzymes higher in the biochemical production of heme, causing a bottleneck at PBG and ALA. These include seizure medications, benzodiazepines, sulfa drugs, birth control pills, and the NSAID ketorolac/Toradol.

Progesterone can also act as a trigger (and in this case study, it was the body’s own progesterone during the luteal phase of the menstrual cycle!)

Toxins That Can Cause Secondary Porphyria

The best known toxin to trigger porphyria is lead poisoning. However,  there are certainly others.

Anecdotally, I have heard that Lyme, Lyme coinfections, and mold toxicity can also act as triggers for porphyria, though that hasn’t made it into the literature yet, that I’ve found.

Testing For Porphyria

Traditional labs can test for PBG and ALA in the urine. It’s best to test during a symptomatic episode, as the test is most likely to be positive at that time. Because porphyrins are light sensitive, it’s also advisable to keep the sample in a dark container until it can be analyzed.

Treating Porphyria

For acute and severe cases of porphyria, treatment is called hemin, which basically replaces heme. This then sends feedback suppression to earlier parts of the pathway (telling the body that there’s enough heme, you don’t need to keep making more of it.) This enables the enzymes to play catch-up.

Other less invasive treatments include carbs, or specifically glucose. This is because glucose will help to shut down one of the enzymes higher in the biochemical pathway, producing more of the heme precursors.

Activated charcoal also seems to work well as a binder, for elimination of porphyrin.

Also, if you’ve experienced porphyria, you may be deficient in Vitamin B12. (This seems to be because one of the downstream products of the heme production process also turns into B12.) Supplementing with B12 after an episode may therefore be helpful.

SUBSCRIBE TO MY NEWSLETTER

Subscribe to my wellness newsletter & get a FREE eBook: "10 Supplements Everyone Should Have."  Plus, get 15% OFF your first order from my new online store! You may unsubscribe at any time.

free_ebook-_ipad

By |2019-01-04T15:26:05+00:00January 4th, 2019|Categories: Articles, Conditions & Treatments|0 Comments

About the Author:

Dr. Lauren Deville is board-certified to practice medicine in the State of Arizona. She received her NMD from Southwest College of Naturopathic Medicine in Tempe, AZ, and she holds a BS in Biochemistry and Molecular Biophysics from the University of Arizona, with minors in Spanish and Creative Writing. She also writes fiction under a pen name in her spare time. Visit her author website at www.authorcagray.com.

FREE RECIPES

Get a week's worth of fast, healthy, kid-friendly recipes for the whole family when you sign up for our free monthly health newsletter!

I hate spam as much as you do. I will never share, sell, or publish your email address.