Porphyria Educational Services
Monthly Newsletter October 2005
All information published in the Porphyria Educational Services Monthly Newsletter is
to provide information on the various aspects of the disease porphyria and it's associated
symptoms, triggers, and treatment.
Columnist and contributors and the information that they provide are not intended as a
substitute for the medical advice of physicians. The diagnosis and treatment of the
porphyrias are based upon the entire encounter between a physician and the individual
Specific recommendations for the confirmed diagnosis and treatment of any individual
must be accomplished by that individual and their personal physician, acting together
Porphyria Educational Services in no way shall be held responsible in part or whole for
any injury, misinformation, negligence, or loss incurred by you. In reading the monthly
newsletters you need to agree not to hold liable any contributing writers.
Chlorine and Chemical Toxins
Chlorine is everywhere. And furthermore chlorine is essential. However for most porphyrics chlorine is considered a chemical toxin which can and does trigger acute porphyria attacks from time to time.
In the world of medicine where porphyric patients find themselves so often, chlorine is lurking just about everywhere. This includes even the medications that most people take commonly and even on a daily basis.
One common drug is acetaminophen. Others include antibiotics. Chlorine is a biggie in anti-cancer drugs including cisplatin, and mintotane.
Other drugs containing chlorine include xanax, vancomycin, lorabid, ceclor, benedryl,chlor-trimeton.
In addition, almost one-third of central nervous system drugs contain chlorine, and 98 percent of gastrointestinal medications are made using chlorine.
At the same time chlorine is what makes our tap water safe and keeps our "whites white" during laundry.
Chlorinated compounds are essential to the development of potent new drug therapies. Of the nearly 400 new drugs approved for therapeutic use in humans since 1984, more than 60 are chlorinated compounds, and many others use chlorine's unique chemical properties in their production.
In the medical world chlorine does not just stop with the pharmaceuticals.Chlorine is essential to a wide variety of medical equipment. An estimated one-fourth of all medical devices in hospitals contain chlorine, ranging from some of the most commonly used to some of the most specialized and advanced.
X-ray and mammography films are made with silver chloride. Chlorine also is a basic building block in the silicon used to make the semiconductors upon which many electronic medical devices depend. And surgical sutures, artificial blood vessels, and osmotic membranes are all made with nylon, a product made using chlorine chemistry.
Chlorine-based plastics also are widely used in medical devices and equipment. Of the 14 families of plastics made using chlorine, the most common is polyvinyl chloride, a plastic known for being light, easy to bend and shape, and inexpensive.
As a porphyria patient, if your are super sensitivity to chemical toxins including chlorid, it is important to be aware that the IV and blood bags are made of chloride.
This precaution also holds true with the oxygen tents some times used in the hospital setting and even prescription eyewear.
Chlorine-based vinyl packaging also adds to the safety of medicine.
Many pharmaceuticals also are supplied in vinyl packaging -- such as the "blister" packs that help extend the shelf life of tablets and capsules and make it easier for patients to take the proper dosage.
Much of the aforementioned is safe for porphyrics however, if contact is limited.
What is neeeded to be mindful of is the chlorination of drinking water, and especially chlorinated water in swimming pools.
This has always been a problem for the general populous and with porphyrics it is even more so.
Skin irritation is a great concern for porphyrics with the cutaneous manifestations.
However AIP also can be affected by chlorine.
Melissa Gilbertson PhD
PN Treatment in Porphyria
The goal of treatment for PN in the acute porphyrias is to manage the underlying condition causing your neuropathy and to repair damage, as well as provide symptom relief.
Along with determining the cause of your neuropathy, the physician may try a variety of medications to see which specific drugs may be beneficial in alleviating the porphyria patient's symptoms.
Controlling a chronic condition may not eliminate your neuropathy, but it can play a key role in managing it.
Porphyria patients who learn to control their triggers and avoid acute episodes find that PN can be controlled and often wll go into remission.
Not unlike those with diabetic neuropathy, acute porphyria patients who carefully watch their carbohydrate intake and avoid acute episodes,both slow the onset and the progression of neuropathy.
It has been found that some porphyria patients experience certain vitamin deficiencies. Such vitamin deficiences can and do cause neuropathy.
If neuropathy is the result of a vitamin deficiency, it is most likely that the neuropathy symptoms will disappear once the deficiency is corrected.
The use of medications can ease pain symptoms of PN, but most have side effects, especially if taken for long periods of time.
If a porphyria patient takes pain medication regularly, including over-the-counter (OTC) products,it is most important for the patient to discuss the benefits and side effects with one's primary care physician as well as a neurologist.
Medications that may help provide pain relief for neuropathy include: pain relievers, tricyclic antidepressants, antiseizure medications, as well as other medications.
The problem with most of the medications is that they have been found to be unsafe for acute porphyria patients.
One drug known to be safe for porphyria patients is that of Neurontin aka Gabapentin. The drug is designed for the treating of seizures associated with PN in porphyria.
Some porphyria patients have claimed that the drug is beneficial in the treatment of pain, however the drug is not
approved for being a pain medication in neuropathy.
Transcutaneous electronic nerve stimulation (TENS) is one of the more used and beneficial treatments for PN in porphyria patients.
Through the use of TENS therapy which helps prevent pain signals from reaching the patient's brain, TENS delivers tiny electrical impulses to specific nerve pathways through small electrodes placed on the skin.
Although safe and painless, TENS doesn't work for everyone or for all types of pain.
TENS is generally more effective for acute pain than for chronic pain and is often used in conjunction with other therapies.
Acupuncture and Biofeedback are other commonly used
treatment therapies for porphyria patients with PN.
Lowell Manchester PA
Neurology & Physical Medicine
Hyponatremia and Porphyria
Hyponatremia deals with one part of the electrolyte imbalance which often occurs as a part of acute attacks of porphyria.
Hyponatremia occurs when the body has less than the normal amount of sodium in the blood.
Realization of this less than normal range of sodium is determined through a collection of blood from a patient.
Most often it is a part of the routine electrolyte panel which is administered as a patient is admitted to the hospital where they will begin intervention therapy for the porphyria.
If hyponatremia is left undiagnosed and untreated the porphyria patient will most likely develp water intoxification. In addition the patient will usually present with confuion and lethargy leading to muscle spasms, convulsions and coma.
Laboratory tests to check the ranges of all electrolytes is most essential to be administered to all porphyria patients during acute attacks.
Hyponatremia is most notable for its frequency and intensity during acute attacks in over 50% of porphyria patients.
Increase in urinary porphobilinogen, is also often observed.
In addition septic complications, such as pneumonia, septicemia, and urinary tract infection, present in over 50% of acute attacks requiring hospitalization..
Administration of needed electrolytes in conjunction with the necessary administration of carbohydrates will most often correct the sodium levels.
It is most important for physicians to followup with further electrolyte testing as the porphyria patient begins into remission.
Karen Simmons, RN, NP
Hepatitus C Infection and Porphyria
Many research studies have been looking at the role of Hepatitus C
Some of the research on Hepatitus C involves the areas of natural history, pathogenesis, therapy and prevention of Hepatitus C.
Hepatitus C is no stranger to various forms of porphyria.
Hepatitus C research would be most beneficial to many porphyria patients, especially thosewith PCT.
A high number of PCT patients also have the Hepatitus C condition in addition to their PCT.
Other hepatic porphyrias are also known to sometimes deal with Hepatitus C.
Chronic liver disease is experience by many porphyria patients as the disease progresses and continues to manifests new or advanced symptomology.
The hepatitis C virus is a major cause of acute and chronic liver disease.
In porphyria cirrhosis or liver failure may also occur.
With the Hepatitus C experienced by so many PCT type porphyria patients the infection goes from the acute stage to the chronic.
Acute hepatitis C leads to chronic infection in approximately
75% of cases according to various studies that have been undertaken.
Chronic hepatitis C is often asymptomatic and can be mild; but in a proportion of patients, the chronic infection leads to progressive liver disease.
Many PCT patients with the Hepatitus C in the past have ultimately incurred cirrhosis and end stage-liver disease, including hepatic carcinoma [liver cancer] known as HCC.
The determinants of outcome and progression of liver disease in hepatitis C
are unknown but may be related either to viral, behavioral, environmental or genetic factors of the
In addition, alcohol use, other medical conditions such as a form of porphyria, and co-infection with other viruses may also affect the disease outcome of hepatitis
Currently the treatment of Hepatitus C is not very good.
Only a small percentage of porphyria patients respond to currently available therapies with long term remission of
Researchers and physicians alike note that the most
effective way to prevent the liver disease of hepatitis C is through
the development of a preventative vaccine.
Thus, the focus of research for porphyria related Hepatitus C is the development of
a vaccine for hepatitis C. A study through NIH is providing partial
funding for such research.
The future for porphyria patients with Hepatitus C lies in
the development of a vaccine for the Hepatitus.
Bonnie Harrison MNS, NP
Hyperpigmentation Seen in Porphyria
Hyperpigmentation is experienced by some type of cutaneous porphyria patients. It often
is an added condition in the patients that experience hitsutism, but not always.
Hyperpigmentation has a close relationship to porphyria as the pigments of the skin are derived from hemoglobin or the products of hemoglobin catabolism.
All of the blood, bile and urinary pigments are derived in this fashion.
Pigments are made up of organic coloring molecules within the body.
Such pigments are found in the bile, blood, urine, the eye, skin and hair.
The endogenous pigments of the choroid, skin and hair are called melanins.
Pigment comes into two basic types.
Eumelanin is a brown/black type and the pheomelanin is red and amber.
. Melanins are synthesized in membrane bound cellular organelles called melanosomes from the amino acid tyrosine into dopa and dopaquinone.
Eumelanin is primarily responsible for the color seen in skin, hair and eyes.
Eumelanin is genetically controlled. The exception to this genetic control is the tanning reaction that occurs with the exposure to UV light. Differences in skin color are due mainly to differences in the number of melanin granules in the keratinocytes.
Pigmentation levels usually increase with age.
For some people, hair may lose all pigmentation early in life, thus they experience gray hair most of their life.
Normal pigmentation may be altered by genetic defects or by acquired diseases or metabolic disease such as porphyria.
In some cutaneous porphyria this means an increase in pigmentation which is known as hyperpigmentation.
Some internal compounds--such as the byproducts of hemoglobin metabolism--may color the skin.