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Porphyria Educational Services
Monthly Newsletter
July 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 patient.

Specific recommendations for the confirmed diagnosis and treatment of any individual must be accomplished by that individual and their personal physician, acting together cooperatively.

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.

The Liver and possible HCC

HCC is a real concern for many porphyria patients with AIP or PCT.
HCC stands for hepatocellular carcinoma, in other words, liver cancer.
Your liver is the largest and most complex internal organ in your body.
The liver is particularly sensitive to the manifestations of the disease porphyria.
The liver weighs between 3 and 4 pounds, is about the size of a football. The liver detoxifies harmful substances, purifies your blood and manufactures vital nutrients.v Primary liver cancer occurs when cancerous (malignant) cells begin to grow in the tissues of your liver.
A far more common type of liver cancer secondary liver cancer occurs when cancer cells spread (metastasize) to the liver from other parts of the body.
The liver is especially vulnerable to invasion by tumor cells and with the exception of the lymph nodes, is the most common site of metastasis.
Liver cancer is rarely discovered early. Because of the delay in detecting HCC the prognosis is often poor.
There are treatments which can help relieve symptoms and improve quality of life. In addition to standard treatments such as surgery, chemotherapy and radiation, new and less invasive therapies may be an option for some people.
However it must be kept in mind that many of these treatments use chemical toxins which are known to be contraindicated for AIP patients due to their ability to trigger porphyria attacks.
For PCT patients, the risk of HCC can greatly be reduced for developing the disease by receiving a vaccine that protects you from the hepatitis B virus (HBV).
Hepatitis B is a highly contagious liver infection and one of the leading causes of liver cancer.
Also preventive lifestyle changes can help prevent other major causes of HCC, such as hepatitis C and cirrhosis.

Rosemary Jenkins MSN, FNP
Hematology & Oncology

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 confusion 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
Intensive Care


Analgesics are presecribed for giving effective pain relief.
At least that is the theory.
Often porphyrics still endure a life of ongoing pain.
Analgesics can be classified by the site of their action.
There are three types: [1] centrally acting; [2] peripherally actng; and [3] locally acting.
The centrally acting analgesics include both opiod analgesics and non-narcotic agents such as Tramadol a.k.a. Ultram.
In porphyria we must avoid the Tramadol/Ultram non-narcotic pain medication because of the side effects of seizures and being noted by some as a "trigger" for acute attacks.
It is also contraindicated with the use of some medications for seizures, muscle relaxants, pain and nausea medications.
It must be said that no one drug is perfect.
Every drug known has their benefits and at the same time has some ricks involved with its use.
Healthcare medical providers must make a determination which drug to use in any given situation.
Often drugs are given to a patient and the medical provider then assesses the individual patient's reaction to a specific drug.
With the porphyric patient, it is much better to use drugs that have been approved for a period of no less than five years.
The reason for this is that it takes a couple of years to assess the general problems with any new pharmaceutical product.
Porphyrics by the very nature of their disease need to be ever mindful of the use of drugs and double check all information on any drug prescribed for them whether it be oral, suppository, injection or intravenous.
The majority of drugs on the market today are newer drugs, and each years countless numbers of new drugs and especially drug samples are left with medical care providers to give to patients to try out.
Be careful of such medications.
Ask for and demand to use pharmaceuticals known to be safe for porphyrics.
Even then, because each person is different and has different sensitivities, a "safe" drug can not be tolerated by everone. Regardless of whether one uses a non-narcotic, an opiod, or NSAID, and informed decision making in the prescribing of such drugs requires an understanding of the pharmacology, efficiacy and more importantly, the safety profile of these agents.
Every porphyric patient should familiarize themselves with the Drugs List by Dr. Michael Moore.
In addition to his endless list of drug names, one would be advised to state the variables of each drug name, whether it be the generic, brand, trade or classification name of a drug.
BuSpar for instance is known as busprione. It does not appear on any drug list, unsafe or safe.
However the classification is such that one would refuse to take the drug. BuSpar is an antianxiety drug, a sedative.
Most drugs dealing with the mental abnormalities such as anxiety, or insomnia are unsafe for porphyrics.
For pain associated with inflammation an NSAID is more often prescribed.

Rodney Graham PhD, RPhm
Pharmacology & Toxicology

Porphyria Cutaneous Tardea [PCT] & Blistering

In the PCT type of porphyria the most common skin problem is that of vesicles which are otherwise known as blisters.
A vesicle is a thin-walled sac filled with a fluid.
The fluid is usually clear and ranging in size from pinpoint to 10 millimeters in diameter. As a rule, the term vesicle is usually used to describe small blisters, while the term bullae is used to describe larger blisters.
Moreover the vesicle is an important term used to describe the appearance of many rashes that typically consist of or begin with tiny-to-small fluid-filled blisters.
Other typical illnesses beside porphyria cutaneous tarda with the onset of vesicular eruptions include the common cold sore, or that of shingles (herpes zoster), and chicken pox.
PCT has the appearance of area or patches or otherwise called crops of vesicles.
Because of the similar crops of vesicles found in other more common dermatological presentations, it sometimes makes a diagnosis of PCT hard when based upon the skin appearance alone.
Contact dermatitis may first show up with tiny vesicles that itch or burn.
A typical example of contact dermatitis would be something like poison ivy.
Such dermatitis begins with tiny vesicles that enlarge rapidly, rupture, ooze for a period of time, and finally crust over and heal.
In PCT these eruptions often lead to massive scarring.
In PCT the skin remains thin and fragile and will easily break.
It is especially important to avoid pharmaceuticals known to trigger PCT as well as avoiiding exposure to triggering chemical toxins, sunlight and uv lighting.

Marilyn Geneseo MNS, NP-C