Make your own free website on

Porphyria Educational Services
Monthly Newsletter
September 2002

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 is a an important focus in the hepatic types of acute porphyria as well as some of the cutaneous forms

The liver is the main organ that keeps a person alive. It is said of the liver that it performs over 100 separate bodily functions. One of these functions is the regulation of the production of porphyrins.

The sheer complexity of the liver makes it susceptible to almost as many different diseases.

Many of these diseases are rare, or like some types of porphyria, not rare, but hardly common either.

Of the common liver diseases some are "household names" that are all too common, including hepatitis, cirrhosis, liver disorders in children, alcohol-related disorders, and liver cancer.

In a report published in a medical journal in 1998, it states that over 25 million people are afflicted with liver and gallbladder disease each year. Furthermore the report states that over 27,000 Americans die from cirrhosis annually. With a mortality census like this, it makes cirrhosis of the liver the third leading cause of death for people between the ages of 25 and 59, and the seventh leading cause of death overall in the United States today.

Viruses, hereditary defects such as porphyria, and reactions to drugs and chemicals are among the known causes of liver breakdown.

Though few treatments are effective for life-threatening liver disease, avoiding alcohol and other substances known to cause damage can do a lot to safeguard this important organ.

Many drugs are unsafe for use in people with liver disease. This is especially true of the the drugs that are known as Cytochrome P-450 drugs.

It is vital to read up on any pharmaceutical before taking it, even if your doctor has presecribed it for you. This is especially true in porphyria. If a drug is metabolized in the liver, you do not want to use it.

Porphyrins are manufactured in the liver. With increased acute attacks or what some call chronic porphyria, the liver may be damaged more readily. Avoiding drugs is imperative. Seeking immediate Intervention Therapy is also imperative to reduce the risk of developing porphyrin crystallization in the liver

The liver converts carbohydrates, fats, and proteins into chemicals essential for life and growth. It manufactures and exports to other organs some of the substances they need to function properly, such as the bile used by the intestines during digestion. The liver caaries out the function of "metabolizing".

It modifies drugs taken to treat disease so that they can be used more easily by the body. And it cleanses the blood of toxic substances either ingested or produced by the body iself.

In porphyria patients, it is necessary to be knowledgeable about one's liver and safeguard the liver from harmful acute attacks, drugs, and other chemical and environmental toxins.

Jerry Schultz PA


Electro encephalograms are commonly known as an EEG. EEG's are used for diagnostic purposes in many medical conditions, but not in porphyria.

In porphyria patients the EEG is not a test for peripheral neuropathy but is carried out to exclude other causes of similar symptoms.

The EEG measures the electrical activity of your brain, as a series of brain waves recorded by an instrument as paper traces. It can display the general state of arousal of different parts of the brain. It can also show areas of the brain that are not functioning correctly either because of too little or too much electrical activity.

Several electrodes will be attached to your scalp using adhesive paste. The test may take up to an hour to complete. It is painless. There are generally no risks involved with an EEG.

Lance Driscoll, MT


Constipation is the number one gastrointestinal complaint in the United States. In a report published during the millenium, constipation accounted for more than two million physician visits a year. In another published reports, it was stated that in the United States alone $725 million a year is spent on over-the-counter laxatives.

For most porphyria patients constipation is a major problem especially during acute attacks, although many find the problem of constipation still lingering during their periods of remission.
Br> By keeping the stool soft and bulky, the fiber in intact grains helps prevent this troubling problem.

Fiber from cereals, as well as from fruits and vegetables, adds bulk to the stool and softens it. Together, these actions decrease pressure inside the intestinal tract and help prevent disease which may perforate the lining of the stomach, colon and bowel.

Eating whole grain products helps keep the body's sugar control system in proper balance. It has been documented that whole grains protect against diabetes. In porphyria patients who need to consume large quanitities of high carbohydrate food, often their intake of sugars is high making them prime targets for diabetes. Consumption of whole grain which are also high in carbohydrate can help to stave off porphyrin overproduction and at the same time lower risks of diabetes by eliminating the consumption of high amounts of sugar when the whole grain will do the job.

Sheryl Wilson MSN, RD

Br> The generic drug Gabapentin which is also known as the brand drug Neurontin, is fast becoming the leading prescribed medication for control of seizures as well as pain especially in patients with metabolic diseases and some inherited genetic diseases.

Gabapentin for the most part is the only known safe anti-seizure medication for use in porphyria patients.

Some of the other anti-seizure or anti-convulsants as they may be classified, have been effective in some porphyria patients, yet at the same time have proven themselves very unsafe for use in a number of other porphyria patients. Some of these drugs include carbamazepine, phenytoin, and valproate. In normally healthy patients these drugs are effective for the treatment of tremor as well as pain.

In studies carried out with the use of Gabapentin results showed that the majority of patients had moderate-to-excellent improvement in symptoms, with the best responses occurring in patients with peripherally mediated neuropathic pain.Most pain in porphyria patients is deemed neuropathic pain.

Other tremor conditions treated that showed some improvement through the use of Gabapentin were benign essential/familial tremor, restless legs syndrome, centrally mediated pain, and periodic nighttime leg movements.

Gabapentin / Neurontin offers an effective, safe alternative therapy for porphyria patients. It is effective in controlling both tremor and for providing some pain relief.

Another key point about Gabapentin / Neurontin is that the drug does not affect the metabolism of other medications and is well tolerated.

David Twiddle RPh

Monthly Drug Update

PES drug information does not endorse drugs, diagnose patients or recommend therapy. PES drug information is a reference resource designed as a supplement to, and not a substitute for, the expertise, skill, knowledge and judgement of healthcare practitioners in patient care. The absence of a warning for a given drug or drug combination in no way should be construed to indicate that the drug or drug combination is safe, effective or appropriate for any given patient.

PAXIL is the brand name for the generic drug PAROXETINE HYDROCHLORIDE. It is used to treat social anxiety disorder and is classified as an antidepressant. This drug is not recommended for persons with liver impairment as this drug is metabolized through the liver.

PROLIXIN is the brand name for the generic drug FLUPHENAZINE HCL which is in the drug class called a TRANQUILIZER. PROLIXIN is a trifluoro-methyl phenothiazine derivative. This drug runs the risk of Tardive Dyskinesia [TD] with prolonged use. Also this drug runs the risk of Neuroleptic Malignant Syndrome [NMS] which is potentially fatal. Best not to be used by persons with convulsive disorders. A phenothiazine classification drug. Not recommended for persons with renal or hepatic disease. PROLIXIN is metabolized through the liver.

SERTALINE is the generic name for the brand name drug ZOLOFT. It is used for major depressive disorder and depression. It is contraindicated in patients with hepatic and renal insufficiency, and should not be used by persons with liver disease.

UROPLUS is a brand name for the generic drug combination of SULFAMETHOXAZOLE and TRIMETHOPRIM. It contains sulfa as an ingredient. The drug carries a warning against use in persons with the disease porphyria.