Porphyria Educational Services
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.
THE IMPORTANCE OF THE LIVER IN PORPHYRIA
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
USE OF ENCEPHALOGRAMS IN PORPHYRIA
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
WHOLE GRAINS /FIBER & PORPHYRIC CONSTIPATION
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
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.
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
Sheryl Wilson MSN, RD
GABAPENTIN EFFECTIVE FOR PAIN & TREMOR
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
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