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.
SUICIDE STILL PREVALENT AMONG PORPHYRICS
Throughout much of the known history of porphyria, there have been those
porphyrics who have ended their lives. Most notable of these porphyrics was
of course the famed Vincent Van Gogh. One wonders "what if" pain control had
been available for Van Gogh, his
remarkable career as an artist would not
have been cut short.
His problem of course was that when he went into a poprhyic attack, he would
drink a thimble of absinthe to dull the severity of the pain. And it may for a
short time, but at the same time was the "trigger" for another acute attack of
porphyria. It happened so often that Van Gogh was chronic and no longer just
a smoldering chronic porphyric but in severe chronic pain.
Finally, unable to handle his condition he commited suicide.
Just at the end of this last summer one young woman, both a wife and mother,
while visiting with family in Europe, was unable to obtain adequate pain control
in an ever progressing VP form of porphyria. Having lost a child from
porphyria complications during pregnancy, and then having another child
diagnosed with porphyria as an infant and still another high suspected of having
VP, and herself progressively worsening and then unable to receive proper medical
treatment, ended her life tragically.
Suicide is NOT a treatment for anything. This same thought was stated
about five years ago in an article in a California mainline newspaper
publication where the "right to die" was being discussed.
Debate started in the California legislature regarding physician-assisted suicide
with the introduction of Assembly Bill 1592. This got all kinds of people into
While all of the protesting was going on, one wonders how much emphasis was
placed on looking at the reasons behind suicide. Lack of pain control has been
one major factor throughout the passage of history.
Rather than debate such issues to end life, it should rather be the issue of
requiring physicians to include courses that familiarize them with the disease
porphyria and addressing pain and symptom management of the various types of
As a former clinician, I am still amazed at how little knowledge is really shared
about what in my day were considered rare diseases, but obviously are by no means
Another issue that needs to be stressed is to be sure that pain medications are
readily available to porphyria patients in pain. Legislation which was passed and
effective January 2001, makes it clear that pain must be addressed and treated
in patients. Legislation allows for a physician caring for porphyric patients to
prescribe appropriate dosages of medications without fear of the wrath of the
law enforcement officials intent on the waging of war on drugs.
Laws have been enacted to allow access to pain medications and reduced the
inefficiancy of the triplicate prescription process for administering drugs to
terminally ill patients. Those suffering from a terminal illness are no longer
subject to long delays in approval of non-formulary medications. In Europe some
countries have mandated the treatment of pain, while most unfortunately have
However, porphyrics are not terminal, but many days it seems as if we wish the
porphyria were terminal. It is not. And suicide is not an answer.
We must be sure that legislation is made that will enable our physicians to
prescribe the medications that we need in order to live a better quality of
All concerned parties should work together for the behalf of porphyrics and
continue down the humanitarian path of treating pain in suffering individuals
both porphyrics and others in severe pain. No porphyria patients in the
United States should ever be dismissed from medical care without proper
treatment options for controlling their pain.
Robert Johnson M.D.
ELECTROLYTE BALANCE ESSENTIAL FOR PORPHYRIA PATIENTS
Electrolytes are very essential to porphyria patients and especially during
an acute attack.
One may ask what are "electrolytes"?
An "electrolyte": is a substance that when dissolved in water conducts an
What makes up the electrolytes?
The normal electrolytes include sodium, chloride, potassium as well as calcium
and magnesium and other trace elements.
When a porphyric loses their electrolyte balance small electric shocks sent
through the nervous system signal changes ahead. Many of these changes
exascebate into changes in our mental well being. They are also thought to
contribute to seizure activity, and muscle spasms.
Various disturbances of fluid and electrolyte balance are seen during the acute
attack. Dehydration may occur, owing to persistent vomiting. Hyponatraemia,
secondary to inappropriate antidiuretic hormone secretion, may also occur,
sometimes first becoming apparent after commencing intravenous fluids.
Hyponatraemia can usually be controlled by restricting fluid intake.
To maintain adequate carbohydrate intake while restricting fluid intake,
it may be necessary to use higher concentrations of glucose, administered via
a central venous line.
When beginning the onset of an acute attack of porphyria it is important to
safeguiard oneself by avoiding the nausea and vomiting by the use of safe
suppositories to avoid the loss of them. The most commonly use and known as
safe for porphyrics is that of compazine.
Potassium is another factor and if potassium is falling in lab tests then an
added bag of iv potassium along with the glucose can be administered. When in
remission potassium can be easily restored to proper levels by the oral intake
of potassium supplements as prescribed by your physician.
Consuming water or clear liquids is also essential during an acute attack.
It is good to remember that cold water is the best, but not ice water.
Cold water instead of ice water is good not only for taste, but the cold water
is absorbed into the system more rapidly.
Electrolytes are very important to porphyric patients and they should be sure
to have them checked regularly and at the beginning of any acute porphyric
attack in order to avoid the unnecessary mental changes and other neurological
changes that can occur.
Patrica Johnson MNS
Medical Charge Nurse
HEPATITUS AND PCT
Hepatitus, an inflammation of the liver, is often associated with porphyria
cutaneous tarda (PCT).
Viral hepatitis, a contagious infection of the liver, afflicts more than 70,000
Americans each year. It is usually caused by one of three different organisms.
Hepatitis A, formerly known as infectious hepatitis, can be contracted by
consuming contaminated water or food, most notably shellfish. Since the virus
is eliminated in the stool, it also spreads through improper hand washing,
especially by restaurant workers or anyone else who handles food.
Although hepatitis A is seldom serious, in one percent of the cases it can
cause severe liver failure and death. It does not cause chronic hepatitis
and will not lead to cirrhosis or other long-term liver problems. This can be
serious for one who all ready has an illness associated with the liver, as some
forms of porphyria are considered.
Hepatitis B, formerly known as serum hepatitis, is found in blood and other
body fluids such as urine, tears, semen, breast milk, and vaginal secretions.
It is usually transmitted in blood, via transfusions, or through illicit
injectable-drug use. But it also can be contracted through a minor cut or
abrasion, or during such everyday acts as toothbrushing, kissing, or having
Infants can contract the disease from the mother at birth, or from
the mother's breast milk. Dental work, ear piercing, and tattooing are other
ways people can get hepatitis B.
Type C hepatitis virus is the cause of a disease known as "non-A, non-B
hepatitis," which is also contracted through contact with contaminated blood,
or through household or sexual contact with an infected person. It affects
approximately 170,000 Americans each year.
This form of hepatitus is most often associated with PCT.
The problem with hepatitis B is that five to 10 percent of those who become
infected with this disease become chronic carriers who can spread it to others
for an indefinite period of time.
At present there are more than a million of these silent carriers in this
country, and their number is growing by two to three percent annually.
Consequently, authorities recommend that all children and anyone with a
high risk of exposure be vaccinated against this dangerous virus.
Chronic carriers usually do not develop chronic hepatitis. If it does develop,
however, cirrhosis and primary cancer of the liver can be long-term
An estimated 4,000 people in the United States die from hepatitis B-related
cirrhosis annually. Carriers are many times more likely to get liver cancer
than are non-carriers.
Treatment for acute hepatitis consists of rest and small, nourishing meals;
fluids; and sometimes anti-nausea drugs such as trimethobenzamide (Tigan).
Chronic cases of hepatitis B and C are now being treated with interferon,
a biotech medicine derived from the human immune system.
For PCT patients who have the hepatitus in addition to the porphyria, it is
most necessary to have adequate medical treatment and proper care of the liver.
Sheila Brandt NP
ABDOMINAL PAIN AND AIP
It has been stated in various listings of statistics on the acute porphyria
and especially in regard to AIP that 90% experience abdominal pain.
Unexplained abdominal pain still remains one of the most constant indicators
Back in 1957 in a study by Waldenstrom 85% of the porphyria patients indicated
Two years later in 1959 Goldberg found in his study that 94 % of the porphyria
patients indicated abdominal pain.
Twenty years later, in 1979 Drs. Stein and Tschudy found that 95% of all
porphyria patients indicate having the abominal pain.
Chest, back, and limb pain may also occur either in the presence of or
absence of abdominal pain.
While not everyone experiences abdominal pain, and while it may not be
present with every acute attack, abdominal pain stil remains one of the
major symptoms of acute intermittent porphyria.
Dr. Robert Johnson
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.
Diflucan is the brand name of the generic drug Fluconazole.
Used for the treatment of candida it has been known to cause serious liver
disease. It is thought to be unsafe for persons with porphyria.
Avandia is the brand name for the generic drug Rosiglitazone Maleate
which is a new diabetes drug. This drug carries warnings for persons with a
history of heart failure and liver disease.
Levaquinis the brand name for the generic drug Levofloxin which
is a Fluoroquinolone. That is the same classification as Coprofloxin,
Norafloxin, Trovan, Penetrex, etc.
Coprofloxin is the mildest of this classification and Levofloxin is next.
It does carry the warning/precaution for people with liver or kidney disease,
but is not known to have caused any attacks. It is one of the more commonly
prescribed antibacterials used for UTIs, soft tissue infection and pneumonia
Celexa is the brand name for the generic drug Citalopram.
In vitro studies using human liver microsomes indicated that CYP3A4 and
CYP2C19 are the primary isozymes involved in the N-demethylation of
citalopram [Celexa].The antidepressant action of Celexa in hospitalized
depressed patients has not been adequately studied. Clinical experience
with Celexa in patients with certain concomitant systemic illnesses is
limited. Caution is advisable in using Celexa in patients with diseases or
conditions that produce altered metabolism or hemodynamic responses.
The use of Celexa in hepatically impaired patients should be approached with
caution. Until adequate numbers of patients with severe renal impairment have
been evaluated during chronic treatment with Celexa, however, it should be
used with caution in such patients