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Porphyria Educational Services
Monthly Newsletter
July 2004

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.

Electrolytes Essential in Porphyria

Electrolytes are very essential to a 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 electric current.
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 in 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 one'sself 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

Abdominal Pain in Acute Porphyria

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 of porphyria. Back in 1957 in a study by Waldenstrom 85% of the porphyria patients indicated abdominal pain.
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
Retired Clinician

Stress Can Trigger Porphyria

It has long been known that stress and pain go together. In fact one could say that stress and pain go hand in hand.
When a person is in pain, that person is less able to handle the stress of everyday life.
Often these little hassles of life become complex and overburdening.
Stress then affects the total person. Anxiety can build up. Restlessness or insomnia may occur. Inability to eat may occur. And, then the next thing a person notices is the presence of the telltale signs and symptoms of an acute attack presenting.
Stress may also cause you to do things that intensify your pain. This is especially an importance aspects for porphyria patients dealing with severe chronic porphyric neuropathy.
When people are under stress they will often tense their muscles. The end result is that pain causes stress, and stress intensifies pain.
Porphyric patients need to learn to deal with stress in order to control their pain and at the same time to avoid triggering further acute attacks. A porphyria patient must break the pain-stres cycle.
How a patient responds to an event is crucial. Learn to control things where you can, if it is something that you can control.
Like porphyria itself, for some porphyrics something may be stressful, yet for another porphyria patient, it is not stressful.
Porphyria patients have to take notes and learn how they each individually respond to stress. Remember that when an individual encounters stress, the person's body responds in a manner similar to a physical threat. It automatically gears up to face the challenge or musters the strength necessary to get out of trouble's way.
Stress can be negative or positive. Positive stress provides a feeling of excitement and opportunity. Positive stress often helps individuals better perform in competition. Negative stress occurs when you feel out of control or under constant or intense pressure.
For porphyria patients, often negative stress is closely associated with the hassles of obtaining proper medical care and treatment of the porphyria condition itself.
Other negative stress signs are shown in trouble concentrating, feeling alone, family negativism toward porphyria, financial problems, inability to keep working, isolation,other health problems including pain.
Continued stress can have a negative effect on your health.

Dr. Kenneth Carlson
Neuropsychiatric Medicine


Pharmacogenetics is one of the most beneficial research programs to acute hepatic porphyria patients.
By using the new science that pharmacogenetics offers, the public is more ably to ascertain pharmaceuticals which are known for their safety due to their drug-metabolizing enzyme activity.
Pharmacogenetics can identify single nucleotide polymorphisms (SNPs) in DNA sequences that cause clinically significant alterations in drug-metabolizing enzyme activities.
WHat once was mostly accomplished by "trial and error" in learning which drugs were safely used by acute porphyria patients without triggering attacks, has now been narrowed by the use of pharmacogeneics.
Recent advances in pharmacogenomic research have begun to elucidate the inherited composiiton of interindividual differences in drug-induced adverse reactions, toxicity, and therapeutic responses.
DNA sequences have been identified which explain some of the variability in drug-metabolizing enzyme activities which contribute to alterations in drug clearance and impact patients' response to drug therapy.
Interestingly enough is that it has been found that drug-metabolizing enzymes often differs among ethnic groups.
Continued research in pharmacogenetics will further our understanding in interindividual differences in drug disposition.

Leonard Hraska R Ph
Pharmacology & Toxicology

PES 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 judgment 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.

ORASONE is a brand name for the generic drug PREDNISONE.
SIDE EFFECTS include: Sodium retention, fluid retention, muscle weakness, and abdominal Increases in alanine transaminase (ALT, SGPT), aspartate transaminase (AST, SGOT) and alkaline phosphatase have been observed.
Other side effects include decreased carbohydrate tolerance; manifestations of latent diabetes mellitus. There may be ocular infections.
.Average and large doses can cause elevation of blood pressure, salt and water retention, and increased excretion of potassium.
Persons who are on drugs which suppress the immune system are more susceptible to infections than healthy individuals.
There is an enhanced effect of corticosteroids on patients with hypothyroidism and in those with cirrhosis and liver disease. It is not recommended for persons with liver disease.

AVANDIA is the brand name for ROSIGLITAZONE MALEATE, a thiazolidinedone (TXD) used to treat type II diabetes. This drug carries the WARNING - Not to be used by persons with liver disease.