Make your own free website on

Porphyria Educational Services
Monthly Newsletter
March 2003

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.

Fuzzy Blurry Eyes Experienced by Many Porphyrics

Fuzzy eyes are often a complaint in the porphyrias involving the CNS. Some clinicians will go so far as to diagnose the "fuzzy eyes" as optic neuritis.

Most fuzzy eye problems in porphyria are basical vision abnormalities which in some cases have presented as temporary blindness, decreased vision, or termed impaired vision or diminished vision.

Fuzzy eyes are the loss of visual acuity or sharpness resulting in unclear visual details.

Vision loss is the inability to perceive visual stimuli. Often patients will liken the fuzzy eyes as watching the snow on television once a channel has left the airwaves.

The spottiness associated with fuzzy eyes are thought to be dark "holes" in the visual field in which nothing can be seen. Such spots become noticeable to the patient when the spot is enlarging.

Things for the porphyria patient to remember is that any loss of visual acuity, should always be evaluated as soon as possible by a medical profesional. This holds true of any changes in vision, or decreased light perception. Fuzzy eyes is thus a real warning that needs to be clinically evaluated.

It is important that when you see an eye specialist that you inform the physician of your diagnosis of porphyria. Other considerations that need to be addressed are any family history of eye diseases, and any history of diabetes.

Diabetic retinopathyis is always a real possibility with hepatic porphyria patients as a result of continued high carbohydrate intake.

Migraines frequentl;y are associated with porphyria patients and likewise often are the cause of fuzzy eye problems.

Any secondary congenital disorders to the porphyria should also be noted.

Some pharmaceuticals are also known to cause fuzzy eyes. Such drugs include the common phenothiazines, thiazide diuretics, antihistamines, reserpine, and chloroquine.

One of the things porphyria patients can do to help themselves with the fuzzy eye problems as well as depression, mood change and general weakness is to increase the amount of light in a room. This does not mean sitting directly in the sunlight of a window, but allowing the light to illuminate the room. This not only allows a person to more fully see, but lifts the spirits and causes a person to feel more physically capable.

Be sure to obtain copies of your visit to an eye specialists and share a copy with your primary care physician as well as keeping a copy in your personal records for future reference.

G.A. Brown, P.A.
Ophthalmology & Optometry

Liver Function Tests are Essential in Most Porphyrias

Abnormal levels of hepatic enzymes including ALP may be found in up to 6% in normally healthy adult individuals. Liver disease will be found only in about 1% of the general population.

In most of the porphyrias liver enzyme testing will be found to be elevated in one if not all of the results.

Liver function tests or liver anzyme testing, is often used to describe a panel of laboratory measures of a variety of hepatic enzymes, including serum aminotransferase (aspartate aminotransferase or AST and alanine aminotransferase or ALT) and alkaline phosphatase (ALP).

ALP elevation can be seen in infectious mononucleosis, bile duct obstruction, hepatitis, heavy alcohol consumption, and fatty liver. (NASH) as well as the hepatic porphyrias.

Rather than a measure of hepatic function, these tests evaluate hepatocyte integrity, as serum levels of these enzymes rise in response to a variety of forms of injury to hepatic cells.

In liver anzyme testing, like every other form of testing in the porphyrias minor elevations often occurs because of improper handling of specimen and delayed testing.

For ALP tests, the ALP may increase in a sample by as much as 5% to 10% after 4 hours of storage. Timing of the testing is essential as well as the handling of the test collection and the assaying of the collection.

When an individual has a confirmed diagnosis of a hepatic porphyria, even if the liver enzymes have fallen back into the normal range after intervention treatment for porphyria, ongoing monitoring and a repeat measurement in 6-12 months should be undertaken routinely.

SGOT, GGT usually parallels changes in ALP in liver disease. The ALP (alkaline phosphatase) along with an elevation in the GGT confirm the likelihood of a hepatic disorder and is often found as such in the diagnosis of the hepatic porphyias.

A common cause of elevations include drug or alcohol-induced liver disease and fatty liver. The alkaline phosphatase usually returns to normal by the end of 10 weeks after triggering drugs have been discontinued.

George Lewis, Ph.D.

Sulfite Sensitivity in Porphyria Patients

Sulfites are sulfur based preservatives. Such sulfites are most generally used to prevent or reduce discoloration of fruits and vegetables. Sulphites are also found in wine. In addition, sulphites are used to prevent mildew and also to inhibit the growth of microorganisms in fermented foods.

It is known that many people even among normally healthy individuals are sulfite-sensitive. This has long been known in association with asthma patients, but is being realized within the porphyria population as well as those with Multi-Chemical Sensitivities.

There is no specific onset time for a person to become sulfite-sensitive. A person can develop sulfite sensitivity at different times in life. Moreover to date medical science does not yet know what triggers the sensitivity.

Sulphites can also be found elsewhere in one's home. Sulfites are also used to bleach food starches,as well as being used to prevent rust and scale in pipes and boiler water that is used to steam food. Food wrap is another place that sulphites are found.

People who are sensitive to sulfites experienced difference reactions. The primary reactions is difficulty in breathing.

In the porphyria patient, the exacerbation can be much worse. Sulfites give off the gas sulfur dioxide, which can cause irritation in the lungs. Often the breathing problems associated with this if coupled with other triggers can caused various negative reactions in porphyrics. Such reactions include nausea, constriction of the chest, bulbar paralysis, abdominal pain, hives. Some patients experience anaphylactic shock.

Poprhyria patients should be aware that sulfites have a variety of names including potassium metabisulfite, sodium sulfite, sulfur dioxide, potassium bisulfite, sodium bisulfite, sodium metabisulfite. All of the aforementioned are dry chemical forms of the sulfur dioxide, a gas.

Rod Stephens, Ph.D.
Bio-Chemistry & Toxicology

Nausea and Vomiting Key Symptoms in Acute Attacks

Abdominal pain in the acute porphyrias is the most commonly experienced symptom, and most often it is closely followed with nausea and then vomiting.

When a person is showing signs of a pending porphyria attack quite often they will notice stomach upset, sometimes feel a little light-headed, a tinge of nausea which will later turn into vomiting.

Nausea is the sensation leading to the urgency to vomit. To vomit is to force the contents of the stomach up through the esophagus and out of the mouth. Women often refer to the sensations as a series of contractions

In porphyria one of the biggest problems stemming from vomiting and/or diarrahea is that of dehydration...Dehydration is the biggest concern in most vomiting episodes.

The rate with which dehydration takes place depends on a number of various factors. One factor is the size of the person, and another factor is the frequency of the vomiting. In cases where diarrhea is also present, the rate of dehydration will increase.

Signs of dehydration are increased thirst, infrequent urination or dark yellow urine, dry mouth, eyes that appear sunken, crying without tears, and skin that has lost its normal elasticity.

When the cause of nausea and vomiting is known, it is important to treat the underlying disorder. In porphyria it is most often the porphyria attack in it's onset stage.

Whatever the cause, it is important for the person who has vomited a great deal to take in as much fluid as possible without upsetting the stomach any further. Sip clear fluids such as water, ginger ale, fruit juices, or Gatorade. In porphyria it is also important to have the electrolytes checked because often potassium levels will fall below normal levels. Electrolytes can be checked in a simple blood test such as a CBC including electrolytes.

For some porphyria patients who have continuously low levels of potassium during attacks it is important to have potassium supplementation along with the glucose or other intravenous fluids.

Many porphyria patients with a history of vomiting during onset of attacks elect to control nausea and use compazine or zofran as an intervention during times of probably acute attacks.

Leslie Sinclaire, MNS, NP
Hydration Therapy

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.

OXYCODAN is the brand name for the generic drug OXYCODONE.This drug can produce drug dependence of the morphine type The administration of Percodan or other narcotics may obscure the diagnosis or clinical course in patients with acute abdominal conditions such as a porphyric attack or other medical conditions.

OXYCODAN should be given with caution to patients such as the elderly or debilitated, with impairment of hepatic or renal function,or/and hypothyroidism. The drug is metabolized through the liver.

PROLIXIN is the brand name for the generic drug FLUPHENAZINE HCU 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.