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Porphyria Educational Services
Monthly Newsletter
April 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.

Reduce Stress & Anxiety in Porphyria

It is known that stress can play a role in a wide variety of medical conditions. Porphyria is no expception!
Medical researchers have been busy the last few years meauring the effects of stress on a variety of medical conditions. Researchers have proven that stress can add up to more than a restless night and pacing of the floor.
Latest studies indicate that stress and anxiety can effect the healing of wounds or how effective a drug will work.
Stress and anxiety effects hormones and those hormones have an effect on the immune system.

When a person becomes stressful that state of anxiety will be the signal to the brain for the release of hormones and other chemicals. Release of these properties will cause a person's heart to beat faster. At the same time a person's attention will become more focused, and eyesight is more focused and clearer. This is all good for a short term state of anxiety and heightened awareness.

But when stress becomes a part of daily life and at times chronic, these hormones and chemicals continue to pump out from nerves and glands and then the effects begin to reverse. The same molecules that mobilized you for the short term become debilitating to the person.

Medical researchers have made studies measuring the effect of stress. Such studies have revealed strong ties between stress and a multitude of physical conditions.

Whether or not stress alone will trigger an acute porphyria attacks, stress from the illness itself causes debilitating effects and often add to a "chronic smoldering" state of porphyria.

Stress and anxiety do not necessitate a desperate, terrible stress levels to see effects on the immune system, and coupled with other manifestations of the CNS can only add to the many and varied problems associated with porphyria such as tachycardia and hpyertension.

Porphyria patients experiencing anxiety and stress should practice a series of relaxation techniques. Visualization techniques can also be quite useful. Walking a treadmill or jogging and other exercise can rlelieve stress.

For those porphyria patients with a high degree of pain, as well as peripheral neuropathy, use of a hot tub for relaxation and stress reduction is encouraged.

Jenny Browning FNP - C
Neuropsychiatric Dept.

Ultrasound Use in Porphyria

As soon as a porphyria patient realizes that they have a medical condition and seeks medical attention, so begins many different medical procedures beyond those of the basic laboratory testing.

Ultrasound is one of the more basic and painless techniques used.
Ultrasound is the simple name for the science of ultrasonography.
Ultrasound is a simple and noninvasive test.
Ultrasound is painless and usually takes less than 30 minutes.
Many aspects of various porphyrias take place in the liver, especially in the hepatic forms of porphyria.
Ultrasound uses sound waves to produce a picture of internal organs, including the liver.
The patient undergoing an ultrasound lies on a bed or examining table. A small a wand-shaped device called a transducer is placed on your body. The transducer emits sound waves that are reflected back from your liver and then transformed into a computer image.
The benefit of ultrasound if that ultrasound is very good at providing information about the shape, texture and makeup of tumors or body masses. This is particular useful in detecting HCC in AIP and PCT patients.

James Seymour PA
Radiology & Nuclear Medicine

Microbial Genomics and Porphyria

One of the major end benefits of the Human Genome Project comes from that of microbial genomics. Through such research it will allow for environmental monitoring to detect pollutants.

Most porphyria patients are very aware of the effect that environmental toxins have upon them. Chemical toxins or neurotoxic substances are often the triggers for acute attacks of porphyria. For those with cutaneous porphyria the toxins enhance their skin rashes, itching, blistering and scarring.

Having genetic knowledge of microbes may increase our ability to use them for industrial purposes which in turn could possibly help in clearing the air of pollutants. or microbes that digest waste products or pollutants.

Genetic research will also lead to better assessment of health damage and risks caused by radiation exposure, including and the health damage and risks caused by exposure to mutagenic chemicals and cancer-causing toxins.

Through such medical and scientific research it can lead to the reduction of the likelihood of damage to DNA, which causes mutations.

In porphyria new mutations are being identified on a regular basis.

The new mutations cause varied responses in testing and diagnosis, as well as treatment. Mutational changes cause different susceptibilities to pharmaceuticals.

Linda Stewart PhD
Genetics & Biochemistry

Understanding Porphyric Neurologic Pain

Pain basically results from a series of exchanges involving three major components: your peripheral nerves, spinal cord and of course your brain.

Pain is a universal experience. All people feel pain.

To the extent to which an individual feels pain and exactly the way this individual reacts to pain, are very individual responses.

Responses to pain, are the results of an individual's own biological, psychological and cultural makeup.

It must be remembered that past encounters with painful injury or illness also can influence an individual's sensitivity to pain.

Unfortunately, when pain persists beyond the time expected for an injury to heal or an illness to end, it can become a chronic condition.

We all know persons with fibromyalgia who suffer with chronic pain. Such can be the case with porphyric neuropathy.

When pain becomes chronic, no longer is the pain viewed as just the symptom of another disease, but as an illness unto itself.

Many porphyria patients find that in addition to the neurologist, they must also seek the professional care of pain management specialists.

The physical sensation of pain comes in many forms: sharp, jabbing, throbbing, burning, stinging, tingling, nagging, dull and aching.

Pain also varies from mild to severe.

Severe pain grabs your attention more quickly and generally produces a greater physical and emotional response than mild pain.Severe pain can also incapacitate you, making it difficult or impossible to sit or stand.

The location of your pain also can affect your response to it.

Remember that your own personal makeup also affect how you interpret you pain and tolerate it.

Your emotional and psychological state, memories of past pain experiences, and your upbringing and attitude also affect how you interpret pain messages and tolerate pain.

There are two types of pain: acute and chronic.

Acute pain is triggered by tissue damage. It's the type of pain that generally accompanies illness, injury or surgery

Acute pain may be mild and last just a moment, or can be severe and last for weeks or months.

Chronic pain hangs on after the injury is healed. Pain is generally described as chronic when it lasts 6 months or longer.

Unlike acute pain, with chronic pain you may not know the reason for the pain. Chronic pain can also occur without any indication of injury.

Frequently, the cause of chronic pain is not well understood. Like other aspects of porphyria, there may be no evidence of disease or damage to your body tissues that doctors can directly link to the pain.

Often chronic pain in porphyria patients is due to the chronic condition of porphyria itself. Such chronic pain produces painful inflammation in your joints and causes aching in your muscles.

Nerve pain that lingers after the original injury heals is called neuropathic. Once damaged, the nerve may send pain messages that are unwarranted.

Doris Bagnette MNS RN
Neurology & Physiatry Department

The Role of Migraines in Porphyria

Porphyria patients often complain about the presence of migraine headaches. Many believe they have suffered migraines as a part of their porphyria acute attack and in some cases separate from the onset of a porphyria attack itself. Currently there is no supportive medical evidence to show a connection between migraines headaches and porphyria itself.

It has been found that diet more than porphyria or other medical conditions, plays a far greater role in a patient having the migraines.

Migraine headaches bring crushing pain to more than 23 million Americans, as cited in various medical publications. Migraines are the chief complaint of many patients seeking medical care in clinical settings.

Migraines can be triggered by certain foods. When starting to identify your triggers of migraines eat suspected foods separately from other suspected foods. Consume a suspected food trigger along with known "safe" foods.

With a little effort it will be possible that avoiding those foods may help you avoid a headache.

Migraine headaches are different and are much more painful than other types of headaches.

Migraine headaches can last from 4 to 72 hours. A migraine is different and more painful than a tension headache you might get after a stressful day. A migraine may include throbbing and sometimes disabling head pain.

Some or all of the following symptoms or signs may accompany the pain of a migraine headache. These include: blurred vision; flashing lights; nausea and vomiting; tingling numbness in your arms or legs; and unusual sensitivity to light, sound or odors.

Exhaust fumes from cars, paint, varnishes, glue, diesel asphalt, tar, adhesives, and perfumes are among the odors which can easily trigger migraine headaches.

Some people with migraines also have food cravings, mood swings, irritability and frequent yawning. Unformatunately many things within the environment can trigger a migraine headache.

It is thought that there may be chemicals in some foods that cause widening of blood vessels in the brains of people who have migraines. It is in this widening, rather than the food itself, that results in migraine pain.

Some known food triggers of migraine headaches include chocolate, caffeine, alcohol, nuts, foods with nitrates, and foods prepared with monosodium glutamate (MSG)

Dr. Robert Johnson
Retired Clinician
Porphyria Investigator

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.

DECORTIN is a brand name for the generic drug PREDNISONE.
SIDE EFFECTS include: Sodium retention, fluid retention, muscle weakness, and abdominal pain. 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.

DIABINESE is a brand name for the generic drug CHLORPROPAMIDE. It is a sulfonylurea.
SIDE EFFECTS: Hypoglycemia, Gastrointestinal disturbances are most common reactions.Porphyria cutanea tarda and photosensitivity reactions have been reported with sulfonylureas. Skin eruptions rarely progressing to erythema multiforme and exfoliative dermatitis have also been reported.
Metabolic REACTIONS - Hepatic porphyria and disulfiram-like reactions have been reported with chlorpropamide.
WARNING: This drug is UNSAFE for porphyria patients