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Porphyria Educational Services
Monthly Newsletter
May 2001 Edition




Disclaimer

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.

Columnists 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 Educations Services in no way shall be held responsible in part or whole
for any injury, misinformation, neglience, or loss incurred by you. In reading the monthly newsletters you
need to agree not to hold liable any contributing writers.




FOCUS: Hyperpigmentation in Cutaneous Porphyrias

Hyperpigmentation in the cutaneous porphyrias may be experienced by some and not by others porphyria patients. It is not common place among all of the cutaneous porphyrias, however it is often found in more than one type.

And what is hyperpigmentation?

Hyperpigmentation is the abnormal darkening of an area of skin. Usually it appears in an area which is exposed to sunlight.

Such a condition can be inherited in the porphyrias. If one is prone to hyperpigmentation they need to take the precaution to avoid direct sunlight or uv lighting which will cause the abnormal darkening of the skin to worsen, although such pigmentation is not caused by the effects of sun itself.

Some pharmaceutical are known to worsen the condition. One class of drugs are those in the family of phenothiazines.

The human body's skin normally contains cells called melanocytes. These melanocytes are what cause the brown skin-coloring pigment melanin. In the cutaneous porphyrias the condition is present in which the melanocytes are abnormal. A patch of skin bearing hyperpigmentation is a darker a darker area meaning that it has more active melanocytes.

Some pigment changes spontaneously return to normal skin color except where that has been a scarring of the skin. There are some OTC nonprescription depigmenting creams are available for lightening the skin. Be sure to check out the ingredients because some ingredients may be contraindicated for porphyria.

Many porphyriacs will use cosmetics in covering a discoloration.

Ann Robertson NP
Dermatology

FOCUS: Enzymes and Porphyria

Enzymes play a significant role in the metabolic inherited disease porphyria. Enzyme are in fact important in all aspects of anything living.

Enzymes occur when chemical bonds are made or broken as atoms, ions, or molecules collide with each other and change occurs within the cell. The chemical reaction cannot occur without the help of a specialized group of proteins, known as enzymes. Yes enzymes are proteins that are found in every cell division within living things.

In porphyria it is the mutations of certain enzymes that cause changes in the heme biopathway. From this come weaknesses which give way to attacks of porphyria.

Enzymes are known as the "reaction catalysts of biological systems." And just what is a catalyst?

A catalyst is defined as "a substance that speeds up the rate of a chemical reaction without being changed by the process.

This chemical reaction is very important in the human body. The need for catalysts in the human body is important because the body operates at a temperature and pressure that is too low for the chemical's reactions to occur at a rate rapid enough to maintain life.

It is through the presence of enzymes that the reaction can speed up. It does this by increasing the frequency of collision, lowering the activation energy, and properly orienting the colliding molecules.

Then in turn, the chemical reaction takes place.

The majority of enzymes consist of a protein portion, known as the apo enzyme, and a nonprotein portion called a cofactor. And what are cofactors?
Cofactors can be such things as metal ions, such as iron, cells, and, of course, blood cells. Each type of cell is unique in its construction, but all contain organelles, such as the nucleus or mitochondria, which perform specific functions geared toward the construction and operation of the cell.

It is most to note that the smallest level of structure in the body is the chemical level, which includes all the atoms and molecules essential for life. Carbon, hydrogen, oxygen, nitrogen, potassium, sodium, and calcium are some examples of the types of atoms which create and maintain life from the chemical level.

Atoms combine to form molecules, such as vitamins, fats, carbohydrates, and proteins. These molecules, in turn, combine to form structures at the cellular level.

Almost every function within the cell involves a protein. There are thousands of different kinds of proteins and each performs a specific function, such as providing the structure of the cell or the capability of movement for the cell. Proteins are also important for their function in chemical reactions.

Through the study of enzymes there is hope one day for gene therapy in the treatment and hopefully a cure for porphyria.


David Wilson, PhD
Biochemist & Genetics


FOCUS: Drugs: What's in a Name?
When it comes to drugs, what is the difference between a "brand" name drug and a "generic" drug? And what is a drug classification?

Drugs are complex substances, and they may have as many as three different names, as well as a classification.

The three various names of drugs are known as [1] chemical, [2] generic, and [3] brand. Most people will find the chemical names are long and difficult to pronounce.

F.D.A [The U.S. Food and Drug Administration] following a series of clinical trials finally approves the generic form of a drug. Variations on the "generic" name or shortened forms of the a “generic" names are usually known by are the "brand" names. The. pharmaceutical companies give their products brand names.

An example we could use here is N-(4-hydroxyphenyl) acetamide. This long name is the chemical name for acetaminophen, a common OTC [over-the-counter] medication. Acetaminophen is the generic name for Tylenol of which most people are familiar with.

Many nonprescription and prescription pain relievers are available under both generic and brand names. Your doctor or pharmacist can tell you the generic name.

Generic pharmaceutical products tend to be less expensive than brand-name drugs. Studies have shown that generic drugs are usually just as effective as the more expensive "brand" name drug..

One of the things that porphyria patients must realize however is that there are differences in manufacturing methods between the "generic" and the "brand" name drug.

Drugs with the same "generic" name produced by different companies may differ in the way they are absorbed by the body. The reason for some of this is the use of different ingredients called "fillers" and "binders".

For this reason, your doctor may prefer that you take a brand-name drug. You might want to ask your doctor or pharmacist if you can use a less expensive medication. However, be sure to ask a complete listing of ingredients. Some people can not tolerate even the coating of a pill.

The majority of drug stores are careful to obtain high-quality generic products. Therefore it is sometimes possible to make substitutions, however be sure to double check the ingredients.

And what of classifications?

The classification of a drug is according to what it is suppose to do for you. Examples are anti-depressants, pain relief, antibiotics, topical anti-fungals and calcium-channel-blockers and many more classifications.

In porphyria certain whole classifications are contraindicated for use by porphyria patients. Be sure to double classifications of drugs as well.

Roy Miller, R.Ph.



Focus: Stress and Porphyria

It has been cited in many medical writing on porphyria that stress is a potential trigger of an acute attack of porphyria.

Today, more than ever, medical research is proving that stress can and does cause even a normally healthy person to become ill. How much truer is this for a person with a acute hepatic porphyria.

It has been long known that persons with a stressful job or family life can lead a person to experiencing restlessness or sleeplessness. With this comes a chronic fatigue and often can lead to depression, body weakness, and a overall blah feeling.

Recent medical and scientific research has shown that stress can affect everything from the healing of wounds to the effectiveness of certain vaccines including the annual influenza vaccines.

The fact that medical researchers are undertaking studies in regard to stress shows the extent to which scientific thinking about the effects of stress has changed in the past few years.

For many years doctors and scientists dismissed the idea that stress could make you sick. Then as scientific studies were undertaken researchers are able to prove the very point that stress does make us sick.

Medical researchers can now measure the effects of stress hormones on the immune system. It can be proven that certain stressful events can trigger the release of a outflow of hormones and other chemicals that cause your heart to beat faster, and a person's attention to become more focused and at the same time a person's vision to become clearer.

Along this same happening, if you prolong the stress, by being unable to control it or making it too potent or long-lived, these hormones and chemicals will continue to pump out from nerves and glands. When this happens then the same molecules that mobilized you for the short term will now debilitate you.

. This finding is not based on a sole study, but rather on a number of scientific studies.

Strong links between stress and a variety of physical ailments have been found.

Stress has been cited numerous times on lists of porphyria triggers. In porphyria one of the areas besides triggering an acute attack itself, is that of memory impairment

Cognitive skills lessen in porphyria patients especially during attacks and during times of remission if the patient is under constant stress.

It has been long known that stress is linked to heart attacks and hypertension, and can render individuals more susceptible to other ailments.


Dr. Kenneth Carlson
Neuropsychiatric Medicine



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.

DEPRIM 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 porphyria.

NOVOXAPAM is a brand name for the generic drug OXAZEPAM. It belongs to the BENZODIAZEPINE class of drugs. The drug contains sulfates. The drug carries a warning against use in persons with the disease porphyria.

NARDIL is the brand name for the generic drug PHENELZINE SULFATE. It is an antidepressant. The drug contains the ingredient sulfa. The drug is a monoamine oxidase inhibitor {MAOI] and is often used to treat depression. his drug is not recommended for persons with thyroid problems, diabetes, seizures, kidney or liver disease.