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Porphyria Educational Services
Monthly Newsletter
December 2001

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.

FOCUS: Depression in Porphyria

     One of the major concerns of many porphyria patients is the challenge of depression. In the hepatic porphyrias, the involvement of the CNS [central nervous system] brings with it a lot of different medical maladies. Depression can be one of them.

     Depression is especially prevalent at this time of the year as well.

     In today's society among the normally healthy, depression is a medical condition that costs the USA billions of dollars in lost productivity each year. When combined with a chronic on-going as well as generally unknown condition such as porphyria, there is a total sense of hopelessness experienced by many. This feeling of hopelessness is the medical condition known as depression.

     With porphyria many are forced from their work and into newer life experiences. Such a change often brings with it a certain amount of depression. Such changes in a person's life causes them to face loss of financial security, ability to fulfill lifelong dreams, ability to raise their families as they would like to, and at the same time increasing medical confinements.

     For many people in this position it brings about feelings of worthlessness and they experience a loss of interest in normal activities such as marital sex, work, or hobbies. Some will find a loss in their faith beliefs.

     Depression medically is causes when the brain communicates with itself through the use of special chemicals called neurotransmitters. These neurotransmitters are known as norepinephrine and serotonin. Much has been written the past few years in regard to serotonin. Medical research has shown a strong correlation between the amount of these chemicals in the brain and a person's mood. If levels of these chemicals get too low people feel depressed.

     There are many drugs on the market today which can help with depression in normally healthy people. Unfortunately in the porphyric patient many of the antidepressants must be avoided because of being P-450 and their ability to perhaps trigger an acute attack of porphyria.

     The most common drugs used to treat depression at the Selective Serotonin Reuptake Inhibitors which are commonly known as SSRI's. The other most common group of drugs used are known as the Tricyclic Antidepressants This later drug group has many side effects and have many contraindications for drug interactions.

     In addition there are MAOI's which is the short for monoamine oxidase inhibitors. The MAOI's have many drug interactions as well as bad food interactions. The drug when used by itself is often very effective but again is not considered for use in porphyric patients.

     In the most current Unsafe Drug lists for porphyria patients one will find that Celexa, Zoloft, Paxil, Nardil, Prozac, and Remeron are all listed as unsafe.

     Therapies such as bright light, goal setting, work productivity, music therapy, and using Preventive Glucose porphyria therapy will all help deal with depression along with the traditional psychotherapy.

Dr.Kennth Carlson
Neuropsychiatric Medicine

FOCUS: Perfumes and Sensitivity in Porphyria

     Aromas have been found throughout all of history. In Biblical times various fragrances and oils were rubbed on the skin usually to cover up or mask other less pleasant body odors that were present before daily bathing, soaps, and deodorants became a standard.

     Fragrances still are found in abundance however. They are in fabric softener sheets placed in the automatic dryer with every laundry. They are the aromatic candles that are currently so popular for soothing the mood, or to arouse sexual feelings.

     But those fragrances also carry with them major problems for many people with various medical conditions ranging from asthma and MCS to the porphyrias.

     Today, more than ever before from department stores to supermarkets a person can find a variety of products with fragrance. People seem to be "into" sniffing and buying various fragrance of just about any kind of product. There are all kinds of scents from which to make a selection.

     Many of the common household products or personal use products used today have a scent or fragrance associated with them. Fragrances have become a standard part of our everyday lives.

     A routine day in a normal American lifestyle brings the average person into contact with fragrances repeatedly throughout the day. Often toilet tissue carries a scent.

     Almost every toothpaste and mouthwash on the market has various scents.

     The morning shower or bath will find a person with a fragrance shampoo, hair conditioner, body soap, or shower gel.

     After drying one may use a hair lacquer or hair spray with fragrance, a facial make-up, lotion or hand cream with fragrance. Women have feminine products which also carry a scent. If an infant is in the household there is also the exposure to the fragrance of the diaper wipes, the diapers themselves, the shampoo, lotion and oils used. For men shaving cream and aftershave have their own distinct fragrances.

     As dishes are washed there is another fragrance. Laundry time brings another whole array of fragrances from laundry soap, bleach, to the fabric softener sheets. Window cleanser, toilet bowl cleanser, shower cleansers, bleach, floor cleanser, waxes, vinyl cleansers all have their own scents and add to the heavy overload on scents all ready present. Add to this the aroma therapy of scented candles, air fresheners, and simmering potpourri.

     A trip to an office waiting room will often find a person face to face with a magazine with a scented perfume ad enclosed. Home to read the mail one will often find the scented stationary.

     The upshot of all of this fragrance is that if you are porphyric, asthmatic or have MCS you will often find your eyes watering, your nose dripping, your throat constricting and having a hard time breathing. Sometimes you will discovered red blotches on your skin, burning and itching sensation and feel just plain miserable, all because of fragrance.

     Many people are unaware that fragrance poses a problem to others. Countless numbers of people just think that fragrance is nothing but pleasant and that it is completely harmless.

     Another whole area of fragrance is that of breath sprays or breath mints or gums. Wintergreen is one scent that is especially hard on a large number of people. Some of the mints are likewise bad. Camphor smell has been known to cause some to have nausea and begin vomiting within several minutes.

          Fragrances can and do cause some very negative effects. The problem lies in the fact that fragrances contained a multitude of different chemicals within them. Some estimates place the figure around 5,000 for the different chemicals that are used by the makers of the various fragrances. One perfume alone can contain hundreds of different chemical ingredients. It is these chemicals that people react to.

     Many of the chemical toxins have not been tested. Many are not regulated by the government to be tested for their toxicity. However at least 100 of the basic chemicals used in the making of fragrances are known to be toxic.

     Research studies indicate that scent chemicals are an instrumental cause of many medical complaints. Cutaneous reactions such as rash, itching, hives, sensitivity, redness, and dermatitis can all be attributed to exposure to fragrance.

     Other studies can document pulmonary reaction to fragrances. People with asthma and COPD can and do experience many unpleasant side effects from exposure to fragrances.

     In porphyria some porphyrics report mental confusion, 'brain fog", watery eyes, drippy nose, and respiratory impairment when exposed to heavy scents. Neurotoxicity has been charted by clinicians because of such exposure to fragrance. Other medical conditions noted in porphyria patient charts include depression, respiratory paralysis and lethargy.

     Porphyria patients have reported shortness of breath, drowsiness, tremor or seizure, wheezing, nausea, vomiting, tachycardia, anxiety, rapid fatigue, lethargy, watery eyes, sneezing, brain fog, inability to concentrate, fuzzy eyes, convulsions, dizziness, headache, and peripheral neuropathy from exposure to fragrance.

     Just by the names of some of the aroma therapies one knows that certain scents can affect stress and mood. Some will alleviate anxiety according to their very own claims. Such physical change within the body will without a doubt cause a chemical change to occur in the brain.

     Fragrances are everywhere. You do not have to wear them. The scents themselves can enter the human body through inhalation, ingestion, and absorption. From their the chemical toxins of the scents enter the bloodstream. Each person will have their own reaction to exposure.

     For porphyria patients it is most important to avoid most scent except for what we know to be safe for the individual themselves.

Shanna Moss-Thompson MNS, NP
Allergy & Immunology

FOCUS: Tingling, Numbness and PN in Porphyria

     What many hepatic porphyria patients know all too well is that PN can become a constant companion. Long before a diagnosis of peripheral neuropathy in porphyria comes the symptoms of tingling and numbness.

     Often when the tingling and numbness is not present, severe pain will be present.

     Such symptoms as tingling and numbness are forms of paresthesia.. Paresthesias are abnormal sensations. Such conditions usually cited are tingling and numbness which can be found along the extremities.

     Some porphyria patients will describe these sensations as a "loss or lack of sensation". Many clinicians will address it as sensory loss or paresthesias.

     Such a medical condition can present in the hepatic porphyrias, yet it is always good to have the condition observed as there are many other underlying causes for conditions of this nature.

     Sometimes there is a lack of blood supply to the area where the loss of sensation takes place. Other times following simple laboratory tests the cause can be a vitamin B-12 deficiency.

     Not to uncommon in the hepatic porphyries is diabetes, another medical condition. Diabetes and other chemical abnormalities in the body can also cause tingling and numbness, especially in the toes.

     Also common to the hepatic porphyrias is the medical condition hypothyroidism. It too will cause the sensations of tingling and numbness.

     Some have expressed such sensation in the feet as liken to walking on cracked egg shells bare foot.

     It has been long known that some pharmaceuticals will also cause nerve damage and give off the tingling and numbness sensations. While most of these drugs are considered unsafe or are contraindicated in porphyria, they are still worthwhile remembering. When it comes to the tingling and numbness as a person may have previous taken the drug for a period of time in which nerve damage took place due to the drug.

     Among these drugs are phenytoin, chloroquine, macrobid, and chemotherapeutic agents, all of which are considered unsafe for porphyria. Other drugs that are known to cause such tingling and numbness include isoniazid, D-penicillamine, and the parenteral gold therapy.      Other causes of the tingling and numbness can be created by the pressure on the nerves, caused by a herniated disk, tumors, abscesses or arthritic bones.

     In PCT patients where the porphyria condition was caused by heavy alcohol use, the tingling and numbness can often be ascribed to the toxic action on nerves from the alcohol. Lead and tobacco can have similar affects as well.

     When dealing with the tingling associated with the porphyric peripheral neuropathy one needs to remember that these conditions are an indication of damage or irritation to the nerves in that area. Likewise when there is numbness, it suggests the nerve is not completely dead or severed but is injured is some way or is experiencing pressure. Pressure can build up with PN because often a person will change their stance or position in order to accommodate for the loss of sensation.

Jim Danielson, RPT
Rehabilitation Medicine

FOCUS: Dizziness as a CNS Porphyria Symptom

     As an acute attack of porphyria begins often a porphyric patient will experience the inability to keep normal balance as they get up from a chair or bed and beginning to walk.

     Going from a laying position to a sitting position in bed a porphyric will often experience lightheadedness

     Dizziness is just one of the many symptoms of the CNS [central nervous system] involvement in an acute attack of hepatic porphyria. Having vertigo, being dizzy or losing one's balance are often cited.

     Quite often a porphyric patient will have the feeling of wanting to faint. And they will at times faint.

     When the accompanying abdominal pain becomes very severe many porphyrics will faint. At other times the fainting will occur after a porphyria patient takes a dosage of propranolol or other medication to reduce their hypertensive state and stop tachycardia.

     One thing to remember during acute attacks however is standing up too quickly after lying down or sitting. This will always bring on the dizziness and imbalance.

     The majority of times the dizziness is very minor. However if severity of the attack continues or becomes worse, it is best to take safeguards. Place extra pillows on the bed to protect from hitting your head on the nightstand. Ask you caretaker to be present when you want to mover from your bed, or stand up from a chair.

     Sometimes things will seem like the things around you are spinning. Given a few minutes the sensation will generally correct itself. Much of the dizziness in porphyria results from insufficient blood flow and oxygen supply to the brain and spinal cord, such as can be caused by a rapid drop in blood pressure. Often this is in association with medications taken at the onset of an attack.

     If seizure activity begins in the acute attack, dizziness may come from the seizure activity as well. Another consideration is that of anxiety about going into another acute attack.

     Lightheadedness is a fainting feeling that is not usually serious. When feeling this way it is best to lay down. Always avoid sudden changes in posture.

Linda M. Evans NP
Vascular Medicine

Focus: Malathion and the Porphyric

     While no research studies focusing on malathion have been specific to persons with porphyria, It is well known that hepatic porphyria patients are more likely to react more severely to malathion.

     It is known that malathion has the potential to produce chromosomal changes including chromosomal aberrations and micronuclei. In humans, the genotoxic effects of malathion are still being studied. Malathion is quite apparent in blood serum chemical toxicology tests which have been carried out at various testing centers.

     It is known that both the rough grade and purified malathion can produce cytogenetic damage. In poprhyria patients there are all ready chromosomal aberrations. With exposure to malathion there is the possibility of even more chromosomal aberrations.

     Some studies of malathion have dealt directly with point mutations in standard gene mutation assays in both plant life and mammalian tests.

     So far health officials are unsure of what exposure to malathion hold for the normally healthy individual. For porphyria patients the probability of risks would be greatly increased. Presently the largest area of concern lies in the medical condition of cancer. Medical journals in the last few years have had numerous reports on the connection of malathion and chromosomal damage in cancer.

Another area of concern has been in reproduction results. Chromosomal aberrations have been documented for a number of years as to be associated with different forms of mental retardation as well as morphological abnormalities.

While to date no conclusive or specific findings have been made in regard to malathion and porphyria as a triggering agent, persons with porphyria are well advised to refrain from any contact with malathion whether inhaled, ingested or absorbed.

Scott Jenson, PhD

Chemical 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.

     DITOMED is a brand name for the generic drug PHENYTOIN. Another name is DILANTIN. It is an antiepileptic drug. It is related to barbiurates in chemical structure. The liver is the chief site of biotransformation of phenytoin; patients with impaired liver function and porphyria should not take this drug.

     EUHYPNOS is a brand name for the generic drug TEMAZEPAM. 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.

     ZAPEX 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.

     CENTRIM 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. LOMAC is a brand name for the generic drug OMEPRAZOLE. In clinical trials this drug was known to elevate liver functions. Some hepatic failure was noted. The drug is metabolized in the liver. Caution is listed for persons with liver impairment.