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Porphyria Educational Services
Monthly Newsletter
June 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 noway 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: QUALITY OF LIFE WITH PREVENTIVE THERAPIES

The Porphyrias can be a progressive disease involving the neurological system and in some forms cutaneous manifestations can occur and become progresive as well.

Not a lot is known about many aspects of the disease yet while some of the causes are still not known, the symptoms are known and can be treated. There is no cure.

The hepatic porphyrias are characterized by symptoms such as abdominal pain, nausea, mental change, blurrd vision as well as the PN [peripheral neuropathy] aspects which include tremor, stiffness, slowness of movement, loss of gait and drop foot.

At one time it was felt that syptoms would lessen in patients as they reached their later years. In the past few years with better diagnostic assessment and data available it is felt that porphyria symptoms continue with patients, but that with adequate interventive and preventive protocols symptoms can be lessened and major acute attacks avoided.

Although there is no cure for the porphyrias there are ways to treat its symptoms.

Most patients with the hepatic porphyrias receive regular glucose infusion. Some with the most severe symptoms or those that will not resolve through the regular conservative therapy protocol of glucose will go to the use of Panhematin. In Europe the use of heme arginate a.k.a Normosang is also used for the more severe cases.

However in the United States only the Panhematin is available.and it does not currently have F.D.A. approval. The use of the Panhematin should be limited only to those porphyria patients which do not respond to the glucose protocol wihtin 48 hours of onset of treatment. Currently to obtain the Panhematin you must have your physician file paperwork through the Compassionate Needs program.

The use of Panhematin for the more severe cases is widely accepted but at the same time it has many draw backs and is largely contraindicated for roughly 37% of the porphyria patients wanting to use it because of previous medical histories of blood clots, DVT, PE and various coagulation diseases.

In the use of Panhematin for those who can qualify for its use safely, it is well to note that as the porphyria progresses, the Panhematin becomes less effective, and complications such as "wearing off" can occur. Sometime this "wearing off" can occur within two years of the initial start of the use of Panhematin.

"Wearing off" is characterized by the reappearance of porphyria symptoms before the time of the next scheduled dose of Panhematin, It then becomes necessary to modify the dosage and the intrevals of treatment in order to maintain consistent control of the symptoms.

In the glucose preventive care protocol the same may be noted infrequently, but with the additon of a single 1,000 ml bag per week symptoms can be kept under control as long as the porphyria patients avoids the major porphyria triggers such as severe dieting, exposure to chemical toxins especially through use of unsafe drugs, and of course other illness.

When glucose or Panhematin is administered, only a small percentage of it ever reaches the liver because it is rapidly broken down by enzymes in the bloodstream.

As the liver over produces porphyrins it becomes even more important to get as much carbohydrate as possible into the liver.

Researchers still advocate the use of timely administered and regularly administered glucose infusion as the primary form of treatment for the hepatic forms of porphyria. Data shows that the use of panhematin slows as a much faster rate than does glucose. Repeated panhematin treatment necessitates more frequent administration as well as additional quanitities of panhematin.

Overall researchers and data collectors have found that the glucose protocol when followed with conformity and avoidance of other triggers, is associated with with improved quality of life and the reduced feelings of depression.

Among other benefits of the preventive glucose treatments participants report that they were able to get around more easily and to communicate more effectively while receiving the regular preventive glucose infusion. They all noted a great reduction in the ongoing pain associated with chronic porphyria, as well as what they termed "brain fog" and "optic neuritis", restlessness, and nausea. They all felt that the preventive regiment improve their mood and enhanced general well-being.

Renee Heaslett RN, MNS



FOCUS: TOUCH--A CARETAKERS NEEDED SPECIALTY

A few months ago I was sharing with the caretaker of a porphyria patient. She lamented on the fact that there were never enough hours in the day to accomplish everything that needed to be done and that just when one bout with porphyria symptoms seemed to be under control, another would "kick in".

Yes, I had heard this before, but what really touched me was when she lamented that with the demands of everyday life and the pain of the porphyria, there was little time for touch. She felt void of loving contact with her spouse that they had once shared before the porphyria came to rule their lives.

Sometimes in the rush of activity that we call necessary living we forget one of the most valuable senses we have. That valuable sense of course is "touch".

There are so many times of celebration throughout the year where people hug and touch freely. Mother's Day, graduation, weddings, baptisms, and numerous other special times. But the sense of "touch" is something that we need to be mindful of on a regular basis. This is especially true when we are caretakers of a porphyria patient.

Many porphyria patients spend long hours alone unable to work and having a lot of time to reflect on life, often the life that is no longer theirs. Loneliness sets in along side the pain that is almost always there. Physical pain and the pain of loss. Here is where touch can be most welcome.

Touching is not only pleasant and loving, it is needed.

Somewhere back in graduate school I remember a seminar where the presentor went on and on in regard to the scientific research support for the need to touch. One example was in the classroom where teachers should make a habit of touching each student at least once a day if but for a second. It is recognition and for many students it was the only time in that given day that that a student would receive any recognition at all.

The same can be said for the porphyria patient who is often left alone for long periods of time. Alone and in pain. Sometime in depression. How welcomed a touch would be. A hug for the porphyria patient often times is more powerful medicine than medicine itself.

Scientific research supports the theory that touch which is stimulation, is absolutely necessary for our physical as well as our emotional well-being.

The loving touch of another can make us feel better about ourselves and our surroundings. It can also cause measurable physiological changes in the toucher and the touched.

The best kind of touch is the hug! Hugging just feels good. Hugging will dispel loneliness. Hugging also opens doors to feeling. Hugging will build up self-esteem so often lacking in those who suffer from the effects of progressing chronic smoldering porphyria.

Some other things that hugging does is help ease tension. Hugging will also affirm physical well being. One very positive affect of hugging is that ir will help fight insomnia which is so prevalent among porphyria patients.

To touch....to hug....makes for happier days. In the lives of a porphyria patient, it makes impossible days possible.

Dr. Kenneth Carlson
Neuropsychiatric



FOCUS: PERFUMES AND SCENTED HOUSEHOLD PRODUCTS

While the effects of the scent of many perfume chemicals upon the health of people is still largely unknown, it is a well-known factor in causing untold reactions on some porphyria patients as well as those suffering from MCS.

One of the things thought is that different fragrances cause different symptoms. In some cases some fragrances probably cause no symptoms at all. From a scientific and medical standpoint these findings may indicate that some chemicals are more toxic than others.

At this point in time, not all perfume chemicals have been tested for their toxcity. Until more is known it would be most advisable for porphyrics to avoid contact with heavy concentrations of perfumes in general. A trip to the shopping mall should avoid the perfume counters in the large department stores or speciality shops specializing in bath and beauty items high in fragrance.

Until all perfume or fragrance chemicals have been tested there is no safe way of knowing which specific products are harmful. Until such a time, when all fragrances and fragance products have been tested it would be most wise to avoid fragranced products as much as possible.

Young women especially are attracted to perfume counters. And young men likewise go for scented "aftershaves". Much of the bath and beauty products used today are perfumed.

Such products most probably containing perfumes or other chemical toxins to be mindful of include shampoos, lotions, hairsprays, perfumes, hair conditioners, skin conditioners, colognes, aftershaves, shaving creams, baby care products, women's personal feminine products, and even make-up.

For a number of years household cleaning products have been toxic to many porphyria patients. Fabric softeners to be used in the clothes dryer, the laundry soap itself, bleach, floor cleaners, air fresheners, and floor wax are all known to cause terrible reactions in some people.

According to some research statistics there are currently over 5,000 different chemicals used today in various products with fragrance. Some are very toxic to people while others may appear to be harmless. Some 20% of these chemicals have been tested and have been found to be toxic to some degree.

Toxic perfume chemicals can cause health effects. COPD and asthma patients suffer with lungs irritation from such chemicals. Porphyria patients with cutaneous manifestations experience rash, burning, itching and blistering from such products.

And mental confusion can occur as well in many hepatic porphyria patients. Headaches are not uncommon after exposure to such chemicals as well as nausea. Some studies cite that "neurotoxcity" occurs after exposure to such fragrance chemicals. Depression is often a common side effect of ongoing exposure to toxic fragrance chemicals. They will also alter a person's mood, elevate a person's stress level and cause anxieyty levels to rise.

Toxic perfume chemicals can enter the body through inhalation and ingestion through the nose and mouth. These chemical are often absorbed through the skin as well.

After these chemical toxins enter the body they are absorbed into the bloodstream and transported throughout the body. Individual sensitivity to the effects of these chemical toxins vary widely from no effect at all to severe symptoms.

Julie Evenson, NP
Allergy & Immunology



FOCUS: FINANCING THE HIGH COST OF PORPHYRIA TREATMENT

A porphyria patient can quickly total up a pile of medical costs in just getting to the point of having a diagnosis of porphyria.

Having a diagnosis at least can put a stop to the repeated testing procedures and exploratory procedures which are very costly and quite often send a porphyria patient spiraling toward their medical insurance coverage "cap"

Some "chronic smoldering" porphyria patients have found themselves unable to obtain the treatment recommended by their physician because personal finances have been stretched to the limit.

Other time medical insurance providers will deny coverage for intervention treatment as well as the preventive protocol for hepaic porphyrias. Some porphyria patients have had ongoing disputes about insurance coverage.

Often such disputes involve complex contractual issues. These kinds of problems can often be handled through a hospital or clinic Patient Representative , however problems involving contractual issues are typically difficult to resolve.

Quite often it benefits a patient to seek out "Case Management" with their insurer. Under such an agreement the patient is obligated for the deductible and co-pay after which all else is paid in full and at the lowest possible charges. Case Management will slow that spiraling progression toward reaching that coverage "cap". Discuss such a possibility with your personal physician if you are finding yourself denying treatment because of lack of financial resources.

Remember that you as a porphyria patient, are already experiencing the stress and anxiety that always accompany serious illness. And stress in porphyria is never good. As a patient you should not be subjected to the additional stress of doing battle over the terms of your medical insurance coverage.

Many hospitals and clinic have staff members that work on behalf of the patient. Patient Representatives work on the premise of education, representation and collaboration.

Tiffany James, BSN
Patient Representative



FOCUS: FOOT DROP, LOSS OF GAIT AND OTHER PN ASPECTS

Many porphyria patients complain about having trouble walking. After a severe acute attack porphyric will often experience to some degree various medical conditions in relation to peripheral neuropathy which stems from the porphyria attack.

Abnormal walking stances, loss of gait, foot drop, or inability to walk, are all a part of the complexities of porphyria.

And what is "gait"?

Gait is the format of how a person walks. There are many different forms of gait abnormalities.

In porphyria patients it is believed that much of the gait abnormalities are due to the exposure to toxic reactions. Toxic pharmaceuticals are the main causes followed by chemical toxins, and alcohol.

At first many do not understand why they are walking in an unusual way, or perhaps why they can not control the way their leg or legs will behave while attempting to walk. Quite often the limbs will experience numbness, other times tingling, and still other times deep bone pain. But always present is a seemingly stumbling mode of operation.

In reviewing clinical charting of numerous porphyria patients it has been found that when an acute attack of porphyria has been triggered by a direct exposure to chemical toxins such as metal dusts, industrial toxins as vinyl, paints and varnish fumes, formaldehyde, carbon monoxide, or a variety of what are termed "unsafe drugs for porphyrics" the problems with walking, loss of gait, foot drop, and the paresis will manifest.

In porphyria patients with sever liver damage often a scissor gait will be noticeable. Sometimes a walking pattern referred to as "spastic" will occur. Those will polyneuropathies will often experience even more walking abnormalities.

Foot dragging and steppage gait, top scraping, or rigid one-sided posture are all a part of walking abnormalities. Upon clinical evaluation loss of deep tender reflexes may be observed.

Most of the walking abnormalities will correct. Sometimes PN will persist and linger for weeks or even months after a porphyria attack. Getting adequate and quality rest is necessary for aiding in recovery.

While in recovery from such defects a porphyria patient should try to be as self-reliant and independent as possible. Caretakers as well as the porphyria patient themselves should allow additional time for daily activities. Do not give up walking, but allow more time for walking and in an area where one does need to navigate steps or uneven surfaces.

Remember that if you experience loss of gait, you will be more susceptible to injuries due to falling because of poor balance. It is well to be advised that you will also most likely make an unconscious effort to always catch up while walking with others.

Physical therapy is advisable. Both active and passive exercises are encouraged. With leg braces and in-shoe splints one can help maintain proper foot alignment for standing and walking. For some porphyria patients with poor balance the use of a cane or a walker is recommended.

Roger Littleton R.P.T.
Rehabilitation 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 judgement 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.

TOFRANIL is the brand name for the generic drug IMIPRAMINE. It is a dibenzazeprine and an antidepressant. This drug is known to be an inhibitor of P-450. The drug should be avoided by patients with liver, or thyorid problems, and seizure activity as well.

EPANUTIN 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.

RESTORIL 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.

BACTRIM 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.

EPIROLE 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.