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Porphyria Educational Services
Monthly Newsletter
June 2002

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.


Hypaesthesia is often experiences by porphyria patients. Hypaesthesia is having a diminished feeling in the extremities, and especially in the fingers and toes, hands and feet.

Anaesthesia in peripheral neuropathy is the absence of any feeling..

In many of the peripheral neuropathies experienced by porphyria patients the neurologist is faced with determining whether such neuropathies are diminished or absent. .

Porphyria patients often can not tell the different or are unaware that there is a difference. Also many times such PN may be confused with PN resulting from other medical conditions such as diabetes, MS, or be the result of chemotherapy treatment for cancer..

Manual dexterity problems can occur with PN. Many porphyria patients learn that their sense of touch and feeling are greatly altered or in some cases non-existent or they have difficulty in walking..

There is also that which is referred to as 'Glove and Stocking’ sensory loss.. Neuropathies such as this give rise to loss of sensation in the hands, feet and lower legs, the distribution resembling gloves and stockings..

The porphyria patient experiencing polyneuropathy may also find that they are not able to carry much because of weakness in their arms. These patients quite often complain of finding themselves dropping things. They have a personal sense of being clumsy which often times is a facade that will mask the correct assessment of the PN and it's progression..

Such poly neuropathetic symptoms are common, and it may mean that the porphyric patient experiencing such symptoms will get frustrated with themself and others around you..

Learning to accept minor limitations and also learning ways of compensating will go a long ways in helping to deal with loss of limb activity..

Often such physical change is temporary and therefore transient. However in some cases where permanent nerve damage has taken place, a good physical therapy and rehabilitation program must be undertaken. .

Renae Stillman PTA
ReHabilitative Medicine


And just what are NSAIDS?

The Non-Narcotic Analgesics NSAID reduce inflammation and relieve pain by affecting arachidonic acid metabolism.

While the NSAIDS are used safely and effectively by millions of people they are often associated with adverse effects, particularly in patients who are in high-risk groups, including porphyrics.

GI complications are the most common adverse effect of NSAIDS.

One of the most used NSAIDS is Acetaminophen [Tylenol], however this drug is metabolized in the liver and should not be used by patients with the hepatic forms of porphyria.

Robert Johnson M.D.
Retired Clinician
Porphyria Investigator


DNA family mapping of porphyrics is being directed by Dr. Robert J. Desnick MD at Mt. Sinai Hospital in Manhattan, New York. Dr. Karl E. Anderson continues on research related to the use of heme arginate in porphyric patients.

Other porphyria studies deal with drug testing, studies focusing on porphyric women and cyclic manifestations. Some studies focus on chemical toxicology. In addition there are countless other porphyria studies worldwide that look at different aspects of the disease.

Dr. Michael Moore's drug reporting site is a simple but most useful research tool. All that is required is to fill in the information on every drug you take, whether the results are good or bad, and that way researchers can deem a drug as "safe" or "unsafe" by looking at the feedback.

Researchers are obligated to protect the rights of people who participate in their research studies. The research participants. themselves, also can protect their own rights by being aware of what those rights are by being assertive in asking questions about the study.

Research is important in improving the healthcare of people, however, it must be conducted in a way that is respectful of the rights of the people participating.

Porphyria patients who are in close proximity to any of the research testing sites or study centers are encouraged to inquire and perhaps to participate in the ongoing investigative studies. Clinical trials in relation to porphyria patients requires porphyria patients to be test subjects

Tsu Lim, PhD
Clinical Investigator


The porphyric patient who experiences peripheral or polyneuropathy, may experience a variety of emotions and changes in self-concept.

Loss of normal everyday functions such as touch, paresis, numbness, and pain become problematic for many as the symptoms first set in.

Some porphyria patients find themselves wanting to shy away from friends, family, work or social gatherings because they find that they cannot lift their feet because of foot drop.

These porphyria patients may find that their legs feel heavy and that they fatigue very easily. Often they feel they can not carry their "normal load" at work or elsewhere. Some feel like they are becoming a burden.

Furthermore the porphyric patient may find walking very difficult and may be prone to tripping.

They may also find that they are not able to carry much because of weakness in their arms. Quite often they complain of finding themselves dropping things.

All of these symptoms are to be expected. Because of this it may mean that you get frustrated with yourself and others around you.

One of the PN aspects that is hard to describe but is experienced at one time or another by most porphyria patients is that of the "loss of position sense." This is another unusual feeling. It is describe by porphyric patients as " your brain knows where your feet should go, but your feet have a mind of their own."

Many times the porphyric patient may become conscious about the way that they walk, but they can not for the life of them understand why it has altered.

Without realizing it the average porphyria patient will probably re-educate the way that they walk, possibly widening their gait or throwing their leg.

They will probably have difficulty keeping their balance especially in the dark and find that they will have to look at the ground to compensate for the loss of position sense.

Most beneficial is having the ability to visit in a small group setting or one-on-one with another person who has experienced the same types of limitations.

Also beneficial is self-realization that peripheral neuropathies do occur but that they need not be self-limiting, in that today more than any other time in our history, people with physical challenges can remain in the mainstream of life.

Roger Collison PA

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.

ALADDIN is a brand name for the generic drug PHENYTOIN. Another name is DILANTIN. It is an antiepileptic drug. It is related to barbiturates 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. ANJAL is a brand name for the generic drug HYDROCHLOROTHIAZIDE TRIAMTERENE. It is classified as a diuretic. It can cause liver enzyme abnormalities. It can also cause renal failure. This drug can also reduce levels of blood serum potassium essential to electrolyte balance. This drug also contains the ingredient of sulfate. 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. PLAQUENIL is a brand name for HYDROXYCHLOROQUINE. It is an antirheumatic and antiprotozoal drug. This drug carries a warning against use by person with liver disease and those with porphyria. ***This drug however is a drug treatment of choice for one specific form of porphyria, but unsafe for all other forms.