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Porphyria Educational Services
Monthly Newsletter
February 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.


     Analgesics are prescribed for giving effective pain relief. At least that is the theory. Often porphyrics still endure a life of ongoing pain.

     Analgesics can be classified by the site of their action. There are three types: [1] centrally acting; [2] peripherally acting; and [3] locally acting.

     The centrally acting analgesics include both opiod analgesics and non-narcotic agents such as Tramadol a.k.a. Ultram. In porphyria we must avoid the Tramadol/Ultram non-narcotic pain medication because of the side effects of seizures and being noted by some as a "trigger" for acute attacks. It is also contraindicated with the use of some medications for seizures, muscle relaxants, pain and nausea.

     It must be said that no one drug is perfect. Every drug known has its benefits and at the same time has some risks involved with its use.

     Healthcare medical providers must make a determination of which drug to use in any given situation.

     Often drugs are given to a patient and the medical provider then assesses the individual patient's reaction to a specific drug.

     With the porphyric patient, it is much better to use drugs that have been approved for a period of no less than five years. The reason for this is that it takes a couple of years to assess the general problems with any new pharmaceutical product. Porphyrics by the very nature of their disease need to be ever mindful of the use of drugs and double check all information on any drug prescribed for them whether it be oral, suppository, injection or intravenous.

     The majority of drugs on the market today are newer drugs, and each years countless numbers of new drugs and especially drug samples are left with medical care providers to give to patients to try out. Be care of such medications. Ask for and demand to use pharmaceuticals known to be safe for porphyrics. Even then, because each person is different and has different sensitivities, a "safe" drug can not be tolerated by everyone.

     Regardless of whether one uses a non-narcotic, an opiod, or NSAID, and informed decision making in the prescribing of such drugs requires an understanding of the pharmacology, efficacy and more importantly, the safety profile of these agents.

     Every porphyric patient should familiarize themselves with the Porphyria UNSAFE DRUG list. In addition to the endless list of drug names, one would be advised to state the variables of each drug name, whether it be the generic, brand, trade or classification name of a drug.
     BuSpar for instance is known as busprione. It does not appear on any drug list, unsafe or safe. However the classification is such that one would refuse to take the drug. BuSpar is an antianxiety drug, a sedative. Most drugs dealing with the mental abnormalities such as anxiety, or insomnia are unsafe for porphyrics.

     For pain associated with inflammation an NSAID is more often prescribed.

Cheryl "Little Flower" Nelson, R.N.
Patient Care Coordinator


A Glossary of Clinical Research Terms.

Clinical Trial
A carefully designed investigation of the effects of drug, medical treatment, or device on a group of patients.

Clinical Investigator
A medical researcher in charge of carrying out a clinical trial's protocol. Researchers are usually doctors, nurses, pharmacists or other health care professionals.

This individual manages the conduct of the clinical trial.

Food and Drug Administration
A government agency that enforces laws on the manufacture, testing, and use of drugs and medical devices. All drugs and medical devices must be approved by the FDA before they can be used by the general public.

Informed Consent
A discussion of all procedures, benefits, risks, and expectations of a clinical trial between clinical investigators and potential patients. The FDA requires all patients to sign an informed consent form before participating in a trial.

Institutional Review Board (IRB)
A board consisting of health care professionals from the institution where the clinical trial takes place, as well as members of the local community. The board scrutinizes all trial activities including recruitment, advertising, and potential risks. The IRB also makes sure that FDA regulations are being followed in a particular trial.

Investigational treatment
The drug or medical device that is tested during a clinical trial.

Drugs and medical devices must pass three segments or phases of testing before they can be eligible for FDA approval.

Principle investigator
A medical professional who is overseeing the treatment of the patients in the clinical trial.

A plan that sets guidelines for a trial and usually involves several different trial locations. A protocol is usually designed by the sponsor of a clinical trial.

The pharmaceutical company, research institution, or other health organization that funds a clinical trial and designs its protocol.

Standard treatment
An FDA-approved treatment currently in wide use. In trials involving new treatments, there may be no pre-existing treatment at all. In these cases, the lack of any treatment is considered the standard treatment. Generally, the goal of a clinical trial is to introduce an investigational treatment that is safer and more effective than the standard treatment.


     Among the rarer forms of porphyria is that of EPP [Erythropoietic Protoporphyria].

     In the EPP there is an involvement of a defect in the hepatic cells, while in both the EPP the cause of the porphyria is due to the a major enzymatic abnormality in the erythropoietic system.

     Before we begin, let us define some of the terms used. The prefix "erythro" is a combining word meaning "red". "Erythrocytes" are red blood cells. So we are talking "red". "Enzymes" are a protein that causes chemical reactions in living matter. Enzymes affect the reactions that take place within the cells of our body.

     In EPP the problem lies in the overproduction of protophyrin in the red blood cells as well as in the liver cells. Other names that it has been known as are "protoporphyria" and "erythrohepatic porphyria."

     EPP is inherited as an autosomal dominant trait. The EPP is though to be due to partial absence of the mitochrondial enzyme ferrochelatase, which is also called the heme syntheses.

     The most prominent feature of this disorder is the photosensitivity. This is thought to be due to the increased levels of plasma protoporphyrin. Excess plasma protoporphyrin results from the overproduction by the hepatocytes along with the erythrocytes.

     Unlike the hepatic porphyrias, protoporphyria [EPP] usually occurs in children younger than four years of age. Whereas females are mostly active in the hepatic forms, it is the males that are mostly affected with the EPP.

     Symptomology of EPP includes burning and itching which are predominately triggered by sunlight. The symptoms are often accompanied by edema, erythema [an abnormal increase into the red blood cells], and /or urticaria [a skin eruption marked by transient wheals of varying shapes and sizes]. 0ther symptoms which are less frequent are blisters and skin ulcers.

     The lesions will have reoccurrence as a result of chronic sun exposure which will lead to scarring, altered pigmentation, lichenification, and the premature aging of the skin. Lichenification is a thickening and hardening of the skin which often results from irritation caused by repeated scratching of a lesion that is itching.

     Another aspect of EPP is that there are increased amounts of protoporphyrin deposited in the liver. There are as a rule, mild liver function abnormalities. Cirrhosis, liver failure, liver transplantation, and hepatosplenomegaly are also noted as well.

     Some EPP patients have a manifestation of gallstones. About half of the EPP patients have a mild hypochromic, microcytic anemia. Some hemolysis may also be present.

     A more severe form of protoporphyria occurs if both parents are heterozygous. The offspring inherit two defective genes. As of 1996 there were at least eleven different EPP mutations found. In EPP the majority of carriers are asymptomatic.

     The diagnosis of EPP is usually made by combining the history and familial occurrence and the increased levels of stool and erythrocyte protoporphyrin. In the testing of the samples the erythrocytes fluoresce on exposure to the Soret band.

     Prevention of EPP symptoms is the avoidance of sunlight.. The avoidance of sun will help to prevent the irreversible scarring. Intervention therapy is the ingestion of beta carotene. What the beta carotene does is to help with the reaching of blood levels high enough to diminish photosensitivity. Sometimes the use of pyridoxine has been advocated, however the drug does not alter porphyrin metabolism.

Dr. Robert Johnson M.D.
Retired Clinician


     Dioxins: One of the multitude of chemical toxins that affect porphyria patients is that of dioxin. What is dioxin?

     Dioxin is a colorless, odorless organic compound containing carbon, hydrogen, oxygen and chlorine.

     The term dioxin refers to a broad family of chemicals, which differ from one another by the location and number of chlorine atoms on the molecule.

     Dioxin has a high affinity for fatty substances and is found adhered to or dissolved in fat tissue, where it can accumulate. In porphyrics the dioxin will remain in the fat while one is in remission. When dieting, losing weight or profusely sweating, the chemical toxins stored in the fat become released. These toxins then trigger acute attacks of porphyria.

     How are humans exposed to dioxin?

     There are innumerable ways that people can be exposed to dioxin. For most people such exposure at mild levels is not harmful. However for porphyrics even mild exposure can be most detrimental.

     Dioxin is an unintended byproduct of natural events such as volcanoes and forest fires as well as man-made processes such as manufacturing, incineration, paper and pulp bleaching, and exhaust emissions.

     In the Pacific Northwestern United States there are higher incidents of chemical toxins involving dioxin. It has been speculated for some time that the higher incidence can be traced to the use of chemical toxins that released in the lumber mills and the manufacturing of paper products.

     Dioxin is ubiquitous in the environment: it is found throughout the industrialized world in air, water, soil as well as in food. Exposure to dioxin can come through working in industries where dioxin is a byproduct, industrial accidents, through food and human breast milk and in drinking water.

     It must be pointed out that skin contact or breathing represent very small sources of dioxin exposure.

     How does dioxin get into the food chain?

     Dioxin can enter the food supply through a number of different routes. In fish, the primary route of exposure is through water. Fish taken from streams downstream from paper mills can be largely suspect for chemical toxins.

     Plants and animals are exposed to dioxin primarily through particulate in the air. Airborne particles of dioxin settle on forage or feed, which is then eaten by animals. This accounts for dioxin traces being found in milk.

     Dioxin concentrates in the fatty tissues of beef and dairy cattle, poultry, pork or seafood. Theoretically, the longer the life span of an animal, the higher potential accumulation of dioxin in its adipose tissue.

     Washing of fresh produce is a must. Dioxin particles that settle on fruits and vegetables as a result of airborne exposure are removed by washing; dioxin does not become systemic in the plant or food source.

     How much dioxin is contained in beef?

     Since the 1950's, the beef industry has made significant strides in responding to public health goals to reduce consumption of dietary fat. For example, leaner cattle are being bred and today's beef has less trimmable fat, and some the meat has zero trimmable fat. Improvements also have occurred in the production and sale of pork and poultry.

     What happens to dioxin when consumed by humans?

     Dioxin is stored in human adipose tissue.

     Scientists recognize that the effects of dioxin vary widely among different animal species. Humans are less susceptible to the consequences of dioxin exposure than many of the animal species tested in laboratories.

     Most research in humans has involved populations involved in occupational or accidental exposures of dioxin several thousand times higher than normal such as the residents of Seveso, Italy.

     What is the Food and Drug Administration (FDA) doing to reduce dioxin exposures?

     FDA has worked with the paper industry to establish a voluntary guideline for lowering dioxin levels in paperboard used for food packaging, such as milk cartons. The agency is also developing new analytic methodologies to improve dioxin monitoring.

     People and especially those who are porphyric who want to minimize their potential exposure to dioxin in the diet should follow advice to consume a low-fat, balanced diet. This includes:       1. Selecting lean cuts of beef, pork and poultry in the meat case;      2. Trimming and discarding fat from beef, poultry or seafood before eating, including any skin;      3. Choosing low-fat dairy products; and      4. Eating moderate portions of a wide variety of foods.      It is well to remember that Dioxins are only one of a large host of chemical toxins that can trigger acute attacks in porphyrics or cause extreme sensitivities for MCS patients.

Dr. Roger Haakensen
Environmental Medicine


ANTISACER 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.

LORAT is a brand name for the generic drug LORAZEPAM. It is a BENZODIAZEPINE. It is used as an antianxiety drug, a sedative and as a hypnotic. Disorientation, depression, nausea, change in appetite, headache, sleep disturbance, agitation, dermatological symptoms, eye-function disturbance, together with various gastrointestinal symptoms and autonomic manifestations can occur with the use of this drug.

Transient amnesia or memory impairment has been reported in association with the use of benzodiazepines. Drug dependence and withdrawl symptoms may occur. This drug is not recommended for use in patients with a primary depressive disorder or psychosis.

The drug is metabolized in the liver. Patients should have periodic blood counts and liver-function tests are recommended for patients on long-term therapy. This drug is not recommended for patients with liver disease.

UROPLUS 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.

XANAX is a brand name for the generic drug ALPRAZOLAM and is a part of the drug classification of BENZODIAZEPINES. The following adverse events have been reported in association with the use of benzodiazepines: dystonia, irritability, concentration difficulties, anorexia, transient amnesia or memory impairment, loss of coordination, fatigue, seizures, sedation, slurred speech, jaundice, musculoskeletal weakness, pruritus, diplopia, dysarthria, changes in libido, menstrual irregularities, incontinence, and urinary retention This drug has a warning on withdrawal reactions including seizures and dependence. The drug is metabolized in the liver. There is a warning concerning use in patients with liver disease.

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