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Porphyria Educational Services
Monthly Newsletter
May 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.


Damage to the motor nerves causes weakness in the upper and lower limbs.

Weakness in the upper and lower limbs is most experienced by patients with a diagnosis of an acute hepatic porphyria.

The porphyric patient may find that they cannot lift their feet because of foot drop.

Furthermore the porphyric patient may find walking very difficult. And the porphyric patient will then begin to notice that they may be prone to tripping.

They will begin to notice that their foot will drag somewhat and that little ridges in flooring such as going from smooth surface to a carpeted surface will find them catching the edge of their shoe, tripping and often falling.

They may find that their legs feel heavy and that they fatigue very easily .

Walking on rocky surfaces,spongy or soft lawns also causes problems for the person with foot drop.

Often the person will learn to compensate by bearing more weight on the good foot and often will find themselves with a buildup of callous or uneven wear on the sole of their shoes because of a change of gait in order to maintain what they perceive as a normal ability to walk and move.

Barbara Rodgers PTA
ReHabilitation Services


Many people wonder what is correct when seeking information concerning pharmaceuticals or various treatments related to porphyria.

As I deal with my patients or consult with the physicians of other porphyria patients I share the following information.

Medicine is a constantly changing science and not all therapies are clearly established.

New research changes drug and treatment therapies daily. In addition no two patients will react in exactly the same way when given a confirmed diagnosis of porphyria.

Currently the best therapy is Preventive Therapy in the acute hepatic porphyrias. Either glucose on a weekly basis or Panhematin on a four to six weeks basis is required to remain in remission.

Metabolism of pharmaceuticals by porphyrics deals with a wide array of issues. Such issues include the degree of malabsorption, ability to ingest pharmaceuticals, contraindications resulting from other medications. Much of that is still unknown in relation to this disease.

As a former practicing clinician it was my thought that the fewer drugs I prescribed the better off my porphyria patient would be because we could not guarantee how a new medication would react, and the patient came ahead of "trial and error" methodology of clinical practice.

The researchers, authors, research or clinical contributors, editors, and publishers of various porphyria websites have used their best efforts to provide information that is up-to-date and accurate and is generally accepted within medical standards at the time of publication.

Keeping information current is a must, and good sites will list their updates. As a contributor to the PES website, I know that it is updated regularly and that the website owner solicits input from the best of pharmaceutical researchers as well as toxicologists.

However, as medical science is constantly changing and human error is always possible, the authors, editors, and publisher or any other party involved with the publication of "UNSAFE DRUG LISTS" do not warrant the information as 100% accurate or complete, nor are they responsible for omissions or errors in the text or for the results of using this information.

Things in medical science are always changing.

Each porphyria patient is ultimately responsible for his or her own self wellbeing. Porphyria is not yet a household word, even in the medical world where one would expect to find it to be. That is unfortunate, but it is reality.

The porphyria patient or caregiver should always confirm the information in such lists from other sources prior to use. Never take any pharmaceutical just because you were handed a prescription. Never assume that the prescribing physician is fully informed about the dangers of porphyria.

Ask questions in regard to any prescription. Have the information checked for contents, including the notorious binders, fillers and coatings of pills. Have your physician be specific as to the ingredients in every medication, no matter how minute an ingredient may be.

Likewise question the pharmacist. Many pharmacies will fill a prescription with a generic or a similar brand which may be the same medication but because of additives, coatings, be contraindicated for a porphyric patient.

In particular, all drug doses, indications, and contraindications should be read and confirmed in the package insert.

Dr. Robert Johnson, M.D. Ph.D.
Consultant and researcher,
Retired clinician.


Glucose can and does diminish the excess or overproduction of heme precursors in the liver.

By using preventive glucose you can prevent an attack or by using the glucose as Intervention therapy you can bring about the recovery from anyone of the acute hepatic porphyrias [AIP, VP or HCP]

Glucose therapy is simple. For those who have only occasional episodes it can be iv accessed through a Hickman. For those who regular run preventive glucose or are chronic porphyrics, and need regular intervention care, the place of a PICC or a PORT is most desirable.

Many porphyrics fail to realize the importance or ignore the importance of their carbohydrate [sugar] intake. A steady daily amount is necessary to suppress disease activity.

Remember that daily requirements run 300 gm or better of carbohydrate daily. During an acute attack a porphyric's requirement runs 500 gm of carbohydrate total including both consumed and iv infusion of carbohydrate.

Quite often porphyria patients when they go to the clinic or hospital while experiencing an acute attack often will relate that they have not felt well enough to eat or have been unable to eat. Ironically it is the very treatment they need, high consumption of carbohydrate containing foods.

Often it is felt that if the porphyria patient had not skipped over the consumption of carbohydrate containing foods, that they would have aborted their own acute attacks and not needed to present for emergency interventional care.

Preventive therapy is most cost effective, as well as most importantly a healthy move.

Preventing acute attacks, a porphyric reduces the risks of renal failure, liver failure, scarring of the liver resulting in cirrhosis or hepatic cancer.

More over safe guarding oneself from the possibility of respiratory failure and death is essential by use of preventive therapy.

JoAnne Nelson RN
Infusion Therapy


Cirrhosis of the liver is found as a condition of some forms of porphyria.

In cirrhosis, liver cells are damaged and replaced by scar tissue which, as it accumulates, hardens the liver, diminishes blood flow, and causes even more cells to die.

The loss of liver function that accompanies this degenerative condition results in gastrointestinal disturbances, jaundice, enlargement of the liver and spleen, emaciation, and accumulation of fluid in the abdomen and other tissues.

Over half of the deaths related to cirrhosis are due to alcohol abuse, hepatitis, and other viruses. The hepatitis is often in relation to the PCT form of porphyria. Chemicals, poisons, too much iron (hemachromitosis) or copper, and blockages of the bile duct also may cause the disease.

Treatment of cirrhosis usually consists of eliminating the underlying cause, if possible, to avoid further damage, and preventing or treating complications. A treatment protocol is mostly supportive, often including a specialized diet, diuretics (water pills), vitamins, and abstinence from alcohol.

In some cases, for patients otherwise healthy, a liver transplant is now a feasible option.

Liver abscesses are caused by bacteria. Strains of bacteria need to be identified and treated with antibiotics.

Most of these disorders causing liver problems are genetic. Among the more common are: Biliary atresia; Chronic active hepatitis; Wilson's disease; Reye's syndrome, and porphyria cutaneous tarda.

Other serious diseases of the liver, fortunately seen less frequently than these discussed above, include fatty liver, hepatic coma, and liver cancer.

Sheila Brandt NP
Gastroenterologist &
Internal Medicine

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.

MERIDA is the brand name for the generic drug SIBUTRAMINE HYDROCHLORIDE MONOHYDRATE. This obesity drug carries with it a warning for those with liver disease, seizures or/and depression. This drug is not recommended for people with hepatic porphyria.

ZOCOR is the brand name for the generic drug SIMVASTATIN. This drug carries a warning that it should not be used by persons with liver disease.

ACETALDEHYDE is a colorless, flammable liquid used in the manufacture of acetic acid, perfumes, and flavors. It is also an intermediate in the metabolism of alcohol. It has a general narcotic action and also causes irritation of mucous membranes. Large doses may cause death from respiratory paralysis. Has been known to trigger acute attacks of porphyria.

DECATONA 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.