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Porphyria Educational Services
Monthly Newsletter
April 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 presecribed 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 tyopes: [1] centrally acting; [2] peripherally acting; and [3] locally acting.

     Analgesics which are centrally acting 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 medications.

     It must be said that no one drug is perfect. Every drug known has their benefits and at the same time has some risks involved with its use. And it must be remembered in porphyria that no two patients will react the same. Just as specific porphyria mutations are different, so too are the varied responses of porphyria patients to the use of the same drugs.

     Healthcare medical providers must make a determination which drug to use in any given situation. It is very important to always refer to the Porphyria Unsafe Drug Lists.

     Often drugs are given to a patient and the medical provider then assesses the individual ppatient'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.

     Porphyria patients, 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 everone.

     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, efficiacy and more importantly, the safety profile of these agents.

     Every porphyric patient should familiarize themselves with the Drugs List by Dr. Michael Moore. In addition to his 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. Often 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.

Robert Johnson M.D.
Retired Clinician
Porphyria Investigator


     lt is well for a porphyria patient to drink water often to avoid dehydration.

     Avoiding dehydration is important to ensure good health at all times. During acute attacks it is extrememly important because dehydration can lead to other problems as well.

     Keep your body well hydrated - in the hot summer months as well as in the dry heated air indoors in the winter. Drink often. It is better to drink eight 8 oz glasses of water throughout the day than to drink three or four 15 - 20 oz glasses. The fluid will stay in your body better.

For porphyria patients using municipal water systems which are heavily chlorinated it is best to purchase spring water or filter all tap water.

     Furthermore, If you are chronically dehydrated, it may take a week of "forcing" fluids before you notice a difference in your hydration. Until then you may just find yourself going to the bathroom more often. On average, however, a person should drink eight 8 oz. glasses per day (about 2 quarts). An overweight person should drink and additional glass for every 25 pounds of excess weight; those who exercise or are experiencing hot or dry weather should increase their intake as well. Water is best cold, but not ice water. Cold water instead of ice water is good not only for taste, but the cold water is adsorbed into the system more rapidly.

     Electrolytes are also very important to porphyric patients and they should be sure to have electrolyte blood serum levels checked regularly and at the beginning of any acute porphyric attack in order to avoid the unnecessary mental changes and other neurological changes that can occur. Avoiding dehydration is important part of this as well.

Cheryl "Little Flower" Nelson RN
Medical Nursing Supervision


     Optic neuritis is a serious eye condition that causes a loss of vision and may be a warning about more serious systemic disease.

     Any porph suspected of having optic neuritis should be examined promptly by an ophthalmologist.

     Optic neuritis means inflammation in the optic nerve. When the optic nerve is inflamed, vision is affected. This happens quite often when a person is in an acute attack of porph. If prolonged the ON will persists during remission [times between attacks].

     This condition can occur at any age.

     Optic neuritis is more common in women than men. The average age that an adult develops the disease is 31 years. However that goes along with the onset age of the associated diseases of ON such as MS and porphyria. They are usually latent therefore so is the ON.

     Optic neuritis is associated with other medical conditions. People who develop optic neuritis may go on to develop multiple sclerosis (MS). The same is true of latent porphs who become acute and moreover become chronic. Then the ON becomes a problem.

     The risk of developing MS is greatest in the first two years after a bout of optic neuritis. About 40% of people with optic neuritis will go on to develop MS. No in depth studies have been undertaken to realize the number of porphyria patients who also have been diagnosed with MS or with other conditions in relation to ON.

     Optic neuritis and multiple sclerosis are called "demyelinating diseases." This can be said of acute porphyrias which develop into the PN [peripheral or polyneuropathy].

     Myelin is a substance that acts like insulation around a nerve. Just as insulation on an electric wire protects the wire, myelin protects nerves.

     When a nerve loses its insulation, the messages in the nerve slow down. This slowing affects the ability of the nerve to do its job.

     Myelin is destroyed by these diseases, resulting in nerve disorders that cause troubles with vision and many other organ systems.

     When someone develops optic neuritis, he or she notices several changes over a period of 2-5 days. The vision in the affected eye becomes blurred. Color vision is lost -- images appear as shades of gray. People here on the list often speak of "fuzzy vision"...and some may even labeled it "brain fog" however that rightfully belongs to the mental change that occurs rather than the ON.

     There may be pain with movement of the eye. There may be blind spots in the field of vision. Vision can get so bad that the person cannot even see light. An ophthalmologist [eye doctor] will be able to see abnormalities in the way the pupil works and sometimes, see swelling and inflammation of the head of the optic nerve, inside the eye.

     Optic neuritis usually gets better, even without treatment. In three quarters of patients, the vision starts to improve to some degree, in a few weeks. It can take several months for it to get to its maximum level. The vision after a bout of optic neuritis is often, nearly as good as it was before. But of course for porph patients you have to avoid having the repeated attacks and that is another good reason for going to "preventive" therapy and if not that at least getting the "intervention" therapy in a timely at onset of an attack and avoid the days of delay in getting the glucose.

Dr. Joseph Olson
Ophthalmology Department
Metabolic Disease Research


     Glucose or dextrose as it is often called, is a sterile, nonpyrogenic, hypertonic solution of dextrose in water for intravenous injection as a fluid and nutrient replenisher.

     The solution of glucose may contain sodium hydroxide and/or hydrochloric acid for pH adjustment. 25 meq K may be added for potassium adjustment.

     The solution contains no bacteriostat, antimicrobial agent or added buffer (except for pH adjustment).

     Glucose is a hexose sugar that is freely soluble in water.

     And what does the glucose accomplish for the patient? In porphyria patiebnts the need for high carbohydrate intake is often accomplished through the means of iv infusion.

     When administered intravenously this solution restores blood glucose levels in hypoglycemia and provides a source of carbohydrate calories. Carbohydrate in the form of dextrose may aid in minimizing liver glycogen depletion and exerts a protein-sparing action. Glucose injection undergoes oxidation to carbon dioxide and water.

     Carbohydrate is essential in porphyria patients for stablizing the production of porphyrins in the liver. Without necessary carbohydrate intake the liver will overproduce porphyrins and trigger acute attacks which will be prolonged without necessary carbohydrate.

     Water is an essential constituent of all body tissues and accounts for approximately 70% of total body weight.

     Water balance is maintained by various regulatory mechanisms. Water distribution depends primarily on the concentration of electrolytes in the body compartments.

     Sodium (Na+) plays a major role in maintaining physiologic equilibrium.

The indication for the use of glucose iv infusion in the porphyria patient is specifically for a source of carbohydrate calories in patients whose oral intake is restricted or inadequate to maintain nutritional requirements.

      Slow infusion of hypertonic solutions is essential to insure proper utilization of dextrose and avoid production of hyperglycemia.

     Clinical evaluation and periodic laboratory determinations are necessary to monitor changes in fluid balance, electrolyte concentrations and acid-base balance during prolonged parenteral therapy or whenever the condition of the patient warrants such evaluation.

     Hyperosmolar syndrome, resulting from excessively rapid administration of concentrated dextrose may cause mental confusion and/or loss of consciousness.

     Glucose injection is hypertonic and may cause phlebitis and thrombosis at the site of injection.

     Significant hyperglycemia and possible hyperosmolar syndrome may result from too rapid administration. The physician should be aware of the symptoms of hyperosmolar syndrome, such as mental confusion and loss of consciousness, especially in patients with chronic uremia and those with known carbohydrate intolerance.

     The intravenous administration of this solution can cause fluid and/or solute overloading resulting in dilution of serum electrolyte concentrations, overhydration, congested states or pulmonary edema. Electrolyte deficits, particularly in serum potassium and phosphate, may occur during prolongedv use of concentrated dextrose solutions. Blood electrolyte monitoring is essential and fluid and electrolyte imbalances should be corrected. Essential vitamins and minerals also should be provided as needed. To minimize hyperglycemia and consequent glycosuria, it is desirable to monitor blood and urine glucose and if necessary, add insulin.

Peggy Leake MNS
Infusion Specialist

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.


PREVACID is the brand name for the generic drug LANSOPRAZOLE.This ulcer medication is metabolized through the P-450 and is questionable for use by persons with hepatic porphyria.

CLARITAN - D 24 HOUR is the brand name for the generic IORATADINE SULFATE. This drug now carries a warning for persons with hepatic or renal insufficiency or those with urinary retention. An allergy drug, this drug is considered questionable for use by hepatic porphyria patients.

PRILOSEC is the brand name for the generic OMEPRAZOLE. This drug is metabolized in the liver and influenced by the P-450. This drug will also showed marked elevations in the liver function tests. It is considered questionable for use by hepatic porphyria patients.

NEXIUM is the brand name for the generic ESOMEPRAZOLE MAGNESIUM. A drug for heart burn and acid reflux, this drug is metabolized in the liver and is considered questionable for use by hepatic porphyria patients.

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