Porphyria Educational Services
Monthly Newsletter
July 2002

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.

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.



PERIPHERAL NEUROPATHY & CNS SYMPTOMS IN PORPHYRIA

Acute intermittent porphyria should be suspected in every patient with acute or episodic neurological or psychiatric distrubances

. In the various varieties one should see porphyrins in urine and/or stool during the attacks, but not necessarily between attacks.

There is often hypercholesterolemia, hyperlipidemia, glucose interlerance and increased CSF protein levels.

Definitive diagnosis requires measuring PBG deaminase activity in erythrocytes.

Since the attacks are episodic but the lesion producing the attacks is fixed, the neuropathy is usually a waxing and waning with permanent changes.

 Leslie McNutt PhD
Neurology & Physiology


PORPHYRIA PAIN AND OPIOD ANALGESICS

Opioid analgesics are centrally acting agents. These opioids provide fast pain relief by either binding or blocking opiate receptors in both the brain and the spinal cord.

An agonist effect is known as binding. The blocking effect is known as an antagonist effect.

Opiods are also known as narcotics.

Opiods can play a role in the management of some chronic pain conditions and this includes many of the acute hepatic porphyric pain.

It is thought by many chronic pain specialists that non-addictive personalities of porphyria patients who use the opiods specifically for their analgesic effect have a very low possibility of addiction. Those patients however who use such opiods because of the their euphoric effects have a far greater possibility of becoming addicted to such drugs.

Nonetheless, because of the social stigma and also in many places the legal issues that of opiods, there continues to be a barrier to both the patient's ability to comply and the physician's ability in prescribing. For these reasons non-narcotic analgesics are often preferred as the first-line of therapy for porphyric patients for their often chronic pain.

Since January1, 2001, all physicians must address the treatment of pain control in all patients presenting with pain. Opiods are just one of several options.

 Robert Johnson, M.D.
Retired Clinician
Porphyria Investigator



LIVER FUNCTIONS AND PORPHYRIA

Liver functions are important when discerning the porphyrias.

One of the liver's many important functions is that it regulates the blood's ability to clot.

The liver also breaks down alcohol, monitors and maintains the right level of numerous chemicals and drugs in the blood. It has been noted that in many of porphyria patients that are found elevated liver function values, specially the alkaline phospatase and the SGOT are often elevated.

Other abnormal findings in porphyria patients have been those of Protein C and Protein S deficiences, and anticoagulaiton abnormalities. The inherited Factor V Leiden has been detected in many AIP patients while blooding irregularities have also been cited.

High cholesterol is also indicated in testing values of nearly 60% of hepatic porphyria patients. The liver controls the production and excretion of cholesterol.

Imbalances in electrolytes are often cited as a leading cause of mental changes in porphyria patients. The liver in normally healthy perosns stores extra vitamins, minerals, and sugars to prevent shortages In porphyria patients potassium will often fall.

 Sheila Brandt NP
Gastroenterologist &
Internal Medicine



ALDEHYDES IN MEDICAL SETTINGS

Aldehydes which are mostly composed of formaldehyde, are found in many substances where we live and work. Moreover they are even found in our medical establishments.

Aldehydes are considered chemical toxins and have been known to causes various symptoms among chemical sensitive persons and to help in the triggering of acute attacks among porphyria patients.

The resins that are used in particle board and fibreboard are loaded with aldehydes. Such fibreboard is often used in the admissions area, business offices,, and ward clerical areas of a hospital or clinic.

While hospitals and clinic need to be comfortable and pleasing to the eye, new carpet, thermal drapes, furnishings, artificial silk, all contain aldehydes.

In many of today's modern clinical laboratories moulded plastics are used exclusively. Such moulded plastics or thermoplastics contain cellulose esters. Not only are the building fixtures toxic, but the actual laboratories are filled with aldehydes such as a fixative for preservation of specimens and tissue samples.

Another place in medical complexes that have been found to be loaded with aldehydes are the radiology departments. Photography for hardening gelatin plates and papers, print flatting solution, hypo test solution, hardeners and toners, all contain large amounts of aldehydes. Often photo labs connected to radiology are interior and not well vented.

Some vaccinations such as pertussis, ’flu vaccinations; synthesis of vitamin A, all contain forms of aldehydes. Aldehydes are also used in the manufacture of antibiotics; chelating agents, and wart paint. Contraceptives are full of aldehyde elements.

Aldehydes are use in preserving and coagulating rubber latex. Vinyl gloves also contain a considerable amount of aldehydes. This often includes iv infusion tubing, wristbands, monitor cuffs, and gurney strapping.

Fabric sizing resins for crease resistant clothes which make fibres wrinkle resistant, water resistant, dye fast, flame resistant, shrink proof, moth proof and more elastic, are loaed with aldehydes.

Aldehydes can be found in rayon, rayon-acetate blends, "wash and wear" cotton, shrinkproof wool, polyester blends, artificial silk. Fabric conditioners, softeners.

Unfortunately aldehydes are found in bed linen which is often used in hospitals.

Hospital print shops are another major source of aldehydes. Printing - etching materials.for chrome printing and developing all contain aldehydes.

 Raymond Nelson PhD
Enviromental 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.
FULVICIN is a brand name for the generic drug GRISEOFULVIN. It is an antifungal drug. This drug carries a warning against use by patients with porphyria.

LEHYDAN 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.

DONNAZYME is a brand name for the generic drug PANCREATIN. It is primarily presecribed to aid in the digestion of food. It carries a warning for patients with 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.

OMIZAC 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.