Porphyria Educational Services
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
Specific recommendations for the confirmed diagnosis and treatment of any individual
must be accomplished by that individual and their personal physician, acting together
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
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
Since the attacks are episodic but the lesion producing the attacks is fixed,
the neuropathy is usually a waxing and waning with permanent
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
An agonist effect is known as binding. The blocking effect is known as an
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
all patients presenting with pain. Opiods are just one of several options.
Robert Johnson, M.D.
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
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
Other abnormal findings in porphyria patients have been those of Protein C and
Protein S deficiences, and anticoagulaiton abnormalities. The inherited Factor V
been detected in many AIP patients while blooding irregularities have also been
High cholesterol is also indicated in testing values of nearly 60% of hepatic
porphyria patients. The liver controls the production and excretion of
Imbalances in electrolytes are often cited as a leading cause of mental changes
porphyria patients. The liver in normally healthy perosns stores extra vitamins,
minerals, and sugars to prevent shortages In porphyria patients potassium will
Sheila Brandt NP
ALDEHYDES IN MEDICAL SETTINGS
Aldehydes which are mostly composed of formaldehyde, are found in many
where we live and work. Moreover they are even found in our medical
Aldehydes are considered chemical toxins and have been known to causes
symptoms among chemical sensitive persons and to help in the triggering of
attacks among porphyria patients.
The resins that are used in particle board and fibreboard are loaded with
Such fibreboard is often used in the admissions area, business offices,, and
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
Such moulded plastics or thermoplastics contain cellulose esters. Not only are
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
are the radiology departments. Photography for hardening gelatin plates and
flatting solution, hypo test solution, hardeners and toners, all contain large
aldehydes. Often photo labs connected to radiology are interior and not well
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
Aldehydes are use in preserving and coagulating rubber latex. Vinyl gloves also
a considerable amount of aldehydes. This often includes iv infusion tubing,
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,
Unfortunately aldehydes are found in bed linen which is often used in
Hospital print shops are another major source of aldehydes. Printing - etching
materials.for chrome printing and developing all contain aldehydes.
Raymond Nelson PhD
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
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
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.