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.
Testing for Porphyric Peripheral Neuropathy
Peripheral neuropathy is one of the biggest problems that porphyria patients
face on a continual basis long after acute attacks are in remission.
And most porphyria patients know that it takes testing and repeated testing
to confirmed their PN in relationship to porphyria. Often it is mistakened as being early MS or another affliction.
For determing Peripheral Neuropathy [PN] testing by a neurologist must be
undertaken. Such tests include:(1) the simple pin sticking checking for nerve
impulse; (2) the rubber tipped hammer to check for tendon reflexes; and (3)
an EMG [Electromyography].
Electromyography is a diagnostic neurolic test to study the potential
[electrically measured activity] of muscle at rest, the reaction to
contraction, and the response of muscle to insertion of a needle. The test
is an aid in ascertaining whether a patient's illness is directly affecting
the spinal cord, muscles or peripheral nerves.
When EMG testing the patient lies at rest while the peripheral nerves in
various locations are stimulated through electrodes, and the electrical
activity in the muscle at rest, on insertion of the needle, and during the
muscle contraction. The test is sometimes employed as a measure of the
muscle tension produced by nercous stress, usually, the muscles of the
forehead are tested, since they can indicate relaxation of generalized body
Electromyoneurogra[hy is the combined use of electromyography and neurography.
The two tests offer a more precise means of finding the exact location of
nerve damage or disorder.
Dynomometry testing uses a dynomometer [most often a dial guage attached to a
spring mechanism that measures the strength of muscles] to ascertain certain
physical abilities such as holding an object in the hand. It helps detect
diseases of the nerves from the spinal cord to the muscle.
There is negligible risk factor in running this test. All that is involved is
the catheter and needle insertion along with the elctrical instruments.
The pain and discomfort from the test focuses on the needle insertion, which
is usually done without local anesthesia. This can be quite uncomfortable and
at times even very painful.
Intrepretation of the tests must be made by a neurologist. In the normal values,
when the muscle is at rest, no electrical activity is observed. When the
muscles contract, the elctromyograph will show a smooth graphic wavelike
respresentation of each contraction. The graph lines are amplified with the
increase in strength of each contraction.
When the values are abnormal,muscle disease will produce a spiked wave pattern.
The shape of the spike depends on the particular disease.
Muscle weakness produces a diminished wave. With myasthenia gravis, for
instance, the waves disappear for a few minutes. Nerve involvement, as
opposed to muscle involvement, usually shows a decreased frequency of
Neurologist consider the test quite reliable and rate it as 90 percent
accurate. As stated by a neurologist, "it is quite quite difficult for a pretender /malingerer to fake to have
muscle pathology when muscles respond to electric stimulation."
Robert Johnson, M.D.
Latex Sensitivity Needs to Put Porphyria Patients on Alert
While most hospitals and medical clinics have changed over to non-latex
gloves and other medical equipment, the incidence of latex allergy continues
to be documented. Among those reporting reactions to the use of latex are many
Medical history citing latex allergy is quite extensive. Beginning in the
fall of 1989, the Food and Drug Administration (FDA) started receiving
medical reports of patients going into anaphylactic shock while receiving
barium enemas. It was soon found that the latex-cuffed enema tip was the
cause of a total of 16 deaths. That was soon remedied and this began an
increased awareness of latex allergy.
The prevalence of latex allergy in porphyria patients ranges much higher.
Porphyria patients with cutaneous symptomology or those with MCS exacerbation
need to be especially aware of the problems with latex. Many porphyria
patients using intervenous treatments have noted alergic reaction to latex
products used. Caretaker who wear latex gloves for prolonged
periods of time also noted allergic reactions to the latex.
Patients with atrophy have higher risk for latex allergy which has been cited.
The reasons for latex allergy has to do with the processing and source of
latex. Latex is the milky sap obtained by tapping the rubber tree. The raw
product is mixed with a preservative, such as ammonia. It is processed and
it is concentrated.
Numerous chemical accelerators reduce the temperature and time required.
These accelerators can cause allergic reactions and are responsible for many
cases of contact dermatitis.
Chemically, latex contains proteins, cis-polyisoprene, water, and lipids. The
proteins cause the severe immediate hypersensitivity reactions. More than 50
different proteins have been implicated in the allergic response, with up to
a total of 240 different proteins found in latex.
Coated or very soft rubber products appear to have the highest content of
latex proteins and, therefore, have the greatest allergenic potential.
Medical devices that have been reported to trigger serious systemic reactions
by cutaneous exposure include anesthetic masks, tourniquets, electrocardiogram electrodes, adhesive tapes, condom catheters, and ileostomy bags.
The symptoms of such a latex reaction can include redness, cracks, fissures,
and scaling. Another type is that of allergic contact dermatitis. There is
also a delayed hypersensitivity, which has an onset 6 to 48 hours after
contact. Another name for this reaction is "Type 4".
Symptoms, which are also caused by the accelerators and chemicals, include
erythema, vesicles, papules, pruritus, blisters, and crusting. These lesions
can resemble those caused by poison ivy or poison oak. Approximately
80 percent of the immunologic reactions are type 4.
The third type of reaction is immediate hypersensitivity, or type 1-IgE
mediated reaction, which is caused by the latex proteins. Its onset occurs,
within minutes and rarely lasts longer than 2 hours. These symptoms include
local and generalized urticaria, light-headedness, angioedema, nausea,
vomiting, abdominal cramps, rhinoconjunctivitis, bronchospasm, and
anaphylactic shock. It is possible to have used latex for years without
problems and suddenly progress to systemic symptoms. Anaphylactic reactions
to latex have been reported in persons who had previously experienced only
irritant or allergic contact dermatitis.
For porphyric patients the exacerbation of reactions can increase dramatically
and can triggers porphyric attacks as well.
Because any product containing latex can trigger a reaction, cautious
investigation of products at home, in the workplace, and at sites of medical
and dental care should occur. A thorough medical history is the cornerstone
of the diagnosis of latex allergy.
If you are aware of itching or other allergic reaction to latex please notify
your primary care provider. Please note this reaction in your medical records
and carry this information in your wallet. Such reactions are as important to
cite as those of an allergic reaction to penicillin.
Dr. Ernesto Gonzales MD
Chlorine Present in Many Pharmaceuticals
Chlorine is a product that is found just about everywhere these days. And
chlorine is essential and is even beneficial overall. But unfortunately for
most porphyria patients chlorine is considered a chemical toxin which can and
does trigger acute porphyria attacks from time to time.
What is often unknown to many porphyria patients is that chlorine is lurking
just about everywhere in the hospital or clinical medical setting.
Porphyria patients find themselves so often in a medical setting, and
yet many are so unaware of that chlorine is lurking just about everywhere.
This includes even the medications that most people take commonly and even
on a daily basis.
One common drug is acetaminophen. Others include antibiotics. Chlorine is
also found in anti-cancer drugs including cisplatin, and mintotane.
Other drugs containing chlorine include xanax, vancomycin, lorabid, ceclor,
In addition, almost one-third of central nervous system drugs contain chlorine,
and 98 percent of gastrointestinal medications are made using chlorine.
At the same time chlorine is what makes our tap water safe and keeps our
"whites white" during laundry.
Chlorinated compounds are essential to the development of potent new drug
therapies. Of the nearly 400 new drugs approved for therapeutic use in humans
since 1984, more than 60 are chlorinated compounds, and many others use
chlorine's unique chemical properties in their production.
In the medical world chlorine does not just stop with the pharmaceuticals.
Chlorine is essential to a wide variety of medical equipment. An estimated
one-fourth of all medical devices in hospitals contain chlorine, ranging
from some of the most commonly used to some of the most specialized and
X-ray and mammography films are made with silver chloride. Chlorine also is
a basic building block in the silicon used to make the semiconductors upon
which many electronic medical devices depend. And surgical sutures,
artificial blood vessels, and osmotic membranes are all made with nylon, a
product made using chlorine chemistry.
Chlorine-based plastics also are widely used in medical devices and equipment.
Of the 14 families of plastics made using chlorine, the most common is
polyvinyl chloride, a plastic known for being light, easy to bend and shape,
As a porphyric if your are super sensitivity to chemical toxins including
chloride be aware that the IV and blood bags are made of chloride. Same with
the oxygen tents and even prescription eyewear.
Chlorine-based vinyl packaging also adds to the safety of medicine. Many
pharmaceuticals also are supplied in vinyl packaging -- such as the "blister"
packs that help extend the shelf life of tablets and capsules and make it
easier for patients to take the proper dosage.
Much of the aforementioned is safe for porphyrics however if contact is
limited. What is neeeded to be mindful of is the chlorination of drinking
water, and especially chlorinated water in swimming pools. This has always
been a problem for the general populous and with porphyrics it is even more so.
Skin irritation is a great concern for porphyrics with the cutaneous
manifestations. However AIP also can be affected by chlorine.
Play it safe, read labels, ask questions, and do not be afraid to refuse use
of some products.
Robert Johnson, MD
The Association of Vitamin B6 and Porphyria
During the past year or so many porphyria patients have concerned Vitamin B6
protocols for controlling their porphyria. As to date there has not been any
scientific research that would point to this conclusion.
Proponents of the Vitamin B6 regiment often cite that porphyria PN is due in
part to a defiency of Vitamin B6. However medical research has yet to make
any findings to substantiate this theory.
Vitamin B6 comprises a group of closely related compounds: pyridoxine,
pyridoxal, and pyridoxamine.
These chemical elements are metabolized and phosphorylated in the body to
pyridoxal phosphate, which functions as a coenzyme in many reactions.
Such reactions include the decarboxylation and transamination of amino acids,
deamination of hydroxyamino acids and cysteine, conversion of tryptophan to
niacin, and metabolism of fatty acids.
Vitamin B6 components are important in blood, CNS(central nervous system) ,
and skin metabolism.
Vitamin B6 is an important factor in erythropoiesis because pyridoxal
phosphate is needed in the formation of -aminolevulinic acid, (ALA) the
rate-limiting step in heme biosynthesis.
The theory that B6 deficiency is the main cause of porphyric PN, really is not
a consideration when you understand that primary deficiency is rare, because
most foods contain vitamin B6.
Secondary deficiency of B6 may result from malabsorption, alcoholism, oral
contraceptive use, chemical inactivation by drugs (eg, isonicotinic acid
hydrazide, cycloserine, hydralazine, penicillamine), excessive loss, and
increased metabolic activity. Malabsorption is found in many porphyria
patients, however none of these patients show a deficit in B6.
Anticonvulsant drugs are the chief offenders, and these drugs are
contraindicated for most porphyria patients.
In event of a B6 deficiency the vitamin B6 antagonist deoxypyridoxine
produces seborrheic dermatosis, glossitis, cheilosis, peripheral neuropathy,
and lymphopenia. Vitamin B6 deficiency can cause convulsions in infants and
anemia in adults.
It is important to keep in mind that several recessive or X-linked states
affect different vitamin B6 apoenzymes, producing symptoms such as convulsions,
mental deficiency, cystathioninuria, sideroblastic (iron overload) anemia,
urticaria, asthma, and xanthurenic aciduria.
Jeff Brown PhD
PES 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 judgment 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.
PES Monthly Drug Update
ZYBAN is the brand name for the generic drug BUPROPION. It is
classified as an aminoketone drug and is used as an anti-depresant and for
smoking cessation. Side effects of this drug include abnormal liver funtion, photosensitivity, jaundice, hepatitus, pancreatitis, edema, peripheral edema, leukocytosis, muscle rigidity, leg cramps, muscle weakness, depersonalization, neuropathy, and liver damage. WARNINGS & PRECAUTIONS: Not recommended for
persons with liver disease.
ZOLOFT is the brand name for the generic drug SERTALINE.Specific
inhibitor of neuronal seratonin re-uptake It is used to treat major depressive
disorders and depression. Side effects of ZOLOFT include disturbances,
nausea, diarrhoea/loose stools, dyspepsia, tremor, dizziness, insomnia,
somnolence, increased sweating, dry mouth, male sexual dysfunction (primary
ejaculatory delay). Palpitations. Headache, paraesthesia, hypoesthesia,
twitching, hypertonia. Agitation, nervousness, anxiety, yawning, female
sexual dysfunction, impaired concentration. skin rash, rhinitis, pharyngitis, Constipation, vomiting, flatulence, anorexia, abdominal pain, appetite increase.
vision abnormalities, tinnitus, taste perversion. micturition frequency, micturition
disorders, fatigue, fever, hot flashes, back pain. Thirst, myalgia. Liver enzymes elevations of SGOT, SGPT Hyponatraemia, possibly due to inappropriate ADH secretion.. This drugs carries a WARNING for patients with diseases or conditions that affect metabolism, patients with cirrhosis , or liver impairment , since SERTALINE is significantly metabolized in the liver.
MEPROLONEL is the brand name for the generic drug METHYLPREDNISOLONE.
This drug is classified as a corticosteroid drug, anti-inflamatory,
anti-arthritic. It has many uses WARNINGS & PRECAUTIONS:
Meprolonel should not be used in persons with an underactive thyroid,
liver cirrhosis, or other liver disease.: Meprolonel may cause
cataracts, glaucoma (increased eye pressure), and eye infections. high blood
pressure, salt and water retention, and potassium and calcium loss or
asthma. May aggravate existing emotional problems or cause new ones