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.
Weakness Experience by Most Acute Porphyrics
Most all acute porphyria patients experience muscle weakness from time to
time and especially during and immediately following an acute attack of
Muscle weakness or a lack of strength is a reduction in the strength of
one or more muscles.
Such weakness in porphyria is a very important symptom.
The feeling of weakness may be subjective.
"Subjective" means that the person feels weak but has no measurable
loss of strength or "concrete evidence" which is defined as a measurable
loss of strength.
Weakness may be generalized or defined as a total body weakness. Weakness
may also be localized to a specific area such as a limb, one side of the
body, a side of the head, and such.
A subjective feeling of weakness usually is generalized and associated
with infectious diseases such as infectious mononucleosis and influenza.
Weakness is particularly important when it occurs in only one area of the
body. Such a weakness is considered a localized or focal weakness.
Localized weakness may follow an acute attack of porphyria. Often such
weakness is found in conjunction peripheral nerve problems.
The common cause of such muscle weakness may result from a variety of
conditions including metabolic, neurologic, and toxic disorders.
This includes most of the porphyrias.
The toxic triggering of muscle weakness has been found to be largely in
part due to pesticides, fungicides, herbicides, rodenticides, and paint
and other chemical inhalations.
In most porphyria patients such weakness results in periodic paralysis
which is potassium related, such as hypokalemic periodic paralysis which
occurs when the potassium blood serum levels diminish.
Also other medical conditions by porphyria patients especially autoimmune
disorders that interfere with the transmission of nerve impulses to
Porphyria patients that use chloroquine will also experience extreme
muscle weakness. and while used to treat one form of porphyria the use of
chloroquine by the other seven main forms of porphyria is contraindicated.
There may be other causes of weakness. This list is not all inclusive, and
the causes are not presented in order of likelihood. . Furthermore, the
muscle weakness may vary based on age and gender of the affected person,
as well as on the specific characteristics of the symptom such as location,
quality, time course, aggravating factors, relieving factors, and
Dr. Robert Johnson M.D. PhD.
Insurance Coverage for Porphyria Testing & Treatment
Disorders of porphyrin metabolism are now widely accepted by the medical
community and coding is firmly establish .
The "porphyrinurias" have been listed by the International Classification
of Diseases since 1920 (currently ICD 277.1).
The diagnostic need for detailed biochemical testing has been well
established in the medical literature (urine and/or stool testing alone
Also among medical insurance providers there is broad agreement that the
primary focus of treatment in acute cases must be on the avoidance of
exposures that may trigger an attack. A few people are having problems
with getting cocerage for home infusion, but the majority are having full
coverage. [In some states home infusion is considered home care and unless
you are HOMEBOUND you can not receive coverage.
It is recommended by medical insurance providers that if this is your case
that you have your PCP contact their office and ask for the specific
"CASE MANAGEMENT" which will allow for home infusion without being
Since so many types of porphyria have such potentially serious
consequences, there is no excuse for denying complete and prompt
evaluation in any suspected case.
If a person has bills where the coverage has been denied the majority of
today's hospital and major clinic have Patient Representatives which will
help patients get proper medical coding and insurance coverage.
Lynette Stevens LSW
Brain Fog is considered a Cognitive Disturbance.
Acute porphyria patients often describe their ability to focus and remember
or function as being in a "brain fog". Such "brain fog" is actually known
as a cognitive disturbance.
Cognitive disturbances involve a difficulty in basic mental operations such
as memory, paying attention or focusing attention on something, and in
They also involve shifting attention from one subject or idea to another.
People with cognitive disturbances have trouble in perceiving accurate
spatial relationships between objects, in comprehending or expressing
language, and performing calculations, and in a number of other areas.
There is a decreased ability to track two processes at once, something we
usually take for granted. This ability requires a rapid shifting of
attention. Many porphyria patients during the height of an acute attack
find this normally simple task as most difficult or impossible.
Suddenly there are two things that you need to monitor and pay attention to
at the same time. This might have come easily to you at one time, but if
you now have 'brain fog," it's very hard.
Brain fog is the having of conflicting emotions inside of you You csuddenly
find that you have the inability to prioritize and use an effective order
in carrying out details.
When there are two different things you want to do at the same time. Other
dimensions of CNS disturbances often are present which leads to sometimes
disorientation, anxiety and the sensation of confusion.
Because of the porphyria patient's cognitive problems one may find it very
difficult to express confusion.
There is also the difficulty in handling sequences. Again prioritizing is
next to impossible. It pertains to the mixing up of words and syllables when
It also includes the transposing or reversing of letters or numbers. In
addition there is the having trouble tracking the flow of a normal
conversation or the sequence of events in a story or article.
Often there is decreased mental stamina. Focus is next to impossible.
Concentration is very hard.
Intellectual concentration is very fatiguing. Many porphyria patients
relate that they find it most difficult if at all possible to finish
watching a 90 minute video.
There is also decreased memory retrieval ability.
Decreased sense of internal certainty. Porphyria patients find them most
unsure of common ordinarily experiences.
Many times porphyria patients reflect on the dimensions of brain fog and
state that they experience a decreased ability to grasp the large whole
For most acute porphyria patients the negative aspects of "brain fog"
disappear as does the acute attack itself.
In those porphyria patients with chronic smoldering effects and especially
for those with chronic pain "brain fog" will often linger. Exercise and
quality rest are the essentials to fighting such "brain fog."
Rosalie Jones FNP
Urine Color An Evidential Clue in Suspecting Porphyria
The first porphyria test that most porphyria patient have had is that of
urine tests. For most patients it was a 24 hour collection, but for others
it has been the simple "window sill" test.
And the "window sill" test is about darkening and various colors. If one
remembers the documentary film "The Maddening of King George III" you will
recall vividly how the royal court paraded around hoisting the "royal pee"
that was said to be a royal wine purple.
However in most people urine color ranges from light yellow to dark amber,
depending on the concentration of solutes in the urine.
Pigment gives the urine the various colors and shades. The pigment is known
as urochrome which characteristically is a yellow color.
Most often the urine of porphyria patients may only appear after the urine
contacts the container or the water in the toilet bowl. Sometimes the urine
has to sit in the sun or a bright light before the color appears.
For most people today it is unusual for individuals to even take note of
coloration since most have been properly trained not to loiter in the
bathroom therefore flushing away the urine, washing our hands and leaving,
urine coloration unnoticed.
What do various urine colors mean in normally healthy persons? White or
cloudy urine is most commonly a result of phosphaturia or due to pyruia.
Pink or red urine should prompt a call to your physician and a dipstick test
for blood carried out. This could mean hemoglobinuria.
Red blood cells in the urine may be from kidney loss (termed "glomerular"
blood cells) in patients with renal disease. Red cells in the urine of these
individuals will usually be misshapen and accompanied by protein in the
urine. If this is ruled out then a full urinalysis needs to be undertaken.
Complete urine testing (called "urinalysis") for the presence of bacteria
and white blood cells, urine culture, cystoscopy, intravenous pyelogram,
and/or other imaging studies my be necessary to clarify the source of the
Some medications can turn the urine shades of red or pink. It is important
to let the physician know every mediciation you have taken including OTC
Orange urine may be produced by phenazopyridine (Pyridium) or ethoxazene
The drugs Rifampin, phenacetin, sulfasalazine, Vitamin C, riboflavin, and
carrots will also turn urine dark yellow to orange.
Blue or green urine may be caused by a blue dye such as methylene blue, a
component in several medications The anesthetic, propofol can also cause
green coloration of the urine. Amitriptyline, indomethacin, resorcinol,
triamterine, cimetidine, phenergan, and several multivitamins also lend a
blue-green tint to the urine.
Brown or black urine may be due to copper or phenol poisoning The eating
of large amounts of rhubarb, fava beans, or aloe can cause dark brownish
black urine. The drugs chloroquine and primaquine, furazolidone,
metronidazole, nitrofurantoin, cascara/senna laxatives, methocarbamol, and
sorbitol can also produce a black urine.
In porphyria patients, again urine will present in various colors.The
general spectrum runs from reddish wine to dark brown tea colors.
Urine collected in a glass container and set in the sunlight will darken
significantly in porphyria patients.
Lillian Jenseon MS
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.
ZYRTEC is a brand name for the generic drug CETIRIZINE which
is a non-sedating antihistamine The drug carries a warning with it for
those who have liver or kidney disease, or electrolyte abnormalities or
HYZAAR is the brand name for the generic drug LOSARTAN POTASSIUM
HYDROCHLOROTHIAZIDE. This drug is used for the treatment of
hypertension. Side effects of this drug include anxiety, anxiety disorder,
ataxia, confusion, depression, insomnia, memory impairment, migraine,
nervousness, panic disorder, paresthesia, peripheral neuropathy,
sleep disorder, somnolence, tremor, vertigo; alopecia, flushing,
photosensitivity, sweating, blurred vision, urinary tract infection.
weakness, jaundice, muscle spasm; restlessness; renal dysfunction,:
Hepatitis has been reported Liver enzymes can be elevated. Electrolyte
imbalance can occur. WARNING: Losartan Potassium-Hydrochlorothiazide:
HYZAAR is not recommended for patients with hepatic impairment who
require titration with losartan. The lower starting dose of losartan
recommended for use in patients with hepatic impairment cannot be given
AVANDIA is the brand name for ROSIGLITAZONE MALEATE, a
thiazolidinedone (TXD) used to treat type II diabetes. This drug carries
the WARNIG - Not to be used by persons with liver disease.