PYROLURIA
Pyroluria
is a genetically determined chemical imbalance involving an abnormality in
hemoglobin synthesis. Hemoglobin is the protein that holds iron in the red
blood cell. Individuals with this disorder produce too much of a byproduct of
hemoglobin synthesis called "kryptopyrrole"
(KP) or "hemepyrrole." Kryptopyrrole
has no known function in the body, and is excreted in urine.
Kryptopyrrole has binds to
pyridoxine (vitamin B6) and zinc and makes them unavailable for their important
roles of co-factors in enzymes and metabolism. These essential nutrients when
bound to kryptopyrrole are removed from the
bloodstream and excreted into the urine as pyrroles. Arachidonic acid (an omega-6 fatty acid) is also deficient.
The
effect of pyroluria can have a mild, moderate, or severe depending on the
severity of the imbalance. Most individuals show symptoms of zinc and/or B6
deficiencies, which include poor stress control, nervousness, anxiety, mood
swings, severe inner tension, episodic anger (an explosive temper), poor
short-term memory and depression. Most pyrolurics
exhibit at least two of these problems. These individuals cannot efficiently
create serotonin (a neurotransmitter that reduces anxiety and depression) since
vitamin B6 is an important factor in the last step of its synthesis. Many of
these persons appear to benefit from SSRI medications such as Prozac, Paxil,
Zoloft, Celexa, etc. However, as with all mind-altering drugs, side effects
occur and the true cause of the mental difficulties remains uncorrected. In
addition these individuals often have frequent infections and are often
identified by their inability to tan, poor dream recall, abnormal fat
distribution, and sensitivity to light and sound. As you can imagine an SSRI
will not correct these metabolic effects. More healthful benefits may be
achieved by giving the appropriate supporting nutrients.
Pyroluria
is detected by chemical analysis of the abnormal pyroles
in urine detectable as a purple (on testing paper) metabolite in called
"the mauve factor." Most persons have less than 10mcg of KP per
deciliter. Persons with 10-20 mcg/dl are considered "borderline" pyroluric and may benefit from treatment. Persons with
levels above 20 mcg/dl are considered to have
pyroluria, especially if the above symptoms are present. The chemical analysis
for KP is difficult due to the tendency for this chemical to decompose.
Sometimes it is necessary to repeat the urine test to properly determine the
level of KP being excreted. To make the initial diagnosis, no vitamins or minerals
should be taken for two days before the urine is collected (This is to avoid
false negative results). The specimen should be handled properly as well -
collected and frozen immediately and protected from any light by being placed
in aluminum foil. A repeat test to determine if the condition has been improved
may be helpful.
People
with mild-moderate pyroluria usually have a fairly rapid response to treatment
if no other chemical imbalances are present. People with severe pyroluria
usually require several weeks before progress is seen and improvement may be
gradual over 3 - 12 months. Features of pyroluria usually recur within 2 - 4
weeks if the nutritional program is stopped.
Pyroluria
is managed in part by restoring vitamin B6 and zinc. The type of replacement
therapy is very important as zinc must be provided in an efficiently absorbed
form. Vitamin B6 is also available in several forms. Both zinc and B6
supplementation need to be directed by the doctor as too much can be toxic, use
of the wrong form will be ineffective, and avoiding competing minerals and
supplements may be necessary. Other nutrients may assist in pyroluria include
niacinamide, pantothenic acid, manganese, vitamins C
and E, omega-6 fatty acids and cysteine. Food sources and nutritional
supplements containing copper and red/yellow food dyes should be avoided.
Because
pyrolurics are stress intolerant, they seem to be
especially vulnerable to cumulative stress over many days. For example, parents
of a pyroluric child should use discipline that is
"short and sweet" rather than "long and lingering." It is
not unexpected that pyroluric patients are prone to
relapses, especially during illness, injury, or emotional stress.
