Phenylketonuria (PKU) - Aspartame
Phenylketonuria (PKU) is a rare, inherited metabolic
disease that results in mental retardation and
other neurological problems when treatment is
not started within the first few weeks of life.
When a very strict diet is begun early and well-maintained,
effected children can expect normal development
and a normal life span.
Elevations of blood phenylalanine
are dangerous for those with PKU, and require
treatment and modification. A child with a level
less than 6 mg/dl does not need to be on a special
diet and is not risk for mental retardation, but
should avoid aspartame at all cost due to the
50% phenylalanine levels.
Scientists have been able to show that carriers
had higher phenylalanine levels in their urine
than non-carriers.
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Phenylalanine Hydroxylase
The disease arises from the absence of a single enzyme
(phenylalanine hydroxylase). This enzyme normally converts
the essential amino acid, phenylalanine, to another
amino acid, tyrosine. Failure of the conversion to take
place results in a buildup of phenylalanine. Through
a mechanism that is not well understood, the excess
phenylalanine is toxic to the central nervous system
and causes the severe problems normally associated with
PKU. Not every child has the same degree of enzyme deficiency,
however; some have enough enzyme activity that the diet
can be quite liberal, while others must have the very
strict diet. The nature of the diet for an individual
child must be determined by an experienced PKU treatment
program.
Phenylketonuria is carried through a "recessive" gene.
The incidence of carriers in the general population
is approximately one in fifty people. Although PKU affects
only one out of every approximately 10,000 babies born
in the US, there are several hundred babies diagnosed
and put on a strict diet each year.
Phenylketonuria Diet
The diet for the most severe form of phenylketonuria
eliminates all of the very high protein foods since
all protein contains phenylalanine. This means that
all concentrated sources of protein must be eliminated
from the diet in order to limit the amount of phenylalanine.
Except in rare circumstances, the diet does not allow
consumption of meat, fish, poultry, milk, eggs, cheese,
ice cream, legumes, nuts, or many products containing
regular flour. A synthetic formula is used as a nutritional
substitute for the eliminated foods.
Phenylketonuria Means NO Aspartame!
In the early days of treating PKU, clinicians believed
that the PKU diet could be stopped at an early age around
6-10 years. However, it is now known that stopping the
diet can result in a variety of serious problems. These
include drops in IQ, learning disabilities, behavior
problems such as hyperactivity and irritability, neurological
problems such as tremors, eczema and other skin disorders,
and personality disorders including schizophrenia, panic
attacks and agoraphobia – all symptoms of aspartame.
As a result of the problems that have developed in young
people who have discontinued the diet, it is believed
that the diet should be maintained for a lifetime.
It is also now believed that keeping blood phenylalanine
levels in the range of 2-6 mg/dl is the safest, especially
in infancy and early childhood. Frequent blood monitoring
to achieve this goal is critical. All individuals, male
or female, who have stopped the diet, would be wise
to return to the diet under the supervision of a PKU
treatment program.
Phenylketonuria and Aspartame
This sugar substitute, sold commercially as Equal and
NutraSweet, was hailed as the savior for dieters who
for decades had put up with saccharine's unpleasant
after taste. There are quite a few problems with aspartame.
The first is phenylketonuria (PKU). One out of 20,000
babies is born without the ability to metabolize phenylalanine,
one of the two amino acids in aspartame. Toxic levels
of this substance in the blood can result in mental
retardation. Beyond PKU, several scientists believe
that aspartame might cause altered brain function and
behavior changes in consumers. And many people have
reported the following side effects from aspartame:
- Fibromyalgia Syndrome and symptoms of Fibromyalgia
- Multiple Sclerosis symptoms
- Dizziness
- Headaches
- Menstrual problems
Are You A Carrier?
Scientists have been able to show that carriers had
higher phenylalanine levels in their urine than non-carriers.
As far as I am aware, more than 100 different mutations
have now been found with corresponding enzyme activities
ranging from zero to normal. So, it’s better to
be safe than sorry. Avoid aspartame’s levels of
phenylalanine and protect yourself and your children’s
health.
Phenylketonuria Links
http://www.pkunews.org/
http://www.yourgenesyourhealth.org/ygyh/mason/ygyh.html?syndrome=pku
http://users.rcn.com/jkimball.ma.ultranet/BiologyPages/P/Phenylketonuria.html
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