The Lucknow Sentinel, 1990-11-14, Page 3LWknew Sentinel* Welliseetint November 14, its — Page 3
Brian's PKU diet limits intake of protein foods
For Laurie and Mike Goetz. of
Lucknow, the diet of their five-
year -old 5on, Brion, is one &poof
their daily routine that receives the
strictest of attention. Brum was.
born with phenyiketonuria better
known as PKU.
PKU an inherited metabolie
disorder where an enzyme
(phenylalanine hydroxylase)
pwduccd by the liver does not
unction properly. This =Yale
nononllY convert./ phenylalanine,
naturally occurring amino acid
• essential for normal growth in in -
tants and for nitrogen balance in
adults, into tyrosine, another amino
acid. With the conversion block,
• phenylalanine aecumulates in the
body. This accumulation,
if left
untreated, interferes withnormals
brain development, .
It is estimated that one Person out
Of 60 is a corder of the PICU gene,
which is an autosomal recessive
gene, When - two carriers - have
childlen, they have a one -in -four
Chance of having children with
PKU. Laurie and Mike found out,
they were carriers when they had
Brian. Their three-year-old twins,
Michelle and Robin don't have
• PKU and tested negative for the
gene.
• Discovery of the disorder is made
• through routine screening of new-
borns through a heel prick blood
OM For, • over. 20 years, die
government has been sponsoring a
program whereby all 'newborns
• -would be tested for PKU..' It is
• estimated that one out of 15,000
children born yearlY in Canada will
have PKU. If left untreated brain
damage and retardation will result,
Because of the testing, • PKU
children NO diagnosed early, diet
•
therapy is initiated and they grow
up normally.
Discovery . 0
PKU. was discovered in 1933 by
Dr. M Folling in Noway, who
Five-year-eld Brion Goetz,. of Ludcnow, was born. with
better known as PKU. As A result of this, Brian's
diet strictly limits hisintake or protein foods. Brian is shown cutting
a sample of his lice„grispiesquares for the photographer to sample.
Brian loves helping his morn, Laurie, prepare rood and. he was
delighted when the sampler declared the treat, "limm, hinm, good"!
(Pat Livingston photo) •
• -
noted ' the unusual odour of the Diet •
- -
urine of some retarded children. Brian's •diet strictly- limits his
He identified it, 48 phenylacetic intake of protein foods. However,
acid.; He then' went on to develop a he still requires some_phenylalanitte
urine test and determined that PKU in order to maintain normal growth,
was au inherited condition. that can which he his certainly done. As a
happen when both parents are car- newborn, Brian was 1480441 on a
riers. •'•• low ylahmine formula -
„
Lofene Due to the period of
In the 50's, a Getman physician, rapid growth aka baby, blood work.
Dr. IlicHe, discovered diet thereby was done 4'041104Y to check his
• to treat PKU. Laurie said it is phenylalanine levels. Outic visits
interesting to note 'that in those were also frequent at that thee.
years, -the diet was very crude and Brian *. is followed by the PKU •
• consisted mainly of Carrots. In 1961 Clinic at ` the „Hospital, for Si*
a Dr. Gu • e developed the method Children in Toronto, and they are
• used , for blood testing to Very pleased With his . progress,
mak; an- early identification of Blood work is done weekly now. •
•
Laurie draws the blood and sends
GIC Rates
0/ ••NOint- • -
MIL INVFSittens
0 , GUARANTEED amotED
1,1R. • TitAtionnE
INVESTMENT '-,
s4 t,. .1,00
by coOriet to the clinic for Satins.
Clinic visits are twice a year now.
Besides that, Laurie says they have
close phoee contact with their
dietician and nurse in Toronto..
Brian's visits to the PKU Clinic in
Toronto, which is one of five in
Gated°, involve specialists check-
ing his diet history, reviewing
phenylalanine levels and any dineseS SIPCO the it visit. His develop-
ment is monitored and of course ,
blood work -completed, checking for
pun zine, tyrosine, sena protein
and other nutritional factors. This is
the one part of this visit Brian isn't
real keen on, but then, what 11,-
yearold,' would be?
Even though he's a youngster,
Brian seems to understand he must
Where- to his diet. Laurie said Brian
has a special rapport with han-
dicapped people and perhaps he ,
realizes, even at his young age, he
is not so bad off. .
Since the centre in Toronto is.
involved in a lot of research into.
PKU. they like to do LQ, tests on
PKU Children end their siblings
yeasty, - •
Solids, such as fruits and
vegetables are introduced:- in
amounta. 'carefully calculated in a
system of equivalents patterned
after the diabetic diet. Brian Still
' has a special formula he must tate
and enjoys it mixed in a glass of
peach think or TangHe is not
allowed Milk. fish, meat, peanut
butter, cheese. eggs and other foods
high in protein Low promIA
are purchased through, 'a 'specialty
food shop in Toronto and include
breads, pasta and other special
'Products. It is very important that
Laurie, and Mike are aware of all,
the food Brian eats each day, in
Order:. that they can calculate the
..protein intake of thefive-year-old.
• While having such a special diet
for one member of the family may .
seem like a chore,. Laurie says. it •
las had its funny moments. She
recalls the first l'inte she made
cookies for Brien, Instead of in-
di'vidual cookies on the pan, site
bad Ooe hose cookie, thanks to the,
lack or eggs. They woody pur-
chaeed their own bread maker, and
now Brian's bread looks just like
their's. Previously they purchased
his bread in a tin and Laurie said in
• no way did it resemble bread as we
know it. Laurie said tremendous
advances have been made in .; the
last five years regarding products
-
and recipes that ate available for
ante on a PKU diet,
Unlike a child with allergies, a
PKU child doe* not feel maim ill
effects if he/she strays off the diet,
unless of C011114 there' is a sig'
nificant deviation. Neurologic
. damage can be more dangerous
than seems evident. For Brian, a
slight lapse in diet, or if he is ill,
will cause his phenylalanine levels
te7rise:LaUrie and Mike' are aware
of these times because Brian's
attention " span . is .shorter, he
becomes more 'irritable and . he .
simply isn't the happy little boy in
theirfamily. •
At a recent clinic visit *Toronto,
at the University of Toronto, Laurie
andBrian were asked to speak to a
gathering of 250 second year
medical students. For the majority
of. the students, it was their first
time to see and talk m a child with
PKU, since it is ,such a rare disor-
der. Brian Was first on the agenda,
and Lurie said hebroke the ice for, .
hero'. when he step* up the
oicroPhone and Said, my nalue
is Brian, I'in five 'and I have a
dog," For Brian discussing his ,pets
.Was of more importance than his .
medical -disorder, Laurie shared her
feelingsas a parent of a PKU child,
reviewed his • medical • history,
management of Brion's diet and
told the students how well their son
. • Turn to page a •
• ,
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