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Polly copes with zero protein diet
When little Polly Smith was days old,
a straightforward heel prick test
revealed that she had PKU, Phenylketonuria.
This occurs in 1 in 50,000 babies and is a
genetically inherited, missing enzyme
condition which means that Polly’s body
doesn’t break down protein effectively
enough for her to have any large amounts
of protein in her diet. This means she has
to have a carefully managed low to zero
protein diet.
Mum, Alice, had to stop breastfeeding immediately
and feed her a special formula. “We were completely
shocked and very anxious about what this might
mean for Polly and how we would manage it as a
family. We were referred to Great Ormond street
where Polly had more tests and a full assessment. “
The family received fantastic support and they
were advised that the treatment would be totally
diet based for life in addition to daily amino acid
supplements. Protein can build up over time, causing
behavioural changes, irritability and neurological
changes which could lead to brain damage so must
be avoided. The early tests were absolutely vital as it
cannot be treated over 9 months old.
her diet is being constantly adjusted
Now, Polly is 7 and overall her condition is managed
well. Her diet obviously plays a huge part in her daily
life, she has to have measures of food dependent
on their protein contents and her diet is being
constantly adjusted. Every two weeks she has blood
tests to check her protein levels. If she has been ill,
the levels will automatically rise so they have to be
managed by diet adjustment.
Alice says “A lot of her food is on prescription and
I do a lot of home baking with special flour and
meats. She likes pizza, veggie sausages and burgers
like most young children but it does mean we can’t
be spontaneous although restaurants go out of their
way to help us so we just have to plan in advance.
Her school, Castle Lower, is very supportive- she
takes packed lunch and they keep her supplements
polly with her younger sister Matilda and brother, Will
for her and also have a provision of her sweets there
for class birthday treats.”
On a day to day basis, Polly copes really well and
understands what she can and can’t have, parties and
Christmas times are difficult as she can’t have party
food. She tells people she has a “special tummy” and
it will make her poorly.
Great Ormond Street hold an annual Christmas
party where all the food is PKU compatible and
Father Christmas comes and Polly gets to join in
all the fun and forget about it for a while! When
she is 8, she has an opportunity to enjoy a youth
hostel adventure weekend aways supported by the
NSPKU Charity where they help her to develop her
confidence and support her to start managing her
diet independantly.
Polly’s family worry it may become harder as she
gets older but for now it’s manageable. Sometimes
the tolerance will improve with age but she may
need to continue her diet into adulthood. For now,
though, they keep it all in perspective and like any
other normal 7-year old, riding her bike and having
fun with her friends is the most important thing!
for more information on pKU visit:
www.pku.com
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