PKU watch
Dr Anita MacDonald
One of the UK's top paediatric dietitians, Anita’s specialism lies
with inherited metabolic disorders. She spends 50 percent of her
Consultant Dietitian in Inherited
professional time in clinical work with children and 50 percent
researching and teaching.
Metabolic Disorders
Weaning: The inside story
Introducing solids into an infant’s diet ‘normal’ feeding development so that
is a process that all parents have to
they can understand why problems
undertake. For many first-time par-
occur, they appreciate that mess and
ents, weaning is approached with
food wastage is inevitable, and that
apprehension, even when there are
it is essential that children are encour-
no dietary restrictions, so in PKU, the
aged to self-feed. In most children the
introduction of low phenylalanine
number of phenylalanine exchanges
solids into an already complex and
permitted is likely to be low, so it may
alien feeding regimen is, understand-
help caregivers to feel in control if they
ably, very daunting.
concentrate on giving low volume,
For many experienced metabolic
well tried and tested favourite ex-
dietitians, the weaning of a baby with
change foods that are easy to ad-
PKU may appear straightforward, but
minister (cereal, yoghurt, custard), but
there are no studies in PKU that report
the main focus of any meal is ‘free,’
solid food introduction, and dietitians
low phenylalanine foods, so the latter
may have to spend endless hours sup-
become the accepted norm for the
porting and helping parents overcome
baby, which are given by self-feeding.
many of the daily issues they face. In
addition, if weaning does not advance
Packaging and choice
as expected, it can have adverse con- ‘I just cried when I saw all the pack-
sequences, affecting both long-term aging. It all looks so bland and all the
diet quality as well as overall metabolic packaging looks the same.’
control. To examine the weaning is-
Mother of a four-month-old baby learning
sues, it is useful to consider some of the
about the choice and availability of special
parents’ and other caregivers’ com-
low protein foods.
ments about this process. Unfortunately there are few spe-
be involved, e.g. grandparents, other
‘I have to continue to spoon feed
cialist weaning foods that have been
relatives, nursery workers, and child-
her, because I know that this way she
developed for infants on low protein
minders. Sometimes the full extent of
has had all her phenylalanine ex-
diets. Many parents find coping with
the involvement of ‘untrained oth-
changes.’
PKU challenging in the first year. They
ers’ does not become apparent until
Parent of a two-year-old child with PKU who
are likely to lack confidence with the
there is a problem with the blood
phenylalanine control. At the outset,
is eating mainly commercial based weaning diet; they may not yet fully understand
foods.
it is useful to offer and promote an
the full range of suitable foods avail-
extended teaching service to other
This is a common problem, with no
able for their child, and they may be
caregivers. If parents are providing
easy solutions. There are many dietary
very vulnerable, emotionally, still trying
dietary advice to others, this may lead
components to a low phenylalanine
to adjust to the diagnosis of their baby.
to ‘over caution’ or even ‘under play’
diet, including administration of protein
The availability and packaging of
the importance of diet and, conse-
substitute, measuring phenylalanine
suitable low protein foods is important.
quentially, any existing dietary issues
exchanges and encouraging low
Parents need reassurance that the
are likely to proliferate. It is perhaps
phenylalanine ‘free’ foods as well as
low phenylalanine diet is acceptable
unfair to expect new parents, who are
paying attention to ‘normal’ feed-
and it is so disappointing that there are
themselves still learning, to be able
ing development. Parents may be so
few appropriately packaged weaning
to fully explain a low phenylalanine
worried about ensuring that the exact
foods that are especially developed
diet to others. Like parents, although
amount of phenylalanine is eaten, for a low phenylalanine diet.
all caregivers need to understand the
they give this excessive attention, and
‘I was so excited the first time I saw
principles of treatment, they prefer
it may even be a precursor of long-
him eat a full dinner; I did not realise
very practical dietary advice.
term food refusal or pernickety eating.
there was so much choice.’ The nursery
Even if a child is not spoon fed, some
cook of an 11-month-old baby. She had Positive attitude and good routine
children are offered only phenylala-
just received formal training about how to
nine exchange foods at mealtime. prepare the low phenylalanine diet. Up until
‘We always eat later after she has
In order to help with this issue, it is
then she had thought that all he could have gone to sleep, so we never eat to-
important that parents understand that
was pureed apple, jam and commercial low
gether.’
protein bread.
during weaning there is a change from
Mother of eight- month-old baby.
dependence on the caregiver to the Much energy is given to the dietary
‘We have no set pattern to feeding;
development of independent feeding education of parents of PKU infants,
each day is different.’
skills. Parents need information about but many other caregivers may also Caregiver of six-month-old baby.
28 NHDmag.com Mar '10 - issue 52
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