An InternationaI Survey of EducationaI TooIs

Used in PKU Dietary CompIiance

L.E. Bernstein
1
, C.J. White
1
, J. HeIm
1
, J.C. Rocha
2
, M.F. AImeida
2
, R. M. Link
3
1
ChiIdren's HospitaI CoIorado, Anschutz MedicaI Campus, USA;
2
Centro de Gentica Mdica Jacinto de MagaIhes, INSA, IP, PORTO, PORTUGAL;
3
Chair SSIEM-DG, WIESBADEN, GERMANY
Methods
An international survey was developed to
examine educational tools available for
patients with PKU ages 3-21 years. The
survey was composed of 14 questions which
were developed by the authors and
distributed via a secure website link. The
answers represent participants from clinics
in the United States, Canada, Europe,
Central America, South America,
Scandinavia, and Australia (Figure 1).
Discussion and ConcIusion
The continuous management of blood
Phenylalanine levels continues to be the
prominent marker of dietary compliance
in PKU patients. However, formula
ingestion, regular monitoring of levels,
and clinic attendance are also important
when defining dietary compliance. The
importance of education is underlined by
the fact that the majority of respondents
offer nutrition education in their current
practice. Nevertheless, the educational
strategies used varied without a clear
adjustment to age. Although the majority
of responders were from the US and
Europe, this survey identified the need to
have a standardized definition of dietary
compliance in order to better define the
educational strategies and tools used with
varying age groups. The authors
concluded that a study addressing the
effectiveness and correlation of
educational tools with regards to PKU
dietary compliance would be very
welcome.
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Figure 2.
Definition of dietary compIiance by the
respondents
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Figure 1. Participating
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CenLral Amerlca
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20
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60
70
80
90
100
Figure 5.
Factors that May Contribute to
Non-compIiance

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Background
Considering the struggles surrounding
dietary compliance of patients with
Phenylketonuria (PKU), education is of vital
importance to the long term success of
dietary compliance. However, limited data is
available regarding the educational tools
routinely used and their impact on dietary
compliance in patients with PKU. The aim of
this survey was to ask dietitians and
physicians to define the term dietary
compliance and to evaluate the level and
types of educational tools currently in use at
international clinics specialized in treating
patients with PKU.
3
3S yrs
1012 yrs
131S yrs
1618 yrs
1820 yrs
69 yrs
21+ yrs
Figure 4. EducationaI Methods
Abstract
An international survey was developed to
examine educational tools available for
patients with Phenylketonuria(PKU), ages 3-
21. This survey represented participants from
the United States, Canada, Europe, Central
America, South America, and Australia.
Dietitians and physicians were asked to
define the term dietary compliance for their
individual practices. Phenylalanine levels
"within treatment range were chosen by
92.1% as a definition of compliance. A
questionnaire was distributed through the
Metabolic Dietitians list serve PNO-
Metabl@listserv.cc.EMORY.EDU
Ninety anonymous responses were analyzed
for data. A majority of those responses
(.9%) offer PKU nutrition education for
patients and families. t was determined that
one-on-one counseling (97.%) is the most
commonly used method for education with
printed material at 4.4%. Nutrition education
is provided every six months by over half
(52.3%) of the respondents with 54% starting
at ages 3-5. Diet management appears to
decline during adolescents, when one-on-one
counseling is the primary tool. n addition,
parents as role models fall from the 7th to
the 17th percentile. Group clinics are the least
utilized educational tool. Despite education,
factors that play a role in dietary compliance
are embarrassment and frustration (9%) and
poor family cohesion (3.3%).
%
%
One-on-one Counseling
Printed Material
Handouts
Hands-on Experiences
Parents as role models
Group Clinic
None of these
ResuIts
The majority of the responders (92.1%)
defined dietary compliance as maintaining
Phe levels within treatment range (Figure 2).
1% of participants offer PKU nutrition
education (Figure 3).
One-on-one counseling and printed material
are the top two methods used for all age
groups, averaging 9.15% and 72.%
responses respectively (Figure 4).
Parents as role models is the primary
educational method used in ages 3-5 but
falls off drastically from 7.5% to 17.7% by
age 1 (Figure 4).
From ages 6-21, one-on-one counseling is
the most commonly used tool ranging from
2%-97.7% of respondents (Figure 4).
Hands-on experiences peaks in use at ages
16-1 years with 51.2% of clinics using this
tool (Figure 4).
42.2% of clinics do not offer cooking
classes.
1% of clinics view embarrassment as a
leading factor affecting compliance (Figure
5).
54.4% of clinics start education at 3-5 yrs.
PKU nutrition education is conducted once
every six months in half of the clinics
(52.3%).
An InternationaI Survey of EducationaI TooIs
Used in PKU Dietary CompIiance
L.E. Bernstein
1
, C.J. White
1
, J. HeIm
1
, J.C. Rocha
2
, M.F. AImeida
2
, R. M. Link
3
1
ChiIdren's HospitaI CoIorado, Anschutz MedicaI Campus, USA;
2
Centro de Gentica Mdica Jacinto de MagaIhes, INSA, IP, PORTO, PORTUGAL;
3
Chair SSIEM-DG, WIESBADEN, GERMANY