Validation of Quality of Life Questionnaire 'Simple Measure of Impact of Lupus Erythematosus in Youngsters (SMILEY)', MEC-2008-086

Pediatric SLE is a chronic, multi-system disease with wide ranging effects on
their quality of life (QOL). The multidimensional aspects of QOL, the
biopsychosocial implications of SLE, children*s evolving needs and changes in
cognitive function, and proxy respondents pose challenges in measuring
SLE-specific QOL in these patients. We developed a brief, new HRQOL measure
that is valid in US English, called *Simple Measure of Impact of Lupus
Erythematosus in Youngsters© (SMILEY©).
Health-related QOL (HRQOL) has been identified as an integral component of a
core set of outcome measures for the evaluation of response to treatment in
patients with pediatric SLE. Recent therapies have significantly prolonged the
survival in pediatric SLE. In order to measure the impact of morbidity of SLE
on their lives and assess the outcome of new therapies, it is critical to have
a uniform measure of SLE-specific QOL across cultures. Moreover, SLE is
prevalent globally in different ethnicities and races. In this study, we
propose to adapt SMILEYÓ to other cultures nationally and internationally.

The primary purpose is validation of the dutch version of a brief
health-related quality of life (HRQOL) tool in children with systemic lupus
erythematosus (SLE). This tool is called *Simple Measure of Impact of Lupus
Erythematosus in Youngsters© (SMILEY©).

Phase II. Cross-sectional arm (Determination of validity and reliability):
After obtaining permission from the attending physician, and obtaining consent
and/ or assent, the study package will be administered to the children and the
guardians. We will collect baseline information including historical,
demographic, medication and SLE-specific information. Corresponding versions
of the SMILEY©, and standard measures (Child Health Questionnaire- CHAQ) will
be administered to the subjects. The physician will complete the measures of
SLE disease activity (SLEDAI, BILAG, PGA ), damage (SDI) and assessment form.
The completion of the initial study package administration will take
approximately 30 minutes. Each subject will then be asked to critically review
the SMILEY© and comment on the relevance, completeness, and comprehensibility.
This will ensure that the adapted version is still retaining its equivalence in
an applied situation and will measure face validity.
Children and parents will be given another copy of the SMILEY© to complete in
10 days time and return by mail (a stamped, self-addressed envelope will be
provided).

Phase III. Prospective arm (Determination of responsiveness to change in
disease activity):
We will re-evaluate children and parents after the initial assessment at 3 (± 1
month leeway) month intervals after the initial visit. For child and
parent/legal guardian subjects, the completion of the subsequent evaluations
for responsiveness will include SMILEY© and an assessment form only and take
about 10 minutes. The physician will complete again the measures of disease
activity (SLEDAI, BILAG, PGA), damage (SDI), and medication use form.

It regards only questionnaires, which do not cause (severe) mental burden to
the participants. The completion of the questionnaires occurs during regular
patient out care visits. During these patient out care visits we collect
routinely blood and urine samples, as we do at every visit. We will not perform
other laboratory investigations besides the investigations that are done at
every patient out care visit.