Objective Neurocognitive assessment of young children with sickle cell disease by eye-tracking

Evidence indicates that Sickle Cell Disease threatens neurodevelopmental
outcome. Previous research suggests that neurocognitive impairment is already
present in toddlers. The future quality of life of children with SCD is highly
dependent on their neurocognitive outcome, that is threatened by the course of
their disease as well as the environment that they grow up in. It is important
to identify as early as possible those individuals who are at the highest risk
of neurocognitive impairment. This will enable early interventions to mitigate
the detrimental effect of SCD on the developing brain. In order to develop such
interventions, a deeper understanding of the underlying pathophysiological
mechanisms is required.

The main aim is to study early neurocognitive functioning and development in
very young children with SCD. The secondary aims are to identify determinants
(risk and protective factors) and biomarkers of early neurocognitive deficits
in SCD and to analyse the relationship between early neurocognitive functioning
and adaptive daily life functioning later in life (quality of life, behavioural
and emotional functioning, general development)

Prospective observational study with an accelerated longitudinal design

There is no additional risk associated with participation in this study. The
burden can be considered minimal. This study will assess the neurocognitive
development of very young children with sickle cell disease. Therefore, this
study can only be performed in this specific group of minors. The control group
of healthy typical developing children will be used as a reference group for
the SCD group and is necessary to elucidate the impact of SCD on the outcome
measures, as corrected for age, sex, and socio-economic status. The available
standardized population norms for some of the outcome measures do not include
the demographic characteristics that can have a considerable influence on the
neurocognitive development of a child [1]. As children with low socio-economic
status are over-represented in the SCD population, the confounding influence of
socio-economic status needs to be controlled, which cannot be done using the
age-standardized population norm. Therefore, this study will need to include a
demographically matched healthy, typically developing control group.
Blood drawings will be taken of children with sickle cell disease. As blood
drawings are part of the patients normal procedure, blood collection will be
combined with regular blood draws to reduce the burden.