Responsiveness of the PRPP (Perceive-Recall-Plan-Perform System of Task Analysis and Intervention)
in children with mitochondrial disease

Engagement in everyday activities is seen as an important determinant for
health, well-being and perceived quality of
life. Children with mitochondrial disorder experience limitations in daily
activities. On the basis of previous research, we
know which activities children with mitochondrial disorder (want to) perform
and what they find important. The variation
in daily activities is large, which requires a personal approach tailored to
the child's level. ln order to make personal
care possible, it is necessary to implement a measuring instrument in daily
(care) practice that makes the quality of
performance of a child's personally relevant activities measurable.
There is one known instrument that has the potential to serve as a generic
instrument for this complex group: the
"Perceive, Recall, Plan and Perform (PRPP) System of task analysis". The PRPP
is a criterion-referenced observation
instrument with which the performance of activities is observed according to an
objective scoring system. Since 2018,
the PRPP has been part of the Mitoroute, in which parents provide videos of
their children carrying out activities. These
videos are analyzed by a PRPP{rained occupational therapist and discussed
during the occupational therapy
consultation and the multidisciplinary consultation within the Mitoroute. As a
result of the recently conducted design
study, the implementation of the PRPP within Mitoroute was optimized. The
manual for the use of the PRPP on the
Mitoroute is currently being finalized. This manual can also be used for other
target groups and care paths in which the
PRPP is used by means of video recordings (instead of real-life observations).
The psychometric properties of the PRPP have been studied in various target
groups, but not for the target group of
children with mitochondrial diseases. Preliminary results are promising
concerning reliability and validity. ln order for
the PRPP to (continue to) be part of the regular care process and for
application in clinical research, it is necessary
to know whether the PRPP is suitable for measuring the course of the disease.
The instrument should measure
differences related to the severity of the disease at the level of functioning
and be responsive to interventions.

Exploring the responsiveness of the PRPP-Assessment in children with
mitochondrial disorder

Multiple case series design with 5-8 children.
There will be a T0, waiting list period, intervention and T1. The intervention
consists of a 6-week home-based video-coaching of parents in which they are
coached how to guide their child so that the child learns to perform the
activity better.
Data will be collected with the PRPP-Assessment, the COPM and the GAS.
Descriptive statistics and correlations are used to compare change scores.

Home-based video coaching consisting of the PRPP-Intervention aimed at coaching
parents how to guide their children in learning the activity

It is a very minimal burden for children; only the pre- and post- interview is
'extra'; the activity is now filmed and practiced, but would otherwise be done.
For parents, the burden is slightly greater (they film the activities, have a
short conversation with the researcher, and are in weekly contact with an
occupational therapist for 6 weeks for coaching on how to support the child in
learning the chosen activities).
There are no risks.