Effects of Exercise in Children with a chronic illness: Inflammatory Bowel Disease

Inflammatory bowel disease (IBD) is one of the most common auto-immune
diseases, including the ulcerative colitis and Crohn's disease. In the
Netherlands 2,500 to 3,000 children live with IBD and around 250 children are
diagnosed each year. Children with IBD have lower levels of physical activity
and are also limited in their endurance compared to healthy children.

Pompe's disease is a rare metabolic disease that affects 1 in 40,000 people.
Children with Pompe's disease have progressive muscle weakness, causing
problems with walking, climbing, playing and eventually they develop
respiratory problems. Whether children with Pompe disease are less active in
daily life compared to healthy children has not been investigated yet, we do
know that children with Pompe's disease are severly limited in their endurance.

Children with a Fontan circulation are born with a heart defect in which only
one heart chamber functions. This occurs in about 3 in 10,000 births. Children
with a Fontan circulation have a reduced quality of life, tire more quickly and
often develop cardiac arrhythmias and heart failure later in life. It is also
known that these children exercise less and have a reduced exercise capacity.

Children with BPD are (premature) born with underdeveloped lungs, leading to
permanent lung damage. The lungs of children with BPD stay very vulnerable.
Children with BPD therefore often suffer from respiratory infections and
continue to have reduced lung function compared to healthy children. Levels of
physical activity have not been investigated yet in this patient group, we do
know that children with BPD have a reduced exercise capacity

The study focuses on children and young people with IBD from 6-18 years old.
These children have lower levels of physical activity and a limited endurance
compared to healthy children. Through this research we want to improve the
endurance of children with IBD, so that these children may also become more
active in daily life. To achieve this, it will first have to be investigated
why children with IBD are less active and have limited endurance. For this we
research the daily (exercise) activities and quality of life and we measure
body composition, disease activity, endurance, muscle strength and energy
balance. On the basis of this data, we make a customized training intervention,
possibly supplemented with dietary advice and psychological guidance. The
primary aim of our study is to measure the effect of this training intervention
on the exercise capacity
of children with IBD. We also check whether the training program can be
followed safely and has no negative influence on the activity of the disease
and we measure the effect of the training program on physical functioning in
daily life, muscle strength, fear of exercise, body composition and resting
metabolism.

Children and young people with Pompe*s disease have lower levels of physical
activity and a limited endurance compared to healthy children. This research
focuses on Children and young people with Pompe*s disease from 6-18 years old.
Through this research we want to improve the endurance of children with
Pompe*s disease, so that these children may also become more active in daily
life. To achieve this, it will first have to be investigated why children with
Pompe*s disease are less active and have limited endurance. For this we
research the daily (exercise) activities and quality of life and we measure
body composition, disease activity, endurance, muscle strength and energy
balance. On the basis of this data, we make a customized training intervention,
possibly supplemented with dietary advice and psychological guidance. The
primary aim of our study is to measure the effect of this training intervention
on the exercise capacity of children with Pompe*s disease. We also check
whether the training program can be followed safely and has no negative
influence on the activity of the disease and we measure the effect of the
training program on physical functioning in daily life, muscle strength, fear
of exercise, body composition and resting metabolism.

Children with a Fontan circulation have lower levels of physical activity move
less in daily life and and a limited endurance compared to healthy children.
This research focuses on Children and young people with a Fontan circulation of
6-18 years. Through this research, we want to improve the exercise capacity of
children with a Fontan circulation, so that these children may also become more
active in daily life. To achieve this, it will first be necessary to
investigate why children with a Fontan circulation are less active and have a
lower exercise capacity. For this we research the daily (exercise) activities
and quality of life and we measure body composition, disease activity,
endurance, muscle strength and energy balance. On the basis of this data, we
make a customized training intervention, possibly supplemented with dietary
advice and psychological guidance. The primary aim of our study is to measure
the effect of this training intervention on the exercise capacity of children
with a Fontan circulation. We also check whether the training program can be
followed safely and has no negative influence on the activity of the disease
and we measure the effect of the training program on physical functioning in
daily life, muscle strength, fear of exercise, body composition and resting
metabolism.

Children with BPD have limited endurance compared to healthy children. This
research focuses on Children and young people with BPD of 6-18 years. Through
this research, we want to improve the exercise capacity of children with BPD,
so that these children may also become more active in daily life. To achieve
this, it will first be necessary to investigate if children with BPD are less
active in daily life. For this we research the daily (exercise) activities and
quality of life and we measure body composition, disease activity, endurance,
muscle strength and energy balance. On the basis of this data, we make a
customized training intervention, possibly supplemented with dietary advice and
psychological guidance. The primary aim of our study is to measure the effect
of this training intervention on the exercise capacity of children with BPD. We
also check whether the training program can be followed safely and has no
negative influence on the activity of the disease and we measure the effect of
the training program on physical functioning in daily life, muscle strength,
fear of exercise, body composition and resting metabolism.

The study is a cross-over intervention study. Each patient group will be
randomized in to two groups. At start, each group will have two assessment
days. Following these assessments a tailor-made training programs will be made,
taking in to account on which of the domains (e.g. 1) endurance, 2) muscle
strength, 3) core stability, 4) inspiratory muscle strength, 5) psychosocial
functioning and/or fear for children and/or their parents and 6) caloric
intake) score less compared to healthy peers or desire advise. One group will
start with 12 weeks of training until assessment 2, and will have a resting
period between assessment two and three. The other group will start with the
resting period and will train between assessment two and three.

During 12 weeks the children will follow a tailor made training program. These
trainings will take place 2-3 times a week for an hour and will supervised by a
local physiotherapist.

The cross-over design was chosen to make sure all children will be able to
participate in the training program and because less patients will be needed
while every child is its own control.


Group 1:
M1 12 weeks training M2 12 weeks *rest* M3
l--l---------------------------------------------l--l---------------------------
-------------------|

Group 2:
M1 12 weeks *rest* M2 12 weeks training M3
l--l---------------------------------------------l--l---------------------------
---------------------l--l

At M1 the volume of physical activity and the possible cause of a decreased
physical functioning will be examined. Depending on the results training
programs will be made. Focus will be on a decreased outcome and the needs of
the patients for: 1) endurance, 2) muscle strength, 3) core stability, 4)
inspiratory muscle strength, 5) psychosocial functioning and 6) caloric intake.
Depending on the results of M1 and earlier research in adult patients training
programs:
We expect to focus on endurance training possibly with strength training
When needed dietary advise and psychosocial training will complete the training
programs.

Earlier research in children and adults with different chronic diseases have
shown that supervised exercise training has beneficial effects on health.
Previous study showed that adults with IBD had increased exercise capacity and
quality of life scores after training. Patients probably may benefit from our
study. We expect that the quality of life and physical activity will increase.

Previous studies in adults with Pompe disease have shown that supervised
training led to an increased quality of life and better endurance. This has not
yet been investigated in children. Patients are likely to benefit from
participating in this study. We expect that both the quality of life and the
physical functioning of the children will increase.

Previous studies in children with lung diseases have shown that supervised
training led to an increased quality of life and better endurance. This has not
yet been investigated in children with BPD. Patients are likely to benefit from
participating in this study. We expect that both the quality of life and the
physical functioning of the children will increase.


Assessments are divided over 3 moments, each moment consisting out of 2
visitis, 4 hours per visit. In between each visit the patients have to answer
questionnaires (n=4, total time 45 minutes taken into account at the assessment
time). Furthermore the patient have to wear an accelerometer for three days,
which isn't uncomfortable. They have to keep a diary on activity for these
three days (30 minutes in total, taken into account in the assessment days)

Risks of the assessments:
- blood sample: vein punction might be painfull, cause hematomas and bleedings
- bicycle ergometer test: can reveal hart rhythm disorders. Heart rhythm and
blood pressure will be monitored by a trained supervisor. The test will be
stopped in case of a heart rhythm disorder or when the supervisor thinks it
isn't safe to continue. Patients can get fatigued or have muscle soreness after
the test.
- 6 minute walk test: can cause fatigue
- indirect calorimetry and BODPOD and MRI wil take place in a small device.
Claustrofobic children might feel uncomfortable.

We don't expect any complications due to the exercise training based on
existing literature. Patients with contra-indications for sport activities will
be excluded. It is always possible to get injured during physical activity but
the risks in this study are reduced due to the supervision of the
physiotherapist. Patients might get fatigued or muscle soreness due to the
exercise training. The training program takes time from children and parents.