1) Improving the (physical) daily functioning of children with HCTD2) Testing feasibility of intervention for child / parents and therapists
ID
Source
Brief title
Condition
- Musculoskeletal and connective tissue disorders congenital
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
The Goal Attainment Scaling (GAS) determines the extent to which a treatment
goal has been achieved. A goal for the treatment is set in accordance with
parents and child. The main goal is included in the analysis. On the basis of
that goal, a 6-point GAS scale is established on which the extent to which this
goal has been achieved can be evaluated. GAS is a generic, evaluative tool that
can describe change per person or in groups with good validity and reliability
The endurance is determined with the Fitkids Treadmill Test. This is a maximum
exercise test. During the test, heart rate is measured with a Polar heart rate
monitor. The degree of fatigue is measured with the OMNI scale. The degree of
pain, whether present or not, is measured with a Visual Analogue Scale (VAS).
The Fitkids Treadmill test has been developed for children with chronic
conditions, is very applicable and has good validity and reliability.
Secondary outcome
Motor functioning and physical capacity
The Bruininsk-Oseretsky Test of Motor Proficiency-2 (BOTMP-2) is used to
measure motor functioning. The test consists of 8 subtests; 4 for gross motor
skills (bilateral coordination, balance, sprint speed and strength and
dexterity) and 4 for fine motor skills. The test has good validity and
reliability.
The Muscle Power Sprint Test (MPST) determines the anaerobic endurance, shown
as Peak and Mean Power. The MPST is a sprint test where 6 x 15 meters is
sprinted as fast as the child can. There is a 10-second rest between sprints.
The time of every 15 meter sprint is registered to 0.1 second with a stopwatch.
The power (power) is then calculated with the time, body weight and distance
traveled. The test has good validity and reliability.
Agility during intensive movement is measured with the 10 x 5 meter sprint,
shown in seconds. The 10 x 5 meter sprint is completed as fast as the child
can. The test has good validity and reliability.
The Childhood Health Assessment Questionnaire (CHAQ-38) is used to measure
limitations at the activity level. The CHAQ-38 was developed and validated by
Lam et al (2004) and consists of 38 items divided into 9 subscales with daily
skills (such as dressing, walking, school activities). This assessment
evaluates 3 components: (a) difficulty to perform activity on a 4 point scale,
(b) use of special tools, and (c) assistance needed to perform the activity.
The CHAQ is a valid, reliable and sensitive measuring instrument to measure
functional disability and has been validated and translated for use in the
Netherlands.
fatigue
The degree of fatigue experienced is measured using the Promis questionnaire.
The Promis short form Fatique V2.0 is for children aged 8 and older. The Parent
proxy report (V2.0-Parent proxy) is taken from the parents / guardians of
children 6 and 7 years old. Promis Fatique is a questionnaire based on the item
response theory to measure the degree of perceived fatigue in children. Promis
Fatique short form is a valid, reliable and sensitive measuring instrument for
children with chronic disability. The Dutch and Flemish translation of the
Promis Fatique short form items were tested and found to be reliable and takes
about 10 minutes.
Physical examination
Date of birth, diagnosis, height (cm), weight (kg) (Tanita MC780), body mass
index (BMI), will be collected for all participants. Joint mobility will be
assessed by the Beighton score, Upper limb assessment and the Lower limb
assessment..
Background summary
Connective tissue disorders (CTD) is a collective term for diseases or
disorders generally characterized by systemic problems affecting the
cardiovascular, muscular and pulmonary systems, hypermobility, extreme skin
flexibility and tissue fragility. Three known types of CTDs are Ehlers-Danlos
Syndromes (EDS), Marfan Syndrome (MFS), and Loeys-Dietz Syndrome (LDS). The
incidence is low, about 1-3 per 10,000 children are diagnosed with a CTD.
Health problems related to the disease can be various and have a profound
impact on physical, social and psychosocial functioning and health-related
quality of life. Joint hypermobility in children will often result in injuries
and pain. This may lead to reduced physical activity, increased absenteeism and
reduced physical and psychosocial functioning.
In a previous study, we examined the impact of the disease in a large group of
children with CTD using a survey and various physical tests. Based on these two
studies we designed an intervention study which is in line with the physical
limitations obtained and experienced by the children and individual needs for
tailored training.
Study objective
1) Improving the (physical) daily functioning of children with HCTD
2) Testing feasibility of intervention for child / parents and therapists
Study design
This is a (not controlled, not randomised) intervention study, feasibility
study.
- T0 Baseline measurement (AMC)
- Training
- 12 weeks 3 times a week training for 45 minutes at the local pediatric
physical therapist
- 3 information meetings at Reade (2 hours each)
- T1 measurement
Intervention
An intensive trainings period of 12 weeks, 3 times a week 45 minutes consist of
High intensity training or Power training and tailored to individual needs.
in addition, there are information meetings about how to deal with complaints
and limitations in daily living.
Study burden and risks
Intensity of the intervention is comparable to a Gym class at school, regular
pediatric physical therapy training or intensive playing outside. The
cardiologist's permission is required for participation in the study.
Meibergdreef 9
Amsterdam 1105 AZ
NL
Meibergdreef 9
Amsterdam 1105 AZ
NL
Listed location countries
Age
Inclusion criteria
Children and adolescents (6-18 years old) who are diagnosed with either Marfan
syndrome, Ehlers-Danlos Syndromes or Loeys-Dietz syndrome, who are treated in
the Amsterdam University Medical Center, location AMC or Reade Amsterdam. The
origin of the children/adolescents and their parents can be of every country
and/or ethnicity .
Exclusion criteria
Children/ adolescents who, next to the Marfan syndrome, Ehlers-Danlos Syndromes
or Loeys-Dietz syndrome, have another prominent chronic disease affecting their
physical functioning, or children who are seriously cognitive impaired or
completely wheelchair dependent.
In addition, for children with MFS, LD and EDS-vascular type permission from
the cardiologist is required for participation in this study
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
No registrations found.
In other registers
| Register | ID |
|---|---|
| CCMO | NL76844.018.21 |