extreme flexibility Heritable Connective Tissue Disorder
Conditions
Interventions
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 meeting
Sponsors
Academisch Medisch Centrum
Eligibility
Age
2 Years to 17 Years
Inclusion criteria
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
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 outcomes
Primary
| Measure | Time frame |
|---|---|
| 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
| Measure | Time frame |
|---|---|
| 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 p | — |
Countries
Netherlands
Outcome results
None listed