Neuromuscular diseases NMD
Conditions
Interventions
Participants will be randomized (ratio 1:1) to the intervention group,
receiving a 6-month personalized physical activity program according to the I*M
FINE strategy, or a control group, receiving us
Sponsors
Academisch Medisch Centrum
Eligibility
Age
18 Years to 99 Years
Inclusion criteria
Inclusion criteria: (1) Prior paralytic poliomyelitis (confirmed by signs of residual weakness and atrophy of muscles on neuromuscular examination, and with EMG) or diagnosis of PPS (according to the March of Dimes criteria), CMT (confirmed by DNA testing or polyneuropathy compatible with CMT and positive family history), or other slowly progressive NMD (with no effective drug therapy). (2) Presence of a question for help indicative of impaired physical fitness or physical inactivity. (3) Minimum age of 18 years.
Exclusion criteria
Exclusion criteria: (1) Contraindication for being physically active (according to the guidelines by the American College of Sports Medicine). (2) Unable to follow verbal or written instructions. (3) Insufficient mastery of the Dutch language. (4) Engaged in an exercise program for a period longer than 4 weeks during the last 6 months.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| (1) Physical fitness The primary endpoint will be the change in peak oxygen uptake directly after intervention. | — |
Secondary
| Measure | Time frame |
|---|---|
| (2) Daily activity Measured through heart rate monitoring during 7 consecutive days, to establish the total time spent in low, moderate and vigorous intensity activities. Activity monitors will be used to determine the total step count during the 7 day period and subjects will also be asked to keep an activity diary. (3) Health-related quality of life Assessed using the Dutch version of the Short Form 36-item Health Survey (SF36). The physical health component scores and mental health component scores will be calculated, using age-correlated means and standard deviations of a healthy Dutch population. (4) Perceived physical functioning Administered with the ACTIVLIM questionnaire. ACTIVLIM is a questionnaire on self-reported activity limitations that was validated using the Rasch model. The questionnaire consists of 22 daily activities of which the perceived difficulty in performing the activity is scored. (5) Muscle strength The maximal voluntary torque (MVT) of the upper extremities elbow flexors and shoulder abductors), or lower extremities (hip flexors, knee extensors and plantar flexors) will be determined, depending on the selected exercise mode for training. Subjects will perform three maximal-effort contractions at an angular velocity of 60°/sec on a fixed dynamometer (Biodex System 4, New York, USA. The highest value of peak torque (Nm) will be used for the analyses. Creatine kinase will be determined from the blood sample. (6) Markers of metabolic syndrome Blood lipids and fasting glucose, assessed from blood samples and blood pressure. (7) Self-efficacy Self-efficacy will be assessed with the following question *How confident are you that you will be physically active in the following situations*, including feeling tired, bad mood, do not have the time, on vacation, and, want to be active outside, but bad weather, rated on a 5-point Likert scale (1=absolutely no confidence, 5=completely confident). (8) Physical capacity As | — |
Countries
Netherlands
Outcome results
None listed