Hypokalemic Periodic Paralysis, Hyperkalemic Periodic Paralysis
Conditions
Brief summary
This study wishes to investigate the effects of strength exercise in patients with either HypoPP or HyperPP. The investigators wishes to include participants already diagnosed with either HypoPP or HyperPP in af 24 week prospective study where the patients will be tested and asked to fill out questionnaires three times. These appointments will be schedueled at week 0, week 12 and week 24. In the time period between week 12 and week 24, the patients will have a personalized strength exercise program, which they will have to follow these 3 months. The exercise will be supervised by one or more of the investigators. We will also assess the muscle structure and function cross sectionally.
Interventions
OTHERStrength exercise
3 weekly supervised trainings with specific strengt exercises
Sponsors
Rigshospitalet, Denmark
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Patients with genetic verified HypoPP or HyperPP * Age minimum 18 years * Able to walk a minimum of 10 meters independently with or without assistive devices and be able to stand from a chair without using hands
Exclusion criteria
* Other significant cause of muscle weakness * Heart or lung disease which, in the investigator's opinion, makes participation in the study training inadvisable Exclusion from muscle biopsy * Anticoagulant treatment * Impaired blood clotting due to disease Exclusion from MRI scanning due to: * Metal implants in the body that contraindicate MRI scanning or are positioned such that the scan quality is significantly affected * Claustrophobia * Pregnancy
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Functional endurance test in the form of the Five Times Sit-to-Stand Test (5STS) | From intervention to end of intervention at 12 weeks |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Functional endurance test in the form of the 6-Minute Walk Test (6MWT) | 12 weeks | — |
| Muscle strength measured with a dynamometer | 12 weeks | Dynamometer used: Biodex |
| Number of attacks of muscle weakness | 12 weeks | Self-reported by participants |
| Myotonia graded using the Myotonia Behaviour Scale (MBS) | 12 weeks | Scale goes from 0-5, where 0 is no stiffness and 5 is incapacitating stiffness, which demands constant movement to not be totally locked up |
| Quality of life measured with the Individualized Neuromuscular Quality of Life Questionnaire (INQoL) | 12 weeks | To assess health-related quality of life of patient suffering from neuromuscular diseases. |
| Quality of life measured with the EuroQol 5 Dimensions 5 Levels (EQ-5D-5L) | 12 weeks | The EQ VAS records the patient's self-rated health on a vertical visual analogue scale where the endpoints are labelled 'The best health you can imagine' and 'The worst health you can imagine'. The VAS can be used as a quantitative measure of health outcome that reflects the patient's own judgement. |
| Fatigue measured with the Multidimensional Fatigue Inventory (MFI-20) | 12 weeks | A questionnaire consisting of 20 questions. The higher the collected score upon answering all the questions indicates a more severe fatigue. |
| Pain graded using the Visual Analog Scale (VAS) | 12 weeks | A pain scale from 0-10, where 0 is no pain and 10 is the worst pain possible |
| Fat fraction of leg muscles | 1 hour | Dixon MRI sequencing of leg muscles. Outcome: fat fraction. Is taken one time to describe the characteristics cross-sectionally. |
| Muscle biopsy | 1 hour | Muscle biopsies will be taken one time to describe the muscle phenotype cross-sectionally. We will look for structural changes, including vacoules. |
| Qualitative interview describing former and current experiences with exercise | 2 hours | After the exercise period, participants will be interviewed about their experiences with the exercise program and former exercise experiences, both personal and through the healthcare system. |
| Timed up and go (TUG) | 5 minutes | We measure the time it takes to raise from a chair, walk 3 meters, turn around, walk back, and sit down. We assess it one time to measure the characteristics cross-sectionally. |
| Sit to rise test (STR) | 5 minutes | We measure number of times the participants can raise and sit from a chair in 10 seconds. We measure it one time to assess the characteristics cross-sectionally. |
Countries
Denmark
Contacts
CONTACTAnnica B Enegaard
Outcome results
None listed