Patients with spinal muscular atrophy who are wheelchair users often experience lower back - and gluteal pain, reduced sleep quality, constipation and reduced quality of life - symptoms that regular exercise could potentially alleviate. However, only very little research has been done on exercise for patients who are wheelchair users. The aim of this study is to explore the impact of cycle exercise on patients with spinal muscular atrophy.
Spinal muscular atrophy (SMA) is a neuromuscular disease affecting the motor neurons of the spinal cord that innervate the skeletal muscles, leading to progressive muscle atrophy and weakness. Consequently, many patients end up having to use a wheelchair from an early age. Besides the impaired motor function, patients with SMA can also experience a variety of symptoms associated with their disease, a sedentary lifestyle and physical inactivity such as lower back and leg pain, obstipation, reduced sleep quality and impacted quality of life. Training can help alleviate these symptoms in patients with neuromuscular diseases. However, there isn´t any validated way for patients with SMA to train as of today. Research has shown that high-intensity interval training (HIIT) has been well tolerated among patients with SBMA, a disease resembling SMA with patients tolerating this form for training well. The aim of this study is to investigate:(1) if HIIT could be exercise modality in SMA patients (2) and to see what positive effects exercise has for their impaired motor function and related challenges. The investigators will test the participants at baseline, which will be followed by a 8-week control period. After the control period the participants will be tested again before the intervention period. During the intervention period the participants will have to train 5 times a week for 10 minutes. After the intervention period, there will be a final test day, where the final results will be compared with those from the baseline.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
20
High intensity interval training on a bike or pedaltrainer from the wheelchair.
Rigshospitalet
Copenhagen, Denmark
RECRUITINGQuestionnaire on fatigue
The Multidimensional Fatigue Inventory (MFI-20) will be used. It measures fatigue in 5 different domains comprised of 4 items each with values ranging from 1 to 5. A higher score indicates more fatigue. Each domain is scored individually, ranging from 4 points (best outcome) to 20 points (worst outcome).
Time frame: 5 minutes
Questionnaire on pain.
A visual pain score for leg and lower back pain with 3 questions with scores ranging from 1 to 10 each, 10 being the worst pain imaginable, will be used.
Time frame: 5 minutes
Questionnaire on constipation
A questionnaire made from the Danish definition of constipation will be used. Four questions will be answered "yes" or "no".
Time frame: 5 minutes
Questionnaire on quality of life
A QoL that has been used in former studies will be used. 16 questions with scores ranging from 1 (very unsatisfied) to 7 (very satisfied) will be scored.
Time frame: 10 minutes
Questionnaire on sleep quality
Minimum Score = 0 (better); Maximum Score = 21 (worse) Interpretation: TOTAL \< 5 associated with good sleep quality TOTAL \> 5 associated with poor sleep quality Minimum Score = 0 (better); Maximum Score = 21 (worse) Interpretation: TOTAL \< 5 associated with good sleep quality TOTAL \> 5 associated with poor sleep quality Minimum score is 0, maximum score is 21. Total over 5 is associated with good sleep quality and under 5 associated with poor sleep quality.
Time frame: 10 minutes
Blood sample
Change in blood sample for blood lipids, mmol/l
This platform is for informational purposes only and does not constitute medical advice. Always consult a qualified healthcare professional.
Time frame: 2 minutes
Blood sample
Change in blood sample for HbA1C, mmol/mol
Time frame: 2 minutes
Blood sample
Change in blood sample for Creatine kinase, U/I. This will be monitored before, in the middle and after the exercise period as a safety measure.
Time frame: 2 minutes
Blood sample
Change in blood sample for fasting blood glucose, in mmol/L
Time frame: 2 minutes
Blood sample
Change in blood sample for fasting insulin, in pmol/L.
Time frame: 2 minutes
Blood sample
Change in blood sample for fasting c-peptide, in pmol/L.
Time frame: 2 minutes
Blood sample
Change in blood sample for ALT (liver parameter) in U/L
Time frame: 2 minutes
Blood sample
Change in blood sample for AST (liver parameter) in U/L
Time frame: 2 minutes
Blood sample
Change in blood sample for alkaline phosphatase (liver parameter) in U/L
Time frame: 2 minutes
Blood sample
Change in blood sample for bilirubin (liver parameter) in umol/L
Time frame: 2 minutes
MRI scan liver size
Assessment of liver size
Time frame: 5 minutes
MR-elastography
Assessment of liver fibrosis (assessed by MR-elastography)
Time frame: 5 minutes
MRI scan liver steatosis
Assessment of liver steatosis
Time frame: 5 minutes
Ultrasound scan
Additional assessment of liver size, fibrosis and steatosis
Time frame: 15 minutes
MRI scan
Assessment of full body muscle size
Time frame: 15 minutes
MRI scan
Assessment of muscle degeneration and subsequent fat replacement (using Dixon method to visualise fat in an optimal way)
Time frame: 15 minutes
Exercise test
Change in time to cycle X km in minutes
Time frame: 7 minutes
Motorscore
Change in motor function score, using the Motor Function Measurement (MFM-32). Assesses motor function in 3 domains, 32 two items in total. A higher score in each domain indicates better function.
Time frame: 20 minutes