Overview
Background: Due to fear of injury and risk of fractures, many people with osteogenesis imperfecta (OI) avoid vigorous exercise that is likely to improve muscle and bone weakness common in this disorder. Low-intensity muscle strength training with blood flow restriction (BFR-LI) via an inflatable cuff leads to similar improvements in muscle size and strength to conventional high-intensity strength training but has not been applied in OI.Objectives:To evaluate the effect of BFR-LI on muscle and bone health and quality of life in adults with OI type I.Methods: For this randomized controlled trial, 40 adults (18-65y) with OI type I will be recruited. Exclusion criteria include pregnancy or \<6 months postpartum, deep vein thrombosis, acute fractures, recent surgery, regular vigorous exercise, or contraindications to exercise. Participants will be randomly assigned to perform BFR-LI or standard care. The BFR-LI group will perform home-based exercises 2-3 times/week for 12 weeks at low training intensity (20% of 1-repetition maximum) which will increase every 4 weeks. Muscle and bone parameters will be evaluated using scans (peripheral computed tomography and dual-energy x-ray absorptiometry) and muscle strength tests, and quality of life (mental well-being, participation, pain and fatigue) by the PROMIS questionnaire at baseline, immediately, three, and six months after training.
Eligibility
Inclusion Criteria:
- Osteogenesis imperfecta type I, aged between 18 and 65 years old
Exclusion Criteria:
- Lower limbs:
Edema; Recent surgery; Acute fractures
- Pregnancy/\<6 months postpartum
- Deep vein thrombosis (now or history of)
- Cardiovascular, respiratory, or neuromuscular diseases
- Recent cardiovascular events/untreated hypertension
- Diseases affecting vascular function (e.g., diabetes)
- Vigorous strength training in year before study
- Other contraindications to perform exercise
