An Intensive Anti-Scoliosis Postural Intervention Supported by an Application for Individuals With Rett Syndrome
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 20
- 试验地点
- 2
- 主要终点
- Scoliosis progression
研究概览
简要总结
Background: Scoliosis is the most common orthopedic comorbidity in Rett syndrome (RTT), with a prevalence of 94% and a mean curve progression of 14-21° Cobb annually. A scoliosis prevention intervention based on daily activity programs was proposed for people with RTT within uncontrolled study designs.
Aim: The current study aims to evaluate the effectiveness of a home-based activity program carried out during daily life to slow the progression of scoliosis in girls with RTT.
Ethics: The proposal was approved by Ariel university IRB. All participants' parents will sign informed consent forms.
Participants: Twenty Italian girls aged between six and 16 years with a genetically confirmed classic RTT and scoliosis at a severity level between 10° and 40° Cobb will be recruited and randomly divided into two groups (immediate intervention - Group 1; wait-list-intervention - Group 2). Both groups will follow the same 10-month intervention program, 10 months apart.
Outcome measures: Participants' scoliosis Cobb's angle, motor functioning, and behavioral characteristics will be assessed three times.
Procedure: Each participant will be evaluated three times: at T1, T2, and T3. Participants in the Group 1 will conduct the intervention for 10 months between T0 and T1. Group 2 will perform the intervention between T1 and T2. The interventions will comprise daily home-based activity programs carried out by participants' caregivers within everyday living environments. An expert therapist will remotely supervise each program through an ad hoc developed smartphone application. Specific strategies that will be implemented during the intervention will include the maintenance of asymmetrical postures that oppose the scoliosis curve during activities and exercises in sitting, standing, and walking positions (according to each participant's functional abilities). These strategies refer to a hypercorrective postural positioning of scoliosis. In addition, activities involving weight bearing on the lower limbs, such as walking and standing for at least two hours a day, will be encouraged, and passive stretching and spinal mobilization exercises will be conducted.
详细描述
Study Aims The current study aims to evaluate the effectiveness of an anti-scoliotic physiotherapy program carried out during daily life and focuses on hypercorrective postures and increasing the level of activity to assess whether or not it is possible to slow the progression of scoliosis in girls with Rett syndrome (RTT).
The current research project has the following research objectives:
- Primary objectives:
- Build, implement and evaluate a physiotherapy program for contrasting scoliosis trajectory adapted to individuals with RTT;
- Develop a secondary deformity prevention program through early intervention for people with RTT.
- Secondary objectives:
- Build and evaluate the efficiency of a support application for carrying out rehabilitation exercises;
- Explore the factors influencing adherence and implementation of intervention from the perspective of both families and therapists in light abovementioned applicationd above.
研究设计
- 研究类型
- Interventional
- 分配方式
- Randomized
- 干预模型
- Crossover
- 主要目的
- Treatment
- 盲法
- Single (Outcomes Assessor)
盲法说明
The orthopedic surgeons who will evaluate the participants' Cobb's angle will be blinded concerning the group to which each participant was assigned.
入排标准
- 年龄范围
- 6 Years 至 16 Years（Child）
- 性别
- Female
- 接受健康志愿者
- 否
入选标准
- •Diagnosis of RTT with confirmed MECP2 gene mutation;
- •Diagnosis of unstructured flexible scoliosis (Cobb angle between 10° and 40°) measured radiologically with an x-ray performed no more than six months before the recruitment;
- •Age between 6 and 16 years.
排除标准
- •Presence of psychomotor developmental deficit evidenced in the first six months of life or diseases of neurometabolic, infectious, or secondary brain damage trauma origin;
- •Previous surgical intervention to the spine or its planning within the study period;
- •Use of a corset during most waking hours;
- •Clinical judgment of the evaluating specialist doctor who certifies unstable health conditions that are not compatible with the performance of the rehabilitation program (e.g., ongoing or recurrent infections, severe gastrointestinal disorders, drug-resistant epilepsy with multi-day seizures).
- •Clinical judgment of the research team suggesting an inability of the home environment to perform the intervention program (change in residence, a planned pregnancy, a pre-planned complex surgical/medical procedure within the period of the planned intervention program for the child or family member) within the intervention period.
结局指标
主要结局
Scoliosis progression
时间窗: Three days 10 months apart from each other, before the start of the Group 1 intervention (T1), before the start of the Group 2 intervention (T2), and at the end of the Group 2 intervention (T3).
The progression of scoliosis will be assessed for each participant by measuring the differences between Cobb's angle measured on three anteroposterior X-rays of the spine collected before and after the intervention of each group. The Cobb's angle will be obtained from the average value of three measurements carried out by independent blinded (to the treatment status of each subject) medical specialists with clinical experience concerning scoliosis in Rett syndrome.
Adherence to the Program
时间窗: 10 months during the Group 2 intervention phase.
Adherence to the Program refers to the amount of treatment conducted by the participants compared to the total amount of treatment established in their program. It will be measured by the ad hoc developed smartphone application which will record the percentage of the performance of each activity foreseen in the programs.
次要结局
- Gross Motor Function Level(30 minutes, at the end of the Group 2 intervention.)
- Caregivers' Burden(15 minutes, at the end of the Group 2 intervention.)
- Behavioral Characteristics(15 minutes, at the end of the Group 2 intervention.)