Pulmonary Rehabilitation in Lymphangioleiomyomatosis

Study Overview
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Brief Summary
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Lymphangioleiomyomatosis (LAM) is a rare disease in which reduced exercise capacity is frequently present. The mechanisms applied are airflow obstruction, abnormal diffusion capacity and dynamic hyperinflation (DH). Pulmonary rehabilitation (PR) has proved benefit in improving exercise tolerance, dyspnea, and quality of life in chronic obstructive pulmonary disease. There are no studies evaluating the impact of PR in patients with LAM. The hypothesis under study is that PR determine improvement in exercise capacity, dyspnea, quality of life, muscle force, functional limitation and DH in these patients.

Official Title
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Evaluation of the Impact of a Pulmonary Rehabilitation Program on Exercise Capacity in Patients With Lymphangioleiomyomatosis

Conditions
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Lymphangioleiomyomatosis

Intervention / Treatment
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* Other: Pulmonary Rehabilitation


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Diagnosis of lymphangioleiomyomatosis established by tissue biopsy and / or a combination of history and high-resolution computed tomography scanning Clinical stability, defined as no exacerbations or hospitalizations related to the underlying disease for a minimum of 6 weeks and no change in the treatment regimen in the last 3 months Low level of physical activity, defined as patients who walk less than 10 thousand daily steps and / or are not performing regular physical activity for at least 4 months before the start of rehabilitation Signature of the free, prior and informed consent for participation in the study Exclusion Criteria: Lung transplant recipients Musculoskeletal disorders that would prevent the patient from performing exercise training Severe (NYHA IV) or uncontrolled heart disease
Sexes Eligible for Study
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Female

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Supportive Care
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Active Comparator: Pulmonary Rehabilitation<br>The intervention group will perform a 12 week pulmonary rehabilitation program consisting of 30 minutes of treadmill aerobic exercise training and 30 minutes of muscle strength training. | Other: Pulmonary Rehabilitation<br> <br> |
| No Intervention: Control<br> | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Endurance time during constant work rate cycle ergometry |  | 12 weeks | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Peak oxygen consumption (VO2 peak) during constant work rate cycle ergometry |  | 12 weeks | 
| Dynamic hyperinflation and ventilatory parameters during constant work rate cycle ergometry |  | 12 weeks | 
| Dyspnea and fatigue in the lower limbs during constant work rate cycle ergometry |  | 12 weeks | 
| Dyspnea and functional disability related to daily life activities | The Modified Medical Research Council Dyspnea Scale (mMRC) and Baseline Dyspnea Index (BDI) will be evaluated pre - and post - intervention | 12 weeks | 
| Health factors related to quality of life | St. George's Respiratory Questionnaire (SGRQ) pre - and post - intervention | 12 weeks | 
| Anxiety and depression | Hospital Anxiety and Depression Scale (HADS) performed pre - and post - intervention | 12 weeks | 
| Daily physical activity | Evaluated using a pedometer for one week pre - and post - intervention | 12 weeks | 
| Changes in six minute walking distance and in desaturation - distance ratio (DDR) |  | 12 weeks | 
| Changes in pulmonary function parameters |  | 12 weeks | 
| Changes in peripheral muscle force | Using one repetition maximum (1 RM) pre - and post - intervention | 12 weeks | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Lymphangioleiomyomatosis, Rehabilitation, Exercise