Lymphangioleiomyomatosis
Conditions
Keywords
Lymphangioleiomyomatosis, Rehabilitation, Exercise
Brief summary
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.
Interventions
OTHERPulmonary Rehabilitation
Sponsors
University of Sao Paulo General Hospital
Study design
Allocation
NON_RANDOMIZED
Intervention model
PARALLEL
Primary purpose
SUPPORTIVE_CARE
Masking
NONE
Eligibility
Sex/Gender
FEMALE
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Endurance time during constant work rate cycle ergometry | 12 weeks |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| 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 | 12 weeks | The Modified Medical Research Council Dyspnea Scale (mMRC) and Baseline Dyspnea Index (BDI) will be evaluated pre - and post - intervention |
| Changes in peripheral muscle force | 12 weeks | Using one repetition maximum (1 RM) pre - and post - intervention |
| Anxiety and depression | 12 weeks | Hospital Anxiety and Depression Scale (HADS) performed pre - and post - intervention |
| Daily physical activity | 12 weeks | Evaluated using a pedometer for one week pre - and post - intervention |
| Changes in six minute walking distance and in desaturation - distance ratio (DDR) | 12 weeks | — |
| Changes in pulmonary function parameters | 12 weeks | — |
| Health factors related to quality of life | 12 weeks | St. George's Respiratory Questionnaire (SGRQ) pre - and post - intervention |
Countries
Brazil
Outcome results
None listed