Clinical Trial: Pulmonary Rehabilitation in Lymphangioleiomyomatosis

Study Status: Completed
Recruit Status: Completed
Study Type: Interventional

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

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.


Detailed Summary:
Sponsor: University of Sao Paulo General Hospital

Current Primary Outcome: Endurance time during constant work rate cycle ergometry [ Time Frame: 12 weeks ]

Original Primary Outcome: Same as current

Current Secondary Outcome:

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


Original Secondary Outcome: Same as current

Information By: University of Sao Paulo General Hospital

Dates:
Date Received: December 4, 2013
Date Started: November 2013
Date Completion:
Last Updated: December 9, 2015
Last Verified: December 2015