Status:

COMPLETED

Orkambi Exercise Study

Lead Sponsor:

University of British Columbia

Conditions:

Lung; Disease

Cystic Fibrosis

Eligibility:

All Genders

19+ years

Brief Summary

Shortness of breath (dyspnea) during exercise is a major source of distress and is a commonly reported symptom in patients with cystic fibrosis (CF). A recent treatment option known as Orkambi, which ...

Detailed Description

Advances in therapies and patient care have led to dramatic improvements in cystic fibrosis (CF) survival. Consequently, CF patients are living longer with varying degrees of lung function impairment....

Eligibility Criteria

Inclusion

  • Confirmed diagnosis of CF and homozygous for the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene
  • Plan to initiate Orkambi™ by the treating physician within 30 days of the enrolment visit
  • Aged 19 years or older
  • Stable clinical status based on clinical judgment of the treating physicians (Drs Wilcox, Quon or Goodwin)
  • Forced Expiratory Volume in 1 second (FEV1.0) \< 90% predicted
  • Body mass index greater than 16 or less than 30 kg/m2
  • Currently non-smoking or a past smoking history of less than 20 pack-years
  • Able to read and understand English

Exclusion

  • A disease other than CF that could importantly contribute to dyspnea or exercise limitation
  • Chronic airway infection with Mycobacterium abscessus, Burkholderia cepacia complex, or other organisms with infection control implications based on the treating physicians
  • Contraindications to clinical exercise testing
  • Use of supplemental oxygen or desaturation less than 85% with exercise
  • Diagnosis of pneumothorax in the past 4 weeks
  • History of organ transplantation

Key Trial Info

Start Date :

July 1 2016

Trial Type :

OBSERVATIONAL

Allocation :

ACTUAL

End Date :

June 1 2020

Estimated Enrollment :

11 Patients enrolled

Trial Details

Trial ID

NCT02821130

Start Date

July 1 2016

End Date

June 1 2020

Last Update

November 17 2020

Active Locations (1)

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Page 1 of 1 (1 locations)

1

UBC James Hogg Research Centre, St. Paul's Hospital

Vancouver, British Columbia, Canada, V6Z1Y6