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Prednisone in Cystic Fibrosis Pulmonary Exacerbations

Phase 3
Completed
Conditions
Cystic Fibrosis Pulmonary Exacerbation
Interventions
Drug: Placebos
Registration Number
NCT03070522
Lead Sponsor
The Hospital for Sick Children
Brief Summary

This will be a 5 year randomized, double blind, placebo controlled trial of 7 days of oral prednisone in cystic fibrosis (CF) patients receiving intravenous (IV) antibiotic treatment for a pulmonary exacerbation at the Hospital for Sick Children and other study sub-sites across Canada. The intervention will be oral prednisone 2 mg/kg/day (max 60 mg) divided twice daily for 7 days as an adjunctive therapy for pulmonary exacerbations in CF patients who have not recovered their baseline forced expiratory volume in 1 second (FEV1) after 7 days of IV antibiotic treatment. The primary outcome will be the proportion of subjects who achieve \>90% of their baseline FEV1 % predicted at day 14 of IV antibiotic treatment for a pulmonary exacerbation in each treatment arm.

Detailed Description

Not available

Recruitment & Eligibility

Status
COMPLETED
Sex
All
Target Recruitment
76
Inclusion Criteria
  1. Diagnosis of CF by newborn screening or at least one clinical feature of CF, AND either (a) or (b) as follows:
  2. A documented sweat chloride ≥ 60 mEq/L by quantitative pilocarpine iontophoresis
  3. A genotype with two identifiable CF-causing mutations
  4. Age > 6 years old.
  5. Acute pulmonary exacerbation treated with IV antibiotics as previously defined 10% relative drop in FEV1 from baseline at the time of exacerbation
  6. Informed consent by patient or parent/legal guardian
  7. Ability to reproducibly perform pulmonary function testing
  8. Ability to comply with medication use including the ability to take capsules, study visits and study procedures as judged by the site investigator
Exclusion Criteria
  1. A respiratory tract culture positive for Burkholderia cenocepacia in the 12 months prior to enrollment
  2. A respiratory tract culture positive for Mycobacterium abscessus in the 12 months prior to enrollment
  3. Treatment with IV or oral corticosteroids within 2 weeks of enrollment or from Day 0-Day 7 of the pulmonary exacerbation
  4. Active allergic bronchopulmonary aspergillosis (ABPA) at the time of enrollment as determined by treating physician
  5. Asthma related exacerbation at enrollment as defined by the treating physician based on clinically compatible symptoms (eg. wheeze)
  6. History of avascular necrosis or pathologic bone fracture
  7. Uncontrolled hypertension with end organ damage
  8. Active gastrointestinal bleeding
  9. Status post lung or other organ transplantation
  10. Pregnancy
  11. Lactose intolerance (contained in placebo)
  12. On Lumacaftor-Ivacaftor (Orkambi) at the time of exacerbation
  13. Investigational drug use within 30 days prior to enrollment visit
  14. Physical findings that would compromise the safety of the subject or the quality of the study data as determined by site investigator

Study & Design

Study Type
INTERVENTIONAL
Study Design
PARALLEL
Arm && Interventions
GroupInterventionDescription
PlaceboPlacebosPlacebo
TreatmentPrednisonePrednisone
Primary Outcome Measures
NameTimeMethod
Lung function recoveryAt 14 days of antibiotic therapy

The proportion of subjects who achieve \>90% of their baseline FEV1 % predicted at day 14 of IV antibiotic treatment for a PEx in each treatment arm.

Secondary Outcome Measures
NameTimeMethod
lung function recovery at follow up visit1 month follow up

The proportion of subjects who achieve \>90% of their baseline FEV1 % predicted

change in pulmonary function testingat day 7, 14 and 1 month follow up

change in pulmonary function testing

quality of life as measured by CFQ-R questionnaireat day 7, 14 and 1 month follow up

quality of life

quality of life as measured by CF Respiratory Symptom Diaryat day 7, 14 and 1 month follow up

quality of life

change in sputum inflammatory markersat day 7, 14 and 1 month follow up

change in sputum inflammatory markers

time to subsequent pulmonary exacerbation1 year follow up time

time to subsequent pulmonary exacerbation

change in serum inflammatory markersat day 7, 14 and 1 month follow up

change in serum inflammatory markers

number of adverse eventsAt day day 14 of antibiotic therapy and 1 month follow up

number of adverse events

length of hospitalizationThrough study completion, up to 100 weeks

length of hospitalization

Duration of antibiotic treatmentThrough study completion, up to 100 weeks

Duration of antibiotic treatment

Trial Locations

Locations (12)

The Governers of The University of Calgary - Alberta Health Services

🇨🇦

Calgary, Alberta, Canada

British Columbia Children's Hospital

🇨🇦

Vancouver, British Columbia, Canada

St. Paul's Hospital

🇨🇦

Vancouver, British Columbia, Canada

London Health Sciences Centre - Lawson Health Research Institute

🇨🇦

London, Ontario, Canada

The Ottawa Hospital

🇨🇦

Ottawa, Ontario, Canada

Unity Health Toronto - St. Michael's Hospital

🇨🇦

Toronto, Ontario, Canada

SickKids

🇨🇦

Toronto, Ontario, Canada

The Centre hospitalier de l'Université de Montréal (CHUM)

🇨🇦

Montréal, Quebec, Canada

Centre hospitalier universitaire Sainte-Justine

🇨🇦

Montréal, Quebec, Canada

CHU de Quebec-Universite Laval

🇨🇦

Quebec City, Quebec, Canada

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The Governers of The University of Calgary - Alberta Health Services
🇨🇦Calgary, Alberta, Canada
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