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This is a prospective observational study to follow a cohort of patients with Cystic Fibrosis and healthy controls for a period of two years. This study will include monitoring the subjects lung clearance index (by performing a breathing test called the multiple breath washout), as well as spirometry and their respiratory symptoms every three months as well as during a pulmonary exacerbation and after their recovery.
This is a prospective observational cohort study. During the 3 year study period, each participant will be followed for a period of 2 years. The study will include quarterly measurements of the lung clearance index (LCI), as well as spirometry, for CF patients at their routine clinic visits. Parents of patients with CF will be asked to call the study nurse or clinical nurse if they experience a worsening of pulmonary symptoms and to come to the clinic for assessment of lung function. CF patients will then be assessed by a CF physician to assess whether they require antibiotic treatment based on a clinicians decisions to treat with antibiotics. The treatment decision will be left to the discretion of the patient's responsible physician, who will be blinded to the MBW results. All patients who meet the symptom based definition of a pulmonary exacerbation, regardless of treatment decision, will have MBW measured after 4 weeks. Following these visits at the time of an exacerbation, patients will have their MBW measured at their next clinic visit (usually within 3 months), and every 3 months thereafter until the end of the 2 year observation period, or the repeat onset of symptoms.This study will capture a maximum of two exacerbations per patient over the 2 year study period.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Healthy Controls | No intervention. Only monitoring LCI |
| |
| Cystic Fibrosis Patients | No intervention. Only monitoring LCI |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| This is an observational study, no intervention used. | Other | No intervention is being used in this study. |
|
| Measure | Description | Time Frame |
|---|---|---|
| Lung Clearance Index | Outcome measure from the Multiple Breath Washout test. | an average of 2 years |
| Measure | Description | Time Frame |
|---|---|---|
| Spirometry | FEV1 | an average of 2 years |
| Respiratory Cultures | Bacterial pathogens IL-8 and neutrophil elastase | an average of 2 years |
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Inclusion Criteria for Healthy Controls:
Inclusion Criteria for CF Patients:
Exclusion Criteria:
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There were 139 subjects recruited in the original preschool longitudinal study, and all participants from this original study will be invited to participate in this follow up study. We would like to recruit an additional 40 CF patients at the Hospital for Sick Children to increase our cohort size.
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| Name | Affiliation | Role |
|---|---|---|
| Felix Ratjen, MD | The Hospital for Sick Children | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hospital for Sick Children | Toronto | Ontario | M5G1X8 | Canada |
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| Respiratory Symptoms | CFQ-R | an average of 2 years |
| Respiratory Symptoms | CFRSD-CRISS | an average of 2 years |