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Idiopathic pulmonary fibrosis (IPF) is a rare chronic progressive disease of unknown etiology that affects physical and emotional well-being. It is characterized by irreversible loss of lung function due to fibrosis, which manifests itself with cough, dyspnea, and impaired quality of life. Lung transplantation is limited to a minority of patients and patients receive anti-fibrotic therapy in addition to supportive/palliative treatments. In light of the broad definition of pulmonary rehabilitation, and by virtue of the multidisciplinary nature of the process, the present study aims to observe the effects of the rehabilitation counseling offered to patients who attend the IPF clinic at the Pneumology Operating Unit of the IRCCS Company Bologna University Hospital, as part of the Intercompany PDTA (diagnostic therapeutic care path) for Pulmonary Fibrosis: the Intercompany PDTA for patients suffering from idiopathic pulmonary fibrosis involves a multidisciplinary approach which, also through therapeutic education/counselling, aims among others to "Offer the patient the opportunity to participate in clinical studies to increase knowledge about the disease and/or have access to innovative treatments". Primary objectives
Evaluate the effectiveness of the rehabilitation intervention contained in the PDTAI (Intercompany diagnostic therapeutic care path )
Evaluate the improvement in functional capacity and cardiorespiratory endurance.
Secondary objectives
Evaluate the progress and evolution of the pathology.
Evaluate the emotional burden of the caregiver in caring for the individual suffering from IPF
Evaluate the improvement in quality of life.
Evaluate the change in disability.
Evaluate the patient's fatigue and its evolution over the course of the disease. Fatigue is associated with dyspnea, impacts daily living activities and quality of life.
Evaluate the progress and evolution of dyspnea at rest and in relation to the performance of common daily activities.
The study plans to enroll 40 patients newly diagnosed with IPF
Inclusion criteria:
Exclusion criteria:
The sessions will be planned following the criteria of frequency, intensity, time and type of exercise:
Frequency: one meeting/every fifteen days in the first month and one meeting every fifteen days (+/- five days) in the second and third months.
Intensity: sixty minutes (with mBorg three dyspnea and minimum peripheral saturation 88%) Time: sixty minutes Type of treatment: exercises from the therapeutic education brochure.
Endpoints measures:
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| Measure | Description | Time Frame |
|---|---|---|
| Effectiveness of the rehabilitation intervention contained in the PDTAI (Inter-company diagnostic and therapeutic pathway) | % of patients participating in the physiotherapy session program, at least that 80% of the total enlisted participate in the cycle of sessions proposed. | 56 months |
| Improvement of functional capacity and cardiorespiratory endurance | Six Minute Walking Test (6MWT). This test is validated for multiple pathologies, including idiopathic pulmonary fibrosis. MCID range from 21.7 to 45 meters. One reduction of more than 50 meters in 6 months increases mortality by three times | 56 months |
| Measure | Description | Time Frame |
|---|---|---|
| % Survival of patients with IPF. | % Survival of patients with IPF. | 56 months |
| Caregiver Strain Index | Evaluate the emotional burden of the caregiver in assisting the affected person by IPF |
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Inclusion Criteria:
Exclusion Criteria:
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All patients newly diagnosed with IPF will be enlisted but only who comply with the criteria for rehabilitation care:
6MWT -meters ≥ 200 (Functionalcapacity); O2 therapy (Lt/min.) ≤ 3; Resting SpO2 ≥ 91% (Peripheraloxygensaturation); FVC (% predicted) ≥ 50% (Pulmonaryfunction); DLCO ≥ 30%(Pulmonaryfunction); mBorg at rest (score) ≤ 4 (Dyspnea); mMRC (score) ≤ 3 (Dyspnea)
As IPF is a rare disease, it is estimated that 20 patients/year can be enrolled on the basis of of the cases relating to previous years, therefore, considering the two years of enrolment, we expect to be able to enroll a total number of 40 patients.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Enrica Cavalli, MD | Contact | 0512142285 | enrica.cavalli@aosp.bo.it |
| Name | Affiliation | Role |
|---|---|---|
| Enrica Cavalli, MD | IRCCS Azienda Ospedaliero-Universitaria di Bologna | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| IRCCS Azienda Ospedaliero-Universitaria di Bologna | Recruiting | Bologna | Bologna | 40138 | Italy |
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| ID | Term |
|---|---|
| D011658 | Pulmonary Fibrosis |
| ID | Term |
|---|---|
| D017563 | Lung Diseases, Interstitial |
| D008171 | Lung Diseases |
| D012140 | Respiratory Tract Diseases |
| D005355 | Fibrosis |
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| 56 months |
| EuroQoL scale (EQ-5D-5L) | Evaluate the improvement in quality of life | 56 months |
| Barthel Index Dyspnea | Evaluate the modification of disability | 56 months |
| Fatigue Assessment Scale (FAS) | Evaluate the patient's fatigue and its evolution during the course of the disease. Fatigue is associated with dyspnea, impacts daily life activities and quality of life. | 56 months |
| Modified British Medical Research Council (mMRC) | Evaluate the trend and evolution of dyspnea at rest and in relation to completion of common daily activities | 56 months |
| D010335 |
| Pathologic Processes |
| D013568 | Pathological Conditions, Signs and Symptoms |