Impact of a Systematic Palliative Care on Quality of Life, in Advanced Idiopathic Pulmonary Fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a rare and severe disease with a survival median between 2 and 4 years which leads to a profound alteration of the quality of life.

In thoracic oncology, the systematic and early intervention of a palliative care team result in an improvement of quality of life for patients.

In the princeps study published in 2010, the early intervention of a dedicated palliative care team was compared to standard care in a randomized trial of 150 patients and shows a significant improvement : (i) of quality of life (main objective), (ii) of depression scores and even overall survival (11.6 months vs. 8.9 months, P = 0.02), (iii) a benefit in terms of understanding the diagnosis and therapeutic goals (3), (iv) diminution of adapted hospitalization in end of life (in emergency or not).

Considering some analogy points between IPF and advanced lung cancer (prognosis, respiratory symptom, psychological burden), it seemed reasonable to assume that the joint systematic intervention of chest physician and palliative care team may provide a significant benefit in terms of quality of life for patients with severe IPF.

Idiopathic pulmonary fibrosis (IPF) is a rare and severe disease with a survival median between 2 and 4 years which leads to a profound alteration of the quality of life. This alteration results from different consequences of the IPF: progressive shortness of breath, irritative cough refractory to treatments, exhaustion, limitation of activity, social isolation, and psychic consequences such as fear, anxiety and depression.

The only current curative treatment of the disease is pulmonary transplantation, but it's only feasible for a minority of patients. Anti-fibrotic drugs, such as pirfenidone and nintedanib, are likely to slow the progression of IPF but have no impact on patients' quality of life.

The symptomatic treatment aimed at relieving respiratory discomfort and the patient's quality of life is therefore fundamental, and the IPF meets in many ways the challenges of lung cancer.

In thoracic oncology, the systematic and early intervention of a palliative care team result in an improvement of quality of life for patients.

In the princeps study published in 2010, the early intervention of a dedicated palliative care team was compared to standard care in a randomized trial of 150 patients and shows a significant improvement : (i) of quality of life (main objective), (ii) of depression scores and even overall survival (11.6 months vs. 8.9 months, P = 0.02), (iii) a benefit in terms of understanding the diagnosis and therapeutic goals (3), (iv) diminution of adapted hospitalization in end of life (in emergency or not).

Considering some analogy points between IPF and advanced lung cancer (prognosis, respiratory symptom, psychological burden), it seemed reasonable to assume that the joint systematic intervention of chest physician and palliative care team may provide a significant benefit in terms of quality of life for patients with severe IPF.

Objective:

To investigate the benefit on quality of life, evaluated after 6 months, of a systematic, formalized and joint intervention of a palliative intervention staff and a chest physician team compared to standard care for patients with severe IPF.

Secondary endpoints

1. To evaluate the benefit of the systematic, formalized and joint intervention of a palliative care team and a chest physician team on:

   * Mood and depression
   * Understanding of diagnosis and therapeutic objectives, frequency of drafting of advance directives regarding end-of-life
   * Respiratory symptoms (cough and dyspnea)
   * The course of care, the use of palliative care stays and the duration of hospital stays (number and duration of hospitalizations).
   * Overall survival and place of death.
2. Carry out a medico-economic study evaluating the incremental cost-utility and cost-effectiveness ratio (overall survival criterion)

Eligibility Criteria:
Inclusion Criteria:

* Age\> 40 years
* Patient with confirmed diagnosis of IPF according to the American Thoracic Society (ATS) / European Respiratory Society (ERS) / Japanese Respiratory Society (JRS) / Latin American Thoracic Association (ALAT) criteria. The patient may be included regardless of the date of diagnosis.
* Advanced IPF with Forced Vital Capacity (FVC) \<50%" of predicted value and / or Diffusing capacity for carbon monoxide ((DLCO) \<30% of predicted value or inability to achieve the Functional Respiratory Investigations (EFR) due to respiratory severity. EFR dated less than 3 months.
* Absence of argument for acute or subacute exacerbation in the last 6 months.
* Patient who can be followed in ambulatory consultation/ outpatient consultation.
* Informed consent signed (signed by the patient or in the presence of a third party for patients who are poorly fluent in French).
* Affiliation to the social security system.

Exclusion Criteria:

* Patient unable to respond to quality of life questionnaires.
* Inability (physical or mental) to give a written informed consent.
* Acute exacerbation of fibrosis in the previous 6 months.
* Patient eligible for a pulmonary transplant.
* Participation in other therapeutic trial
* Patient cannot be followed in ambulatory consultation.
* Patient under trustee