Treatment With Elexacaftor/Tezacaftor/Ivacaftor, in Patients With Cystic Fibrosis and Caloric Intake

University Hospital, Toulouse120 enrolled

Overview

The aim of the study is to describe the evolution of caloric intake in patients with cystic fibrosis with an indication to start treatment with Elexacaftor/Tezacaftor/Ivacaftor according to the Marketing Authorization, between the start of treatment and at 12 months.

Cystic fibrosis is a genetic pathology linked to a dysfunction of the CFTR protein. Undernutrition is common in the natural course of cystic fibrosis, it is linked to exocrine pancreatic insufficiency but also to the increase in energy expenditure due to respiratory damage. At a time when these CFTR modulators are greatly modifying the prognosis and management of cystic fibrosis, it is important to describe how caloric and nutritional intake evolve under treatment with Elexacaftor/Tezacaftor/Ivacaftor in order to be able, in the long term, to adapt nutritional recommendations under treatment with CFTR modulators. The aim of the study is to describe the evolution of caloric intake in patients with cystic fibrosis with an indication to start treatment with Elexacaftor/Tezacaftor/Ivacaftor according to the Marketing Authorization, between the start of treatment and at 12 months.

Study Type

OBSERVATIONAL

Enrollment

120

Conditions

Cystic Fibrosis

Interventions

nutritional intake questionnaireOTHER

patients completed a nutritional intake questionnaire before to start the treatment, 3mounths after and twelve months after

Eligibility

Sex: ALLMin age: 2 YearsMax age: 100 Years

Medical Language ↔ Plain English

Inclusion Criteria: * Patient with cystic fibrosis * Patient with an indication to start treatment with Elexacaftor/Tezacaftor/Ivacaftor according to the Marketing Authorization. * Patient who has not received treatment with CFTR modulators in the 6 months preceding inclusion. * No opposition expressed by the patient (if ≥ 18 years) or by at least one of the holders of parental authority and the child (if \< 18 years). * Be affiliated to a social security scheme or be a beneficiary of such a scheme. Exclusion Criteria: \-

Locations (3)

UHBordeaux

Bordeaux, France

UHLimoges

Limoges, France

UHToulouse

Toulouse, France

Outcomes

Primary Outcomes

caloric intake 12 months after the beginning of treatment

Describe the evolution of caloric intake in patients with cystic fibrosis with an indication to start treatment with Elexacaftor/Tezacaftor/Ivacaftor according to the Marketing Authorization, between the start of treatment and at 12 months.

Time frame: 12 months

Secondary Outcomes

caloric intake 3 months after the beginning of treatment

Time frame: 3 months

Data from ClinicalTrials.gov

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