Purpose

This clinical trial is examining the action and effects of several new drugs in the treatment of cystic fibrosis in children. In addition, several genetic factors are examined. The hope is that the ability to determine prior to treatment those individuals who will or will not respond to existing therapies will avoid needless risk of side effects and the high cost of a potentially ineffective treatment regimen. Understanding the way these drugs work in the body and the best way to study them is critical to expanding the use of these drugs to all patients with cystic fibrosis (CF).

Condition

Eligibility

Eligible Ages
Over 3 Years
Eligible Sex
All
Accepts Healthy Volunteers
No

Inclusion Criteria

  • documentation of CF diagnosis per CFF diagnostic criteria and known CFTR genotype - age 2 years and older - ability to provide written informed consent and/or assent (by subject and/or legal guardian) - on a stable dose of triple combination CFTR modulator therapy for at least two weeks prior to Visit 1 - clinically stable lung disease, defined as no documented acute decrease in FEV1 > 10%, OR use of additional antibiotics (intravenous [IV] or oral [PO]) within 4 weeks prior to screening

Exclusion Criteria

  • recent significant unintentional weight loss, as determined by the investigator, in the 4 weeks prior to screening - pregnant or breastfeeding female - history of alcohol or substance abuse in the 6 months prior to screening - participation in a study involving an investigational intervention within 28 days (or 5 half-lives, whichever is longer) prior to screening - in the opinion of the Investigator, medical or psychiatric illness, or other conditions that would interfere with participation

Study Design

Phase
Phase 4
Study Type
Interventional
Allocation
N/A
Intervention Model
Single Group Assignment
Intervention Model Description
This is a pilot and feasibility study to assess the association of drug concentration with side effects, and to assess the feasibility of the use of therapeutic drug monitoring to adjust dosing in individuals with side effects.
Primary Purpose
Treatment
Masking
None (Open Label)

Arm Groups

ArmDescriptionAssigned Intervention
Other
Single Arm
Participants may be enrolled in an observational one-visit study for association of concentration with side effects. Participants may proceed to a single arm study if they have side effects to assess the feasibility of adjusting dose to maintain concentrations within an estimated effective range. Once within the range, dosing is no longer adjusted. Side effects will be evaluated as described in the protocol.
  • Drug: Elexacaftor / Ivacaftor / Tezacaftor
    This study will examine different dosing strategies and outcomes for triple combination CFTR modulator therapy using the drug(s) elexacaftor, tezacaftor, and/or ivacaftor in patients with cystic fibrosis.
    Other names:
    • Triple Combination CFTR Modulator Therapy
    • ETI - Elexacaftor/Tezacaftor/Ivacaftor
  • Other: therapeutic drug monitoring
    Participants who consent to the therapeutic drug monitoring study will have their dose adjusted to remain within estimated effective concentrations.

Recruiting Locations

The University of Alabama at Birmingham
Birmingham, Alabama 35233
Contact:
Stefanie Krick, MD

More Details

Status
Recruiting
Sponsor
Arkansas Children's Hospital Research Institute

Study Contact

Jennifer S Guimbellot, Medical Degree and License
501-364-5365
jguimbellot@uams.edu

Detailed Description

Understanding variation in genetic response to pharmacological treatments and personalized CFTR modulator response is crucial to the optimization of the use of these novel compounds; expansion to all patients who might benefit from them; and development of predictive biomarkers. In addition, the ability to determine prior to treatment those individuals who will or will not respond to existing therapies will avoid needless risk of side effects and the high cost of a potentially ineffective treatment regimen. Understanding the way these drugs work in the body and the best way to study them and the downstream effects is critical to expanding the use of these drugs to all patients with cystic fibrosis (CF).

Notice

Study information shown on this site is derived from ClinicalTrials.gov (a public registry operated by the National Institutes of Health). The listing of studies provided is not certain to be all studies for which you might be eligible. Furthermore, study eligibility requirements can be difficult to understand and may change over time, so it is wise to speak with your medical care provider and individual research study teams when making decisions related to participation.