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Colorado Cystic Fibrosis Research & Development Program

Clinical Core

Our goals are to help develop evidence-based translational strategies and improved outcomes in the prevention and treatment of nontuberculous mycobacteria (NTM) in cystic fibrosis (CF).

The Clinical Research Core is led by Stacey Martiniano, MD at the University of Colorado and Children's Hospital Colorado and Jerry Nick, MD at National Jewish Health. The Clinical Research Core supports investigators in all aspects of clinical and translational research related to NTM infection in CF. This includes assistance with study design, and implementation of trials through help with study coordination, screening, recruitment, biostatistical and bioinformatics support. Resources are available to collect, store and distribute data and samples, with a particular focus on biomarker development. The Clinical Core is also available for consultation concerning challenging aspects of NTM treatment.

The Clinical Research Core is currently supporting the following NTM trials:

Isolation of NTM from CF sputum is relatively common, and of uncertain significance, as clinical sequelae can range from undetectable to severe. In the context of CF, the diagnosis of NTM disease and the decision to treat currently requires more than one positive culture accompanied by evidence of accelerated clinical decline. Achieving a standardized diagnosis of NTM disease is essential for the conduct of therapeutic trials and to identify individuals that will benefit from treatment. The primary objective of this trial is to develop and test feasibility of a standardized diagnostic protocol to assist with identification of CF patients requiring treatment for NTM disease that is based on recent guidelines from the CF Foundation (CFF) and the European CF Society (ECFS). Patients with a positive culture for NTM in the past year are eligible. The study’s primary endpoint is diagnosis of NTM disease at 6, 12, and 24-months following enrollment.

 

No NTM treatment protocol has been prospectively validated in the setting of CF. This primary objective of this trial is to develop and test the feasibility of a standardized treatment algorithm for confirmed disease by either Mycobacteria avium complex (MAC) or Mycobacteria abscessus complex (MABSC) that is also based on recent CFF/ECFS guidelines. First-line treatment consists of combinations of 3 or 4 antibiotics, determined by the type of infection and severity of presentation. The algorithm also includes scheduled testing for medication-induced toxicities, as well as second- and third-line options in the setting of treatment failure. The primary endpoint is 12 months of negative NTM cultures following completion of therapy.

 

NTM pulmonary infections are difficult to treat and treatment failure is common, possibly in-part due to sub-therapeutic drug levels. Clinical pharmacokinetics (PK) and pharmacodynamics (PD) have been shown to be altered in CF, however no specific studies have been performed for the antimycobacterial drugs in CF. The purpose of this study is to determine oral antimycobacterial drug PK and PD in patients with CF. Our hypothesis is that CF patients have altered drug absorption and metabolism leading to sub-therapeutic drug levels and that taking drugs with food and supplemental enzymes may improve drug levels by improving intestinal absorption. The aims of the proposal are (1) compare PK of oral antimycobacterial drugs (azithromycin, rifampin and ethambutol) under both fasting conditions and when taken with food plus enzymes in 20 subjects with pancreatic insufficient CF to 10 healthy controls, (2) begin to investigate the influence of host characteristics and drug metabolism on the PK of antimycobaterial drugs, and (3) estimate an optimized dosing regimen for the antimycobacterial drugs against MAC. Upon completion of this study we will determine if and why PK of the antimycobacterial drugs are altered in CF and may lead to CF-specific guidelines to achieve therapeutic goals with recommendations for drug dosing, enzyme use, and timing of therapeutic monitoring to be used for future treatment of NTM lung disease in CF. 

 

  • Healthcare-Associated Links in Transmission of Nontuberculous Mycobacteria in CF (HALT NTM)

If you are interested in participating in this study, please contact Jane Gross.Sources of NTM infection and modes of transmission among CF patients are poorly understood. Healthcare-associated transmission of NTM among CF patients has been suspected and is of growing concern for CF Centers. There is a need for a systematic evidence-based approach to investigating potential episodes of healthcare-associated transmission. Clusters of highly similar strains of NTM in CF patients cared for at the same CF Center may arise from healthcare sources including patient-to-patient transmission and/or acquisition from water sources within a healthcare setting. The primary objective of the study is to facilitate a standardized process by which CF Centers may perform data abstraction on patients identified with highly similar NTM isolates and determine if clustered NTM strains are related to strains isolated from healthcare setting water biofilm sources. HALT NTM is available to the entire CF Foundation Care Network, under a collaborative agreement, to initiate a standardized, independent, confidential, internal NTM outbreak investigation. Patients that are identified by whole genome sequencing as having highly similar NTM strains and receiving care in the same CF Care Center are eligible. The study’s primary endpoint is to identify potential modes and sources of healthcare-associated acquisition of CF NTM, thereby revealing risk factors for NTM acquisition.

If you are interested in participating in this study, please contact Jane Gross.

The Clinical Research Core has extensive experience with designing and performing clinical studies, standardizing specimen collection, maintaining clinical and genetic databases, investigating novel biomarkers and providing biostatistical and bioinformatics support for CF investigators. In addition to the three NTM trials highlighted above, we are currently conducting over 60 additional clinical trials within the Colorado CF Center, including both CFF Therapeutic Development Network (TDN) multicenter treatment and observational studies, as well as investigator-initiated trials. In many studies we provide support in bioinformatics, biostatistics, patient recruitment and study coordination. Almost all studies have been performed in conjunction with the Colorado Clinical and Translational Sciences Institute (funded through an NIH CTSA).