Study of Peripherally Inserted Venous Catheters in Cystic Fibrosis Patients

According to data from the U.S. CF Foundation Patient Registry (CFFPR), more than 25% of
children and 40% of adults were treated with intravenous (IV) antibiotics for pulmonary
exacerbations (PEx) in 2016; this translated to 20,286 care episodes. Case series have
identified important complications of PICCs and TIVADs in CF patients such as deep venous
thrombosis (DVT) and infection; central venous stenosis; and other types of mechanical
dysfunction. The incidence of PICC-associated DVT in CF patients ranges from 2 to 8%.
Proposed risk factors in CF patients fall into several broad categories. First are
catheter-related factors: larger catheter size, number of catheter lumens, and catheter
composition; second are patient factors: lung function, nutritional status, respiratory
flora, diabetes status, thrombophilia; and number of previously placed catheters; and third
are catheter-management factors: insertion technique and site selection, inpatient versus
outpatient care, anticoagulant use, blood sampling practices, and duration of line use. The
importance of some of these factors remains controversial, leading some to call for detailed
prospective studies of PICC use in specific patient populations. To date, no multicenter
trial has prospectively studied PICC and midline complications in adult and pediatric CF
patients. The investigators hypothesize that the rate of PICC and midline vascular
complications is associated with specific patient level and line level factors as well as
with line insertion and management practices. The proposed trial is a multicenter,
prospective surveillance study to evaluate (patient level-, catheter level- and catheter
management-) risk factors associated with various complications of PICCs and midlines in
pediatric and adult CF patients.

Inclusion Criteria:

- All ethnic groups

- Females and males

- 6 years of age and above (in order to perform spirometry on all study participants)

- Undergoing treatment for PEx with IV antibiotics via hospital-placed PICC or midline
catheter

- Ability to communicate with pertinent staff.

- Ability to understand and willingness to comply with the requirements of the trial
(allow repeated assessment of the catheter insertion site, photographs of the site,
extremity measurement and face-to face assessment on the day of line removal).

- Ability and willingness to give verbal consent (with the assistance of a parent or
guardian, if appropriate) or assent (for pediatric patients)

- Diagnosis of cystic fibrosis consisting of both:

- sweat sodium or chloride > 60 mEq/L by the pilocarpine iontophoresis method or
cystic fibrosis genotype (homozygous for CFTR mutation or compound heterozygous
for CFTR mutations)

- clinical manifestations of cystic fibrosis

Exclusion Criteria:

- Under age 6

- Planned use of a TIVAD or peripheral catheter for IV therapy for the full course of
therapy

- History of >10 PICCs prior to study entry

- IV treatment anticipated to extend beyond 21 days at the time of line insertion

- Patient taking anticoagulant medication (other than NSAIDs) at the time of screening

- Inability/unwillingness to provide consent or assent