About this trial
The goal of this clinical trial is to learn if drug SPL84 is safe for adult patients with cystic fibrosis (CF). It will also learn if the drug works to treat works to treat CF with a specific mutation (3849 +10kb C--\>T).
The purpose of this research study is to test the safety and effectiveness of multiple doses of the study drug, SPL84.
Researchers will compare drug SPL84 to a placebo (a look-alike substance that contains no drug) to see if drug SPL84 is safe and if it works to treat CF. In cohorts 1-3, SPL84 will be tested as a monotherapy, and in Cohort 4, SPL84 will be tested in participants who are already stable on CFTR modulator therapy.
Participants will take drug SPL84 or a placebo by inhalation every week for 9 weeks (cohorts 1-3) or 12 weeks (cohort 4) and visit the clinic approximately weekly for checkups and tests.
Eligibility criteria
This trial does not accept healthy volunteersQualifiers
Diagnosis of CF and two CF causing mutations; 3849+10 Kb C->T mutation on one allele in the CF transmembrane conductance regulator (CFTR) gene (homozygote or compound heterozygote). Source documentation from a certified genetic laboratory is required.
Body mass index (BMI) of ≥ 17 kg/m2.
FEV1 40-90% predicted at screening.
Non-smokers or vapers for at least 180 days (6 months) prior to screening, per participant report.
Disqualifiers
Use of Kalydeco, Orkambi, Symdeko/Symkevi or Trikafta/Kaftrio within 30 days of first dose with study intervention.
Use of any investigational drug (other than SPL84) or device within 30 days of first dose with study intervention.
Use of systemic steroids over 3 consecutive months in the last 6 months prior to screening, or use of systemic steroids in the last month prior to screening. Use of inhaled steroids above 1 mg.
Use of CF medications, e.g. inhaled antibiotics, dornase alfa (Pulmozyme), hypertonic saline and physiotherapy should be on stable regimen for the period 28 days prior to screening; those participants taking inhaled antibiotics for prophylaxis must be on a stable regimen of these drugs for at least 90 days prior to first dose with study intervention.
Trial design
Sequential
Treatments tested in this trial
SPL84
DrugSPL84 solution for nebulization
Placebo
Other interventionPlacebo solution for nebulization
Treatment groups
Trial outcomes
Primary outcomes
Safety and Tolerability of SPL84 as evaluated by number of subjects with at least one treatment-related adverse event (AE) or serious adverse event (SAEs)
Incidence, nature, and severity of AEs and SAEs
Safety and Tolerability of SPL84 as assessed by number of participants with abnormal heart rate
Safety and Tolerability of SPL84 as assessed by number of participants with abnormal respiratory rate
Safety and Tolerability of SPL84 as assessed by number of participants with abnormal systolic and diastolic blood pressure
Secondary outcomes
Characterization of pharmacokinetics (PK) of SPL84: maximum serum concentration (Cmax)
Characterization of PK of SPL84: Time to Cmax (Tmax)
Characterization of PK of SPL84: terminal elimination half-life (t1/2)
Characterization of PK of SPL84: Area under the curve to the final sample (AUC0-t)
Sponsors and contacts
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