Ketone Supplementation in Cystic Fibrosis

This study will examine the effects of a novel ketone monester supplement on patients with cystic fibrosis experiencing an acute pulmonary exacerbation requiring hospitalization. Patients will undergo standard testing along with blood sampling to examine concentrations of inflammatory markers. Patients will then receive the ketone supplement for 5 days followed by post-testing to examine changes in pulmonary function and inflammatory markers

Up to 25 hospitalized patients with cystic fibrosis will take part in this study at UAB. Patients will be randomly assigned to receive either an exogenous ketone or placebo supplement. Participants will take the ketone or placebo for 5 days during hospitalization. Participants will drink one 60 mL bottle twice daily of either the ketone or placebo. On day 1 and day 5, before the patient drinks the ketone (or placebo) the patient will be asked to fast overnight for 8 hours. Following administration of the first morning dose of ketones (or placebo) blood samples will be obtained from a finger stick before (time 0 min) and following (15, 30, 60, 90, and 120 min) after they drink the supplement. A commercially available ketone meter (Keto-Mojo, Napa, CA) that provides instant assessment of circulating ketone concentrations will be used to measure ketone concentrations in the blood. Medical history history will be obtained at hospitalization. The study team will also examine the body for any abnormal signs and symptoms. Sputum and blood will be collected prior to and following the ketone supplement or placebo. The hypothesis is that ketone supplementation will reduce inflammation via a well known inflammatory pathway to improve outcomes in patients with cystic fibrosis experiencing an acute pulmonary exacerbation

The ketone used is from KetoneAid and is commercially available as KE4 and will be consumed at 30 mL twice daily (15 g / dose)

A placebo is being manufactured by KetoneAid to match the taste and other characteristics of the ketone supplement for blinding purposes

A questionnaire will be used to assess quality of life and patient outlook 30 days following the intervention

Inclusion Criteria: Diagnosis of CF Age >19 Colonization with P. Aeruginosa Acute pulmonary exacerbation requiring inpatient care Exclusion Criteria: Concurrent or recent (within 28 d of enrollment) use of corticosteroids Inability to produce sputum Acute respiratory failure Chronic liver or renal disease Pregnancy