Ketone Supplementation in Cystic Fibrosis

Study Overview
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Brief Summary
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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

Detailed Description
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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

Official Title
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Ketone Monoester Supplementation in Cystic Fibrosis: A Pilot and Feasibility Study

Conditions
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Cystic Fibrosis

Intervention / Treatment
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* Dietary Supplement: Ketone Monoester


Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 89 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Allocation: Randomized
Intervention Model: Parallel Assignment
Interventional Model Description: Two-way ANOVA with repeated measures on time
Masking: Quadruple


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Ketone Monoester<br>The ketone used is from KetoneAid and is commercially available as KE4 and will be consumed at 30 mL twice daily (15 g / dose) | Dietary Supplement: Ketone Monoester<br>* Ketone supplement will be taken by mouth at a dose of 15 mL twice per day<br>|
| Placebo Comparator: Placebo<br>A placebo is being manufactured by KetoneAid to match the taste and other characteristics of the ketone supplement for blinding purposes | Dietary Supplement: Ketone Monoester<br>* Ketone supplement will be taken by mouth at a dose of 15 mL twice per day<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Pulmonary Function Testing | FEV1.0 (L/sec) | 5 days | 
| Inflammatory markers | Interleukin 1Beta (ug/mL) | 5 days | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Assessment of quality of life, respiratory symptoms, and patient outlook | A questionnaire will be used to assess quality of life and patient outlook 30 days following the intervention | 5 days |