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Search Criteria:
	Post Transplant Studies Only:
Trials matching criteria:		104
Studies matching criteria:		32
International trials/studies matching criteria:		7 Trials / 2 Studies

Interventional

Recruitment Status: Not Yet Recruiting

1.	Arikace Compared to TOBI in People with CF with Chronic Pseudomonas Aeruginosa Infections This trial will look at whether Arikace is effective in treating chronic lung infections caused by Pseudomonas aeruginosa in people with Cystic Fibrosis. The effectiveness, safety and tolerability of Arikace will be compared to TOBI, an inhalation antibiotic already available for use. Study Drugs: Arikace Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: >= 25 Percent Predicted Trial Results Not Yet Available

2.	Study of Arikace in People with CF with Chronic Lung Infection Due to Pseudomonas Aeruginosa Liposomal amikacin for inhalation (Arikace) was developed as a possible treatment for chronic infection due to Pseudomonas aeruginosa in people with CF. The purpose of this double-blind, placebo controlled study is to determine whether Arikace is effective in treating chronic lung infections caused by Pseudomonas aeruginosa in people with CF. Study Drugs: Arikace Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: >= 25 Percent Predicted Trial Results Not Yet Available

3.	Long term study of Arikace in people with CF with Chronic Pseudomonas Aeruginosa Lung Infection This study will look at the long term safety and tolerability of Arikace in people with Cystic Fibrosis for up to approximately 2 years. Study Drugs: Arikace Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: >= 25 Percent Predicted Trial Results Not Yet Available

4.	Burlulipase in People with CF and Pancreatic Insufficiency This is a Phase 2 multi-center, randomized, double-blind trial looking at the effectiveness of burlulipase in people with CF and pancreatic insufficiency who are ages 12 years and above. Study Ages: >= 12 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

Recruitment Status: Recruiting

5.	Aztreonam for Inhalation Solution (AZLI) taken in a Continuous Alternating Therapy Regimen for the Treatment of Chronic Pseudomonas aeruginosa lung infections in people with CF This is a Phase 3, Randomized, Double-Blind, Placebo-Controlled, Multicenter study of Aztreonam for Inhalation Solution (AZLI) in a Continuous Alternating Therapy (CAT) regimen of inhaled antibiotics for the treatment of chronic pulmonary Pseudomonas aeruginosa infection in people with CF ages 6 years and older. Study Drugs: Cayston Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 25 - 75 Percent Predicted Trial Results Not Yet Available

6.	AeroVanc for the treatment of MRSA in people with CF This trial will look at the safety and effectiveness of AeroVanc in treating persistent MRSA lung infections in people with cystic fibrosis. Study Drugs: Inhaled Vancomycin Study Ages: >= 12 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: 30 - 90 Percent Predicted Trial Results Not Yet Available

7.	Arikace for Nontuberculous Mycobacteria (NTM) The purpose of this study is to determine whether Arikace is effective in treating recalcitrant nontuberculous mycobacterial lung disease. The safety and tolerability of Arikace in this patient population will also be assessed. Study Drugs: Arikace Study Ages: 12 Years - 75 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

8.	Tobramycin Inhalation Powder (TIP) in People with Cystic Fibrosis This study will assess the long term safety for the use of tobramycin inhalation powder (TIP) in people with cystic fibrosis who are 6 years of age and older and who have a chronic pulmonary infection with Pseudomonas Aeruginosa. Study Drugs: Tobramycin Inhalation Powder Study Ages: >= 6 Weeks Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 25 - 75 Percent Predicted Trial Results Not Yet Available

9.	Early Treatment of New Onset of Methicillan Resistant Staphylococcus Aureus (MRSA) Growth in Respiratory Culture This study will evaluate an early eradication protocol to eliminate Methicillan Resistant Staphylococcus Aureus (MRSA) from the lungs of people with CF who grow this bacteria for the first time in a respiratory culture. Study Drugs: Multiple systemic antibiotics Study Ages: 4 Years - 45 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 30 Percent Predicted Trial Results Not Yet Available

10.	Persistent Methicillan Resistant Staphylococcus Aureus (MRSA) Growth in Respiratory Culture This study will look at the safety and effectiveness of a 28-day course of vancomycin for inhalation in getting rid of the bacteria, Methicillan Resistant Staphylococcus Aureus (MRSA), from the respiratory tract of people with CF and persistent MRSA infection. Study Drugs: Multiple systemic antibiotics Study Ages: >= 12 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 30 Percent Predicted Trial Results Not Yet Available

11.	KB001A in People with CF and Pseudomonas Aeruginosa This is a Phase 2 multi-center randomized, double-blind, placebo-controlled trial looking at the safety and effectiveness of KB001A to increase time-to-need for antibiotic treatment for worsening respiratory tract signs and symptoms compared to placebo in people with CF ages 12 - 50 with chronic Pseudomonas aeruginosa lung infection. Study Drugs: KB001A Study Ages: 12 Years - 50 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 40 - 80 Percent Predicted Trial Results Not Yet Available

12.	Inhaled Alpha-1 HC in People with CF The purpose of this study is to look at the safety and tolerability of 100 mg and 200 mg of inhaled Alpha-1 HC administered once a day for three weeks in people with CF who are age 18 years and older. Study Drugs: Alpha 1 Anti-Trypsin Study Ages: >= 18 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: 40 - 90 Percent Predicted Trial Results Not Yet Available

13.	Vertex Lumacaftor (VX-809) and Kalydeco in people with CF who are aged 12 years and have two copies of the delF508 CFTR mutation (TRAFFIC) This is the first of two nearly identical Phase 3 trials looking at the safety and effectiveness of the study drug VX 809 in combination with Kalydeco in people with CF who are aged 12 years and older and have two copies of the F508del-CFTR mutation. Study Drugs: VX-809/VX-770 Study Ages: >= 12 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

14.	Vertex Lumacaftor (VX-809) and Kalydeco in People with CF aged 12 years and older and have two copies of the delF508 CFTR mutation (TRANSPORT) This is the second of two nearly identical Phase 3 trials looking at the safety and effectiveness of the study drug VX 809 in combination with Kalydeco in people with CF who are aged 12 years and older and have two copies of the F508del-CFTR mutation. Study Drugs: VX-809/VX-770 Study Ages: >= 12 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

15.	Ivacaftor Open Label Follow on Study in People with CF who have a Non-G551D CFTR Mutation This trial is looking at the safety of long term ivacaftor treatment in people with CF who have a non-G551D CFTR mutation. Study Drugs: Kalydeco Study Ages: >= 6 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

16.	Ivacaftor in People with CF age 2 to 5 years with a CFTR Gating Mutation This study will look at the safety, pharmacokinetics (PK), and pharmacodynamics (PD), of ivacaftor in children with CF who are 2 through 5 years of age and have a CFTR gating mutation in at least 1 allele. Study Drugs: Kalydeco Study Ages: 2 Years - 5 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

17.	Ivacaftor (Kalydeco) in People with CF who have a Non G551D Gating Mutation (KONNECTION) The purpose of this trial is to look at the safety and effectiveness of ivacaftor (trade name Kalydeco) in people with cystic fibrosis (CF) who have a non-G551D cystic fibrosis transmembrane regulator (CFTR) gating mutation (any one of the following CFTR mutations: G178R, G551S, S549N, S549R, G970R, G1244E, S1251N, S1255P, or G1349D). Study Drugs: Kalydeco Study Ages: >= 6 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 40 - 105 Percent Predicted Trial Results Not Yet Available

18.	Ivacaftor (Kalydeco) in People with CF who have the R117H-CFTR mutation (KONDUCT) This study will look at the safety and effectiveness of ivacaftor (Kalydeco) in people with CF who have the R117H-CFTR mutation. Study Drugs: Kalydeco Study Ages: >= 6 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 40 - 105 Percent Predicted Trial Results Not Yet Available

19.	VX-661 alone and in combination with Kalydeco in People with Cystic Fibrosis and F508del-CFTR mutation This Phase 2 trial will look at the safety, effectiveness of VX-661 alone and in combination with Kalydeco in people with CF who have two copies of the F508del-CFTR mutation. Additionally this trial will look at the pharmacological properties of VX-661 in the body - how well the body absorbs, breaks down, and gets rid of VX-661. Study Drugs: VX-661/VX-770 Study Ages: >= 18 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 40 - 90 Percent Predicted Trial Results Not Yet Available

20.	VX-770 Expanded Access Program People with CF who have at least one copy of the G551D-CFTR mutation and who are in critical medical need and who are not eligible for participation in other Vertex-sponsored studies may be able to receive VX-770 through an expanded access program. Study Drugs: Kalydeco Study Ages: >= 6 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 1 - 40 Percent Predicted Trial Results Not Yet Available

21.	PULMOZYME delivered by the eRapid Nebulizer This is a phase 4 multi-center, randomized, open-label trial looking at the safety and effectiveness of Pulmozyme delivered by the eRapid nebulizer system. Study Drugs: Pulmozyme Study Ages: >= 6 Years Type of Therapy: Mucus Alteration Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

22.	Vitamin D for enhancing the immune system in people with CF This multi-center trial will look at the effect of a one time high-dose vitamin D to rapidly correct vitamin D deficiency in people with CF who are over 16 years of age admitted to the hospital for pulmonary exacerbation. Study Drugs: Vitamin D Study Ages: >= 16 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 20 Percent Predicted Trial Results Not Yet Available

23.	Study of N6022in People with CF who have two copies of the F508del-CFTR mutation This trial will look at the safety, tolerability and pharmacokinetics and effects CFTR biomarkers of N6022 injected once daily for 7 days in people with CF who are 18 years and older and have two copies of the F508del-CFTR mutation. Study Drugs: N6022 (N30) Study Ages: >= 18 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

24.	Home Monitoring of Lung Function This study will look at the monitoring lung function and lung symptoms at home. Study Ages: >= 14 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 26 Percent Predicted Trial Results Not Yet Available

25.	Effects of Nutrition and Lung Disease on Growth in Preschoolers with CF The purpose of this study is to examine the relationship between a newly developed nutritional method, patient growth, and lung disease. Study Ages: 2 Years - 6 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

26.	Acid Suppression therapy in People with CF This study will look at whether bacteria in the throat changes when people with CF take acid suppression medications, proton pump inhibitors (PPI). Study Ages: 3 Months - 21 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

Recruitment Status: Recruiting Completed

27.	Aztreonam (AZLI) in Infants and Children with CF and New Onset Pseudomonas Aeruginosa This an open-label, multi-center trial looking at the effectiveness of Aztreonam Inhalation Solution given to infants and children with CF, age 3 months to less than 18 years, who have a newly detected Pseudomonas Aeruginosa lung infection based upon respiratory culture results. Study Drugs: Cayston Study Ages: 3 Months - 17 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: >= 90 Percent Predicted Trial Results Not Yet Available

28.	VX -770 in People with CF age 12 and older with 2 copies of the F508del-cftr mutation The purpose of this study is to assess the safety and effectiveness of VX-770 in people with cystic fibrosis (CF) who have two copies of the F508del-cystic fibrosis transmembrane conductance regulator (CFTR) mutation for 16 weeks. Study Drugs: Kalydeco Study Ages: - Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: Trial Results Not Yet Available

29.	Vertex 809/770 in People with Cystic Fibrosis and F508del-CFTR mutation This study looked at the safety and effectiveness of VX 809 and VX770 when given in combination to people with CF who have either one or two copies of the F508del-CFTR mutation. Study Drugs: VX-809/VX-770 Study Ages: >= 18 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

30.	Ataluren (PTC 124) Open Label Extension Study in Cystic Fibrosis This study is a Phase 3 extension trial that will evaluate the long-term safety of ataluren (PTC124) in adult and pediatric patients with nonsense mutation CF, as determined by adverse events and laboratory abnormalities. Study Drugs: PTC124 Study Ages: >= 6 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 40 - 90 Percent Predicted Trial Results Not Yet Available

31.	Tiotropium Bromide (Spiriva) Safety and Effectiveness in People with Cystic Fibrosis This study will look at the safety and effectivesness of tiotropium bromide (Spiriva) inhaled once a day using the Respimat device in people with Cystic Fibrosis. Study Drugs: Tiotropium Bromide Study Ages: >= 1 Days Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 26 Percent Predicted Trial Results Not Yet Available

32.	Cystic Fibrosis Nutritional Education and Behavioral Training This study will look at the effectiveness of a behavioral and nutritional treatment to improve BMI and lung functioning in children with cystic fibrosis ages 3 to 6 years old. Study Ages: 3 Years - 12 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

Recruitment Status: Trial Completed

33.	Aztreonam Inhalation Solution (AZLI) Safety Study in Children with CF and Chronic Pseudomonas Aeruginosa This study will look at the safety of Aztreonam Inhalation Solution (AZLI) given three times daily for three 28 day on/ 28 day off cycles in children < 13 years of age with Cystic Fibrosis (CF) and chronic Pseudomonas Aeruginosa (PA) infection in the lower airways. Study Drugs: Cayston Study Ages: 1 Days - 12 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

34.	Levofloxacin Inhalation Solution (Aeroquin) compared to TOBI in people with Cystic Fibrosis This study will look at the safety and effectiveness of the study drug, Levofloxacin Inhalation solution, (Aeroquinâ?¢) compared to Tobramycin Solution for Inhalation (TOBIÂ®) people with CF that have chronic Pseudomonas aeruginosa lung infection. Study Drugs: Levofloxacin (Inhaled) Study Ages: >= 12 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: 25 - 85 Percent Predicted Trial Results Not Yet Available

35.	Levofloxacin Inhalation Solution (Aeroquinâ?¢) compared to placebo in people with Cystic Fibrosis This trial will study the safety and effectiveness of the aerosolized form of the antibiotic, levofloxacin, (AEROQUINâ?¢) in people with Cystic Fibrosis. Study Drugs: Levofloxacin (Inhaled) Study Ages: >= 12 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: 25 - 85 Percent Predicted Trial Results Not Yet Available

36.	Amikacin for Inhalation (Transave) This study looked at a new way an inhaled antibiotic can be made for people with CF and a positive pseudomonas aeruginosa lung infection. Study Drugs: Arikace Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: >= 40 Percent Predicted Trial Results Exist

37.	Inhaled Fosfomycin/Tobramycin for People with CF and P. aeruginosa The purpose of this study is to evaluate the safety and efficacy of 2 dose combinations of fosfomycin/tobramycin for inhalation (FTI), following a 28-day course of Aztreonam for Inhalation (AZLI) in patients with cystic fibrosis and Pseudomonas aeruginosa lung infection. Study Drugs: Fosfomycin-Tobramycin (Inhaled) Study Ages: >= 18 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: 25 - 75 Percent Predicted Trial Results Exist

38.	Tobramycin Inhalation Powder Compared to TOBI in People with CF Study Drugs: Tobramycin Inhalation Powder Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 25 - 75 Percent Predicted Trial Results Exist

39.	Inhaled Levofloxacin (MP-376) Pediatric Safety Study This study will look at the safety, tolerability and how the body processes MP-376 Inhalation Solution given to children 6 to 16 years of age with Cystic Fibrosis. Study Drugs: Levofloxacin (Inhaled) Study Ages: 6 Years - 16 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 25 - 95 Percent Predicted Trial Results Not Yet Available

40.	Inhaled Levofloxacin (MP-376) in CF Patients with P. aeruginosa (Phase 2 Study) MP-376 is a form of levofloxacin being developed to be inhaled to control chronic pulmonary infections due to Pseudomonas aeruginosa and other bacteria. Study Drugs: Levofloxacin (Inhaled) Study Ages: >= 16 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 25 - 75 Percent Predicted Trial Results Exist

41.	Azithromycin in Patients with CF and Culture Negative for Pseudomonas aeruginosa This study evaluated the effect of azithromycin in children with CF who are not infected with Pseudomonas aeruginosa. Study Drugs: Azithromycin Study Ages: 6 Years - 18 Years Type of Therapy: Anti-Infective Pseudomonas Status: Negative B Cepacia Status: Negative FEV1% Status: >= 50 Percent Predicted Trial Results Exist

42.	Safety Study of Azithromycin in Cystic Fibrosis Patients Negative for P. aeruginosa This study was an open-label extension study of the Azithromycin study in adolescents and children with CF who were not infected with P. aeruginosa. This study provided addiitonal information regarding the safety of longer term treatment. Study Drugs: Azithromycin Study Ages: 6 Years - 18 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 30 Percent Predicted Trial Results Exist

43.	Early Pseudomonas Infection Control (EPIC) This study examined how often antibiotics should be administered to produce the greatest reduction in respiratory infections and pulmonary exacerbations. Study Drugs: TOBI Study Ages: 1 Years - 12 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

44.	Inhaled Ciprofloxacin for People with CF and Pseudomonas aeruginosa This study will compare the change in FEV1 from baseline to Day 28 between Cipro Inhale-treated and placebo-treated subjects. Study Drugs: Ciprofloxacin DPI Study Ages: >= 12 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 35 - 75 Percent Predicted Trial Results Not Yet Available

45.	Biomarker of Pulmonary Exacerbation in Patients with CF This study will look to determine if biomarkers indicate progression of CF lung disease. Study Drugs: Multiple systemic antibiotics Study Ages: >= 10 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

46.	Antibiotic Susceptibility Testing Using the Standard vs. the Biofilm Method This study compared the effectiveness of selecting antibiotic treatments through either a standard laboratory test or a new testing method. Study Drugs: Multiple systemic antibiotics Study Ages: >= 18 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: >= 1 Percent Predicted Trial Results Exist

47.	Inhaled Aztreonam Vs. TOBI in People with CF and P. aeruginosa The purpose of this study was to look at the safey and effectiveness of Aztreonam for Inhalation Solution versus Tobramycin Nebulizer Solution in adult and pediatric patients with CF and pulmonary Pseudomonas Aeruginosa. Study Drugs: Cayston Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 1 - 75 Percent Predicted Trial Results Exist

48.	AZLI (Inhaled Aztreonam) for People with Cystic Fibrosis and Chronic Burkholderia Infection This study looked at the safety and effectiveness of Aztreonam Inhalation Solution in people with Cystic Fibrosis and chronic burkholderia cepacia infection. Study Drugs: Cayston Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Positive FEV1% Status: >= 1 Percent Predicted Trial Results Exist

49.	Expanded Access Program for Aztreonam Lysine for Inhalation Aztreonam Lysine for Inhalation is now available to people with CF who meet specific criteria through an expanded access program. Study Drugs: Cayston Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 1 - 50 Percent Predicted Trial Results Not Yet Available

50.	Long-term Safety Study of Aztreonam Lysinate in CF Patients with P. aeruginosa (AIR-CF3) The purpose of this study was to evaluate the safety of an inhaled antibiotic, aztreonam, in CF patients who have respiratory infections caused by the Pseudomonas aeruginosa bacteria Study Drugs: Cayston Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: 25 - 75 Percent Predicted Trial Results Exist

51.	Aztreonam Lysinate for Inhalation in Individuals who Utilize Inhaled Tobramycin Frequently (AIR-CF2) The purpose of this study was to evaluate the safety and efficacy of an inhaled formulation of aztreonam lysine in CF patients who have respiratory infections caused by the Pseudomonas aeruginosa bacteria. Study Drugs: Cayston Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 25 - 75 Percent Predicted Trial Results Exist

52.	Aztreonam Lysinate for Inhalation in Cystic Fibrosis Patients with P. aeruginosa (AIR-CF1) The purpose of this study was to evaluate the safety and efficacy of an inhaled antibiotic, Aztreonam, in CF patients who have respiratory infections caused by the Pseudomonas aeruginosa bacteria. Study Drugs: Cayston Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 25 - 75 Percent Predicted Trial Results Exist

53.	Aztreonam Lysinate for Inhalation in Cystic Fibrosis Patients with Mild Lung Disease and P. aeruginosa (AIR-CF4) This study looked at the safety and use of a 28-day treatment with an inhaled antibiotic, aztreonam in CF patients with mild lung disease and positive Pseudomonas aeruginosa culture. Study Drugs: Cayston Study Ages: >= 6 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: >= 75 Percent Predicted Trial Results Exist

54.	Effect of Simvastatin on CF Airway Inflammation This study examined the safety and efficacy of simvastatin in decreasing inflammation of the respiratory airways in patients with CF. Study Drugs: Simvastatin Study Ages: >= 10 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 60 Percent Predicted Trial Results Exist

55.	PharmaNAC Phase 2b: N-Acetylcysteine Effects in CF This study will look at the effects of an investigational product called N-acetylcysteine (NAC) on the basic processes that cause inflammation in CF lung disease. Study Drugs: N-Acetylcystiene (Oral) Study Ages: >= 7 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 40 - 85 Percent Predicted Trial Results Not Yet Available

56.	Safety Study of KB001 in People with CF and Pseudomonas aeruginosa This study looked at what a single-dose of an investigational drug, KB001, does to Pseudomonas aeruginosa in the CF lung. Study Drugs: KB001A Study Ages: >= 12 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: >= 40 Percent Predicted Trial Results Exist

57.	Single Dose Safety Study of SB-656933 in People with CF This study will look at the safety and pharmacokinetics of one dose of SB-656933 in people with CF. Study Drugs: SB-656933 Study Ages: >= 16 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

58.	Multiple Dose Safety Study of SB-656933 in People with Cystic Fibrosis The purpose of this study was to determine the safety, tolerability and pharmacodynamics of SB656933 in patients that have cystic fibrosis. Study Drugs: SB-656933 Study Ages: >= 18 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: 40 - 110 Percent Predicted Trial Results Exist

59.	Infant Formula Fortified with DHA The purpose of this study is to see if feeding infants diagnosed with CF through newborn screening with a formula fortified with DHA can slow damage from CF. Study Drugs: Docosahexaenoic Acid (DHA) Study Ages: 1 Days - 56 Days Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

60.	Vertex 770 - a phase 2 trial evaluating the effect of VX- 770 on Lung Clearance Index (VX 770-106) This trial will study the effect of VX-770 on lung clearance index in people with CF ages 6 years and older with the G551D mutation and an FEV1 >90%. Study Drugs: Kalydeco Study Ages: >= 6 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 90 Percent Predicted Trial Results Not Yet Available

61.	VX-770 in People with CF and G551D Mutation - Open Label Safety Study (PERSIST) This study will look at the long-term safety of VX-770 in people with CF and the G551D mutation. Subjects will receive VX-770 for 96 weeks during this study. Study Drugs: Kalydeco Study Ages: >= 6 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

62.	Safety Study of VX 809 in People with CF and Homozygous for Delta F508 Gene Mutation The purpose of this study was to evaluate the safety, tolerability, pharmacokinetics, and pharmacodynamics of VX-809 in subjects with cystic fibrosis who have the delta F508-CFTR gene mutation. Study Drugs: VX-809 Study Ages: >= 18 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Exist

63.	Vertex-770 in People with CF with Homozygous Delta F508 Mutation (DISCOVER) This study looked at the safety and effectiveness of VX-770 in people with Cystic Fibrosis (CF) age 12 years and older, with homozygous Delta F508 mutation. Study Drugs: Kalydeco Study Ages: >= 12 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Exist

64.	Vertex 770 in Children with CF and the G551 Mutation (ENVISION) This study looked at the safety and effectiveness of VX-770 in people with CF aged 6 to 11 years with the G551D mutation. Study Drugs: Kalydeco Study Ages: 6 Years - 11 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 40 - 90 Percent Predicted Trial Results Exist

65.	Vertex-770 and the G551D mutation (STRIVE) This study looked at the effectiveness of VX-770 in people with CF and the G551D mutation by evaluating an absolute change from baseline in % predicted FEV1 through week 24 and safety over a longer treatment period than previously studied. Study Drugs: Kalydeco Study Ages: >= 12 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 40 - 90 Percent Predicted Trial Results Exist

66.	VX-770 Phase 2 study in People with CF and Genotype 551D This Phase 2 clinical trial looked at the safety and tolerability of VX-770 in people with CF and the genotype G551D. Study Drugs: Kalydeco Study Ages: >= 18 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Exist

67.	Ataluren (PTC 124) in Cystic Fibrosis This study was a Phase 3 trial that evaluated the clinical benefit of Ataluren (PTC 124) in adult and pediatric patients with CF due to a nonsense mutation. Study Drugs: PTC124 Study Ages: >= 6 Years Type of Therapy: CFTR Modulation Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 40 - 90 Percent Predicted Trial Results Exist

68.	Effect of PulmozymeÂ® Withdrawal on Exercise Tolerance in People with Cystic Fibrosis This study looks at the ability to do exercise, pulmonary function tests and other effects in people with CF when PulmozymeÂ® is withdrawn for 2 weeks. Study Drugs: Pulmozyme Study Ages: >= 14 Years Type of Therapy: Mucus Alteration Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

69.	Pulmozyme in 3-5 Year Old Children with CF This study looked at the effect of Pulmozyme on preschool aged (3-5 year old) children. Study Drugs: Pulmozyme Study Ages: 3 Years - 5 Years Type of Therapy: Mucus Alteration Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

70.	Study of delayed released pancrelipase capsules in people with CF and pancreatic insufficiency This study will assess the effect of pancrelipase delayed release 24,000 unit capsules on fat and nitrogen absorption in people with Cystic Fibrosis and pancreatic insufficiency. Study Drugs: Creon Study Ages: >= 12 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

71.	Study of Pancrease MT Capsules in People with CF and Pancreatic Insufficiency The purpose of this study was to assess the effectiveness and safety of oral pancrelipase MT in the treatment of adult and pediatric/adolescent cystic fibrosis (CF) patients with clinical symptoms of exocrine pancreatic insufficiency (EPI). Study Drugs: Pancrease MT Study Ages: 7 Years - 60 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

72.	Pancrecarb MS-16 in children and adults with CF The purpose of this study was to determine if PANCRECARBÂ® MS-16 (pancrelipase) was safe and effective in reducing steatorrhea in children and adults with cystic fibrosis and pancreatic insufficiency Study Drugs: Pancrecarb MS-16 Study Ages: >= 7 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

73.	Efficacy and Safety of Pancrelipase Delayed Release Capsules in Young Children with CF This study will look at the effect a new formulation of an enzyme replacement therapy will have on fat absorption in young children with CF and pancreatic insufficiency. Study Drugs: Creon Study Ages: 1 Months - 6 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

74.	Safety and Effectiveness of the Eurand Pancreatic Enzyme Supplement in People with CF In this study, researchers examined the effect of a pancreatic enzyme drug on fat absorption. Study Drugs: PEP Microtabs Study Ages: >= 7 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

75.	Safety and Efficacy of Eurand PEP Microtabs in Pediatric Patients with CF Pancreatic Insufficiency This open-label study evaluated the safety and efficacy of Eurand pancreatic enzyme product (PEP) microtabs in pediatric patients under age 7 with Cystic Fibrosis and Exocrine Pancreatic Insufficiency. Study Drugs: PEP Microtabs Study Ages: 1 Days - 6 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

76.	Use of Utrase MT 12 Enzymes in Young Children with CF Related Pancreatic Insufficiency This study will look at how effective Ultrase MT 12 can minimize excess fat in the bowel movements of children with CF ages 2- 6 years Study Drugs: Ultrase MT12 Study Ages: 2 Years - 6 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

77.	Phase 3 Study of the Safety and Effectiveness of ULTRASE MT20 for Pancreatic Insufficiency This study will assess the safety and efficacy of a pancreatic enzyme capsule designed for the absorption of nutrients in patients with CF. Study Drugs: Ultrase MT20 Study Ages: >= 7 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

78.	Efficacy and Safety of Ultrase MT20 in Children with CF and Pancreatic Insufficiency This study is being conducted to demonstrate the efficacy and safety of ULTRASE MT20 in children with CF and pancreatic insufficiency, following the new FDA guidelines. Study Drugs: Ultrase MT20 Study Ages: 7 Years - 11 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

79.	ALTU-135 for the Treatment of CF Exocrine Pancreatic Insufficiency Researchers evaluated people with CF taking ALTU-135, an oral enzyme replacement therapy, for safety concerns during a period of one year. Study Drugs: Liprotamase Study Ages: >= 7 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

80.	Efficacy and Safety of ALTU-135 in Patients with CF Related Exocrine Pancreatic Insufficiency (DIGEST E) This study examined if people with CF taking ALTU-135, an oral enzyme replacement therapy, would achieve statistically significant improvements in the absorption of fat when compared to people not taking the enzyme. Study Drugs: Liprotamase Study Ages: >= 7 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

81.	Effect of Tiotropium Bromide (Spiriva) in People with CF This study looked at the effects of tiotropium bromide inhaled once a day using the Respimat device. Study Drugs: Tiotropium Bromide Study Ages: >= 5 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 25 Percent Predicted Trial Results Exist

82.	Effect of Proton Pump Inhibitors on CF Exacerbations This study will determine whether treatment of GERD affects frequency of respiratory exacerbations in CF. Study Drugs: Esomeprazole Magnesium Study Ages: >= 18 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

83.	Effect of Insulin or Repaglinide on Body Weight and Pulmonary Function in Patients with CF Related Diabetes The study is being conducted to determine whether treatment with either insulin or Repaglinide (a medication that increases insulin levels) will improve healthy body weight and pulmonary function in cystic fibrosis patients who have CF-related diabetes. Study Drugs: Repaglinide Study Ages: >= 16 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

84.	Denufosol in People with CF ages 2 - 4 years The purpose of this study was to look at the safety and tolerability of denufosol aerosolized three times a day in pediatric CF patients 2 to 4 years of age. Study Drugs: Denufosol Study Ages: 2 Years - 4 Years Type of Therapy: Restore Airway Surface Liquid Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 1 Percent Predicted Trial Results Exist

85.	Denufosol Inhalation Solution in People With Mild CF Lung Disease (TIGER 2) The purpose of this trial was to evaluate the safety and effectiveness of inhaled Denufosol designed to enhance the hydration and clearance of mucus in the lungs of people with CF. Study Drugs: Denufosol Study Ages: >= 5 Years Type of Therapy: Restore Airway Surface Liquid Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: 75 - 110 Percent Predicted Trial Results Exist

86.	Denufosol (TIGER 2) Open Label Follow On This open-label follow on study to TIGER 2 will be evaluating the long term safety of Denufosol in patients with CF. Only enrolling participants by invitation. Study Drugs: Denufosol Study Ages: >= 5 Years Type of Therapy: Restore Airway Surface Liquid Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

87.	Denufosol Inhalation Solution for People with CF and Mild Lung Disease (TIGER 1) The purpose of this trial was to evaluate the safety and effectiveness of inhaled Denufosol designed to enhance the hydration and clearance of mucus in the lungs of CF patients. Study Drugs: Denufosol Study Ages: 5 Years - 999 Years Type of Therapy: Restore Airway Surface Liquid Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 75 Percent Predicted Trial Results Exist

88.	Inhaled Dry Powder Mannitol in Cystic Fibrosis This study looked at how safe and how well treatment with inhaled dry powder mannitol worked to clear mucus in the lungs and improve lung function of people with cystic fibrosis. Study Drugs: Bronchitol Study Ages: >= 6 Years Type of Therapy: Restore Airway Surface Liquid Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 30 - 89 Percent Predicted Trial Results Exist

89.	Infant Study of Inhaled Saline (ISIS) The purpose of this study was to determine whether 7% hypertonic saline (HS) was an effective and safe therapy in infants with CF. Note this study included infants 4 months to 59 months at the enrollment visit. If participated in the Infant Pulmonary Function testing (selected sites), age 4 months to < 16 months at Enrollment visit. Study Drugs: Hypertonic Saline Study Ages: 4 Months - 5 Years Type of Therapy: Restore Airway Surface Liquid Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

90.	Pilot Safety Study of Inhaled 7% Hypertonic Saline in Infants with CF This safety study evaluated the tolerability of hypertonic saline given to infants for up to 14 days. Study Drugs: Hypertonic Saline Study Ages: 12 Months - 30 Months Type of Therapy: Restore Airway Surface Liquid Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

91.	Vestibular and Auditory Sensory Loss in People with CF This study will include patients with CF who are starting a course of IV Tobramycin for a lung infection in order to test inner ear function during the treatment. Study Drugs: Tobramycin (IV) Study Ages: >= 10 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

92.	Treatment of early Pseudomonas Aeruginosa This study will look at the effects of treatment with intravenous antibiotics compared to inhaled tobramycin in clearing mucoid Pseudomonas aeruginosa and reducing inflammation in the airways. Study Drugs: TOBI Study Ages: 6 Months - 16 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

93.	Early Intervention in Pulmonary Exacerbation in Cystic Fibrosis This study will compare routine care for a pulmonary exacerbation to a program of early identification and treatment of pulmonary exacerbation in adults with CF. Study Drugs: Multiple systemic antibiotics Study Ages: >= 18 Years Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: >= 25 Percent Predicted Trial Results Not Yet Available

94.	The Effect of Azithromycin on TGF-b1 This study will examine if TGF-b1, a gene responsible for regulation of immune function and tissue repair, is reduced when taking Azithromycin. Study Drugs: Azithromycin Study Ages: >= 8 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

95.	Sildenafil in CF lung disease This single center trial will look at the effect of Sildenafil (RevatioÂ®), a drug already used in other types of lung disease, on symptoms related to lung inflammation and infection in people with CF who are 12 years of age and older. Study Drugs: Sildenafil Study Ages: >= 12 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

96.	Pioglitizone for the Treatment of CF Lung Disease Researchers studied whether a drug called pioglitazone could decrease inflammation in cystic fibrosis lung disease. Study Drugs: Pioglitazone Study Ages: >= 18 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Exist

97.	Low-Dose Methotrexate for the Treatment of CF Lung Disease Researchers studied whether a drug called methotrexate could decrease iflammation in cystic fibrosis lung disease. Study Drugs: Methotrexate Study Ages: >= 14 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Exist

98.	Hydroxychloroquine for the Treatment of CF Lung Disease Researchers studied whether a drug called hydroxychloroquine could decrease inflammation in cystic fibrosis lung disease. Study Drugs: Hydroxychloroquine Study Ages: 16 Years - 65 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Exist

99.	Effect of Sulforaphane in Broccoli Sprouts on Nrf2 activation This single center study looked at whether sulforaphane released from well chewed broccoli sprouts activates Nrf2 in the cells of people with CF who are between the ages of 18 to 49 years. Study Ages: 18 Years - 49 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

100.	Alendronate in adults with CF related osteoporosis This study will look at the bioavailability and biologic effect of alendronate on bone metabolism in people with cystic fibrosis and assess the safety and effectiveness of the study treatment regimen in improving osteoporosis in people with CF. Study Ages: >= 18 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: 20 - 90 Percent Predicted Trial Results Not Yet Available

101.	Pilot Study of Zinc Supplementation in Children with CF This study will examine the level of zinc absorption, excretion, and balance in children with cystic fibrosis. Researchers would like to find out if children with CF who are given zinc supplements will have a higher zinc balance than CF children who do not take these supplements. Study Drugs: Zinc Study Ages: 8 Years - 14 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

102.	Surfactant Aerosol Pilot Study This study will looked at whether inhaled aerosol drugs used to treat CF might work better if special chemicals called surfactants, were added to them. Study Ages: >= 18 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 65 Percent Predicted Trial Results Exist

103.	Surfactant and Hypertonic Saline This study will look at the ability of inhaled surfactant to lessen the obstruction of CF airways and to improve mucus clearance. The study will also compare the effect of surfactant treatment to surfactant plus hypertonic saline treatment. Study Drugs: Hypertonic Saline Study Ages: >= 14 Years Type of Therapy: Restore Airway Surface Liquid Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

104.	Hypertonic Saline in Infants and Young Children with Cystic Fibrosis (Pilot) This single center pilot study assessed the safety of Hypertonic Saline (HS) infants and young children with CF. Study Drugs: Hypertonic Saline Study Ages: 1 Months - 8 Years Type of Therapy: Restore Airway Surface Liquid Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 60 Percent Predicted Trial Results Exist

Observational

Recruitment Status: Recruiting

1.	Fibrosing Colonopathy in Cystic Fibrosis This is a long-term study to assess the occurrence and risk factors for a rare bowel disorder called fibrosing colonopathy (narrowing of the large intestine) in people with cystic fibrosis who take pancreatic enzymes and are participating in the Cystic Fibrosis Patient Registry. Study Ages: >= 1 Days Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

2.	BONUS - Baby Observational and Nutrition Study A multi-center observational study following the growth of infants with CF for a duration of 12 months. Data such as length, weight, blood, urine will be collected at routine clinical care visits. This study will help gain a better understanding of the factors that interfere with good growth in infants with CF. Study Ages: 1 Days - 15 Weeks Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

3.	Sweat Testing in CF Newborns The purpose of this study is to evaluate the sweat testing results of newborns and their fluid and salt balance. This study is being conducted because it is known that fluid and salt balance may affect sweat testing but this has not been fully determined in babies. Study Ages: 2 Weeks - 16 Weeks Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

4.	Prediction by Ultrasound of the Risk of Hepatic Cirrhosis in Cystic Fibrosis This study will look at whether using abdominal ultrasounds predicts the progression of liver disease in children with CF. Study Ages: 3 Years - 12 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

5.	Genetic Modifiers of Cystic Fibrosis: Sibling Study The purpose of the study is to find genetic and environmental factors affecting the variability in CF. Study Ages: >= 1 Days Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

6.	Genetic Modifiers of Cystic Fibrosis Liver Disease This study examines â??modifier genesâ?? that may play a role in the development of CF liver disease. Study Ages: >= 1 Days Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

7.	Impaired Glucose Tolerance in People with Cystic Fibrosis This study will look at identifying the frequency of diagnosing cystic fibrosis related diabetes (CFRD)and impaired glucose tolerance (IGT) using oral glucose tolerance test screening (OGTT). Study Ages: 8 Years - 25 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

8.	Lung Disease in Infants with CF This study will seek to identify what factors impact lung function in infants with CF. Study Ages: 1 Years - 3 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

9.	A Novel Test to Measure Response to Treatment of a CF Pulmonary Exacerbation To see whether a novel blood test measuring white blood cell proteins can measure a change in inflammation of lungs after therapy for an exacerbation. Study Ages: 18 Years - 999 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: 1 - 99 Percent Predicted Trial Results Not Yet Available

Recruitment Status: Recruiting Completed

10.	Study to Monitor the Susceptibility to Aztreonam of Pseudomonas Aeruginosa in People with CF This is a 5-year study that will enroll 500 subjects from the existing Cystic Fibrosis Foundation (CFF) patient registry. Eligible subjects will be patients who receive care at CFF-accredited centers nationwide. Each enrolled subject will provide samples for microbiological evaluation, obtained upon enrollment and then once per year thereafter for 5 years. Study Ages: >= 1 Years Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: 1 - 90 Percent Predicted Trial Results Not Yet Available

11.	Five Year Extension Study to Evaluate Risk Factors for and Inpact of P. aeruginosa Acquisition This study is a five year extension study to continue to evaluate the impact of P. aeruginosa acquistion on clinical outcomes in Cystic Fibrosis. Enrollment is limited to patients who completed the original study. Study Ages: 1 Days - 12 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

12.	iCARE study (I Change Adherence and Raise Expectations Study) The iCARE study is learning which tools and strategies help cystic fibrosis (CF) care teams communicate better with their patients and improve their patientsâ?? CF management. Study Ages: 11 Years - 20 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

13.	Biomarkers of Lung Injury in CF Children The purpose of the study is to learn if a urine and sputum test can be used to measure if lung damage occurs over a long period of time (two years) or when patients are sick. Study Ages: 8 Years - 25 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 35 Percent Predicted Trial Results Not Yet Available

Recruitment Status: Trial Completed

14.	Observational Study in People with CF with the G551D mutation This is a multi-center observational trial collecting clinical information such as height and weight as well as specimens such as blood and urine in people with CF who have the G551D mutation. Study Ages: >= 6 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

15.	Standardization of Mucociliary Clearance Methodology Comparing the measurement techniques for mucociliary clearance across 3 CF care centers. Study Ages: 18 Years - 40 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 50 Percent Predicted Trial Results Not Yet Available

16.	Assessing Quality of Life in CF After Lung Transplant This study will compare the quality of life perceptions in children with CF who receive a lung transplant versus those children with CF who are not lung transplant candidates. Study Ages: >= 6 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: Trial Results Not Yet Available

17.	Characterization of MRSA infection/colonization in CF This study seeks to define if sub-types of MRSA differ in regards to effect on exacerbations, lung function and nutrition. The study also tests which antibiotics would be most useful for treating MRSA and if children and their parents would need to also have disinfection of nose and skin to avoid re-infection. Study Ages: 1 Years - 18 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

18.	Longitudinal Assessment of Risk Factors for and Impact of Pseudomonas aeruginosa and Early Anti-Pseudomonal Treatment in Children with CF This observational study was to learn what may lead to lung infections caused by Pseudomonas aeruginosa (Pa) in children with CF. Study Ages: 1 Days - 12 Years Pseudomonas Status: Negative B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

19.	Detection of Airway CFTR Develop new biomarkers for the detection of CFTR activity in the airway of CF patients. Study Ages: >= 8 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

20.	Depression and Anxiety in CF Patients and Caregivers This study will look at depression and anxiety in patients with CF and parent caregivers. Depression and anxiety may have an effect on health outcomes such as change in FEV1, BMI, and number of exacerbations and hospitalizations. Study Ages: >= 1 Days Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

21.	Antimicrobial Resistance in CF This study enrolled people with CF and compared their sputum microbiology and susceptibility data with data obtained years ago from tobramycin clinical trials. The comparisons between current and previous infections help scientists to determine if use of certain antibiotics has contributed to antibiotic resistance. Study Ages: >= 6 Years Pseudomonas Status: Positive B Cepacia Status: Negative FEV1% Status: 25 - 75 Percent Predicted Trial Results Exist

22.	Management of Bacterial Air Contamination in CF Clinics The main purpose of this study is to see whether mask use by CF patients during office visits can reduce contamination of examination room air. Study Ages: >= 6 Years Type of Therapy: Other Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

23.	Transition Readiness Assessment in CF This study will compare two health care transition questionnaires to gain knowledge about factors to consider when determining transfer of care from a pediatric to adult CF care program. Study Ages: 14 Years - 22 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

24.	Infants Exhaled Breath Condensate This study sought to determine if the use of the non-invasive technique, exhaled breath condensate (EBC), could detect airway inflammation in infants lungs. Study Ages: 5 Months - 35 Months Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Exist

25.	Immune Responses and Pulmonary Phenotype This study will look at variation in specific immune responses among individuals with identical CFTR genotypes. Study Ages: >= 18 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

26.	SCN Secretion in CF This study will seek to identify a defect in the airway fluid of patients with CF. Study Ages: >= 18 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 70 Percent Predicted Trial Results Not Yet Available

27.	Pseudomonas Aeruginosa Infections by ELISA Testing This study will look at developing a test to detect antibodies against Pseudomonas aeruginosa in the saliva of young children with CF. Study Ages: 1 Months - 16 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

28.	Neutrophils in CF Exacerbation This study will look at changes in neutrophils during a CF exacerbation. Study Ages: >= 7 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

29.	Inflammation and Oxidant Stress in Cystic Fibrosis Related Diabetes This study will look at oxidant and inflammatory effects in the development of Cystic Fibrosis Related Diabetes. Study Ages: >= 18 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

30.	Early Staphylcoccal and Other Bacterial Infections in CF This study will look at what bacteria in the airway of young children grows from oropharyngeal swab samples. It will also look to see if S. Aureus, P. aeruginosa and anaerobic bacteria effect airway disease progression. Study Ages: 1 Days - 4 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

31.	Biomarkers in Breath Condensate in Children with CF This study will look at exhaled breath condensate as a measurement of airway inflammatory biomarkers. Study Ages: 6 Years - 21 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

32.	Anaerobic Bacteria in CF The goal of the project is to understand how each germ found in the lungs of CF patients is involved in disease and the best way to treat lung infections in CF. Study Ages: >= 6 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

International Only

1.	Azithromycin Infant Study This study will look at the safety and effectiveness of 3 years of treatment with azithromycin compared to placebo in infants with CF. Study Drugs: Azithromycin Study Ages: 3 Months - Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: Trial Results Not Yet Available

2.	Probiotic Use in People with Cystic Fibrosis This study will look at the effect of probiotic use for 1 year in children with CF. Study Ages: 2 Years - 16 Years Type of Therapy: Nutritional-GI Therapies Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: >= 50 Percent Predicted Trial Results Not Yet Available

3.	Prevention of Chronic Pseudomonas Infection This study will look at the effectiveness of two different medication treatment plans in preventing chronic pseudomonas aeruginosa lung infections. Study Drugs: Ciprofloxacin (oral) Study Ages: >= 1 Months Type of Therapy: Anti-Infective Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: Trial Results Not Yet Available

4.	Effect of Azithromycin in People with CF and B. Cepacia This study will examine the effectiveness of azithromycin in improving the lung function in people with CF infected with the bacteria Burkholderia cepacia complex. Study Drugs: Azithromycin Study Ages: >= 19 Years Type of Therapy: Anti-Infective Pseudomonas Status: Not applicable B Cepacia Status: Positive FEV1% Status: >= 30 Percent Predicted Trial Results Not Yet Available

5.	Inhaled Glutathione in People with CF This study will look at the tolerability and effect of inhaled gluthathione on FEV1 in people with CF. Study Drugs: Glutathione (Inhaled) Study Ages: >= 8 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Negative FEV1% Status: 40 - 90 Percent Predicted Trial Results Not Yet Available

6.	Glutamine Supplementation for Cystic Fibrosis This study willevaluate the safety and tolerability of aerolized glutathione compared with normal saline. The main outcome measure is pulmonary function testing (PFT) Study Drugs: Glutamine Study Ages: >= 14 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Positive B Cepacia Status: Not applicable FEV1% Status: >= 40 Percent Predicted Trial Results Not Yet Available

7.	Choline Supplementation in Children with CF This research will look to find out if choline supplements over a longer duration of 6 months will improve and maintain normal choline status in children. Study Drugs: Choline Study Ages: 5 Years - 17 Years Type of Therapy: Anti-Inflammatory Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

8.	Early Predictors of Lung Disease This study will look at different factors that affect lung function in infants. Infants will be followed until 4 years of age. Study Ages: 14 Days - 4 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available

9.	Breath Glucose Biomarker This study will find out how useful breath glucose is as a measure of CF lung disease. Study Ages: >= 18 Years Pseudomonas Status: Not applicable B Cepacia Status: Not applicable FEV1% Status: >= 1 Percent Predicted Trial Results Not Yet Available