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Please use the below scale to rate your current level of confidence in your ability to: *

1=Not at all confident, 5=Extremely confident, N/A=Not applicable for me

	1	2	3	4	5	N/A
Understand the principles of precision medicine when managing patients with cystic fibrosis.	Understand the principles of precision medicine when managing patients with cystic fibrosis. - 1	Understand the principles of precision medicine when managing patients with cystic fibrosis. - 2	Understand the principles of precision medicine when managing patients with cystic fibrosis. - 3	Understand the principles of precision medicine when managing patients with cystic fibrosis. - 4	Understand the principles of precision medicine when managing patients with cystic fibrosis. - 5	Understand the principles of precision medicine when managing patients with cystic fibrosis. - N/A
Identify predictors of disease progression in patients with cystic fibrosis.	Identify predictors of disease progression in patients with cystic fibrosis. - 1	Identify predictors of disease progression in patients with cystic fibrosis. - 2	Identify predictors of disease progression in patients with cystic fibrosis. - 3	Identify predictors of disease progression in patients with cystic fibrosis. - 4	Identify predictors of disease progression in patients with cystic fibrosis. - 5	Identify predictors of disease progression in patients with cystic fibrosis. - N/A

The GOAL study demonstrated significant clinical and physiologic improvements, including reduced infection with P. aeruginosa in patients age ≥ 6 years-old treated with ivacaftor. The patients in this study harbored which of the following mutations? *

a. G551D mutation

b. R117H mutations

c. F508del-CFTR mutation

d. All mutations

Which of the following are not key indicators of cystic fibrosis regarding its pathophysiology? *

ASL dehydration

Hyperviscous mucus

Pulmonary inflammation

Excessive bowel gas

The PERSIST trial was a 96-week continuation study from the STRIVE and ENVISION trials. Which of the following represents findings at 144 weeks? *

Ataluren was well tolerated with no safety concerns

Lumacaftor was well tolerated with no safety concerns

Ivacaftor was well tolerated with no safety concerns

Tobramycin + ataluren was well tolerated with no safety concerns

Which of the following manifestations of cystic fibrosis would be a reversible organ-specific CFTR protein dysfunction? *

Salt loss

Insulin resistance

Mucus plugging

Cirrhosis

Like any chronic disease, early intervention is key for the improvement of patient outcomes. Newborn screening has proven to have established benefits for infants with suspected cystic fibrosis. *

True

False

What are physicians trying to achieve with modern cystic fibrosis management? *

Maintain pulmonary health

Limit burden of treatment

Ensure normal growth, development and nutrition

All of the above

Patients who were treated with ataluren in addition to existing tobramycin therapy show significant improvements in FEV1. *

True

False

Using the scale below, please indicate how often you use each of the following practice strategies for patients with cystic fibrosis. *

Use scale: 1=Never, 2=Not often, 3=Sometimes, 4=Often, 5=Always, N/A= Not applicable to me

	1	2	3	4	5	N/A
Utilize precision medicine as it pertains to CFTR modulation therapeutics	Utilize precision medicine as it pertains to CFTR modulation therapeutics - 1	Utilize precision medicine as it pertains to CFTR modulation therapeutics - 2	Utilize precision medicine as it pertains to CFTR modulation therapeutics - 3	Utilize precision medicine as it pertains to CFTR modulation therapeutics - 4	Utilize precision medicine as it pertains to CFTR modulation therapeutics - 5	Utilize precision medicine as it pertains to CFTR modulation therapeutics - N/A
Manage cystic fibrosis patients appropriately to specific gene mutations	Manage cystic fibrosis patients appropriately to specific gene mutations - 1	Manage cystic fibrosis patients appropriately to specific gene mutations - 2	Manage cystic fibrosis patients appropriately to specific gene mutations - 3	Manage cystic fibrosis patients appropriately to specific gene mutations - 4	Manage cystic fibrosis patients appropriately to specific gene mutations - 5	Manage cystic fibrosis patients appropriately to specific gene mutations - N/A
Monitor the impact CFTR modulators have on the gastrointestinal system	Monitor the impact CFTR modulators have on the gastrointestinal system - 1	Monitor the impact CFTR modulators have on the gastrointestinal system - 2	Monitor the impact CFTR modulators have on the gastrointestinal system - 3	Monitor the impact CFTR modulators have on the gastrointestinal system - 4	Monitor the impact CFTR modulators have on the gastrointestinal system - 5	Monitor the impact CFTR modulators have on the gastrointestinal system - N/A
Perform assessments in addition to FEV1 measurements to ensure accurate diagnosis	Perform assessments in addition to FEV1 measurements to ensure accurate diagnosis - 1	Perform assessments in addition to FEV1 measurements to ensure accurate diagnosis - 2	Perform assessments in addition to FEV1 measurements to ensure accurate diagnosis - 3	Perform assessments in addition to FEV1 measurements to ensure accurate diagnosis - 4	Perform assessments in addition to FEV1 measurements to ensure accurate diagnosis - 5	Perform assessments in addition to FEV1 measurements to ensure accurate diagnosis - N/A

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Elsevier CME

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