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A Review on the Use of Cystic Fibrosis Transmembrane Conductance Regulator Gene Modulators in Pediatric Patients

      INSTRUCTIONS
      To obtain continuing education credit:
      • 1
        Read the article carefully.
      • 2
        Read each question and determine the correct answer.
      • 3
        Visit PedsCESM, ce.napnap.org, to complete the online Posttest and evaluation.
      • 4
        You must receive 70% correct responses to receive the certificate.
      • 5
        Tests will be accepted until June 30, 2020.
      OBJECTIVES
      • 1
        Describe the six major classes of mutation of the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
      • 2
        Review the mechanisms of action of ivacaftor, lumacaftor/ivacaftor, and tezacaftor/ivacaftor.
      • 3
        Compare the indications and efficacy among the three CFTR modifying drugs.
      • 4
        Identify the counseling points for the three CFTR modifying drugs.
      Contact hours: 1.0
      Passing score: 70%
      This continuing education activity is administered by the National Association of Pediatric Nurse Practitioners (NAPNAP) as an Agency providing continuing education credit. Individuals who complete this program and earn a 70% or higher score on the Posttest will be awarded 1.0 contact hour of which 1.0 is pharmacology content.
      Earn FREE CE Contact Hours Online.
      Contact Hours for this online activity are FREE for NAPNAP Members. Non-Members will be charged a fee of $10 to receive contact hours for this online activity through PedsCESM. Payment can be made by credit card through PedsCESM.
      • 1
        To take the Posttest for this article and earn contact hours, please go to PedsCESM at ce.napnap.org.
      • 2
        In the Course Catalog, search for the name of the CE article.
      • 3
        If you already have an account with PedsCESM, login using your username and password. If you are a NAPNAP member, log in with your username and password. If you are a first-time user and NAPNAP non-member, click on ‘‘New Customer? Click Here.’’
      • 4
        Once you have successfully passed the Posttest and completed the evaluation form, you will be able to print out your certificate immediately.
      CE test Questions
      • 1
        What is true regarding the CFTR protein?
      • A
        It is normally present within the basolateral surface of the plasma membrane
      • B
        It regulates the production of exocrine fluids
      • C
        It allows transport of chloride ions across the cellular membrane
      • D
        The gene that encodes the CFTR protein is found on chromosome 17
      • 2
        Which of the following characterizes a class I mutation?
      • A
        CFTR protein is not synthesized
      • B
        CFTR protein is misfolded
      • C
        CFTR protein is rapidly degraded
      • D
        CFTR protein cannot open properly
      • 3
        What is the most common genetic mutation found in cystic fibrosis patients?
      • A
        I507del
      • B
        G542X
      • C
        F508del
      • D
        G551D
      • 4
        Which of the following is routinely utilized as supportive care for cystic fibrosis?
      • A
        Chest physiotherapy
      • B
        Inhaled sodium chloride 0.9%
      • C
        Ipratropium
      • D
        Oral corticosteroids
      • 5
        In addition to improvement in ppFEV1, lumacaftor/ivacaftor and tezacaftor/ivacaftor have been proven to significantly improve which of the following?
      • A
        Tidal volume
      • B
        Pulmonary exacerbations
      • C
        Mortality
      • D
        Forced vital capacity
      • 6
        Which of the following statements is true regarding tezacaftor compared to lumacaftor?
      • A
        Tezacaftor treats a lower number of CFTR gene mutations
      • B
        Tezacaftor has a higher incidence of respiratory symptoms
      • C
        Tezacaftor provides better improvement in ppFEV1
      • D
        Tezacaftor dosing can be affected by concomitant moderate CYP3A4 inhibitors
      • 7
        How much improvement in ppFEV1 has been observed in Phase-2 clinical trials for the next generation of CFTR correctors?
      • A
        5-6%
      • B
        8-9%
      • C
        13-14%
      • D
        20-22%
      • 8
        Which of the following is true regarding the use of ivacaftor?
      • A
        It should be taken on an empty stomach
      • B
        It is approved for use in children older than 6 months of age
      • C
        Dose adjustments are required for strong CYP2C19 inhibitors
      • D
        Common adverse events include nasopharyngitis, upper respiratory tract infections, and headache
      • 9
        Which of the following is an appropriate counseling point in patients taking CFTR targeted therapy?
      • A
        Routine eye exams are only necessary if the patient has a history of cataracts
      • B
        These medications are administered once daily
      • C
        These medication should be administered with a high-fat containing meal
      • D
        The most common side effect of these medications is renal dysfunction
      • 10
        KS is a 3-year-old female with CF found to have a G551D mutation in the CFTR gene. Which of the following is an appropriate treatment option for KS?
      • A
        Ivacaftor
      • B
        Lumacaftor/Ivacaftor
      • C
        Tezacaftor/Ivacaftor
      • D
        KS does not qualify for treatment with a CFTR targetedtherapy