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108 PharmacyTimes.org December 2015 CURRENT AND EMERGING THERAPEUTICS IN CYSTIC FIBROSIS: ENSURING CONTINUITY OF CARE WITHIN THE NEW TREATMENT PARADIGM 1. What is the most common patho- gen isolated in the respiratory tract of patients with cystic fibro- sis (CF)? a. Pseudomonas aeruginosa b. Methicillin-resistant Staphylococcus aureus (MRSA) c. Escherichia coli d. Haemophilus influenza e. Group B Streptococcus 2. The median age of survival of patients with CF today is approxi- mately: a. 18 years b. 24 years c. 28 years d. 31 years e. 41 years 3. Which agent can be used long term to decrease the inflamma- tion of the airway in CF due to chronic infection? a. Prednisone b. Azithromycin c. Dexamethasone d. Cephalexin e. Dornase alfa 4. Which inhaled antibiotic regimen is often used for chronic sup- pressive therapy of P. aeruginosa infection in patients with CF? a. Daily for 6 months b. Daily for 28 days, then stop therapy for 28 days c. The first 7 days of each month d. Every other day for 28 days, then off for 28 days e. Daily for 1 year, then reevalu- ate 5. Which agent is FDA-approved for patients with CF who are homo- zygous for the most common mutation, F508del? a. Lumacaftor b. Ataluren c. Lumacaftor/ivacaftor d. Ivacaftor e. There is no FDA-approved agent 6. The incidence of pulmonary infec- tion with which microorganism has increased in patients with CF over the past 2 decades? a. P. aeruginosa b. MRSA c. E. coli d. H. influenzae e. Group B Streptococcus 7. Class I mutations may eventually be treated by an agent called an activator. Which agent falls into this category? a. Ivacaftor b. Lumacaftor c. VX-661 d. Ataluren e. Ivacaftor/lumacaftor 8. Liam, a 3-year-old, 15-kg boy with CF, is being seen at his rou- tine medical visit. His CF muta- tions are G551D and F508del, as detected on his newborn state screen. His mother inquires about new treatments for patients with CF and certain genetic mutations. What drug and dose would Liam qualify for at this time? a. Lumacaftor/ivacaftor 200/125- mg tablets b. Ivacaftor 150-mg tablet c. Ivacaftor 50-mg oral granules d. Ivacaftor 75-mg oral granules e. He is not old enough for the current therapy 9. Kristin is a 16-year-old female being seen in clinic for her base- line visit before starting luma- caftor/ivacaftor therapy. The pharmacist reviews all of her cur- rent therapies. Kristin's current CF care includes albuterol as needed, hypertonic saline 7% twice daily, dornase alfa once daily, TOBI Podhaler on/off cycles, long-term azithromycin, Creon 24 with meals/snacks, and AquADEK. When asked if she is taking any additional medications, she men- tions she takes Ortho Tri-Cyclen Lo, an oral hormonal contracep- tive. Among her treatments listed, are there possible drug interactions with lumacaftor/iva- caftor about which Kristin should be counseled? a. Yes, drug interaction with azithromycin b. Yes, drug interaction with dor- nase alfa c. No drug interactions present d. Yes, drug interaction with Ortho Tri-Cyclen Lo e. Yes, drug interaction with Creon 24 10. Which therapy listed below showed a change in baseline predicted FEV 1 of 10.6%? a. Lumacaftor in homozygous F508del b. Ivacaftor in homozygous F508del c. Lumacaftor/ivacaftor in homozygous F508del d. Ataluren in Class I mutations e. Ivacaftor in G551D This is a i o s a ( M R ) a . b . c . e r t h P . a M R E . c r u g i n o s A o l i e s ? a z i t h . Y e s , d n a s e a N sample of f s u r v i v a o d a y i s a l o f p p r o x i - b e t r e a t e a c t i v a t o r . h i s c a t e g b y a n a g W h i h a g o r y ? o r a y e e n t e n t a l l e d a n a l l s i n t o C r e o n W h i c h t i C y r u g i n n 2 4 h e r a the online e u s e d l o n g e . 8 L i n f t o r / l u m a y e a r - o l c a f t o r 1 5 - k g b o y F b . I v F 5 L u d e l a c a f t o r i n 0 8 d e l a c a f t posttest. s n e t e c t e d c r e e n . H e w t r e a t d 5 1 o n h i s m o h m e n t s i n d n e w b o e r i n f o r p a i r n s t a t u i r e s a b o t i e n t s w t u t t h e . I v INSTRUCTIONS FOR EARNING CREDIT • Begin the activity by reading the content in its entirety. • Go to PharmacyTimes.org to access the posttest and activity evaluation form. • Complete the electronic posttest and activity evaluation form. • Once completed, click on "submit posttest." Your posttest will auto- matically be graded. • If you successfully complete the posttest (score of 70% or higher) and the evaluation form, this information will be uploaded into CPE Monitor. • You must complete these steps before the activity expires in order to receive your credit. • You may view your credit within 48 hours at www.mycpemonitor. net.