Pediatric Grand Rounds: Cystic Fibrosis

Dr. Orenstein provides an overview of the history of Cystic Fibrosis. He discusses the drug development process, discontinued drugs, and the effects of the newest drug: Ivacaftor. The financial aspect of the drug is also touched on.

Educational objectives:

Upon completion of this activity, participants should be able to:

• Review our evolving understanding of cystic fibrosis as inherent to tissues (not circulating factors)
• Describe to patients the physiology behind newest CF drug, Ivacaftor
• Discuss financial background of Ivacaftor development

Reading Resources:

1. The ease of breathing test tracks clinical changes in cystic fibrosis. Kazmerski T, Orenstein DM. J Cyst Fibros. 2012 Apr 9
2. Comparative efficacy and safety of 4 randomized regimens to treat early Pseudomonas aeruginosa infection in children with cystic fibrosis. Treggiari MM, Retsch-Bogart G, Mayer-Hamblett N, Khan U, Kulich M, Kronmal R, Williams J, Hiatt P, Gibson RL, Spencer T, Orenstein D, Chatfield BA, Froh DK, Burns JL, Rosenfeld M, Ramsey BW; Early Pseudomonas Infection Control (EPIC) Investigators. Arch Pediatr Adolesc Med. 2011 Sep;165(9):847-56.
3. Osmolytes and ion transport modulators: new strategies for airway surface rehydration. Goralski, Boucher, Button. Current Opinion in Pharmacology 2010, 10:294–299

Disclosures:

Dr. Orenstein has no relevant relationships with any entities producing healthcare goods or services.

Accreditation Statement:

The University of Pittsburgh School of Medicine is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.

The University of Pittsburgh School of Medicine designates this enduring material for a maximum of 1.0 AMA PRA Category 1 CreditsTM. Each physician should only claim credit commensurate with the extent of their participation in the activity. Other health care professionals are awarded (0.1) continuing education units (CEU) which are equivalent to 1.0 contact hours.

For your credit transcript, please access our website 4 weeks post-completion at http://ccehs.upmc.edu and follow the link to the Credit Transcript page. If you do not provide the last 5 digits of your SSN on the next page you will not be able to access a CME credit transcript. Providing your SSN is voluntary.

Release Date: 9/13/2012 | Last Modified On: 3/25/2015 | Expires: 3/25/2016