August 2011

Infection control knowledge, attitudes, and practices among cystic fibrosis patients and their families
Miroballi Y, Garber E, Jia H, Zhou JJ, Alba L, Quittell LM, Angst D, Cabana M, Saiman L, CF Infection Control Study Consortium

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June 2011

Mannitol-guided delivery of ciprofloxacin in artificial cystic fibrosis mucus model
Yang Y, Tsifansky MD, Shin S, Lin Q, Yeo Y.

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April 2011

Challenges and advances in the development of inhalable drug formulations for cystic fibrosis lung disease
Ibrahim BM, Tsifansky MD, Yang Y, Yeo Y.

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August 2010

PGD for all cystic fibrosis carrier couples: novel strategy for preventive medicine and cost analysis
Tur-Kaspa I, Aljadeff G, Rechitsky S, Grotjan HE, Verlinsky Y

Over 1000 children affected with cystic fibrosis (CF) are born annually in the USA. Since in-vitro fertilization (IVF) with preimplantation genetic diagnosis (PGD) is an alternative to raising a sick child or to aborting an affected fetus, a cost-benefit analysis was performed for a national IVF-PGD program for preventing CF. The amount spent to deliver healthy children for all CF carrier-couples by IVF-PGD was compared with the average annual and lifetime direct medical costs per CF patient avoided. Treating annually about 4000 CF carrier-couples with IVF-PGD would result in 3715 deliveries of non-affected children at a cost of $57,467 per baby. Because the average annual direct medical cost per CF patient was $63,127 and life expectancy is 37 years, savings would be $2.3 million per patient and $2.2 billion for all new CF patients annually in lifetime treatment costs. Cumulated net saving of an IVF-PGD program for all carrier-couples for 37 years would be $33.3 billion. A total of 618,714 cumulative years of patients suffering because of CF and thousands of abortions could be prevented. A national IVF-PGD program is a highly cost-effective novel modality of preventive medicine and would avoid most births of individuals affected with debilitating genetic disease.

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September 2008

Barriers to adherence to cystic fibrosis infection control guidelines
Garber E, Desai M, Zhou J, Alba L, Angst D, Cabana M, Saiman L; CF Infection Control Study Consortium

In 2003, the American Cystic Fibrosis (CF) Foundation published revised, evidence-based guidelines for infection control. We sought to assess potential barriers to adherence to these guidelines experienced by health care professionals (HCPs) caring for CF patients. From April 2004 to December 2005, a knowledge, attitude, and practice survey was administered to HCPs at randomly selected CF centers in the United States to explore potential barriers to adherence to selected guidelines. The survey was completed by 528 HCPs from 25 sites (5-50 respondents per site). Only 60% of respondents were aware of the guidelines, but despite awareness, 31-47% were unfamiliar with the specific guidelines. Self-reported adherence was low; only 23-63% of respondents reported practicing the selected guidelines >75% of the time/opportunities. Lack of self-efficacy, that is, confidence in adequately performing the guidelines, was commonly experienced by respondents. Access to a copy of the guidelines was associated with increased agreement with the recommendations and increased self-efficacy. In conclusion, strategies to reduce barriers to adherence to CF infection control guidelines are needed.

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