AHEAD OF THE CURVE: Cystic Fibrosis

In 2002, the CF Foundation convened a consensus conference. This consensus conference developed specific recommendations for the prevention of bone disease and the optimal supplementation of vitamin D for patients with cystic fibrosis. The original consensus guidelines did not have any actual demonstration in cystic fibrosis patients, and most of this was expert opinion. And at this time we are actually trying to test these guidelines to see if they hold true in those with cystic fibrosis. Since patients with cystic fibrosis are living longer, they are developing more complications, such as bone disease, and insuring that vitamin D supplementation is adequate and figuring out new ways to prevent bone disease is becoming more and more important for CF patients. In the discussion today, Peter Mogayzel, MD & Dianna Green, MD, of the Johns Hopkins University School of Medicine, will focus on the basis of the current vitamin D recommendation for patients with cystic fibrosis, describe the potential for success of various strategies for treatment of vitamin D insufficiency, and compare the potential benefits and risks of using bisphosphonates for the treatment of osteoporosis in patients with cystic fibrosis.

Improvements in nutrition have been a key factor in reducing morbidity and mortality in patients with cystic fibrosis (CF.) In this issue, Dr. Sarah Jane Schwarzenberg will summarize current progress in the definition and epidemiology of 2 CF GI-related issues—small intestinal bowel overgrowth (SIBO) and distal intestinal obstruction syndrome. The discussion continues with case studies to illustrate causes of nonpancreatic enzyme–related defects in fat absorption, including the use of omega-3 supplements.

In this edition Dr. Richard Moss will help expand our understanding of allergic bronchopulmonary aspergillosis (ABPA) with the discussion some typical case scenarios. ABPA is a major pulmonary complication in patients with cystic fibrosis (CF). The impact of ABPA on the course of CF lung disease poses special challenges, with chronic Aspergillus respiratory infection, even in the absence of ABPA, playing a significant role, according to data from 2 recent retrospective cohort studies. Dr. Moss' use of current diagnostic criteria provides perspective also on the limitations.

In September of 2009, the Cystic Fibrosis Foundation held a third CFRD consensus conference, co-sponsored by the American Diabetes Association and the Lawson Wilkins Pediatric Endocrine Society, providing a new set of recommendations for treating cystic fibrosis-related diabetes. Johns Hopkins provided evidence review so there can be evidence-based guidelines. Diagnostic & treatment criteria are presented in this podcast.

The eCysticFibrosis Review podcast is a clinical discussion between our January authors, Stephanie D. Davis, MD, Jessica Pittman, MD and Robert Busker, eCysticFibrosis Reviews Medical Editor. The topic is Measurement of Early Lung Disease in Children With Cystic Fibrosis. Participants can now receive 0.5 credits per podcast after completing an online post-test via the links provided on this page via eCysticFibrosisReview.org

The eCysticFibrosis Review podcast is a clinical discussion between our November authors, Michael Boyle, MD, Elliott Dasenbrook, MD and Robert Busker, eCysticFibrosis Reviews Medical Editor. The topic is Emerging Pathogens in Cystic Fibrosis. Participants can now receive 0.5 credits per podcast after completing an online post-test via the links provided on the website page, eCysticFibrosisReview.org

The eCysticFibrosis Review podcast is a clinical discussion between our September authors, Garry Cutting, MD, Barbara Karczeski, MS and Robert Busker, eCysticFibrosis Reviews Medical Editor. The topic is How to Interpret Genetic Testing for Cystic Fibrosis. Participants can now receive 0.5 credits per podcast after completing an online post-test via the links provided on the website page, eCysticFibrosisReview.org

Presented at NACFC Anaheim 2011 this symposium presentation offers information on the current treatment options for patients with cystic fibrosis. Mike Boyle, MD, from Johns Hopkins University School of Medicine offers a 25-minute look at the basics of CF, then describes genotype and CF phenotype relationships. The presentation includes a review the influences of severity of lung disease and outcomes in CF, followed up by issues related to adherence.

Presented at NACFC Anaheim 2011 this symposium presentation offers information on the current treatment options for patients with cystic fibrosis. Mike Boyle, MD, from Johns Hopkins University School of Medicine offers a 25-minute look at the basics of CF, then describes genotype and CF phenotype relationships. The presentation includes a review the influences of severity of lung disease and outcomes in CF, followed up by issues related to adherence.

Presented at NACFC Anaheim 2011 this symposium presentation offers information on the current treatment options for patients with cystic fibrosis. Mike Boyle, MD, from Johns Hopkins University School of Medicine offers a 25-minute look at the basics of CF, then describes genotype and CF phenotype relationships. The presentation includes a review the influences of severity of lung disease and outcomes in CF, followed up by issues related to adherence.