AHEAD OF THE CURVE: Cystic Fibrosis

In this podcast guest author Dr. Margaret Rosenfeld from the University of Washington School of Medicine, discusses the rationale for early Pseudomonas aeruginosa eradication, Identifies the inhaled and oral antibiotics that have been compared in clinical trials for early Pseudomonas eradication, and describes the potential adverse effects of early Pseudomonas eradication therapy.

Nonadherence to any medical regimen is common particularly among adolescents and young adults, and cystic fibrosis (CF) is no exception. There are many barriers to following the complex daily regimen that CF management requires, and researchers and clinicians have not yet found the ideal approach for counseling and supporting patients to improve and maintain their level of adherence. In this podcast Dr. Kristin Riekert, of The Johns Hopkins Adherence Research Center discusses best practices for determining which clinic patients are nonadherent and Identifies risk factors for nonadherence.

Dr. Elizabeth Yen offers a clinical perspective to her review on how new research that highlights the strong positive impact of early behavioral and nutritional education and predictors of long-term response to such interventions. In this podcast she will expand on that information by discussing case scenarios.

In this issue, Dr. Elizabeth Yen reviews new research highlighting the strong positive impact of early behavioral and nutritional education. Exploring predictors of long-term response to such interventions she discusss how body image may affect compliance with nutritional recommendations in adolescents; and, then present data describing positive outcomes with gastrostomy tube placement for supplemental enteral intake.

Drs. Cutting and Collaco will help expand our understanding of the genotypic and phenotypic modifiers in the treatment of cystic fibrosis, with the discussion some typical case scenarios.

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

Clinical practice guidelines have become a regular component of health care. The Cystic Fibrosis Foundation has supported the development of several recent clinical practice guidelines to review the evidence that endorses many of the therapies currently in use.

Although numerous clinical manifestations of cystic fibrosis (CF) exist, the principal causes of disease-related morbidity and mortality are due to pancreatic insufficiency and chronic airway infection. Recommendations are now available on the assessment and treatment of nutritional disease, use of airway clearance therapies, and use of chronic medications to maintain lung health. Acute complications of CF lung disease include pulmonary exacerbations, hemoptysis, and pneumothorax; guidelines on the management of these complications are currently available as well. Finally, as newborn screening for CF has become standard in all states, we can expect that most patients will be diagnosed in the absence of symptoms. Infant care guidelines should provide primary care physicians with the information needed for appropriate assessment and treatment of newly diagnosed patients.

In this podcast recording, Dr. Patrick Flume utilizes patient cases to summarize the clinical practice guidelines for individuals with CF, to aid clinicians in improving the quality and consistency of the care they provide.

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 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.