AHEAD OF THE CURVE: Cystic Fibrosis

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.

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.

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.

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.

This podcast has been developed for clinicians caring for patients with issues related to cystic fibrosis. You can also read the companion newsletter. In this edition Dr. Chris Goss will explain the use of new inhalation therapies for 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.