Notes+on+cystic+fibrosis+overview+of+the+treatment+of+lung+disease+2-4-13

Notes on cystic fibrosis overview of the treatment of lung disease 2-4-13

q CF Care Centers (most with dedicated adult care programs)

q In the United States, more frequent caregiver-patient interaction (visit frequency, monitoring, and interventions for pulmonary exacerbations) is associated with improved outcomes

q A subgroup of CF patients also have airflow obstruction from bronchial hyperreactivity; many, but not all, of these patients show typical signs and symptoms of asthma, such as chest tightness, wheezing, and cough following exercise or exposure to allergens or cold air

q Inhaled DNase I (dornase alfa) — The endonuclease DNase I can decrease the viscosity of purulent CF sputum by cleaving long strands of denatured DNA that are released by degenerating neutrophils.

A metaanalysis of randomized trials concluded that DNase treatment improves lung function and is well tolerated [30].

q By contrast, hypertonic saline cannot be considered to be part of the routine care of CF children under the age of six years.

q DNase costs approximately 30-fold more than hypertonic saline

q Of special note is DNase, which is inactivated when mixed with 7 percent saline.

q Inhaled N-acetylcysteine — N-acetylcysteine, a free sulfhydryl reagent that cleaves disulfide bonds within mucus glycoproteins, can liquefy CF sputum in vitro. ^airway inflammation side effect

q In our practice, we recommend using azithromycin for all patients with CF older than six years of age regardless of P. aeruginosa infection status who have clinical evidence of airway inflammation such as chronic cough, or who have any reduction in FEV1.

q The guidelines committee of the CF Foundation suggests the use of high-dose ibuprofen in children older than 6 years of age who have good lung

q We agree with the guidelines committee of the CF Foundation, which recommends against the routine chronic use of oral corticosteroids for children with CF aged 6 to 18 years, in the absence of asthma or allergic bronchopulmonary aspergillosis, because of the associated adverse effects.

not sure what a glucocorticoid is classified as (I guess it decreases inflammation though)

q Based on efficacy in other populations, annual vaccination against viral influenza is recommended for all patients with CF older than 6 months of age,

q Supplemental oxygen during exercise was associated with improved exercise duration and peak performance.

q The procedure does not address the non-pulmonary problems associated with CF.

q 115 CF Centers within the United States