One Center's Guide to Outpatient Management of Pediatric Cystic Fibrosis Acute Pulmonary Exacerbation

Abstract

Cystic fibrosis (CF) is a chronic disorder characterized by acute pulmonary exacerbations that comprise increased cough, chest congestion, increased mucus production, shortness of breath, weight loss, and fatigue. Typically, severe episodes are treated in the inpatient setting and include intravenous antimicrobials, airway clearance therapy, and nutritional support. Children with less-severe findings can often be managed as outpatients with oral antimicrobials and increased airway clearance therapy at home without visiting the specialty CF center to begin treatment. Selection of specific antimicrobial agents is dependent on pathogens found in surveillance culture, activity of an agent in patients with CF, and the unique physiology of these patients. In this pediatric review, we present our practice for defining acute pulmonary exacerbation, deciding treatment location, initiating treatment either in-person or remotely, determining the frequency of airway clearance, selecting antimicrobial therapy, recommending timing for follow-up visit, and recognizing and managing treatment failures.

Telemedicine in Pediatric Infectious Diseases

“…A study conducted in a large US center on children with CF tried to apply telemedicine in the management of oral antibiotic therapy in pediatric patients with mild to moderate relapses. A modified form of the Akron Children’s Hospital CF Pulmonary Exacerbation Score (PES) has been adopted as a telephone triage tool [ 46 ]. The telephone PES used systemic signs, including fever, fatigue, appetite, absence from school and increased lung work; signs such as coughing, dyspnea and hemoptysis, although without objective data (e.g., results of lung-function tests, weight); and objective examination results.…”

“…This approach is also needed in developing countries. A pediatric study was conducted in Bogota, Colombia, where most patients are concentrated in rural areas, often far from the main facilities [ 46 ]. The system has reduced the number of patient transfers to higher-level hospitals by 83%, as well as waiting times for transfers, improving healthcare at a reasonable cost.…”