The 2013 Infection Prevention and Control (IP&C) Guideline for Cystic Fibrosis (CF) was commissioned by the CF Foundation as an update of the 2003 Infection Control Guideline for CF. During the past decade, new knowledge and new challenges provided the following rationale to develop updated IP&C strategies for this unique population:1. The need to integrate relevant recommendations from evidence-based guidelines published since 2003 into IP&C practices for CF. These included guidelines from the Centers for Disease Control and Prevention (CDC)/Healthcare Infection Control Practices Advisory Committee (HICPAC), the World Health Organization (WHO), and key professional societies, including the Infectious Diseases Society of America (IDSA) and the Society for Healthcare Epidemiology of America (SHEA). During the past decade, new evidence has led to a renewed emphasis on source containment of potential pathogens and the role played by the contaminated healthcare environment in the transmission of infectious agents. Furthermore, an increased understanding of the importance of the application of implementation science, monitoring adherence, and feedback principles has been shown to increase the effectiveness of IP&C guideline recommendations.2. Experience with emerging pathogens in the non-CF population has expanded our understanding of droplet transmission of respiratory pathogens and can inform IP&C strategies for CF. These pathogens include severe acute respiratory syndrome coronavirus and the 2009 influenza A H1N1. Lessons learned about preventing transmission of methicillin-resistant Staphylococcus aureus (MRSA) and multidrug-resistant gram-negative pathogens in non-CF patient populations also can inform IP&C strategies for CF.

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Airway clearance therapy (ACT) is used in a variety of settings for a variety of ailments. These guidelines were developed from a systematic review with the purpose of determining whether the use of nonpharmacologic ACT improves oxygenation, reduces length of time on the ventilator, reduces stay in the ICU, resolves atelectasis/consolidation, and/or improves respiratory mechanics, versus usual care in 3 populations. For hospitalized, adult and pediatric patients without cystic fibrosis, 1) chest physiotherapy (CPT) is not recommended for the routine treatment of uncomplicated pneumonia; 2) ACT is not recommended for routine use in patients with COPD; 3) ACT may be considered in patients with COPD with symptomatic secretion retention, guided by patient preference, toleration, and effectiveness of therapy; 4) ACT is not recommended if the patient is able to mobilize secretions with cough, but instruction in effective cough technique may be useful. For adult and pediatric patients with neuromuscular disease, respiratory muscle weakness, or impaired cough, 1) cough assist techniques should be used in patients with neuromuscular disease, particularly when peak cough flow is < 270 L/min; CPT, positive expiratory pressure, intrapulmonary percussive ventilation, and high-frequency chest wall compression cannot be recommended, due to insufficient evidence. For postoperative adult and pediatric patients, 1) incentive spirometry is not recommended for routine, prophylactic use in postoperative patients, 2) early mobility and ambulation is recommended to reduce postoperative complications and promote airway clearance, 3) ACT is not recommended for routine postoperative care. The lack of available high-level evidence related to ACT should prompt the design and completion of properly designed studies to determine the appropriate role for these therapies.

Aerosol delivery equipment used to administer inhaled medications includes the nebulizer, positive expiratory pressure devices added to the nebulizer, and valved holding chambers (spacers). These devices are semi-critical medical devices, and as such, infection prevention and control (IPC) guidelines recommend that they be cleaned, disinfected, rinsed with sterile water, and air-dried. There is confusion surrounding the care of aerosol devices because of inconsistencies in the various published IPC guidelines, lack of a standard of practice among institutions and respiratory therapists (RTs), and manufacturer's instructions for use of these devices are not always compatible with guidelines or practice. Challenges lie in awareness of IPC guidelines and establishing a standard for the care of aerosol delivery devices among all stakeholders/manufacturers, governments, vendors, and users. The latest IPC guideline from the Cystic Fibrosis Foundation, reviewed and endorsed by the Society for Healthcare Epidemiology of America and the Association for Professionals in Infection Control, has a recommendation for disposable nebulizers and a recommendation for reusable nebulizers. Reusable nebulizers should be cleaned, disinfected, rinsed with sterile water (if using a cold disinfectant), and air-dried between uses. The mouthpiece/mask of disposable nebulizers should be wiped with an alcohol pad, the residual volume should be rinsed out with sterile water after use, and the nebulizer should be replaced every 24 h. The RT plays a significant and responsible role in providing and teaching aerosol therapy to patients. The RT and all stakeholders need to work together to provide a standard of care for the safe use of aerosol delivery devices.

Cystic fibrosis (CF) is a complex genetic disease characterized by lung infections that lead to early morbidity and death. Pathogens that commonly infect the lungs of patients with CF include Staphylococcus aureus, Haemophilus influenzae, Pseudomonas aeruginosa, and Burkholderia cepacia. Aggressively treating pulmonary infection with antibiotics has contributed to improved survival in patients with CF but has also promoted multiple-drug-resistant bacteria. Other complexities include the ability of bacteria to form biofilms, which makes them more resistant to antibiotics, and emerging pathogens in CF, of which the clinical importance is not yet clear. Increasing evidence of patient-to-patient transmission of CF pathogens led the Cystic Fibrosis Foundation to produce evidence-based infection-control recommendations, which stress 4 principles: standard precautions, transmission-based precautions, hand hygiene, and care of respiratory equipment. Respiratory therapists need to know and follow these infection-control recommendations. Cohorting patients infected with B. cepacia complex is one of several interventions successful at keeping the spread of this pathogen low, but cohorting patients who are infected/colonized with other microbes is controversial, the main argument of which is not being certain of a patient's present respiratory culture status at any given patient visit.