Infection Prevention and Control Clinical Care Guidelines

Updated from 2003, these guidelines on infection prevention and control for cystic fibrosis provide recommendations for people with CF, their families, and health care providers to help reduce the spread of germs in the clinic and hospital setting, as well as in everyday life.

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Summary
  • The 2013 guidelines for infection prevention and control (IPC) for cystic fibrosis provide an update to the 2003 guidelines.
  • The guidelines were created by an interdisciplinary team using a systematic process to review and grade evidence for each recommendation.

Infection Prevention and Control Clinical Care Guidelines

Infection Prevention and Control Guideline for Cystic Fibrosis: 2013 Update 
Saiman L, Siegel JD, LiPuma JJ, Brown RF, Bryson EA, Chambers MJ, Downer VS, Fliege J, Hazle LA, Jain M, Marshall BC, O'Malley C, Pattee SR, Potter-Bynoe G, Reid S, Robinson KA, Sabadosa KA, Schmidt HJ, Tullis E, Webber J, Weber DJ. Infect Control Hosp Epidemiol. 2014 Aug;35 Suppl 1:S1-S67. Epub 2014 Jul 1.PMID: 25025126

The 2013 guidelines for infection prevention and control (IPC) for cystic fibrosis provide an update to the 2003 guidelines. The guidelines were created by an interdisciplinary team using a systematic process to review and grade evidence for each recommendation. Key changes in the 2013 guidelines include the recommendations for:

  • Contact precautions for all CF patients regardless of pathogen status.
  • Mask use by patients in common areas in health care settings.
  • A minimum six-foot distance between patients.
  • Standards for reducing infection risk with pulmonary function testing.
  • Auditing the cleaning and disinfection of environmental surfaces.

This update also recommends against universal mask use by health care workers.

Recent studies on airborne spread of CF pathogens and guidelines for nontuberculous mycobacteria (NTM) in CF patients support the 2013 guidelines. Key unanswered questions regarding IPC for CF patients include how to schedule patients in clinic and what Airborne Precautions to take for cases of suspected NTM infection.

Purpose and Background

In 2014, an update to the 2003 Guideline for Infection Prevention and Control for CF was published. The rationale for the guideline update was to incorporate:

  • New knowledge and understanding of person-to-person transmission of CF pathogens, the association of this transmission with adverse outcomes, and the changing epidemiology of CF pathogens.
  • New knowledge about person-to-person transmission of pathogens in non-CF populations that may be of relevance to CF patients and publication of new evidence-based IPC guidelines that included effective strategies to prevent transmission.
  • Expanded understanding of droplet and airborne transmission of respiratory pathogens.

Methodology

In 2011, an interdisciplinary committee of health care professionals, parents of children with CF, and an adult with CF developed the scope of the guidelines using the population, intervention, comparison, and outcomes (PICO) format. A systematic review of the literature was performed to address the PICO questions. The committee used the Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) system to describe the strength of evidence for recommendations adopted from other sources such as the Centers for Disease Control and Prevention and World Health Organization. However, limited evidence was found by the systematic review for other recommendations and the GRADE system could not be used. These IPC recommendations for CF were made based on expert consensus (anonymous voting with at least 80 percent agreement by the committee members).

Selected recommendations from the 2013 guideline are presented. An * denotes those recommendations that were modified or new when compared to the 2003 guideline. Relevant studies published since the 2013 guideline are also provided and support the recommendations.

Recommendations

Recommendations Evaluation of the Evidence
Education: Provide education to all health care personnel and people with CF and their families regarding IPC practices. Educational materials should be age-appropriate and language-appropriate. Grade IB
Audits of IPC practices: CF care teams should collaborate with their IPC teams to develop protocols, checklists, and audits to standardize implementation of practices for cleaning and disinfecting multiuse items and surfaces in the health care environment.* 100% Committee Consensus
Hand hygiene: All health care professionals and people with CF and family members and friends should perform hand hygiene with alcohol-based hand rub or antimicrobial soap and water when hands could be potentially contaminated with pathogens. Grade IA
Contact precautions: All health care personnel should implement Contact Precautions (i.e., wear a gown and gloves) when caring for all people with CF, regardless of respiratory tract culture results.* 100% Committee Consensus
Six-foot rule: All people with CF, regardless of their respiratory tract culture results, should be separated by at least six feet (two meters) from other people with CF in all settings, to reduce the risk of droplet transmission of CF pathogens. This does not apply to members of the same household.* 100% Committee Consensus
Isolation precautions for nontuberculous mycobacteria: The CF Foundation concludes that there is insufficient evidence at the time of publication of this document for or against placing people with CF who are infected with NTM on Airborne Precautions.* 2013 CF IPC guideline
Certainty: Low
Mask use: All people with CF should wear a surgical (procedure, isolation) mask when in health care settings to reduce the risk of transmission or acquisition of CF pathogens.* 100% Committee Consensus
Anticipating psychosocial impact of IPC guidelines: Centers should identify specific concerns for the potential psychosocial impact of the IPC guidelines for people with CF and identify strategies including an available counselor, to minimize the negative impact.* 100% Committee Consensus

Microbiology and Molecular Epidemiology

Recommendations Evaluation of the Evidence
Microbiology surveillance: All CF centers should review their quarterly surveillance reports to assess the incidence and prevalence of respiratory tract pathogens at their centers.* 100% Committee Consensus
Molecular typing: Molecular typing should be performed using an appropriate genotyping method (e.g., pulsed-field gel electrophoresis, random-amplified polymorphic, DNA polymerase chain reaction (PCR), repetitive sequence-based PCR, or multi-locus sequence typing).*  

Outpatient Settings

Recommendations Evaluation of the Evidence
Minimize time in common areas of CF clinic: CF clinics should schedule and manage people with CF to minimize time in common waiting areas by using staggered clinic schedules, placing people with CF immediately into an exam room, using a pager system to alert people with CF that an exam room is available, and keeping people with CF in their exam room while the care team rotates into the room. 100% Committee Consensus
Pulmonary function tests (PFTs): PFTs should be performed in one of the following ways:
In the exam room at the beginning of the clinic visit
In a negative-pressure room
In a PFT laboratory with either portable or integrated high-efficiency particulate air (HEPA) filters
In a PFT laboratory without HEPA filtration, allowing 30 minutes to elapse before the next person with CF enters the PFT laboratory*
100% Committee Consensus3, 7

Inpatient Settings

Recommendations Evaluation of the Evidence
Hospital room: People with CF should be placed in a single-patient room.* 100% Committee Consensus
Activities outside hospital room: Centers should evaluate people with CF case-by-case and in accordance with institutional IPC policies regarding the pursuit of activities outside the hospital room.* 100% Committee Consensus
Handheld disposable nebulizers: Handheld disposable nebulizers should be managed as follows:
After each use, rinse out residual volume with sterile water and wipe the mask or mouthpiece with an alcohol pad
Discard the nebulizer every 24 hours*
100% Committee Consensus8
Non-Health Care Settings  
Recommendations Evaluation of the Evidence
Events and activities: Only one person with CF should attend any camp, educational retreat, or CF Foundation-sponsored indoor event. Family members without CF and individuals with CF who live together in the same household may attend these activities.*  
   

* Denotes those recommendations that were modified or new when compared to the 2003 guideline

Unanswered Questions

Several areas had insufficient evidence to make a recommendation and additional research is needed.

  • When should a person with CF who previously had Burkholderia species isolated from respiratory tract cultures be considered Burkholderia-free?
  • Should CF clinics be routinely scheduled based on specific respiratory tract pathogens?
  • Should people with CF who are infected with NTM be placed on Airborne Precautions (negative pressure single room, more than 12 air exchanges per hour, air exhausted to outside)?
  • Should solid organ transplant recipients with CF be placed in a Protective Environment (i.e., a positive pressure room with HEPA filtration)?

Further Reading

Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.

  1. Savant AP, O'Malley C, Bichl S, McColley SA. Improved patient safety through reduced airway infection rates in a paediatric cystic fibrosis programme after a quality improvement effort to enhance infection prevention and control measures. BMJ Qual Saf. 2014 Apr;23 Suppl 1:i73-80.PMID:24608553
    This quality improvement initiative found that the use of Contact Precautions for all people with CF, regardless of respiratory tract culture results and other IPC measures, decreased Pseudomonas aeruginosa and methicillin-resistant Staphylococcus aureus (MRSA) infection rates from 30 percent to 21 percent and from 10.8 percent to 8.7 percent, respectively. Findings support using Contact Precautions for all people with CF.
  2. Knibbs LD, Johnson GR, Kidd TJ, Cheney J, Grimwood K, Kattenbelt JA, O'Rourke PK, Ramsay KA, Sly PD, Wainwright CE, Wood ME, Morawska L, Bell SC. Viability of Pseudomonas aeruginosa in cough aerosols generated by persons with cystic fibrosis. Thorax. 2014;69(8):740-45. Epub 2014 Apr 17.PMID: 24743559
    P. aeruginosa was detected four meters from 17 of 18 subjects (94 percent) after forced coughing and remained viable for 45 minutes in 14 of 18 subjects (78 percent). Findings support maintaining at least six feet between people with CF. 
  3. Johnston DI, Chisty Z, Gross JE et al. Investigation of Mycobacterium abscessus outbreak among cystic fibrosis patients, Hawaii 2012. J Hosp Infect. 2016;94(2):198-200.PMID:24743559
    This study describes a Mycobacterium abscessus outbreak in nine children with CF. The outbreak was attributed to possible lapses in cleaning and disinfection of bronchoscopes and spirometers. After obtaining dedicated pediatric equipment and a respiratory therapist, only one additional child acquired the outbreak strain. Findings suggest possible patient-to-patient transmission, but the transmission route was not fully elucidated.
  4. Kapnadak SG, Hisert KB, Pottinger PS, Limaye AP, Aitken ML. Infection control strategies that successfully controlled an outbreak of Mycobacterium abscessusat a cystic fibrosis center. Am J Infect Control. 2016;44(2):154-59.PMID:26442462
    This study further describes an M. abscessus subspecies massiliense outbreak that occurred in 2009. Findings strongly suggest patient-to-patient transmission, but the transmission route was not fully elucidated.
  5. Harris KA, Underwood A, Kenna DTD, Brooks A, Kavaliunaite E, Kapatai G, Tewolde R, Aurora P, Dixon G. Whole genome sequencing and epidemiological analysis do not provide evidence for cross-transmission of Mycobacterium abscessus in a cohort of pediatric cystic fibrosis patients. Clin Infect Dis 2015 Apr 1;60(7):1007-16.PMID:25452595
    This study evaluated 27 isolates of M. abscessus from 20 people with CF and only found a sibling pair with the same isolate. No cross transmission of M. abscessus was demonstrated within this health care setting.
  6. Driessche KV, Hens N, Tilley P, Quon BS, Chilvers MA, de Groot R, Cotton MF, Marais BJ, Speert DP, Zlosnik JE. Surgical masks reduce airborne spread of Pseudomonas aeruginosa in colonized patients with cystic fibrosis. Am J Respir Crit Care Med. 2015;192(7):897-99.PMID: 26426786.
    This study found that masks reduced detection of infectious aerosols by 88 percent in six people with CF who produced aerosols of P. aeruginosa. Findings support mask use by people with CF.
  7. Zuckerman JB, Clock SA, Prato BS, McDevitt JJ, Zhou JJ, Leclair LW, Lucas FL, Saiman L. Air contamination with bacteria in cystic fibrosis clinics: implications for prevention strategies. Am J Respir Crit Care Med. 2015;191(5): 598-601.PMID: 25723827
    This study demonstrated that compared with the air in exam rooms, the air of spirometry rooms was more likely to be contaminated, but cleared by 30 minutes. Findings support recommended strategies for performing PFTs.
  8. Weber DJ, Gergen ME, Sickbert-Bennett EE, Short KA, Lanza-Kaduce KE, Rutala WA. Frequency of contamination of single-patient-use nebulizers over time. Infect Control Hosp Epidemiol. 2014;35(12):1543-1546. Epub 2014 Nov 10.PMID: 25419779
    This study provides evidence that, as an alternative to discarding disposable nebulizers in the hospital after 24 hours, nebulizers that have been cleaned after each use may be discarded after 48 or 72 hours of use, as contamination rates with epidemiologically important pathogens were low and the same after 24, 48, and 72 hours of use.
  9. Somayaji R, Waddell B, Workentine ML, Surette MG, Brager NP, Rabin HR, Parkins MD. Infection control knowledge, beliefs and behaviours amongst cystic fibrosis patients with epidemic Pseudomonas aeruginosa. BMC Pulm Med. 2015;15:138.PMID:26542115
    This study demonstrated that individuals with CF infected with an epidemic P. aeruginosa strain were more likely to have attended CF camp, have a history of CF fundraising, and are more likely to continue to associate with others with CF despite counseling. These findings support avoiding socialization and providing alternatives to face-to-face methods of communication and support. 
  10. Bowmer G, Latchford G., Duff A., Denton M, Dye L, Lawton C, Lee T. Adherence to infection prevention and control guidelines: A vignette-based study of decision-making and risk-taking in young adults with cystic fibrosis. J Cystic Fibros 2017 Jan;16(1):146-50. Epub 2016 Sep 22.PMID:27666487
    Adults with CF make decisions that discriminate between risk levels, but risk perception is associated with willingness to participate in behaviors associated with transmission and acquisition of CF pathogens. These findings support improving educational strategies.
  11. Jain M, Saiman LM, Sabadosa K, LiPuma JJ. Point: does the risk of cross infection warrant exclusion of adults with cystic fibrosis from cystic fibrosis foundation events? Yes. Chest. 2014; 145(4):678-80.PMID: 24135971
    A pro-con debate on whether more than one adult with CF should be excluded from CF Foundation and CF center-sponsored events. This debate highlighted the complexity of this issue.
  12. Shepherd SL, Goodrich EJ, Desch J, Quinton PM. Counterpoint: does the risk of cross infection warrant exclusion of adults with cystic fibrosis from cystic fibrosis foundation events? No. Chest. 2014;145(4):680-83.PMID: 24135999
    A pro-con debate on whether more than one adult with CF should be excluded from CF Foundation and CF center-sponsored events. This debate highlighted the complexity of this issue.
  13. O'Malley CA. Device cleaning and infection control in aerosol therapy. Respir Care2015;60(6):917-30.PMID: 26070583
    This paper provides enhanced details of care for disposable and reusable nebulizers and the role of respiratory therapists and augments the recommendations for nebulizer care. 
  14. Hohenwarter K, Prammer W., Aichinger W, Reychler G. An evaluation of different steam disinfection protocols for cystic fibrosis nebulizers. J Cystic Fibros 2016;15(1):78-84. Epub 2015 Jul29.PMID: 26233897
    This study evaluated six protocols using six different steam disinfectors to assess the best methods of steam disinfection of nebulizer parts. An increased risk of recontamination was associated with manipulation of the parts by active drying. These findings augment the recommendations for nebulizer care.

This executive summary was prepared by:

Alex H. Gifford, MD, FCCP (Dartmouth-Hitchcock Medical Center, Lebanon, N.H.), William Stoudemire, MD (University of North Carolina, Chapel Hill, N.C.), and Lisa Saiman, MD, MPH (Columbia University Medical Center and New York-Presbyterian Medical Center, New York)

The guidelines were published in July 2014, they were reviewed in April 2019 and it was determined that no update is needed at this time.
 

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What is IPC? Download PDF
For Clinicians: 11 Ways to Guard Against Germs in Health Care Settings Download PDF
References for CFF IPC policy Download PDF
For Patients: 7 Ways to Guard Against Germs in Health Care Settings Download PDF
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