Infection Prevention and Control Cystic Fibrosis
Publication Date: August 1, 2014
Last Updated: March 14, 2022
Core Recommendations
The CF Foundation recommends that all healthcare personnel caring for people with CF (eg, the CF care team, inpatient staff, environmental services staff, research staff, and staff in diagnostic and therapeutic areas, including pulmonary function test [PFT] laboratories, radiology, phlebotomy, operating room, and physical therapy) receive education regarding IP&C for CF, using principles of adult learning. Education should be repeated at intervals each center deems appropriate. (IB)
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The CF Foundation recommends that the CF care team develop strategies to monitor adherence to IP&C practices by healthcare personnel and provide feedback. Feedback to the CF care team includes immediate feedback to an individual when a lapse in practice is observed and feedback to the entire CF care team of trends of overall adherence rates at regular intervals (eg, quarterly) on the basis of consistency of practices. (IB)
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The CF Foundation recommends that all people with CF and their families receive education regarding IP&C for CF, using age appropriate tools and reading/language level appropriate to the target audience. Involve people with CF and their families in the development of educational programs and implementation of recommended practices. Education should be repeated at intervals each center deems appropriate. (II)
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Partnering with Institutional IP&C Teams
The CF Foundation recommends that CF care teams collaborate with their institutional IP&C teams to implement the recommendations in this guideline. (IB)
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The CF Foundation recommends that CF care teams collaborate with their institutional IP&C teams to develop protocols, checklists, and audits to standardize implementation of practices for the following:
- a. Single-patient-use, disposable items
- b. Cleaning and disinfecting multiuse items (eg, patient care equipment, oximeters, iPads and similar tablets, and computers)
- c. Cleaning and disinfecting surfaces in the healthcare environment (eg, CF clinics, PFT rooms, hospital rooms, and sinks and showers).
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The CF Foundation recommends ensuring that dust containment during renovation and construction and water-leak remediation policies and practices are followed according to institutional and national guidelines in all ambulatory care areas and inpatient settings where people with CF receive care. ()
(IB/IC)
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The CF Foundation recommends that healthcare personnel assume that all people with CF could have pathogens in respiratory tract secretions that are transmissible to other people with CF. (IA)
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Practices for Healthcare Personnel
The CF Foundation recommends that all healthcare facilities caring for people with CF ensure ready availability of alcohol-based hand rub or antimicrobial soap and water in all patient rooms, PFT rooms, and waiting areas.
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The CF Foundation recommends that healthcare personnel perform hand hygiene (either using alcohol-based hand rub or washing hands with antimicrobial soap and water), as per CDC and WHO guidelines, in the following clinical situations:
- a. Before entering the room and when leaving the room of any patient
- b. Before and after direct contact with any patient
- c. Before putting gloves on and after removing gloves, for both sterile and nonsterile procedures
- d. After contact with patient’s skin, mucous membranes, respiratory secretions, or other body fluids
- e. After contact with inanimate objects (including medical equipment) in the vicinity of the patient that may be potentially contaminated with respiratory secretions.
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The CF Foundation recommends that healthcare personnel should not wear artificial fingernails or nail extenders when having direct contact with people with CF. (IA)
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The CF Foundation recommends that healthcare personnel should disinfect their stethoscopes before and after use on each patient in accordance with institutional IP&C policies. Stethoscopes that remain in the patient’s room and are dedicated for use only for that patient do not need to be disinfected before and after use. (IB)
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The CF Foundation recommends that healthcare personnel caring for people with CF should not be routinely screened for MRSA colonization unless they are epidemiologically linked to a cluster of MRSA infections in accordance with institutional IP&C policies and national guidelines. (IB)
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Isolation Precautions
The CF Foundation recommends that all healthcare personnel implement Contact Precautions (ie, wear a gown and gloves) when caring for all people with CF regardless of respiratory tract culture results, in ambulatory and inpatient settings. ()
(IB/IC)
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The CF Foundation does not recommend that healthcare personnel wear a mask routinely when caring for people with CF. However, the CF Foundation recommends mask use per CDC guidelines, as follows:
- a. Surgical (procedure, isolation) masks are worn by healthcare personnel caring for any patient under Droplet Precautions with suspected or confirmed pathogens that are transmitted by the droplet route (eg, adenovirus, rhinovirus, influenza virus, or Mycoplasma pneumoniae).
- b. Masks and eye protection should be worn by healthcare personnel if splashes or sprays of respiratory tract secretions are anticipated as per Standard Precautions.
- c. N-95 respirators (masks) or powered air-purifying respirators (PAPRs) are worn by healthcare personnel caring for any patient under Airborne Precautions (in an airborne infection isolation room [AIIR]) for suspected or confirmed infection with Mycobacterium tuberculosis.
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The CF Foundation recommends placing people with CF who are acid-fast bacilli (AFB) smear positive for the first time under Airborne Precautions (AIIR requirements: negative-pressure single room, more than 12 air exchanges per hour, air exhausted to the outside) in ambulatory and inpatient settings until M. tuberculosis infection has been excluded. Alternatively, in geographic locations with a very low incidence of TB, a risk assessment that includes the likelihood of exposure to individuals with TB (eg, travel or visitors from high-prevalence areas) may be used to guide the use of AIIRs. Consult with institutional IP&C staff and/or infectious disease physicians. ()
(IA/IC)
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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 under Airborne Precautions. (Low)
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Practices by People with CF and Family Members/Friends
The CF Foundation recommends that all people with CF, regardless of their respiratory tract culture results, be separated by at least 6 feet (2 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. ()
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The CF Foundation recommends that all people with CF and their family members/friends perform hand hygiene (with either alcohol-based hand rub or antimicrobial soap and water) when there is potential for contamination of hands with pathogens, such as the following:
- a. Entering and exiting CF clinics, clinic exam rooms, or hospital rooms
- b. Hands become contaminated with respiratory secretions (eg, after coughing or performing PFTs or chest physiotherapy).
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The CF Foundation does not recommend that people with CF wear gowns or gloves in CF clinics, in other ambulatory healthcare settings, or while hospitalized. ()
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The CF Foundation recommends that people with CF be instructed to follow Respiratory Hygiene practices to contain their secretions when coughing or sneezing (ie, cough into a tissue, immediately discard soiled tissue into a trash receptacle, and perform hand hygiene after disposing of soiled tissues). A covered trash receptacle with a foot pedal is preferred. (IB)
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The CF Foundation recommends that all people with CF wear a surgical (procedure, isolation) mask when in a healthcare facility to reduce the risk of transmission or acquisition of CF pathogens. Masks should be worn throughout the facility, including in restrooms. Masks should not be worn during pulmonary function testing, in the clinic exam room, or in the patient’s hospital room. If the optimal size mask is not available (eg, for small infants), use the smallest mask available. If a mask is not tolerated by an individual with CF who is having respiratory distress, encourage that person to follow Respiratory Hygiene practices. Masks should be changed when wet. (IB)
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The CF Foundation recommends that all people with CF who do not live in the same household avoid activities and risk factors that are associated with transmission of CF pathogens in nonhealthcare and healthcare settings, including the following:
- a. Social contact between people with CF
- b. Physical contact between people with CF (eg, handshakes, kissing, and intimate contact)
- c. Car rides with another person with CF
- d. Sharing hotel rooms with another person with CF
- e. Fitness class with another person with CF.
Activities that all people with CF, including those who live in same household, should avoid include the following:
- a. Sharing personal items (eg, toothbrush and drinking utensils) with another person with CF
- b. Sharing respiratory therapy equipment.
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The CF Foundation recommends that
- a. Tap water or well water that meets local public health standards, distilled water, or bottled water may be used by people with CF
- i. For drinking
- ii. For bathing
- iii. For cleaning nebulizers and other respiratory equipment (eg, airway clearance devices, spacers, and neti pots) if followed by disinfection
- iv. For the water needed for heat disinfection (eg, boiling, microwaving, and steam sterilizing)
- b. Only sterile water be used for nasal rinses (eg, neti pots), filling of humidifier reservoirs, and as a final rinse of respiratory equipment (eg, after cold disinfection)
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Immunizations/Influenza Chemoprophylaxis
The CF Foundation recommends that, as per CDC/ACIP recommendations, all healthcare personnel (unless there is a medical contraindication to immunization) should be immunized or have evidence of immunity to mumps, measles, rubella, varicella, pertussis (Tdap), and hepatitis B and receive an annual influenza immunization. ()
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The CF Foundation recommends that, as per CDC/ACIP recommendations, all people with CF and their family members/close contacts receive recommended vaccines at the recommended schedule, age, dose, and route of administration unless there is a medical contraindication. ()
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The CF Foundation recommends use of antiviral chemoprophylaxis or treatment (eg, oseltamivir) for prevention or treatment of influenza according to ACIP recommendations. (IA)
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Research Settings
The CF Foundation recommends that for all research activities, people with CF, their family members/friends, and healthcare personnel follow relevant IP&C recommendations for that healthcare setting. ()
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Recommendations for Microbiology and Molecular Epidemiology
Review Center-Specific Microbiology Data
The CF Foundation recommends that all CF centers obtain and review center-specific quarterly surveillance reports (eg, data from the local clinical microbiology laboratory or the CF Foundation Patient Registry) of the incidence and prevalence of respiratory tract pathogens at their centers. This review should be conducted in collaboration with institutional IP&C teams and microbiology laboratory directors. (IB)
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Molecular Typing
The CF Foundation recommends that CF isolates of Burkholderia spp. be sent to the laboratory at the University of Michigan (US) for confirmation of identification, speciation, and molecular typing, as follows:
- a. All initial isolates from every patient
- b. At least 1 isolate per patient per year
- c. Any isolates suspected of being associated with transmission or an outbreak
- d. Any other nonfermenting gram-negative organism for which species identification remains equivocal after routine analysis should be sent for confirmation of identification.
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The CF Foundation recommends that molecular typing of B. cepacia complex isolates and other microorganisms (eg, P. aeruginosa and NTM) be performed when epidemiologically indicated (eg, suspected patient-to-patient transmission). (IB)
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The CF Foundation recommends that molecular typing be performed using an appropriate genotyping method (eg, pulsed-field gel electrophoresis, random-amplified polymorphic DNA polymerase chain reaction [PCR], repetitive sequence-based PCR, or multilocus sequence typing). (IB)
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Surveillance
The CF Foundation and European CF Society (ECFS) recommend that screening cultures for NTM should be performed annually in individuals with a stable clinical course. Culture and smears for AFB from sputum should be used for NTM screening. In the absence of clinical features suggestive of NTM pulmonary disease, individuals who are not capable of spontaneously producing sputum do not require screening cultures for NTM. The CF Foundation and ECFS recommend against the use of oropharyngeal swabs for NTM screening. ()
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The CF Foundation concludes that there is insufficient evidence at the time of publication of this document to recommend criteria by which to consider a person with CF who previously had Burkholderia species isolated from respiratory tract cultures to be Burkholderia-free. (Low)
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III. Recommendations for CF Clinics and Other Ambulatory Care Settings
Scheduling in CF Clinics
The CF Foundation recommends that CF clinics schedule and manage people with CF in ways to minimize time in common waiting areas. Such strategies include the following:
- a. Stagger clinic schedule
- b. Place people with CF regardless of their respiratory culture results in an exam room immediately on arrival to the clinic
- c. Use a pager system or personal cell phone to alert people with CF that an exam room is available
- d. Keep a person with CF in one exam room while the CF care team rotates through the exam room
- e. Do not share common items (eg, clinic computer and toys), and request that people with CF bring their own recreational items to clinic appointments.
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The CF Foundation recommends that infants under 2 years of age be separated from other people with CF in CF clinics until adequate infection control education has been provided to and is understood by the caregivers. (Low)
(benefit: moderate)
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The CF Foundation recommends that all newly diagnosed people with CF be separated from other people with CF in CF clinics until adequate IP&C education has been provided to and is understood by newly diagnosed individuals and their caregivers. ()
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The CF Foundation concludes that there is insufficient evidence at the time of publication of this document for or against routinely scheduling CF clinics on the basis of specific pathogens isolated from respiratory tract cultures. (Low)
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Pulmonary Function Testing
The CF Foundation recommends that PFTs be performed in one of the following ways:
- a. In the exam room at the beginning of the clinic visit
- b. In a negative-pressure room (AIIR)
- c. In a PFT laboratory with either portable or integrated HEPA filters
- d. In a PFT laboratory without HEPA filtration, allowing 30 minutes to elapse before the next person with CF enters the PFT laboratory
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Environmental Practices
The CF Foundation recommends that exam rooms be cleaned and disinfected between patients using a 1-step process and Environmental Protection Agency (EPA)–registered hospital-grade disinfectant/detergent designed for housekeeping in accordance with institutional IP&C policies. (IB)
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Designing a New CF Clinic
The CF Foundation recommends that the leadership staff of CF centers collaborate with the institutional IP&C and planning design and construction departments when designing a new CF clinic to ensure a design that includes the following:
- a. Provision for management of people with CF who require Airborne Precautions
- b. Appropriate number of exam rooms
- c. Single-person restrooms
- d. Adequate space for personal protective equipment (eg, masks, gowns, and gloves) at the point of use
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Recommendations for Inpatient Settings
Room Placement
The CF Foundation recommends that people with CF be placed in a single-patient room. Only people with CF who live in the same household may share a hospital room. (IB)
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The CF Foundation recommends placing people with CF who are solid-organ transplant recipients in a single-patient room in accordance with institutional policy and national guidelines. There is insufficient evidence to recommend for or against Protective Environment (ie, positive pressure room and HEPA filtration) for solid-organ recipients. (U)
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Practices for People with CF and Their Families
The CF Foundation recommends evaluating people with CF on a case-by-case basis in accordance with institutional IP&C policies for participation in activities outside the hospital room (eg, walking in the hallway, going to the playroom, physical therapy, exercise room, or school room) only when no other person with CF is present and under the supervision of a trained staff member. Considerations include the capability of a person with CF to contain his or her respiratory tract secretions, age, endemic levels of pathogens in an individual center, and adherence to the following practices:
- a. Perform hand hygiene and put on a mask immediately before leaving patient rooms
- b. After a person with CF has left a hospital activity room, clean surfaces and touched items with an EPA-registered hospital disinfectant/detergent
(IB/II)
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The CF Foundation recommends that all people with CF perform all respiratory interventions (eg, aerosol therapy, airway clearance, and collection of respiratory tract cultures) in the patients’ rooms. If 2 people with CF who live in the same household are sharing a room, these procedures should be performed when the second person is not in the room, whenever possible. (IB)
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The CF Foundation recommends that airway clearance devices (eg, flutter, acapella, pep device, and therapy vest) be for single-patient use only, in accordance with institutional IP&C policies. (II)
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The CF Foundation recommends following institutional IP&C policies for the use of masks, gowns, and gloves by individuals who are visiting hospitalized people with CF. (U)
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Care of Nebulizers in the Hospital
The CF Foundation recommends the following:
- a. Nebulizers are for single-patient use only
- b. Aseptic technique is always followed when handling the nebulizer and dispensing medications
- c. Single-dose vials of medication used in nebulizers are always preferred
- d. Handheld disposable nebulizers are managed as follows:
- i. After each use, rinse out residual volume with sterile water and wipe mask/mouthpiece with an alcohol pad
- ii. Discard the nebulizer every 24 hours.
- e. Handheld reusable nebulizers (eg, home equipment) are managed as follows:
- i. After each use, clean, disinfect, rinse with sterile water (if applicable, following cold disinfection method), and air dry away from sink
- ii. After each use, the nebulizer can be reprocessed (eg, by steam sterilization) if the reprocessing is performed according to the manufacturer’s instructions and the CF Foundation recommendations for home care (rec. 59) and if the nebulizer can be returned to the patient in time for the next treatment.
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Animals
The CF Foundation recommends that people with CF can participate in animal-assisted (“pet”) therapy in accordance with institutional policies. (II)
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Designing New Inpatient Facilities
The CF Foundation recommends that the leadership staff of CF centers collaborate with the institutional IP&C and the planning, design, and construction departments when designing a new inpatient unit to ensure a design that
- a. Provides an adequate number of single-patient rooms to care for people with CF
- b. Includes a provision for people with CF who require possible Airborne Precautions
- c. Provides access to exercise during hospitalization (eg, adequate space for exercise equipment)
- d. Provides adequate space for personal protective equipment (eg, masks, gowns, and gloves) at the point of use.
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Recommendations for Nonhealthcare Settings
Families with More than 1 Person with CF
The CF Foundation recommends that it is preferable that people with CF who live in the same household perform airway clearance with only 1 person with CF in the room during treatment. (II)
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Events and Activities
The CF Foundation recommends against CF-specific camps or CF-specific educational retreats for groups of people with CF. Only 1 individual with CF should attend any camp or educational retreat unless they live in the same household. However, family members who do not have CF may attend educational retreats. People with CF are encouraged to participate in camps and sports with non-CF individuals. (IB)
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People with CF and their parents or legal guardians are not obligated to disclose the diagnosis of CF or the results of respiratory tract cultures to school or day care personnel. However, the CF Foundation recommends disclosure so that school or day care personnel can be made aware of the importance of IP&C principles and practices for the protection of students with CF and can make the recommended accommodations. Such information must be maintained as confidential medical information unless the person with CF and/or parent or legal guardian choose to make this information known. (II)
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The CF Foundation recommends that people with CF attending the same day care and/or school should not be in the same room at the same time unless they live in the same household. The CF Foundation recommends education of day care/school personnel on the principles of IP&C for CF so they can work with people with CF and/or parents or legal guardians to develop strategies to minimize contact between people with CF (eg, assignment to separate classrooms and separation during other scheduled common activities, including lunch, physical education, and recess). (II)
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The CF Foundation recommends that only 1 person with CF attend CF Foundation–sponsored, healthcare-sponsored, or CF center–sponsored indoor events (eg, CF Education Days) unless they live in the same household, to reduce the risk of person-to-person transmission of CF pathogens. ()
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The CF Foundation recommends developing and utilizing alternative CF education programs, (eg, videotapes, video conferencing, CD-ROM web-based learning, and apps) that do not require face-to-face meetings among people with CF. (II)
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The CF Foundation recommends that people with CF can attend CF Foundation–sponsored, healthcare-sponsored, or CF center–sponsored outdoor events (eg, Great Strides) providing they maintain a distance of at least 6 feet (2 meters) from others with CF. ()
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MRSA
The CF Foundation recommends that people with CF should avoid direct contact with people with skin and soft-tissue infections caused by MRSA unless wounds are covered, hand hygiene is performed frequently, personal items (eg, towels) are not shared, sports equipment is cleaned between use, and cleaning protocols for environmental surfaces are established to reduce the risk of MRSA transmission. ()
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The CF Foundation recommends that people with CF and respiratory cultures positive for MRSA should not be restricted from contact with people without CF in congregate settings (eg, sports teams, classrooms, and the workplace) if the person with CF performs appropriate hand and respiratory hygiene. ()
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Nebulizers: Cleaning and Disinfecting
The CF Foundation recommends that the following steps be performed for nebulizers used in the home as soon as possible after each use:
- a. Clean the nebulizer parts with dish detergent soap and water
- b. Disinfect the nebulizer parts using one of the following methods:
Heat methods:
-
a. Place in boiling water and boil for 5 minutes
-
b. Place in a microwave-safe receptacle submerged in water and microwave for 5 minutes
-
c. Use a dishwasher if the water is more than or equal to 70°C or 158°F for 30 minutes
-
d. Use an electric steam sterilizer.
Cold methods:
-
a. Soak in 70% isopropyl alcohol for 5 minutes
-
b. Soak in 3% hydrogen peroxide for 30 minutes
-
i. Rinse off the cold-method disinfectant using sterile water, not tap water; the final rinse must be with sterile or filtered (less than or equal to 0.2-micron filter) water
-
ii. Air dry the nebulizer parts before storage.
-
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The CF Foundation recommends that nebulizers used in the home should not be disinfected with acetic acid (vinegar), bleach solutions, or benzalkonium chloride (eg, “Control III”). ()
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Leisure Activities
The CF Foundation recommends that people with CF should limit prolonged and/or repeated exposure to activities that generate dust from soil and organic matter (eg, gardening and lawn mowing) to decrease exposure to potential soilborne pathogens (eg, Burkholderia spp. and Aspergillus spp.). ()
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The CF Foundation recommends that people with CF should avoid exposure to construction and renovation activities that generate dust to decrease exposure to potential pathogens (eg, Aspergillus spp.). ()
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The CF Foundation recommends that people with CF can swim in pools or water parks with adequate disinfection (eg, chlorination). ()
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The CF Foundation recommends that people with CF avoid activities in hot tubs, whirlpool spas, and stagnant water. ()
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There is insufficient evidence at the time of publication of this document for the CF Foundation to recommend for or against people with CF avoiding activities in natural bodies of water that are not stagnant (eg, ocean, ponds, and hot springs). (Low)
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Contact with Pets or Farm Animals
The CF Foundation recommends that people with CF perform hand hygiene after changing the litter, handling feces, cleaning and disinfecting the cages or fish tanks of their pets, or interacting with farm animals. ()
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The CF Foundation recommends that people with CF avoid cleaning stalls, pens, or coops. ()
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Recommendations for Healthcare Personnel with CF
The CF Foundation recommends that healthcare personnel with CF should not provide care for other people with CF. ()
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The CF Foundation recommends that people with CF interested in a career in healthcare receive counseling from their CF care team regarding specialty areas wherein job duties minimize the risk of transmission or acquisition of potential pathogens. (II)
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The CF Foundation recommends that healthcare personnel with CF consider informing their employers’ workforce health and safety department about their diagnosis of CF to ensure that job duties are assigned and care practices are adopted that minimize the risk of acquisition or transmission of potential pathogens. This disclosure is legally required to be kept confidential. ()
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The CF Foundation recommends that when it is known that a healthcare provider with or without CF is infected/colonized with MRSA, work assignments should be made according to local hospital policy. (II)
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The CF Foundation recommends that healthcare personnel with CF be assigned to care for patients without CF on a case-by-case basis, considering health- and behavior-related factors, such as:
a. Frequency and severity of coughing episodes, quantity of sputum production during these episodes, and ability to contain respiratory tract secretions
b. Ability to use barrier precautions and adhere to IP&C guidelines, Centers for Medicare & Medicaid Services, HICPAC, and CDC guidelines; and
c. Risk of transmission of pathogens by healthcare personnel with CF in the context of specific job duties.
(II)b. Ability to use barrier precautions and adhere to IP&C guidelines, Centers for Medicare & Medicaid Services, HICPAC, and CDC guidelines; and
c. Risk of transmission of pathogens by healthcare personnel with CF in the context of specific job duties.
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Recommendations for Psychosocial and Medical Impact of IP&C
The CF Foundation recommends educating, when appropriate, friends, teachers, employers, and coworkers about the rationale for the IP&C recommendations. (II)
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The CF Foundation recommends identifying CF center–specific concerns for the potential psychosocial impact of the IP&C guideline for people with CF in the hospital, clinic, community, school, and home and strategies, including an available counselor, to minimize the negative impact. (II)
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The CF Foundation recommends that the CF care team inform people with CF and their parents or legal guardians of their microbiologic status. People with CF and their parents or legal guardians will then determine whom they will inform. (II)
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The CF Foundation recommends collaboration with the child life staff to ensure individualized programs consistent with the recommended IP&C guidelines. (II)
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The CF Foundation recommends making accommodations (eg, providing entertainment, enhancing communication with the outside world, facilitating visits with non-CF individuals, and adapting child life programs) to relieve the psychosocial stress of inpatient and outpatient IP&C guidelines without placing people with CF at risk for transmission or acquisition of pathogens. (II)
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Recommendation Grading
Overview
Title
Infection Prevention and Control Cystic Fibrosis
Authoring Organization
Cystic Fibrosis Foundation
Publication Month/Year
August 1, 2014
Last Updated Month/Year
June 27, 2023
Document Type
Guideline
External Publication Status
Published
Country of Publication
US
Inclusion Criteria
Female, Male, Adult, Older adult
Health Care Settings
Emergency care, Hospital, Outpatient
Intended Users
Respiratory therapist, nurse, nurse practitioner, physician, physician assistant
Scope
Prevention, Management
Diseases/Conditions (MeSH)
D003550 - Cystic Fibrosis
Keywords
cystic fibrosis, infection prevention
Methodology
Number of Source Documents
500
Literature Search Start Date
June 1, 2012
Literature Search End Date
August 1, 2012