Infection Prevention for Patients with Cystic Fibrosis

The Cystic Fibrosis Foundation (CFF) has published recommendations regarding infection prevention and control practices for patients with CF. Read the "Infection Prevention and Control Guidelines for Cystic Fibrosis: 2013 Update"

These recommendations are available to CF Centers to use as a guideline in caring for their patients with cystic fibrosis. The recommendations are qualified as a “work in progress” because as new information on infection prevention and control becomes available, changes in the way we do things may be necessary.

Infection prevention and control practices are especially important to follow for people who have CF. People with CF are born with an altered CF gene that causes an abnormal movement of salt and water in and out of the cells that line their lungs. As a result, the mucus layer lining the lungs is thick and sticky, and it is more difficult to clear this mucus from the lungs. Germs like this environment.

Germs are everywhere. Germs are the bacteria, viruses, molds and fungi that make us sick if we “catch” them. Viruses, for example, are germs that cause the common cold. Everyone catches a cold now and then, and it generally runs its course without too much trouble, even for a person with cystic fibrosis. However, a person with CF is more vulnerable to bacterial growth and infection, so it is important to try and prevent catching or spreading germs as best you can.

There are many types of germs. The germs called Pseudomonas aeruginosa and Burkholderia, for example, are bacteria that get particular attention in CF. Pseudomonas aeruginosa shows up in many places and is commonly found in the environment, especially wet places. It can be found in contaminated respiratory equipment, on surfaces, in sinks and in showers. For reasons that are not known, Pseudomonas is a common lung infection among people with CF. According to the 2016 CFF Patient Registry Data Report, about 20% of children less than two years of age and 50% of 18-year-olds have Pseudomonas in their lungs. And in most cases, it is unknown where the initial source of a Pseudomonas infection came from.

Burkholderia is a germ that is found in the natural environment and infects plants but also people. There are several species of Burkholderia referred to as “B.cepacia complex” and some species appear to be more harmful than others. It can infect the lungs of people with CF and has been found on contaminated respiratory equipment. In the United States, less than 3% of people with CF are infected with Burkholderia, which makes it a less common bacterial infection in CF. However, it is very serious because it can lower lung function quickly and can be easily passed between people with CF.

The goal in practicing infection prevention and control measures is to minimize exposure to germs. Less exposure means less chance of infection. Your healthcare team at Lurie Children's want to work with you to reduce the spread of germs. There are ways to do this in the outpatient setting, in the hospital, at school, at home and in public. 


How Germs Spread

Let's begin with a review of the basics about the spread of germs that can cause infection. The three main ways germs spread are through contact, droplet and airborne transmission.

Contact Transmission

Contact transmission is the most common way germs are spread. There is direct contact and indirect contact.

Direct contact happens when a person transfers their germs to you by actually touching you with their germs. Kissing, hugging and shaking hands are good examples of direct contact.

Indirect contact occurs when you touch something that already has germs on it. Sharing a toothbrush or touching an item that may have germs on it — such as money, toys and doorknobs — are examples of indirect contact. If you touch your eyes, your nose or your mouth with dirty hands, you could indirectly have contact with these germs. That is why it is so important to wash your hands often.

Droplet Transmission

Germs can spread to others by traveling in the air on very small drops of liquid. When a person is coughing, sneezing, talking, laughing or even singing, droplets from their secretions can go into the air. These droplets can travel up to six feet. Droplet transmission happens if the droplets are carrying germs, and you are close enough for the germs to get in your eyes, nose or mouth. If you are within six feet, these droplets can land on you. Some germs in the droplets that land on surfaces can live for hours on a surface.

Airborne Transmission

Airborne transmission happens when germs are carried on tiny particles that are small enough to float in the air for a long time and travel long distances and a person breathes in the airborne germs. Some examples of diseases that are spread this way are tuberculosis (TB), measles, SARS and chickenpox. Molds and fungi can also infect a person through airborne transmission.

“Droplet nuclei” is a recent concern in CF. Droplet nuclei are much smaller droplets that can be produced when a person coughs. In experimental situations, it has been shown that people with CF do generate droplet nuclei when coughing and these droplets can stay suspended in the air for longer periods of time, depending upon the air currents in the room. Although there is no evidence that these droplet nuclei spread germs between people with CF, prevention measures are recommended by the Cystic Fibrosis Foundation. For example, in CF clinic the pulmonary function lab follows strict guidelines to clear the air in the PFT rooms between patients.

Remember: CF germs are spread by contact and droplet transmission. It is important to contain respiratory secretions in order to prevent the spread of germs.

Tips for Preventing Infections

Learn more about the ways to protect yourself from infections in a variety of different settings. 

What We Do

During your appointment, you will be escorted directly into an exam room. We want to avoid having our patients in the same area to reduce the risk of spreading germs between patients.

  • We clean and disinfect the exam room surfaces and the medical equipment — such as the pulse oximeter, the stethoscope and the pulmonary function devices — between patients.
  • We wash our hands or use alcohol-based hand sanitizers and gown and glove before and after contact with each patient.
  • We have measures in place in the Pulmonary Function Lab to assure that the air is clear of droplet nuclei between patients, such as the use of negative pressure or HEPA filters in the testing rooms.

What You Can Do

Put on a mask and wash your hands with soap and water or use alcohol-based hand sanitizers (hand gel) when you arrive for your appointment and when you leave. Alcohol-based hand sanitizers are very effective in killing germs on hands only when the hands are not visibly soiled. If hands look or feel dirty, then hand washing with soap and water is best.

If you or your child are coughing or sneezing, please use a tissue and throw it away in the trash. Afterwards, wash your hands again.

Remember: Washing your hands is one of the best things you can do to keep from getting sick and avoid spreading germs to others.

What We Do

All individuals with CF admitted to the hospital are placed in contact isolation.

Contact isolation means that we are taking precautions against the possible spread of germs from other patients and your surroundings.

All healthcare providers who enter your child’s isolation room must wash their hands or use the hand sanitizing gel and wear a clean gown and gloves. (If your child is in droplet and contact isolation, then the provider will also put on a mask). This protection works both ways; it protects from germs coming in and protects others from germs going out.

Transmission-based precautions for all patients is recommended, which is health care workers using appropriate barriers (e.g., gowning, gloves, mask) with anticipated exposure to bodily fluids.

Everyone is required to wash their hands before entering and leaving your room, including you and any visitors.

What You Can Do

Since you are placed in contact Isolation, you will not be permitted to leave your room unless you need to go for a test or procedure. Do not enter other patient rooms, especially a person with CF. Practice the six-foot rule when near any patient. Wear a mask when you leave your room, and change it when it becomes moist.

Wash your hands often and see that your child does as well. Always make sure hands are washed before eating, after coughing, sneezing or nose-blowing (after airway clearance therapy!), after bathroom use and before leaving the hospital room.

If you are not sure that a healthcare provider who has entered your room has washed their hands, ask them. This way you can be reassured that all precautions are being followed.

Remember: The hospital policy for infection prevention and control for patients with cystic fibrosis may seem strict and difficult at first, but it is for your child’s protection and safety.

What We Do

Your health and the health of your child is a private matter. Everyone's medical information is confidential, and it is a personal decision to share this information with others.

  • We can help you decide what information your school needs to know about your child’s care.
  • We can provide education about CF to the school and the school nurse and staff.
  • We have literature available for your school about CF.

What You Can Do

  • Children with CF may attend the same school; however, they should NOT share the same classroom if at all possible.
  • Teach your child to practice the six-foot rule if he happens to be near another student with CF.
  • Check to see if your child can keep alcohol hand sanitizers in his or her desk, backpack or locker. Encourage your child to use this between classes or before lunch whenever hand washing is not available.

Remember: Hand washing is the easiest and best way to prevent the spread of germs.

What You Can Do

Clean, disinfect and air-dry your respiratory equipment after each use. This would include any equipment that touches your child’s mouth and therefore may have germs on it from secretions. Your child’s nebulizer, PEP device and spacer all need to be cared for according to the manufacturer's directions. Please review cleaning and disinfecting instructions with your healthcare team.

Wash your hands prior to preparing your child’s medications. Prepare your child’s inhaled medications in a clean area in order to prevent contamination of the medication and of the nebulizer.

If there are other family members with CF, be careful not to spread your germs to one another. Do treatments separately if possible. Cough into a tissue and dispose of the tissue in the trash can, especially when you are doing your airway clearance therapy.

Do not allow your child to share any of his personal items, such as eating/drinking items, toothbrush or respiratory equipment. Keep your home clean and dry; excessive moisture in the home may breed bacteria, molds and fungi. Do not use a vaporizer.

What You Can Do

  • Teach your child to wash their hands well and often. Make sure they carry alcohol hand sanitizers.
  • Teach your child to cough, sneeze and blow their nose into tissue and dispose of the tissue properly; then wash hands.
  • Maintain a distance of at least six feet from others with CF or other people who appear to have an active infection, like a common cold.
  • Avoid prolonged contact with others with CF in an enclosed space, such as a long car ride or at a meeting in a small room.
  • People with CF should not attend indoor CF events such as Family Education Days because of the serious health risk to one another.
  • Avoid people who are sick.
  • See that your child gets all of his vaccinations, including the flu vaccine each year.

Remember: Every person benefits from understanding how germs spread.

Although it is important to practice ways to avoid “catching” germs, it is impossible to have complete control over your environment. Being educated about infection prevention and control practices and being reasonably cautious is the best you can do.