Caring for Someone With CF

Caring for someone with cystic fibrosis (CF) as a parent or caregiver? There are ways to adapt your approach as they move through different stages of their life.

Keep in mind that it’s always a good idea to meet with the professionals at your child’s CF Care Center to develop a care plan that fits your child’s specific needs. Visiting your child’s CF Care Center on a regular basis is a crucial part of managing their health.

Here are some age-specific tips to keep top of mind while providing care for your loved one.

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Infants

There’s a lot to learn as a parent of a newborn with CF. To help make sense of it all, here is a timeline of some things your child’s care team may have told you to expect during the first year.

Learning about CF caregiving

"1 month icon

During this first month, the care team will help you learn how to address your child’s CF-related needs. This includes diet, physiotherapy, and respiratory cultures.

Diet

It's important to make sure your child is gaining enough weight so they can maintain their health. If your child isn't gaining weight, they may need a high-calorie diet to help them catch up. This helps to combat the CF-related mucus that clogs the pancreas and inhibits nutrients from being absorbed.

The care team will guide you on what to feed your child and how to adjust their diet to make sure they achieve a healthy weight.

Here are a few things to discuss with your child's healthcare provider:

  • Some children may benefit from nutritional supplements if they are not gaining or maintaining weight
  • Many children with CF may also benefit from salt supplements and vitamin A, D, E, and K supplements
  • Some children with CF may develop exocrine pancreatic insufficiency (EPI), where the pancreas doesn’t produce enough enzymes to break down and absorb nutrients. This can be managed with pancreatic enzyme replacement therapy (PERT)

Be sure to talk with your child’s care team about supplements, PERT, and how to best support their nutritional needs.

Physiotherapy

Keeping the lungs clear is a big part of managing your child’s CF. One way to do this is through chest physiotherapy, which your child's care team will help you learn early on—as soon as a week after their diagnosis.

Chest physiotherapy uses a combination of gravity and percussion to clear mucus from the lungs. Clapping or vibrating your child’s chest will loosen mucus and move it to larger airways where your child can cough it out of the body.

Here are some airway clearance tips that may help:

  • Always do it before feeding your child to avoid reflux
  • Make sure your child’s head is fully supported during treatment

Respiratory cultures

Your child’s healthcare provider may collect a respiratory culture (also known as sputum culture) by having them cough up sputum for bacterial analysis. If your child isn’t coughing to produce sputum on their own, the healthcare provider may use a swab to collect a sample for culture from the back of their mouth.

These cultures allow your child’s healthcare provider to understand what type of bacteria is in the lungs so they can decide how to treat it. Your care team may perform a culture every 3 months, starting in your child’s first month after birth.

Action items:

  • Talk to your child’s care team about scheduling their first respiratory culture
  • Get tips on physiotherapy

Getting to know the CF Center

"3 month icon

By the third month, you should be familiar with visiting your child's care team at the CF Center. Regular visits to the center are an important part of monitoring and managing your child's CF. 

As an infant, most of your child's visits will include:

  • Measurement of vital signs 
  • A respiratory culture

Sometime between 3 and 6 months, your child may also have their first chest x-ray at a clinic visit. They may have another chest x-ray before they turn 2. Chest x-rays are used to monitor possible changes in your child's lungs.

Next steps:

  • Talk with the care team about scheduling your child’s first chest x-ray and second respiratory culture
  • Learn more about regular CF Center visits

Solid foods & the flu vaccine

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By 6-months-old, your child may start eating solid foods. You may need to provide enzymes with certain foods. Talk to your child's care team to discuss an appropriate plan. 

Action items:

  • Consider scheduling your child’s seasonal flu vaccine and another respiratory culture around 6 months
  • Ensure everyone in your family receives their seasonal flu vaccine

Maintaining care

"12 month icon

By the time your child is 1-year-old, you may be thinking about a longer term CF care routine. You can build on all the knowledge you’ve gained throughout the year to help your child.

Follow up:

  • Talk with your child’s doctor about scheduling another chest x-ray and another respiratory culture
  • Plan for the year ahead by taking another look at the Cystic Fibrosis Foundation’s Clinical Care Schedule for newborns to 5-year-olds

If you want more information about your child’s first year of life with CF, talk to their CF care team.

CF fact or fiction
Your CF Care Center will teach you how to perform airway clearance techniques after your child turns 1.
Fact
or
Fiction
CF Fiction CF Fact

Here's the fact: Your care team will help you perform airway clearance techniques as soon as a week after your child's diagnosis.

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Children Ages 2-5

Continuing to care for your child in these early years brings a new set of challenges and opportunities. Here are some important things to keep in mind along the way:


 

Stay up to date with vaccinations. Make sure your child gets routine immunizations, including the first dose of Pneumococcal polysaccharide vaccine (PPSV23). You, all the members of your household, and all caregivers should also receive the annual influenza vaccinations


 

Monitor your child’s respiratory health. Check in every few months with your child’s care team to talk about your care plans and treatment goals. Take your child in for regular testing, including:

  • Spirometry tests. These will help your care team check for pulmonary exacerbations and see how your child is responding to treatments. Healthcare providers may start to perform these tests with children at 3 years of age. However, young children may not be able to perform it at this age. Once your child is able to do the test, it is recommended that it be performed at every visit

  • Chest x-rays. These should be done at least every other year

  • Chest computed tomography (CT) scans. You and your care team may decide to have your child get a CT scan every 2 to 3 years, using the lowest dose of radiation possible

  • Throat cultures. These tests are done on younger children because they are not able to cough up enough sputum into a cup to be studied. A throat culture should be done every 3 months to monitor the microorganisms in your child’s airways

 


 

Perform airway clearance techniques daily and increase when ill as directed by your care team

When your child experiences a pulmonary exacerbation, you may need to:

  • Perform airway clearance techniques more frequently

  • Give your child oral, inhaled, and/or intravenous (IV) antibiotics

Learn more about pulmonary exacerbations

 


 

Check your child’s weight regularly. There are many different ways to make sure your child is maintaining a healthy weight, including:

  • Weight-for-age. Your child should be within the 10th percentile

  • Body mass index (BMI percentile). This can be used to check your child’s weight-for-height. Your child should be within the 50th percentile

  • Calorie intake. Discuss with your care team nutritionist what the appropriate amount of calories, protein, and fat per day is for your loved one

 

If your child is not able to maintain a healthy weight, talk with your healthcare provider right away. There are many ways you can help your child gain weight.

Learn about CF

Getting your child familiar with CF from an early age is crucial to helping them take control of their care later. This educational series offers a fun and engaging way to learn all about CF.

 

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Children Ages 6-12

As your child continues to grow and eventually reaches their pre-teen years, they’ll become more aware of their CF and how it affects their life. Here are a few tips to help you navigate the changes you may see during this time.


 

Help your child learn what causes CF and the importance of knowing about their disease and their genotype. Take a deeper look

 

Begin to teach your child about how CF impacts the body
 

 

Encourage your child to participate more actively in their care. You can start by allowing them to take medicines and do vest therapy on their own while you observe

 

Help your child learn why it is important to perform airway clearance techniques. Learn how CF affects the lungs 

Support for Your Child at School

Keep your child's teachers in the know about CF by sharing this guide at the start of the school year.

 

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Children Ages 13-17

The teenage years are hard enough when you don't have CF; having CF only makes things harder. Here are a few tips to consider as you continue to provide your best care for a loved one on the brink of adulthood.


 

Encourage your teen to play an active role during appointments by:

  • Asking and answering questions on their own

  • Knowing the reasons for yearly checkups and tests

  • Learning the right food options from a nutritionist


     


 

Begin the steps for transitioning your teen from pediatric to adult care by:

  • Having your teen connect via phone, text, and/or video chat with an adult with CF to help guide them in this transition. The CF care team can help find a person to share their CF experiences and successes with your loved one

  • Encouraging them to take more responsibility—and trusting them to start taking care of their health on their own. For example, they should know the names of their medications, and they should be honest and forthcoming when talking about their symptoms with you and their care team

  • Getting to know the adult center you’re considering. Not all adult centers offer the same exact services, so it’s important to find out what specialists and services are available at this center
     


 

Teach your teen to recognize when their symptoms get worse and how a pulmonary exacerbation can lead to lung damage

Learn how pulmonary exacerbations lead to lung damage

 


 

Help your teen keep up with their digestive health by:

  • Reminding them to take their digestive enzymes. Most teens need to take enzymes to help them with digestion. If they miss a dose of their enzymes, they may have abdominal pain, constipation, or even a type of acute intestinal blockage called distal intestinal obstruction syndrome (DIOS)

 


 

Be on the lookout for diabetes by:

  • Knowing which signs and symptoms to watch out for. Roughly 15% to 20% of teens with CF will develop diabetes, which can cause them to lose calories, weight, and energy. It may also cause a drop in lung function. Your teen may feel drained without knowing why, may feel thirsty all the time, or may have to get up to use the bathroom in the middle of the night. Talk to your care team right away if you or your teen notices any of these symptoms. Even if your teen does not have any of these symptoms, they should be screened once a year for diabetes.

    Learn more about cystic fibrosis-related diabetes (CFRD) here

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Adults

As your loved one reaches adulthood, they might already have a good understanding of their CF. The key is to make sure they are equipped to manage their own care as they become fully independent.

Communicate with your loved one’s care teams. Talk to your loved one’s pediatric care team and adult care team, and make sure they are both communicating with one another

 

Prepare your loved one for the transition to adult care. Try to talk to your loved one about how their care will change as they get older—and have these conversations as early as you can

 

Give your loved one more responsibility over time. Encourage your loved one to keep learning everything they can about CF, practice self-care, and make decisions about their health. Taking more of an active role in managing CF is just one of the many ways they can become more independent!

 

Take it slow. Some young adults may be more eager for independence than others. It might be a good idea to accompany your loved one to their first visit at their adult care center  

 

Make sure your loved one knows:

  • How to fit their treatments into their daily routine

  • What their medications do, the potential side effects, and how to get refills

  • About lung function and how to prevent infections Learn about the impact of CF on the lungs

  • The signs and symptoms of cystic fibrosis-related diabetes (CFRD)  Learn more about CFRD

  • When they need to call the healthcare provider

 

Encourage your loved one to manage their CF by:

  • Getting sputum cultures every few months

  • Having posterior/anterior and lateral chest X-rays every 2 to 4 years, or as needed (for example, during pulmonary exacerbations)

  • Performing airway clearance and exercising regularly to keep their lungs healthy

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