Moving Forward After
a CF Diagnosis

If your loved one just received a cystic fibrosis (CF) diagnosis, it’s best to talk to their CF care team to better understand their individual needs and develop a plan. Below you will find information to help navigate through the early stages of diagnosis and into your loved one's preschool years.

Month 1:

Learning about caregiving for a newborn with CF

This first month may be a big learning curve as your child’s care team walks you through addressing CF-related needs. Start by getting an understanding of diet, physiotherapy, and respiratory cultures below.

Diet
It’s important to ensure your child is gaining weight to maintain their health. A high-calorie, high-fat diet can help with gaining weight, which is made difficult with CF as buildup of CF-related mucus inhibits nutrients from being absorbed. Your care team will be there to guide you on what to feed your child and how to modify their diet to ensure they reach a healthy weight. What are some things you can discuss with your child’s care team?
- Nutritional supplements: Some children may benefit from nutritional supplements if they’re struggling to gain or maintain weight
- Salt and vitamin supplements: Salt and vitamin A, D, E, and K supplements may also be beneficial
- Exocrine pancreatic insufficiency (EPI): EPI is when the pancreas fails to produce enough enzymes to digest and absorb nutrients. This condition can be managed with pancreatic enzyme replacement therapy (PERT)
Physiotherapy
Chest physiotherapy (CPT) plays an integral role in the management of your child's CF, utilizing a mix of gravity and percussion (rhythmic tapping) to clear mucus from the lungs and keep them clear. By clapping or vibrating the chest, mucus loosens and moves to larger airways where it can be coughed out. Your child’s care team will help you learn these techniques early on. In preparation, it’s important to do CPT before your child eats to avoid reflux, and to ensure your child’s head is supported the entirety of treatment by placing them on a pillow on your lap.
Respiratory cultures
Respiratory cultures, also known as sputum cultures, can help a doctor determine what type of bacteria is in your child’s lungs to decide how to treat it. If your child is unable to cough up sputum on their own, your doctor may discuss using another method of collection with you. Your care team may repeat this process every 3 months, starting the first month after your child’s birth.

Action items:

Ask your child’s care team about treatment options for managing their CF
Schedule your child’s first respiratory culture
Get tips on chest physiotherapy

Month 3:

Getting familiar with the CF Center

Regular visits to the CF Center play an important part in monitoring and managing your child’s CF. By the third month, you should be familiar with visiting your child’s care team. While your child’s an infant, most of your visits will include a measurement of vital signs and a respiratory culture.

Between month 3 and 6, your child may have their first chest x-ray, serving as a tool to monitor possible structural changes in their lungs. Before turning 2 years old, another chest x-ray may be scheduled for your child.

Action items:

Schedule your child’s first chest x-ray and second respiratory culture
Learn more about regular CF Center visits

Month 6+:

Solid foods and the flu vaccine

By the time your child is 6 months old, they may start eating solid food. You may need to provide pancreatic enzymes to help digest and absorb certain foods. Discuss this with your child’s care team to develop an appropriate plan.

Action items:

Consider scheduling a flu vaccine for your child, as well as another respiratory culture
Ensure everyone in your household and caregivers receive their flu vaccine

Month 12:

Maintain care

Once your child is 1-year-old, you may start to think about a longer-term CF care routine. You can use all the knowledge you acquired over the year to help support your child and their CF.

Action items:

Talk to your care team about scheduling another chest x-ray and respiratory culture
Plan for the next year by taking a look at the Cystic Fibrosis Foundation’s Clinical Care Schedule for newborns to 5-year-olds

Years 2 to 5:

Continuing care for your preschooler

New challenges and opportunities come along as your child enters the preschool phase. Below you’ll find helpful tips on vaccines, respiratory health, airway clearance, and maintaining body weight.

Vaccines
Ensure your child has received the proper vaccinations, including their first dose of Pneumococcal polysaccharide vaccine (PPSV23). All of your household members and caregivers should also receive their annual flu vaccine.
Respiratory health
Continue to monitor your child’s respiratory health by checking in with their care team every couple of months and ensuring they undergo regular testing.
- Chest x-rays should be done at least every other year
- Chest computed tomography (CT) scans can be done every 2 to 3 years, using the lowest dose of radiation possible. Talk to your care team to see what is best for your child
- Throat cultures should be done at least every 3 months in younger children, as they are not able to cough up enough sputum to be studied
Airway clearance
Air clearance techniques acquired through CPT should be performed daily and increased if your child is ill, as directed by your care team. If your child has a pulmonary exacerbation (respiratory symptoms flare up), you may have to perform airway clearance techniques more often and provide antibiotics for your child.
Maintaining body weight
Check your child’s weight regularly to ensure they’re maintaining a healthy weight by doing any of the following methods:
- Weight-for-age: Your child should fall at or greater than the 10th percentile, which you reference through WHO’s weight-for-age standards
- Body mass index (BMI percentile): BMI is used to measure weight-for-height. Your child should be at or greater than the 50th percentile
- Calorie intake: Talk with your care team to determine the appropriate amount of calories, fat, and protein per day your child should be consuming

You should also review your child’s pancreatic enzyme replacement therapy (PERT) with their doctor, to see if they need to be changed as your child’s diet evolves as they grow older.

Talk to your child’s doctor right away if your child struggles to maintain a healthy weight. There are many strategies to assist your child in gaining weight effectively.

Stay ahead of the game!

Work with your care team to develop a treatment plan for your child as early as possible. Getting familiar with CF for both you and your child at a young age is important for effectively managing CF and helping them take charge of their own care in the future.

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