Health Maintenance Recommendations for Infants with Cystic Fibrosis

 1. Regular clinic visits: Frequent visits with the CF team when your infant is young are needed for close monitoring of growth and nutrition. These visits also allow time for the CF team to teach you how all the aspects of care involved in keeping your infant healthy. 

Anticipate that visits will be scheduled based on your infant’s needs:

      Every 2-4 weeks until about 3 months of age.

      Every 1-2 months until about one year of age.

      Every 2-3 months from one year of age forward.

 This is just a rough guideline for your reference.  Since each CF clinic and child is different, the timing for your next visit will be determined as you are leaving each visit. 

2. Laboratory Evaluations: While we know having blood drawn can be stressful for both infants and parents there are certain labs that are important to do to help your CF Team  best care for your child. These are usually done between 3 and 6 months of age and routinely every year as determined by your CF Center's guidelines.

    Blood tests:

1. Vitamin levels: Fat soluble vitamins can be poorly absorbed in babies with CF, but are very important for infant development.  Vitamin A, E and D levels will be checked.  Vitamin K is also a fat soluble vitamin but there is not a test for Vitamin K levels. 
2. Kidney function: Many medications are processed in the kidneys.  In order to make sure the kidneys are working well and can process any medications that your child is taking, kidney function should be checked.
3. Liver function: The liver can have a back-up of thick secretions that can make affect how well it works. Many medications are also processed in the liver, so it's important to check liver function.
4. Nutrition tests:  Your CF Team will monitor your child’s growth closely  but there are also blood tests to check that your child has enough protein to build muscle and grow normally.
5. Blood counts: Red blood cells are checked because children with CF can have anemia (not enough red blood cells to carry oxygen).  White blood cells are checked because a change in them can mean infections.  Platelets are also checked as a marker of inflammation and to see how well your child’s blood clots.   

       Stool studies:

Elastase: This checks to see how well the pancreas is working to absorb nutrients. Most infants with CF do not have the ability to absorb all nutrients because their pancreas does not work correctly.  Usually this happens shortly after birth. However, some children do not lose the ability of their pancreas to work correctly until later in the first year of life.  Depending on your CF Center, this test will be done when your child is diagnosed and repeated again in the first year of life if it is normal the first time it is checked.  

3. Evaluations of Lung Health:

1. Respiratory cultures: Children with CF can have many different bacteria (germs) in their airways.  It is not easy to get a culture from the lungs so a cotton throat swab is used to see what bacteria might be in the airways. It is important to know what bacteria are in your child’s airways so we can give them the right medications when they are sick.  Respiratory cultures are repeated every 3 months and/or with any new respiratory symptoms.
2. Pulmonary Function Testing (PFTs): This is a test of lung volume and function.  A special machine is used for infants.  The first test is done at 4 months of age and then repeated every 6 months depending on your CF Center's guidelines and equipment availability.
3. Chest X-ray: An x-ray of your child’s lungs are generally taken in the first year of life. This helps your CF Team monitor any changes in the lungs over time. 

This information was kindly provided by The Cystic Fibrosis Center at Children’s Memorial Medical Center in Chicago. It is based on the Current Clinical Practice Guidelines as established by the CF Foundation for accredited care centers as of 2010 and is subject to revision. Check with your CF Team for the most current guidelines.