CFTR Modulator Drugs
Many children with CF are now eligible for CFTR modulator medications. Modulators can improve pancreatic function, and as a result your child’s nutritional needs may change when they commence this medication.
Your child may start absorbing more fat and nutrients from food and find it easier to gain weight. As a result, we have seen a shift amongst CF dietitians away from the traditional CF diet of high fat, high calorie, high salt food, that was once recommended to compensate for malabsorption and increased energy needs.
New recommendations are moving towards an individual approach that encourages a balanced nutrient-dense diet to support long-term health. Working closely with your child’s CF dietitian and multidisciplinary team to monitor their nutritional needs can help you find a balance that best supports your child.
Taking Modulator Medications
Modulators need to be taken with a meal or snack that contains fat to help the body absorb the medicine. Examples include foods that contain milk, yoghurt, cheese, eggs, avocado, peanut butter, or a meal with oil or butter.
Most modulators are taken morning and evening and work best when they are taken at approximately the same time each day. They should be taken consistently, even when your child feels well.
Changes to Medications
For some children, CFTR modulators can change how their body works and may affect their dosages of other medications, such as pancreatic enzymes, vitamin supplements and salt. These needs can change over time and should be regularly monitored by your child’s CF care team.
Enzymes
A small stool sample, knows as a faecal elastase test, is a simple test used to check how well the pancreas is producing digestive enzymes. It may be performed before or around the time your child starts a modulator to provide a baseline and then repeated around six months later to see whether their pancreatic function has changed. Ongoing testing may also be recommended as part of their regular review.
It is important to remember that improvements in pancreatic function may not always be permanent. If your child is able to reduce or stop enzymes, their pancreatic function will continue to be monitored, as they may need to restart enzymes in the future. Never reduce or stop your child’s enzymes without guidance from their CF care team.
For some children, modulator medicines can improve how the pancreas works, meaning they may be able to reduce or sometimes even stop enzyme replacement therapy under medical guidance.
Modulator therapy can also influence how well the body absorbs vitamins and how much salt is lost through sweat. These changes will be monitored during clinic visits, and any medication adjustments will be advised by your CF care team.
Salt & Vitamins
Modulator therapy can also influence how well the body absorbs vitamins and how much salt is lost through sweat. These changes will be monitored during clinic visits, and any medication adjustments will be advised by your CF care team.
Bone Health
Children with CF, even those on modulators, are at increased risk of reduced bone mineral density (BMD) for a range of reasons. Therefore, good nutrition during childhood is particularly important, as this is when children are building the strong bones they will carry into adulthood.
CF teams are now monitoring children from a younger age (8 years) in order to detect low BMD and implement strategies to target this prior to puberty. This might include reviewing their diet and enzyme/vitamin supplements to ensure they are being absorbed effectively as well as encouraging regular weight bearing exercises.
BMD is measured through a DEXA scan. Depending on your child’s results, they may be reviewed annually, 3 yearly or 5 yearly.
Bowel Health
CFTR modulators may also have positive effects on the digestive system by reducing gut inflammation, minimising bloating and reflux symptoms and supporting an overall healthier balance of gut bacteria.
Some children may notice changes in digestion or bowel habits, however, these changes vary from child to child, and their CF care team will continue to monitor closely. A diet high in fibre and fluid is important to encourage good bowel health.
More Information
Click on the links below for more information and recipe ideas.
For more information, discuss with your CF dietitian.