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CFTR Modulator Drugs

Modulators for Infants

CFTR modulators are not routinely available for babies, with most being accessible to children aged 6 years and older. Currently Kalydeco is the only modulator for younger babies, being available to eligible babies aged 1 month and older, while Orkambi is available to eligible babies from 12 months of age. Both need to be upon referral from your CF team.

Dosage

Kalydeco and Orkambi are both supplied as oral granules for babies. They are available in different strengths, and your CF care team will prescribe the appropriate dose based on your baby’s weight.

How is it given?

CFTR modulators need fat to be absorbed properly. For babies, this typically means giving the dose with:

  • Breastmilk
  • Formula or
  • Puree (when solids start)

Give them immediately before feeding.

Changes/benefits

Possible benefits of Kalydeco/Orkambi to babies can include:

  • Improved fat absorption
  • Better weight gain
  • Reduced need for very high-calorie strategies

Monitoring

You CF team will closely monitor your baby while taking Kalydeco/Orkambi. They will monitor:

  • Liver function (blood test)
  • Growth (weight/length)
  • Stool patterns (digestion)
  • Tolerance (other side effects)

Based on these, they will decide if any changes need to be made to their nutritional or medication requirements. Be sure to let the team know of any side effects you notice or concerns you might have.

More information

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