CHOA Nutrition Support Lecture: Cystic Fibrosis Nutrition -Changing in the Age of ‘Miracle Drug’

Recently a terrific review of Cystic Fibrosis and Nutrition was presented at our CHOA nutritional colloquium.

To access this ~45 minute webinar: Next Nutrition Support Colloquium Webex on Tuesday, 2/23, at 1300-1400: “Nutrition and Cystic Fibrosis”-20210223 1812-1

Password: 5DgsMXqY

Key points:

  • Advances in Cystic Fibrosis (CF) have led to improvement in longevity, now ~46 years in 2019
  • Newer CFTR modulator therapies need to be aligned to the specific mutations. With new therapies, a high calorie diet may not be required and instead follow a standard healthy balanced diet.
    • These agents appear to be driving a big drop in hospitalizations
    • Now some patients need counseling for obesity and NAFLD
  • Better nutrition has been linked to better lung outcomes.
  • Pancreatic enzyme replacement therapy needed for 85-90% of patients with CF
    • CFTR modulator therapies may allow lower dosing PERT dosing
  • Fat soluble vitamin supplementation (A, D, E, K) is needed
    • Vitamin D (25-OH) goal (by CF Foundation) is >30 ng/mL
  • High salt diet is recommended due to excessive losses
  • Targeted nutritional support needs to be based on nutritional status; some patients may need G-Tube placement. CHOA algorithm developed.
  • High sugar diet may increase the development of CF related diabetes (CFRD)
  • Fibrosing colonopathy is a very rare effect of high dose pancreatic enzymes and may have been in part due to impurities in older preparations of panrcreatic enzymes.

Some of the slides:

Related blog posts:

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