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:

• Data on Immobilized Lipase Cartridge for Patients with CF
• Cystic Fibrosis Expert Update 2017
• Nutrition Pearls: Fiber and Cystic Fibrosis
• Big Advance for Cystic Fibrosis -Who Will Benefit?
• Nutrition Guidelines for Cystic Fibrosis
• Complex Family of CFTR-Associated Disorders | gutsandgrowth
• N2U -Part 3: EoE, IBD, and Cystic Fibrosis | gutsandgrowth
• “Origins of Cystic Fibrosis Lung Disease” | gutsandgrowth
• Breaking down lung defenses in Cystic Fibrosis | gutsandgrowth
• Fluctuating Elastase Levels in Infants with Cystic Fibrosis …