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
- 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
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