Nutrition – Patient Task Force Meeting

By: Melissa Shiffman

Growing up, I had an unhealthy relationship with food. My brother and I both were diagnosed with Cystic Fibrosis as children. He was pancreatic insufficient, painfully thin and took handfuls of enzymes. Meal times were filled with constant fighting as my parents tried to force him to eat. Meanwhile, I was pancreatic sufficient with a very healthy appetite. Our body shapes could not be more different! He was skin and bones and I was chubby. As I got older, “chubby” was socially unacceptable. I was twelve the first time I went on the “hotdog diet!”

I have no real memory of fresh fruit and vegetables being readily available. Instead, our refrigerator was filled with high calorie, high fat foods to encourage my brother to eat: Kit Kats, Nestle Crunch, ice cream, Sustacal puddings. I was told not to eat his food, so I snuck it and ate in my room. I gained more weight. My brother made fat faces to get under my skin as I sucked in my cheeks back to him.

Like a normal pre-teen, I became self conscious about my appearance.Everyone talked about fad diets and losing weight. I absorbed that being thin was ideal. By the end of 8th grade, I convinced myself that, if I read the ingredients in things like Oreos, I would find a way to be disgusted. Eventually, I talked myself out of eating most foods except for grapefruit and canned tuna without mayo. I ate minimally and cut out sweets. I lost a lot of weight, started to look more like my brother (but less sickly) and revelled in the compliments on my weight loss.  

I know this is not the normal CF story. However, it is so important to recognize our relationship with food and to rectify our lack of education on eating well when you are underweight or gaining weight on modulators. Therefore, I am looking forward to Dr.Tonja Gonska (gastroenterologist at Sick Kids Hospital in Toronto) discussing healthy eating vs. high fat/high caloric intake, body composition vs. BMI and how nutrition plays a role in healthy weight gain for women with CF at the CFReSHC Patient Task Force meeting on Thursday March 21, 2019 from 3-5 pm EST.

Attendees can share their personal experiences, help develop potential research questions to be addressed by the CFReSHC research advisory panel and receive an Amazon gift card for their time.  For more information on CFReSHC and for login information email CFReSHC at info@CFReSHC.org.  

Mutations in Genes Regulating Digestion Prevalent in CF Patients with Pancreatitis

By Vijaya Iyer

In addition to mutations in the CFTR gene, cystic fibrosis (CF) patients with pancreatitis also have a high prevalence of mutations in genes regulating pancreatic function, according to researchers.

Conducted by a research team in Italy, the study, “Trans-heterozygosity for mutations enhances the risk of recurrent/chronic pancreatitis in patients with Cystic Fibrosis,” was published in the journal Molecular Medicine Continue reading Mutations in Genes Regulating Digestion Prevalent in CF Patients with Pancreatitis

Making it Matter Podcast – CF Nutrition

Making it Matter Podcast Ep. 10 – CF Nutrition

Nutrition is something I love to talk about (well…. ever since I got my feeding tube placed in 2011). It is a vital part of CF care, something that Julia and I approach differently. While my pancreas doesn’t seem to work, Julia knows she is Continue reading Making it Matter Podcast – CF Nutrition

UDCA Supplementation Enhances Lipid Digestion and Absorption in Pancreatic Insufficient Patients with Cystic Fibrosis

http://www.hcplive.com/

Results from a trial involving 23 cystic fibrosis patients with pancreatic insufficiency and mild liver involvement indicate that supplementation with ursodeoxycholic acid can increase the digestion and absorption of fat. Continue reading UDCA Supplementation Enhances Lipid Digestion and Absorption in Pancreatic Insufficient Patients with Cystic Fibrosis

Galapagos starts SAPHIRA Phase 2 study with GLPG1837 in cystic fibrosis patients

Galapagos NV (Euronext & NASDAQ: GLPG) announced today the first dosing in its Phase 2 exploratory program of GLPG1837 in patients with cystic fibrosis (CF).

GLPG1837 is a candidate CFTR potentiator drug in clinical development for the treatment of Class III mutations in cystic fibrosis. The SAPHIRA Phase 2 Continue reading Galapagos starts SAPHIRA Phase 2 study with GLPG1837 in cystic fibrosis patients

Pancreas Transplant for Cystic Fibrosis Patients with Exocrine Insufficiency

An analysis of organ transplant records between 1987 and 2014 suggests that cystic fibrosis patients who lose pancreatic function are failing to avail themselves of operations that could replace their damaged organs and restore their ability to digest food and manage blood sugar. Continue reading Pancreas Transplant for Cystic Fibrosis Patients with Exocrine Insufficiency

Pancreatic Enzyme Products May Benefit Cystic Fibrosis Patients with Malabsorption

After the Food and Drug Administration (FDA) issued rules requiring approval of pancreatic enzyme products (PEPs) in 2004, the cost and availability of the products added another hardship to the lives of cystic fibrosis patients, some of whom use PEPs to prevent uncomfortable and Continue reading Pancreatic Enzyme Products May Benefit Cystic Fibrosis Patients with Malabsorption