Cystic Fibrosis by Amanda Seguin, Dietetic Intern

     While working with the pediatric dietitians during my internship, I’ve gained experience working in the cystic fibrosis (CF) clinic.  CF is a genetic disease that affects 30,000 people in the United States and most commonly affects the Caucasian population, though it can affect all races.  This disease causes mucus to build up in the lungs and the pancreas, along with many other organs.
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     While there is no cure for CF, there are many therapies patients can go through to help alleviate symptoms like, airway clearance treatments and pancreatic enzyme supplementation, but it is very important to have a good nutrition status.  Typically, there is a connection between higher body weights and improved lung function.
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     Many CF patients perform airway clearance treatment at least one time per day.  These treatments help patients breathe easier as it
 loosens the thick, sticky lung mucus so it can be cleared by coughing.  It is important to clear airways to reduce the risk of lung infections while improving lung function.  Another great way to break-up the mucus is by exercising.
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     Children and adults with pancreatic insufficiency must take pancreatic enzyme supplements with every meal and snack to improve digestion and absorption of the food they eat.  People with cystic fibrosis have higher nutritional needs due to inappropriate release of enzymes, therefore, a high-calorie and high-protein diet is beneficial for growth and development.  Drinking supplements with regular meals and snacks is encouraged to help meet nutrition needs, along with compliance of pancreatic enzyme regimen.  Because of the decreased absorption, CF patients must also take a daily multi-vitamin along with fat-soluble vitamins in their water-soluble for
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     Many complications improve with compliance of medication regimen, exercise, and eating appropriately.  Overall, I’ve enjoyed my experience working with the clinic and it is interesting to see how the nutrition status can change from patient to patient.
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Resource: Cystic Fibrosis Foundation, http://www.cff.org

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