Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines

Abstract

Nutrition is integral to the care of individuals with cystic fibrosis (CF). Better nutritional status is associated with improved pulmonary function. In some individuals with CF, enteral tube feeding can be useful in achieving optimal nutritional status. Current nutrition guidelines do not include detailed recommendations for enteral tube feeding. The Cystic Fibrosis Foundation convened an expert panel to develop enteral tube feeding recommendations based on a systematic review of the evidence and expert opinion. These guidelines address when to consider enteral tube feeding, assessment of confounding causes of poor nutrition in CF, preparation of the patient for placement of the enteral feeding tube, management of the tube after placement and education about enteral feeding. These recommendations are intended to guide the CF care team, individuals with CF, and their families through the enteral tube feeding process.

Use of an In‐line Digestive Cartridge With Enteral Nutrition Improves the Weight Trajectory of 2 Children With Cystic Fibrosis Complicated by Another Medical Diagnosis

“…The use of oral enzymes with cyclic or continuous EN therapy has been based on best practice experience in an effort to maximize calorie absorption for people who otherwise cannot digest macronutrients in formula. Different techniques to administer enzymes with EN exist and vary among institutions . However, these techniques are not approved by the Food and Drug Administration (FDA) based on manufacturer labeling and have not been evaluated in a clinical trial .…”

“…Different techniques to administer enzymes with EN exist and vary among institutions . However, these techniques are not approved by the Food and Drug Administration (FDA) based on manufacturer labeling and have not been evaluated in a clinical trial . One manufacturer recently updated the package insert, outlining directions for administering pancrelipase microspheres through a gastrostomy tube (GT) .…”

Impact of nutritional status on pulmonary function after lung transplantation for cystic fibrosis

“…5 Vitamin D deficiency has been associated with impaired lung function in non-transplanted CF patients, 6,7 and might be associated with an increased risk of rejection and infections after LT. 8 Subsequently, nutritional support has been shown to defer impairment of pulmonary function and improve survival in LT candidates. [9][10][11][12] However, data on the impact of nutritional status on lung function in LT recipients are limited.…”

Percutaneous Endoscopic Gastrostomy Tubes May Be Associated With Preservation of Lung Function in Patients With Cystic Fibrosis

“…Cystic fibrosis (CF) is the most common life‐shortening autosomal recessive genetic disease in Caucasians, with an incidence of ≈1000 new cases per year in the U.S . Malnutrition is a common complication of CF because patients are often unable to meet their nutrition demands . Multifactorial mechanisms, including inadequate oral intake, increased energy requirements due to pulmonary disease, exocrine pancreatic insufficiency, and increased resting energy expenditure, contribute to increased caloric demands .…”

“…Optimization of the nutrition status of patients with CF is an integral part of their multidisciplinary care and is vital to improving long term outcomes . Enteral tube feeding, including surgical enteral tubes such as percutaneous endoscopic gastrostomy (PEG) tubes and nasoenteric tubes, has been used since the 1980s to supplement oral intake and improve growth in this population . By 2016, Cystic Fibrosis Foundation (CFF) guidelines have been adjusted to guide the care of patients aged 2–5 years during their optimal growth and development phase and emphasize the importance of nutrition, including possible use of enteral tubes, including PEG tubes …”