Update on Enteral Nutrition Support for Cystic Fibrosis

Abstract

Cystic fibrosis (CF) is an inherited disease affecting the respiratory, gastrointestinal, hepatobiliary, and reproductive systems. Nutrition status in persons with CF is often compromised due to increased energy needs, frequent infections, pancreatic insufficiency, lung disease, or CF-related diabetes. Maintaining good nutrition status has been associated with better pulmonary function, reduced hospitalizations, and increased longevity. Nutrition support as oral supplementation (used in >37% of the CF population) or tube feeding (used in >13% of the CF population) is often required for children and adults with CF. The purpose of this update is to describe current consensus and evidence for enteral nutrition support guidelines, reported complications of enteral feeding in the CF population, evidence of expected outcomes, and to discuss related areas requiring further research. A case report is provided to illustrate potential outcomes of aggressive enteral support.

Malnutrition in Cystic Fibrosis

“…Calculations used by nutrition professionals are just estimates and need to be adjusted according to actual intake and growth. It has been stated in multiple resources that the calorie needs of patients with CF are 110‐200% higher than the healthy population 4 , 40 – 42 . The 1992 nutrition assessment and management in CF: a consensus report provided recommendations on using a formula that took into account physical activity, pulmonary function and degree of malabsorption 41 …”

“…If all other nutrition related symptoms have been ruled out or treated and a patient is unable to meet nutrition needs, the third step is usually nutrition support. Nutrition support can be provided as oral supplementation (seen in >37% of the CF population) or tube feeding (seen in >13% of the CF population) as described in the update on enteral nutrition (EN) support for CF 40 . At this time there are no published guidelines on when it is appropriate to initiate gastrostomy tube (GT) placement and start tube feedings; however the 1992 CF nutrition consensus report recommended starting tube feeding if a patient was below 85% of their ideal body weight and failed other nutrition intervention 40 …”

“…CF patients with a GT for supplemental nutrition therapy typically get night time tube feeding providing about 50%‐75% of estimated energy needs 37 , 42 . GT feedings are well tolerated and helpful in improving nutrition status, but there are risks involved (decreased lung function, and poor pulmonary status) that will prevent weight gain even with EN supplementation 40 . Bradley et al conducted a retrospective study that found at 6 months post GT placement patients were 10 times more likely to reach a BMI at or above the 50th percentile.…”

“…Results from a small retrospective study demonstrated that GT feedings do not significantly improve lung function or decrease the number of pulmonary exacerbations 37 . Patients with advanced lung disease can have poor clinical outcomes following GT placement related to poor growth and little improvement of lung function; patients with less severe lung disease will receive a greater benefit from GT placement 37 , 40 . Young children with poor growth will benefit from early intervention with GT before malnutrition becomes an issue 40 …”

Chronic Illness and Disordered Eating: A Discussion of the Literature

“…Long-term treatment for these individuals includes increased energy intakes with 35% to 40% of total calories coming from fat, along with vitamin and mineral supplementation, pancreatic enzyme replacement therapy, and regular chest examinations (16). Sometimes oral and enteral tube feedings may be necessary if patients cannot meet the demands of nutrient needs from food sources (48). Additionally, 1 of 4 cystic fibrosis patients older than 20 y of age also experience the onset of type 1 diabetes, otherwise known as cystic fibrosis-related diabetes, which can make dietary management even more challenging (49).…”