If a patient’s weight gain is inadequate or appetite poor, dietary supplements may help to improve energy intake. A wide variety of supplements are available, including calorie only supplements e.g. Maxijul®, Polycal® , Duocal®; calorie and protein only supplements, e.g. Scandishake®, Calshake®; semi-complete supplements, e.g. Enlive® and Fortijuice® (fruit based), and nutritionally complete supplements e.g. Ensure Plus®, Entera®, Fortisip® (milk based). All are available on prescription.
The type and amount of supplement recommended depends on the patient’s age, preference and requirements, and should therefore be prescribed on an individual basis. Supplements should be taken in addition to normal food to increase total daily energy intake and should not replace a meal. Ideally they should be given with a snack between meals, as a drink after meals, or later in the evening. Multi-centre UK trials have shown that their use can promote significant weight gain (Skypala et al, 1998) and improvements in energy intake (Poustie et al, 2006). However, oral supplements should not be considered as an essential long term treatment in mildly malnourished children where dietary advice to optimise intake may be sufficient (Smyth & Walters, 2007). The efficacy of short term use of oral calorie supplements in the acute situation, or the long term use in adults or those with advanced lung disease, has not been fully assessed (Smyth & Walters, 2007). However, within our own adult clinic we have shown that use of supplements results in significantly greater energy and protein intakes (White et al, 2004).
Nasogastric and enterostomy feeds
Some patients, usually those with more severe chest involvement, may be unable to achieve or maintain an adequate energy intake and normal growth, even with a high fat diet and dietary supplements. Indications for more invasive forms of nutritional support include:
• Failure to achieve an adequate weight gain over a period of six months
• Weight-for-height below 90% (children)
• BMI less than 19 kg/m 2 (adults)
• Significant weight loss during an acute exacerbation
• Inadequate weight gain during pregnancy
Invasive nutritional support can be provided via a fine bore nasogastric tube (Holden et al, 1991) or a gastrostomy tube placed directly into the stomach (Steinkamp & von der Hardt, 1994). More rarely a jejunostomy tube (placed in the upper small bowel) may be required (Dalzell et al, 1992). The nasogastric tube can be left in place or passed each time a feed is given and feeding via this route has proved both effective and acceptable to patients (Bellwood et al, 1991). Gastrostomy feeding is now the preferred method of enteral tube feeding due to ease of endoscopic tube placement and the use of gastrostomy buttons. The incidence of gastrostomy feeding is reported as between eight and 20% of clinic populations (Bell et al, 1998; Richardson et al, 2000; Morton et al, 2000) and has increased steadily during the last 10 years. In Leeds, 13% of patients attending the paediatric unit and 12% of those attending the adult unit are currently tube fed, the majority via gastrostomies. This is double the incidence reported to the CF database from other centres in the UK (UK CF Database, 2003) and reflects our belief that optimising nutritional status is of paramount importance to help preserve lung function. Gastrostomy feeding has been shown to be more effective than nasogastric feeding in terms of resultant weight gain (Morton & Conway, 1996) and can stabilise lung function in the longer term (Morton et al, 2004; Efrati et al, 2006).
Early discussion and placement of a gastrostomy is an important factor in subsequent weight gain. Those with advanced disease may benefit less (Van Biervliet et al, 2004; Oliver et al, 2004). Although patients may express concerns regarding body image (Abbott et al, 2007) positive attitudes have also been reported to gastrostomy placement (Gunnell et al, 2005). Early discussion, allowing adequate time to make decisions regarding enteral tube feeding is therefore important.Enteral tube feeds are usually administered overnight, allowing the patient to eat normally through the day. Our patients seem to tolerate lower volumes of more highly concentrated preparations such as the whole protein feeds e.g. Ensure Plus®, Ensure TwoCal®, Fresubin TwoCal®, Fresubin HP Energy® or Nutrison Concentrated® , rather than more dilute feeds given at a higher rate, as is often necessary with elemental feeds. Whatever the type of feed used, careful monitoring of the patient’s blood glucose level is essential when feeds are first introduced. Hyperglycaemia (high blood glucose levels) requiring insulin therapy may occur in patients given night time enteral tube feeds (Smith et al, 1994), irrespective of the carbohydrate content (Kane & Black, 1989). This tendency is exacerbated if the patient is also receiving oral corticosteroids.
Orally administered pancreatic enzymes are necessary with all fat containing enteral tube feeds including elemental preparations and those containing medium chain triglyceride fat (Durie et al, 1980). As the feed is continuously administered over a long period of time, it is likely that the enzyme requirement is less than that calculated when comparing the fat content of the feed and the patient’s usual enzyme dose with food.
• Individualised use of dietary supplements may help to improve the overall calorie (energy) intake and nutritional quality of the diet
• Enteral tube feeding may be required if dietary intakes are insufficient to achieve a good nutritional status
Abbott J, Morton AM, Musson H, et al. Nutritional status, perceived body image and eating behaviours in adults with cystic fibrosis. Clin Nutr 2007; 26: 91-99. [PubMed]
Bell SC, Bowerman AR, Davies CA, et al. Nutrition in adults with cystic fibrosis. Clin Nutr 1998; 17: 211-215. [PubMed]
Bellwood E, Crosse BA, Conway SP. Enteral feeding in adolescent and adult cystic fibrosis patients. An argument for the nasogastric route. 17th European CF Conference, Copenhagen, 1991. Poster 137.
Dalzell AM, Shepherd RW, Dean B, et al. Nutritional rehabilitation in cystic fibrosis: a 5 year follow up study. J Pediatr Gastroenterol Nutr 1992; 15: 141-145. [PubMed]
Durie PR, Newth CJ, Forstner GG, et al. Malabsorption of medium chain triglycerides in infants with cystic fibrosis. Correction with pancreatic enzyme supplements. J Pediatr 1980; 96: 862-864. [PubMed]
Efrati O, Mei-Zahav M, Rivlin J, et al. Long term rehabilitation by gastrostomy in Israeli patients with cystic fibrosis: clinical outcome in advanced pulmonary disease. J Pediatr Gastroenterol Nutr 2006; 42: 222-228. [PubMed]
Gunnell S, Christensen NK, McDonald C, et al. Attitudes toward percutaneous endoscopic gastrostomy placement in cystic fibrosis patients. J Pediatr Gastroenterol Nutr 2005; 40: 334-338. [PubMed]
Holden CE, Puntis JWL, Charlton CPL, et al. Nasogastric feeding at home: acceptability and safety. Arch Dis Child 1991; 66: 148-151. [PubMed]
Kane RE, Black P. Glucose intolerance with low-, medium-, and high carbohydrate formulas during night time enteral feedings in cystic fibrosis patients. J Pediatr Gastroenterol Nutr 1989; 8: 321-326. [PubMed]
Morton A, Conway SP. Enteral feeding by gastrostomy is more effective than by nasogastric route in patients with cystic fibrosis. Israel J Med Sci 1996; 32(Suppl): S218.
Morton AM, Wolfe SP, White H, et al. Nutritional status of patients attending a Regional Cystic Fibrosis Unit. Abstract book XIIIth International Cystic Fibrosis Conference Proceedings 2000; 112.
Morton A, White H, Peckham D, et al. Effects of enteral tube feeding on clinical and nutritional status in adults with cystic fibrosis. J Cyst Fibros 2004; 3(Suppl 1): Abstract 310.
Oliver MR, Heine RG, Ng CH, et al. Factors affecting clinical outcome in gastrostomy-fed children with cystic fibrosis. Pediatr Pulmonol 2004; 37: 324-329. [PubMed]
Poustie VJ, Russell JE, Watling RM, et al. Oral protein energy supplements for children with cystic fibrosis: CALICO multicentre randomised controlled trial. BMJ 2006; 332: 632-636. [PubMed]
Richardson I, Nyulasi I, Cameron K, et al. Nutritional status of an adult cystic fibrosis population. Nutrition 2000; 16: 255-259. [PubMed]
Skypala IJ, Ashworth FA, Hodson ME, et al. Oral nutritional supplements promote significant weight gain in cystic fibrosis patients. J Hum Nutr Dietet 1998; 11: 95-104.
Smith DL, Clarke JM, Stableforth DE. A nocturnal nasogastric feeding programme in cystic fibrosis adults. J Hum Nutr Diet 1994; 7: 257-262.
Smyth R, Walters S. Oral calorie supplements for cystic fibrosis. Cochrane Database Syst Rev 2007; 1: CD000406. [PubMed]
Steinkamp G, von der Hardt H. Improvement of nutritional status and lung function after long-term nocturnal gastrostomy feedings in cystic fibrosis. J Pediatr 1994; 124: 244-249. [PubMed]
UK CF Database Annual Data Report 2003. [link]
Van Biervliet S, De Waele K, Van Winckel M, et al. Percutaneous endoscopic gastrostomy in cystic fibrosis: patient acceptance and effect of overnight tube feeding on nutritional status. Acta Gastroenterol Belg 2004; 67: 241-244. [PubMed]
White H, Morton AM, Peckham DG, et al. Dietary intakes in adult patients with cystic fibrosis – do they achieve guidelines. J Cyst Fibros 2004; 3: 1-7. [PubMed]