3 Nutritional Challenges in Special Conditions and Diseases Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
3.19 Nutrition in Cystic Fibrosis Michael Wilschanski
Cystic fibrosis · Nutritional status · Pancreatic enzymes · Gastrostomy
Key Messages • Survival from cystic fibrosis (CF) has substantially improved over the past four decades. The advance in nutritional management is one factor which has contributed to this change • This chapter reviews the basic defect of CF and how it influences nutritional status • Normal growth and development can be achieved in CF, and to this end, nutritional counseling is paramount at all ages. The prevention and early detection of growth failure is the key to successful nutritional counseling • An approach to the CF patient who is not thriving and an outline of nutritional management is pro© 2015 S. Karger AG, Basel posed
Introduction
Cystic fibrosis (CF) is the most common lifethreatening autosomal recessive disease in Caucasians with an incidence of 1 in 2,500 live births. The disease is caused by mutations in the cftr gene on chromosome 7, which codes for a cAMP-regulated chloride channel [1]. Nonfunctioning CFTR protein affects epithelial ion and water
transport in a variety of organs including the respiratory, gastrointestinal, hepatobiliary, reproductive and sweat glands. The lack of CFTR function in the pancreatic duct is responsible for obstruction and autodigestion of the pancreas in utero, leading to exocrine pancreatic insufficiency (PI) in 85% of CF infants. The early growth of infants with PI due to CF is dependent on the age at diagnosis. Clinical diagnosis may be difficult unless meconium ileus occurs, typically in only 15% of cases. The remaining patients are diagnosed later, mainly presenting with failure to thrive with steatorrhea, accompanied in some cases with respiratory symptoms. An increasing number of countries have initiated newborn screening for CF using a serum marker of PI, and this has been shown to facilitate an earlier diagnosis with better growth and nutritional status [2]. Longer-term studies after neonatal screening are now revealing reduced pulmonary disease progression [3]. Numerous studies have shown that underweight and poor linear growth in children and malnutrition in adults are independent factors predicting mortality [4, 5]. Together with this, undernutrition has been shown to have an adverse effect on the outcome of lung transplantation [6]. These data reinforce the importance of the prevention and early detection of growth failure, leading to the aggressive management of nuDownloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
Key Words
Infants
The evaluation of an infant with CF should be expedited. If PI is established by tests for steatorrhea and indirect pancreatic function tests, pancreatic enzyme replacement therapy should be initiated as soon as possible. Breast milk can provide complete nutritional support to infants with CF for the first 4–6 months of age, though supplemental energy may sometimes be required by fortifying a portion of the breast milk feeds with formula or by fortifying formulas to a more concentrated energy level for those infants on a combination of breast milk and formula or on formula alone [9]. Regular cow’s milk-based infant formulas can be used if breastfeeding is not an option or if supplementation is required; there is no need for a predigested formula in most instances. Enzymes are given with all foods and milk products including predigested formulas containing medium-chain triglyceride. Infants require powder, which should be taken with fruit sauce with lubricant pretreatment to the mouth and perianal area to avoid skin excoriation. The enzymes should be administered at the beginning of and during the meal. The initial dose of enzymes should be approximately 5,000 IU lipase/ kg/day. The dose may be gradually increased according to symptoms and objective assessment of growth and fat absorption. In many instances, the caloric density needs to be increased, and this may be achieved by fortifying breast milk, adding fat or carbohydrate or concentrating the formula. Once solid food is introduced, enzymes should be titrated by fat intake. The maximum dose is 10,000 IU lipase/kg/day. Fat-soluble vitamin (ADEK) supplementation should be initiated according to the current recommendations [7, 8]. New guidelines from the North American Cystic Fibrosis Foundation Committee on Vita-
Table 1. Recommended dietary macronutrient composi-
tion in CF and non-CF patients (% of energy intake)
Protein Carbohydrate Fat
Non-CF
CF
10 – 15 55 – 60 30
15 35 – 40 45 – 50
min D advise higher supplemental amounts of vitamin D than are found in the currently available CF vitamin supplements. Additional vitamin D supplements are therefore recommended to keep the lower limit of 25-hydroxyvitamin D at 30 ng/ ml (75 nmol/l) [10]. Yearly monitoring of serum vitamin levels is recommended for vitamin A, vitamin E and vitamin D. Hyponatremic alkalosis may occur in infants, especially during the summer months; supplementation with sodium chloride is recommended. Zinc supplementation may be considered in the child who is not thriving [9].
Toddlers
As infants are introduced to table foods, it is important that the diet be balanced, with moderately increased fat and protein content (table 1). Parents need to be in control, routinely adding calories to maintain growth. The child with CF should avoid low-fat food and ‘grazing’. The dietician should promote positive interactions around meals. The mealtime must not turn into a battleground which is the catalyst for poor feeding behavior.
3
School-Age Children
School age is the age at which encourage the child to obtain a basic knowledge of the physiological processes, eventually leading to taking increasing responsibility for practical enzyme and nutritional management.
Nutrition in Cystic Fibrosis
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
245
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
tritional deficits at all ages. This has led to the publication of nutritional guidelines in Europe and in North America [7, 8].
Pancreatic Intestinal Biliary
Cellular defect?
Needs
+
Losses
Iatrogenic Psychogenic Gastrointestinal
+
Intake
Anorexia
Vomiting
Energy deficit
Pulmonary infections
Weight loss
Respiratory muscles
Lung parenchyma
Immune dysfunction
Deteriorating lung function
Fig. 1. The pathogenesis of energy imbalance in CF.
This stage is associated with increased growth, puberty and increased physical activity. This adds up to markedly increased nutritional requirements which are often difficult to attain. Pulmonary infections are more common, as is the onset of CF-related diabetes and, in a small minority of cases, CF-related liver disease. Female patients are at a greater risk of nutritional failure at this age [11]. This may be partly due to dissatisfaction with weight and body shape in healthy adolescent females. Growth retardation and pubertal delay occur with increased social pressure and psychosocial stress. These factors must be considered when nutritional advice is provided to teenagers. Ideally, dietary advice should be passed on before conception as a low prepregnancy BMI is associ-
246
ated with reduced birth weight. Nutrition should be optimized throughout pregnancy and vitamin levels should be monitored [12].
Bone Health
A decrease in bone mineral density (BMD) in patients with CF may begin at a young age [13]. There are many factors that influence bone health both in healthy individuals and in patients with CF; these include nutritional status, calcium, vitamins D and K, pulmonary infection, exercise, glucocorticoids and the class of CFTR mutation. The causes of poor BMD, a reflection of bone health, are thus multifactorial. Evidence for the efficacy of treatments for maintaining and improving bone health is lacking in CF; however, consensus
Wilschanski
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
Adolescence
guidelines have been established. It is recommended that monitoring of BMD and ensuring factors related to bone health be addressed at routine visits.
Poor weight gain
Assess pulmonary status/disease activity
Follow-Up Nutritional assessment
When Things Go Wrong
Figure 1 demonstrates the pathogenesis of malnutrition in CF [16]. As pulmonary disease worsens and resting energy expenditure increases, other factors predispose to an energy deficit. The frequency and severity of infections increase, inducing anorexia and/or vomiting, in turn reducing intake. Weight loss results in causing loss of muscle tissue; respiratory muscle wasting reduces effective coughing, further contributing to the deterioration in lung function. Malnutrition is known to cause immune dysfunction. Taken together, a vicious cycle is established, leading to further deterioration.
Management of the Malnourished Child
Once poor growth is identified, patients must be evaluated more frequently. The visits must include medical, nutritional and behavioral input. Figure 2 shows an algorithm for the work-up.
Feeding behavior evaluation Maximize dietary intake
Psychosocial consultation (adherence) 72-hour fecal fat balance
Other etiologies: • GERD • CFRD • Constipation • DIOS • Bacterial overgrowth • IBD • Etc.
Assess enzyme dose
Consider acid suppression
Enteral feeding
Fig. 2. Plan of action for poor weight gain in CF. GERD =
3
Gastroesophageal reflux disease; CFRD = CF-related diabetes; DIOS = distal intestinal obstruction syndrome; IBD = inflammatory bowel disease.
Nutritional Intervention
If the reason for the poor weight gain is poor intake, the first strategy must be to gradually increase calories at mealtime. At the same time, nutritional intervention with high-calorie supplements may be made. The long-term effect of supplements is controversial and they must not take the place of meals [17]. If this fails, enteral feeding should be commenced [18]. The choice of access should be made together with the family, but, generally, nasogastric feeding is started before gastrostomy
Nutrition in Cystic Fibrosis
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
247
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
A formal dietary assessment should be undertaken annually. This should incorporate a review of nutritional intake, the enzyme dose, the timing of administration as well as vitamin supplement intake. Anthropometry should be performed regularly and at length, and BMI percentile charts should be used for the interpretation of nutritional status. Bone health is an increasing concern in CF [14]. BMD and body composition should be assessed by dual-energy X-ray absorptiometry [15].
placement. Calorically dense formulas (1.5–2.0 kcal/ml) are well tolerated, and, initially, nocturnal infusion is encouraged to promote normal eating behavior during the day. Our experience is that once families see a success after 6–8 weeks of nasogastric feeding, gastrostomy placement is welcomed. Patients with excessive nausea, bloating or vomiting may benefit from prokinetic drugs or semielemental or elemental formula.
Growth Hormone and Appetite Stimulants
The efficacy of growth hormone therapy in CF has recently been reviewed [19]. While growth parameters and pulmonary function seem to improve in treated patients, the overall benefits to health cannot be determined from the moderate evidence available. One recent multicenter trial in which growth hormone was administered for 12 months to patients with reduced growth and bone age indicated its effectiveness in improving growth and lung volumes [20]. Larger trials with appropriate patient selection are needed in order to establish its safety and effectiveness. Appetite stimulants (AS) are often requested by individuals with CF, or by parents of a child with CF, in anticipation of an enhanced appetite
and increased energy intake to promote weight gain [21]. While megestrol acetate is one of the most studied AS in CF, results are not conclusive on the use of AS in patients with CF at this time, and larger, controlled studies are needed.
Conclusions
• The overall goal is that every patient with CF should achieve normal growth. This requires regular surveillance including age-specific individualized expert advice with nutritional care plans to suit each patient. Nutritional intervention should be appropriately timed to influence the evolution of the disease • Nutritional support is an integral part of the care of patients with CF • At diagnosis, all patients require pancreatic and nutritional assessment • Patients must be carefully monitored and dietary counseling provided • Nutritional evaluation and support is age related • Patients who fail to respond require enteral supplementation • Nutritional status impacts on the progression of CF
References
248
3 Sims E, Clark A, McCormick J, et al: Cystic fibrosis diagnosed after 2 months of age leads to worse outcomes and requires more therapy. Pediatrics 2007; 119:19–28. 4 Kraemer R, Rudelberg A, Hadorn B, Rossi E: Relative underweight in cystic fibrosis and its prognostic value. Acta Paediatr Scand 1978;67:33–37. 5 Sharma R, Florea VG, Bolger AP, et al: Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax 2001;56:746–750.
6 Snell GI, Bennetts K, Bartolo J, et al: Body mass index as a predictor of survival in adults with cystic fibrosis referred for lung transplantation. J Heart Lung Transplant 1998;17:1097–1103. 7 Sinaasappel M, Stern M, Littlewood J, et al: Nutrition in patients with cystic fibrosis: a European consensus. J Cyst Fibros 2002;2:51–75. 8 Borowitz D, Baker RD, Stallings V: Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002;35:246–259.
Wilschanski
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
1 Welsh MJ, Tsui LC, Boat TF, et al: Cystic fibrosis; in Scriver C, Beaudet AL, Valle D (eds): The Metabolic and Molecular Basis of Inherited Disease, ed 7. New York, McGraw-Hill, 1995, pp 3799– 3876. 2 Farrell PM, Kosorok MR, Laxova A, et al: Nutritional benefits of neonatal screening for cystic fibrosis. Wisconsin Cystic Fibrosis Neonatal Screening Study Group. N Engl J Med 1997;337: 963–969.
9 Borowitz D, Robinson KA, Rosenfeld M, et al: Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr 2009;155(suppl):S73–S93. 10 Tangpricha V, Kelly A, Stephenson A, et al: An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab 2012;97:1082–1093. 11 Lai HC, Kosorok MR, Sondel SA, et al: Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: evaluation of various criteria used to identify malnutrition. J Pediatr 1988;132:478– 485.
12 Edenborough FP, Borgo G, Knoop C, et al: Guidelines for the management of pregnancy in women with cystic fibrosis. J Cyst Fibros 2008;7(suppl 1):S2– S32. 13 Bianchi ML, Romano G, Saraifoger S, et al: BMD and body composition in children and young patients affected by cystic fibrosis. J Bone Miner Res 2006;21: 388–396. 14 Buntain HM, Schluter PJ, Bell SC, et al: Controlled longitudinal study of bone mass accrual in children and adolescents with cystic fibrosis. Thorax 2006; 61:146–154. 15 Kerem E, Conway S, Elborn S, Heijerman H; Consensus Committee: Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 2005;4:7–26. 16 Durie PR, Pencharz PB: A rational approach to the nutritional care of patients with cystic fibrosis. J R Soc Med 1989; 18(suppl 16):11–20.
17 Kalnins D, Corey M, Ellis L, et al: Failure of conventional strategies to improve nutritional status in malnourished adolescents and adults with cystic fibrosis. J Pediatr 2005;147:399–401. 18 Jelalian E, Stark LJ, Reynolds L, Seifer R: Nutritional intervention for weight gain in cystic fibrosis: a meta-analysis. J Pediatr 1988;132:486–492. 19 Phung OJ, Coleman CI, Baker EL, et al: Recombinant human growth hormone in the treatment of patients with cystic fibrosis. Pediatrics 2010;126:e1211– e1226. 20 Stalvey MS, Anbar RD, Konstan MW, et al: A multi-center controlled trial of growth hormone treatment in children with cystic fibrosis. Pediatr Pulmonol 2012;47:252–263. 21 Chinuck RS, Fortnum H, Baldwin DR: Appetite stimulants in cystic fibrosis: a systematic review. J Hum Nutr Diet 2007;20:526–537.
Nutrition in Cystic Fibrosis
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
249
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
3
3.19 Nutrition in Cystic Fibrosis Michael Wilschanski
Cystic fibrosis · Nutritional status · Pancreatic enzymes · Gastrostomy
Key Messages • Survival from cystic fibrosis (CF) has substantially improved over the past four decades. The advance in nutritional management is one factor which has contributed to this change • This chapter reviews the basic defect of CF and how it influences nutritional status • Normal growth and development can be achieved in CF, and to this end, nutritional counseling is paramount at all ages. The prevention and early detection of growth failure is the key to successful nutritional counseling • An approach to the CF patient who is not thriving and an outline of nutritional management is pro© 2015 S. Karger AG, Basel posed
Introduction
Cystic fibrosis (CF) is the most common lifethreatening autosomal recessive disease in Caucasians with an incidence of 1 in 2,500 live births. The disease is caused by mutations in the cftr gene on chromosome 7, which codes for a cAMP-regulated chloride channel [1]. Nonfunctioning CFTR protein affects epithelial ion and water
transport in a variety of organs including the respiratory, gastrointestinal, hepatobiliary, reproductive and sweat glands. The lack of CFTR function in the pancreatic duct is responsible for obstruction and autodigestion of the pancreas in utero, leading to exocrine pancreatic insufficiency (PI) in 85% of CF infants. The early growth of infants with PI due to CF is dependent on the age at diagnosis. Clinical diagnosis may be difficult unless meconium ileus occurs, typically in only 15% of cases. The remaining patients are diagnosed later, mainly presenting with failure to thrive with steatorrhea, accompanied in some cases with respiratory symptoms. An increasing number of countries have initiated newborn screening for CF using a serum marker of PI, and this has been shown to facilitate an earlier diagnosis with better growth and nutritional status [2]. Longer-term studies after neonatal screening are now revealing reduced pulmonary disease progression [3]. Numerous studies have shown that underweight and poor linear growth in children and malnutrition in adults are independent factors predicting mortality [4, 5]. Together with this, undernutrition has been shown to have an adverse effect on the outcome of lung transplantation [6]. These data reinforce the importance of the prevention and early detection of growth failure, leading to the aggressive management of nuDownloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
Key Words
Infants
The evaluation of an infant with CF should be expedited. If PI is established by tests for steatorrhea and indirect pancreatic function tests, pancreatic enzyme replacement therapy should be initiated as soon as possible. Breast milk can provide complete nutritional support to infants with CF for the first 4–6 months of age, though supplemental energy may sometimes be required by fortifying a portion of the breast milk feeds with formula or by fortifying formulas to a more concentrated energy level for those infants on a combination of breast milk and formula or on formula alone [9]. Regular cow’s milk-based infant formulas can be used if breastfeeding is not an option or if supplementation is required; there is no need for a predigested formula in most instances. Enzymes are given with all foods and milk products including predigested formulas containing medium-chain triglyceride. Infants require powder, which should be taken with fruit sauce with lubricant pretreatment to the mouth and perianal area to avoid skin excoriation. The enzymes should be administered at the beginning of and during the meal. The initial dose of enzymes should be approximately 5,000 IU lipase/ kg/day. The dose may be gradually increased according to symptoms and objective assessment of growth and fat absorption. In many instances, the caloric density needs to be increased, and this may be achieved by fortifying breast milk, adding fat or carbohydrate or concentrating the formula. Once solid food is introduced, enzymes should be titrated by fat intake. The maximum dose is 10,000 IU lipase/kg/day. Fat-soluble vitamin (ADEK) supplementation should be initiated according to the current recommendations [7, 8]. New guidelines from the North American Cystic Fibrosis Foundation Committee on Vita-
Table 1. Recommended dietary macronutrient composi-
tion in CF and non-CF patients (% of energy intake)
Protein Carbohydrate Fat
Non-CF
CF
10 – 15 55 – 60 30
15 35 – 40 45 – 50
min D advise higher supplemental amounts of vitamin D than are found in the currently available CF vitamin supplements. Additional vitamin D supplements are therefore recommended to keep the lower limit of 25-hydroxyvitamin D at 30 ng/ ml (75 nmol/l) [10]. Yearly monitoring of serum vitamin levels is recommended for vitamin A, vitamin E and vitamin D. Hyponatremic alkalosis may occur in infants, especially during the summer months; supplementation with sodium chloride is recommended. Zinc supplementation may be considered in the child who is not thriving [9].
Toddlers
As infants are introduced to table foods, it is important that the diet be balanced, with moderately increased fat and protein content (table 1). Parents need to be in control, routinely adding calories to maintain growth. The child with CF should avoid low-fat food and ‘grazing’. The dietician should promote positive interactions around meals. The mealtime must not turn into a battleground which is the catalyst for poor feeding behavior.
3
School-Age Children
School age is the age at which encourage the child to obtain a basic knowledge of the physiological processes, eventually leading to taking increasing responsibility for practical enzyme and nutritional management.
Nutrition in Cystic Fibrosis
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
245
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
tritional deficits at all ages. This has led to the publication of nutritional guidelines in Europe and in North America [7, 8].
Pancreatic Intestinal Biliary
Cellular defect?
Needs
+
Losses
Iatrogenic Psychogenic Gastrointestinal
+
Intake
Anorexia
Vomiting
Energy deficit
Pulmonary infections
Weight loss
Respiratory muscles
Lung parenchyma
Immune dysfunction
Deteriorating lung function
Fig. 1. The pathogenesis of energy imbalance in CF.
This stage is associated with increased growth, puberty and increased physical activity. This adds up to markedly increased nutritional requirements which are often difficult to attain. Pulmonary infections are more common, as is the onset of CF-related diabetes and, in a small minority of cases, CF-related liver disease. Female patients are at a greater risk of nutritional failure at this age [11]. This may be partly due to dissatisfaction with weight and body shape in healthy adolescent females. Growth retardation and pubertal delay occur with increased social pressure and psychosocial stress. These factors must be considered when nutritional advice is provided to teenagers. Ideally, dietary advice should be passed on before conception as a low prepregnancy BMI is associ-
246
ated with reduced birth weight. Nutrition should be optimized throughout pregnancy and vitamin levels should be monitored [12].
Bone Health
A decrease in bone mineral density (BMD) in patients with CF may begin at a young age [13]. There are many factors that influence bone health both in healthy individuals and in patients with CF; these include nutritional status, calcium, vitamins D and K, pulmonary infection, exercise, glucocorticoids and the class of CFTR mutation. The causes of poor BMD, a reflection of bone health, are thus multifactorial. Evidence for the efficacy of treatments for maintaining and improving bone health is lacking in CF; however, consensus
Wilschanski
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
Adolescence
guidelines have been established. It is recommended that monitoring of BMD and ensuring factors related to bone health be addressed at routine visits.
Poor weight gain
Assess pulmonary status/disease activity
Follow-Up Nutritional assessment
When Things Go Wrong
Figure 1 demonstrates the pathogenesis of malnutrition in CF [16]. As pulmonary disease worsens and resting energy expenditure increases, other factors predispose to an energy deficit. The frequency and severity of infections increase, inducing anorexia and/or vomiting, in turn reducing intake. Weight loss results in causing loss of muscle tissue; respiratory muscle wasting reduces effective coughing, further contributing to the deterioration in lung function. Malnutrition is known to cause immune dysfunction. Taken together, a vicious cycle is established, leading to further deterioration.
Management of the Malnourished Child
Once poor growth is identified, patients must be evaluated more frequently. The visits must include medical, nutritional and behavioral input. Figure 2 shows an algorithm for the work-up.
Feeding behavior evaluation Maximize dietary intake
Psychosocial consultation (adherence) 72-hour fecal fat balance
Other etiologies: • GERD • CFRD • Constipation • DIOS • Bacterial overgrowth • IBD • Etc.
Assess enzyme dose
Consider acid suppression
Enteral feeding
Fig. 2. Plan of action for poor weight gain in CF. GERD =
3
Gastroesophageal reflux disease; CFRD = CF-related diabetes; DIOS = distal intestinal obstruction syndrome; IBD = inflammatory bowel disease.
Nutritional Intervention
If the reason for the poor weight gain is poor intake, the first strategy must be to gradually increase calories at mealtime. At the same time, nutritional intervention with high-calorie supplements may be made. The long-term effect of supplements is controversial and they must not take the place of meals [17]. If this fails, enteral feeding should be commenced [18]. The choice of access should be made together with the family, but, generally, nasogastric feeding is started before gastrostomy
Nutrition in Cystic Fibrosis
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
247
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
A formal dietary assessment should be undertaken annually. This should incorporate a review of nutritional intake, the enzyme dose, the timing of administration as well as vitamin supplement intake. Anthropometry should be performed regularly and at length, and BMI percentile charts should be used for the interpretation of nutritional status. Bone health is an increasing concern in CF [14]. BMD and body composition should be assessed by dual-energy X-ray absorptiometry [15].
placement. Calorically dense formulas (1.5–2.0 kcal/ml) are well tolerated, and, initially, nocturnal infusion is encouraged to promote normal eating behavior during the day. Our experience is that once families see a success after 6–8 weeks of nasogastric feeding, gastrostomy placement is welcomed. Patients with excessive nausea, bloating or vomiting may benefit from prokinetic drugs or semielemental or elemental formula.
Growth Hormone and Appetite Stimulants
The efficacy of growth hormone therapy in CF has recently been reviewed [19]. While growth parameters and pulmonary function seem to improve in treated patients, the overall benefits to health cannot be determined from the moderate evidence available. One recent multicenter trial in which growth hormone was administered for 12 months to patients with reduced growth and bone age indicated its effectiveness in improving growth and lung volumes [20]. Larger trials with appropriate patient selection are needed in order to establish its safety and effectiveness. Appetite stimulants (AS) are often requested by individuals with CF, or by parents of a child with CF, in anticipation of an enhanced appetite
and increased energy intake to promote weight gain [21]. While megestrol acetate is one of the most studied AS in CF, results are not conclusive on the use of AS in patients with CF at this time, and larger, controlled studies are needed.
Conclusions
• The overall goal is that every patient with CF should achieve normal growth. This requires regular surveillance including age-specific individualized expert advice with nutritional care plans to suit each patient. Nutritional intervention should be appropriately timed to influence the evolution of the disease • Nutritional support is an integral part of the care of patients with CF • At diagnosis, all patients require pancreatic and nutritional assessment • Patients must be carefully monitored and dietary counseling provided • Nutritional evaluation and support is age related • Patients who fail to respond require enteral supplementation • Nutritional status impacts on the progression of CF
References
248
3 Sims E, Clark A, McCormick J, et al: Cystic fibrosis diagnosed after 2 months of age leads to worse outcomes and requires more therapy. Pediatrics 2007; 119:19–28. 4 Kraemer R, Rudelberg A, Hadorn B, Rossi E: Relative underweight in cystic fibrosis and its prognostic value. Acta Paediatr Scand 1978;67:33–37. 5 Sharma R, Florea VG, Bolger AP, et al: Wasting as an independent predictor of mortality in patients with cystic fibrosis. Thorax 2001;56:746–750.
6 Snell GI, Bennetts K, Bartolo J, et al: Body mass index as a predictor of survival in adults with cystic fibrosis referred for lung transplantation. J Heart Lung Transplant 1998;17:1097–1103. 7 Sinaasappel M, Stern M, Littlewood J, et al: Nutrition in patients with cystic fibrosis: a European consensus. J Cyst Fibros 2002;2:51–75. 8 Borowitz D, Baker RD, Stallings V: Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002;35:246–259.
Wilschanski
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
1 Welsh MJ, Tsui LC, Boat TF, et al: Cystic fibrosis; in Scriver C, Beaudet AL, Valle D (eds): The Metabolic and Molecular Basis of Inherited Disease, ed 7. New York, McGraw-Hill, 1995, pp 3799– 3876. 2 Farrell PM, Kosorok MR, Laxova A, et al: Nutritional benefits of neonatal screening for cystic fibrosis. Wisconsin Cystic Fibrosis Neonatal Screening Study Group. N Engl J Med 1997;337: 963–969.
9 Borowitz D, Robinson KA, Rosenfeld M, et al: Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr 2009;155(suppl):S73–S93. 10 Tangpricha V, Kelly A, Stephenson A, et al: An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab 2012;97:1082–1093. 11 Lai HC, Kosorok MR, Sondel SA, et al: Growth status in children with cystic fibrosis based on the National Cystic Fibrosis Patient Registry data: evaluation of various criteria used to identify malnutrition. J Pediatr 1988;132:478– 485.
12 Edenborough FP, Borgo G, Knoop C, et al: Guidelines for the management of pregnancy in women with cystic fibrosis. J Cyst Fibros 2008;7(suppl 1):S2– S32. 13 Bianchi ML, Romano G, Saraifoger S, et al: BMD and body composition in children and young patients affected by cystic fibrosis. J Bone Miner Res 2006;21: 388–396. 14 Buntain HM, Schluter PJ, Bell SC, et al: Controlled longitudinal study of bone mass accrual in children and adolescents with cystic fibrosis. Thorax 2006; 61:146–154. 15 Kerem E, Conway S, Elborn S, Heijerman H; Consensus Committee: Standards of care for patients with cystic fibrosis: a European consensus. J Cyst Fibros 2005;4:7–26. 16 Durie PR, Pencharz PB: A rational approach to the nutritional care of patients with cystic fibrosis. J R Soc Med 1989; 18(suppl 16):11–20.
17 Kalnins D, Corey M, Ellis L, et al: Failure of conventional strategies to improve nutritional status in malnourished adolescents and adults with cystic fibrosis. J Pediatr 2005;147:399–401. 18 Jelalian E, Stark LJ, Reynolds L, Seifer R: Nutritional intervention for weight gain in cystic fibrosis: a meta-analysis. J Pediatr 1988;132:486–492. 19 Phung OJ, Coleman CI, Baker EL, et al: Recombinant human growth hormone in the treatment of patients with cystic fibrosis. Pediatrics 2010;126:e1211– e1226. 20 Stalvey MS, Anbar RD, Konstan MW, et al: A multi-center controlled trial of growth hormone treatment in children with cystic fibrosis. Pediatr Pulmonol 2012;47:252–263. 21 Chinuck RS, Fortnum H, Baldwin DR: Appetite stimulants in cystic fibrosis: a systematic review. J Hum Nutr Diet 2007;20:526–537.
Nutrition in Cystic Fibrosis
Koletzko B, et al. (eds): Pediatric Nutrition in Practice. World Rev Nutr Diet. Basel, Karger, 2015, vol 113, pp 244–249 DOI: 10.1159/000367876
249
Downloaded by: Deakin University 198.143.35.1 - 8/10/2015 8:43:01 PM
3
