- Email: [email protected]
Strategies for improving early nutritional outcomes in children with oesophageal atresia and congenital diaphragmatic hernia
Accepted Manuscript Clinical usefulness Strategies for Improving early nutritional outcomes in children with oesophageal atresia and congenital diaphragmatic hernia Dominic A. Fitzgerald, Andrea Kench, Lucy Hatton, Jonathan Karpelowsky PII: DOI: Reference:
S1526-0542(17)30040-4 http://dx.doi.org/10.1016/j.prrv.2017.05.001 YPRRV 1215
To appear in:
Paediatric Respiratory Reviews
Please cite this article as: D.A. Fitzgerald, A. Kench, L. Hatton, J. Karpelowsky, Strategies for Improving early nutritional outcomes in children with oesophageal atresia and congenital diaphragmatic hernia, Paediatric Respiratory Reviews (2017), doi: http://dx.doi.org/10.1016/j.prrv.2017.05.001
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Clinical Usefulness
Strategies for Improving early nutritional outcomes in children with oesophageal atresia and congenital diaphragmatic hernia. Dominic A. Fitzgerald 1,2 Andrea Kench 1,3 Lucy Hatton 1 Jonathan Karpelowsky 2,4 1. Department of Respiratory Medicine, The Children’s Hospital at Westmead, Sydney 2. Discipline of Child & Adolescent Health, Sydney Medical School, University of Sydney 3. Department of Nutrition and Dietetics, The Children’s Hospital at Westmead, Sydney 4. Department of Surgery, The Children’s Hospital at Westmead, Sydney
Summary Text Figures References
107 words 1923 3 18
Key words: Growth, feeding guides, oesophageal atresia, congenital diaphragmatic hernia
Educational Aims The reader will come to appreciate that: • Feeding is often compromised in repaired oesophageal atresia and diaphragmatic hernia during early childhood • Normal growth outcomes should be expected in the majority of infants • Written information sheets for parents may reinforce nutritional strategies for parents to implement in their children.
1
Summary Post-natal growth in surgical lung conditions, such as congenital diaphragmatic hernia and oesophageal atresia with tracheo-oesophageal fistula, is often sub-optimal in the early years of life when lung growth is occurring. Whilst constitutional, behavioural and mechanical factors may contribute to poor feeding and weight gain, there is a common path of management with greater caloric supplementation that may change growth trajectories and potentially lead to better respiratory, anthropometric and cognitive outcomes. We provide simple, single page, feeding supplementation sheets in three age groups: 0-6 months, 6-12 months and 12-24 months that have proven useful for enhancing weight gain in our patients.
2
The growth of infants with congenital surgical lung conditions such as diaphragmatic hernia [CDH] and oesophageal atresia / tracheo-oesophageal atresia [OA/TOF] is compromised from the outset with days to weeks of intravenous fluids and often parenteral nutrition. Preterm delivery and an underlying syndrome, such as VACTERL or Trisomy 21, may further complicate the clinical course and this may be reflected in the delay in establishing sucking feeds [1]. An underlying syndrome may also impact growth potential and trajectory A lengthy period of hospitalisation will ensue for more complicated cases , typically lasting for three to four weeks in the neonatal intensive care unit [NICU] [1-5]. A significant subset of infants will leave hospital dependent, at least partially, on nasogastric feeding. Those with CDH may have ongoing pulmonary hypertension and pulmonary hypoplasia necessitating the use of low flow nasal oxygen supplementation or even the use of noninvasive ventilation during sleep [3,4]. The early wells of transition from the NICU to the home can be challenging and feeding issues with slow weight gain can be anticipated.
There will be typically be frequent reviews with medical, surgical and nursing health care professionals regarding their respiratory and feeding status with an appropriate emphasis on establishing full sucking feeds in the early weeks and months following discharge. With all these appointments, the early focus on feeding may prompt health care professionals to accept slower than normal weight gains. However, if this slow weight gain persists, then a significant weight lag develops that may take months to years to overcome.
The consequences of poor growth in animal models suggest that protein malnutrition impairs lung growth and development [4]. In humans, early postnatal weight gain is driven by growth hormone [5]. Urinary growth hormone levels has been demonstrated to be low in infants with chronic neonatal lung disease [CNLD] who have compromised growth and tachypnoea and to improve with reduction in tachypnoea and improved growth trajectories [6]. In common with CNLD, infants with diaphragmatic hernia and oesophageal atresia/ tracheo-oesophgeal fistula with poor growth have increased caloric requirements due to tachypnoea and mild hypoxaemia and their growth improves
3
with reduction in respiratory symptoms after infancy7,8. Consequently, as improvement in oxygenation improves growth in CNLD [9], so the same may be applicable for infants with CDH and OA/TOF who have mild hypoxaemia. Thus, poor growth in CDH and OA/TOF is likely due to inadequate caloric intake to meet the infant’s elevated nutritional requirements that may reflect a combination of challenges with maintaining adequate caloric delivery as well as mild hypoxaemia.
Growth patterns in children with CDH Growth failure is common in CDH and has been attributed to increased metabolic demand related to elevated work of breathing, gastro-oesophageal reflux disease [GORD] and oral aversion [3, 1012]. Growth failure is reported in up to 50% of CDH babies, even when oral caloric intake is optimised [10-13]. Many such references date back over 10 to 20 years and the risk factors for poor growth include need for ECMO and discharge on home oxygen.
Despite evidence that aggressive nutritional management can bring about significant catch-up growth [3,7,10-13,], there is ongoing evidence in a more contemporary cohort of 45 CDH survivors [Born 2005 -2011] that growth remains a problem [7]. In this cohort, from our group at The Children’s Hospital at Westmead, Sydney, followed to 12 months of age, 56% had failure to thrive [FTT: Weight z score - 2 standard deviations for age] at 6 months of age and this had dropped to 21% at 12 months of age, with days of mechanical ventilation being predictive of FTT at 6 months of age. This result should be seen as being from a single centre which does not provide ECMO for CDH babies, but has an 82% survival rate for all comers [7]. The weight gain showed an improvement in weight z scores [+/- SD] from -2.07 +/- 1.17 to -0.85 +/- 1.32 between 6 and 12 months with feeding strategies including added calories in 58%, nasogastric feeds in 42% and gastrostomy tubes in 8% during the first 12 months. Oral aversion may be made worse by the prolonged use of nasogastric tubes and the use of gastrostomy tubes and so considerable time with speech pathology intervention may be required to establish adequate oral caloric intake [12,13] However, there is a lack of consensus about the best approach to this problem with
4
strategies ranging from observation to using increased intake of calorie dense foods, use of nasogastric tubes and consideration of gastrostomy tubes for more severe cases.
Growth patterns in children with OA/TOF Some infants with OA/TOF will have significant challenges to establishing feeds related to anastomotic leaks from the initial surgery, strictures requiring dilatation and episodes of pulmonary aspiration and pneumonia [1,5]. In an American series of 3479 infants [median birth weight 2.7 kg at 37 weeks gestation] managed in 43 US centres over 13 years to 2012, there was a outcome data to age 2 years in 83% [2]. The median length of stay following delivery was 27 days. Only 0.8% of patients received ECMO. The authors reported a 5.4% in hospital mortality rate, 91.5% received total parental nutrition [TPN] for a period of time, 11.4% had a repeat operation for their oesophageal construction, and 11.7% had a fundoplication. Interestingly, 17% had a readmission within 30 days, 55% of the cohort had readmission within 2 years and this included half who had multiple readmissions, 12.7% who had pneumonia on readmission, and 6.8% having at least five readmissions [2]. Unfortunately, longitudinal growth data was not presented in this paper.
With regard to growth, there are several groups who have presented their data, usually as part of a multidisciplinary follow-up clinic. In a single US centre study involving 69 infants born between 1972 and 1990, approximately 25% had height and or weight < 5% at age 5 years, and 15% at 510 years and 15% at > 10 years of those followed [5]. In another single centre series of 334 patients aged 1 to 37 years from Australia, born between 1948 and 1986, included 62 patients aged 0-5 years, 54 aged 5-10 years, 54 aged 10-15 years and 164 aged over 15 years [14]. Daily feeding difficulties were reported in approximately 10% in all ages and the proportion with gastrooesophageal reflux was reported as approximately 45%, 15%, 20% and 25% respectively across the age groups. Wasting rather than stunting was seen under the age of five years with approximately 18% having a weight < 10% and 13% having height < 10%, yet adult height and weight distribution was normal [14]. More recently, a small study from Holland compared infant lung function testing and noted no difference in lung function or growth up to two years of age
5
whether an open thoracotomy or a thoracoscopic [minimally invasive] approach was used [15]. This suggests a degree of growth restriction related to sub-optimal caloric intake in the early years. In more recent reports demonstrate a similar pattern of slow initial growth [8-11]. The French reported their outcomes to 12 months for 307 infants born in 2008-2009 and demonstrated that 15% were undernourished at 12 months of age [16]. A repeated association with poor growth is the lack of established feeds at the time of discharge from hospital after the initial surgery [16,17]. This reinforces our need to focus on optimising caloric intake in the first 12 months of life in order to optimise nutritional, respiratory and cognitive outcomes.
Methods of increasing calories for infants and young children with CDH and OA/TOF 0-6 months Exclusive breastfeeding is encouraged and supported for mothers of infants aged 0-6 months [18]. However, breastfeeding often isn’t feasible for infants with CDH and OA/TOF, especially during the
initial post-operative period in the NICU. It isn’t uncommon for these infants to receive periods of total parenteral nutrition (TPN) followed by enteral tube feeding (NG feeds) as oral suck feeds are slowly introduced. Where possible, a dietitian should be involved to assess growth and calculate estimated energy and protein requirements for these infants. Individualised feeding plans should be implemented in consultation with the multidisciplinary team (MDT). Expressed breastmilk (EBM) or standard infant formula alone, often doesn’t meet the elevated nutritional requirements for infants with CDH and OA/TOF. Commonly used supplements for EBM fortification include human milk fortifier (HMF), carbohydrate and/or fat additions and powdered infant formula. Care should be taken when fortifying EBM with carbohydrate and/or fat alone as this will result in a reduced protein to energy ratio of the feed and can hinder potential for optimal weight gain. Concentrated infant formulas with/without carbohydrate or fat additions and high energy/high protein ready to feed infant formulas may also be required for this population group where EBM isn’t available. Where possible, suck feeds should also be encouraged, even if supplementary nasogastric feeding is
6
required, in an attempt to prevent oral aversion longer term. Early input from the broader MDT, including speech pathology may also help with the progression of oral feeding and prevention of oral aversion [Figure 1].
6-12 months The introduction of solids is recommended from around 4-6 months of age whereby most children will be physiologically and developmentally ready [18]. It is important to assess the readiness for solids on and individualised basis for infants with CDH and OA/TOF. National infant feeding recommendations should be used to guide recommendations. In Australia, that includes introducing any food in any order whilst promoting iron containing foods e.g. fortified rice or pureed meat as a first food [18]. As an infant progresses with the introduction of solids and a greater portion of their intake is in the form of food, fat fortification of solids may assist with optimizing weight gain. Options for the fortification of solids are listed in Figure 2. Progression with textures should also be encouraged according to the child’s developmental stage. A speech pathologist may again be required to assist with strategies to combat oral aversion.
12-24 months Ideally children will have progressed to family foods, including an element of self-feeding by 12 months of age [18]. If children with CDH and OA/TOF have not established catch-up weight during infancy, further strategies to optimise growth may be required [Figure 3]. These include ongoing fortification of meals and the transition from fortified EBM or concentrated infant formula, to a 1.01.5kcal/ml paediatric formula. Fussy eating can also be a challenge for children of this age and can be particularly challenging when combined with an element of oral aversion and the need for ongoing supplementary enteral feeding. In addition to commonly employed fussy eating strategies, food play is a strategy employed by dietitians and speech pathologists to assist with oral aversion. For the more complex feeders, a referral to a MDT feeding clinic may be required for nasogastric tube weaning strategies.
7
Conclusion In this paper we have addressed the common clinical problem of slow weight gain in infants with structural surgical lung conditions such as diaphragmatic hernia and oesophageal atresia tracheooesophageal fistula. Through our experience in a multi-disciplinary clinic we have developed some feeding strategies with information sheets for parents that have proven helpful to families in an Australian setting. These are designed to be used in addition to recommended medical therapies, such as the treatment of gastro-oesophageal reflux where appropriate. Whilst food preferences and feeding practices may differ in various parts of the world, we hope these resources may prove beneficial for families.
Acknowledgements We would like to thank Dr Robert Halliday, Professor Nadia Badawi and Dr Karen Walker for their assistance in developing the parent information sheets.
8
Figures Figure 1
Parent information sheet for feeding infants 0 to 6 months
Figure 2
Parent information sheet for feeding infants 6 to 12 months
Figure 3
Parent information sheet for feeding children aged 12 to 24 months.
9
References
1. Holland AJ, Fitzgerald DA. Oesophageal atresia and tracheo-oesophageal fistula: current management strategies and complications. Paediatr Respir Rev. 2010; 11:100–106. 2. Sulkowski JP, Cooper JN, Lopez JJ, Jadcherla Y, Cuenot A, Mattei P et al. Morbidity and Mortality in Patients with Esophageal Atresia. Surgery 2014; 156: 483-491. 2. Robinson PD, Fitzgerald DA. Congenital diaphragmatic hernia. Paediatr Respir Rev. 2007; 8: 323-35. 3. Leeuwen L, Fitzgerald DA. Congenital diaphragmatic hernia. J. Paediatr Child Health. 2014; 50: 667-73. 3. Little DC, Rescola FJ, .Grosfeld JL, West KW, Scherer LR, Engum SA. Long Term Analysis of Children with Esophageal Atresia and Tracheoesophageal Fistula. J Pediatr Surg 2003; 38: 852-856. 4. Gaultier C. Malnutrition and Lung Growth. Pediatr Pulmonol 1991; 10: 278-286. 5. Gluckman PD. Fetal Growth: An Endocrinie Perspective. Acta Paediatr Scand 1989; 349: 2125. 6. Fitzgerald DA, Van Asperen PP, O’Leary P, Feddema P, Leslie G, Arnold J et al. Sleep, respiratory rate and growth hormone in chronic neonatal lung disease. Pediatr Pulmonol 1998; 26: 241-249. 7. Leeuwen L, Walker K, Halliday R, Karpelowsky J, Fitzgerald DA. Growth in children with congenital diaphragmatic hernia during the first year of life. J Pediatr Surg 2014; 49: 13631366. 8. Roberts K, Karpelowsky J, Fitzgerald DA, Soundappan SS. Outcomes of oesophageal atresia and tracheo-oesophageal atresia fistula repair. J Paediatr Child Health 2016; 52: 694-698. 9. Groothius JR, Rosenberg AA. Home oxygen promotes weight gain in infants with bronchopulmonary dysplasia. Am J Dis Child 1987; 1987: 141: 992-995.
10
10. Gien J, Murthy K, Pallotto EK, Brozanski B, Chicoine L, Zaniletti I et al. Short-term weight gain velocity in infants with congenital diaphragmatic henria (CDH). Early Hum Dev 2017; 106: 7-12. 11. Jaillard SM, Pierrat V, Dubois A, Truffert P, Lequiien P, Wurtz AJ et al. Outcome at 2 years of infants with congenital diaphragmatic hernia: a population based study. Ann Thorac Surg 2003; 75: 250-256. 12. Muratore CS, Utter S, Jaksic T, Lund DP, Wilson JM. Nutritional morbidity in survivors of congenital diaphragmatic hernia. J Pediatr Surg 2001; 36: 1171-1176 13. Cortes RA, Keller RL, Townsend T, Harrison MR, Farmer DL, Lee H et al. Survival of severe congenital diaphragmatic hernia has morbid consequences. J Pediatr Surg 2005; 40: 36-45. 14. Checuti P, Phelan PD. Gastrointestinal morbidity and growth after repair of oesophageal atresia and tracheo-oesophageal fistula. Arch Dis Child 1993; 68: 163-166. 15. Spoel M, Meeussen CJ, Gischler SJ, Hop WC, Bax NM, Wijnen RM et al. Respiratory Morbidity and growth after thoracotomy or thoracoscopic repair of oesophageal atresia. J Pediatr Surg 2012; 47: 1975-1983. 16. Schneider A, Blanc S, Bonnard A, Khen-Dunlop N, Auber F, Breton A et al. Results from the French National Esophageal Atresia Register: one year outcome. Orphanet J. Rare Dis 2014; 9: 206. 17. Castilloux J, Noble AJ, Faure C. Risk factors for short and long term morbidity in children with oesophageal atresia. J Pediatr 2010; 156: 755-760. 18. https://www.eatforhealth.gov.au/sites/default/files/files/the_guidelines/n56b_infant_feeding_su mmary_130808.pdf.
11
Figure 1
Parent information sheet for feeding infants 0 to 6 months
Feeding your CDH or OA/TOF baby: birth to 6 months
Babies born with Congenital Diaphragmatic Hernia (CDH) [abdominal contents in the chest through a gap in the diaphragm muscle] or oesophageal atresia / trachea-oesophageal fistula [OA/TOF] sometimes have trouble getting enough food. So many babies with CDH or OA/TOF need extra calories to help them grow. Food provides the energy to allow babies to grow.
Babies with CDH or OA/TOF need to work harder to breathe which means they need more food for energy. A dietician will probably tell you to give your baby formula as well as breast milk, special formula with extra nutrition or to feed your baby more often.
You won’t need to do this all the time, so talk to the dietician about how long your baby will need extra milk or solids [4-6 months onwards].
Never give your baby extra food without talking to a doctor or dietician first.
Sometimes, when babies work hard to breathe, they have trouble keeping food in their tummy, so they may start to vomit during or after feeding. If this happens, you may be advised by a dietician or doctor how to feed your baby smaller amounts of food more often, alter your breast-feeding approach or to give your baby special formula.
Some babies need to be fed through a tube that goes into their stomach through their nose. This is called a nasogastric (NG) tube.
If a baby has an NG tube for a long time, they may get an ‘oral aversion’, which means they don’t like food or bottles going near or in their mouth. Helping your baby to eat normally as they grow will help them get rid of the NG tube in time. As your baby grows, they will stop needing the NG tube.
Your baby’s doctor or dietician will give you lots of help and information if your child has an NG tube.
Regularly see a community health nurse or your local GP to check how your baby is growing.
If you have any questions or you are worried about anything, please speak to a doctor, nurse or dietician.
There are two more information sheets about feeding a child with CDH that may help you. Ask for a copy of the information sheet about babies aged 6 to 12 months and 1 to 2 years.
12
Figure 2
Parent information sheet for feeding infants 6 to 12 months
Feeding your CDH or OA/TOF baby: 6 to 12 months Babies born with Congenital Diaphragmatic Hernia (CDH) or oesophageal atresia / trachea-oesophageal fistula (OA/TOF) may be smaller than other babies but they usually start to catch up after six months. Good nutrition is important for all babies, but especially for babies with CDH as it can help improve breathing and lung function. Around six months of age, babies are usually ready to start eating solids. Some doctors or dietitians may tell you to give your baby solids before they are six months old, if they are having trouble getting enough milk. You will know your baby is ready to try solids if they: seem hungry between milk feeds look interested in the food others are eating have good head and neck control can sit up, if they have enough support. Even when your baby starts to eat solids, milk will probably still be the main type of nutrition for a while. For all babies, starting solids is something new and different. To help them get used to this new experience, here are a few tips:
When you first start, give your baby some solids after they’ve had some milk. At first your baby will probably only have a few teaspoons. Make sure things are quiet and relaxed around your baby. Your baby will tell you when they’ve had enough by pushing away the spoon, turning their head away or refusing to open their mouth.
DON’T FORCE FEED. Your baby won’t enjoy it and it can lead to feeding problems.
WHAT TYPE OF FOODS SHOULD YOU GIVE YOUR BABY? Food that is high in iron, such as fortified rice cereal or pureed meat, should be some of the first foods you offer your baby. As your baby gets used to eating soft food, you can start to slowly give your baby different types of food with bigger pieces. By the time your baby has their first birthday, they should be eating the same food as the rest of the family. Your doctor or dietician may suggest you give your baby fortified milk to grow better If your baby is breastfed, or doesn’t need special foods, it is good to add some healthy fats to their diet. See the table below for some ideas. This is a good way to help them gain more weight. When your baby reaches normal weight, and is growing normally, you can stop. FOOD/FLUID Oil (olive/canola)
Margarine / butter Avocado Dairy products Cheese Cream
Add 1-2 teaspoons into pureed baby foods (homemade or commercial) Drizzle over the top of rice, noodles, pasta and vegetables Add to pureed foods or spread thickly on sandwiches, toast, crackers and biscuits Add to pureed foods or mash with sour cream and use as a dip with vegetables Full fat dairy products are recommended for children under 2 years old Grate into pureed vegetables, pasta, rice, bolognaise sauce, casseroles or baked beans Add 1-2 teaspoons to purees, mashed potato, soups, casseroles, cereals, custard or yoghurt
There are two more information sheets about feeding a child with CDH or OA / TOF that may help you. Ask for a copy of the information sheet about babies aged birth to 6 months and 1 to 2 years.
13
Figure 3
Parent information sheet for feeding children aged 12 to 24 months.
Feeding your CDH or OA/TOF baby:12 to 24 months
By their first birthday, some babies (around 1 in 4) with congenital diaphragmatic hernia (CDH) or oesophageal atresia / trachea-oesophageal fistula (OA/TOF) still grow more slowly than other babies do. However, all children do start to grow slower when they reach toddler age.
Children do not need a lot of food to grow well, but they do need a range of healthy foods to make sure they get the best nutrition.
Toddlers love to explore and try new things and mealtimes can be very messy as toddlers throw, squash and play with their food. This is an important and natural part of toddler development.
Choosing and refusing food is a normal way for children show their independence.
Not all toddlers will eat the same amount every day – it is normal for them to eat lots one day and little the next.
It can be stressful if your child is a fussy eater, especially if it has been important part of managing their growth and health.
As a parent or carer, you are responsible for what, when and where your child eats. But you cannot make them eat – how much (if any) your child eats is up to them. Give your child the best food you can and try to not to worry too much if they are fussy.
Tips for dealing with a fussy eater: 1. Do not prepare a ‘special’ separate meal for your toddler 2. Don’t spend too long trying to make them eat. Stop after half an hour. 3. You may need to cut up food that is hard to chew. 4. Meals and snacks should be eaten at the table, with no distractions 5. Do not force feed as your toddler will hate it and it may cause feeding problems later. 6. Don’t give extra foods or favourite foods as a ‘reward’ for eating 7. Be patient – it can take time for your child to eat well. 8. Give your child small amounts of food. Your child may feel worried if they see too much food on their plate. 9. Talk about interesting things during meals, rather than focussing on food
If your child does not want to eat, say ‘have you finished eating?’ If they have, tell them to move away from the table and make sure they know that they won’t be having any more food until the next mealtime. 14
Try not to worry; your child will eat if they are hungry.
If you think your child’s is growing too slowly, you can add some healthy fats to their diet. See the table below for some ideas. This is a good way to help them gain more weight. When your child reaches normal weight, and is growing normally, you can stop.
FOOD/FLUID Oil (olive/canola) Butter/Margarine Avocado, humus or dips Dairy products
Cheese
Cream
WHERE TO USE Add 1-2 teaspoons into meals such as pasta and sauces Add to meals or spread thickly on sandwiches, toast, crackers and biscuits Add to meals or mash with sour cream and use as a dip with vegetables Full fat dairy products are recommended for children under the age of 2 years old, including milk, yoghurt, cheese, cream cheese, custard, ice cream and sour cream Ideas: serve yoghurt/custard with mashed banana/fruit pieces to dip or use cream cheese as a dip Grate or melt cheese over vegetables, pasta, rice, bolognaise sauce, casseroles or baked beans Add 1-2 teaspoons to fruit or vegetable purees, mashed potato, soups, casseroles or cereals
There are two more information sheets about feeding a child with CDH or OA/TOF that may help you. Ask for a copy of the information sheet about babies aged birth to 6 months and 6 months to 12 months.
15
S1526-0542(17)30040-4 http://dx.doi.org/10.1016/j.prrv.2017.05.001 YPRRV 1215
To appear in:
Paediatric Respiratory Reviews
Please cite this article as: D.A. Fitzgerald, A. Kench, L. Hatton, J. Karpelowsky, Strategies for Improving early nutritional outcomes in children with oesophageal atresia and congenital diaphragmatic hernia, Paediatric Respiratory Reviews (2017), doi: http://dx.doi.org/10.1016/j.prrv.2017.05.001
This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.
Clinical Usefulness
Strategies for Improving early nutritional outcomes in children with oesophageal atresia and congenital diaphragmatic hernia. Dominic A. Fitzgerald 1,2 Andrea Kench 1,3 Lucy Hatton 1 Jonathan Karpelowsky 2,4 1. Department of Respiratory Medicine, The Children’s Hospital at Westmead, Sydney 2. Discipline of Child & Adolescent Health, Sydney Medical School, University of Sydney 3. Department of Nutrition and Dietetics, The Children’s Hospital at Westmead, Sydney 4. Department of Surgery, The Children’s Hospital at Westmead, Sydney
Summary Text Figures References
107 words 1923 3 18
Key words: Growth, feeding guides, oesophageal atresia, congenital diaphragmatic hernia
Educational Aims The reader will come to appreciate that: • Feeding is often compromised in repaired oesophageal atresia and diaphragmatic hernia during early childhood • Normal growth outcomes should be expected in the majority of infants • Written information sheets for parents may reinforce nutritional strategies for parents to implement in their children.
1
Summary Post-natal growth in surgical lung conditions, such as congenital diaphragmatic hernia and oesophageal atresia with tracheo-oesophageal fistula, is often sub-optimal in the early years of life when lung growth is occurring. Whilst constitutional, behavioural and mechanical factors may contribute to poor feeding and weight gain, there is a common path of management with greater caloric supplementation that may change growth trajectories and potentially lead to better respiratory, anthropometric and cognitive outcomes. We provide simple, single page, feeding supplementation sheets in three age groups: 0-6 months, 6-12 months and 12-24 months that have proven useful for enhancing weight gain in our patients.
2
The growth of infants with congenital surgical lung conditions such as diaphragmatic hernia [CDH] and oesophageal atresia / tracheo-oesophageal atresia [OA/TOF] is compromised from the outset with days to weeks of intravenous fluids and often parenteral nutrition. Preterm delivery and an underlying syndrome, such as VACTERL or Trisomy 21, may further complicate the clinical course and this may be reflected in the delay in establishing sucking feeds [1]. An underlying syndrome may also impact growth potential and trajectory A lengthy period of hospitalisation will ensue for more complicated cases , typically lasting for three to four weeks in the neonatal intensive care unit [NICU] [1-5]. A significant subset of infants will leave hospital dependent, at least partially, on nasogastric feeding. Those with CDH may have ongoing pulmonary hypertension and pulmonary hypoplasia necessitating the use of low flow nasal oxygen supplementation or even the use of noninvasive ventilation during sleep [3,4]. The early wells of transition from the NICU to the home can be challenging and feeding issues with slow weight gain can be anticipated.
There will be typically be frequent reviews with medical, surgical and nursing health care professionals regarding their respiratory and feeding status with an appropriate emphasis on establishing full sucking feeds in the early weeks and months following discharge. With all these appointments, the early focus on feeding may prompt health care professionals to accept slower than normal weight gains. However, if this slow weight gain persists, then a significant weight lag develops that may take months to years to overcome.
The consequences of poor growth in animal models suggest that protein malnutrition impairs lung growth and development [4]. In humans, early postnatal weight gain is driven by growth hormone [5]. Urinary growth hormone levels has been demonstrated to be low in infants with chronic neonatal lung disease [CNLD] who have compromised growth and tachypnoea and to improve with reduction in tachypnoea and improved growth trajectories [6]. In common with CNLD, infants with diaphragmatic hernia and oesophageal atresia/ tracheo-oesophgeal fistula with poor growth have increased caloric requirements due to tachypnoea and mild hypoxaemia and their growth improves
3
with reduction in respiratory symptoms after infancy7,8. Consequently, as improvement in oxygenation improves growth in CNLD [9], so the same may be applicable for infants with CDH and OA/TOF who have mild hypoxaemia. Thus, poor growth in CDH and OA/TOF is likely due to inadequate caloric intake to meet the infant’s elevated nutritional requirements that may reflect a combination of challenges with maintaining adequate caloric delivery as well as mild hypoxaemia.
Growth patterns in children with CDH Growth failure is common in CDH and has been attributed to increased metabolic demand related to elevated work of breathing, gastro-oesophageal reflux disease [GORD] and oral aversion [3, 1012]. Growth failure is reported in up to 50% of CDH babies, even when oral caloric intake is optimised [10-13]. Many such references date back over 10 to 20 years and the risk factors for poor growth include need for ECMO and discharge on home oxygen.
Despite evidence that aggressive nutritional management can bring about significant catch-up growth [3,7,10-13,], there is ongoing evidence in a more contemporary cohort of 45 CDH survivors [Born 2005 -2011] that growth remains a problem [7]. In this cohort, from our group at The Children’s Hospital at Westmead, Sydney, followed to 12 months of age, 56% had failure to thrive [FTT: Weight z score - 2 standard deviations for age] at 6 months of age and this had dropped to 21% at 12 months of age, with days of mechanical ventilation being predictive of FTT at 6 months of age. This result should be seen as being from a single centre which does not provide ECMO for CDH babies, but has an 82% survival rate for all comers [7]. The weight gain showed an improvement in weight z scores [+/- SD] from -2.07 +/- 1.17 to -0.85 +/- 1.32 between 6 and 12 months with feeding strategies including added calories in 58%, nasogastric feeds in 42% and gastrostomy tubes in 8% during the first 12 months. Oral aversion may be made worse by the prolonged use of nasogastric tubes and the use of gastrostomy tubes and so considerable time with speech pathology intervention may be required to establish adequate oral caloric intake [12,13] However, there is a lack of consensus about the best approach to this problem with
4
strategies ranging from observation to using increased intake of calorie dense foods, use of nasogastric tubes and consideration of gastrostomy tubes for more severe cases.
Growth patterns in children with OA/TOF Some infants with OA/TOF will have significant challenges to establishing feeds related to anastomotic leaks from the initial surgery, strictures requiring dilatation and episodes of pulmonary aspiration and pneumonia [1,5]. In an American series of 3479 infants [median birth weight 2.7 kg at 37 weeks gestation] managed in 43 US centres over 13 years to 2012, there was a outcome data to age 2 years in 83% [2]. The median length of stay following delivery was 27 days. Only 0.8% of patients received ECMO. The authors reported a 5.4% in hospital mortality rate, 91.5% received total parental nutrition [TPN] for a period of time, 11.4% had a repeat operation for their oesophageal construction, and 11.7% had a fundoplication. Interestingly, 17% had a readmission within 30 days, 55% of the cohort had readmission within 2 years and this included half who had multiple readmissions, 12.7% who had pneumonia on readmission, and 6.8% having at least five readmissions [2]. Unfortunately, longitudinal growth data was not presented in this paper.
With regard to growth, there are several groups who have presented their data, usually as part of a multidisciplinary follow-up clinic. In a single US centre study involving 69 infants born between 1972 and 1990, approximately 25% had height and or weight < 5% at age 5 years, and 15% at 510 years and 15% at > 10 years of those followed [5]. In another single centre series of 334 patients aged 1 to 37 years from Australia, born between 1948 and 1986, included 62 patients aged 0-5 years, 54 aged 5-10 years, 54 aged 10-15 years and 164 aged over 15 years [14]. Daily feeding difficulties were reported in approximately 10% in all ages and the proportion with gastrooesophageal reflux was reported as approximately 45%, 15%, 20% and 25% respectively across the age groups. Wasting rather than stunting was seen under the age of five years with approximately 18% having a weight < 10% and 13% having height < 10%, yet adult height and weight distribution was normal [14]. More recently, a small study from Holland compared infant lung function testing and noted no difference in lung function or growth up to two years of age
5
whether an open thoracotomy or a thoracoscopic [minimally invasive] approach was used [15]. This suggests a degree of growth restriction related to sub-optimal caloric intake in the early years. In more recent reports demonstrate a similar pattern of slow initial growth [8-11]. The French reported their outcomes to 12 months for 307 infants born in 2008-2009 and demonstrated that 15% were undernourished at 12 months of age [16]. A repeated association with poor growth is the lack of established feeds at the time of discharge from hospital after the initial surgery [16,17]. This reinforces our need to focus on optimising caloric intake in the first 12 months of life in order to optimise nutritional, respiratory and cognitive outcomes.
Methods of increasing calories for infants and young children with CDH and OA/TOF 0-6 months Exclusive breastfeeding is encouraged and supported for mothers of infants aged 0-6 months [18]. However, breastfeeding often isn’t feasible for infants with CDH and OA/TOF, especially during the
initial post-operative period in the NICU. It isn’t uncommon for these infants to receive periods of total parenteral nutrition (TPN) followed by enteral tube feeding (NG feeds) as oral suck feeds are slowly introduced. Where possible, a dietitian should be involved to assess growth and calculate estimated energy and protein requirements for these infants. Individualised feeding plans should be implemented in consultation with the multidisciplinary team (MDT). Expressed breastmilk (EBM) or standard infant formula alone, often doesn’t meet the elevated nutritional requirements for infants with CDH and OA/TOF. Commonly used supplements for EBM fortification include human milk fortifier (HMF), carbohydrate and/or fat additions and powdered infant formula. Care should be taken when fortifying EBM with carbohydrate and/or fat alone as this will result in a reduced protein to energy ratio of the feed and can hinder potential for optimal weight gain. Concentrated infant formulas with/without carbohydrate or fat additions and high energy/high protein ready to feed infant formulas may also be required for this population group where EBM isn’t available. Where possible, suck feeds should also be encouraged, even if supplementary nasogastric feeding is
6
required, in an attempt to prevent oral aversion longer term. Early input from the broader MDT, including speech pathology may also help with the progression of oral feeding and prevention of oral aversion [Figure 1].
6-12 months The introduction of solids is recommended from around 4-6 months of age whereby most children will be physiologically and developmentally ready [18]. It is important to assess the readiness for solids on and individualised basis for infants with CDH and OA/TOF. National infant feeding recommendations should be used to guide recommendations. In Australia, that includes introducing any food in any order whilst promoting iron containing foods e.g. fortified rice or pureed meat as a first food [18]. As an infant progresses with the introduction of solids and a greater portion of their intake is in the form of food, fat fortification of solids may assist with optimizing weight gain. Options for the fortification of solids are listed in Figure 2. Progression with textures should also be encouraged according to the child’s developmental stage. A speech pathologist may again be required to assist with strategies to combat oral aversion.
12-24 months Ideally children will have progressed to family foods, including an element of self-feeding by 12 months of age [18]. If children with CDH and OA/TOF have not established catch-up weight during infancy, further strategies to optimise growth may be required [Figure 3]. These include ongoing fortification of meals and the transition from fortified EBM or concentrated infant formula, to a 1.01.5kcal/ml paediatric formula. Fussy eating can also be a challenge for children of this age and can be particularly challenging when combined with an element of oral aversion and the need for ongoing supplementary enteral feeding. In addition to commonly employed fussy eating strategies, food play is a strategy employed by dietitians and speech pathologists to assist with oral aversion. For the more complex feeders, a referral to a MDT feeding clinic may be required for nasogastric tube weaning strategies.
7
Conclusion In this paper we have addressed the common clinical problem of slow weight gain in infants with structural surgical lung conditions such as diaphragmatic hernia and oesophageal atresia tracheooesophageal fistula. Through our experience in a multi-disciplinary clinic we have developed some feeding strategies with information sheets for parents that have proven helpful to families in an Australian setting. These are designed to be used in addition to recommended medical therapies, such as the treatment of gastro-oesophageal reflux where appropriate. Whilst food preferences and feeding practices may differ in various parts of the world, we hope these resources may prove beneficial for families.
Acknowledgements We would like to thank Dr Robert Halliday, Professor Nadia Badawi and Dr Karen Walker for their assistance in developing the parent information sheets.
8
Figures Figure 1
Parent information sheet for feeding infants 0 to 6 months
Figure 2
Parent information sheet for feeding infants 6 to 12 months
Figure 3
Parent information sheet for feeding children aged 12 to 24 months.
9
References
1. Holland AJ, Fitzgerald DA. Oesophageal atresia and tracheo-oesophageal fistula: current management strategies and complications. Paediatr Respir Rev. 2010; 11:100–106. 2. Sulkowski JP, Cooper JN, Lopez JJ, Jadcherla Y, Cuenot A, Mattei P et al. Morbidity and Mortality in Patients with Esophageal Atresia. Surgery 2014; 156: 483-491. 2. Robinson PD, Fitzgerald DA. Congenital diaphragmatic hernia. Paediatr Respir Rev. 2007; 8: 323-35. 3. Leeuwen L, Fitzgerald DA. Congenital diaphragmatic hernia. J. Paediatr Child Health. 2014; 50: 667-73. 3. Little DC, Rescola FJ, .Grosfeld JL, West KW, Scherer LR, Engum SA. Long Term Analysis of Children with Esophageal Atresia and Tracheoesophageal Fistula. J Pediatr Surg 2003; 38: 852-856. 4. Gaultier C. Malnutrition and Lung Growth. Pediatr Pulmonol 1991; 10: 278-286. 5. Gluckman PD. Fetal Growth: An Endocrinie Perspective. Acta Paediatr Scand 1989; 349: 2125. 6. Fitzgerald DA, Van Asperen PP, O’Leary P, Feddema P, Leslie G, Arnold J et al. Sleep, respiratory rate and growth hormone in chronic neonatal lung disease. Pediatr Pulmonol 1998; 26: 241-249. 7. Leeuwen L, Walker K, Halliday R, Karpelowsky J, Fitzgerald DA. Growth in children with congenital diaphragmatic hernia during the first year of life. J Pediatr Surg 2014; 49: 13631366. 8. Roberts K, Karpelowsky J, Fitzgerald DA, Soundappan SS. Outcomes of oesophageal atresia and tracheo-oesophageal atresia fistula repair. J Paediatr Child Health 2016; 52: 694-698. 9. Groothius JR, Rosenberg AA. Home oxygen promotes weight gain in infants with bronchopulmonary dysplasia. Am J Dis Child 1987; 1987: 141: 992-995.
10
10. Gien J, Murthy K, Pallotto EK, Brozanski B, Chicoine L, Zaniletti I et al. Short-term weight gain velocity in infants with congenital diaphragmatic henria (CDH). Early Hum Dev 2017; 106: 7-12. 11. Jaillard SM, Pierrat V, Dubois A, Truffert P, Lequiien P, Wurtz AJ et al. Outcome at 2 years of infants with congenital diaphragmatic hernia: a population based study. Ann Thorac Surg 2003; 75: 250-256. 12. Muratore CS, Utter S, Jaksic T, Lund DP, Wilson JM. Nutritional morbidity in survivors of congenital diaphragmatic hernia. J Pediatr Surg 2001; 36: 1171-1176 13. Cortes RA, Keller RL, Townsend T, Harrison MR, Farmer DL, Lee H et al. Survival of severe congenital diaphragmatic hernia has morbid consequences. J Pediatr Surg 2005; 40: 36-45. 14. Checuti P, Phelan PD. Gastrointestinal morbidity and growth after repair of oesophageal atresia and tracheo-oesophageal fistula. Arch Dis Child 1993; 68: 163-166. 15. Spoel M, Meeussen CJ, Gischler SJ, Hop WC, Bax NM, Wijnen RM et al. Respiratory Morbidity and growth after thoracotomy or thoracoscopic repair of oesophageal atresia. J Pediatr Surg 2012; 47: 1975-1983. 16. Schneider A, Blanc S, Bonnard A, Khen-Dunlop N, Auber F, Breton A et al. Results from the French National Esophageal Atresia Register: one year outcome. Orphanet J. Rare Dis 2014; 9: 206. 17. Castilloux J, Noble AJ, Faure C. Risk factors for short and long term morbidity in children with oesophageal atresia. J Pediatr 2010; 156: 755-760. 18. https://www.eatforhealth.gov.au/sites/default/files/files/the_guidelines/n56b_infant_feeding_su mmary_130808.pdf.
11
Figure 1
Parent information sheet for feeding infants 0 to 6 months
Feeding your CDH or OA/TOF baby: birth to 6 months
Babies born with Congenital Diaphragmatic Hernia (CDH) [abdominal contents in the chest through a gap in the diaphragm muscle] or oesophageal atresia / trachea-oesophageal fistula [OA/TOF] sometimes have trouble getting enough food. So many babies with CDH or OA/TOF need extra calories to help them grow. Food provides the energy to allow babies to grow.
Babies with CDH or OA/TOF need to work harder to breathe which means they need more food for energy. A dietician will probably tell you to give your baby formula as well as breast milk, special formula with extra nutrition or to feed your baby more often.
You won’t need to do this all the time, so talk to the dietician about how long your baby will need extra milk or solids [4-6 months onwards].
Never give your baby extra food without talking to a doctor or dietician first.
Sometimes, when babies work hard to breathe, they have trouble keeping food in their tummy, so they may start to vomit during or after feeding. If this happens, you may be advised by a dietician or doctor how to feed your baby smaller amounts of food more often, alter your breast-feeding approach or to give your baby special formula.
Some babies need to be fed through a tube that goes into their stomach through their nose. This is called a nasogastric (NG) tube.
If a baby has an NG tube for a long time, they may get an ‘oral aversion’, which means they don’t like food or bottles going near or in their mouth. Helping your baby to eat normally as they grow will help them get rid of the NG tube in time. As your baby grows, they will stop needing the NG tube.
Your baby’s doctor or dietician will give you lots of help and information if your child has an NG tube.
Regularly see a community health nurse or your local GP to check how your baby is growing.
If you have any questions or you are worried about anything, please speak to a doctor, nurse or dietician.
There are two more information sheets about feeding a child with CDH that may help you. Ask for a copy of the information sheet about babies aged 6 to 12 months and 1 to 2 years.
12
Figure 2
Parent information sheet for feeding infants 6 to 12 months
Feeding your CDH or OA/TOF baby: 6 to 12 months Babies born with Congenital Diaphragmatic Hernia (CDH) or oesophageal atresia / trachea-oesophageal fistula (OA/TOF) may be smaller than other babies but they usually start to catch up after six months. Good nutrition is important for all babies, but especially for babies with CDH as it can help improve breathing and lung function. Around six months of age, babies are usually ready to start eating solids. Some doctors or dietitians may tell you to give your baby solids before they are six months old, if they are having trouble getting enough milk. You will know your baby is ready to try solids if they: seem hungry between milk feeds look interested in the food others are eating have good head and neck control can sit up, if they have enough support. Even when your baby starts to eat solids, milk will probably still be the main type of nutrition for a while. For all babies, starting solids is something new and different. To help them get used to this new experience, here are a few tips:
When you first start, give your baby some solids after they’ve had some milk. At first your baby will probably only have a few teaspoons. Make sure things are quiet and relaxed around your baby. Your baby will tell you when they’ve had enough by pushing away the spoon, turning their head away or refusing to open their mouth.
DON’T FORCE FEED. Your baby won’t enjoy it and it can lead to feeding problems.
WHAT TYPE OF FOODS SHOULD YOU GIVE YOUR BABY? Food that is high in iron, such as fortified rice cereal or pureed meat, should be some of the first foods you offer your baby. As your baby gets used to eating soft food, you can start to slowly give your baby different types of food with bigger pieces. By the time your baby has their first birthday, they should be eating the same food as the rest of the family. Your doctor or dietician may suggest you give your baby fortified milk to grow better If your baby is breastfed, or doesn’t need special foods, it is good to add some healthy fats to their diet. See the table below for some ideas. This is a good way to help them gain more weight. When your baby reaches normal weight, and is growing normally, you can stop. FOOD/FLUID Oil (olive/canola)
Margarine / butter Avocado Dairy products Cheese Cream
Add 1-2 teaspoons into pureed baby foods (homemade or commercial) Drizzle over the top of rice, noodles, pasta and vegetables Add to pureed foods or spread thickly on sandwiches, toast, crackers and biscuits Add to pureed foods or mash with sour cream and use as a dip with vegetables Full fat dairy products are recommended for children under 2 years old Grate into pureed vegetables, pasta, rice, bolognaise sauce, casseroles or baked beans Add 1-2 teaspoons to purees, mashed potato, soups, casseroles, cereals, custard or yoghurt
There are two more information sheets about feeding a child with CDH or OA / TOF that may help you. Ask for a copy of the information sheet about babies aged birth to 6 months and 1 to 2 years.
13
Figure 3
Parent information sheet for feeding children aged 12 to 24 months.
Feeding your CDH or OA/TOF baby:12 to 24 months
By their first birthday, some babies (around 1 in 4) with congenital diaphragmatic hernia (CDH) or oesophageal atresia / trachea-oesophageal fistula (OA/TOF) still grow more slowly than other babies do. However, all children do start to grow slower when they reach toddler age.
Children do not need a lot of food to grow well, but they do need a range of healthy foods to make sure they get the best nutrition.
Toddlers love to explore and try new things and mealtimes can be very messy as toddlers throw, squash and play with their food. This is an important and natural part of toddler development.
Choosing and refusing food is a normal way for children show their independence.
Not all toddlers will eat the same amount every day – it is normal for them to eat lots one day and little the next.
It can be stressful if your child is a fussy eater, especially if it has been important part of managing their growth and health.
As a parent or carer, you are responsible for what, when and where your child eats. But you cannot make them eat – how much (if any) your child eats is up to them. Give your child the best food you can and try to not to worry too much if they are fussy.
Tips for dealing with a fussy eater: 1. Do not prepare a ‘special’ separate meal for your toddler 2. Don’t spend too long trying to make them eat. Stop after half an hour. 3. You may need to cut up food that is hard to chew. 4. Meals and snacks should be eaten at the table, with no distractions 5. Do not force feed as your toddler will hate it and it may cause feeding problems later. 6. Don’t give extra foods or favourite foods as a ‘reward’ for eating 7. Be patient – it can take time for your child to eat well. 8. Give your child small amounts of food. Your child may feel worried if they see too much food on their plate. 9. Talk about interesting things during meals, rather than focussing on food
If your child does not want to eat, say ‘have you finished eating?’ If they have, tell them to move away from the table and make sure they know that they won’t be having any more food until the next mealtime. 14
Try not to worry; your child will eat if they are hungry.
If you think your child’s is growing too slowly, you can add some healthy fats to their diet. See the table below for some ideas. This is a good way to help them gain more weight. When your child reaches normal weight, and is growing normally, you can stop.
FOOD/FLUID Oil (olive/canola) Butter/Margarine Avocado, humus or dips Dairy products
Cheese
Cream
WHERE TO USE Add 1-2 teaspoons into meals such as pasta and sauces Add to meals or spread thickly on sandwiches, toast, crackers and biscuits Add to meals or mash with sour cream and use as a dip with vegetables Full fat dairy products are recommended for children under the age of 2 years old, including milk, yoghurt, cheese, cream cheese, custard, ice cream and sour cream Ideas: serve yoghurt/custard with mashed banana/fruit pieces to dip or use cream cheese as a dip Grate or melt cheese over vegetables, pasta, rice, bolognaise sauce, casseroles or baked beans Add 1-2 teaspoons to fruit or vegetable purees, mashed potato, soups, casseroles or cereals
There are two more information sheets about feeding a child with CDH or OA/TOF that may help you. Ask for a copy of the information sheet about babies aged birth to 6 months and 6 months to 12 months.
15