Polish experience in nutrition support in children

Polskie doświadczenie w leczeniu żywieniowym dzieci

Justyna Laskowska, Joanna Friedman-Gruszczyńska, Katarzyna Popińska, Małgorzata Łyszkowska1, Janusz Książyk
Department of Pediatrics
Head of Department: prof. dr hab. n. med. J.B. Książyk
1Department of Surgery
Head of Department: prof. dr hab. n. med. P. Kalicinski
The Children’s Memorial Health Institute, Warsaw, Poland

Abstract
Organization of enteral nutrition programme in Poland has developed rapidly in the last years, however, the underdiagnosis and late diagnosis of malnutrition are still the major challenges. For those children who are unable to tolerate enteral diet, intravenous support is required. Main achievements in our parenteral nutrition programme (PN) consist in decreasing sepsic complication rate and introducting fish oil based emulsions for prevention of PN-related liver disease. The challenge to combat in the future comprises development of a network of nutritional centres covering the whole country, which will take care of patients requiring nutrition support. The Children’s Memorial Health Institute in Warsaw will remain the reference centre for the management of the most complicated cases. The organization of postgraduate courses should lead to continuous reduction of nutrition related complications rate. The important problem remains the relatively low number of patients weaned off PN. The widespread introduction of intestine rehabilitation programme is essential to improve this issue.

Key words: parenteral nutrition, enteral nutrition, children

Streszczenie
Rozwój leczenia żywieniowego w Polsce u dzieci, od wielu lat podąża za wytycznymi opracowywanymi przez międzynarodowe organizacje, towarzystwa naukowe i wiodące ośrodki żywieniowe na świecie. Zdecydowana większość dzieci wymagających sztucznego żywienia w Polsce żywiona jest jedynie drogą dojelitową. W ostatnich latach rozwój żywienia dojelitowego uległ znacznemu przyspieszeniu, pomimo to nadal głównym problemem pozostaje zbyt rzadkie i zbyt późne rozpoznawanie niedożywienia. Dzieci, które nie mogą być żywione drogą przewodu pokarmowego, włączane są do programu żywienia drogą dożylną. Do najważniejszych osiągnięć naszego ośrodka w zakresie przewlekłego żywienia pozajelitowego należą znaczące zmniejszanie liczby powikłań septycznych oraz wprowadzenie emulsji lipidowych z zawartością oleju rybiego w profilaktyce choroby wątroby związanej z żywieniem pozajelitowym. Dużym wyzwaniem na przyszłość jest stworzenie sieci ośrodków żywieniowych obejmujących całą Polskę, tak aby ułatwić dzieciom dostęp do leczenia żywieniowego. Centrum Zdrowia Dziecka pozostawałoby ośrodkiem koordynującym i referencyjnym dla najbardziej skomplikowanych pacjentów. Celem organizowanych szkoleń podyplomowych jest w pierwszej kolejności zmniejszenie ilości powikłań leczenia żywieniowego. Ważnym problemem pozostaje stosunkowo mała liczba pacjentów odłączanych od przewlekłego żywienia pozajelitowego. Aby poprawić tę sytuację wdrożono program rehabilitacji jelit, który dzięki systemowi szkoleń w niedługim czasie ma zostać rozpowszechniony w ośrodkach prowadzących leczenie żywieniowe.

Słowa kluczowe: żywienie pozajelitowe, żywienie dojelitowe, dzieci

INTRODUCTION
Nutrition support is defined as the provision of nutrients orally, enterally or parenterally with therapeutic intent (1). Proper nutrition is a basic issue in paediatric healthcare. Not only must it provide sufficient nutrient intake to maintain nutritional status but also sustain growth and neurodevelopment. The progress of nutrition support in children in Poland has closely followed trends selected by international organizations. Several comprehensive evidence-based guidelines have been published recently. Current Guidelines on Paediatric Parenteral Nutrition of the European Society of Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN), and the European Society for Clinical Nutrition and Metabolism (ESPEN) supported by European Society of Peadiatric Research (ESPR) have been available since 2005 (2). The clinical practice guide for pediatric enteral nutrition was published by ESPGHAN Committee on Nutrition in 2010 (3). On the basis of available evidence the ASPEN recommendations of parenteral and enteral nutrition were published as well (4). Polish standards worked out by the Polish Society of Parenteral and Enteral Nutrition complete international guidelines and adapt them to national regulations and reality (5). Despite the acknowledgement about the importance of proper feeding, malnutrition still exists and is an underestimated problem. It concerns 15-30% of pediatric patients in European countries according to the recent data (6). Significant effort has been put to improve nutritional care, but still there is a field for improvement.

Historical background
Twenty five years ago Polish physicians, pharmacists and dieticians organized the Section of Parenteral and Enteral Nutrition of the Polish Physician Association, which then became an independent society. Sixteen years ago the first Polish handbook about nutrition management was published (7). THe development of nutrition support in paediatric hospitals has been observed in all academic centres as a result of educational efforts brought about byThe Children’s Memorial Health Institute, Warsaw. Since 1992 home parenteral nutrition has been applied in Polish children and hundreds of patients have been treated at homes. In the last 2 years two additional centres of home parenteral nutrition were established (in Gdańsk and Cracow). In the last 5 years we observe constant increase in the rate of enteral support both at the hospital level and at homes.

Enteral nutrition. Current status and perspectives
Enteral nutrition support is defined as delivery of food beyond the oesophagus via tube, either to the stomach or postpyrolically, however in recent guidelines (8) the definition includes also special oral nutrition supplements. this method of nutrition support is indicated in situations, when energy and nutrient requirement cannot be met by regular oral food intake in a patient with at least partially functional gut.There are many clinical conditions in which enteral nutrition might be needed, including diseases leading to inadequate oral intake such as neurologic impairment or congenital abnormalities of the upper gastrointestinal tract; disorders of digestion and absorption, diseases related to increased nutritional requirement or losses such as cystis fibrosis, chronic organ failure (3).
Thus this problem seems to be frequent, however it is still fairly underdiagnosed. Currently there are several centres in Poland providing home enteral nutrition programmes. Administration of enteral nutrition, as was already stressed above, may be gastric or postpyloric, via tubes or via stomas.The choice of access should take into consideration the prognostic duration of enteral nutrition, functional integrity of gastrointestinal tract and the risk of aspiration. Whenever enteral feeding is expected for longer than 6 weeks, a stoma should be considered (3). Gastro- and enterostomies can be placed via laparotomy, laparoscopy or endoscopy.The advantage of endoscopy is lower cost and less invasive procedure, less complications, shorter time to feeding restart, however, certain contraindications such as laparatomies in medical history, patient’s low weight or the need to proceed fundoplication or pyroloplasty alongside with stoma placement propose to choose surgical method. In our centre, before stoma placement, gastrointestinal tract radiological examinations are performed in order to identify patients with GER (gastroesophagous re"ux), slower gastric emptying or anatomical abnormalities.There is no evidence in the literature whether neurologically impaired patients with higher risk of GER need to have fundoplication performed routinely, thus different centres vary in standards in that matter (9, 10, 11).
Antibiotic prophylaxis before each procedure is indicated regardless of which method of stoma placement has been eventually chosen (3). Complications of stoma placement, however rare, include perforations, wound infection, peritonitis, hepatic or colonic injury. Early refeeding (after 6 hours) after gastrostomy insertion is recommended (3). Enteral nutrition formulas are a balanced mix of all essential nutrients that can serve as a sole source of nutrition. Different preparation vary in proportions of nutrients, caloric density, degree of protein hydrolisation, type of lipid used (LCT vs MCT), osmolality and added supplements (such as fibre). The choice of the formula for enteral nutrition depends on age (children require a reduced renal solute load and a higher concentration of vitamins and minerals – adult formulas can be used only after the age of 10 years), on underlying disease and on absorptive capacity of the intestine. Polymeric feeds, based on cow milk protein, are adequate for most patients. Hydrolyzed or elemental formulas are indicated in situations of food intolerance or impairment of intestinal absorption and digestion. Enteral nutrition can be delivered by intermittent bolus feeding or by a continuous infusion. The first method is more physiological, providing cyclical surges of gastrointestinal hormones that have a trophic e"ect on the intestinal mucosa. Continuous infusion feeding is indicated in case of postpyloric feeding and in patients, who fail to tolerate gastric bolus feedings (for example because of GER) – this method of feeding is more complicated with the need to use a special pump. After enteral nutrition introduction, complications, such as feeding intolerance manifested by emesis, diarrhea, bloating or refeeding syndrome, should be carefully monitored. During the hospitalization when enteral nutrition is introduced, parents are trained in methods of enteral nutrition delivery, nursing the enteral access and early identification of any complication, including tube-related, local irritation or infection. If any of these complications is suspected after discharge, parents have a hot-line telephone number to the nutrition team of our hospital available 24 hours a day. Control visits in the Outpatient Nutrition Clinic are planned in order to monitor nutrition status and enteral access condition – enteral tubes are changed if needed. After maturation of gastrostomy (4 months after insertion) a skin level low-profile devices are preferred. On the basis of the experience of our Nutrition Ward, recommendations concerning enteral tube nursing and change were published (12).
Organization of enteral nutrition programmes in Poland has developed rapidly in the last years. But still underdiagnosis and late diagnosis of malnutrition are major challenges for the near future. Another problem which needs to be resolved is the lack of refunding of enteral nutrition formulas for patients able to be treated orally with no enteral access. This situation leads to significant financial problems for most of the families, nevertheless this method of nutrition treatment is encompassed by the ESPEN definition of Enteral Nutrition.

Parenteral nutrition
Parenteral nutrition (PN) is the supply of fluids, calories, proteins, vitamins, minerals and trace elements directly into the vein. This intervention is to provide essential nutrients to patients who are unable to tolerate adequate enteral feeding or for whom enteral feeding is insu%cient or contraindicated to maintain nutritional status, growth and development. This kind of treatment has become the routine management for children with intestinal failure. Parenteral nutrition is fully available for children in hospitals. The National Health Fund covers full cost of this procedure.
The majority of children require PN for limited period of time while the digestive and absorptive function of the gastrointestinal track either matures or recovers. For the children who require prolonged intravenous nutritional treatment, home parenteral nutrition programme has been created.

Home parenteral nutrition
The Children’s Memorial Health Institute in Warsaw is the centre looking after the vast majority of children on home parenteral nutrition in Poland. Recently, there are two other centres – in Cracow and in Gdansk.
They are taking over the care of some patients from within the area of North and South part of Poland.
Children are qualified to the home parenteral nutrition (HPN) programme when their general health condition is stable and does not require hospitalisation, the expected time of PN dependency exceeds 3 months, parents are motivated, responsible and able to cope with technical and medical problems associated with artificial nutrition. Proper home conditions are also obligatory. Children referred to HPN require special venous access to supply nutritional mixture. Tunneled central venous catheters type Broviac or Groshong are surgically inserted in all our patients. On the basis of the experience gained in our Pediatric Department, recommendations on this catheter nursing were published and became national standards (13).
Close cooperation between parents and nutritional support team is essential during home parenteral nutrition. Children’s discharge is preceded by the parents’ training programme, which takes place in our hospital during hospitalization of the children. It lasts 3-6 weeks depending on caregivers’ abilities and progress in skills. Process of learning includes central venous catheters management, setting parameters of the infusion pump, line connection and disconnection, nutrient solutions storage. Most parents have to learn to prepare the mixtures from basic ingredients. Our hospital pharmacy is able to supply ready nutritional mixtures for 24 patients only. The rest of the children have their nutrition bags prepared by parents at home due to organizational reasons.
The special multidisciplinary nutritional care team including the physician, pharmacist, dietitian, nurse and psychologist trains, supports parents and looks after the children. Parenteral nutrition is a life-saving procedure but children must be carefully monitored to avoid or minimize the risk of several complications. These can be broadly divided into two main groups: catheter-related and metabolic complications. The main complications include: sepsis, exit site infections, thrombotic complications.
Long-term parenteral nutrition is the risk factor for metabolic complications. Parenteral nutritionassociated liver disease and metabolic bone disease constitute two main problems. The pathomechanism of these complications is multifactorial and includes underlying diagnosis, components and proportions of parenteral mixture, previous complications. One of the methods to prevent and reverse parentaral nutrition associated liver disease is to use fish oil as a component of the mixture.

MATERIAL AND METHODS
The Outpatient Nutrition Clinic of Children's Memorial Health Institute in Warsaw has one hundred one children on enteral nutrition under it's care. Children's age varies from eight months to twenty three years (mean age – seven years). Most of them – twenty six percent are patients with neurological impairment associated with swallowing disorders.
Currently, there are one hundred twenty eight children on HPN under the care of our Institute. The youngest child is four months old, the oldest one is three hundred twenty three months old, the mean age is seventy eight months. Hundred twenty three of them have primary digestive disorder and five of them have non-primary digestive disorder. The main diagnosis is short bowel syndrome caused most often by neonatal complication like necrotizing enterocolitis or congenital disease (gastroshisis, bowel atresia etc.). The length of the remaining bowel can not always be su"ciently estimated by the surgeons. The indications for home parenteral nutrition is listed in table I.

RESULTS

Assessment of enteral support is di"cult because of the heterogeneity of the group studied. On average, nine patients (7%) each year wean off parenteral nutrition. The number of patients referred to our centre is similar each year but the number of children remaining on home PN grows. This is due to the fact that less children are weaned off PN then are included in the HPN programme.
In our 5-year retrospective observations set up to assess the causes of removal of tunneled central venous catheters in children on home parenteral nutrition conducted from the March 2004 to February 2009, there were 184 patients under the Paediatric Department care. The total of 163267 catheter-days were analyzed. Twenty four thrombotic complications were noted with the incidence of 0.15 per 1000 catheter-days. Most of them were successfully treated with alteplase and associated with catheter displacement. During the same period of time, 196 catheter- related sepsis and 75 exit-site and tunnel infections were observed with the incidence of 1.2 per 1000 catheter-days and 0.6 per 1000 catheterdays, respectively. There was need of catheter removal in 11% of bloodstream infections and 78% of exit-site and tunnel infections (14). These results are comparable with other data from European and American studies (15, 16). Our results have improved compared to previous data published eleven years ago (17). Surprisingly, as we concluded in the prospective, one year observation study, the risk of catheter-related sepsis was lower in children receiving parenteral mixture prepared by their trained parents at home in comparison to children on mixture delivered from hospital pharmacy (18).
In the prospective study conducted in our department, the use of the fish oil based emulsion as a single source of lipids was associated with signi cant reduction of bilirubin, aminotrasferase and GGTP level after 1 to 44 weeks of administration (19).

CONCLUSIONS
Main challenges to combat in the future comprise development of a network of nutritional centres covering the whole country to improve the care of patients requiring nutritional support. This aims to reduce the incidence of malnutrition and facilitates access to specialized nutritional teams. The Children’s Memorial Health Institute in Warsaw will remain the reference centre for the management of the most complicated cases. The organization of postgraduate courses should lead to continuous reduction of the complication rate. The important problem remains the relatively low number of patients weaned off PN. The widespread introduction of intestine rehabilitation programme is essential to improve this issue.


REFERENCES
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Adres do korespondencji / Address for correspondence:
Justyna Laskowska Department of Peadiatrics Children’s Memorial Health Institute Al. Dzieci Polskich 20, 04-730 Warsaw