Sodexo Dietetic Intern

Sodexo Dietetic Intern
Nutrition Interventions for Pediatric Patients with Short Bowel Syndrome Jackie Costantino Sodexo Dietetic Intern

I want you to imagine its your 6th birthday
I want you to imagine its your 6th birthday. You’re sitting in front of your cake, the candle is lit and all of your friends and family are sounding, singing and waiting for you to make a wish and blow out your candle. Knowing that everyone will enjoy that cake except for you, your only wish is for a cure.

“I just want to eat everything.”
Austin Rath This is the unfortunate reality of Austin Rath, a six-year old boy from Pittsburgh who suffers from SBS. He’s had 90% of his bowel removed and has never been able to eat or drink anything. His only wish is to eat a cupcake and experience the simple enjoyment of food. Something that we easily take for granted. I just think he is so inspiring, and he has motiviated me to find a SBS patient at St. Christopher’s as the focus of my case study. “I just want to eat everything.”

Outline Discussion of SBS and current treatments
Medical Nutrition Therapy Case Study Patient Questions -First, I’m going to talk about what SBS is, what causes it, the signs and symptoms, the pathophysiology and the current treatments. -Then, I will talk about medical nutrition therapy and how it applies to SBS -Followed by my case study patient and his course of care at St. Christophers Hospital for Children - And at the end, I will take any questions you may have

What is Short Syndrome? Bowel
So what is short bowel syndrome? Is it something that you’re all familiar with? Have you seen sbs pts or learned about it in class? Do you know someone with sbs?

What is SBS? Significant loss of bowel length leading to malabsorption of fluid and nutrients 7 out of 1,000 live births for neonates with birth weights <1500g Risk  with  birth weight & gestational age Outcome based on many variables: length, anatomy of bowel resection, functional mass May be accompanied by intestinal failure (IF) -Well basically it’s a significant loss of the length of the bowel, which then leads to loss of the ability to digest and absorb fluid and nutrients. -In my research, I wasn’t able to find a good statistic on the prevalence of SBS. I did find that SBS occurs in 7 out of 1000 live births for neonates with birth wts <1500g. However, across the board, I found that it is difficult to describe the prevalence of this disorder. -The outcomes for SBS patients depend on many variables such as the length and anatomy of the remaining bowel and the mass that remains functional. Outcome based on many variables: NO SBS patient is the same! -Importantly it’s a very serious condition, and often accompanied by intestinal failure and definitely is life altering for a patient.

SBS Associated Intestinal Failure
Definition in the pediatric population: Insufficient intestinal mass to… Absorb and digest fluid and nutrients Maintain fluid, protein-energy and micronutrient balance for normal growth and development Acute IF: Dependent on PN for 4-6 weeks Chronic IF: Dependent on PN >90 days Major difference between adult and pediatrics is the concern for proper growth and development That is the major difference between SBS in the adult population compared to the pediatric population. We’re obviously very concerned with proper growth and development in infants and children when they are at such high risk for malnutrition.

Etiologies Squires R et al . J. Pediatric. 2012
The etiologies of SBS are another major difference between the adult and pediatric population. Adult’s typically get SBS from Crohn’s disease. SBS in pediatrics is usually caused by a congenital defect that in some way leads to intestinal tissue death that requires a surgical bowel resection. Etiologies most often seen in pediatrics include necrotizing enterocolitis (NEC), gastrochisis, intestinal atresia, a volvulus, aganglionosis and others. SBS may also result from a combination of these conditions. As you can see form this pie chart, NEC is the most prevalent cause which is intestinal tissue death. Squires R et al . J. Pediatric. 2012

Gastroschisis Congenital defect when an infant's intestines protrude from the body through one side of the umbilical cord Although it is possible to fix this situation through surgical procedures, there will most likely be some resulting tissue death. A performed silo is one way to fix gastroschisis, which gradually places the intestines back into the body. During a performed silo, a flexible ring is inserted into the abdomen while the intestines sit inside of a bag. The bag is made smaller and smaller as the intestines go into the abdomen until they are fully inserted and the hole is closed. Another option is to surgically enlarge the hole, place the intestines into the abdomen and close the hole. The method of surgical treatment for gastroschisis is determined on a case-by-case basis. (7) birthdefects/Gastroschisis-graphic.html

Midgut Volvulus Involves the entire midgut twisting around the super mesenteric artery (SMA), cutting off the blood supply Midgut includes: Distal duodenum Ileum Colon Transverse colon A volvulus can occur anywhere along the GI tract from the stomach to the colon, but the most common is a midgut volvulus. The midgut includes the distal duodenum, all of the jejunum and ileum, and the colon up to the transverse colon. The superior mesenteric artery (SMA) supplies this entire section of the GI tract. A midgut volvulus involves the entire midgut twisting around the SMA, cutting off the blood supply. (8)

Signs & Symptoms: Pre-resection
Dependent on the etiology of SBS Broad signs and symptoms bilious vomiting abdominal pain abdominal distention tachycardia tachypnea shock bloody stools Generally, GI disturbances

Complications Post-resection
Intolerance and malabsoption Diarrhea Steatorrhea Nutritionl deficiencies Weight loss (acute malnutrition) Growth stunting &  head circumference (chronic) Dry scaly skin Brittle hair and nails Poor wound healing Post-resection, SBS patients may experience various signs and symptoms of feeding intolerance and malabsorption due to decreased surface area, shorter transit time. 2 signs of intolerance and malabsorption: diarrhea and steatorrhea. Diarrhea can result from carbohydrate malabsorption, decreased transit time, high osmotic load or excessive digestive secretions into the intestines. Steatorrhea- fat that malabsorbed and lost in feces secondary to malabsorption of bile acids. **These signs and symptoms usually occur when fluid and nutrient intake exceed the absorptive capacity of the remaining bowel. Specific nutrient deficiencies depend on the section of missing bowel because certain digestive secretions and nutrient absorption occur in different locations along the GI tract. (12)

Absorption Of Nutrients Along the GI Tract
Risk for specific nutritional deficiencies depend on the anatomy of the small bowel resection -There is a lot of detail on this graphic, but it’s important because it shows that the risk for specific vitamin and mineral deficiencies depends on the section of bowel missing. -For example, Iron absorbed in duodenum and jejunum, if you’re missing the part that absorption of iron at risk for iron deficiency

Pathophysiology: 3 Phases
Immediate post-operative phase (1-7 days) Loss of communication between stomach and small intestine Poor absorption Loss of fluid and electrolytes Adaptation Intestinal growth and morphological development EN is initiated critical to adaptation Can increase absorptive capacity by 4X the initial capacity Intestinal Autonomy 100% EN is achieved -A large volume of fluid and electrolytes may be lost and require replacement through intravenous (IV) isotonic fluid to maintain hydration. (2) -Adaptation is when the body attempts to make up for what it lost, the cells in the intestinal lining are multiplying, the villi are growing and all of this increase in mass increase absorptive capacity. The time can vary for how long this occurs. It can take up to 24 months or longer. It’s critical here to introduce EN. -Intestinal Autonomy: Unforunately, this is not always achieved, and they remain dependent on pn for months or years. Some may never reach intestinal autonomy

Labs & Tests LFTs BMP CBC Prealbumin & CRP Tryglycerides
Calcium, phosphorus, magnesium Fat soluble vitamins (ADEK) Vitamin B12 Serum zinc levels Endoscopy & colonoscopy Labs are obviously very important to continuly monitor, here are a list of labs and test that are typically. Bc at risk for liver damge, lfts are important, nutritionally important is prealbumin and crp and the vit and minerals Important labs include a liver panel (bilirubin, ALT, AST, total protein and albumin), a basic metabolic panel (BUN, CO2, creatinine, glucose, serum chloride, potassium and sodium), prealbumin, triglycerides (TG), calcium, phosphorus, magnesium, a complete blood count (white blood cells, red blood cells, hemoglobin, and hematocrit), fat soluble vitamins (A, D, E, K), Vitamin B12 and serum zinc levels. Bilirubin is a byproduct of the breakdown of red blood cells. It normally passes through the liver for breakdown and into the stool for excretion. When the liver is damaged, this process may not occur leading to increased levels of bilirubin in the bloodstream. The normal value is 0-1.5mg/dL (1 month to adult) for total bilirubin and <1.5 mg/dL (newborns) and mg/dL (1 month to adult) for conjugated bilirubin.

Treatment Options Surgical interventions
Intestinal transplantation Intestinal lengthening procedures Substances indicated to promote adaptation Growth hormone (GH) Glutamine Glucagon-like peptide 2 (GLP-2) The treatment options are limited and there is no cure. A surgical transplant makes sense, sounds like a good plan, however many challenges and survival rates not so good. Mortality rate in US: 40% 5-years later, 50% 10 years later Talk about lengthening procedures in a minute. There are also substances that may promote adaption, none of which that are widely used currently.

Intestinal Lengthening Procedures
Bianchi Procedure STEP Procedure 2 procedures that are done to inc intestin length. Diff cuts, longitud into 2 loops, the second cuts diagnal, which also gives more length, good, but don’t have absoprtive capacity, so they are helpful to increase length but not for absorption. The Boston group’s 2009 data shows a mean increase in bowel length of 9138%. They also reported that EN tolerance improved by 1.4% per month, and 6 patients (38%) transitioned off TPN. Both lengthening procedures are promising management tools to improve intestinal function in SBS patients. (14) STEP- serial transverse enteroplasty

Substances Indicated to Increase Adaptation
GH (FDA approved in adults) Zorbtive® (somatropin rDNA origin for injection) 191 amino acid peptide hormone GH + glutamine may stimulate intestinal growth GLP-2 (not FDA approved) Gattex® (teduglutide) 33 amino acid peptide and growth hormone Adult studies show  dependence on TPN So the substances that promote adaptation are GH, glutamine and GLP-2 GH is growth hormone approved for use in adults, but the efficacy has been question based on the research that was done before approval GLP2- glucagon-like peptide 2 has shown to dec dependence on tpn, but it is not yet FDA approved in adults and neither have been studied in a pediatric population.

Medical Nutrition Therapy
Crucial Component to SBS Management

Role of the RD Evaluate nutritional status
Identify malnutrition and growth failure Improve patients nutritional status through interventions Obviously rd’s play a major in the managem Growth and dev so important at this time The rd has to work on the management team, so even if they are not directly involved in the central line or the labs, they need to be aware of all the pieces of the

Goals of the RD Goals of the RD
To ensure patient is receiving 100% nutritional needs for proper growth and development Initiate EN as soon as medically appropriate Wean patient from TPN to reduce associated risks End goal 100% EN

ADIME Assessment Diagnosis Interventions Monitoring and Evalulation
Adime an acrro youre all familiar with Over the next few min I’m going to talk about how it is used in sbs and later I’m

Assessment Patient’s history Anthropometrics “Ins and Outs”
Stool characteristics Feeding access points Food history Estimated needs Physical observations Medications and supplements Laboratory and diagnostic tests For the assessment, it’s important to collect all of the important information that can build the story of the patient’s case. The type of information gathered in a pediatic patient is similar to an adult patient as you can see here but I just want to point out some of the key differences. Important to find out birth history, especially in SBS because SBS usually stems from a congenital defect. You also want to note if the patient was born full term or preterm bc that will affect how you assess antrhopometrics and estimated needs. 2) For anthropometrics, you plot the patient’s wt, ht and head circumference on a growth chart 3) Also, estimated needs vary widely depending on many variables such as age, gender and medical status…next slide

Assessment Estimated Needs Pediatric Nutrition Care Manual:
Calories: Estimated Energy Requirement (EER)  1.2 Protein: DRI  1.3 Pediatric Reference Guide of Texas Children’s Hospital: Calorie needs: DRI x There are multiple ways to estimate a child’s needs, but here are 2 recommendations specific to SBS

Diagnosis Common problems for SBS: Example PES statement SBS:
Increased nutrient needs (NI-5.1) Altered gastrointestinal function (NC-1.4) Impaired nutrient utilization (NC 2.1) Example PES statement SBS: Altered gastrointestinal function related to short bowel syndrome (___cm remaining), as evidenced by inability to tolerate full enteral feeds and need for parenteral nutrition support. Once the patient has been thoroughly assessed, an RD can use the signs and symptoms to make a nutrition diagnosis. A nutrition diagnosis is written in the form of a PES statement: Problem, Etiology and Signs/Symptoms.

Interventions Parenteral Nutrition Enteral Nutrition Cycling
Lipid Reduction Therapy Omega-3 fatty acids for PN lipids Ethanol lock therapy Enteral Nutrition Nutrition source Continuous vs. Bolus Modulars

Total Parenteral Nutrition (TPN)
Essential when intestinal failure (IF) is present Necessary for proper growth and development, but NOT ideal route for nutrition! Associated with 2 main causes of death among SBS PN-associated liver disease (PNALD) Central line infections Extent of IF varies for each patient. Patients with extensive short bowel resections will require PN in the immediate post-operative phase, and may be dependent for years. **Major difference between adult and pediatric SBS is that we are very concerned for proper growth and development Remember goal #1 Patient must grow, TPN is necessary for growth and development, but NOT ideal route for nutrition because it’s associated with PNALD and centrall line infections, the 2 main causes of death among SBS syndrome patients

PN-Association Liver Disease (PNALD)
Most prevalent and severe complication of long term PN 27% in children and 85% in neonates Risk of death  8 fold when cholestasis is present Largest predictor of death in SBS patients PNALD is the most prevalent and severe complication of long-term PN dependence. It is the largest predictor of death in SBS patients. De Meijer et al. reports the prevalence of PNALD is approximately 27% in children and 85% in neonates on long-term PN. The progression of PNALD begins with steatosis when fatty acid deposits build up in the liver. Then, cholestasis occurs when the flow of bile from the liver is slowed. The risk of death increases 8 fold when cholestasis is present as seen in Figure 9 (14). Cholelithiasis presents next, which is the development of gallstones from bile acids. Ursodeoxycholic acid (Actigall) can be given to treat gallstones or prevent their development. Liver injury triggers hepatic fibrosis, which then progresses to biliary cirrhosis, portal hypertension and end-stage liver failure (ESLD), subsequently. About 15% of patients on long-term TPN progress all the way to ESLD (25). Once ESLD is reached, the only treatment options are liver transplant or a combined liver-small bowel transplant, which are both associated with a high mortality rate.

PN-Associated Liver Disease (PNALD)
Nutritional interventions to reduce risk of PNALD: Wean from TPN (#1) Cycling TPN Lipid reduction therapy Omega-3 fatty acids for PN lipids

Lipid Reduction Therapy
Reducing lipids to 1g/kg/day 3 times per week has shown to improve bilirubin levels and resolve cholestasis in SBS patients without causing EFAD. The University of Michigan showed If full TPN dependence is required, reducing parenteral lipid load to 1g/kg three days per week has shown to reduce cholestasis without causing essential EFAD. A prospective study on the reduction of standard lipid administration was conducted at the University of Michigan from August 2005 to September The treatment group included 31 surgical NICU patients on TPN with a direct bilirubin of 2.5 mg/dL. The control group included 27 historical NICU patients with PNALD. A case match revealed no significant differences between the groups (i.e. diagnosis, GA, birth weight), except the treatment group contained 74% males compared to 34% in the control group. The control group received daily administration of standard lipid protocol, 3g/kg/day of soybean-based lipids. For the treatment group, lipid regimen was reduced to 1g/kg/day and administered two days per week. As demonstrated in Figure 10, results show a decrease in predicted total bilirubin levels among the treatment group (slope of , p=0.046) compared to a slight increase in the control group (slope of 0.007, p=0.574). The difference in slopes was significant (p=0.0394).

Lipid Reduction Therapy
Prospective study at the University of Michigan 31 NICU patients on PN with direct bili of 2.5 mg/dL Treatment group: 1g/kg/day 2 times per week Control group: 3/kg/day daily EFAD monitored monthly results showed a  in total bilirubin levels among the treatment group compared to a slight increase in the control group. The difference in slopes was significant (p=0.0394).

Results Treatment group:  bili levels
Control group: slight  bili levels Treatment group developed mild EFAD, but resolved when lipids increased to 1g/kg/d 3days/week No difference in growth results showed a  in total bilirubin levels among the treatment group compared to a slight increase in the control group. The difference in slopes was significant (p=0.0394). Conclusion: if total bili levels are elevated, lipid- reduction therapy should be utilized

Omega-3 Fatty Acids Use of omega-3 fatty acids as an alternative to standard lipid emulsions may  risk for PNALD Theory: omega-3 fatty acids have less pro-inflammatory effects and potential anti-inflammatory properties Omegaven® is the only current lipid emulsion made from 100% fish oil Diamond et al. Changing the Paradigm: Omegaven for the Treatment of Liver Failure in Pediatric Short Bowel Syndrome. Another strategy that has been studied to reduce PNALD is the use of linolenic-omega-3 monounsaturated fatty acids (MUFA) as an alternative to standard parenteral lipid emulsions. IV lipid emulsions available in the U.S. are Intralipid (distributed by Fresenius Kabi/Baxter) and Liposyn III (distributed by Hospira), both made from 100% soybean oil. Soybean oil is a rich source of omega-6 polyunsaturated fatty acids (PUFA). Some researchers believe omega-6 PUFAs from plant sources, such as soybean oil and safflower oil may influence the development of liver damage due to its pro-inflammatory effects. One the other hand, omega-3 MUFAs found in fish oil have less pro-inflammatory effects and potential anti-inflammatory and antioxidant effects, which may reduce risk of liver damage. Omegaven (distributed by Fresenius Kabi, Germany) is the only current lipid emulsion made from 100% fish oil. (26)

Central Line Infections
10-35% mortality associated with line infections More common in children  risk for sepsis Can cause loss of central venous access for PNrisk for malnutrition CVAD infections, also referred to as line infections, are another common and serious complication of PN. Mortality associated with line infections is 10-35% (14). Line infections are more common in children than adults because children have a weaker immune system and are more susceptible to infection. There are 7-9 infections per 1,000 catheter days in children versus <1 infection per 1,000 catheter days in adults (14). When a line infection occurs, there is a high risk for bloodstream infection (BSI) and loss of CVA. A BSI is a life-threatening condition and the patient must be hospitalized immediately. Line infections can cause loss of CVA, which is a dangerous situation because vascular sites are limited in children. Without CVA, PN is no longer possible, which places the patient at risk for dehydration and malnutrition. For these two reasons, it is critical to take preventive actions to avoid line infections and react quickly with a treatment plan when a line infection occurs. The RD has little involvement with the actual management of CVADs and treatment of BSI; however, line infections inhibit PN and if the child is dependent on PN, dehydration and nutritional status will falter. in children ich.edu/pediatric/clinical/patient_content/a-m/broviac_placement.shtml

Central Line Infections
Ethanol lock therapy Dramatically reduces rate of a blood stream infections Can be initiated in patients when weight is >5kg and TPN cycling is achieved (at 22 hours) Most effect when given daily for at least 2 hours NOT compatible with heparin NOT compatible with polyurethane catheters ELT is the most current treatment used for line infections, and it is also used as a preventative method for future line infections. One advantage of ELT is the potential to reduce the use of broad antibiotics. Also, the effects of ELT are not dependent on sensitivity to antibacterial agents, which may be beneficial for infections with multi-resistant organisms. ELT also lyses biofilm that builds on and around the catheter. There are also disadvantages of ELT including risk for intoxication, catheter weakness and breakage, and line occlusion. It is important to note the type of catheter being used (i.e. polyurethane, silicone etc.). ELT cannot be used with polyurethane catheters because it will weaken the line. ELT can be used with silicon catheters even though the risk for leakages and tears does increase. Also, TPN must be cycled to at least 22 hours to allow 2 hours for ELT to effectively kill bacteria. The Children’s Intestinal Rehabilitation Program at The University of Michigan used ELT in an outpatient setting to test the effectiveness of ELT. Teitlebaum et al. used ELT in 15 children weighing >5kg with a history of CVAD infections and limited vascular access sites for almost 2 years (July April 2008). BSI rate before ELT averaged 8 infections per 1,000 catheter days. This rate drastically decreased after ELT to a mean of 1.3 infections per 1,000 catheter days. ETL had a significant impact on the reduction of BSIs. Since 2008, this institution continues to place all SBS patients on ELT when weight is >5kg and TPN cycling is achieved.

Enteral Nutrition Introduce EN as soon as possible
EN provides several beneficial effects on the GI tract Fuel for enterocytes Stimulates hyperplasia Promotes peristalsis- decreases bacterial overgrowth Stimulates flow of GI secretions EN has several beneficial effects on the GI tract leading to better outcomes for SBS patients. EN provides fuel for enterocytes and stimulates hyperplasia; promotes peristalsis, which decreases bacterial overgrowth; and stimulates flow of GI secretions. Initiation of enteral feeding differs for every patient, and full EN dependence may take a few days to years. Determining factors for initiation of enteral feeding includes the length, anatomical section and function of the remaining bowel. Malabsorption is likely to occur when EN feeds are started, which may increase energy needs up to 150% of the DRI. It’s important to remember that every patient has different needs, and the feeding regimen should be structured to meet those individualized needs.

Initiating EN Initiate trophic feeds of one of the following:
Mother expressed breast milk (MEBM) Donor expressed breast milk (DEBM) Protein Hydrosylate formulas Semi-elemental Elemental If the patient is medically appropriate for enteral feeds, trophic feeds should be initiated. MEBM is always the first choice, if available because it can: -promote intestinal growth -increase immune function and -protect against infection. When MEBM is not available, the second best choice is DEBM. In the instance that EBM cannot be provided, protein hydrosylate formulas (semi-elemental and elemental) are the next appropriate choice for EN feedings.

Formulas Semi-Elemental Elemental Infant Pediatric Alimentum
Peptamen Jr. Pregestimil Peptamen 1.5 Nutramigen Pediasure Peptide Elemental Infant Pediatric Neocate Infant Neocate Jr. Elecare Infant Elecare Jr. Nutramigen Infant Vivonex Semi-elemental and elemental formulas are designed for easier digestion and better tolerated among SBS patients. They are made for both infant and pediatric patients (>1 year old). Standard formulas contain complete intact proteins, whereas semi-elemental and elemental do not. Semi-elemental formulas contain hydrolyzed proteins, which are partially broken down into di- and tripeptides for easier digestion. Elemental formulas contain completely hydrolyzed proteins, meaning the proteins are broken down into single amino acids for even easier digestion. Infants should be started on an elemental formula like Elecare, Neocate or Nutramigen AA when breast milk is not an option. Children and adolescents can start on an elemental formula like Elecare or Neocate Junior or a semi-elemental formula such as Peptamen Jr. or Pediasure Peptide. Formula tolerance should be monitor and changed accordingly.

Continuous vs. Bolus Continuous Bolus
Preferred method in infants and children with SBS Causes less stress and demand on intestinal function Provides constant saturation of intestinal wall may promote adaptation Bolus More physiological More often used in older children Less tolerated in infants Depends on the individual’s tolerance level Enteral feedings can be administered on either a bolus or continuous feeding schedule, depending on tolerance. A continuous feeding is advantageous for the patient as it causes less stress and demand on the intestine’s function, and is the preferred feeding method for infants and toddlers with short bowel syndrome (SBS) (Parker 1981; Christie 1975; Vanderhoof 2008). This method promotes constant saturation of the transporters, which may help promote the adaptation process (Vanderhoof 2008; Parker 1981; Christie 1975). Bolus feeds seem to be used in older children more often and are poorly tolerated in infants (Ching 2007

Modulars Pectin Benefiber Beneprotein Duocal Polycose MCT oil
Human Milk Fortifier There are several modulars that can be added to feeds such as pectin, Benefiber, Resource Beneprotein, Duocal powder, Polycose powder and MCT oil. For patients with loose stools, pectin and Benefiber can be added to feeds to help solidify stools. Pectin is a soluble fiber that binds contents in the intestines and adds bulk to stools. Recommendations for pectin range from 1% to 3% (1ml/100mL to 3mL/100mL). Benefiber provides 3g of fiber per serving (1 packet or 2tsp) and can be used in tube feeds. Resource Beneprotein can be added for patients with extra protein needs that cannot be met through the feeding regimen. Resource Beneprotein contains 25 kcal and 6g of protein per scoop/packet (7g), which is equivalent to 1½ tbsp. Duocal powder is a source of extra calories made from carbohydrates and fat (35% MCT, 65% LCT). It dissolves in water, liquids and most moist foods, such as pudding, scrambled eggs and yogurt. It can also be added to tube feeds. One scoop of Duocal powder provides 25 calories. Polycose powder provides additional calories with a low osmolality and minimal sweetness. It provides 8 kcal per teaspoon and 23 kcal per tablespoon. MCT oil is made from modified coconut and/or palm oil and provides 7.7 kcal/mL. MCT oil is beneficial for patients with fat malabsorption because it is absorbed directly into the portal system without the use of bile salts and lipase for digestion. (5)

Monitoring and Evaluation
 Trend anthropometrics Monitor labs closely vitamin/mineral deficiencies for decreased liver function Monitor I/Os Adjust feeding regimen accordingly to meet 100% needs    MNT for SBS is long-term and requires constant monitoring and evaluation with ongoing interventions to achieve the patient’s nutritional goals. (5, 20)

Case Study

Presentation of Patient
CM 13 months old Full term, no significant history Twin brother Diagnosed with SBS at 15 weeks

CM’s Course of Care at SCHC
Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos Note that CM is also seen at SCHC’s GI outpatient clinic

CM’s Hospital Course Admitted with abdominal distention
Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos Admitted with abdominal distention Diagnosed with midgut volvulus 160 cm bowel resection 16 cm remaining with ICV & colon Broviac & G-tube placement TPN & trophic feeds initiated Abdominal distention Midgut volvulus 160 cm bowel resection Broviac & G-tube placement TPN main source of nutrition Trophic feeds of Elecare 20 initiated 20 weeks GA, 125 30 weeks GA, 200 Term (>37 weeks), 275 1 year, 380 5 years, 450 10 years, 500 20 years, 575

Initial Nutrition Assessment
CM’s Hospital Course PES: Altered GI function related to short bowel syndrome as evidenced by 16cm remaining bowel and dependence on TPN/G-tube feeds to meet nutritional needs. Recommended Interventions: Continue D13P3.2L1 TFV of 550mL/day, Lipids M/W/F Provide HAL over 16 per home feeding regimen (tapered) 9.3mL/hr 1st and 16th hour, 18.5mL/hr 2nd and 15th hour, 37/hr 3rd-14th hour Max GIR= 8.18 Continue current G-tube feeding regimen Daily weights, strict I/Os, monitor labs Goals/evaluation: Appropriate wt gain for age (11-12g/day) Tolerates feeds Chief Complaint: Broviac infection Medications: ELT, Gentamycin, Heparin Diet order: (G-tube) Elecare 24ml/hr with 3tsp Benefiber Nutrition Support: D13P3.2L mL 32.2 mL/hr X 18 Current Intake: (4/30) 495 mL HAL, 35mL IL, 596mL Elecare, 263mL NS with meds Anthropometrics: Weight: 9.8 kg (50th%ile) Length: 79 cm (95th%ile) Wt/Lgth: 10-25th%ile Head circumference: 50 cm (>95th%ile) Estimated Daily Needs: 960 kcal (98 kcal/kg)- RDA 16g pro (1.6g/kg)- RDA 980mL fluid (100mL/kg)- Holiday-Segar Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos Moderate nutritional risk Note: NO nutritionally significant labs (4/30) Na-136, K-42, Cl-104, CO2-24, BUN-15H, Cr- 0.14L, Glu-97, Ca-9.1, PO4, 5.7, Mg-1.6, Gram neg. rods, Gram  cocci in pairs, Pending labs- Staph areus, E. Coli, Enterococcus Per SBS rounds, lipids will be changed to 1g/kg 3X/week (M/W/F).

Initial Nutrition Assessment F/U Nutrition Assessment
CM’s Hospital Course Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos May 8, 2012 F/U Nutrition Assessment Age: 10 ¾ mos

Initial Nutrition Assessment F/U Nutrition Assessment
CM’s Hospital Course Monitoring/Evaluation: Meet 100% needs Wt gain 11-12g.day Bowel movements WNL  5 BM/day Tolerate TPN/G-tube feeds Diagnosis: Altered GI function related to SBS as evidenced by need for TPN/G-tube feeds Interventions: Continue current TPN regimen Continue current EN order, increase per home schedule T/C holding feeds for one hour and provide formula PO Continue daily weights, strict I/Os, monitor labs RD to follow Wt: (5/7) 9.65kg, wt decreased 150g (21g/d X 7 days) TPN order: D13P3.2L1, TFV increased to 550ml/day EN order: Elecare 20 with 3 tsp Benefiber: 20 28mL/hr 672mL (69.6mL/kg), 448 kcal (46.4 kcal/kg), 13.8g pro (1.4g/kg) Intake (5/7): 712mL Elecare 20, 235mL D13P3.2, 19.5mL IL 670 kcal (69 kcal/kg), 27.8g Pro, 966mL (100mL/kg) Output (5/7): 1076mL (UOP= mL/kg/hr), BM X2 Meds: Gentamycin, Ampicillin, ELT, Heparin Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos May 8, 2012 F/U Nutrition Assessment Age: 10 ¾ mos Note: No new labs CM is a 10-¾ month old male admitted with Broviac infection and SBS 2/2 mid-gut volvulus (16cm left). Pt is FT twin on continuous G-tube feeds of Elecare 20 and TPN X 16 home. Since last nutrition note, G-tube feeds increased to 28mL/hr X 24 hours per home schedule (increases 2mL/hr/wk). CM continues on antibiotics. Anticipated discharge 5/10 when antibiotics finished. Note: Mom OK to hold feeds over 1 hour and provide formula PO.

CM’s Hospital Course Estimated Daily Needs:
991 kcal (98 kcal/kg), 16.2g pro (1.6g/kg), 1012mL fluid (100mL/kg) PES: Altered GI function related to SBS as evidenced by 16cm remaining small bowel and dependence on TPN/G-tube feeds to meet nutritional needs. Recommended Interventions: Continue current TPN with lipids M/W/F Continue current EN regimen T/C increasing Elecare 20 kcal/oz to 30mL/hr if BM WNL Monitor daily weights, labs, I/Os and BM Please re-check length (inconsistency) Chief Complaint: Fever with Broviac Medications: ELT, Cefotaxime, Vancomycin Diet Order: Elecare 28mL/hr via G-tube, Baby food PO ad lib Nutrition Support: D13P mL x 19 31.6mL (8AM-5PM) based on 10kg; x 20 M/W/F Current Intake: (5/13) HAL, 672mL Elecare 20 ( I/O)= /663 Anthropometrics: Weight: kg (50-75th%ile Wt/age) (5/1) 9.8kg, (4/7) 9.65kg Length/Height: 70 cm (~5th%ile Ht/age) (4/26) 73.5, (5/1) 79cm inconsistency Wt/Ht: >95th%ile Head circumference: 49 cm (>95th%ile HC/age) Oct 10- Nov 21, 2011 Diagnosis of SBS Age: 3 ¾ mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos May 8, 2012 F/U Nutrition Assessment Age: 10 ¾ mos May 13, 2012 Readmitted w/Central Line Infection Age: 11 mos **I/Os- retaining? Want to highlight Ht Goals are the same

CM’s Hospital Course Oct 10- Nov 21, 2011 Diagnosis of SBS
Age: 3 ¾ mos Dec 5, 2011 – June 21, 2012 GI Outpatient Visits Age: 5 ¾ mos- 12 mos May 1,2012 Initial Nutrition Assessment Age: 10 ½ mos May 8, 2012 F/U Nutrition Assessment Age: 10 ¾ mos May 13, 2012 Readmitted w/Central Line Infection Age: 11 mos SO as you can see, CM received many of the interventions I discussed earlier: TPN cycled Lipids reduced to 3 days/wk ELT G-tube feeds Benefiber to help loose stools

GI Outpatient Visits Mom has gradually increased G-tube feeds 2mL/hr every week as tolerated (start rate) 2mL/hr (current rate) 34mL/hr Gradually weaned from TPN Feeds held 2-3 times per day to allow PO Baby foods slowly introduced Benefiber consistently in feeds secondary to loose stools , CM has consistently gained weight with no setbacks, which is shown in Table 6 (p.62). At times, he was gaining excessive weight, which was addressed by cutting back on his TPN regimen. CM has been taking Elecare Infant formula since he was in the hospital after his short bowel resection staring at a continuous rate of only 2mL/hr via G-tube. CM’s mother has gradually increased his G-tube feeding regimen by 2mL/hr every week as tolerated to his current rate of 34mL/hr. CM has done an excellent job with the increased EN feeds with no tolerance issues per mom. G-tubes feeds are held 2-3 times per day to allow PO feeds. Mom has also slowly introduced baby foods. PO feeds are important for normal oral development, and CM tolerates them well. Benefiber has been consistently added to his feeds secondary to loose stool output, which has been a successful solution. As CM’s EN intake has increased, his TPN regimen has been gradually decreased at each visit. The progression of his EN versus PN intake can be seen in Table 7 (p.62).

Update on CM Current EN: Current PN: Plan:
Elecare Jr mL/hr with Benefiber Current PN: 30g Dextrose per day (No amino acids or lipids) Plan: To gradually concentrate Elecare Jr. by 2 kcal per week as tolerated to goal concentration of 30 kcal/oz To continue to wean TPN

CM’s Progression from PN to EN
Date Age (mo) EN Regimen % Kcal from EN PN Regimen % Kcal from PN TOTAL Oct 2011 4 ¼ None D17 P3 L2.99 100 Nov 2011 5 2mL/hr 6 D16 P3 L2.5 94 Jan2012 7 ¼ 10mL/hr 27 *Lipids 3d/wk 73 April 2012 9 24ml/hr 50 D13 P3.2 L1 June 2012 12 34mL/hr 61 D13 P3.2 39 12 ¼ *Elecare Jr. 22 50g D, 14g AA Present 13 ¾ *Elcare Jr. 27 90 30g D 10 Lipids reduced Need to condense and highlights importance Lipids D/C’d AAs D/C’d

Critical Comments Anthropometrics- inconsistent height
Estimated kcal needs Medications: ELT & heparin Laboratory values: suggestive of anemia 5 days prior to admission on April 26th where his length= 73.5cm (50th percentile for length-for-age) and (50-75th percentile for wt/ht). On May 1st: 79cm= 95th lgth/age, 10-25th wt/ht. Readmit- 70cm - I did catch the inconsistency at this time and recommended to recheck CM’s length GI outpatient appointment on May 24th, his length was recorded as 77cm, which is an increase of 0.875cm/week times four week. Estimated needs: used RDA (98kcal/kg) DRI x 1-1.5= kcal/kg NCM= EER x 1.2 – takes ht into consideration, and because we don’t know cm’s ht, this equation wouldn’t be appropriate in this situation Overall, these equations are just estimates and starting points, and the key is to closely monitor weight changes and then make adjustment accordingly Iron- should be held IV during infection, possibly orally

Summary

Key Points  Goal #1- Meet 100% needs for proper growth and development Goal #2- Start EN as soon as medically appropriate Goal #3- Reduce risk of PNALD and line infections Wean TPN as EN increases Reduce lipids to 1g/kg/day 3X/week when cholestasis is present  

Austin’s Cupcake Fund Raised 61,000 dollars for research Dr. David Hackman at Children’s Hospital of Pittsburgh

Questions?

References Cole CR. Pathophysiology and Medical Management of Intestinal Failure in Childhood. Cincinnati Children’s Hospital Medical Center 2012. Beattie LM, Barclay AR, Wilson DC. Short bowel syndrome and intestinal failure in infants and children. Paediatrics and Child Health 2010; 20:10. Teitlbaun H. “Pediatric Intestinal Failure: Approaches to Optimize Care.” PASPEN (Philadelphia Area Society for Parental and Enteral Nutrition) Spring Conference 2012. Gastroschisis [CHOP]. Philadelphia: The Children’s Hospital of Philadelphia; c [updated 2012 Feb; cited June 10]. Available from diagnoses/gastroschisis.html. Intestinal Malrotation and Volvulus [Cincinnati Children’s]. Cincinnati: Cincinnati Children’s Hospital Medical Center; c (updated 2012 Aug; cited 2012 June]. Available from: Bunting KD, Mills J, Phillips S, Ramsey E, Rich S, Trout S. Pediatric Nutrition Reference Guide. 9th ed. Houston: Texas Children’s Hospital; 2010. Pediatric Nutrition Care Manual. Short Bowel Syndrome. Available from: McMellen M, Wakeman D, Longshore S, et al. “Growth Factors: Possible Roles for clinical Management of the Short Bowel Syndrome.” Semin Pediatr Surg 2010; 19 (1): Tee C, Wallis K, Gabe S, et al. Emerging treatment options for short bowel syndrome: potential role of teduglutide. Clinical and Experimental Gastroenterology 2011:

Omegaven Diamond et al.’s retrospective cohort study
12 pediatric SBS patients with advanced PNALD All being considered for liver transplant Treatment: 1g/kg Intralipid, 1g/kg Omegaven (total lipids=2g/kg) Intralipid decreased or d/c’d if PNALD worsening Diamond et al.’s study also showed positive results with the use of Omegaven. This retrospect cohort included 12 pediatric SBS patients with advanced PNALD (median age 7.5 months). Criteria for participation included a conjugated bilirubin of >100 mol/L (5.9 mg/dL) or a serum conjugated bilirubin >50 mol/L (2.9mg/dL) with other evidence of liver damage. Participants received 1g/kg of Intralipid and 1g/kg Omegaven, which is a total of 2g of lipids/kg. However, if PNALD was not improving or worsening, Intralipid dose was decreased or discontinued. Patients were on PN for a median duration of 28.4 weeks prior to initiation of Omegaven, and all were being considered for liver transplant. Median conjugated bilirubin was initially 137mol/L. Results showed 9 out of the 12 patients completely resolved hyperbilirubinemia within a median of 24 weeks with conjugated bilirubin levels of 0 mol/L. Out of those 9 patients, 4 achieved resolution of hyperbilirubinemia with the combination of Intralipids and Omegaven, while 5 achieved resolution after discontinuation of Intralipids. Also, all 12 patients were no longer being considered for liver transplantation at the conclusion of this study. The interesting and unique factor in this study was the use of both Intralipids and Omegaven, opposed to the sole use of omega-6 or omega-3 fatty acids. Diamond et al. explain that the ratio of omega-3 to omega-6 fatty acids is important in the regulation of inflammation, and the optimal range is 1:1-4:1. The goal ratio for the study was 1:1-1:2. (27)

Results 9 out of 12 completely resolved hyperbilirubinemia within a median of 24 weeks Out of those 9 patients: 4 achieved resolution with combination of Intralipid and Omegaven 5 achieved resolution after Intralipids discontinued All 12 patients were no longer considered for liver transplant Results showed 9 out of the 12 patients completely resolved hyperbilirubinemia within a median of 24 weeks with conjugated bilirubin levels of 0 mol/L. Out of those 9 patients, 4 achieved resolution of hyperbilirubinemia with the combination of Intralipids and Omegaven, while 5 achieved resolution after discontinuation of Intralipids. Also, all 12 patients were no longer being considered for liver transplantation at the conclusion of this study. The interesting and unique factor in this study was the use of both Intralipids and Omegaven, opposed to the sole use of omega-6 or omega-3 fatty acids. Diamond et al. explain that the ratio of omega-3 to omega-6 fatty acids is important in the regulation of inflammation, and the optimal range is 1:1-4:1. The goal ratio for the study was 1:1-1:2. (27) Hard to determine if lipid reduction had more to do with improved liver function than the composition

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