Pediatric Case StudyBy: Sarah Iske

Elliot Hospital

• Elliot Health System (EHS) is the largest provider of comprehensive healthcare services in Southern New Hampshire.

• Established in 1890, Elliot Hospital offers Southern New Hampshire communities caring, compassionate and professional patient service.

• 296-bed acute care facility located in Manchester.

Upon Admission…

• Initial Screening Questions:• Have you lost weight in the past 3-6 months? If yes,

how much?• Has there been no nutritional intake for greater than

five days?• Difficulty swallowing?• Non-healing wound or pressure sore?• Admitted on nutrition support (TF/TPN)?

• Malnutrition Universal Screening Tool (MUST) Score:• BMI >20 = score 0, 18.5-20 = score 1, <18.5= score 2

Elliot Registered Dietitians

• Prioritize all new patients

• Perform an initial assessment• Estimate nutrition requirements• Determine level of care• Create nutrition diagnosis statements• Determine nutrition status• Determine goal of therapy• Create intervention• Monitor and evaluate

• Re-evaluate every 1-7 days

• Provide nutrition educations

• Coordinate care with medical staff and attend rounds/pre-rounds

Initial Screening

• Initial Screening Questions:• Have you lost weight in the past 3-6 months?

If yes, how much? NO• Has there been no nutritional intake for

greater than five days? NO• Difficulty swallowing? aspiration

precaution• Non-healing wound or pressure sore? NO• Admitted on nutrition support (TF/TPN)? YES

Patient History

• Caucasian, Male

• Born on 3/29/15

• Premature 33 5/7 weeks

• L&D Complication: Full resuscitation at

birth including intubation and chest

compressions

• Born with Fetal Hydrops (treated by

paracentesis)

• Previously admitted at MGH

• No known allergies

• Nutrition Status: TPN before transitioning

to NG Tube using Enfaport formula

Social History

• Lives with parents and 4 year old sister

• Apartment is not baby safe

• Planning to move to a new apartment

• Family doesn’t have a car

• Medical team concerned about Mom-infant bond

Anthropometrics from Medical Chart

• Birth Weight: 3.2 kg

• Length since admission: 52 – 59 cm

• Weight since admission: 4.87 – 6.38 kgs

• BMI: 18.33 kg/m2

Elliot Problem List

1. Chylothorax/Chylous Ascites• d/t Noonan’s Syndrome?

2. Hypogammaglobulinemia

3. Hypercoagulability

4. Chronic Lung Disease (CLD)

5. Iatrogenic Drug Withdrawl

What is Chyle?

• Chyle: alkaline solution produced during digestion of food. Contains chylomicrons, triglycerides, and fat soluble vitamins.

• 70% of ingested long-chain triglycerides are absorbed via the lymphatic system and the lymph flow increases after each meal.

Chyle Leak

• Rare condition

• Abnormally high amount of drainage from the lymphatic system into a body cavity or externally through a chest tube.

• Three types:1. Chylous Ascities (abdominal

cavity)

2. Chylothorax (pleural cavity)

3. Chylopericardium (cardiac cavity)

Causes of Chyle Leaks

• Select malignancies (60%)• Lymphoma• Mediastinal lymph node

metastasis • Carcinomas (gastric,

ovarian, colon)

• Trauma (25%)

• Congenital abnormalities

• Cirrhosis

• Diagnosis confirmed by lab assessment:

• Lipid content of the leak is greater than the lipid content of plasma.

• Protein content of the leak is 50%+ greater than the protein content of plasma.

Complications of Chyle Leaks

• Symptoms: dyspnea, tachypnea, cough, chest pain, abdominal distention.

• Malnutrition • Depletion of nutrients (fat, protein, fat soluble

vitamins, albumin, lymphocytes)• Increased risk for immunosuppression with potential

risk for pneumonia and sepsis.• Hypovolemia (2.5 L losses/day)• Metabolic acidosis• Electrolyte imbalance (hypernatremia, hypocalcemia)• Hemodynamic instability

Treatment of Chyle Leaks

• Surgical Treatment• Swift closure

• Medical Treatment:• Goal = spontaneous closure• Paracentesis• Reducing the amount of chyle (diuresis with a fat-

modified enteral diet)• Treating the underlying cause• Nutrition support• Goal: decrease production of chyle, replace fluid and

electrolytes, and maintain or replete nutrition status

Nutritional Management

Two Options:

1. Enteral Nutrition/Oral Nutrition• Very low fat or fat free elemental formula/diet• 2-5% of energy from essential fatty acids to prevent deficiency

• <10g of LCT a day

• Multivitamin/Mineral supplement• High Protein (fat free protein supplement)• MCT oil supplementation

2. NPO with Parenteral Nutrition• Attempt EN or Oral feedings first• Only if unable to use gut, intolerance to oral or EN feeds, or

failure of EN or oral diet to close chyle leak.

Low Fat Specialized Formulas

Formulas Uses Kcals/

100 ml%

MCT/LCTProtein/100 ml **Cost

Enfaport (liquid

or powder)

For infants 100 84:16 3.5 $

Monogen

(powder)

For infants or child

supplementation

74.2 90:10 2 $$$

Portagen

(powder)

Children and adults. Not for

infants99 87:13 3.6 $$

**Prices depend on contract – scale in chart show relative expense from the most affordable ($) to the most expensive ($$$)

Changing Enfaport Caloric Density

Cal/fl oz Water Enfaport To Make

20 4 fl oz (118 ml) 8 fl oz 12 fl oz (355 ml)

22 2.9 fl oz (86 ml) 8 fl oz 10.9 fl oz (322 ml)

24 2 fl oz (59 ml) 8 fl oz 10 fl oz (296 ml)

26 1.2 fl oz (35 ml) 8 fl oz 9.2 fl oz (272 ml)

27 0.9 fl oz (27 ml) 8 fl oz 8.9 fl oz (263 ml)

28 0.6 fl oz (18 ml) 8 fl oz 8.6 fl oz (254 ml)

Noonan’s Syndrome(Also called Turner Syndrome)

• An autosomal dominant congenital disorder that affects both males and females

• Mutations in the PTPN11, SOS1, RAF1, KRAS, NRAS and BRAF genes that provide instructions for making proteins.

• Characterized by: mildly unusual facial characteristics, short stature, heart defects, blood clotting problems, skeletal malformations.

• Affects 1 in 1,000 to 2,500 people

• Testing: Molecular genetic tests (or gene tests) that study single genes or short lengths of DNA to identify variations or mutations that lead to a genetic disorder.

• Patients results were inconclusive.

• Nutrition Management: No specific nutrition management.

• Treatment: Based on individual signs and symptoms. Any cardiac or respiratory treatment is similar to treatment in general population.

Hypercoagulability

• Increased risk for blood clots developing in the arteries and veins.

• In most cases, treatment is only needed when a blood clot develops in a vein or artery.

• Anticoagulants decrease the blood’s ability to clot and prevent the formation of additional clots.• Warfarin (Coumadin)• Heparin (IV or subcutaneous)• Fondaparinux (Arixtra)

Secondary to Chylothorax/Fetal Hydrops: Hypogammaglobulinemia

• A deficiency of gamma globulins and especially antibodies in the blood causing immunosuppression.• Other causes: medications, nephrotic

syndrome, intestinal lymphangiectasia, B-cell lineage malignancies, severe burns, and dystrophic myotonia.

• Normal ranges for IgG are 176 - 601 mg/dL for 2-3 month old baby

• Treated with replacement therapy of immunoglobulin G (IgG) through IVIG or SCIG

Chronic Lung Disease (CLD)

• A general term for long-term respiratory problems in premature babies. The lungs of newborn babies are fragile and are easily damaged. Injury causes inflammation and scarring leading to breathing difficulties.

• Causes:1. Prematurity2. Low amounts of surfactant3. Oxygen use4. Mechanical ventilation5. Maternal womb infection6. A family history of asthma7. Development of an infection during or shortly after birth

• Symptoms:• Respiratory distress (rapid breathing, flaring of the nostrils, grunting, chest retractions)• Continued need for mechanical ventilation or oxygen after a premature baby reaches 36

weeks gestation

• Treatment: • Oxygen • Mechanical ventilation• Surfactant replacement• Medications: Bronchodilators, Steroids, Diuretics, Antibiotics• Nutrition for proper growth

Chylothorax/Chylous Ascites

Hypogammaglobulenimia

Hypercoagulability

Noonan’s Syndrome?

CLD

Fetal Hydrops

Patient Admission 6/5

• Admitted to NICU

• Weight: 4.87 kgs

• Current Problems: CLD, Chylothorax/chylous ascities, hypercoagulability, hypogammaglobulinemia, possible Noonan’s Syndrome.

• On NCPAP for CLD

• Chylothorax/Chylous Ascites – resolving on current diet• Continue Enfaport 26 cal/oz TF regimen 135-140 ml/kg/day• No PO allowed d/t respiratory status

• IVC/RA junction clot (hypercoagulability)

• NAS scoring system for drug withdrawl

• Practicing comfort measures: supportive positioning, pacifier, and sucrose per unit policy.

Medication Medical Use Drug-Nutrient Interaction

LovenoxAnticoagulant

(IVC/RA junction clot)

Interacts with vitamin K. Do not exceed UL for Vitamin E,

A. Avoid alcohol.

PhenobarbitolBarbituate

(Iatrogenic Drug Withdrawl)Avoid alcohol and Excessive

Vitamin C intake

BactrimAntibiotic

(Hypogammaglobulinemia)Avoid alcohol

Prednisone Steroid(CLD)

Avoid alcohol

Morphine Narcotic

(Pain Reliever)Avoid alcohol

TylenolAnalgesic and Antipyretic(With Phenobarb wean)

Avoid alcohol

ZantacH 2 Blocker

(Reflux)Avoid excessive alcohol, caffeine and spicy foods

Mylicon Anti-flatulent None

MycostatinAntifungal antibiotic

(diaper rash)Brewer’s Yeast

Hydoduril LasixDuieretic

(Fluid accumulation) Avoid alcohol

Vit DSupplement(Low Vit D)

None

Initial NICU Nutrition Assessment 6/9

• Weight: 5.225 kgs

• Diet: NPO• Nutrition Support: NG tube of Enfaport 24 cal/oz @ 135 ml/kg over 1 hr via pump.

Decreased from 26 cals/oz d/t large weight gain. • Vit D labs requested. Abnormal at 18 ng/ml – added 400 IU Vit D

• Estimated Nutrition Needs:• Energy: 110 – 120 kcal/kg for corrected to term/post term based on estimated enteral needs.• Protein: 3-4 gm for corrected to term/recovery needs based on estimated enteral needs. • Yield of current TF: 135 mls/kg provides 564 kcals/day, 108 kcals/kg and 3.5 gm

protein/kg/day.

• Nutrition Diagnosis:• Altered GI function related to chylothorax evidenced by need for specialized low chain fatty

acid formula (Enfaport)• Delayed/impaired oral feeding related to hx of prematurity and extensive

medical/respiratory support as evidenced by NPO status/full TF dependence at this time.

• No Malnutrition Identified

• Goal of Therapy:• Promote ~0.025-0.035 kgs/day weight gain• Promote proportionate growth velocity

• Check TF daily and Reassess in 7 days

• Labs: Glucose 96, BUN 16, Creat 0.4, Na 134, K 3.3

Enfaport Formula Calculation

Nutrition Support: NG tube of Enfaport 24 cal/oz @ 135 ml/kg over 1 hr via pump. • Energy Requirements: 110 – 120 kcal/kg and 3-4 gm protein• Weight: 5.225 kg

• 24 cals/oz x 30 ml/oz = 720 mls/ 5.225 kg = 137.7 ml/kg• 135 ml/kg x 5.225 kg = ~705 ml• 705 ml/ 30 ml/oz = 23.5 ounces x 24 cals/oz = 564 kcals• 564 kcals / 5.2 kg = 108 kcals/kg

• 705 mls x 0.026 gm protein/ml = 18.33 gms protein• 18.33 grams protein/ 5.2 kg = 3.5 gm protein/kg

• Yield of current TF: 135 mls/kg provides 564 kcals/day, 108 kcals/kg and 3.5 gm protein/kg/day.

Updates 6/10-6/16

• Labs: Glucose 99, BUN 15, Creat 0.2, Na 134, K 4.3

• TF remains at 135-140 ml/kg/day with Enfaport 24 cal/oz over 1 hr via pump and no PO intake

• NAS score daily• Plan for gradual wean off phenobarbitol

• Weights checked daily – 0.395 kg weight increase

• Stooling well for age

• Prednisolone for respiratory issues d/c’ed

Nurtition Re-Eval 6/16• Weight: 5.62 kgs

• Diet: NPO• Nutrition Support: NG tube of Enfaport 22 cal/oz @ 130 ml/kg over 1 hr via

pump. Decreased from 24 cals/oz d/t large weight gain. • Diuretic also adjust for weight gain and fluid accumulation may be source of

weight gain.

• Estimated Nutrition Needs:• Energy: 100 – 110 kcal/kg for corrected to term/post term based on estimated enteral

needs.• Protein: 2.2-3 gm for corrected to term/recovery needs based on estimated enteral

needs. • Yield of current TF: 130 mls/kg provides 535 kcals/day, 95 kcals/kg and 3.4 gm

protein/kg/day.

• Nutrition Diagnosis:• Altered GI function related to chylothorax evidenced by need for specialized low chain

fatty acid formula (Enfaport)• Delayed/impaired oral feeding related to hx of prematurity and extensive

medical/respiratory support as evidenced by NPO status/full TF dependence at this time.

• Check TF daily and Reassessment in 7 days

Updates 6/17 – 6/24

• Transferred to the PICU from NICU

• On the TF with Enfaport but cleared by MD to trial PO intake.• Noticed increased WOB, nasal flaring, head bobbing• Plan to have SLP evaluate

• Labs: Glucose 85, BUN 10, Creat 0.14, Na 133, K 4.3

• Multiple loose stools

• Not weaning from respiratory support as expected• Hold PO when attempting to wean

SLP Evaluation

• Noted immature suck of 4-5 sucks and then a breathing break.

• Tires quickly after 15 minutes and sucks decrease to 3 before breathing break.

• Risk of aspiration is very high!

• Plan: Allow PO feeds with slow flow nipple d/t no evidence of aspiration

• Recommendations: SLP Therapy and Modified Barrium Swallow• Modified Barium Swallow findings: at very high risk for

aspiration given issues with feedings, respiratory needs, and delay in developmental milestones.

Nutriton Re-Eval 6/24• Weight: 5.9 kgs

• Diet: NPO• Nutrition Support: NG tube of Enfaport 22 cal/oz @ 130 ml/kg over 1 hr via pump. • If weight gain suboptimal, can increase TF to meet low end needs of 100 kcals/kg. • Promote ~ 20-25 g/day weight gain

• Estimated Nutrition Needs:• Energy: 100 – 110 kcal/kg for corrected to term/post term based on estimated enteral needs.• Protein: 2.2-3 gm for corrected to term/recovery needs based on estimated enteral needs. • Yield of current TF: 130 mls/kg provides 562 kcals/day, 95 kcals/kg and 3.4 gm protein/kg/day.

• Nutrition Diagnosis:• Altered GI function related to chylothorax evidenced by need for specialized low chain fatty acid

formula (Enfaport)• Delayed/impaired oral feeding related to hx of prematurity and extensive medical/respiratory

support as evidenced by NPO status/full TF dependence and SLP evaluation.

• Check TF daily and Reassessment in 7 days

• GI consultation planned today

GI Consultation

• Aspiration will continue to worsen lung function and prevent alveolar growth• Possible PEG placement• Possible Nissen Fundoplication to protect

airway

• Plan to continue NG tube feeds and we reevaluate when closer to d/c and respiratory status improves

Nissen Fundoplication

A laparoscopic procedure performed for patients with GERD or reflux

Updates 6/25 – 6/29

• Low weight gain – increased TF from 95 ml q3 to 100 ml q3• Yield of Enteral Nutrition: 800 mls of Enfaport 22 kcals/ounce

will yield 587 calories/day 98 kcals/kg and 20 grams protein/day.

• 98% energy needs and 3.3 gm Pro/kg/day

• Multiple, loose stools

• Had episode of 2 emesis after feeding

• Patient discovered to have inguinal hernia – reduced by MDs.

• Labs: Glucose 93, BUN 11, Creat 0.31, Na 136, K 4.4

Nutrition Re-Eval 6/30• Weight: 6 kgs

• Diet: NPO• Nutrition Support: NG tube of Enfaport 22 cal/oz @ 100 ml q3

Estimated Nutrition Needs:• Energy: 100 – 110 kcal/kg for corrected to term/post term based on

estimated enteral needs.• Protein: 2.2-3 gm for corrected to term/recovery needs based on estimated

enteral needs. • Yield of Enteral Nutrition: 800 mls of Enfaport 22 kcals/ounce will

yield 587 calories/day 98 kcals/kg and 20 grams protein/day or 3.5 gm protein/kg.

• Nutrition Diagnosis:• Altered GI function related to chylothorax evidenced by need for

specialized low chain fatty acid formula (Enfaport)• Delayed/impaired oral feeding related to hx of prematurity and extensive

medical/respiratory support as evidenced by NPO status/full TF dependence and SLP evaluation.

• Check TF daily and Reassessment in 7 days

Updates 6/31 – 7/6

• Parents are more involved in patient care – learning how to use feeding pump.

• Plan for hernia repair with PEG placement when respiratory status improves.• Of all options (NG, GT, GT/fundo, G-J) chose G-J because feeds will be

postpyloric and will protect airway.• Planned for 7/7

• Abnormal Lab 7/1: IgG 140 mg/dL – given IVIG (last dose on 7/6)• Average ranges between 176-601 for 2-3 month old

• Weaned off NC

• Vomit x 2 (likely related to NGT position)

• Poor weight gain: TF increased from 100 ml q3 to 105 ml q3 of Enfaport 22 kcals/ounce• Provides 840 mls , 616 calories/day and 22 grams protein

Nutrition Re-Eval 7/6• Weight: 6.195 kgs

• Diet: NPO

• Nutrition Support: NG tube of Enfaport 22 cal/oz @ 105 ml q3

• Estimated Nutrition Needs:

• Energy: 100 – 110 kcal/kg for corrected to term/post term based on estimated enteral needs.

• Protein: 2.2-3 gm for corrected to term/recovery needs based on estimated enteral needs.

• Yield of Enteral Nutrition: 840 mls of Enfaport 22 kcals/ounce will yield 616 calories/day 99 kcals/kg and 22 grams protein/day providing 99% of energy needs and 3.5 gms protein/kg/day

• Nutrition Diagnosis:

• Altered GI function related to chylothorax evidenced by need for specialized low chain fatty acid formula (Enfaport)

• Delayed/impaired oral feeding related to hx of prematurity and extensive medical/respiratory support as evidenced by NPO status/full TF dependence and SLP evaluation.

• GJ tube placement and hernia repair planned for 7/8• With J tube in place, switch to continuous feeds and consider goal rate of 35 ml/hr for 24 hrs to provide 840 ml,

616 kcals (99 kcals/kg current weight), 22 grams protein.

• Once established, may wish to shorten TF rate to 20 hours daily (44 ml/hr) for more motility at home

• Monitor and Evaluate: new feeding tube, TF restart, events/plans for surgery

• Check TF daily and Reassessment in 2-4 days

Updates 7/7 – 7/8

• Patient intubated and sedated in OR: **laparoscopic gastrojejunostomy tube placement, hernia repair, small amount chylous ascities noted in RUQ• Feeds started at 9 ml/hr with plans for gradual

increase by 9 ml/hr until goal of 35 ml/hr for continuous feeds.

• Parents involved in care

Nutrition Re-Eval 7/9

• Weight: 6.38 kgs

• Diet: NPO

• Nutrition Support: GJ tube of Enfaport 22 cal/oz @ 35 ml/hr

• Estimated Nutrition Needs:

• Energy: 100 – 110 kcal/kg for corrected to term/post term based on estimated enteral needs.

• Protein: 2.2-3 gm for corrected to term/recovery needs based on estimated enteral needs.

• Yield of Enteral Nutrition: 840 mls of Enfaport 22 kcals/ounce will yield 616 calories/day 97 kcals/kg and 22 grams protein/day providing 97% of energy needs and 3.4 gms protein/kg/day

• Nutrition Diagnosis:

• Altered GI function related to chylothorax evidenced by need for specialized low chain fatty acid formula (Enfaport)

• Delayed/impaired oral feeding related to hx of prematurity and extensive medical/respiratory support as evidenced by NPO status/full TF dependence and SLP evaluation

• Check TF daily and Reassessment in 2-4 days

Updates 7/10 – 7/12

• Possible d/c on 7/13

• Home feeding equipment delivered

• Officially weaned off phenobarbitol

Nutrition Re-Eval 7/12

• Weight: 6.38 kgs

• Diet: NPO

• Nutrition Support: GJ tube of Enfaport 22 cal/oz @ 35 ml/hr

• Estimated Nutrition Needs:

• Energy: 100 – 110 kcal/kg for corrected to term/post term based on estimated enteral needs.

• Protein: 2.2-3 gm for corrected to term/recovery needs based on estimated enteral needs.

• Yield of Enteral Nutrition: 840 mls of Enfaport 22 kcals/ounce will yield 616 calories/day 97 kcals/kg and 22 grams protein/day providing 97% of energy needs and 3.4 gms protein/kg/day

• Nutrition Diagnosis:

• Altered GI function related to chylothorax evidenced by need for specialized low chain fatty acid formula (Enfaport)

• Delayed/impaired oral feeding related to hx of prematurity and extensive medical/respiratory support as evidenced by NPO status/full TF dependence nd SLP evaluation

• Check TF daily and Reassessment in 2-4 days

• Monitoring and Evaluation: May want to measure head circumference prior to discharge and once well established, may wish to shorten TF to 20 hrs daily for 42 ml/hr for more mobility at home.

Updates 7/12 – 7/13

• G tube flushing, J tube not flushing• Attempted flushing with warm water• Cleared with warm water and cola

• Parents received full training of formula prep and feeding tube/pump instructions. • Also given recipe for clearing clogged tube

• Labs: IgG re-checked - at 281mg/dl• Average ranges between 176-601 for 2-3

month old

Discharge Summary 7/13

• Active problems: CLD, hypercoagulability, hypogammaglobulinemia

• Resolved problems: drug withdrawal, Chlythorax/Chylous ascites managed with Enfaport

• Medications: Vit D, hydrodiuril, zantac, and bactrim

• Outpatient Care:• Noonan’s Syndrome? F/U genetics testing at MGH• Plan for flow cytometry for IgG levels • Referrals for hematology, GI, pulmonary, cardiology

Questions??

ReferencesSources:1. Vogt, Annelie M., and Rebecca Brody. "Nutritional Management of Chyle Leaks." Support Line 31.3. 2. http://www.stanfordchildrens.org/en/topic/default?id=chronic-lung-disease-90-P023483. http://www.uofmhealth.org/health-library/hw1793544. http://emedicine.medscape.com/article/136471-overview5. http://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/transient-hypogammaglobulinemia-of-infancy/ ????6. http://www.ohsu.edu/xd/outreach/occyshn/training-education/upload/Infant-Pediatric-Formulas-Alisa-de-DeWeese.pdf7. http://www.merriam-webster.com/medical/hypogammaglobulinemia8. http://ghr.nlm.nih.gov/condition/noonan-syndrome9. http://www.ncbi.nlm.nih.gov/gtr/conditions/C0028326/10. http://my.clevelandclinic.org/services/heart/disorders/hypercoagstate11. http://costellosyndromeusa.org/2013Resources/Tools%20To%20Help%20Your%20Childs%20Nutrition%20-%20Karjoo%202013.pdf12. http://www.fodsupport.org/documents/Monogenarticle6.07.pdf13. http://www.nutricia.ie/products/view/monogen14. http://www.nlm.nih.gov/medlineplus/ency/article/003544.htm15. http://www.ext.colostate.edu/pubs/foodnut/09361.html16. http://www.nlm.nih.gov/medlineplus/druginfo/meds/a682007.html17. http://edis.ifas.ufl.edu/pdffiles/HE/HE77600.pdf18. Mead Johnson Nutrition Pediatric Product handbook19. https://rarediseases.info.nih.gov/gard/10955/noonan-syndrome/resources/820. http://www.nlm.nih.gov/medlineplus/ency/article/007308.htm21. https://my.clevelandclinic.org/health/treatments_and_procedures/hic_Fundoplication_Procedure_for_Children22. http://www.stanfordchildrens.org/en/topic/default?id=chronic-lung-disease-90-P0234823. https://www.healthcare.uiowa.edu/path_handbook/Appendix/Heme/PEDIATRIC_NORMALS.html

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