Pediatric Case Study

Pediatric Case Study A Closer Look at Trisomy 18 and Tetralogy of Fallot

Ashley Spence University of Maryland Dietetic Intern

Objectives 

Understand the Disease



Meet the Patient



Nutrition Assessment



Prognosis, Outcomes, Summary

Understanding the Disease Trisomy 18 and Tetralogy of Fallot

Trisomy 18 •

3 copies of chromosome 18 in every cell of the body.

•

Humans usually have two copies of chromosome 18.

T18 Characteristics 

Complex medical problems and birth defects 

Heart defects



Brain, spinal cord, and other internal organs



Cleft lip or palate



Club foot, hernia



Renal abnormalities



Difficulty regulating breathing and temperature



Feeding difficulties



Intrauterine growth retardation and low birth weight

Tetralogy of Fallot •

Type of congestive heart defect characteristic of four defects

•

Cyanotic heart legion resulting in decreased/below normal oxygenated blood flow

CHD/ToF Characteristics 

Group of four structural defects resulting in decreased amounts of oxygenated blood circulating through out the body



Heart is forced to work harder



Increased work of breathing



Increased energy needs



Decreased po intake due to fatigue with feeds



Poor absorption of nutrients from the digestive tract



Growth disturbances noted in weight and height



FTT Woldu et al, 2014

Nutrition for CHD 





Energy Needs: 

0-.5 months- 120-150 kcal/kg day



Based on Catch Up Growth for Children with CHD

Protein Needs: 

0-.5months- 2.2-3.5 g/kg/day



Based on Catch Up Growth for Children with CHD



Intubated with TPN~ 80 kcals/kg



Extubated with TPN~ 90-100 kcal/kg



Intubated with EN~90 kcal/kg



Extubated with EN ~ 120-150 kcal/kg

Fluid needs: 

100 mL/kg



(Based on Holiday Segar Method (100 mL/kg)

Abad-SindenA, 2001

MEET THE PATIENT 

Patient is referred to as J.W.



2.6 months old



Male



Multiple medical conditions: 

Trisomy 18 (T18)



Tetralogy of Fallot (ToF, a Congestive Heart Disease defect)

Report Upon Admission 

Hospitalized at birth for CHD



Transfer patient from CHKD 



Determined not a good candidate for surgical correction

Receiving NG /ND tube feeds 

Enfamil/BM, 24 kcal @ 23 mL/hr x24 hrs



3.5 kg or 3500g 



50.5 cm 



Weight for age %tile- <3

Height for length %tile- <2

Head Circumference-36.5 cm 

HC for age %tile- <2

Report Upon Admission Cont’d Past Medical History 

  

Episodes of bradycardia with desaturation

Left club foot Hernia Mom attempting bottle feeds but not going well due to desaturation

Significant Medications 

Epinephrine



Vasopressin



Furosemide



NaCl, KCl



Dopamine, Fentanyl, Prednisolone



Caffeine



Sodium Bicarbonate

Nutrition Assessment

Diet History 

Prior to admission: patient is a transfer from Children’s Hospital of the King’s Daughter, CHKD, southeastern VA.



Feeding history: 

Home diet included breast feeding-breast milk and formula.



Prior to admission, the patient’s mother reported she was attempting bottle feeds. However, they were not doing well. The patient was then transitioned to NG feeds and later advanced to ND feeds 2/2 issues with desaturation.

Diet History Cont’d 

Previous Hospital : enteral feeds  Enfamil/BM



Caloric Density: 24 kcal/oz



Schedule: 23 mL/hr x 24 hrs



Tolerance issues (nausea/vomiting, diarrhea/constipation)-None documented



Vitamin/Mineral Supplements: None listed



Food Allergies: none reported

Weight Assessment 



Weight for Age 

3.5 kg



<3 percentile

Height for Age 

50.5 cm



<2 percentile

Weight Assessment 



Head Circumference for Age 

36.5 cm



<2 percentile

Weight for Length 

50th percentile

Lab Values Labs

2/5

2/6

2/7

2/8

2/9

2/10

2/11

2/12

2/13

Na

139

145 H

144 H

139

137

145 H

146 H

144 H

136

K+

4.0

3.8

3.9

4.1

3.7

3.9

3.1 L

3.4 L

6.0 H*

Cl

102

104

105

102

104

108

112 H

109 H

102

CO2

26 H

25 H

27 H

20

21

24 H

22

23

22

BUN*

9

13 H

17 H

26 H

29 H

39 H

30 H

29 H

42 H

Cr*

0.4

0.6 H

0.8H

1.0H

0.6 H

0.7 H

0.5

0.3

0.7 H

Glucose

80

146 H

129 H

115

105

102

134 H

107

95

Ca

9.7

11

9.4

8.3 L

9.5

9.8

9.3

9.9

9.7

Mg

2.2

1.8

Medications 

Patient has several continuous medications given via IV/fluid



Medications administered using much of maintenances fluid needs



Epinephrine- feeding is not recommended while on “pressors” due to complications with perfusion, nutrient absorption, and potentially ischemic gut.



Furosemide- “Lasix” can deplete potassium, sodium, and electrolyte stores.

ď&#x201A;§

NI 1.4

ď&#x201A;§

Inadequate energy intake related to OR/need for surgery as evidenced by NPO status.

PES Statement

Estimated Needs 

Energy  Kcals/kg: 120-150 kcal/kg/day



Protein  Grams protein/kg: 2.2-3.5 g/kg/day



Fluid  mL/day to meet maintenance fluid needs: 100 mL/kg

Recommendations 

Initial Consult: 



When medically able resume ND feeds of 24 kcal/oz BM/enfamil @ 23 mL/hr x 24 hrs to provide 158 mL/kg fluid, 126 kcal/kg, 2.7 gm PRO.

Order Implemented: TPN 

95 mL/kg fluid, D 13% (GIR 3.3 mg/kg/min), 2 gm/kg lipids provides 42 kcal/kg/day



Monitor for ability to liberalize fluid restriction and advance TPN by GIR of 1-2 mg/kg/day daily to goal of 10-12 mg/kg/day.



Advance protein to goal of 2.5-3 gm/kg/day



Advance lipids by 1 gm/kg/day to goal of 3 gm/kg/day



Advance to goal intubated kcals of ~80 kcal/kg/day or extubated kcals 90-100 kcal/kg/day

Risk Level 

High/Severe 

NPO status and need for TPN



Intubated



Surgery



Open chest



Poor po PTA

Intake at Follow Up 

2/9 



2/12 



D19, 34 kcal/kg, 85 mL/kg, 1.5 gm/kg protein, 1 gm/kg lipid

D 13, 42 kcal/kg, 95 mL/kg, 2 gm/kg protein, 2 gm/kg lipid

2/13 

D18, 48 kcal/kg, 95 mL/kg, 2 gm/kg protein, 2 gm/kg lipid

At Last Follow Up 

2/19 

Patient intubated on TPN



TPN advanced to goal



Received 100% of nutrition needs in last 24 hours  110 mL/kg, D22, GIR 10.1, 2.5 gm PRO/kg/day, 2.5 gm lipids/kg/day



Significant weight gain due to fluid status (4.19 kg)



No actual weight gain since admission

Follow Up Recommendations 

Recommendations:



Monitor for the ability to initiate ND feeds with BM/Enfamil 20 kcal/oz @ 3mL/hr. Advance by 3 mL Q8H to goal intubated rate of 20 mL/hr (137 mL fluid, 91 kcal/kg, 1.9 gm/kg protein)



Once extubated advance to 23 mL/hr and concentrate BM by 2 kcal/oz/day to goal of 24 kcal/oz (home regimen)

Future Plan 

Overall-Ideally patient would be transitioned from TPN to po feeds when stable.



Parenteral nutrition- when medically able, patient will be weaned from TPN to enteral feeds.



Enteral nutrition- when medically stable, patient will be transitioned to enteral feeds via NG, then condensed.

Summary ď&#x201A;§

T18 and Tof have the potential to negatively impact growth and development.

ď&#x201A;§

CHD plays a vital role in determining estimated needs due to need for catch up growth.

ď&#x201A;§

Early nutritional intervention and surgical correction are strongly recommended.

References 

Children’s National Pediatric Nutrition Assessment Slides. January 25, 2015



Children’s National Pediatric CICU/HKU Manual. February 2015



Abad-SindenA, Sutphen JL. Growth and Nutrition . In: Emmanoullides GC, Riemenschneider TA, Allen HD, Gutgessel HP, eds. Moss and Adams Heart Disease in Infants, Children, and Adolescents, Including the Fetus and Young Adult. 6th edition. Baltimore Md:Lippincott, William and Wilkins; 2001:324331



Wessel JJ, Sam PQ. Cardiology. In Samour PQ,King K eds. Handbook of Pediatric Nutrition 3rd edition. Sudbury, MA: Jones and Bartlett Publishers Inc; 2005: 407-420



Woldu, Krista, Arya Bhawna, Emile Bacha, Isamee Williams. Impact of Neonatal versus Nonneonatal Total Repair of Tetralogy of Fallot on Growth in the First Year of Life. The Society of Thoracic Surgeons. New York, New York.

2014: 1399-404.

Thank You!