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.
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NI 1.4
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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.
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CHD plays a vital role in determining estimated needs due to need for catch up growth.
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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!