Interdisciplinary Case

Presentation

DR. BRETT TURNER

SARAH SMITH, RN, PNP STUDENT
KATE MORTON, RD
LIZ PALLOTTA, SW STUDENT
 Objectives

Get to know E.G.F.

Gather an improved knowledge EGs diagnoses
Improve on understanding of how multidisciplinary
teams factor in to patient care
Consider nutrition for EGF
Consider social and psychological factors for EGF
 E.G.F.

2 year old female

Chosen for this project because she has a
combination of diagnoses that are beneficial to learn
from
Why pulmonary?
Tracheo-esophageal fistula (TEF)
Obstructive Sleep Apnea Syndrome
Tracheomalacia
Recurrent pneumonia
 E.G.F

Medical background
EG is a now 2-year old with a complex past medical
history who is followed closely by the Pulmonary Service
at UAB/COA.

Past Medical History:

Birth Hx:
Born at 39 weeks gestation via spontaneous vaginal
delivery to a 23 year-old G1P0 mother.
Apgars 8/9

Birth Weight was 3969 grams

Pre-natal diagnosis of cleft-palate confirmed at the time of

delivery
 Past Medical History

NICU Course:
After delivery, an attempt to place a nasal enteric feeding tube
was unsuccessful.
EG transferred to COA on DOL1 for pediatric surgery evaluation.
After evaluation, EG was found to have a tracheo-esophageal
fistula and subsequently underwent surgical repair on DOL 4.
She continued to undergo extensive work-up for evaluation of an
underlying disorder causing her multiple congenital anomalies
Family left the NICU without a diagnosis.

After leaving the NICU, EG has had multiple readmissions to

COA (30 on most recent count), including multiple PICU
admissions.
 Medical Overview

Presenting symptoms:
Copious amounts of secretions requiring suctioning

Diagnosed with obstructive sleep apnea in hospital and on

supplemental oxygen at night
Failed swallow study at initial visit

Heart murmur
 Physical Exam

Weight: 12.41 kg. Height: 76 cm. BMI: 21.52. HR: 138. RR:
34. Oxygen Saturation: 98%.

General: Awake, alert and in no apparent distress. Chronically ill

appearing child.
HEENT: MMM, PERRL, EOMI. Tympanic membranes clear
bilaterally. Dysmorphic facies.
Neck: Supple without lymphadenopathy.
Chest: Coarse congested breath sounds without wheezing or
increased work of breathing.
CV: Regular rate and rhythm, no murmur. Capillary refill brisk.
Abdomen: Soft, non-distended. Gastrostomy tube is present without
surrounding erythema or drainage.
Extremities: Warm and well perfused. No digital clubbing.
Neurologic: Normal tone and strength for age.
Skin: No rashes or obvious lesions.
 Surgical History

Past Surgical History:

TEF repair Day of life 4
Subsequent esophageal dilations 11
Gastrostomy tube placement
Cleft lip repair
Tympanostomy tube placement 2
Flexible Bronchoscopy 2
Nissen fundoplication
Tonsillectomy
Adenoidectomy
 Diagnoses

Current Chronic Diagnoses:

TEF, s/p repair

Bilateral cleft lip and palate, s/p repair

Gastrostomy tube dependence

GERD

Abnormal hearing

Obstructive Sleep Apnea Syndrome

Tracheomalacia

CHARGE Syndrome

Recurrent aspiration pneumonia

 Embryology of the airway

Foregut arises as one

common tube early in
development.

Within the first few weeks

of gestation, the lung bud
has separated from the
esophagus into two distinct
tubular structures.
 Tracheo-Esophageal Fistula

Tracheoesophageal
fistula is a common
congenital anomaly of
the respiratory tract
Approximately 1:3500
live births
Typically occurs with
esophageal atresia.
TEF/EA occur as part of
the VACTERL or
CHARGE associations in
about 50% of cases and
is associated with
congenital heart and
genito-urinary defects.
 CHARGE Syndrome

Coloboma
Heart Anomalies
Atresia of the Choana
Mental Retardation
Genital/renal
abnormalities
Ear Abnormalities
 Tracheomalacia associated with TEF

Tracheo-esophageal fistula (TEF)/oesophageal

atresia is one of the most common and serious
congenital malformationsmorbidity and mortality
still remains quite high (Pal, 2014).
A form of tracheomalacia that occurs in patients with
TEF is due to the lumen of the trachea taking on a
D-shaped configuration because of an abnormally
wide membranous portion of the trachea (Pal,
2014).
Tracheomalacia
Flexible Airway Bronchoscopy
 As an outpatient, EG sees the following general and specialty
clinics:
Pediatrician
Pulmonary
General Surgery
Plastic Surgery
ENT
Audiology
Nutrition
Genetics
 Medications

Albuterol Sulfate (2.5mg/3mL) nebulize 1 vial twice a day and q4h

as needed for wheezing/coughing
ProAir - 4puffs, every 4 hours as needed
Hypersal 3.5% - Nebulize 1 vial twice daily
Culturelle - 1 packet daily
Poly-Vi-Sol 1mL daily
Lansoprazole
Azithromycin
Dulera 100-5 mcg/act
Robinul (Glycopyrrolate)
Ipratropium Bromide 0.02%
 Current Respiratory Support

Dulera 100/5 2 puffs twice daily with aerochamber

and mask
Albuterol MDI 4 puffs every 4 hours as needed for
cough, wheezing, increased work of breathing or
shortness of breath
Azithromycin 125 mg via G-tube M, W, F for
prophylaxis against recurrent respiratory infections
Oxygen by nasal cannula at 0.5 lpm while sleeping
Nutrition Considerations
 Anthropometrics

Length:74.1cm = <3rd%ile (z score = -2.89)

Weight: 13.5kg = 93rd%ile (z score = 1.27)
Due to CHARGE syndrome

Weight for Length: >97th%ile (z scores = 3.65)

Growth Chart
 Nutrition Diagnosis

Gastroesophageal reflux disease (GERD), with out

esophagitis
TEF
Failure to Thrive
Dysphagia, Oropharyngeal Phase
 GERD without Esophagitis

Can contribute to poor growth

Required fundoplication surgery to reduce GERD &
frequent formula changes
 Feeding Complications in TEF

Tracheoesophageal fistula (TEF) leads to:

Difficulty swallowing

Inability to digest food safely

 Dysphagia, Oropharyngeal Phase

Result of Swallow studies

04/17/2015: Aspiration without cough

08/20/2015: There was silent aspiration with all consistencies

with-nasopharyngeal regurgitation
 Dysphagia, Oropharyngeal Phase

Problems with chewing, initiating the swallow, or

propelling the food through the pharynx to the
esophagus
 Gastrostomy tube placement

Date: 1/ 26/2015
Age: 3 days old

Type: 14-French x 1-cm button gastrostomy

 Feeding Related Procedures

1/26/2015 : G Tube Placement

3/3/2016 : G J Tube Placement
5/20/2016: Fundoplication
9/29/2016: Fundoplication w/ G Tube Placement
G J Tube
Nissen Fundoplication
 Reason for G - tube

TEF
Dysphagia, Oropharyngeal Phase
Craniofacial Abnormalities
 Current Feeding Rx

Formula Pediasure 1.0

Nutrition Rx
05/20/2016
150 mL x 3 feedings+ night drips 60ml/hr X 12hrs = 1170
mL/day

09/29/2016
1170 mL x 1.0 kcal = 1170kcals/day 15% (decrease feeding by
15%)
 Implications of Over Feeding

EGFs weight increased 5kgs in 6 months

Weight for age %ile change = 43rd%ile 98th%ile

28 grams/day weight gain

Recommended weight gain for 19-24 months is 6

grams/day.
 Estimated Nutritional Needs
Based on the Estimated Energy Requirements (EER)

Macronutrient needs:
~ 80 kcal/kg

1+gram/kg protein

80 kcals x 13.5kg = 1080 kcals/day

1 gm pro x 13.5kg = 13.5 gm pro/day

Fluid needs: 1500-1800ml/m2

 Overfeeding?

= 1170 kcals/ day = 90 kcals/ day too much

Non-medical/ psychosocial
factors
 January- February, 2015

First child born to mom and dad

Employment:
Mom is on maternity leave from Medical Center, where she works as a
receptionist
Dad is employed full-time as pipe fitter

Insurance:
All members on Blue Cross Blue Shield policy through fathers employer

Physical environment:
Currently live in Muscle Shoals, AL. In the meantime, residing in an
apartment offered through the Homewood Church of Christ
Parents were well prepared for childs discharge from hospital.
Appropriate crib, car seat, and additional resources were in place.
 January, 2016

Living environment:
Residing in Tuscumbia, AL (Colbert Co)
Child does not attend daycare

Employment:
Mother had to quit job as a receptionist in order to stay home
with child
Father continues to be employed full-time as a pipe fitter.
Father travels often for work.

Social/Financial Support:
Parents express financial concern due to buildup of medical
bills they have for their child. Co-payment for each
hospitalization is $250.
All members have BCBS through fathers employer
 January, 2016

Social/Financial Support:
Parents applied for SSI. Child was deemed disabled due to medical
condition however was denied SSI based on exceeding income
maximum.
They were encouraged to apply again as the first application
included mothers income when she was working.
Social support includes maternal and paternal grandparents. Family
recently moved in with maternal grandparents due to financial
situation. Paternal grandparents are currently renovating fathers
childhood home. Future plan is for child, mother and father to
reside in this home once completely renovated.

Services:
Child is receiving in-home therapy services through Early
Intervention (Easter Seals). Therapies include OT and PT
 March, 2016

Services:
Child is assigned to a worker from Elderly& Disabled Medicaid
Waiver. Child qualifies for skilled respite and homemaker services.

Employment:
Father loses job. Major concerns as this was familys primary source
of income
Father reports he will draw unemployment in the meantime until he
finds another job

Insurance:
Insurance will not be affected as insurance is through the Pipe
Fitters Union
Family proceeds to apply for Medicaid and SSI starter kit
 July, 2016

Employment:
Father obtains new job as a Plumber/Pipe fitter. Requires that he travels
often and at this time was in Iowa.

Physical environment:
Continues to reside with maternal grandparents

Insurance:
Did not qualify for SSI/ Disability after two attempts.
Did not qualify for Elderly and Disabled Waiver through Medicaid. Reason
was due to missing documentation. New application has been resent.

Social Support:
Social support includes family and church members. Mom seeks support
through support groups through Facebook for CHARGE and for Cleft Lip
Palette.
 October, 2016

Employment:
Dads work is now local and does not require him to
travel anymore

Insurance:
E&D Waiver paperwork was not received by E&D
caseworker, therefore case was closed.
New E&D waiver application was filled out, with
Social worker present. (3rd time)
 December, 2016

Insurance:
Social worker contacted mom to follow up on E&D
Medicaid waiver however mother was unable to be
reached.
 What determinants/factors influenced childs
outcome?

Healthcare: Access to healthcare, travel time is 2+

hours, one way

Individual behavior: Compliance with medications.

Compliance with appointments/ phone calls with
medical staff

Social environment: Maternal and paternal

grandparents are heavily involved. Involvement with
church. Support groups

Physical environment: Safe living environment.

Stable home. No current smokers in the home.
Distance to Childrens of Alabama
 Are there any disparities in outcomes for children
affected by this diagnosis and/or special need?

CHARGE Syndrome: TEF:

Cognitive impairments Cardiovascular
Sensory deficits Digestive
Learning disabilities Genitourinary
Increased mortality Musculoskeletal
 What policies or programs could influence the outcomes for
the population affected by this diagnosis or special need?

Primary prevention: Aims to prevent disease or injury

before it ever occurs. Ex: Genetic testing (CDH7 highly
recommended, although not perfect), Prenatal care/
screenings, Funding for research

Secondary prevention: Aims to reduce the impact of a

disease or injury that has already occurred. Ex: complying
with medical appointments, surgeries, medications, hearing
amplifications

Tertiary prevention: Aims to soften the impact of an

ongoing illness or injury that has lasting effects. Ex: Early
Intervention, Support groups, educational resources, AIDB
 Reference

1. Burrow TA, Saal HM, de Alarcon A, Martin LJ, Cotton

RT, Hopkin RJ. Characterization of congenital
anomalies in individuals with choanal atresia. Arch
Otolaryngol Head Neck Surg. 2009;135(6):543.
2. Clark, DC. Esophageal atresia and tracheoesophageal
fistula. Am Fam Physician 1999;59:910. TEF types
classified according to the scheme developed by EC
Vogt in 1929, as modified by Gross.
3. Pal, K. (2014). Management of associated anomalies of
oesophageal atresia and tracheo-oesophageal fistula.
African Journal Of Paediatric Surgery, 11(4), 280-286.
doi:10.4103/0189-6725.143127.
The End