A Case of Recurrent Wheeze MT AAP Roundup September 28, 2019 - - PowerPoint PPT Presentation

A Case of Recurrent Wheeze

MT AAP Roundup September 28, 2019

Allison Young MD Lauren Wilson MD Paul Smith DO Carol Cady MD/PhD Lauren Willis MD

Primary Care – Allison Young, MD

Introducing Baby G

• Born AGA at 39 1/7 weeks at CMC to G7P5->6 mom.
• Two previous children in my care. Last infant died by homicide in

care of mom’s male partner.

• Benign newborn course. Discharged home in DCFS custody to

foster parent.

• Admitted to CMC at 11 days of age for hypoxemia and wheeze – dx.
• Bronchiolitis. Intubated and eventually transferred to Seattle due to

possible need for ECMO.

• Between birth and hospital admission, was having mandatory visits

with biological mom at DCFS and concern raised about second hand smoke exposure.

• Maternal functional status low. Older half sibling with cutis aplasia

and craniosynostosis.

Hospitalist – Lauren Wilson, MD

Admission for fever

• 11 day old male, presenting with fever to 100.5 and

grunting/congestion, poor feeding.

• Mom GBS positive adequately treated.
• Birth history: Born to 29 yo G7P6 mother at term. HIV neg, Heb

B/C neg. No HSV hx. BW 3.146 kg. Uneventful vaginal delivery. Discharged to foster care.

• CRP 1.39 (normal <0.5), U/A neg, WBC 7.1 with 8% bands,

rhino/enterovirus positive.

• LP without pleocytosis.

Admission for fever

• Escalated to CPAP on HD 4
• Continue to worsen, required intubation HD 5
• HD 7 had mucous plugging event, required brief compressions,

worsening ventilation, transferred to Seattle in case of deterioration needing ECMO

• Thought to be severe viral pneumonia, bacterial superinfection

(H flu + Moraxella grew from tracheal aspirate). HIV negative.

• Treated with Unasyn, did not require ECMO, transferred back to

Missoula 6 days later

• Uneventful extubation and recovery

Subsequent admissions

• Readmitted 1 month later with clinical bronchiolitis, 5 day stay,

max support 0.5 L/m NC O2

• Additional history: Lives in a house with adoptive mom and brother, 2

years older than the patient. They have 1 dog who is in the bedroom, and 1 cat not allowed in the bedroom. There is no humidifier, no water

• r mold damage. He is not in day care. Tobacco smoke exposure with

supervised visits with bio mom.

• Video fluoroscopic swallow study showed vallecular pooling but no

laryngeal penetration or aspiration, much better with Dr. Brown preemie nipple, caregivers trained

• pH Probe = no GERD
• Some response to beta 2 agonists in hospital

Recurrent bronchiolitis

Question: How many episodes of bronchiolitis are too many?

• Readmission within 30 days for acute bronchiolitis (multiple

studies): 2.1 - 6%

• 12 month follow up shows 52.7% of infants with rhinovirus

bronchiolitis have recurrent wheezing, vs 10.3% of controls (Midulla et al 2012)

Image Source: Trendmicro.com

So what do we do?

• Phone a friend or two

“Don’t just do something. Stand there.” - Lauren Wilson, personal motto

Pulmonology – Paul Smith, DO

Wheezy Infant: Differential Diagnosis

Infectious bronchiolitis – WARI, unlucky repeats Tracheo-bronchomalacia Bronchopulmonary Dysplasia Aspiration syndromes – GERD Abnormal swallowing Anatomic (TEF, Laryngeal Clefts) Protracted bacterial bronchitis of Pediatrics

“Not all that wheezes is asthma” Hippocrates “Not all that wheezes is bronchiolitis” Allison Young, MD

Wheezy Infant: Differential Diagnosis

Cystic Fibrosis Childhood Interstitial Lung Diseases NEHI – Neuroectodermal Hyperplasia of Infancy Primary Ciliary Dyskinesia (PCD) Congenital Heart Diseases – Pulmonary overcirculation, Congestion Large VSD’s, ASD’s, Pulmonary Vein Stenosis Airway Anomalies – Rings, Slings, Stenosis Immunodeficiency – Congenital, Acquired (HIV)

Wheezy Infant: Evaluation

Physical Exam – where, when and how is the noise Timing of noise – inspiratory, expiratory, biphasic Location of noise – upper vs. lower Constancy or associated events (feeding, position) Pulse Oximetry Heart Sounds Weight, Growth

“Not all that wheezes is wheezing” Smith

Wheezy Infant: Evaluation

CXR Focal infiltrates – infection, aspiration Peri-bronchial cuffing – infection, aspiration, PBB Ground glass appearance – chILD, CHD, CF Interstitial pattern – chILD, CHD, CF Heart size Swallow Study / Esophagram – TEF, Rings/Slings ECHOcardiogram Sweat Test CT Chest – sedated, controlled inflation Laryngoscopy/Bronchoscopy Genetics – CF, Surfactant defects, PCD

Workup for our patient

• CXR – When ill: LUL atelectasis, later resolved. Mild perihilar

bronchial wall thickening, mild hyperinflation.

• Sweat chloride test - Normal
• Swallow study – Essentially normal
• Echocardiogram – When ill, mild septal flattening suggestive of

elevated pulmonary / RV pressures – Resolved on follow up

• EGD - Normal
• Bronchoscopy – Mild laryngomalacia, mild tracheomalacia but no

tracheal compression, grade 1 subglottic stenosis. Fluid later grew S pneumoniae.

• Immunology consultation (to follow)

Primary Care follow up

• Continues with frequent respiratory infections
• During this time – immunodeficiency workup in process
• Genetic testing – Carnitine deficiency identified, unclear

significance

• Notable for period of poor growth between ~18 months and 21

months

• Clinical history involved dysphagia and poor oral intake

Gastroenterology – Lauren Willis, MD

GI: History

• Formula fed as an infant
• Supplemented as a toddler to current with amino acid based

formula 20-24oz a day

• Frequent coughing and choking with feeds – especially liquids
• Likes a wide variety of foods but has early satiety
• Mother notes he does best with puréed textures

GI: Workup

• Upper GI series 15 months – normal
• Esophagogastroduodenoscopy (EGD) with bx 2 years –

mild reactive changes esophageal mucosa; no inflammation

• pH impedance 26 months – normal
• Video swallow study with SLP
• SLP Eval and therapy sessions – Requires Maximal

Effort for Minimal Intake; demonstrates avoidance/refusal behavior

GI: Workup

• Video swallow study with SLP:
• Frank Esophageal Dysmotility - Moderate amount of contrast lined

esophagus between swallows, pooling contrast, retrograde bolus movement

• Reduced oral initiation and awareness → premature spillage thin

liquids to vallecula and piriform sinuses

• Flash laryngeal penetration with thin liquids only; no aspiration with

multiple consistencies

VSS – Esophageal Dysmotility

VSS – Esophageal Dysmotility

GI: Feeding problems

• Oral aversion, oral defensiveness – differential is vast
• Pretty common
• NICU course with instrumentation
• Behavioral
• Developmental delay with oropharyngeal weakness/incoordination
• GER
• Eosinophilic Esophagitis
• Aspiration
• Congenital heart disease
• Food allergies

GI: Feeding problems

• Less Common
• Anatomic
• Stricture
• Vascular sling

“It’s important to eat dessert. The sweet and the fat signal satiety and let you know you are done with your meal.” - Lauren Willis, personal motto

Immunology – Carol Cady, MD, PhD

Immunology

• Newborn screening normal TRECs (T cells present)
• On appropriate treatment for reactive airways
• Allergy testing for cat and dog negative
• Absolute lymphocytes low normal

Immunology

 Infections:

Rhinovirus/enterovirus (pulmonary) 2 wks of age H influenza B & Moraxella catarrhalis (pulmonary) 3½ wks of age Human metapneumovirus (pulmonary) 3 months of age H influenza B (skin) 3 months of age RSV (pulmonary) 10 months of age

• IgG, IgA, IgM and complement function normal (9 months)
• Lymphocyte mitogen function normal (12 months)
• IgG, IgA, IgM re-checked and normal (17 months)

Immunology

• BAL fluid positive for Streptococcus pneumoniae (22 months)
• Re-consider functional deficiency of IgG – check vaccine titers

Deficient Strep pneumonia IgG titers despite infection AND Prevnar 13

• Low response to tetanus and H influenza B vaccines

10 months IgG 327 IgA 29

Immunology

• Treatment options: prophylactic antibiotics vs. IgG infusions
• IgG infusions started at age 2
• Follow-up 4 months later: no significant infections

Primary Care follow up

• G tube placed. Began IVIG.
• Growth improved. Now typically developing.
• MP2 created with mom.

Lessons Learned

• Frequent URIs not usually a red flag for immune deficiency
• HOWEVER: growth failure, more severe infections were flags here
• Important to talk to your colleagues, and don’t be afraid to refer
• Important to follow up and be persistent
• I.E. quant IgGs were normal twice, but didn’t explain symptoms
• Keep an open mind, doubt your diagnosis
• Sometimes you have more than one problem

References

• Kemper A, Kennedy E, Dechert R et al. Hospital Readmisison

for Bronchiolitis. Clinical Pediatrics. 44(6):509-513

• Midulla F, Pierangeli A, Cangiano G et al, Rhinovirus

bronchiolitis and recurrent wheezing: 1-year follow up. European Respiratory Journal 39:396-402