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 or 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 10 months IgG 327 IgA 29 • 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
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
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