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Pediatric Pulmonary Pearls Terrence W. Carver, Jr., M.D. Children’s Mercy Hospitals and Clinics Medical Director of Respiratory Care and Pulmonary Medicine Associate Professor of Pediatrics University of Missouri-Kansas City © The Children's Mercy Hospital, 2016 Disclosures • Many medications used in pediatric patients are not specifically approved by the FDA. • Many medications used in cystic fibrosis (CF) patients are not specifically approved by the FDA. Educational Objectives • Review management of (some) pulmonary concerns encountered in primary care practice. – Recognize a typical clinical scenario for foreign body aspiration. – Review evaluation for hemoptysis in a noncystic fibrosis patient. – Evaluation of a pediatric patient with chronic cough. Selfish Goal • My goal is to get invited back! Foreign Body Aspiration • Typical presentation (acute) – Toddler – Cough/choke episode – Unilateral wheeze Foreign Body Aspiration • O2 saturation and respiratory rate • Retractions/distress • CXR • Lateral decubitus views – Air trapping What’s wrong with this picture? Foreign Body Aspiration • Witnessed • Suspected • Possible Foreign Body Aspiration • Solid/particulate – ENT or Surgery for rigid bronchoscopy • NPO • Write checks that I don't cash. • Oils/Volatiles – Supportive care and oxygen (likely admission) • Not bronchoscopic removal New Hemoptysis—non CF • Amount and color – Bright red blood, clots, blood streaked mucus • Source – Lung, nose, GI • Bronchitis/pneumonia • Epistaxsis • Liver disease, GERD New Hemoptysis—non CF • Urine output/blood – Pulmonary/Renal Syndrome • Rash, abnormal joints, chronic/recurrent fever – Rheumatologic New Hemoptysis—non CF • CXR findings and oxygen saturation • CBC and reticulocyte count • PT/PTT/LFTs/platelet count • Guaiac stool • Urinalysis/BUN/creatinine – Blood and casts Umm…Dr. Segeleon… New Hemoptysis—non CF • CT Chest with contrast or CT angiogram • Bronchoscopy/BAL • P and C-ANCA, RF, ANA, ESR, CRP, type and cross, brain natriuretice peptide, Ddimer, sputum culture/AFB/TB evaluation? • Echocardiogram New Hemoptysis—non CF • Consults; "Who you gonna call?" – Rheumatology, Renal, ID, Surgery, ENT, GI • • • • Positive pressure ventilation Lung biopsy Lung resection Blood transfusion Stridor • Think airway (ENT) • Laryngomalacia • Recurrent croup • Vocal Cord Dysfunction (VCD) Laryngomalacia • Infant • Growing well and no distress – Some chest wall retractions esp. supine • If more severe consider subglottic stenosis, laryngeal papillomatosis, others • Scope (ENT) if suspicious for others or severe Recurrent Croup • Rule out anatomic abnormality (ENT) • Occasionally responds to "reactive airway disease" medications (Pulmonary) – Trial of inhaled corticosteroids Vocal Cord Dysfunction • Adolescent • Type A personality • Stress related (games vs. practice) • Confused with exercise induced asthma – Dysphonia, throat/neck vs. chest, choking sensation – Video on cell phone • Speech Therapy for breathing exercises Chronic/Recurrent Cough • History – How long – When • AM/PM • While asleep – Associated feeding • Good growth or failure to thrive Chronic/Recurrent Cough • Triggers – Upper respiratory tract infections, allergies, exercise,cold air, etc. • Seasonal – Previous steroids Chronic/Recurrent Cough • Symptoms of GERD/aspiration • Family history of cystic fibrosis, asthma, or allergic rhinitis • Vocal Cord Dysfunction with “wheeze” but normal oxygen saturation Chronic/Recurrent Cough • Asthma – Early morning around 0200-0500 • GERD – Feeding/chest rattle – Family often says "wheeze" Chronic/Recurrent Cough • Allergic Rhinitis/Sinusitis – On awakening • Aspiration – Cough/choke with po feeds vs. silent aspiration • CF – Loose – Difficulties gaining weight Chronic/Recurrent Cough • Vascular ring – Stridor, dysphagia • Immune dysfunction – Other sites of infection, failure to thrive • Habit – Loud, honking, resolves with sleep Chronic/Recurrent Cough • TB – Weight loss, night sweats • Large tonsils/upper airway obstruction – Hard to judge • Primary cilia dyskinesia – recurrent sinusitis and otitis media, situs inversus, respiratory difficulties as term newborn Chronic/Recurrent Cough • Vitals and oxygen saturation • Exam of nose and lungs, etc. • Type of cough • Any stridor Chronic/Recurrent Cough • Labs – CXR – UGI – Sweat test • Newborn screening began: – MO 7/1/07 SD 6/1/05 – KS 7/1/08 NE 1/1/06 What’s wrong with this picture? Chronic/Recurrent Cough • TST • Immune evaluation • Swallow study • pH probe • CT Chest with contrast Chronic/Recurrent Cough • Bronchoscopy/BAL – Anatomy – Lipid laden macrophages Chronic/Recurrent Cough • Cilia biopsy by ENT – Structure typically but not function Pneumothorax • First or recurrent • Same side • Connective tissue disease • CT Chest (with contrast) – Blebs • Surgery consult What’s wrong with this picture? Tracheostomy & Cough • Color, amount of sputum • Increased suctioning • Fever • Acts sick • Increased oxygen need Tracheostomy & Cough • Previous organisms and sensitivities • Previous antibiotics that helped or didn’t • Ever Pseudomonas or MRSA Tracheostomy & Cough • Vitals and oxygen saturation • Type and size of tracheostomy • Breath sounds • CXR compared to previous Home Ventilator • Similar to tracheostomy • Ventilator settings and times of day used • Cuffed vs. uncuffed tubes • Blood gas evaluation and bicarbonate level from basic metabolic panel Tracheostomy Concerns • ENT generally – Too big – Too small – Can’t get back in • Critical vs. inconvenient • Cuff problems Snoring/Obstructive Sleep Apnea • ENT • Large tonsils/adenoids • Needs Polysomnogram (PSG aka Sleep Study) – Sleep or Pulmonary Clinic • Consider admit Pectus Excavatum • Pulmonary Fuction Testing • Surgery Clinic • Cardiology Clinic – Exercise study CF with Cough • Color and amount of sputum • Change in taste--anaerobes • Fever--not typically noted • Associated symptoms – Fatigue – Weight loss – URI CF with Cough • History of ABPA (Allergic Bronchopulmonary Aspergillosis) • Other pertinent history • Home medications • Last antibiotic course/dosing • Last admit therapy--especially if admitting CF with Cough • Oxygen saturation • Fever--atypical for standard exacerbation • Respiratory rate/distress • Breath sounds – Crackles – Reduced air movement – Wheeze uncommon CF with Cough • Previous culture results • CXR results compared to previous • Other labs – WBC, RSV, Influenza – Pulmonary function tests with reduced FEV1 Pseudomonas aeruginosa • • • • Frequent pathogen Resistance pattern important Consider ciprofloxan (Cipro) Often admit for IV antibiotics – Ceftazidime and tobramycin unless resistant MRSA • Increased incidence in CF not unlike overall community • Decreased survival • Consider linezolid (Zyvox) or Bactrim • Medication interactions CF with Cough • What does family want? – Home antibiotics – Admit – Repeat sputum or throat culture CF with Hemoptysis • Amount, color – < 5 ml vs. more – Bright red blood vs. streaks in mucus • New or many times • Can patient tell where the bleeding is coming from? CF with Hemoptysis • Home medications – ADEK • Other recent history – Pulmonary exacerbation • Other pertinent history – Liver disease/portal HTN CF with Hemoptysis • Oxygen saturation • Respiratory rate/distress • Breath sounds – Crackles – Reduced air movement – Wheeze CF with Hemoptysis • PT • PTT • CBC • Liver function testing • Type and cross for PRBC transfusion CF with Hemoptysis • CXR compared to previous • Bronchiectasis CF with Hemoptysis • Positive pressure ventilation • Bronchial artery embolization • Lung resection • Stop NSAIDS • FFP and/or vitamin K CF with Hemoptysis • Hold airway clearance • Antibiotics • PRBC transfusion CF with Hemoptysis • What does family want? – Home antibiotics – Admit – Repeat sputum or throat culture CF with Abdominal Pain • Location and duration • Lack of stooling • With eating (constipation, DIOS) • Spitting up (GERD) • Hematemesis (liver disease) CF with Abdominal Pain • Home medications • Previous history – Meconium ileus (adhesions/bowel obstruction) • Previous KUB/abdominal ultrasound • Previous liver enzymes CF with Abdominal Pain • Considering (more likely) – DIOS (Distal Intestional Obstructive Syndrome) – Constipation – Missing enzymes – GERD CF with Abdominal Pain • Considering (less likely) – Gall stones – Portal hypertension – Liver disease – Pancreatitis • Pancreatic sufficient CF with Abdominal Pain • Exam – Tenderness – Mass • Palpable stool – Rebound CF with Abdominal Pain • KUB • Abdominal ultrasound • Liver enzymes • Lipase/amylase • PT CF with Abdominal Pain • GI or Surgery services – Cautious with narcotics • Enema • GoLYTELY po or NG • Increase constipation medications – MiraLax – Fluids CF with Abdominal Pain • What does the family want? – Trial of therapy at home – Admit Suggested Reading • Kendig’s and Chernick's Disorders of the Respiratory Tract in Children, 8th ed., 2012. • Respiratory Disease in Children: Diagnosis and Management, ed. Loughlin and Eigen, 1994. • Care of the Child with a Chronic Tracheostomy; Am J Respir Crit Care Med. 2000;161:297-308 • Management of Spontaneous Pneumothorax; Chest. 2001;119:590-602 Suggested Reading • Cystic Fibrosis Pulmonary Guidelines Chronic Medications for Maintenance of Lung Health; Am J Respir Crit Care Med. 2007;176:957-969 • Cystic Fibrosis Pulmonary Guidelines Pulmonary Complications: Hemoptysis and Pneumothorax; Am J Respir Crit Care Med. 2010;182:298-306 Suggested Reading • Association Between Respiratory Tract Methicillin-Resistant Staphylococcus aureus and Survival in Cystic Fibrosis; JAMA. 2010;303(23):2386-2392 • Update on treatment of pulmonary exacerbations in cystic fibrosis; Current Opinion in Pulmonary Medicine. 2006;12:440-444 Suggested Reading • Guidelines for Evaluating Chronic Cough in Pediatrics: ACCP Evidenced-Based Clinical Practice Guidelines; Chest. 2006;129:260S283S • Recommendations for the assessment and management of cough in children; Thorax. 2008;63:1-15 Suggested Reading • UpToDate, April 2016. • Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis, Pediatrics. 2016;137(4):e20151784 • https://tools.cff.org/aboutcf/testing/newbor nscreening/screeningforcf/