Wheeze, Stridor, Cough, and Other Respiratory Noises Miles Weinberger, M.D. Professor of Pediatrics www.uihealthcare.com/allerpulm Wheeze Continuous musical sounds, typically expiratory in nature But What Do Parents Mean by “Wheeze” ¾Less than 50% agreement between parents and clinicians (Cane et al. Arch Dis Child 2000;82:327) ¾Wheeze most commonly used term for respiratory sounds until imitated or shown video (Elphick et al. Arch Dis Child 2001;84:35) ¾30% of parents used other words for wheeze and 30% labeled other sounds as “wheeze” (Cane et al. Arch Dis Child 2001;84) Causes of Wheezing ¾Bronchiolitis ¾Asthma ¾Cystic fibrosis ¾Foreign body (monophonic) ¾Bronchomalacia Causes of Wheezing ¾Bronchiolitis ¾Asthma ¾Cystic fibrosis ¾Foreign body (monophonic) ¾Bronchomalacia Patient GH ¾5 m.o. with wheezing and retractions ¾Onset 6 weeks of age ¾No response to albuterol Patient GH Patient GH Patient GH ¾BAL from RUL n 52% neutrophils n 400,000 cfu/ml Moraxella catarrhalis n 400,000 cfu/ml Haemophilus influenzae ¾Coughing and wheezing resolved with Augmentin ES600, 90 mg/kg/day divided BID ¾Stridor and retractions when excited persisted from the mild laryngomalacia Bronchomalacia Bronchomalacia Noises and Clinical Consequences Associated with Bronchomalcia Kompare M, Weinberger M. Protracted bacterial bronchitis in young children: association with airway malacia. J Pediatr 2012;160:88-92). ¾ Protracted bacterial bronchitis – most with bronchomalacia ¾ BAL for persistent presence of cough, wheeze, or noisy breathing n Inflammation (neutrophilia) n High colony counts bacteria – S. pneumo – H. influenza – M. catarrhalis ¾ Improved with Augmentin, but recurrences common AirwayAirway MalaciaMalacia Bronchomalacia Tracheomalacia 26%26% Both 41%41% None seen 13%13% 20%20% Stridor Continuous inspiratory musical sound of variable pitch Laryngomalacia ¾Common to varying degrees – about 60% of stridor in infants ¾Represents a delay in maturation of supporting laryngeal structures. ¾Generally resolves by 2 years of age ¾Rare cases result in pulmonary hypertension ¾Laser laryngoplasty for very occasional severe cases affecting feeding or growth What is “croup”? ¾ Transient inflammation of the laryngeal area causing upper airway obstruction characterized by inspiratory stridor - generally from viruses ¾ Commonly associated with inflammation of the trachea and bronchi, hence the term “laryngotracheobronchitis” ¾ Typically accompanied by harsh barking cough from the tracheobronchitis – but a “croupy” cough alone is not croup ¾ Responds to epinephrine aerosol with transient relief and dexamethasone for sustatined benefit Etiologic Agents Recovered from Children with Croup N = 360 M Pneumoniae Influenza A 4% Paraflu 2 Influenza B Paraflu 3 4% Misc. vir. 9% 3% 18% 5% Paraflu 1 47% RSV 10% Denny et al, Pediatr 71:871, 1983 Epiglottitis ¾Bacterial infection n H. influenza group B n S. pyogenes (beta hemolytic streptococcus, group A) ¾Life threatening ¾Medical emergency ¾Treatment n Establish an airway n Antibiotics Jennifer J - 15 Y.O. Girl With 3 Weeks of recurrent stridor and dyspnea ¾First episode while detasseling - Rx with epinephrine in ER successful ¾2nd episode next day while detasseling - same treatment with same result ¾Then daily episodes without detasseling and without response to epinephrine ¾Respiratory sound called wheezing ¾Rx with inhaled bronchodilators, prednisone, and hospitalization without response Jennifer J - Spirometry Before and After Treadmill Exercise ¾Treadmill exercise induced “wheezing” (actually a high pitched stridor) ¾Normal pre-exercise; decreased flow only on inspiration post- post-exercise exercise ¾Diagnostic of pre-exercise extrathoracic airway obstruction Sarah J - 15 Y.O. Girl With 1.5 Year History Recurrent Severe “Wheezing” and Dyspnea ¾ Multiple paramedic calls for ER Rx because of severe respiratory distress ¾ Rx with bronchodilators, inhaled and oral corticosteroids ¾ Several hospitalizations ¾ No consistent response to any treatment Sarah J - spirometry before and after preparing to perform bronchoprovocation ¾ “Wheezing” (actually a high before pitched inspiratory stridor and a monophonic expiratory wheeze) and dyspnea began while preparing to perform bronchoprovocation after ¾ Initially normal; then severe airflow obstruction apparent on both inspiration and expiration Vocal Cord Dysfunction Syndrome ¾Commonly mis-diagnosed as asthma ¾May be present along with asthma ¾Two clinical patterns (Doshi D. Weinberger M. Long-term outcome of vocal cord dysfunction. Ann Allergy Asthma Immunol 2006;96;794-799) n Exercise induced (can be blocked with anti-cholinergic aerosol) n Spontaneous ¾Treatment of spontaneous VCD – vocal cord training by a speech therapist familiar with the disorder History of D.D. ¾17 m.o. girl with 8 months of harsh nocturnal stridor and coughing ¾Rx with albuterol and corticosteroids ¾No response to treatment ¾Flexible bronchoscopy showed intermittent paradoxical vocal cord movement MRI of D.D. Chiari 1 malformation with herniation of cerebellar tonsil below foramen magnum Causes of Stridor ¾Croup ¾Epiglottitis ¾Laryngomalacia ¾Chiari malformation ¾Vocal cord paralysis ¾Laryngeal foreign body ¾Vocal cord dysfunction syndrome Cough Forceful exhalation after quickly raising thoracoabdominal pressure, often to > 100 cm H20 Pertussis Whooping Cough ¾Most dangerous in unimmunized infants ¾Occurs in immunized adolescents and adults n 26% of 130 college students with persistent cough > 6 days (Mink et al, Clin infect Dis 1992;14:464-71) n 21% of 75 adults with cough > 2 weeks (Wright et al, JAMA 1994;273:1044-6) n 12% of 153 adults with cough > 2 weeks (Nennig et al, JAMA 1996;275:1672-4) Nasal Swab for Pertussis PCR Habit Cough Syndrome Clinical Characteristics ¾Loud, repetitive, dry, barking cough ¾Duration of weeks or months ¾Irritating and disturbing to others - prevents school attendance ¾Commonly misdiagnosed as asthma ¾No response to medication ¾Absent once asleep Habit Cough Rx With Suggestion Therapy - Outcome Lockshin, Lindgren, Weinberger, Koviach. Ann Allergy 1991;67:405 (from U of IA Ped All/Pulm Clinic) ¾8/9 patients treated successfully with 15 minute session, 1/9 required 30 minutes ¾Minor relapses controlled with autosuggestion technique ¾1 required a 2nd session 9 days later ¾All remained asymptomatic for median of 2.2 years Habit Cough Syndrome Natural History Rojas, Sachs, Yunginger, O’Connell. Annals of Allergy 1991;66:106 (from the Mayo Clinic) ¾Follow-up of 62 pts (34 m & 26 f) for mean length of 7.9 years ¾Ages 4.6-15.6 (mean 10.5) ¾Mean duration till Dx - 7.6 months ¾Mean duration till resolution 6.1 months ¾16 pts still coughing for mean of 5.9 years from time of Dx Habit Cough Syndrome Rx With Suggestion Therapy - Method ¾Physician must have confidence in success ¾Focus patient’s attention on suppressing cough ¾Explain cough ¾Reinforce even brief successes ¾Emphasize that patient, not physician, is controlling cough ¾Success usual in about 15 minutes ¾Emphasize autosuggestion for minor relapses Donna E - 9 Y.O. Girl With Cough for 2 Years ¾Treated as asthma ¾Oral corticosteroids n Growth retarded n Cushingoid n Hirsute ¾Several hospitalizations with IV antibiotics and corticosteroids ¾No response to any of the therapy Patient MW ¾14 m.o. coughing day and night ¾4-5 spasms of coughing a day lasting 4-5 minutes ¾Interferes with activity ¾Coughing disturbs sleep, especially when lying on her back Patient MW Patient MW Patient MW ¾No inflammation on BAL ¾Referred to pediatric surgery ¾Aortopexy performed Tracheomalacia - treatment ¾Behaviorally – successful for some mild cases (Wood RE. Localized tracheomalacia or bronchomalacia in children with intractable cough. J Pediatr 1990;116:404-406) ¾Aortopexy – stenting the trachea using a stitch between the sternum and the aorta to place tension on the connective tissue between the aorta and anterior wall of the trachea Aortopexy Patient MW ¾Cough now not troublesome ¾She now sleeps well at night on her back History of A.K. ¾ 4 y.o. with harsh barking cough ¾ Present since infancy ¾ Dx as asthma ¾ Allergy skin testing negative ¾ Rx with cromolyn, Singulair, prn albuterol, and multiple courses of steroids ¾ Equivocal response to all treatment Upper Airway of A.K. Through Flexible Bronchoscope Najada A, Weinberger M. Unusual cause of chronic cough in a four-year-old cured by uvulectomy. Pediatr Pulmonol 2002;34;144-146. 3.y.o. girl with chronic cough Gurgel RK, Brookes JT, Weinberger MM, Smith RJ. Chronic cough and tonsillar hypertrophy. Ped Pulmonol 2008;43:1147–1149. epiglottis ¾ No response to bronchodilators ¾ No response to prednisolone ¾ No response to antibiotic ¾ No airway inflammation on BAL ¾ No bacteria on BAL Tonsillar tissue Other Causes of Cough ¾Acute viral bronchitis (or tracheobronchitis) ¾Asthma (1215 tomorrow) ¾Protracted bacterial bronchitis ¾M. Or C. Pneumoniae ¾Aspiration ¾Cystic fibrosis ¾Primary ciliary dyskinesia ¾Habit cough syndrome (Saturday at 1300) What about GERD and Cough??? Chang et al. Cough and reflux esophagitis in children: their co-existence and airway cellularity. BMC Pediatr 2006 Feb 27;6:4. Chang et al. An objective study of acid reflux and cough in children using an ambulatory pHmetry–cough logger. Arch Dis Child. 2011 May;96(5):468-72. Chang et al. Gastro-oesophageal reflux treatment for prolonged non-specific cough in children and adults. Cochrane Database Syst Rev. 2011 Jan 19;(1) Controlled clinical tirals have not shown PPIs to improve cough associated with GERD symptoms in