Outpatient Management of Common Respiratory Problems in a NICU graduate

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1 Outpatient Management of Common Respiratory Problems in a NICU graduate Filomena Hazel Villa, MD Division of Pediatric Pulmonology University of Texas Health Sciences Center- San Antonio

2 I have no relevant financial relationships with the manufacturer of any commercial product and/or provider of commercial services discussed in this CME activity. I do not intend to discuss an unapproved or investigative use of a commercial product or device in my presentation

3 Objectives To review common respiratory problems of a NICU graduate To discuss outpatient interventions of respiratory disorders in infants with chronic lung disease of infancy To present outpatient diagnostic studies that will assist in the management of an infant with chronic lung disease

4 Definition Bronchopulmonary dysplasia (BPD)- pathologic process; infants still requiring oxygen (O2) at 28 th day of life. Chronic lung disease of infancy (CLDi) continued requirement of O2 at 36 weeks post menstrual age (PCA) CLDi- better predictor of clinical respiratory difficulty in the first year of life.

5 Definition NHLBI: infants <32 weeks Mild BPD treatment with O2 of >21% for at least 28 days, but in room air at 36 weeks PCA Moderate - O2 of >21% for at least 28 days; O2 <30% at 36 weeks PCA Severe- treatment with O2 >21% for at least 28 days and require >30% O2 or positive pressure ventilation at 36 weeks.

6 Definition Common to both BPD and CLDi: lung immaturity, injury from O2, mechanical ventilation, lung inflammation

7 Statistics Approximately 1 out of every 9 live births occurs prematurely. 90% of preterm infants survive. BPD/CLDi remains the most common complication of extreme prematurity Increased morbidity in the first 2 years of life.

8 Common respiratory disorders in the outpatient Signs and Symptoms Wheezing Apnea Increase work of breathing Desaturation Differential Diagnoses Airway reactivity; Bronchiolitis; Aspiration syndromes; Airway obstruction; Pulmonary edema Apnea of prematurity; ALTE; Aspiration; Airway obstruction Subglottic damage; Pneumonia; Aspiration syndromes; Pulmonary edema Pulmonary hypertension; Airway obstruction; Pulmonary edema

9 Airway Reactivity Episodic Dramatic clinical response to bronchodilator. Evidence of bronchodilator responsiveness seen in lung function testing: - Infant pulmonary function testing if available can be done in up to 2 years of age. - Impulse oscillometry for toddlers - Spirometry for >= 5 years old

10 Airway Reactivity Intervention: 1. Short acting beta agonist (SABA) PRN 2. +/- Inhaled corticosteroid ** Metered dose inhaler with mask spacer is as good or even better as nebulized form when correctly used. It is convenient and requires a lower dose. ** 1.25 to 2.5 mg nebulized Albuterol over minutes per dose versus 216 mcg to 432 mcg (2-4 puffs of HFA form) per dose over a span of 30 seconds

11 BRONCHIOLITIS Etiology- any respiratory virus. Respiratory syncytial virus (RSV) bronchiolitis potential severe complication in premature infants with CLDi. Diagnosis- History and physical examination. Intervention: Hydration/Nutrition Supplemental O2 or increase supplemental O2.

12 BRONCHIOLITIS Prevention: Palivizumab x 5 consecutive months during RSV season Recommendation: Administer to infants with Mild to Severe BPD (NHLBI definition) in the 1 st year of life. Administer to infants with Mild to Severe BPD IF on chronic steroid use, diuretic therapy or supplemental O2.

13 ASPIRATION SYNDROMES 1. Aspiration from above (swallowing dysfunction) 2. Aspiration from below (gastroesophageal reflux disease- GERD)

14 Swallowing Dysfunction In premature infants, disorganized sucking persists up to 34 weeks gestational age. Exclude other gross anatomic abnormalities. Coordination of sucking, swallowing and breathing - a complex process. Dysphagia is from immaturity of this process.

15 Swallowing dysfunction Intervention: 1. Referral to occupational/speech therapist for feeding strategy. 2. Video-fluoroscopic swallowing study. 3. Thickening of feeds. ** Risk of necrotizing enterocolitis on the use of xantham gum for <37 weeks to 44 weeks PCA.

16 GERD Clinically significant GER frequent vomiting, aspiration pneumonia, irritability; failure to thrive Silent aspiration worsens pulmonary status recurrent airway inflammation reflex bronchospasm. Prevalence is unknown.

17 GERD Diagnostic studies: Upper GI radiography ph probe monitoring, impedance Upper endoscopy

18 GERD Intervention Choose Wisely campaign: Avoid routine use of anti-reflux medications. 1. Reduce volume of feeding, increase frequency. 2. Upright or prone position (only when awake) 3. Acid suppressant: H2 antagonist or Proton pump inhibitor 4. Fundoplication

19 Airway obstruction 1. Dynamic airway obstruction Tracheomalacia Tracheobronchomalacia 2. Fixed airway obstruction Subglottic stenosis Tracheobronchial stenosis

20 Tracheomalacia/Tracheobronchomalacia Tracheomalacia is the most common congenital anomaly of the trachea. Primary (congenital)- faulty foregut division Secondary (acquired, more common)- degeneration of normal tracheal cartilages. More commonly seen in premature infants.

21 Tracheomalacia/Tracheobronchomalacia Result of prolonged intubation, recurrent infection, increased airway pressure. Premature infants have immature supporting structures. Frequently associated with cardiovascular abnormalities. 50% of infants with tracheomalacia have GERD.

22 Tracheomalacia/Tracheobronchomalacia Signs and symptoms: Wheezing not responding to SABA. Recurrent croup. Characteristic barky cough. Dying spells ; BPD spells - when patient is active, upset and more apparent with Valsalva maneuver. Impaired mucus clearance, inefficient cough mucus plugging atelectasis

23 Tracheomalacia/Tracheobronchomalacia Diagnostic studies: Fluoroscopy Chest CT Flexible Bronchoscopy dynamic airway evaluation

24

25 Tracheomalacia/Tracheobronchomalacia Intervention: Wait and see, feed and grow for mild cases. Use of Ipratropium bromide (Gallagher et al 2011 ) for moderate cases. Adjustable PEP (positive expiratory pressure) valve- improve efficiency of cough (Sirithangkul et al 2005) Surgery for severe cases: tracheostomy; aortopexy; external splinting, internal stent

26 Subglottic stenosis/tracheobronchial stenosis Acquired stenosis is more common than congenital form. History of prolonged or repeated endotracheal intubation. Subglottic stenosis occurs in 1.7 to 8% of neonates with history of intubation. Tracheobronchial stenosis from repeated mucosal injury from suction catheters.

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28 Subglottic/Tracheobronchial stenosis Diagnostic studies: CT scan (neck and chest) Rigid bronchoscopy Intervention: Tracheostomy Endoscopic dilation with steroid injection Anterior cricoid split Laryngotracheal reconstruction

29 Pulmonary Edema Factors contributing to lung edema in CLDi Increased capillary permeability resulting from lung injury Patent ductus arteriosus Fluid overload Commonly used in the NICU for the following reasons: Improve lung compliance. Reduce number of mechanical ventilation days. Furosemide up to 5 days. Thiazide- Spironolactone up to 42 days.

30 Pulmonary Edema Furosemide is used not more than 5 days. Nephrocalcinosis is a known complication. Chronic diuretic use: Thiazide- Spironolactone Cochrane review: Chronic use of thiazide-spironolactone significantly decreased mortality rate. Improvement of lung compliance at 4 weeks of treatment, but none thereafter. Reduced the use of Furosemide.

31 Pulmonary Edema Intervention: Treat underlying cardiac disease (refer to Cardiology) if hemodynamically unstable. Fluid restriction. Adjust dose for age, if still with recurrent signs and symptoms of pulmonary congestion. Consider giving acute doses of Furosemide if with exacerbations. No chronic diuretics in between.

32 Apnea of Prematurity - Occurs in less than 37 weeks PCA. Consider other diagnoses if patient s PCA is term - Secondary to immaturity of respiratory center. - With concomitant upper airway obstruction in most cases.

33 Apnea of Prematurity Diagnostic study: Polysomnography (PSG) determines type of apnea. - central, obstructive or mixed Titration of O2 and/or non-invasive positive pressure ventilation (NIPPV) during PSG

34 Apnea of Prematurity Intervention: Caffeine NIPPV in the form of either CPAP or BIPAP. Oxygen ** Choosing wisely campaign: Avoid routine use of pneumograms for pre-discharge assessment of ongoing apnea of prematurity.

35 Apparent Life Threatening Event (ALTE)/SIDS Patients with CLDi are at high risk for ALTE/SIDS. Deaths are likely from unrecognized hypoxemia. Study of Bancalari et al on premature infants weeks gestational, those given home O2 survived. Peripheral chemoreceptors function is abnormal in CLDi. Ventilatory and arousal responses to hypoxia are blunted.

36 Apparent Life Threatening Event/SIDS Diagnosis of ALTE is based on symptomatology. Diagnostic studies may be extensive to exclude primary etiology. Intervention: Depends on what system is involved. Back to sleep campaign

37 Pulmonary hypertension New BPD- reduced number of alveoli (alveolar simplification). Also reduced arteries and abnormal distribution in the lungs impaired gas exchange hypoxemia pulmonary vasoconstriction increase pulmonary vascular resistance pulmonary hypertension (PH)

38 Pulmonary hypertension Pulmonary hypertension is about 27-37% of infants with BPD. (An, 2010, Slaughter 2011) Diagnostic studies: Echocardiogram Cardiac catheterization

39 Pulmonary hypertension Intervention: Treat lung disease and other ongoing issues contributing to recurrent hypoxia. Treat underlying cardiac lesions if present. Pulmonary vasodilators: Oxygen Sildenafil Bosentan Calcium channel blockers

40 Pneumonia Most common etiology is viral. Premature infants follow a complicated course with RSV pneumonia. Streptococcus pneumoniae common bacteria in community acquired pneumonia.

41 Pneumonia Diagnostic studies: Check for Influenza/RSV from viral panel. CXR if with moderate to severe respiratory distress and persistent hypoxemia.

42 Pneumonia Intervention: Antibiotic (Amoxicillin) if stable to discharge home. Close follow up is important. Hospitalize if + increasing respiratory distress, poor feeding, lethargy and O2 saturation < 91%. Prevention: - Keep immunization up to date.

43 Weaning Home Oxygen If patient has documented pulmonary hypertension (PH) - DON T WEAN! No consensus on the best way to wean. Consider weaning from O2, if infant is about 12 months old and: keeping it in is not practical. is moving a lot during sleep and strangulation from the O2 tubing becomes a hazard.

44 Weaning Home Oxygen Weaning criteria (Fitzgerald, 2012) No increase work of breathing No evidence of pulmonary hypertension. Target minimum O2 saturation (SPO2) to at least 93-95%. Adequate weight gain.

45 Weaning Home Oxygen ATS Statement 2002 : If patient is thriving start weaning at daytime if SPO2 is adequate wean night time O2 if SPO2 adequate Completely wean O2. Another option : PSG before weaning night time O2.

46 Traveling by air Aircraft cabins are pressurized up to 8000ft (equivalent FIO2 of 15%) High Altitude Simulation Test (HAST) If HAST is not accessible, increase O2 by 0.25LPM if flight is > 1 hour.

47 REFERENCES 1. American Academy of Pediatrics. Clinical Practice Guideline: Diagnosis, Management, and Prevention of Bronchiolitis. Pediatrics 2014; 134 (5): e1474 e American Academy of Pediatrics. Updated guidance for Palivizumab prophylaxis among infants and young children at increased risk of hospitalization for respiratory syncytial virus infection. Pediatrics 2014; 134 (2): American Thoracic Society Documents. Statement on the care of the child with chronic lung disease of infancy and childhood. Am J Respir Care Med 2003; 168: Fitzgerald DA, Massie RJH, Nixon G, Jaffe A, Wilson A, Landau L, Twiss J, Smith G, Wainwright C, Harris M. Infants with chronic neonatal lung disease: recommendations for the use of home oxygen therapy. M J Aust 2008 Nov 17; 189(10): Carden KA, Boiselle PM, Waltz DA, Ernst A. Tracheomalacia and tracheobronchomalacia in children and adults. An in depth review. Chest March 2005; 127 (3); Gallagher T, Maturo S, Fracchia S, Hartnick C. An analysis of children with tracheomalacia treated with Ipratropium bromide (Atrovent). The Laryngoscope 01/2011; 121(S4). DOI: /lary Sirithangkul S, Ranganathan S, Robinson P, Robertson CF. Positive expiratory pressure to enhance cough effectiveness in tracheomalacia. J Med Assoc Thai Nov;93 Suppl 6:S Randel A. Practice Guideline: AAP releases guideline for the management of gastroesophageal reflux in children. Am Fam Physician Mar 1;89(5): Goldfield EC, Buonomo C, Fletcher K, Perez J, Margetts S, Hansen A, Smith V, Ringer V, Richardson MJ, Wolff PH. Premature infants swallowing: Patterns of tongue-soft palate coordination based on videofluoroscopy. Infant Behav. Dev 2010 April; 33 (2): A Stewart, LP Brion, I Ambrosio-Perez. Diuretics on the distal renal tubule for preterm infants with (or developing) chronic lung disease (Review). The Cochrane Collaboration Issue Linda Y Fu, Rachel Y Moon. Apparent life threatening events: An update. Pediatrics in Review 2012; 33; Slaughter JL, Stenger MR, Reagan PB. Variation in the use of diuretic therapy for infants with bronchopulmonary dysplasia. Pediatrics Apr 2013; 131(4): An, HS, Bae, EJ, Kim, GB. Pulmonary hypertension in preterm infants with bronchopulmonary dysplasia. Korean Circ J. 2010; 40(3): Slaughter, JR, Pakrashi, T, Jones, DE. South, AP, Shah, TA. Echocardiographic detection of pulmonary hypertension in extremely low birth weight infants with bronchopulmonary dysplasia requiring prolonged positive pressure ventilation. J. Perinatol 2011 Feb 10 (Epub). 14. Stokowski LA, Ho T. Just don t do it (routinely). Choose wisely when caring for newborns. Choosing Wisely Campaign August American Thoracic Society Documents. An official American Thoracic Society workshop report: Optimal lung function tests for monitoring cystic fibrosis, bronchopulmonary dysplasia, and recurrent wheezing in children less than 6 years of age. Ann Am Thorac Soc Apr 2013; 10 (2):S1- S11 Apr Slaughter JL, Stenger MR, Reagan PB. Variation in the use of diuretic therapy for infants with bronchopulmonary dysplasia. Pediatrics Apr 2013; 131(4):

48 Thank you.

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