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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

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Outpatient Management of

Common Respiratory

Problems in a NICU graduateFilomena Hazel Villa, MD

Division of Pediatric Pulmonology

University of Texas Health Sciences Center- San Antonio

• 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

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

Definition

• Bronchopulmonary dysplasia (BPD)- pathologic process; infants still

requiring oxygen (O2) at 28th 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.

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.

Definition

• Common to both BPD and CLDi: lung immaturity, injury from O2,

mechanical ventilation, lung inflammation

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.

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

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

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 10-15 minutes per dose versus 216 mcg

to 432 mcg (2-4 puffs of HFA form) per dose over a span of 30 seconds

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.

BRONCHIOLITIS

• Prevention: Palivizumab x 5 consecutive months during RSV season

• Recommendation:

• Administer to infants with Mild to Severe BPD (NHLBI definition) in the

1st year of life.

• Administer to infants with Mild to Severe BPD IF on chronic steroid use,

diuretic therapy or supplemental O2.

ASPIRATION SYNDROMES

1. Aspiration from above (swallowing dysfunction)

2. Aspiration from below (gastroesophageal reflux disease- GERD)

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.

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.

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.

GERD

Diagnostic studies:

• Upper GI radiography

• pH probe monitoring, impedance

• Upper endoscopy

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

Airway obstruction

• 1. Dynamic airway obstruction

• Tracheomalacia

• Tracheobronchomalacia

• 2. Fixed airway obstruction

• Subglottic stenosis

• Tracheobronchial stenosis

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.

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.

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

pluggingatelectasis

Tracheomalacia/Tracheobronchomalacia

Diagnostic studies:

• Fluoroscopy

• Chest CT

• Flexible Bronchoscopy – dynamic airway evaluation

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

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.

Subglottic/Tracheobronchial stenosis

• Diagnostic studies:

• CT scan (neck and chest)

• Rigid bronchoscopy

• Intervention:

• Tracheostomy

• Endoscopic dilation with steroid injection

• Anterior cricoid split

• Laryngotracheal reconstruction

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.

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.

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.

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.

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

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.”

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 26-33 weeks gestational,

those given home O2 survived.

• Peripheral chemoreceptors function is abnormal in CLDi. Ventilatory

and arousal responses to hypoxia are blunted.

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”

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)

Pulmonary hypertension

• Pulmonary hypertension is about 27-37% of infants with BPD. (An,

2010, Slaughter 2011)

• Diagnostic studies:

• Echocardiogram

• Cardiac catheterization

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

Pneumonia

• Most common etiology is viral.

• Premature infants follow a complicated course with RSV pneumonia.

• Streptococcus pneumoniae –common bacteria in community acquired

pneumonia.

Pneumonia

• Diagnostic studies:

• Check for Influenza/RSV from viral panel.

• CXR if with moderate to severe respiratory distress and persistent

hypoxemia.

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.

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.

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.

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.

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.

REFERENCES

1. American Academy of Pediatrics. Clinical Practice Guideline: Diagnosis, Management, and

Prevention of Bronchiolitis. Pediatrics 2014; 134 (5): e1474 – e1500.

2. 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): 416-420.

3. 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: 356-396.

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):578-82.

4. Carden KA, Boiselle PM, Waltz DA, Ernst A. Tracheomalacia and tracheobronchomalacia in children and adults. An in depth review. Chest March 2005; 127 (3); 984-1005.

5. 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: 10.1002/lary.22097

6. Sirithangkul S, Ranganathan S, Robinson P, Robertson CF. Positive expiratory pressure to enhance cough effectiveness in tracheomalacia. J Med Assoc Thai. 2010 Nov;93 Suppl 6:S112-8.

7. Randel A. Practice Guideline: AAP releases guideline for the management of gastroesophageal reflux in children. Am Fam Physician. 2014 Mar 1;89(5):395-7.

8. 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): 209-218.

9. 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. 2011 Issue 9.

10. Linda Y Fu, Rachel Y Moon. Apparent life threatening events: An update. Pediatrics in Review 2012; 33;361.

11. Slaughter JL, Stenger MR, Reagan PB. Variation in the use of diuretic therapy for infants with bronchopulmonary dysplasia. Pediatrics Apr 2013; 131(4): 716-723.

12. An, HS, Bae, EJ, Kim, GB. Pulmonary hypertension in preterm infants with bronchopulmonary dysplasia. Korean Circ J. 2010; 40(3): 131-136.

13. 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 2011.

15. 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 2013.

16. Slaughter JL, Stenger MR, Reagan PB. Variation in the use of diuretic therapy for infants with bronchopulmonary dysplasia. Pediatrics Apr 2013; 131(4): 716-723.

Thank you.