Airway Clearance in Children Youth and Adolescents – Does

The Device Really Matter?

Chris Landon MD FAAP,FCCP,CMDChris Landon MD FAAP,FCCP,CMDDirector of Pediatrics Ventura County Medical CenterDirector of Pediatrics Ventura County Medical CenterPediatric Pulmonary Center Director Mid CoastPediatric Pulmonary Center Director Mid CoastClinical Associate Professor of Pediatrics USC School of MedicineClinical Associate Professor of Pediatrics USC School of MedicinePediatric Pulmonary Department ChildrenPediatric Pulmonary Department Children’’s Hospital Los Angeless Hospital Los Angeles

DisclaimerDisclaimer

Scientific Advisory BoardsScientific Advisory Boards• Hill-RomHill-Rom

Objectives• I. Review of the rationale for airway clearance

therapy and basic principles

• II. Review the evidence for efficacy of airway clearance therapy in pediatrics

• III. Minimal to no benefit in the treatment of children with acute asthma, bronchiolitis, hyaline membrane disease, and those on mechanical ventilation for respiratory failure in the pediatric intensive care unit, and it is not effective in preventing atelectasis in children immediately following surgery.

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Neuromuscular Diseases: Overview

Children who experience varying degrees of neurological/neuromuscular dysfunction

Diagnoses include: cerebral palsy, muscular dystrophy, spinal muscular atrophy, brain injury, consequences of infectious disease, inherited metabolic disorders, etc.– One child in 1000 is institutionalized as a result

of profound disability

Neuromuscular Diseases: Overview

NeuromuscularGastroesophagealImmune systemRespiratoryPsychosocial

Multi-system assessment necessary to determine risk of pulmonary involvement:

Neuromuscular Diseases

• Neuro assessment – Oral motor weakness

– Muscular dystrophies

– Myopathies

– Neuromuscular junction disorders

– Anterior horn cell disorders

Typical symptoms

– Too weak to swallow

– Too weak to cough

– Easily fatigued

– Head position dependent

Assessment of complications that predispose to pulmonary involvement

Neuromuscular Diseases

• Neuro assessment – Increased secretions– Autonomic dysfunction

– Medication effects

– Frequent seizures

Typical symptoms

– Constant drooling

– Worse with stress or infection

– Drowning in drool

Assessment of complications that predispose to pulmonary involvement

Oral Motor Weakness

MyopathiesMuscular dystrophiesNeuromuscular junction disordersAnterior horn cell disorders

Typical symptoms– Too weak to swallow– Too weak to cough– Easily fatigued– Head position dependent

Central Neurogenic Hypoventilation

Diffuse cortical damagePoor hypoxic responsePoor hypercarbic responseWorse with stress or infection

Thoracic Weakness

• Myopathies

• Muscular dystrophies

• Neuromuscular junction disorders

• Anterior horn cell disorders

Gastroesophageal Function and Complications

The Upper Airway-Swallowing and Aspiration

Aspiration Associated Pneumonias

Lower Esophageal Aspiration, Gastric Distention and Airway Remodeling

Gastroesophageal Reflux Disease (GERD)

Fundoplication Versus Medication and Airway Clearance

Nutrition and the Immune System

The Faces of Dysfunction

Arching

Irritability

Regurgitation

Gagging and Choking Refusing Feedings

Failure To Thrive

The Immune System

Genetic Abnormalities

Nutritional Compromise of the Immune System

Stress and Immune Response

Recurrent Infection and Frequent Use of Antibiotics: The Impacts

Allergies Reactive Airway

Disease (RAD) Airway Clearance

Therapy

Respiratory Medical History

– Number of Pulmonary Infections Annually

– Number of Hospital Admissions Annually

– Number of ER Admissions Annually

– Number of Courses of Antibiotics for Respiratory Infections Annually

– Immunization History

– History of Recurrent Infections with Respiratory Syncytial Virus (RSV)

High Risk For Post-Operative Complications

Atelectasis Pneumonia Respiratory Failure Need for prolonged

ventilation Tracheostomy Death

Problems

Weak cough Dyscoordinated swallow Aspiration Difficulty clearing secretions Increased lower respiratory tract

infections

Respiratory Weakness

May not be apparent on physical exam Respiratory failure when work of

breathing is increased

Chronic Respiratory Muscle Weakness

Reduced lung volumes Microatelectasis V/Q mismatch Scoliosis Decreased compliance of the chest wall Decreased pulmonary compliance Hypoxemia only during sleep Hypoventilation due to muscle weakness Hypoventilation due to central hypoventilation

Thorough History

Frequency and severity of respiratory tract infections

Pulmonary complications of previous surgeries

History suggestive of reactive airways disease– Even mildly increased airway obstruction

may lead to respiratory failure in the postoperative period in a patient with severe respiratory muscle weakness

Physical Examination

Gag reflexCoughAdequacy of aerationPresence of adventitial lung

sounds

Ability To Cooperate With Post-Operative Pulmonary Therapy

General muscle strength Physical and intellectual capacity

Laboratory Examinations

Chest x-rayArterial blood gases or mixed

venous gas measurements and oximetry

Complete blood count

Pulmonary Function Tests

All children who are capable of performing them– Lung volumes– Pre and post bronchodilator– Maximal inspiratory and expiratory mouth

pressures• frequently decreased more than lung

volumes and flows• do not correlate with general muscle

strength

Impaired Airway Clearance: Factors

• Ineffective mucociliary clearanceIneffective mucociliary clearance

• Excessive secretionsExcessive secretions

• Thick secretionsThick secretions

• Ineffective coughIneffective cough

• Restrictive lung diseaseRestrictive lung disease

• Immobility / inadequate exerciseImmobility / inadequate exercise

• Dysphagia / aspiration / gastroesophageal Dysphagia / aspiration / gastroesophageal refluxreflux

Results of Impaired Airway Clearance

• Airway obstructionAirway obstruction

• Mucus pluggingMucus plugging

• AtelectasisAtelectasis

• Impaired gas exchangeImpaired gas exchange

• InfectionInfection

• InflammationInflammation

Disease States with Compromised Airway Clearance

• Primary Ciliary DyskinesiaPrimary Ciliary Dyskinesia

• Neuromuscular DiseaseNeuromuscular Disease– Predisposes to respiratory failurePredisposes to respiratory failure– Distinct risk factor for morbidity and mortalityDistinct risk factor for morbidity and mortality

• Severe neurologic insultsSevere neurologic insults

• Cystic FibrosisCystic Fibrosis

• BronchiectasisBronchiectasis No proven benefit for airway clearance therapy in pneumonia, No proven benefit for airway clearance therapy in pneumonia,

asthma not complicated by atelectasis, bronchiolitisasthma not complicated by atelectasis, bronchiolitis

Airway Clearance Devices

• CPT for infantsCPT for infants

• PEP ValvePEP Valve

• FlutterFlutter

• AcapellaAcapella

• Cough AssistCough Assist

• The Vest SystemThe Vest System

Chest Physiotherapy for Infants

• No definitive data to support use in No definitive data to support use in asymptomatic CF infant.asymptomatic CF infant.

• Most likely age group to have adverse Most likely age group to have adverse effects, especially GER effects, especially GER ++ aspiration. aspiration.

• Must modify postural drainage to minimize Must modify postural drainage to minimize side effectsside effects

• Significant time commitment for families and Significant time commitment for families and Healthcare teamsHealthcare teams

PEP Valve

• Positive Expiratory PressurePositive Expiratory Pressure

• Action: Splints airways during exhalationAction: Splints airways during exhalation

• Can be used with aerosolized medicationsCan be used with aerosolized medications

• Technique dependentTechnique dependent

• PortablePortable

• Time required: 10-15 minutesTime required: 10-15 minutes

Flutter

• Action: Loosens mucus through expiratory Action: Loosens mucus through expiratory oscillation; positive expiratory pressure splints oscillation; positive expiratory pressure splints airways.airways.

• Used independentlyUsed independently• Technique dependent – has to be held at a Technique dependent – has to be held at a

precise angle to maximize oscillationprecise angle to maximize oscillation• PortablePortable• May not be effective at low airflowsMay not be effective at low airflows• Time required: 10-15 minutesTime required: 10-15 minutes

Acapella

• Combines benefits of PEP and airway Combines benefits of PEP and airway vibrations to mobilize secretionsvibrations to mobilize secretions

• Similar to flutter except has a valve-Similar to flutter except has a valve-magnet device to interrupt expiratory flow magnet device to interrupt expiratory flow and thus can be used at any angle.and thus can be used at any angle.

Contraindications for PEP, Acapella and Flutter

• PneumothoraxPneumothorax• Perforated ear drumPerforated ear drum• HemoptysisHemoptysis• Post-operative lung surgery as may lead to air Post-operative lung surgery as may lead to air

leak or if fresh transplant may break down leak or if fresh transplant may break down anastomoses siteanastomoses site

• Severe cardiac diseaseSevere cardiac disease• Esophageal varicesEsophageal varices• Pulmonary embolusPulmonary embolus

Cough Assist

• Action: Creates mechanical Action: Creates mechanical ““coughcough”” through the use through the use of high flows at positive and negative pressures.of high flows at positive and negative pressures.

• Positive / negative pressures up to 60cm of waterPositive / negative pressures up to 60cm of water• Used independently or with caregiver assistanceUsed independently or with caregiver assistance• Technique independentTechnique independent• PortablePortable• Primary use in muscular weaknessPrimary use in muscular weakness

Airway Clearance Vest Systems(High Frequency Chest Wall Oscillation)

High Frequency Chest Wall Oscillating Devices

• Action: Uses pulses of air pressure Action: Uses pulses of air pressure applied to the chest wall to produce applied to the chest wall to produce shearing at the air-mucus interface and shearing at the air-mucus interface and compression causes repetitive peak compression causes repetitive peak expiratory flows to expel mucus like small expiratory flows to expel mucus like small coughscoughs

• The chest wall is only compressedThe chest wall is only compressed

• The air in the airways only oscillatesThe air in the airways only oscillates

Contraindications to Vest Therapy

• Head and/or neck injury which has not Head and/or neck injury which has not been stabilizedbeen stabilized

• Active pulmonary hemorrhageActive pulmonary hemorrhage

• Hemodynamic instabilityHemodynamic instability

Clear secretions effectively and consistentlyClear secretions effectively and consistently Preserve lung functionPreserve lung function Reduce infectious exacerbationsReduce infectious exacerbations Reduce dependence on antibiotic therapy and Reduce dependence on antibiotic therapy and

other medicationsother medications Reduce need for hospitalization and auxiliary Reduce need for hospitalization and auxiliary

medical servicesmedical services Delay disease progressionDelay disease progression Reduce the burden of careReduce the burden of care Enhance the quality of lifeEnhance the quality of life

Quality Airway Clearance Therapy Should

Adverse Effects – Airway Clearance

• Oxygen desaturationOxygen desaturation• Gastroesophageal refluxGastroesophageal reflux• AspirationAspiration• HyperventilationHyperventilation• Airway obstruction from mobilized secretionsAirway obstruction from mobilized secretions• BarotraumaBarotrauma• Pain and discomfortPain and discomfort• Guilt from lack of adherenceGuilt from lack of adherence

Therapy Adjuncts

• AntibioticsAntibiotics

• BronchodilatorsBronchodilators

• Anti-inflammatory drugsAnti-inflammatory drugs

• MucolyticsMucolytics

• NutritionNutrition

HFCWO Clearance System CaseHFCWO Clearance System Case5 Year Old Girl With Spinal 5 Year Old Girl With Spinal

Muscular Atrophy Second PICU Muscular Atrophy Second PICU Hospitalization in Two Months Hospitalization in Two Months

History Of IllnessHistory Of Illness

• 5 year old girl with Spinal Muscular Atrophy5 year old girl with Spinal Muscular Atrophy• Fever and oxygen saturations in the high 80Fever and oxygen saturations in the high 80’’ss• Discharged from PICU two weeks priorDischarged from PICU two weeks prior• Poor oral intakePoor oral intake

Clinical FindingsClinical Findings

• X-ray at PMDX-ray at PMD’’s office showed RUL Pneumonias office showed RUL Pneumonia• Transferred by PMD to Santa Barbara PICU by Transferred by PMD to Santa Barbara PICU by

ambulance from the officeambulance from the office• Temperature 38.7 Pulse 158 BP 105/53Temperature 38.7 Pulse 158 BP 105/53• Respiratory Rate 29 Respiratory Rate 29 • Chest clear to auscultation bilaterally. No Chest clear to auscultation bilaterally. No

audible wheezingaudible wheezing• Neuro – Alert and cooperative, extremely thin Neuro – Alert and cooperative, extremely thin

with muscular atrophywith muscular atrophy

Treatment IssuesTreatment Issues• Social stressors of divorcing parentsSocial stressors of divorcing parents• Parents not recognizing that patient is having difficulties Parents not recognizing that patient is having difficulties

with eatingwith eating• Last authorized Pediatric Pulmonary visit 1/23/05 VEST Last authorized Pediatric Pulmonary visit 1/23/05 VEST

prescribed prescribed • Despite multiple attempts to fit VEST, patient reported to Despite multiple attempts to fit VEST, patient reported to

mother it was uncomfortable. Mother blamed father for mother it was uncomfortable. Mother blamed father for imposing it. Parents feel uncomfortable with CPT due to imposing it. Parents feel uncomfortable with CPT due to patients reported discomfortpatients reported discomfort

• Unable to generate sufficient flow for Flutter and AcapellaUnable to generate sufficient flow for Flutter and Acapella• Patient has nebulizer for albuterol and uses 1992 Patient has nebulizer for albuterol and uses 1992

Coffalator left by DME company Coffalator left by DME company

Exam Date:Exam Date: 1/19/2006 13:55 1/19/2006 13:55 HoursHours

PROCEDURE: X-RAY OF CHEST PROCEDURE: X-RAY OF CHEST ONE VIEW, PORTABLEONE VIEW, PORTABLE

COMPARISON:12/3/05.COMPARISON:12/3/05.INDICATIONS: Fever, cough.INDICATIONS: Fever, cough.FINDINGS: When compared with FINDINGS: When compared with

the previous study there is now the previous study there is now an area of opacity in the right an area of opacity in the right upper lobe.upper lobe.

CONCLUSION: Right upper lobe CONCLUSION: Right upper lobe pneumonia.pneumonia.

Medical Interventions at Time of Medical Interventions at Time of PresentationPresentation

• BiPap with supplemental oxygen for sleeping BiPap with supplemental oxygen for sleeping and nappingand napping

• Cefuroxime and AzithromycinCefuroxime and Azithromycin• Patient receives albuterol and CPT to upper lobePatient receives albuterol and CPT to upper lobe

PROCEDURE:PROCEDURE: X-RAY OF CHEST X-RAY OF CHEST ONE VIEW, PORTABLEONE VIEW, PORTABLE

COMPARISON:COMPARISON: 1/20/061/20/06INDICATIONSINDICATIONS PneumoniaPneumoniaFINDINGS: There is rotation to the right. FINDINGS: There is rotation to the right.

There is increased opacity in the right There is increased opacity in the right hemithorax. There is evidence of shift hemithorax. There is evidence of shift of the mediastinal contents to the right of the mediastinal contents to the right but this is accentuated due to rotation. but this is accentuated due to rotation. The relatively rapid increase in opacity The relatively rapid increase in opacity is suspicious for atelectasis. The left is suspicious for atelectasis. The left lung remains clear.lung remains clear.

CONCLUSIQN~ Marked increase in CONCLUSIQN~ Marked increase in parenchymal opacity in right parenchymal opacity in right hemithorax with evidence for volume hemithorax with evidence for volume probably representing lobar probably representing lobar atelectasis.atelectasis.

Medical Interventions at Time of Medical Interventions at Time of PresentationPresentation

• BiPap with supplemental oxygen for BiPap with supplemental oxygen for sleeping and nappingsleeping and napping

• Cefuroxime and AzithromycinCefuroxime and Azithromycin

• Changed to cefepime on Day 3Changed to cefepime on Day 3

• Pediatric Hospitalist reviews care and X-Pediatric Hospitalist reviews care and X-ray reports by phone with Pediatric ray reports by phone with Pediatric PulmonologistPulmonologist

• Coffalator brought from homeCoffalator brought from home

Exam Date: 1/21/2006 9:41 HoursExam Date: 1/21/2006 9:41 HoursCOMPARISON; 1/20/06COMPARISON; 1/20/06INDICATIONS: PneumoniaINDICATIONS: PneumoniaFINDINGS: There is rotation to the right. FINDINGS: There is rotation to the right.

There is increased opacity in the right There is increased opacity in the right hemithorax. There is evidence of shift of hemithorax. There is evidence of shift of the mediastinal contents to the right but the mediastinal contents to the right but this is accentuated due to rotation. The this is accentuated due to rotation. The relatively rapid increase in opacity is relatively rapid increase in opacity is suspicious for atelectasis. The left lung suspicious for atelectasis. The left lung remains clear.remains clear.

CONCLUSION: Marked increase in CONCLUSION: Marked increase in parenchymal opacity in right hemithorax parenchymal opacity in right hemithorax with evidence for volume loss, probably with evidence for volume loss, probably representing lobar atelectasis.representing lobar atelectasis.

Medical Interventions at Time of Medical Interventions at Time of PresentationPresentation

• BiPap with supplemental oxygen for sleeping BiPap with supplemental oxygen for sleeping and nappingand napping

• Cefuroxime and AzithromycinCefuroxime and Azithromycin• Changed to cefepime on Day 3Changed to cefepime on Day 3• Coffalator brought from homeCoffalator brought from home• Custom VEST brought from home and used in Custom VEST brought from home and used in

conjunction with Coffalatorconjunction with Coffalator

PROCEDURE: X-RAY OP CHEST PROCEDURE: X-RAY OP CHEST ONE VIEW, PORTABLE 1/25/06ONE VIEW, PORTABLE 1/25/06

COMPARISON: 1/23/06COMPARISON: 1/23/06INDICATIONS: PNAINDICATIONS: PNAFINDINGS:FINDINGS: There is patchy There is patchy

right lung infiltrate which is slightly right lung infiltrate which is slightly improved since the previous exam improved since the previous exam with improved volume loss in the with improved volume loss in the right chest. The patient is rotated. right chest. The patient is rotated. Gracile ribs suggest muscular Gracile ribs suggest muscular disease. The heart and disease. The heart and mediastinum are relatively mediastinum are relatively unremarkable.unremarkable.

CONCLUSION:CONCLUSION: Improving right Improving right lung infiltrate with re-expansion of lung infiltrate with re-expansion of the right lung since the previous the right lung since the previous exam.exam.

Exam Date:Exam Date: 1/27/20061/27/2006PROCEDURE:X-RAY OF CHEST PROCEDURE:X-RAY OF CHEST

ONE VIEW, PORTABLEONE VIEW, PORTABLECOMPARISON:COMPARISON: 1/25/2006.1/25/2006.INDICATIONS:INDICATIONS: Pneumonia.Pneumonia.FINDINGS:The patient is rotated. FINDINGS:The patient is rotated.

There is evidence of There is evidence of neuromuscular disease with neuromuscular disease with gracile ribs and humeri.gracile ribs and humeri.

There is further clearing of right There is further clearing of right lung infiltrate since previous lung infiltrate since previous examination.examination.

CONCLUSION:CONCLUSION: Further interval Further interval clearing of right lung infiltrate.clearing of right lung infiltrate.

New AchievementsNew Achievements

• PROBLEM: PROBLEM:

• Recurrent Intensive Care Unit Recurrent Intensive Care Unit Admissions for Patients with Admissions for Patients with Neuromuscular Disease, Cerebral Neuromuscular Disease, Cerebral Palsy, and Anoxic Brain DamagePalsy, and Anoxic Brain Damage

Respiratory Management of Pediatric Patients with Chronic Pulmonary Involvement  AMDA's 27th Annual Symposium March 24, 2004

Data Sources• Medical claims data from 2007-2009. 233,562 patients

identified with neuromuscular diseases. 446 patients received The Vest System.

• Thomson Reuters MarketScan Data Base reflecting the health experiences of employees and dependents covered by the health benefits of large employers employers.

• Milliman’s Consolidated HCG Database (CHSD) containing detailed claims and membership information from Milliman’s data contributors

• Claims and membership from the Center of Medicare and Medicaid Services (CMS) 5% sample of the Medicare population

This report was prepared by Milliman on January 16, 2012

Medical Claims Analysis• Medical claims analysis to examine the

effectiveness of High Frequency Chest Wall Oscillation (HFCWO) in reducing medical costs.

• The analysis covered 446 neuromuscular disease patients that had received HFCWO therapy between 2007 and 2009 comparing health care costs incurred before and after HFCWO therapy intervention.

• The results of the study show lower claim costs for patients with neuromuscular disorders after the initial insurance claim for HFCWO.

This report was prepared by Milliman on January 16, 2012

Milliman Actuarial Analysis

57

Commercial Claims• Overall Per Member Month (PMPM) claims costs

(excluding RX) were lower by 10.0%• Inpatient admissions per 1000 were lower by 21.3%• Inpatient days per 1000 were lower by 38.3%• Decrease in average length of stay from 11.6 to 9.1

days

Medicare Claims• Overall PMPM claim costs were lower by 8.2%• Inpatient admissions per 1000 were lower by 16.1%• Inpatient days per 1000 were lower by 37.8%• Decrease in average length of stay from 10.1 to 7.5

days

Medical claims analysis examined effectiveness of HFCWO in reducingmedical costs to 446 NM pts. Receiving HFCWO between 2007-2009

ReferencesReferences

• Deboeck et al. Airway clearance techniques to treat acute Deboeck et al. Airway clearance techniques to treat acute respiratory disorders in previously healthy children – where is the respiratory disorders in previously healthy children – where is the evidence? evidence? European Journal of Pediatrics. 2009European Journal of Pediatrics. 2009

• Fuhrman et al. Pediatric Critical Care. 4Fuhrman et al. Pediatric Critical Care. 4thth Edition. 2011. Edition. 2011.• Light et al. Pediatric Pulmonology. 2011Light et al. Pediatric Pulmonology. 2011• Mcilwaine, M. Physiotherapy and airway clearance techniques and Mcilwaine, M. Physiotherapy and airway clearance techniques and

devices. devices. Pediatric Respiratory Review.Pediatric Respiratory Review. 2007. 2007.• Morrison and Agnew. Oscillating devices for airway clearance in Morrison and Agnew. Oscillating devices for airway clearance in

people with cystic fibrosis. people with cystic fibrosis. Cochrane Database of Systematic Cochrane Database of Systematic Reviews.Reviews. 2009. 2009.

• Taussig et al. Pediatric Respiratory Medicine. 1999.Taussig et al. Pediatric Respiratory Medicine. 1999.• West. Respiratory Physiology – The Essentials. 8West. Respiratory Physiology – The Essentials. 8 thth Edition 2008. Edition 2008.• Landon. Novel methods of ambulatory physiologic monitoring in Landon. Novel methods of ambulatory physiologic monitoring in

patients with neuromuscular disease. Pediatrics 2009patients with neuromuscular disease. Pediatrics 2009