Home Non-Invasive Positive Airway Pressure Devices for the Treatment of Respiratory Insufficiency and Failure

POLICY NUMBER

A.8.01.64

DESCRIPTION

Respiratory failure is characterized by low arterial blood oxygen (hypoxemia, PaO₂) and/or high arterial carbon dioxide (hypercapnia, PaCO₂ >45 mmHg). Chronic respiratory insufficiency or failure can occur with chronic obstructive pulmonary disease (COPD), thoracic restrictive disorders, and hypoventilation syndromes, and may result in poor quality of life, sleepiness, hospital admission, intubation, and death. Non-invasive positive airway pressure ventilation (NPPV) including continuous positive airway pressure (CPAP), bilevel positive airway pressure (BPAP), and home mechanical ventilators (HMV) that are pressure, rate and volume targeted are proposed for the treatment of COPD and other forms of chronic hypoventilation.

Chronic Obstructive Pulmonary Disease

Chronic obstructive pulmonary disease (COPD) is a common condition, affecting more than 5% of the population, and is associated with high morbidity and mortality. COPD is the third leading cause of death in the United States. It is a clinical syndrome with multiple etiologies that is characterized by chronic respiratory symptoms, structural pulmonary abnormalities, and/or lung function impairment. Chronic obstructive pulmonary disease is most frequently associated with cigarette smoking or other air pollutants, and a majority of patients with COPD in the United States have a history of cigarette smoking. Chronic obstructive pulmonary disease is progressive, with expiratory airflow limitation, air trapping/hyperinflation, and destruction of alveoli (emphysema). The Global Initiative for Chronic Obstructive Lung Disease (GOLD), defines COPD as "a heterogeneous lung condition characterized by chronic respiratory symptoms (dyspnea, cough, sputum production and/or exacerbations) due to abnormalities of the airways (bronchitis, bronchiolitis) and/or alveoli (emphysema) that cause persistent, often progressive, airflow obstruction."

Respiratory failure in patients with COPD is characterized by the inability to sustain normal gas exchange, leading to low arterial blood oxygen (hypoxemia, PaO₂) and/or high arterial carbon dioxide (hypercapnia, PaCO₂). Hypercapnia develops in about one-third of patients with COPD and is associated with poor quality of life, sleepiness, frequent hospital admissions due to exacerbations, and an increase in mortality compared to patients with COPD who are normocapnic. The hypercapnia is due in large part to poor lung biomechanics including low inspiratory muscle reserve, high CO₂ production, and a reduced ventilatory capability. The imbalance between the respiratory load and respiratory capability may in turn affect the ventilatory control center in the brain stem. Physiological changes in responsiveness to hypoxemia and hypercapnia during sleep can be particularly pronounced in patients with COPD, with overnight increases in PaCO₂ affecting daytime PaCO₂, possibly through bicarbonate retention or changes in cerebrospinal fluid. Patients with COPD may also have comorbid obstructive sleep apnea and/or obesity hypoventilation syndrome due to decreased ventilatory motor output and upper airway muscle activity during sleep.

Thoracic Restrictive Disorders Due to Neuromuscular Disease

Thoracic restrictive disorders result from a variety of underlying diseases all characterized by restrictive patterns on pulmonary function testing. Neuromuscular disorders such as muscular dystrophy, amyotrophic lateral sclerosis (ALS), polio, and phrenic neuropathies can result in weakness of the respiratory muscles affecting inspiration and expiration, ultimately resulting in hypoventilation. Impaired cough and swallowing associated with neuromuscular disease increases the risk of respiratory complications in these patients. Nocturnal hypoventilation due to muscular atonia during sleep leads to nocturnal hypercapnia. Frequent nocturnal episodes can result in renal compensation and ultimately result in daytime hypercapnia. Non-invasive positive airway pressure ventilation (NPPV) is often necessary for patients with thoracic restrictive disorders due to neuromuscular disease.

Hypoventilation Syndromes

Hypoventilation syndromes are nonspecific disorders characterized by hypercapnia (PaCO₂>45 mm Hg) that is not otherwise categorized. Obesity hypoventilation syndrome (OHS), central respiratory depression due to substance or medication use, and decompensated hypercapnic respiratory failure that is not COPD are all included in this category. In patients with OHS, weight loss is useful in normalizing PaCO2; however, NPPV should be initiated early while weight loss is attempted.

Treatment With Non-invasive Positive Airway Pressure

A major goal of management of patients with chronic hypoventilation is to reduce hospitalizations and mortality. Long-term oxygen therapy is recommended for patients with poor clinical status and noninvasive positive airway pressure ventilation (NPPV) devices for patients with severe chronic hypercapnia and a history of hospitalization for acute respiratory failure. Noninvasive positive airway pressure ventilation devices include nocturnal continuous positive airway pressure (CPAP) for individuals with hypercapnia due to obstructive sleep apnea or hypoventilation and bilevel positive airway pressure (BPAP) devices or non-invasive home mechanical ventilators that are pressure, rate, and volume targeted. The objective of this policy is to describe which features of NPPV are required to improve the net health outcome in patients with COPD, thoracic restrictive disorders due to neuromuscular disease, or those with hypoventilation syndromes.

Benefits of nocturnal NPPV persist into the daytime with improved breathing patterns (lower frequencies and larger tidal volumes) and improved gas exchange. Explanations for the improvement in daytime respiration with nocturnal NPPV include increased respiratory drive, improved diaphragm function by unloading the respiratory muscles during sleep, increased CO₂ sensitivity, and reduction in air trapping and hyperinflation. It is not known which factors (eg, muscle unloading, gas exchange normalization, decrease in hyperinflation) underlie the benefits of NPPV on health outcomes. It is also unclear if the reduction in PaCO₂ has an effect on health outcomes or if it is only a marker of effective ventilation.

Respiratory Assist Devices

The Centers for Medicare and Medicaid Services (CMS) defines respiratory assist devices (RADs) as bilevel devices with or without back-up respiratory rate capability. While CPAP devices provide continuous air at a pressure that prevents the collapse of the airway during inspiration, BPAP devices work by increasing pressure during inspiration and lowering it during expiration (pressure cycled). In some devices a backup respiratory rate is triggered when the patient's nocturnal respiratory rate decreases below a set threshold. The backup rate is typically set 2 breaths below the patient's spontaneous respiratory rate during wakefulness.

Terminology on device features is described in Table 1.

Table 1. Device Features

Home Mechanical Ventilators

In some patients, nocturnal respiratory assist devices are insufficient to address the respiratory failure. Non-invasive home mechanical ventilators (HMV) are proposed for the treatment of chronic respiratory failure that is refractory to a respiratory assist device. Mechanical ventilators are devices that deliver more controlled breathing with bilevel ventilation at a higher pressure. The ventilators may also have additional features compared to BPAP machines such as alarms and battery backup power. Home mechanical ventilators can be used for patients with tracheostomy in the home, but may also be used with a non-invasive interface such as a mask or mouthpiece in patients who do not depend on 24 hour ventilation for survival. Current technology has decreased the size of home ventilators to around 10 pounds. In addition, some models may be wireless with battery backup, allowing greater mobility during the day.

Titration

Early studies with low intensity NPPV did not demonstrate health benefits in patients with hypercapnia. More recent studies have reinforced the importance of high-intensity NPPV (>18 cm H₂O) that is titrated to decrease hypercapnia. A high respiratory backup rate that is increased to the level of spontaneous breathing has also been shown to be important to achieve positive health outcomes. Manually set, laboratory or hospital titration of NPPV with pressure control and backup rate have been recommended for stable hypercapnic COPD. The goal of titration of inspiratory positive airway pressure is to achieve normocapnia, a reduction in transcutaneous CO₂, or maximum tolerable inspiratory pressure. A fast rise in inspiratory pressure (rise time) allows enough time for expiration within the normal rate of breathing. In patients with air trapping and hyperinflation, use of positive end-expiratory pressure can also be beneficial.

A suggested protocol for in-laboratory titration of NPPV in patients with COPD in the U.S. is described by Orr and colleagues. Titration of NPPV is usually performed in a monitored environment after the patient has stabilized, as studies have not found an improvement in health outcomes when NPPV is started soon after an acute exacerbation. Polysomnography or respiratory monitoring may be used during titration to evaluate the presence of obstructive sleep apnea or hypoventilation. The inspiratory pressure is typically started at 6 to 8 cm H₂O of pressure support above the expiratory pressure and titrated to reduce hypercapnia. A Bilevel-ST (with backup rate) or a VAPS (volume assured) may be used if a Bilevel-S (without backup rate) fails to adequately reduce hypercapnia. Although titration in European studies has been performed with a hospital stay, this is not feasible in the U.S., and titration might be performed over several weeks in the patient's home by an external durable medical equipment (DME) provider.

Pulmonary Rehabilitation

Pulmonary rehabilitation is a personalized intervention that includes physical activity (eg, activities of daily living, endurance exercises and muscle strengthening), health education, and psychological support. It may be performed in the hospital, outpatient clinic, or home, and has been shown to reduce mortality, exacerbation rate, intensive care admissions, and emergency department visits. Pulmonary rehabilitation is common in Europe but is less frequently provided in the U.S.

Numerous CPAP and BPAP devices are available in the U.S. Examples of HMV devices that have both invasive and non-invasive interfaces and are available in the U.S. are described in Table 2.

Table 2. Select Home Mechanical Ventilators with Non-invasive Interface

POLICY

Nocturnal bilevel positive airway pressure (BPAP) with backup rate may be considered medically necessary for individuals with COPD and chronic respiratory failure (see Policy Guidelines) who meet either of the following:

• Chronic stable daytime (awake) hypercapnia (PaCO₂≥52 mmHg) OR

• Daytime (awake) hypercapnia (PaCO₂≥52 mmHg) at least 2 weeks after discharge from the hospital for an acute exacerbation with decompensated respiratory acidosis.

Non-invasive home mechanical ventilation may be considered medically necessary for individuals with COPD who meet the following criteria:

• Qualify for a BPAP device AND meet at least one of the following:

• • Higher pressure is needed to reduce hypercapnia than can be achieved with a bilevel device during titration (typically >25 cm H₂O); OR

  • Severe hypoxemia requiring fraction of inspired oxygen (FIO₂) >40% or >5 L/min; OR

  • Daytime use (battery operated unit) is required to reduce hypercapnia.

Individuals with COPD who are started on BPAP at discharge from hospitalization may continue for up to 3 months to provide time to stabilize and complete reevaluation.

Non-invasive positive airway pressure for COPD is considered investigational under all other conditions.

Bilevel positive airway pressure may be considered medically necessary for individuals with thoracic restrictive disorders due to neuromuscular disease who meet any of the following:

• Pulmonary function tests:

  • Spirometry (upright or supine) with vital capacity <50% predicted or <80% predicted with associated symptoms (orthopnea, dyspnea, morning headaches, excessive daytime sleepiness, or unrefreshing sleep); OR

  • Maximal inspiratory pressure <60 cm H₂O or maximum expiratory pressure (MEP) <40 cm H₂O; OR

  • Peak cough flow (PCF) <270 L/min for age ≥12 years or PCF <5th percentile for age <12 years; OR

  • Sniff nasal inspiratory pressure (SNIP) <70 cm H₂O in males, SNIP <60 cm H₂O in females for age ≥12 years.

• Hypercapnia

  • Chronic stable daytime (awake) hypercapnia with PaCO₂ ≥45 mmHg (capillary blood gas can be used in children); OR

  • Venous blood gas PCO₂, end-tidal PCO₂, or transcutaneous PCO₂, ≥50 mmHg; OR

• Hypoxia

  • Overnight oximetry in-laboratory or home sleep test with saturation <88% for 5 minutes;

  • Overnight oximetry: SpO2 ≤90% for ≥2% of sleep time.

Non-invasive home mechanical ventilation may be considered medically necessary for individuals with thoracic restrictive disorders due to neuromuscular disease who meet the following:

• Qualify for a BPAP device; AND

  • BPAP fails; OR

  • Have extreme loss in function with vital capacity <30%; OR

  • Non-invasive ventilation is needed for >10 hours per day; OR

  • Severe breathlessness (e.g., with speaking at rest); OR

  • Worsening daytime hypercapnia with need for mouthpiece ventilation; OR

  • Daytime use (battery operated unit) is required to reduce hypercapnia or dyspnea.

Bilevel positive airway pressure may be considered medically necessary for individuals with hypoventilation syndromes who meet the following criteria:

• Awake or sleep hypoventilation with hypercapnia (one of the following is met):

  • Awake hypoventilation with chronic stable daytime (awake) hypercapnia (PaCO₂ ≥45 mmHg); OR

  • Venous blood gas PCO₂, end-tidal PCO₂, or transcutaneous PCO₂ ≥50 mmHg; OR

  • Sleep hypoventilation with hypercapnia:

    • ≥10 mmHg increase from baseline awake PCO₂ and to a value >50 mmHg for ≥10 min; OR

    • PCO₂ ≥55 mmHg for ≥10 min; AND

• Low clinical suspicion for COPD or neuromuscular disease; AND

• One of the following conditions are met:

  • Obesity with body mass index (BMI) ≥30 kg/m²; OR

  • Decreased respiratory drive due to opioid or substance use; OR

  • Advanced lung disease other than COPD (e.g., end-stage or advanced interstitial lung disease); AND

• Individual was discharged from inpatient stay with persistent awake hypoventilation (hypercapnia) on BPAP.

  • A reassessment with a provider within 3 months (30 to 90 days) is required and an attended polysomnogram (PSG) should be performed to assess appropriateness of positive airway pressure modality (home sleep apnea test is acceptable if attended PSG is not obtainable); OR

• Individual is ambulatory and sleep study indicates that BPAP is necessary for sleep-disordered breathing, or individual with severe obstructive sleep apnea is continuous positive airway pressure intolerant or continuous positive airway pressure was proven ineffective.

Non-invasive home mechanical ventilation may be considered medically necessary for individuals with hypoventilation syndromes who meet the following:

• Qualify for a BPAP device and at least one of the following:

  • Higher pressure is needed to reduce hypercapnia than can be achieved with a bilevel continuous positive airway pressure device during titration (typically >25 cm H₂O); OR

  • Severe hypoxemia requiring FIO₂ >40% or >5 L/min; OR

  • Daytime use (battery operated unit) is required to reduce hypercapnia; OR

• Tried and failed BPAP device with persistent hypercapnia despite 3 months of adequate adherence to prescribed positive airway pressure therapy with:

  • Awake PaCO₂ ≥45 mmHg; OR

  • Awake venous blood gas PCO₂, end-tidal PCO₂, or transcutaneous PCO₂ ≥50 mmHg.

POLICY EXCEPTIONS

Federal Employee Program (FEP) may dictate that all FDA-approved devices, drugs or biologics may not be considered investigational and thus these devices may be assessed only on the basis of their medical necessity.

POLICY GUIDELINES

The coverage guidelines outlined in the Medical Policy Manual should not be used in lieu of the Member's specific benefit plan language.

Respiratory failure in individuals with chronic disease is characterized by the inability to sustain normal gas exchange, leading to low arterial blood oxygen (hypoxemia, PaO₂) and/or high arterial carbon dioxide (hypercapnia, PaCO₂). Assessment of hypoxemia would lead to supplemental oxygen administration. Stable clinical state is defined as free of exacerbations for at least 4 weeks with pH over 7.35.

Compliance with treatment of at least 4 hours per 24 hours should be documented after the first 3 months of use. There are limited data on which to base compliance assessment. Assessment could be further based on an average of at least 4 hours per 24 hours over a consecutive 30-day period or use of 4 hours per 24 hours for at least 65% of the days in a consecutive 30-day period.

The Centers for Medicare and Medicaid Services (CMS) classifies a respiratory assist device as a bilevel positive airway pressure (BPAP) device with or without backup respiratory rate capability. Treatment modalities that are reported with the E0471 code include BiPAP ST, ASV, BiPAP AutoSV, iVAPS, AVAPS. BPAP units with batteries have a battery life that is shorter than home mechanical ventilators and are infrequently used in the U.S.

CMS defines non-invasive mechanical ventilators as life supporting/sustaining devices used in various settings, including home, hospital, and institutional settings. The non-invasive mechanical ventilators should have at least 6 pressure modes and 3 volume modes, and allow for both invasive or non-invasive use.

Although most individuals with comorbid COPD and obstructive sleep apnea can be effectively treated with continuous or auto-adjusting positive airway pressure, approximately 10% of individuals will need BPAP to tolerate the required pressure.

Respiratory therapy in the home may be provided for individuals who are treated with E0466, E0470, or E0471 devices.

Medically Necessary is defined as those services, treatments, procedures, equipment, drugs, devices, items or supplies furnished by a covered Provider that are required to identify or treat a Member's illness, injury or Mental Health Disorders, and which Company determines are covered under this Benefit Plan based on the criteria as follows in A through D:

1. consistent with the symptoms or diagnosis and treatment of the Member's condition, illness, or injury; and

2. appropriate with regard to standards of good medical practice; and

3. not solely for the convenience of the Member, his or her Provider; and

4. the most appropriate supply or level of care which can safely be provided to Member. When applied to the care of an Inpatient, it further means that services for the Member's medical symptoms or conditions require that the services cannot be safely provided to the Member as an Outpatient.

For the definition of medical necessity, “standards of good medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. BCBSMS makes no payment for services, treatments, procedures, equipment, drugs, devices, items or supplies which are not documented to be Medically Necessary. The fact that a Physician or other Provider has prescribed, ordered, recommended, or approved a service or supply does not in itself, make it Medically Necessary.

Investigative is defined as the use of any treatment procedure, facility, equipment, drug, device, or supply not yet recognized as a generally accepted standard of good medical practice for the treatment of the condition being treated and; therefore, is not considered medically necessary. For the definition of Investigative, “generally accepted standards of medical practice” means standards that are based on credible scientific evidence published in peer-reviewed medical literature generally recognized by the relevant medical community, and physician specialty society recommendations, and the views of medical practitioners practicing in relevant clinical areas and any other relevant factors. In order for equipment, devices, drugs or supplies [i.e, technologies], to be considered not investigative, the technology must have final approval from the appropriate governmental bodies, and scientific evidence must permit conclusions concerning the effect of the technology on health outcomes, and the technology must improve the net health outcome, and the technology must be as beneficial as any established alternative and the improvement must be attainable outside the testing/investigational setting.

POLICY HISTORY

05/01/2022: New policy added. Approved by the Medical Policy Advisory Committee.

04/04/2024: Policy title changed from "Non-Invasive Positive Airway Pressure for Chronic Obstructive Pulmonary Disease" to "Home Non-Invasive Positive Airway Pressure Devices for the Treatment of Respiratory Insufficiency and Failure." Policy description updated to add information regarding chronic obstructive pulmonary disease, thoracic restrictive disorders, and hypoventilation syndromes. Added medically necessary policy statements for thoracic restrictive disorders due to neuromuscular disease and hypoventilation syndromes when the specified criteria are met. Policy Guidelines updated with minor changes. Code Reference section updated to add ICD-10 diagnosis codes E66.2, G47.35, and J98.4.

04/23/2024: Policy reviewed; no changes.

04/22/2025: Policy description updated regarding COPD. Policy statements unchanged. Policy Guidelines updated.

SOURCE(S)

Blue Cross Blue Shield Association policy # 8.01.64

CODE REFERENCE

This may not be a comprehensive list of procedure codes applicable to this policy.

The code(s) listed below are ONLY medically necessary if the procedure is performed according to the "Policy" section of this document.

Medically Necessary Codes

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