Home Mechanical Ventilation
Howard B. Panitch, MD
Introduction
•Home mechanical ventilation (HMV) is used to support patients with chronic respiratory failure (CRF).
•The aim of HMV is to increase quality of life, reduce morbidity and mortality, and reduce health care costs (Box 116-1).
•The demographics of children receiving HMV have evolved since its introduction with the polio epidemics of the 1940s and 1950s, reflecting both the diseases that lead to CRF and the attitudes toward providing ventilatory support to children with chronic progressive conditions like Duchenne muscular dystrophy and spinal muscular atrophy.
•The number of children who receive HMV continues to increase, with estimates in 2011–2012 being 4.2–6.7 per 100,000 children <18 years of age.
•Methods of support have also shifted, with an increasing number of children receiving noninvasive ventilation versus ventilation via tracheostomy.
Box 116-1. Goals of Home Mechanical Ventilation
•Extend life and enhance its quality
•Reduce morbidity
•Improve physiological function
•Achieve normal growth and development whenever possible
•Reduce health care costs
Pathophysiology of CRF
•CRF is defined as the need for ≥4 hours per day of mechanical ventilatory support for ≥1 month, despite efforts to wean the patient from support.
•CRF can result from inadequacy of the respiratory pump, including the respiratory muscles, rib cage, and abdominal wall; abnormalities in the respiratory drive; extrathoracic and central airway lesions; and pulmonary parenchymal and vascular lesions (Box 116-2).
Box 116-2. Conditions That Necessitate Chronic Mechanical Ventilation
•Respiratory pump disorders
—Neuromuscular and central nervous system conditions
▪Myopathies
▪Spinal cord injuries
▪Encephalopathies
▪Botulism
▪Metabolic disorders
—Thoracic insufficiency syndrome
▪Chest wall malformations
▪Kyphoscoliosis
•Abnormalities of the respiratory drive
—Congenital central hypoventilation syndrome
—Brainstem lesions
•Extrathoracic and central airway lesions
—Upper-airway obstruction
▪Laryngomalacia
▪Craniofacial abnormalities
—Tracheobronchomalacia
•Pulmonary parenchymal and vascular lesions
—Bronchopulmonary dysplasia
—Chronic aspiration
—Interstitial lung disease
—Congenital heart disease
—Congenital abnormalities
▪Diaphragmatic hernia
▪Pulmonary hypoplasia
▪Tracheoesophageal fistula
—Bronchiectasis
•Pump failure results in hypercapnia, while parenchymal disease causes hypoxemia.
•Suitability for HMV is more a function of the degree of medical stability and the extent of support available than the underlying cause of the CRF.
Clinical Features of CRF
•CRF can arise from an acute illness from which the child does not completely recover or a gradual progression of a chronic disease, such as cystic fibrosis or neuromuscular disease.
•CRF can progress in a stereotypical way in children with neuromuscular disease, beginning with sleep arousal and fragmentation, followed by sleep hypoventilation and, ultimately, diurnal respiratory failure.
•Inadequate respiratory support can result in growth and developmental delay or failure.
•Episodic or chronic hypoxemia from inadequate support can also lead to pulmonary hypertension, cor pulmonale, and right-sided heart failure.
• Infants and young children who require >16 hours per day of ventilatory support usually undergo tracheostomy placement; older children can be supported with noninvasive ventilation, even when they require support 24 hours per day.
Indications and Eligibility
•Suitability for HMV requires medical stability, caregiver support, an adequate home environment, commitment by third-party payers to support the endeavor, and a medical team to provide necessary care and guidance (Box 116-3).
•Two adults must agree to learn all aspects of the child’s care.
•Health care professionals must be identified, including a primary care physician, a skilled nursing agency, a durable medical equipment (DME) company, and a local ambulance service.
•If the child lives a distance from the tertiary care center, an emergency care facility should also be identified if stabilization of the child may be necessary.
•The home is assessed by the DME company to determine whether there is adequate space and electrical service for all HMV equipment and accessories.
Management
•Before discharge, negotiations with the insurance company, state Medicaid, or model waiver programs must ensure payment for durable and disposable equipment and establish hours of skilled nursing care and other therapies and services.
•Skilled nursing care should be arranged for any infant or child (a) who would experience life-threatening respiratory compromise if the ventilator interface became displaced, disconnected, or obstructed by secretions and (b) who could not correct the problem without assistance.
•The amount of nursing care will vary from 8 to 24 hours per day on the basis of the complexity of the child’s condition and other comorbidities, the demands on nonskilled caregivers (employment, other children at home), family preference, and point in the child’s overall course (having nursing care 24 hours per day is reasonable for the first 1–2 weeks after initial hospital discharge to ease the transition to the home, with a reduction in nursing care hours thereafter).
Box 116-3. Patient Eligibility
•Medical stability
—Clinical
▪Positive trend on the growth curve
▪Stamina for periods of play
▪No frequent fevers or infections
—Physiological
▪Stable airway
▪Pao2 ≥60 mm Hg in Fio2 ≤0 .4
▪Pco2 <50 mm Hg
▪Frequent ventilator changes not required
▪Individualization: Many of these guidelines can be modified to facilitate the discharge of a child who wishes to go home and receive hospice care, for example .
•Social, environmental
—Family members willing to
▪Help care for the patient
▪Be included in the planning and selection of professional
caregivers
▪Commit to the plan
—Home environment
▪Enough space
▪Access (eg, ramps for children confined to wheelchairs)
▪Adequate heat, electricity, and water
▪Working telephone
▪Area resources (emergency room, ambulance service)
•Reimbursement by third-party payers, Medicaid, and Model Waiver Programs to fund the following:
—Durable medical equipment
—Disposable supplies
—Nursing salaries
—Occupational, physical, speech, feeding, and developmental therapists
•Organizational: home ventilation team
—Members
▪Medical, nursing, respiratory therapy, social services, nutrition, support services
—Functions
▪Coordinate care with community medical caregivers
▪Review treatment plans
▪Advocate for patient and family
▪Provide medical direction
▪Guarantee access to tertiary care
Fio2, forced expiratory volume in 1 second; Paco2, partial pressure of carbon dioxide, arterial; Pao2, arterial oxygen pressure .
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