Care of Ventilator-Dependent Children with Chronic Lung Disease

Steven Abman, MD,
Director, Pediatric Heart Lung Center , The Children’s Hospital
Professor, Pulmonary Medicine, University of Colorado Denver School of Medicine

Progressive developments in respiratory care have markedly improved the outcomes of sick newborns and children with severe lung diseases, including complications of premature birth, acute neonatal respiratory failure, acute respiratory distress syndrome (ARDS), congenital heart disease and other diverse diseases. These children include survivors of the neonatal and pediatric intensive care units, infants with congenital airway abnormalities, children with neuromuscular disease, severe scoliosis, obstructive sleep apnea and many other conditions. Despite these advances in respiratory therapies, there is a persistent need to improve our overall approach to the care of children with chronic respiratory failure who need prolonged ventilator support.

One of the major groups of such infants includes survivors of extreme prematurity and acute neonatal intensive care, who develop the chronic lung disease, known as bronchopulmonary dysplasia (BPD). Although most infants with BPD wean successfully from respiratory support, we continue to treat infants with severe chronic respiratory failure who require tracheostomy and long-term ventilator care. Most infants with ventilator-dependent BPD remain in NICUs, however, the nature of their lung disease and clinical strategies to optimize care are often quite distinct from the general approaches used in preterm and term infants with acute respiratory failure.

It has been long appreciated that there are major issues with the management of infants with severe BPD in the intensive care setting, leading to the initiation of chronic ventilator units at many centers. These issues include several problems managing chronic disease in an acute care setting, such as receiving less attention than patients with acute life-threatening problems who require urgent attention. Also, BPD infants have complex cardiopulmonary physiology, requiring different ventilator and medical strategies than are generally applied in the NICU.

Neurodevelopmental outcomes for these infants have become a major area of concern, with cognitive, speech and language, motor and socio-emotional outcomes as well as oral feeding being particularly affected. Long-term hospitalization for these infants with their associated invasive procedures, during critical periods of brain development as well as repeated hospitalizations once discharged from the Newborn Center (NBC) contribute to significant adverse neurodevelopmental outcomes that continue to affect children into school age. Parents’ reactions to having a hospitalized, chronically ill and frequently technology dependent infant also cause significant psychological, social, emotional and financial disruptions requiring an interdisciplinary approach to provide supports for families.

As noted above regarding infants in the NBC, providing care for older children with chronic respiratory failure in an acute care setting, such as the PICU, is also problematic. Not infrequently, children previously discharged from the NBC who were born prematurely or who have established BPD require prolonged ventilation and subsequently the need for chronic ventilator support. Numerous patients with a variety of congenital airway, respiratory, orthopedic or systemic disease are acutely admitted to the PICU and subsequently develop the need for chronic ventilator support as well (see Table 1 on next page). Our recent experience in providing pulmonary hypertension (PH) consults from the Section of Pulmonary Medicine and the Pediatric Heart Lung Center in the NBC and PICU has led to the development of more comprehensive consultations to care for these complex patients, and has re-emphasized the need to establish a more comprehensive, multidisciplinary approach to providing care to these children. Furthermore, these patients remain in the hospital setting for prolonged periods due to the lack of community resources, private duty nurses and others.

The Children’s Hospital continues to provide support for children with diverse causes of chronic respiratory failure, leading to the need for similar approaches to chronic tracheostomy and ventilator patients. Such diagnoses include neuromuscular disease, congenital lung disease, severe airway anomalies, complex cardiovascular disease, obstructive sleep apnea and others (see Table 1).

There has been a special need to establish and emphasize the multidisciplinary approach towards patients with chronic tracheostomies who require home ventilation. Not only do their issues extend well beyond their respiratory status, but many medical problems that include cardiac, gastrointestinal and nutrition, and neurodevelopmental concerns remain especially important. In the past, our standard approach to the care of technology-dependent children did not encourage a strong multidisciplinary approach that is attentive to meeting these needs for our chronic patients. This is especially a problem when we think about transitioning patients from the intensive care setting to the ward, and finally, transitioning from in-hospital care to home care. The gaps in our ability to manage medical, developmental and social issues have been especially apparent. The need to improve the continuity of care, to increase communication among specialists, staff and primary care providers, and to provide better support and advocacy for parents and their families, led to a clear need for The Children’s Hospital to develop a multidisciplinary team to implement a thorough and effective approach to the management of these children.

In addition, the number of infants requiring non-invasive ventilation (such as BiPAP or nasal CPAP) has increased in recent years, including patients who are recovering from acute disease, but do not require tracheostomy for long-term care. For example, older children with chronic lung diseases, such as cystic fibrosis, interstitial lung disease, obstructive sleep apnea, obesity with hypoventilation, restrictive lung disease that is worsened following scoliosis repair, and others, require non-invasive ventilator support as part of their care at Children’s. Advances in the use of non-invasive ventilation has allowed for prolonged respiratory support in patients who are otherwise stable and do not require care in an intensive care setting. The use of non-invasive ventilation continues to increase at Children’s, and a comprehensive approach to the management of infants with chronic respiratory failure requires multidisciplinary teams for better care.

Causes of respitory failure in children:

• Bronchopulmonary Dysplasia (BPD)
• Cystic Fibrosis (CF)
• Interstitial Lung Diseases

• Lung Hypoplasia
   - Congenital Diaphragmatic Hernia
   - Primary Lung Hypoplasia

• Neuromuscular Disease
   - Muscular Dystrophy
   - Spinal Muscular Atrophy
   - Diaphragm Paralysis

• Central Hypoventilation Syndromes

• Congenital Airway Abnormalities
   - Tracheomalacia, bronchomalacia
   - TE Fistula
   - Tracheal Stenosis

• Spinal Cord Injury

• Obstructive Sleep Apnea

• Severe Scoliosis, abnormal thorax

• Severe Congenital Heart Disease

• Central Nervous System (CNS) Disease
   - Head trauma, asphyxia, infection
   - Severe MRCP

• Osteogenisis Imprefecta

The Ventilator Care Program

As a result, we established the Ventilator Care Program (VCP) at The Children’s Hospital. The overall goal of this program is to launch a multidisciplinary program that will include pulmonary, respiratory therapy, nurse specialists, nursing staff who are especially interested in the care of these infants, neonatology, intensivists, developmentalists, occupational therapists, speech therapists, physical therapists and other care providers. This program includes three overlapping components.

Inpatient Unit

The Pediatric Respiratory Care Unit (PRCU), will involve dedicated space for a centralized, inpatient ventilator care center for tracheostomy and/or ventilator-dependent children, children who require non-invasive ventilation, and other patients who may require specialized respiratory care services and related technologies, such as nasal CPAP and BiPAP. Currently, patients are on the ninth floor of The Children’s Hospital as part of the Breathing Center , as plans for the PRCU are still underway. The same teams managing our chronic tracheostomy and ventilator patients are involved with the care of such specialized patients. This encourages cross-talk between respiratory therapists, nurses and physicians involved in their care, and would help develop cutting-edge approaches to respiratory care of complex patients.

Inpatient Consultation

An inpatient consultation service provides regular, ongoing consultation service for patients who require ventilator support and related airway care within the NBC, PICU and CICU, as well as patients in the neuro-rehabilitation program and other units. These consults are more comprehensive than past support provided by the pulmonary service by including a diverse multidisciplinary team which includes a focus on developmental issues impacting infants and young children with pulmonary compromise. Neurobehavioral assessment from the time of inclusion in the Ventilator Care Program while the infant is in the NICU, throughout the infant’s hospital stay, and during clinic visits after discharge will be included with consultation with our neurocognitive team.

Outpatient and Home Care

An outpatient program that links inpatient care and outpatient consults or referrals from other centers with long-term outpatient and home care. The Special Care Clinic plays a very important role in the outpatient program along with our ongoing pulmonary clinics. Special Care Clinic, as led by Drs. Ellen Elias and Adam Rosenberg, currently follows a large number of patients with tracheostomies and chronic ventilation. Dr. Jason Gien (neonatology) plays a critical role as an important liaison not only in the newborn center but in the outpatient program as well. This group is actively involved with our inpatient conferences and will be very aware of the transition of our patients from inpatient to outpatient settings and can help coordinate care with the rest of the team. Respiratory therapy is central to the care of these children and the clinic itself provides multidisciplinary care in a well-coordinated fashion. This group is also very experienced in knowing the community resources that are available for our patients and have been successful in helping to coordinate appointments for patients so that they can see pulmonary, ENT, and other specialists on the same day as much as possible. Nutrition and other therapies are also involved.

Thus, the VCP is multidisciplinary and provides key features that are required for the successful care of children with chronic ventilator needs by linking inpatient and outpatient care, and by developing a multidisciplinary team of care providers with the experience and focused interest in providing the highest quality of care to these patients and their families. Other aspects of this program include the development of clinical care guidelines with quality improvement approaches, patient advocacy and education, training and research.

For questions about the Ventilator Care Program or patient referrals, please contact Robin Mascotti in the Pediatric Heart Lung Center at (720) 777-5821.