Caring for Ventilator-Dependent Children

Robert Graham, MD, hung up the phone after a lengthy conversation with the mother of a young child. The stress in her voice was evident and understandable. The woman and her husband had just brought their son home from the hospital. Not yet a year old, he remained far from full health.

The boy had been born with a complex heart lesion that had required a transplant, and the operation had led to further complications. He’d gone home with a tracheotomy and a ventilator, a feeding tube, and, because heart problems had led to renal problems, he was on peritoneal dialysis. The parents, thrust into the role of caring for an infant with a complex array of health challenges, felt anything but prepared.

Graham reflected that at least now he was in a better position to assist the family. At Boston Children’s Hospital, Graham had just launched a new program in the form of a small team dedicated full time to children who were dependent on ventilators. This baby was the program’s first patient.

In 2016, nine years after that launch, the Critical Care, Anesthesia, Perioperative Extension (CAPE) and Home Ventilation program looked after more than 300 patients, roughly 80 percent of whom had been diagnosed with cerebral palsy, muscular dystrophy, or spinal muscular atrophy. The team visited patients in their homes, answered inquiries by phone, and helped coordinate care needs. Anytime a patient was hospitalized, the team became even more deeply engaged, guiding the family through medical decisions and advising hospital caregivers on the patient’s health condition and full life context.

The program’s intent was to stave unnecessary escalations of care that these patients and their families frequently experienced. Typical local communities have few, if any, health professionals who are confident treating children dependent on life-support technologies like ventilators. As a result, even when a simple in-home intervention might suffice, these patients frequently are referred to hospitals and emergency rooms and, subsequently, to specialty hospitals and ICUs.

The escalation is not just expensive; it is dangerous. The normal risk of a hospital-acquired infection, for example, is far more consequential for these vulnerable patients. Furthermore, every transport risks stranding a child with urgent needs on the highway, because of traffic, weather, or any number of unforeseen circumstances.

During medical school, Graham’s curiosity drew him towards chronic, complex, and unusual diseases, as well as the many interrelationships between the body’s organ systems. Through exposure to intensive care units in his residency, he saw that patients with chronic ailments, especially children, became particularly vulnerable when beset by some sort of acute episode.

Graham chose a fellowship in pediatric critical care at Boston Children’s, one that allowed him to shuttle back and forth between multiple settings and to help guide patients through full episodes—from intensive care to rehabilitation to ongoing care in the community. The experience shone a spotlight on the various ways in which the system falls short for these patients. Transitions are fraught, access to care can be difficult in communities, and avoidable acute episodes are commonplace.

After completing his fellowship in 2004, Graham accepted a full-time job with Boston Children’s. He took a particular interest in a set of patients who were particularly vulnerable—those who were dependent on ventilators. In caring for these patients, he came into frequent contact with a colleague, Lauren Perlman, a respiratory therapist. He learned that Perlman was going well out of her way to assist these patients and their families, even stopping by their homes after hours.

In 2007, Graham approached Perlman and suggested that they start a program focused on these patients. Although their initial proposal lacked much detail beyond that it would include home visits, Graham’s division chief and department head were intrigued and wanted to give it a try.

Many years later, Graham still appreciated the occasional case that presented him with the task of learning about a rare disease unfamiliar to him. But his more routine preoccupation was finding ways to make the system work better for his patients. He also derived a great deal of satisfaction from helping to guide families through their challenging lives.

Caring for children dependent on ventilators is difficult. Parents are not just parents, they are also medical providers and care coordinators. While the technical aspects of the care that parents provide are learned readily enough, they are nonetheless demanding. Many state governments have programs that provide nursing care in the home, but never 24-7, and some patients must be monitored continuously to ensure their airways remain clear. Parents often are unavoidably absent from work or, at the very least, their productivity suffers. It is common for one parent to give up entirely on a career, and the entire family is affected by the reality of so much time and energy directed towards a single child. Few parents are able to accurately anticipate what is coming.

Nonetheless, parents adjust. Few slip into despair or hopelessness. And the children generally are able to achieve at least some semblance of normalcy in their lives. Schools have become ever more adept at mainstreaming children with handicaps, and many children who live on ventilators are unencumbered intellectually and readily able to keep up with classwork.

Activities outside of school are another story. In general, these children cannot keep up socially or physically; they can’t play sports or even go with friends to the mall. They often blend into the background in their communities, unnoticed by many.

Children dependent on ventilators also spend a lot more time than their peers as patients. Though most live without pain from day to day, they may need a great deal of physical therapy. Furthermore, they get sick more often than other children, and maladies that would be minor for most children can be serious and involve complicated recoveries.

As they approach young adulthood, most of these patients find their health is deteriorating. A certain fraction of any person’s energy is dedicated to the basic mechanics of supporting life, such as breathing and circulating blood. For individuals who live on ventilators, that fraction is higher, and this siphons energy from other activities, including growing or overcoming illness.

The impact worsens with age. These patients routinely aspirate saliva and bits of food, and they struggle to clear secretions. Damage to the lungs is inevitable and cumulative. Lung infections are common, scar tissue builds, and it becomes ever harder to eliminate foreign matter. It can become a vicious cycle. Few patients live into their fourth decade.

Home visits lie at the core of the CAPE program. In the program’s early years, through 2011, the team included only Graham and Perlman. They made most home visits together. Generally, each visit included some respiratory testing and, as needed, adjustments to ventilator settings. Perlman’s expertise as a respiratory technologist was crucial. Graham could specify desired outcomes (and to some degree act in place of a pulmonologist), but he did not operate the equipment. Ventilators had advanced over the years in both capability and complexity. There were many different models and a wide range of adjustments that could be made to ensure proper ventilation and oxygenation.

The program also offered training and assistance during home visits to day-to-day care providers, including family members and nurses who provided help in the home. Some of the nurses were quite skilled and had years of experience with patients on ventilators; others had very little.

Some home visits were scheduled proactively and at a frequency that depended on need. Such visits might include services, such as immunizations, that normally would be handled in a primary care office. In other cases, home visits were a reaction to illness and arranged to avoid unnecessarily moving or hospitalizing the patient.

To enable the program to serve more patients, Graham expanded the CAPE team and refined each team member’s role. By 2016, the team had grown to include Julie Shurtleff, a nurse practitioner; Yudy Muneton, a social worker; Maura Morrissey, program coordinator; and David Casavant, MD, an intensivist and pain doctor who allocated 30 percent of his time to the program.

The team’s schedule was mostly fluid and prioritized daily. Shurtleff’s focus was on outpatient care. She answered a steady stream of inbound phone calls from families each day, assessed the urgency of their needs, made some treatment decisions and referred others to Graham or Casavant. Shurtleff occasionally joined Graham and Perlman on home visits and handled some home visits on her own. Muneton took on a variety of roles. She worked with schools, helped families with insurance, coordinated mental health needs, sought affordable housing for families, and even linked families to charitable programs that might help with expenses, such as vans equipped to handle children on ventilators.

To further increase their capacity and impact with outpatients, the team had started seeing patients and their families through Internet video. Graham and Casavant shared their early findings in the Journal of Telemedicine and Telecare (“Trial of telemedicine for patients on home ventilator support: Feasibility, confidence in clinical management and use in medical decision-making,” December 2014, 20(8), pp. 441–449). Families reported higher confidence with telemedicine as compared to telephone calls. Graham and Casavant agreed, feeling that they could get a much fuller sense of what was going on in the home by using video. The authors estimated that 27 telemedicine encounters prevented 23 clinic visits, three ER visits, and one hospitalization.

CAPE had no physical clinic at Boston Children’s. However, any time a CAPE patient visited for an appointment with another provider, team members went out of their way to see the patient face-to-face.

On any given day, it was likely that a few CAPE patients were hospitalized. Graham or Casavant or both were always closely involved. They worked with other treating physicians on a care plan for the full episode of care, contributing their knowledge of the patient, the patient’s medical history, the family, the dynamics among family members, and the goals of care. Simply having observed the patient on multiple occasions could be powerful. For example, a doctor meeting the patient for the first time might remark that the patient appeared to be in terrible shape. Graham might respond that this was more or less how the patient looked every day.

Graham paid particular attention to supporting families with weighty medical decisions. Parents typically had a poor understanding of the consequences of the decisions they were asked to make. The decisions were complex and uncertain, as the rate of disease progression could be hard to predict.

Although each team member had workspaces within reasonable physical proximity of each other (all in the same building, though not all on the same floor), they came together just once a month for a staff meeting and discussion of the program’s growth and evolution. They relied heavily on daily phone calls and electronic messaging. That said, there was plenty of time for conversation about specific patients while driving to and from home visits. A typical visit generated multiple follow-up actions, often handled by cell phone from the car.

Graham wanted to expand CAPE. He estimated that within the six New England states, there were another 300 to 600 families that could benefit from its services. But he also knew that as the team took on more patients, its daily operations slipped from proactive to reactive and overall effectiveness declined. At a bit beyond 300 patients in 2016, he felt the team was close to full capacity. There was no way to double or triple the patient panel without similarly expanding the size of the team.

And yet, Graham knew he’d been fortunate to get as far as he had. He’d had the right experiences during his residency and fellowship. He’d met the right partner in Perlman. He was lucky to have the support of the senior leaders at Boston Children’s. The system at large, however, was not set up for a program like CAPE. Most egregiously, there was no means of reimbursement for many of the services CAPE provided. Roughly speaking, CAPE’s reimbursements covered only the cost of the non-physician staff. The remainder of the budget, Graham’s and Casavant’s salaries, had to be covered by the hospital or by outside grant support.

Graham and his team had begun gathering data to prove the program’s value. They logged every activity and estimated impact on costs and outcomes. They partnered with a research institute at Tufts University and shared their data with a coalition of payers.

Through their work, they learned that hospitalizations and emergency room visits were the overwhelming cost drivers for patients like those whom CAPE served. Furthermore, for these patients nationwide, the probability that an emergency room visit would lead to a hospitalization was at least 40 percent—and possibly as high as 90 percent—compared to just a few percent for the for the population at large.

Hospitalizations were extremely expensive and typically included a stay in the ICU. Indeed, one local payer’s data showed that 10 of their 20 most costly patients were served by CAPE. Total cost of care exceeded $1 million per year for some of these patients.

Graham and Casavant estimated that although they were only attentive to what was best for the patient and never made cost reduction an explicit consideration in their care, they saved the system between $7 million and $10 million per year. This was, of course, many multiples of the program’s unreimbursed costs—that is, Graham’s and Casavant’s salaries.

One might reasonably expect a payer’s reaction to such news would be to joyfully figure out how to help Graham expand CAPE. That hasn’t always been the case. In Graham’s recollection, one payer was more interested in the possibility of reducing the program’s cost by using nurse practitioners, not intensivists, for home visits. (Graham is interested in maximizing the role of nurse practitioners and acknowledges that the optimal roles for intensivists and nurse practitioners remains an open question. At the same time, he sees substantial value in intensivists making home visits and notes that because it would take several nurse practitioners to cover call 24-7, it might not reduce costs.)

Conversations have progressed but are still in early stages. Sharing financial risk is particularly tricky with these patients. The population is small and single-care episodes can be extremely expensive, so trying to forecast the total annual cost of care is a dicey proposition.

Graham had been treating the patient, a young adult with a complex heart disease and a lung malformation, for seven months. The family had called Graham because of an arrhythmia. Because there was also a high fever, Graham felt he had no choice but to ask the family to come to the emergency room. He was involved throughout the subsequent hospitalization, directing some tests that were needed, helping to avoid some care steps that weren’t needed, and doing everything possible to shorten what was inevitably a lengthy hospital stay.

During that stay, Graham had engaged the family members in several in-depth conversations about what it would take to help the patient thrive as best possible in the home. Three weeks after discharge, Graham was pleased. He’d visited the family in their home twice, fully enjoying his time with them on both occasions. The family had adapted their routine and had even made it possible to get the patient out and about a bit in the neighborhood. Graham would continue to closely monitor the patient’s recovery for at least another week.

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