Taking Children Home With Mechanical Ventilation

Vol. 14 •Issue 5 • Page 30
Home Pathways

By James Royall, MD

Advances in pediatric and neonatal intensive care have improved survival for critically ill children. Unfortunately, not all of these children can be free of dependence on technology, most commonly long-term mechanical ventilation. Children “stuck” in intensive care units were the stimulus for the development of organized programs that allow safe ventilation support in the home.

Additional children and families have benefited from these programs. Primarily these are children with limitation to ventilation due to processes outside of the lung such as inadequate central drive to ventilation or limited lung inflation due to neuromuscular weakness or thoracic cage defects. These children may progress to home ventilation without a phase of acute respiratory failure requiring care in an ICU.

Common reasons why children may require long-term ventilation are listed in Table 1, page 32. For some, like children with bronchopulmonary dysplasia, the usual expectation is slow improvement and eventual freedom from ventilator support. For some, like high spinal cord injury, the usual expectation is ongoing requirement for ventilation support but in a stable fashion. And for others, like brain stem tumors unresponsive to therapy, the usual expectation is terminal progression of the primary disease process in the foreseeable future.

For the latter group, time at home is precious, and training for home ventilation can be relatively brief. For the first two groups, however, we need to create a “mini-ICU” environment in the home.

TAKING AN ORGANIZED APPROACH

Our home ventilation program focuses on the need for continuous ventilatory support at home via tracheostomy tube. However, a similar organized approach can be taken to noninvasive ventilatory support, which is an option for some patients even with fairly significant respiratory compromise.

We developed and started our program in 2000. Our initial estimate that we would enroll about four patients per year has been quite accurate.

We partnered with a home care agency so that the team who initiated the program in the hospital also would conduct the patient’s discharge and provide outpatient care. This relationship has worked extremely well and has allowed us to deliver continuous, high-quality service. This team developed an organizational manual for the program in addition to manuals for teachers and learners to assure that the educational process was complete and consistent.

INTAKE PROCESS

The intake process is very important. Any leverage the medical director or team has in defining the home support environment is much reduced once the family leaves the hospital. The process usually begins with contact from the patient’s physician with a request for consultation. General patient guidelines are listed in Table 2, page 32.

The child should be “rock solid” in terms of medical support. The parents should not be placed in a position of making daily (or even weekly) significant medical support decisions. It is not optimal if the disease process has inherent variability such that periods of physiologic instability may be expected.

For example, we have evaluated an infant with severe congestive heart failure on very low ventilator settings. However, the child had limited cardiac reserve, and episodes of severe decompensation would occur. The child’s primary physicians communicated that there were options for further cardiac care. Thus, home ventilation wasn’t appropriate even though the respiratory support itself was relatively low.

Alternatively, we have accepted children with bronchopulmonary dysplasia on fairly high ventilator settings and significant CO2 retention. These children are older (often 9 to 12 months), well past their acute phase of respiratory failure, clearly have severe fibrotic lung disease that will take considerable time to improve, and have a long period of observation in the neonatal ICU such that stability has been demonstrated.

One should realize that home ventilation isn’t required for any child. Other venues (usually institutional) exist for long-term ventilation. The primary goals are stability, growth and recovery. This usually, but not always, can be accomplished in the home.

BASIC REQUIREMENTS

If the child is a good medical candidate, a social worker conducts a psycho-social and environmental evaluation. The home must have a telephone, heat, air conditioning, electricity, running water and transportation.

At least two responsible adults in the home must be capable of handling the tasks needed for home ventilation and willing to commit 24 hours per day to a task that will continue for at least several years. At all times, one of the adults must be in the home. Everyone independently caring for the child must complete home ventilation training.

Next is a meeting between the family and the home care team. The aspects of the program and training are discussed. Ideally, the family will take some time to consider the commitment they plan to make before proceeding with the program. If the family decides to proceed, then other steps are needed.

There also must be an upfront commitment from the insurance provider to support the program. The biggest issue for the insurance provider is home nursing. We require 24 hours per day at the time of discharge with an expectation that this will be available for two weeks then incrementally decreased to a minimum of 12 hours per day.

THE BIGGEST SAFETY FACTOR

We have never accepted a child into the home ventilation program without a commitment to this support. The reason is quite simple. For the child dependent on technology, the biggest safety factor is monitoring. Monitoring isn’t a monitor (like a pulse oximeter); monitoring is having a reasonably skilled individual with the child in continuous line of sight.

In a common arrangement, one parent works outside the home generating income, and the other parent works inside the home caring for the child. The 12 hours of nursing allows the parents to sleep and conduct normal household tasks such as bathing (that’s right, if parents can’t see the child they can’t sit in a bath).

In most regions, it isn’t reasonable to expect most home nurses have PICU or NICU experience. The number of nurses who would be needed with these highly focused skills is simply not available, especially in smaller communities. While the nurses are very competent providers, it’s considered absolutely acceptable, and even expected, for the nurse to arouse the parents from sleep to come deal with problems related to home ventilation. We emphasize this concept of continuous monitoring.

All it takes is a few changes — an adult decides to move from the home, a few nursing shifts are uncovered (this is expected to happen on occasion) — and suddenly a dedicated solo parent is trying to make it alone. Even if the child has been stable for some time, that might be the moment when the tracheostomy tube becomes dislodged. With no one to recognize and correct this immediately, an irreversible tragedy has occurred. Thus we maintain an unwavering requirement for two parents and home nursing to ensure adequate monitoring.

PREPARING FOR DISCHARGE

When a decision is made to begin the home ventilation program, the child is changed to the home ventilation system, observed in the ICU for stability, and transferred to the regular inpatient ward. The family has the learner’s manual that contains generic information as well as information specific to their child. The educators follow the teacher’s manual.

There are lists of skills under certain topics such as tracheostomy care that must be learned then re-demonstrated. Proficiency is documented by direct observation of two members of the home care team, each of whom will sign off on that task as being completed.

It isn’t surprising that this education process usually takes one to two months and sometimes longer. In our current health care environment, we’re often pressured to say exactly how long. But this comprehensive educational process for the family isn’t unlike other education processes such as college. A person continues education until the task is completed to adequate achievement (in college to a certain number of credits, in home ventilation to mastery of all the needed skills).

This initial educational process is the basis for fairly complex care important to the child for a period of a few years to a lifetime and shouldn’t be rushed. It’s expected that the duration of home ventilation training for the family will vary depending on how many education contacts are possible per week (easier for parents in town versus those who live at a distance) and how fast the parents achieve competence. Parental attitude is as important as previous educational status.

As training nears completion, the family is given total responsibility for the child’s care (as much as allowed under hospital policy) for a period of three to seven days. During this time, the family will do transport of the child around the hospital. These are the last steps prior to discharge.

You can imagine that discharge to home is a big event and potentially quite stressful for the family. It helps tremendously to have the home nurse and respiratory therapist meet the family as they arrive. We generally see the family in clinic at two to four weeks after discharge and then monthly. The visits are gradually extended to every three months.

The process I have described has gone extremely well. Noah was the first child to enter our home ventilation program. He began home ventilation when he was 9 months old. By 4 years, he was free of both ventilator and tracheostomy tube. Now I see Noah most often in the halls as he goes to other clinics, and he no longer requires routine care in our pulmonary center.

Resources

1. American College of Chest Physicians. Mechanical ventilation beyond the intensive care unit: report of a consensus conference of the American College of Chest Physicians. Chest. 1998;113(5):289S-344S.

2. American Thoracic Society. Statement on the care of the child with chronic lung disease of infancy and childhood. Am J Respir Crit Care Med. 2003;168(3):356-96.

3. Core guideline for the discharge home of the child on long-term assisted ventilation in the United Kingdom. Thorax 1998;53(9):762-7.

4. Panitch HB, Downes JJ, Kennedy JS, et al. Guidelines for home care of children with chronic respiratory insufficiency. Pediatr Pulmonol. 1996;21(1):52-6.

James Royall, MD, is board certified in pediatrics, pediatric critical care, and pediatric pulmonology. He’s currently the C.R. Anthony Professor and chief of pediatric pulmonology at the University of Oklahoma Health Sciences Center, Oklahoma City, where he’s also director of the Oklahoma Cystic Fibrosis Center and director of the Pediatric Home Ventilation Program of Oklahoma.

Acknowledgment: Much credit for development of the Pediatric Home Ventilation Program of Oklahoma goes to: Cynthia Gray, BS, RRT, RCP, vice president of respiratory care, CV Medical Solutions, and her staff, Yolanda Sellers, RRT, RCP, and Shirley Gresham, RRT, RCP; to Julia Vana, ARNP, and Prue Armstrong, CRT, RCP, from the pediatric pulmonology section; and to the Oklahoma Health Care Authority for cooperative discussion about support for home care.

Table 1: Common causes for mechanical ventilator dependence in children

Primary Cardiopulmonary Processes

Bronchopulmonary dysplasia

Other post-inflammatory fibrotic lung processes

Severe congestive heart failure (usually also some pulmonary disease)

Airway abnormality (usually malacia or stenosis of lower trachea or bronchi)

Limited Ventilation From Other Processes

Central nervous system or spinal cord dysfunction

Phrenic nerve paralysis

Neuromuscular weakness (e.g., muscular dystrophy, spinal muscular atrophy)

Thoracic cage defects (e.g., asphyxiating thoracic dystrophy, spondylothoracic dystrophy)

Table 2: General guidelines for the medical status of children acceptable for home ventilation