Section 4: Home monitoring and follow-up of home-ventilated children

This article is related to:

Pediatric home mechanical ventilation: A Canadian Thoracic Society clinical practice guideline executive summary

Introduction

Children receiving home mechanical ventilation (HMV) are at risk of acute deterioration and adverse events.^1–8 Although caregivers represent the first-line monitoring for the child to identify acute events that could be potentially life threatening, monitoring equipment is often used. This section reviews the literature on the use of home monitoring. The objective of the review is to focus on the use of external monitoring devices; however, as ventilators used in the home environment have their own internal alarm system to alert caregivers, we will also briefly review this topic.

Randomized controlled studies evaluating the usefulness of monitoring devices do not exist and will be very difficult to pursue; therefore, consensus or expert opinion is the highest level of evidence available. In addition, studies pertaining to monitor use in children receiving HMV with evaluation of benefits and pitfalls are likely scarce. To gain an insight on the potential need for monitoring at home for these complex children, it is therefore important to know the incidence of adverse events (morbidity and mortality).

Results

We retrieved 95 publications that were relevant to our inclusion criteria. After eliminating publications that dealt only with in-hospital data and publications that had no details on mortality or cause of mortality, we were then left with 30 publications dealing specifically with causes of death in cohorts of infants and children on HMV; there were four publications with data on morbidity. Most of these studies on mortality and morbidity on HMV in children represented the experience of a single institution but some related the experience of a state, a province or a whole country. We found no publications on the topic of monitoring (by healthcare personnel or with external devices) and HMV in children. Home monitoring was mentioned in 20 publications concerning long-term follow up or outcome of cohorts of patients on HMV.

Mortality data

Data on tracheostomy- or ventilator-related deaths occurring at home in children on HMV originated from 13 countries (Australia, Canada, Germany, Italy, Japan, Korea, Portugal, Serbia, Taiwan, The Netherlands, Turkey, the UK and the USA) (Table 1).^{1,3–7,9–32} One study presented data on mechanical ventilation starting in the 1960s; there were five studies with data starting in the 1970s, six starting in the 1980s, eleven starting in the 1990s and three starting in the 2000s; the years of the study was not mentioned in three. Eighteen studies spanned more than one decade. None of the studies spanning more than one decade reported the deaths per decade; therefore, we cannot determine whether the risk of death that is tracheostomy- or ventilator-related is changing over time.

Table 1. Literature review: Unexpected deaths of infants and children on HMV.

Author (Year)	Type of study	Country of study	Years of study	N patients at home	N on invasive ventilation	Total number of deaths	Unexpected deaths at home	Tracheostomy deaths	Ventilator associated deaths	Tracheostomy & ventilator related deaths (%)
Frates (1985)^15	Retrospective cohort, one institution	USA	1962–1983	54	33	17	9	0	4	12.1
Oren (1987)^23	Retrospective, case series	USA	NA	6	6	0	0	0	0	0
Iannaccone (1988)^19	Retrospective cohort, one institution	USA	1979–1986	7	4	4	0	0	0	0
Robinson (1990)^26	Point survey	UK	1983 & 1987	9	NA	1	1	0	0	0
Fields (1991)^14	Retrospective, regional cohort	USA	1984–1988	23	23	6	2	1	0	4.3
Marcus (1991)^20	Retrospective, case series	USA	NA	11	11	0	0	0	0	0
Canlas-Yamsuan (1993)^10	Retrospective cohort, one institution	Canada	1979–1992	22	22	7	2	0	0	0
Wheeler (1994)^30	NA, likely retrospective cohort	USA	1990–2000	44	44	4	0	0	0	0
Simonds (2000)^1	NA, likely retrospective cohort	UK	NA	40	40	2	0	0	0	0
Sasaki (2001)^27	Retrospective cohort, one institution	Japan	1990–2000	14	14	0	0	0	0	0
Appierto (2002)^9	Retrospective cohort, one institution	Italy	1985–2001	46	34	7	1	0	0	0
O'Brien (2002)^21	Retrospective cohort, one institution	USA	1992–1998	19	19	0	0	0	0	0
Gilgoff (2003)^16	Retrospective cohort, one institution	USA	1980–2000	39	39	8	5	0	2	5.1
Edwards (2004)^12	Retrospective cohort, one institution	UK	1995–2002	27	27	7	NA	0	0	0
Gowans (2007)^17	Retrospective cohort, one state	USA	1996–2004	77	77	13	NA	0	0	0
Oktem (2008)^22	Retrospective cohort, one institution	Turkey	2001–2006	34	34	11	0	0	0	0
Pekcan (2010)^24	Retrospective cohort, one institution	Turkey	2003–2007	27	14	11	0	0	0	0
Tibballs (2010)^29	Retrospective cohort, one institution	Australia	1979–2008	102	48	36	2	0	1	2.1
Edwards (2010)^13	Retrospective cohort, one institution	USA	1977–2009	228	228	47	14	9	0	3.9
Reiter (2011)^25	Retrospective cohort, one institution	Germany	1983–2006	54	45	4	2	1	1	4.4
Goodwin (2011)^3	Retrospective regional cohort	UK	1994–2009	106	43	11	NA	0	0	0
Paulides (2012)^4	Retrospective cohort, one institution	The Netherlands	1979–2009	197	100	43	NA	0	2	2.0
Sovtic (2012)^28	Retrospective cohort, one institution	Serbia	2001–2011	29	11	7	0	0	0	0
Hsia (2012)^18	Retrospective cohort, one institution	Taiwan	1998–2006	21	21	2	0	0	0	0
Amin (2013)^6	Retrospective cohort, one institution	Canada	1991–2011	379	66	55	NA	5	0	7.6
Cristea (2013)^11	Retrospective cohort, one institution (BPD)	USA	1984–2010	91	91	19	NA	2	0	2.2
Com (2013)^5	Retrospective cohort, one institution	USA	1987–2006	91	91	15	5	0	1	1.1
Kherani (2015)^7	Retrospective cohort, one institution	Canada	1991–2014	51	26	2	1	0	0	0
Cancelina (2015)^31	Retrospective cohort, one institution	Portugal	1993–2013	31	6	6	NA	0	0	0
Han (2015)^32	Retrospective cohort, one institution	Korea	2000–2013	57	48	13	7	1	0	2.1

There were 1936 children cared for at home in the studies reporting on the cause of death, and 1265 of these children (65.3%) were on invasive mechanical ventilation (tracheostomy and ventilator). A total of 358 children died, the cause of death being most often from progression of disease or from an acute illness. From the 23 studies for which we had data on unexpected death, 24.1% of all deaths occurred unexpectedly at home. Nineteen of the 51 unexpected deaths were related to the tracheostomy (tracheal obstruction, false channel, tracheal bleed) and 11 deaths were ventilator-associated (accidental decannulation or ventilator disconnection in all except one, which was due to a power failure). The 30 deaths that were tracheostomy- or ventilator-related represented 2.4% of the children who were on HMV but 8.4% of the total number of deaths — a significant proportion. None of the studies was very explicit regarding the exact circumstances of death and the use of monitoring (nursing personnel or external monitor use) at the time of death. For children on NIV a rare but potential cause of death is displacement of the mask.⁶ Monitoring by caregivers is essential to avoid this preventable cause of death.

Morbidity data

All patients on HMV can have deterioration due to their underlying disease. Children old and strong enough should be able to alert their caregivers, but unexpected events can occur. Usually, well-trained caregivers will be able to identify clinical deterioration and they should be adequately trained to respond to emergencies.

Three publications^25,33,34 provided data on emergencies experienced by caregivers of children on invasive mechanical ventilation at home (Table 2). The studies differed with respect to outcomes and study duration. One study was solely on children and spanned 23 years.²⁵ There were 68 severe emergencies reported (0.2 per patient-year). Respiratory causes were found in 48 of these 68 emergencies; 15 were tracheostomy-related, and three were ventilator failures. These events led to four deaths and significant morbidity in one patient (seizures). One additional study, from Australia,²⁹ reported serious adverse effects on 48 children on invasive HMV over a 29-year period. In the four studies reporting on morbidity, a description of how the children on invasive HMV were monitored was generally not provided. It was the clinical condition or the ventilators alarms that alerted the patient or caregivers to a potential problem. Two studies highlighted the fact that in many cases of perceived malfunction of the ventilator, no malfunction was found after thorough evaluation and a clinical deterioration was likely responsible.^33,34 Only one study²⁵ reported a death due to ventilator disconnection that did not trigger an alarm; the authors reported that the pulse oximeter was not used in view of “too many false alarms in the past.”

Table 2. Literature review on morbidity.

Author (Year)	Population	Study duration	Method	N of children	N of emergencies	Adverse outcome
Srinivasan^34 (1998)	Program in the greater Los Angeles area, USA	1 year	Review of all emergency calls to the program for ventilator failure	44/150	189 reported cases, 73 were real ventilator failures	None reported, but 2 hospitalizations
Chatwin^33 (2010)	Program of the Royal Brompton Hospital, London, UK	6 months	Prospectively manually entered data from the telephone helpline calls concerning ventilator failure	96/1211	86 calls to emergency help line, 188 emergency visits at home	None reported, 13 patients with no failure identified had clinical deterioration
Reiter^25 (2011)	HMV program in Munich, Germany	23 years	Questionnaire, review of admissions, review of emergency calls	54/54	68 severe emergencies, 15 tracheostomy related, 16 ventilator failures	4 deaths, 1 significant morbidity

Monitoring

Monitoring by health professional

Most programs emphasize the need for support to caregivers. This is usually provided, in most programs, through nursing. Consensus statement and clinical practice guidelines do make recommendations about the need for nursing assistance to caregivers and the need for appropriate training to respond to alarms and emergency situations.^35–37

In the literature reviewed, 15 publications^{9,11–17,19,22–25,30,38} give data regarding the availability of health professionals but details were incomplete for three.^12,14,22 There were three studies that mention there was no help available to parents;^19,22,24 four mentioned full-time availability of nursing personnel, but usually only for a minority of families.^15,16,25,30 In general, only part-time nursing (at night) was available.^{9,11,13,17,38}

Use of external monitors

There is no study evaluating the usefulness of external monitors. There are, therefore, no evidence-based recommendations on the use of external monitors for HMV. The American Thoracic Society (ATS) statement ‘Care of the Child with a Chronic Tracheostomy’, published in 2000, recommends that children at high risk of airway complications could benefit from external monitors.³⁹ Its authors stressed that while both cardiorespiratory and end tidal CO₂ monitors alert the caregivers to respond to their child, the delays in the response time are unacceptable. They do not comment about the use of pulse oximeters. The report of the 1998 consensus conference on mechanical ventilation published by the American College of Chest Physicians⁴⁰ recommended the use of external monitors for patients with absent or severely impaired spontaneous breathing efforts. Similar recommendations have been put forward for children on HMV in the UK.³⁵ The ATS consensus statement ‘Respiratory Care of the Patient with Duchenne Muscular Dystrophy,’ published in 2004, recommends the use of pulse oximeters for patients with tracheostomy to detect mucus plugs.⁴¹ Recent textbook chapters also highlight the lack of evidence-based data and do not recommend monitoring for all patients.^42,43 There appears to be some consensus however, that external monitoring should be used if it is known that the child will not awaken or breathe spontaneously if the ventilator fails or becomes disconnected, or if the child has significant airway obstruction.

Published large series from different countries report that children on HMV are on external monitors, but details are scarce. In some programs, including that of Munich, Germany,²⁵ the Children’s Hospital of Los Angeles, USA,¹⁸ and the Hospital for Sick Children in Toronto, Canada (invasive and noninvasive ventilation),⁷ all patients on invasive mechanical ventilation are on pulse oximeters. For other programs,^2,44 although the proportion of children under monitoring is at or near 100%, other means of monitoring are used (cardiorespiratory monitors, end-tidal CO₂ monitors, continuous direct observation by a nurse), although to a much lesser degree than programs using pulse oximeters.

Monitoring of oxygenation status is, in theory, better than monitoring with ECG and thoracic movement, as obstructive events will not be detected with monitors that use variations in thoracic movement for respiration detection, and significant hypoxemia will precede bradycardia.⁴⁵ The old generation of oximeters was, however, infamous for false alarms.⁴⁶ The new generation of oximeters triggers fewer false alarms. There is some Canadian literature supporting the very low rate of false alarms experienced by parents with the new-generation oximeters.⁴⁷ Although pulse oximeters are theoretically well suited as external monitors for children on invasive home ventilation, there is still no evidence showing that monitoring will decrease mortality or morbidity.

There are also no studies evaluating the alarm limits to be used to ensure timely intervention by the caregivers while avoiding the non-significant decreases in oxygenation that trigger repeated alarms. Ultimately these limits may be very patient-specific and difficult to generalize.

Discussion

One might find it surprising that accidental decannulation or ventilator disconnection are the most frequent causes of unexpected death in the publications reviewed and that these events were not detected by the low-pressure alarm on the ventilator. It has been reported that during disconnection from the ventilator, the ventilator hub might be sufficiently obstructed by the patient or by bedding material to allow creation of enough back pressure not to trigger the alarms.⁴⁸ This may also be the case with smaller tracheostomy tubes. Indeed, one study simulated tracheostomy tube decannulation in an experimental setting with tracheostomy tube ranging from 3.0 to 6.0 mm in internal diameter.⁴⁹ These investigators showed that the low inspiratory-pressure alarm was not triggered during simulated decannulation with small tracheostomy tubes (<5.0 mm). The authors suggested setting the low-inspiratory-pressure alarm at 4 cm H₂O below the desired peak inspiratory pressure; in their study, this improved the ability of the alarms to detect simulated decannulation, although decannulation was not always detected.

Because of the number of reported alarms for ventilator failure in the available studies (even if there was no failure eventually discovered), most large programs of HMV for children advocate the availability of a second ventilator for any child who is unable to cope for six hours off the ventilator.^34,40 Battery-operated ventilators or the use of a generator is recommended to overcome the problem of power failure.

It is important to understand, on one hand, the quality of the information derived from external monitors and, on the other hand, the burden to a family who uses these devices. There is now experience with more than 40 years of monitoring with cardio-respiratory monitors (respiratory movement by transthoracic impedance and heart rate through ECG) in the home environment for prematurely born infants or for infants judged at risk of severe apnea.⁵⁰ The use of cardio-respiratory monitors has taught us many lessons: the repeated alarms, which are often false, disrupt family life^51,52 and monitors are not a protection against death.^53,54 Prolonged episodes of hypoxemia can occur without being detected by the cardio-respiratory monitors.^45,55 In addition, compliance is often an issue.⁵⁶ We have also learned that cardio-respiratory monitors with downloadable memory, which allow for the review of events, are very useful to judge the clinical conditions during events and the validity of the triggered alarms.^57–59 This body of experience should be considered in the decision to use or not external monitors.

Conclusion

Most large studies reported mortality due to accidental decannulation, ventilator disconnection or tracheostomy-related deaths. Home surveillance by health professionals, when available, and the use of external monitors are strategies to potentially decrease mortality and morbidity not due to progression of the underlying condition. It is, however; still not clear whether these deaths can be prevented by using home surveillance and monitoring devices.

Research questions — Monitoring

1. What is the severity (perceived by the caregiver vs. documented) of the deterioration in clinical status at the time alarms are triggered on the ventilators or on external monitors?

2. What is the optimal and least burdensome way to monitor a child receiving home ventilation in order to minimize adverse events?

Recommendations for home monitoring in children using long-term mechanical ventilation at home

1. An awake and alert trained caregiver 24 hours a day and 7 days a week will provide the first-line monitoring of a child receiving ventilation in a situation where interruption of ventilation is anticipated to lead to an adverse event, including all invasively ventilated children as well as those on NIV that are medically fragile (at risk of imminent adverse events if the NIV mask is displaced or equipment malfunctions). (Consensus)

2. The built-in alarms of the home ventilators should be adjusted for each patient to maximize safety and minimize nuisance alarms. (Consensus)

3. The low minute ventilation alarm and disconnect alarm should be set optimally to maximize the detection of circuit disconnection and decannulation. (Consensus).

4. For children receiving invasive ventilation via tracheostomy at home through a tracheostomy tube that is smaller than 5 mm ID, an external monitor should be used in view of the potential failure of internal monitors to detect circuit disconnection and decannulation, in addition to an awake and alert trained caregiver. (GRADE 1C)

5. Patient not able to awaken, breathe spontaneously and/or at risk of adverse events if disconnected from the ventilator should have an external home monitor in addition to an awake and alert trained caregiver. (Consensus)

6. Home cardio-respiratory monitoring with respiration detected with transthoracic impedance techniques should not be used as external monitors as they fail to detect obstructive events. Similarly, caregivers should not rely on the detection of bradycardia with these monitors because bradycardia is a very late event. (GRADE 1C)

7. Pulse oximeters with downloadable memory should be the monitor of choice for patients identified as needing an external home monitor. (Consensus)

8. Recurrent alarms should be dealt with promptly to correct the problem, whether equipment-related or patient-related. (Consensus)

9. Routine periodic maintenance of the home ventilators and monitoring equipment is recommended as per the manufacturer’s recommendations.

Follow-up of home-ventilated patients

Introduction

Follow-up care entails the reevaluation of the child on HMV, including assessment of clinical status, effectiveness of ventilation and evaluation of proper functioning of equipment. Follow-up care can be done through a combination of clinic visits, home evaluation and telemedicine.

The frequency of follow-up visits for children on HMV will depend upon many factors including: the baseline disease and its severity, the stability of the child, the expected natural course through the disease process, the distance between home and the follow-up facility, the availability of follow-up care in the community (physicians and other health professionals) and the support parents are receiving for the care of their child (nursing support in the home).

Literature review

In the few published statements from health authorities and in clinical practice guidelines,^35,36,39,60 we found no clear evidence-based recommendations concerning the frequency and content of follow-up visits and evaluation of effectiveness of ventilation for children on HMV. Therefore, any recommendations will be based on consensus among experts.

Data and discussion

Follow-up visits

Clinic visits

It is usually suggested that the first assessment occur in the first month and no later than three months after discharge home. One recent study⁶¹ suggested close follow-up (within one month) or delay of initial discharge, for infants and children who had changes in their management close to their discharge date. Indeed, in that study, 42% of the children who had an unplanned re-admission within one month of discharge home had a pre-discharge recent management change (change in ventilator settings; change in chronic medication; antibiotics initiation; change in their tracheostomy tube size).

After the first visit, the interval between assessments will depend upon the status of the child and the disease process. A few reviews and practice guidelines have suggested a global reassessment every six to twelve months.^35,36 Many infants and young children may need more frequent assessments. Additional unscheduled visits might be necessary in case of deterioration of clinical status or in case of frequent ventilator or external monitor alarms of unclear cause.

During clinic visits, access the internal ventilator data provides information concerning compliance and effectiveness of ventilatory therapy. When a memory card for the ventilator and any other monitoring equipment is available, it should be brought and the data accessed. It should be noted, however, that no studies have evaluated whether the information retrieved alters management or outcomes. As recurrent alarms (especially if they are perceived as false alarms) can desensitize the caregivers to the importance of the monitoring system, it is essential to ask the caregiver about alarms on the ventilator or other devices used. For children on invasive HMV, the ATS statement ‘Care of the Child with a Chronic Tracheostomy,’ published in 2000,³⁹ gives some recommendations for routine evaluation by rigid or flexible bronchoscopy every six to 12 months to assess the airways and detect and treat complications. From a practical perspective, evaluations are typically limited to scenarios where symptoms or signs suggest an airway issue

Home visit

We found no publication in the available literature comparing clinic visits to home visits for follow-up care. Many centers have teams of professionals (physicians, nurses, respiratory therapists, for instance) that can visit the child at home between hospital visits. Other families receive services from homecare companies (but insurance coverage is needed).

Evaluation of the effectiveness of ventilation

Again, there is no published evidence on the optimal ways to evaluate the effectiveness of ventilation in children receiving HMV. The available recommendations are from consensus statements;^36,40,60 two publications have evaluated the frequency of changes in ventilator settings following recordings done in hospital or at home.^62,63

For the assessment of effectiveness of ventilation, it is usually recommended that, at minimum, oxygen saturation (SpO₂) and CO₂ levels should be recorded. It is important that the overnight assessment include CO₂ levels, as normal oxygen saturation is not necessarily an indication of effective ventilation.^40,63 The same evaluation needs to be done following an admission to the hospital for acute deterioration and where parameters were changed.

Some children with nighttime ventilation only, for various conditions that might deteriorate progressively with time, will need assessment of effectiveness of ventilation and gas exchange during the daytime as their disease progresses.

There seems to be a consensus that regular assessment of the effectiveness of ventilatory support and gas exchange can be done at home. The advantage of home assessment is that the child is in his usual sleeping environment for the study. Stores et al.⁶⁴ reported better sleep quantity and quality at home in a sample 60 children age 5–16 years. Felemban et al.⁶⁵ compared recordings performed in the hospital and at home in 29 pairs of recording on children with noninvasive ventilation. SpO₂ and transcutaneous CO₂ values did not differ significantly between the hospital and the home. This study showed that parents are able to perform these studies quite well. In many areas, however, either homecare companies or personnel of the HMV program are in charge of such studies, decreasing the burden on the caregivers.

Performing polysomnography for reassessment of effectiveness of ventilation is usually recommended on a six-month or yearly basis.⁶⁰ A study by Tan et al.⁶² reported changes in the settings in 66% of children on respiratory support (only 35% of these children were on HMV). Our review of the experience published by large centers showed that not all are doing polysomnography,^6,44,66,67 and the tests are not done regularly on all patients. This could be due in part to the availability of a sleep laboratory or to the reliance to home SpO₂ and CO₂ studies.

Telemedicine

We could not identify strong evidence on the use of telemedicine for follow-up care of children on HMV. Only three publications reported the telemedicine experience in such children^68–70 Even in adults, evidence is scarce. There were two systematic reviews on telemedicine that provided some important conclusions; one was solely on patients with diabetes⁷¹ and the other presented data on four conditions, including pulmonary conditions.⁷² Recent randomized trials are also available for the adult population on HMV^73-75 and there is also a randomized trial with children afflicted with major congenital heart disease.⁷⁶

These studies were often limited to small samples, and the follow-up care was of short duration. For studies dealing with HMV in adultS, telemedicine compared favorably to clinic visits and was acceptable to the patients and families, as well as being user-friendly.^73,75,77 It decreased admissions, clinic visits and emergency room visits^73–75,77 and was cost-effective.^74,75 A recent small pilot study from Boston Children’s Hospital in the US enrolled 14 patients followed by telemedicine, of whom nine were children on HMV (invasive and noninvasive).⁷⁰ The study showed a high degree of acceptance by the families and felt that the use of telemedicine avoided clinic and emergency room visits.

The use of telemedicine for follow-up care of home-ventilated children dates back to the 1990s⁶⁸ and has now been implemented in many centers to reach patients and families living in remote areas, or to decrease the number of clinic visits. These telemedicine ‘visits’ involved real-time discussion with the patient, the family and professional caregivers, if available. In addition, there have been several initiatives of transmission through telemedicine of pulse oximetry and clinical data gathered by patients or their family, though mainly in adults.^78–81 There are also reports on transmission of more complex data such as the download from bi-level ventilators for follow-up of adult patients.^82,83

Ideally, however, data that are used in the hospital setting for monitoring the effectiveness of ventilation and the child’s health status is the same data that should be accessible during follow up. The best method to access the data may be through transmission directly from the home. This data could include not only SpO₂ and CO₂ recordings, but also ventilator flow loops as well as inspiratory and expiratory volumes — parameters that help evaluate the patient’s current pulmonary mechanics. If data from the ventilator could be obtained through telemedicine, changes would likely be detected earlier, allowing for intervention and, potentially, avoidance of hospital re-admission. Remote ventilator interface tools have been developed for home use, some with real-time accessibility over a secure wireless Internet connection. So far, however, many of these systems have been used only with a patient simulator^84,85 or only in a pilot study.⁸⁶ One study by Zhou et al. from China⁶⁹ reported on the feasibility of a remote system that transfers information on status and performance of the ventilator to an Internet server via a land-line phone cable. The 14 patients in that study were in the pediatric age group. The authors reported caregiver and physician satisfaction with the system and, in several instances, the use of this technology decreased the number of clinic visits.

Conclusion

The frequency and content of follow-up care to be provided by an HMV team is dependent on multiple factors, including: the age of the child, the disease process, presence of co-morbidities, access to competent community-based providers, family caregiver capacity and the evolution of the clinical condition. The right combination of clinic and home visits will also be dependent on these factors as well as the resources available. Whether the evaluation of the effectiveness of ventilation is done at home or in hospital, the assessment needs to be done on a regular basis. Although in general, telemedicine is well accepted by patients and physicians, there is still no clear evidence that the use of telemedicine, despite its many advantages, actually decreases cost or improves outcome.

Research questions — follow-up care

1. What are the benefits (benefits to the family, financial benefits, improved outcome) of home visits vs. clinic visits for children on HMV?

2. What is the optimal use of telemedicine in terms of decreasing costs, avoiding clinic or emergency room visits or hospitalization, as well as providing a better outcome?

3. Does the use of telemedicine increase access to care for the HMV population both for those children using HMV but also for those not using it that may have resulting increased clinic visits?

4. What is the optimal interval for and content of follow-up care for children on HMV?

Recommendations for follow-up care for children using long-term mechanical ventilation at home

Given the paucity of published data and the lack of recent clinical practice guidelines, the following are consensus recommendations.

1. The first visit and the frequency of subsequent visits should be tailored to the child and family’s need. The first visit should occur shortly within three months after discharge home based on patient acuity and subsequent visits be at a minimum of every six months. (Consensus)

2. Effectiveness of ventilation should be assessed regularly. (Consensus)

   1. A polysomnogram (ideal) or an assessment of oximetry and capnography, if polysomnogram is not available, should be performed yearly.

   2. The home ventilator (invasive or noninvasive NIV) should be downloaded during clinic visits or at home to review the effectiveness of ventilation as well as to review the logs of alarms, their type and frequency, and to review adherence.

3. Quality of Life and/or other parameters of family and patient well-being should be regularly measured. (Consensus)

Disclosure statement

No potential conflict of interest was reported by the author(s).