Home mechanical ventilation (HMV) is a viable and effective treatment strategy for patients with chronic respiratory failure and has been associated with a survival benefit [
Health related quality of life (HRQL), constrained hospital resources, and increasing demands for prolonged mechanical ventilation have motivated providers, patients, and families to create a sustainable home environment for ventilatory assisted individuals (VAIs). While clinical practice guidelines to standardize the management of HMV have been developed, their implementation is uncertain [
While survival is indeed an important long-term outcome, use of HMV should also focus on additional patient-centered and system-level outcomes including HRQL, hospitalization needs, health resource utilization, and the role of caregivers. We therefore conducted a systematic review of the literature to examine all available studies evaluating at least one clinically relevant patient-centered and health resource utilization outcome in patients receiving HMV.
A comprehensive search of MEDLINE, EMBASE, CINAHL, and the Cochrane library (1996 to August 2013) was performed with an experienced librarian (see Supplementary Methods in Supplementary Material available online at
Two trained reviewers (Erika J. MacIntyre and Leyla Asadi) independently conducted an initial eligibility screen of all retrieved titles and abstracts. Studies were selected for full text review if they met the following criteria: (1) study design (original research, other than case reports); (2) population (cohorts with CRF; studies examining COPD exclusively were not included as the use of HMV in stable COPD is currently not recommended in Canada); (3) intervention (invasive tracheostomy ventilation (TV) or NIV); (4) setting (noninstitutionalized residence); and (5) outcomes (patient- and/or family-centered outcomes other than survival and measures of health resource utilization; physiologic measures including blood gases and pulmonary function were not considered patient-centered; initial screening was broad and selection subsequently narrowed to our specific outcomes of interest).
Full text review, data extraction, and methodological quality scoring were independently performed by the same two reviewers on standardized data collection forms for studies that met inclusion criteria. Disagreements between reviewers were resolved through discussion and if consensus could not be achieved, discrepancies were resolved by a third reviewer (S.M.B.).
Methodological quality was scored using a modified version of the Downs and Black checklist (see Supplementary Methods) [
All patient- and family-centered outcomes and health resource outcomes for this systematic review were determined a priori. Our primary outcomes included health related quality of life (HRQL) measured quantitatively by a validated HRQL assessment tool and hospitalization rates including number of admissions and days in hospital. Secondary outcomes consisted of family caregiver (FCG) burden defined by use of standardized or validated questionnaires, HRQL assessment tools or structured interviews grouped by domain, and health service utilization (including any interaction with a physician, allied health professional, or clinical test). Cost implications to either the system or families, sleep quality assessed by quantitative measures, and incidence of decannulation were also considered important secondary outcomes.
The database search generated 1371 citations, of which 36 met inclusion criteria. An additional 6 articles, identified through other sources, were included in the full text review for a total of 42 articles. German language articles (
Outline of study selection process. HMV, home mechanical ventilation; HRQL, health related quality of life.
After screening, we included 1 randomized control trial (RCT) [
Study characteristics by study design and year of publication.
Study | Year | Study design | Location |
|
f/u (mo) | Quality |
---|---|---|---|---|---|---|
Bourke et al. [ |
2006 | RCT | SC UK | 41 | 12 | 18 |
Tsolaki et al. [ |
2011 | BA | SC Greece | 91 | 24 | 15 |
Windisch et al. [ |
2008 | BA | MC Germany | 85 | 12 | 16 |
Dellborg et al. [ |
2008 | BA | MC Sweden | 35† |
9† |
13 |
Farrero et al. [ |
2006 | BA | SC Spain | 43 | 36 | 16 |
Gonzalez et al. [ |
2003 | BA | SC Spain | 16 | 3y | 8 |
Janssens et al. [ |
2003 | BA | MC Switzerland | 211 | 1–88 | 15 |
Nauffal et al. [ |
2002 | BA | SC Spain | 62 | 18 | 12 |
Hein et al. [ |
1999 | BA | SC Germany | 27 | 2–30 | 13 |
Bach et al. [ |
1998 | BA | MC USA | 684 | V | 13 |
Janssens et al. [ |
1998 | BA | SC Switzerland | 6 | 17–55 | 14 |
Chatwin et al. [ |
2010 | C | SC UK | 1211 | 6 | 14 |
Budweiser et al. [ |
2007 | C | SC Germany | 231 | 29 | 17 |
Marchese et al. [ |
2007 | C | SC Italy | 77 | V | 17 |
Evans et al. [ |
2012 | CSS | SC Canada | 12 | NA | 12 |
Chang et al. [ |
2010 | CSS | SC New Zealand | 45 | NA | 10 |
Fernández-Álvarez et al. [ |
2008 | CSS | SC Italy | 66 | NA | 10 |
López-Campos et al. [ |
2008 | CSS | MC Spain | 115 | NA | 15 |
Vitacca et al. [ |
2007 | CSS | MC Italy, Spain | 792 | NA | 13 |
Tsara et al. [ |
2004 | CSS | SC Greece | 50 | NA | 10 |
Kaub-Wittemer et al. [ |
2003 | CSS | SC Germany | 53 | NA | 5 |
Markström et al. [ |
2002 | CSS | SC Sweden | 91 | NA | 9 |
van Kesteren et al. [ |
2001 | CSS | SC Netherlands | 38 | NA | 8 |
Sevick and Bradham [ |
1997 | CSS | MC USA | 277 | NA | 9 |
Moss et al. [ |
1996 | CSS | MC USA | 50 |
NA | 6 |
Bötel et al. [ |
1997 | D | SC Germany | 16 | NA | 4 |
A total of 4425 patients were studied. There were three broad disease states contributing to CRF and establishment of HMV: NMD (patients,
Patient characteristics.
Study | Age (mean) | Male ( |
|
NMD ( |
RTD ( |
OHS ( |
Other‡‡ ( |
NIV ( |
|
---|---|---|---|---|---|---|---|---|---|
Bourke et al. [ |
63 |
24 | 41 | 41 | NA | NA | NA | 22 | |
Tsolaki et al. [ |
65† | 61 | 91 | 11 | 17 | 28 | 35 | 91 | |
Windisch et al. [ |
59† | 49 | 85 | 17 | 29 | 9 | 27 | 85 | |
Dellborg et al. [ |
63‡ | 15‡ | 35‡ | 12‡ | 21‡ | NA | 2‡ | 35 | |
58 |
4 |
11 |
7 |
4 |
0 |
||||
Farrero et al. [ |
77 |
20 | 43 | 9 | 26 | 8 | 0 | 43 | |
Gonzalez et al. [ |
57 | NR | 16 | NA | 16 | NA | NA | 16 | |
Janssens et al. [ |
63† | NR | 211 | 40 | 42 | 71 | 58 | 211 | |
Nauffal et al. [ |
50† | 33 | 62 | 27 | 35 | NA | NA | 62 | |
Hein et al. [ |
56 | 16 | 27 | 8 | 8 | NA | 11 | NR | |
Bach et al. [ |
NR | NR | 684 | 650 | 19 | 3 | 12 | 636 | |
Janssens et al. [ |
79 | 2 | 6 | 0 | 4 | 1 | 1 | 6 | |
Chatwin et al. [ |
46 | 642 | 1211 | NR | NR | NR | NR | 1199 | |
Budweiser et al. [ |
63 | 146 | 231 | 15 | 49 | 69 | 98 | 226 | |
Marchese et al. [ |
58 | 54 | 77 | 41 | 8 | 3 | 25 | 0 | |
Evans et al. [ |
45 | NR | 12 | 12 | NA | NA | NA | 0 | |
Chang et al. [ |
55 | 24 | 45 | 7 | 6 | 27 | 3 | 39 | |
Fernández-Álvarez et al. [ |
61 | 34 | 66 | 10 | 11 | 27 | 18 | 64 | |
López-Campos et al. [ |
62 | 58 | 115 | 18 | 45 | 37 | 15 | 115 | |
Vitacca et al. [ |
67 | 562 | 792 | 375 | 128 | NA | 289 | 634 | |
Tsara et al. [ |
61 |
NR | 50 | 15 | 0 | 6 | 29 | 44 | |
Kaub-Wittemer et al. [ |
61 | 42 | 53 | 53 | NA | NA | NA | 32 | |
Markström et al. [ |
59 | 40 | 91 | 49 | 13 | 0 | 29 | 55 | |
van Kesteren et al. [ |
34 | 24 | 38 | 34 | 4 | NA | NA | 12 | |
Sevick and Bradham [ |
45 | NR | 277 | 177 | 0 | 4 | 96 | 48 | |
Moss et al. [ |
59 | 34 | 50 | 50 | NA | NA | NA | 7 | |
Bötel et al. [ |
NA | NA | 16 | 16 | NA | NA | NA | NR | |
|
|||||||||
Aggregate data |
|
60 | 1880 | 4425 | 1687 | 481 |
293 | 748 | 3682 |
%†† | NA | 59 | 100 | 53 | 15 |
9 | 23 | 85 |
Overall quality was variable (Table
Eleven studies provided data on HRQL using one or more general or disease-specific tools, including the Short Form-36 (SF-36), Sickness Impact Profile (SIP), Severe Respiratory Insufficiency (SRI) questionnaire, Profile of Mood States (POEMS), Munich Quality of Life Dimensions (MLDL), Health Index (HI), Sense of Coherence (SOC) scale, Saint George Respiratory Questionnaire (SGRQ), and the Chronic Respiratory Disease Questionnaire (CRQ) (see Supplementary Results) [
HRQL was generally described as good. Five studies examined HRQL before and after HMV using the SF-36 [
Change in SF-36 domains at 12 mo with home ventilation compared to baseline across studies and disease states.
Disease state | Study |
|
Physical capacity | Mental capacity | ||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|
PF | RP | BP | GH | PCS | Vi | SF | RE | MH | MCS | |||
RTD | Windisch et al. [ |
29 | NC | NC | NC | NC | NC | + | + | + | + | NC |
Hein et al. [ |
8 | NC | NC | NC | NC | NR | + | NC | NC | + | NR | |
Nauffal et al. [ |
35 | NC | + | NC | NC | NR | NC | + | + | NC | NR | |
Tsolaki et al. [ |
17 | NR | NR | NR | NR | + | NR | NR | NR | NR | + | |
|
||||||||||||
NMD | Windisch et al. [ |
17 | NC | NC | NC | NC | NC | NC | + | NC | NC | NC |
Hein et al. [ |
8 | NC | NC | NC | NC | NR | NC | NC | NC | + | NR | |
Nauffal et al. [ |
27 | — | NC | NC | NC | NR | NC | NC | NC | NC | NR | |
Bourke et al. [ |
41 | NC | NC | NC | + | NC | + | NC | NC | + | + | |
Bourke et al. [ |
20 | NC | NC | NC | + | NC | + | + | + | + | + | |
Tsolaki et al. [ |
11 | NR | NR | NR | NR | NC | NR | NR | NR | NR | NC | |
|
||||||||||||
OHS | Windisch et al. [ |
9 | + | + | NC | + | + | + | NC | + | + | + |
Tsolaki et al. [ |
28 | NR | NR | NR | NR | + | NR | NR | NR | NR | + |
BP, bodily pain; GH, general health; MH, mental health; NC, no change; NMD, neuromuscular disorders; NR, not reported; OHS, obesity hypoventilation syndrome; PF, physical function; RE, role emotional; RP, role physical; RTD, restrictive thoracic diseases; SF, social functioning; Vi, vitality; +, statistically significant improvement; —, statistically significant deterioration.
Improved HRQL was more consistent across the mental compared to the physical domains. With respect to mental capacity scores, every study examining OHS or RTD reported improvement in at least one domain or summary score following the establishment of HMV. There was no evidence of a clinically important deterioration in any group. Physical component scores were more heterogeneous. In NMD, one study found deterioration in the domain of physical functioning and improvement only seen in the item of general health in a single study; otherwise there was no change. The majority of the studies examining the remaining disease states reported benefit in at least one physical domain and/or overall physical component score.
The remaining 6 studies were of mixed design and applied different HRQL tools [
Nine studies (36%) examined hospitalization rates following the institution of HMV (patients,
Hospitalization rates before and after HMV.
Study | Disease state |
|
Before |
After† |
---|---|---|---|---|
#admissions/y | #admissions/y | |||
Windisch et al. [ |
NMD, RTD, COPD, OHS | 85 | NR | 0.1 |
Nauffal et al. [ |
NMD | 27 | 1.1 (1.2) | 0.3 (1.2) |
RTD | 35 | 1.2 (1.8) | 0.8 (1.2) | |
Farrero et al. [ |
NMD, RTD, OHS | 43 | 2.2 (2.4) | 0.5 (0.6) |
Bach et al. [ |
NMD, RTD | 654 | 1.5 (2.5)‡ | <0.6 |
Vitacca et al. [ |
NMD | 375 | NR | 0.5 (0.4) |
RTD | 128 | NR | 0.8 (0.5) |
|
|
||||
d in hospital/pt/y | d in hospital/pt/y | |||
|
||||
Tsolaki et al. [ |
NMD | 11 | NR | 2.4 (NR) |
RTD | 17 | NR | 0 | |
OHS | 28 | NR | 3.8 (5.7) | |
Janssens et al. [ |
NMD, RTD | 77 | 22 ( |
17 ( |
OHS | 32 | 26 ( |
17 ( |
|
Gonzalez et al. [ |
RTD | 16 | 10.9 (13.3) | 0 |
d, day; pt, patient; NMD, neuromuscular disorders; NR, not reported; OHS, obesity hypoventilation syndrome; RTD, restrictive thoracic diseases; y, year.
Family caregiver (FCG) characteristics are presented in Table
Primary caregiver characteristics.
Study |
|
Age (mean) | Male |
Spouse |
Parent |
Child |
Employed ( |
Would choose HMV again ( |
NIV ( |
---|---|---|---|---|---|---|---|---|---|
Studies included caregivers of patients with NMD and RTD only | |||||||||
Evans et al. [ |
12 | 55 | 3 (25) | 6 (50) | 5 (42) | 1 (8) | NR | NR | 0 |
Kaub-Wittemer et al. [ |
52 | 56 | 10 (19) | 51 (98) | 0 | 1 (2) | 34 (65) |
46 (88)‡‡ | 32 (62) |
van Kesteren et al. [ |
31‡ | NR | NR | 12 (39) | 19 (61) | 0 | NA | 29 (94) | 23 (74) |
Moss et al. [ |
36 | NR | 10 | NR | NR | NR | NA | 30 (83) | 7 (19) |
Aggregate data ( |
131 | 56 | 23 (23) | 69 (73) | 24 (25) | 2 (2) | NA | 105 (88) | 62 (47) |
|
|||||||||
Studies included caregivers of patients with NMD, RTD, and other conditions | |||||||||
Marchese et al. [ |
77 | NR | 15 (19) | 55 (71) | 18 (23) | 3 (4) | NR | 42 (55) |
77 (100) |
Fernández-Álvarez et al. [ |
20 |
51 | 5 (25) | NR | NR | NR | 14 (70) | NR | 19 (95) |
Tsara et al. [ |
50 | 48 |
NR | 21 (42) | NR | 20 (40) | 24 (48) |
NR | 44 (88) |
Sevick and Bradham [ |
277 | 53 | 69 (25) | 127 (46) | 111 (40) | NR | 119 (43)†† | NR | 48 (96) |
Aggregate data ( |
424 | 51 | 89 (24) | 203 (54) | 129 (34) | 20 (5) | 157 (45) | 42 (55) | 188 (44) |
|
|||||||||
All studies examining caregiver burden | |||||||||
Aggregate data total ( |
555 | 53 | 112 (24) | 272 (55) | 153 (31) | 25 (5) | 191 (48) | 157 (80) | 250 (45) |
HMV, home mechanical ventilation; NA, not assessed; NR, not reported.
Health resource use and the economic impact of HMV, beyond hospitalization, were assessed in 7 studies [
Crude cost estimates.
Study/disease state | Annual cost, mean and/or range |
Cost coverage | ||
---|---|---|---|---|
Home care | Home care + equipment | Out-of-pocket and/or lost wages | ||
Kaub-Wittemer et al. [ |
NR | NR | 3,624–29,942† |
All were eligible for federally funded nursing care |
|
||||
Sevick and Bradham [ |
116,222 | NR | 14,412 |
NR |
|
||||
Moss et al. [ |
208,738 | 15,829 |
91% privately insured | |
|
||||
Bötel et al. [ |
552,260 | 732,250 | NR | NR |
ALS, amyotrophic lateral sclerosis; COPD, chronic obstructive pulmonary disorders; NMD, neuromuscular disorders; NR, not reported; OHS, obesity hypoventilation syndrome; SCI, spinal cord injury.
All studies presenting data on sleep quality documented improvements in either overall sleep quality or sleep related symptoms including morning headaches, nocturnal dyspnea, and daytime somnolence with HMV [
In our scoping review we identified 26 unique studies reporting data on at least one patient-centered outcome other than survival in 4425 HMV users.
We found that HMV generally had a favorable impact on HRQL. Not surprisingly, improvement was more prominent and consistent for mental domains compared with physical domains across HRQL measures, particularly in those with NMD. We found HMV may be associated with an initially low and a subsequently reduced rate of hospitalization and days in hospital following implementation. While poorly described, FCG burden appears quite high. This likely relates in part to the financial strain associated with keeping VAIs in a home environment and may contribute to the unwillingness of some caregivers to choose HMV for their loved one again if given a second opportunity.
We believe our findings require thoughtful interpretation given that they are based on relatively few studies of mixed design and methodological quality describing small cohorts of heterogeneous patients. In general, pooled quantitative analyses could not be performed due to heterogeneity across studies, particularly in terms of the measures of HRQL, hospitalization requirements and caregiver burden that were applied. In addition, our findings are also likely confounded by concomitant cointerventions associated with entry into a multidisciplinary HMV program, such as improved medical attention, education, airway clearance strategies, home care, and provision of mobility aids [
For NMD, there are plausible reasons for the lack of improvement in HRQL association with initiation of HMV. First, many NMD are progressive and can be associated with rapid clinical deterioration. In ALS, HRQL is generally worse in those with bulbar muscle dysfunction and HMV may be of limited benefit [
We also examined several clinically important secondary outcomes. Family care givers were often younger, female, and of spousal or parental relation. Most FCGs were caring for loved ones receiving TV, suggesting a greater burden associated with invasive ventilation. Importantly, nearly one-fifth of all FCGs would not choose the option of HMV again, if given the opportunity, suggesting that the perceived burden is unmanageably high for many. It would seem that there are differences in caregiver perceptions of burden across disease states with burden being the least in caregivers of patients with NMD and RTD, particularly in the case of parents, slightly higher in ALS where the FCG is more often a spouse, and highest in caregivers of mixed cohorts. Moreover, these studies reinforce that FCG burden for HMV patients is poorly evaluated and understood.
There is limited data on the health economic implications of HMV; however, we can make several observations. There appears to be wide variation in the policies, procedures, and patterns of practice around the assessment, initiation, and maintenance of patients on HMV across health jurisdictions. Ambulatory service provision is necessary to maintain patients in a home environment, but this is currently not standardized. Some centers have specialized multidisciplinary home ventilation units that follow all VAIs, whereas in others follow-up can be infrequent and may not even involve a physician. The costs of maintaining VAIs at home are high; however, this is likely significantly less than the cost of long-term institutionalization. Many FCG suffered out-of pocket expenses and financial strain was highlighted in all 8 studies where caregiver burden was examined. This would strongly suggest that the current level of home support is inadequate.
No studies reported on decannulation. This is likely due to the following: (1) NIV use became the preferred first-line method of HMV by patients and providers for which weaning may be less relevant compared with invasive TV. Moreover, elective “ramping” of up to 24 hours of NIV may often obviate the need for invasive TV; (2) HMV patients are referred from chronic ventilation units where patients may have already failed to wean; and (3) in many patients, respiratory recovery is unlikely.
A prior systematic review aimed to evaluate the impact of HMV on hospitalization and sleep quality in NMD and RTD; however, the paucity of data limited the capacity for clear inferences [
Our study also has notable limitations. Firstly, small sample size, heterogenous study design, and variable methodological quality weaken the strength of inferences from our study. Secondly, our outcomes may have been influenced by methods of ventilation and compliance. Compliance is generally considered use for >4 hours/night, 5 nights/week, and while this was an item in our assessment of methodological quality, duration and usage were not fully taken into consideration. Thirdly, many of our outcomes were prone to bias, particularly survivorship bias. Fourthly, not all relevant patient-centered outcomes were considered and we acknowledge that not all outcomes are of equal value to patients and/or FCGs. Fifthly, there is heterogeneity within disease categories and our findings may not be applicable across all specific disease states. Lastly, as previously stated, concomitant cointerventions associated with entry into a multidisciplinary program confound and may exaggerate the beneficial effects of HMV.
The utilization of prolonged mechanical ventilation in CRF secondary to NMD, RTD, and OHS is increasing and likely represents a significant escalation in the complexity and cost of home care. Uncertainty surrounding optimal HMV service provision on patient-centered outcomes is an important knowledge gap and lack of evidence undoubtedly contributes to wide variation in clinical practice, including patterns of home ventilation initiation and titration, which may incur considerable costs to the health system. A large RCT evaluating HMV versus a nonventilated control would be challenging and perhaps unethical. We therefore will need to rely on high quality observational data and randomized trials of selected processes of care to further our understanding of the ideal method of HMV to optimize patient-centered outcomes. Then resources can be appropriately allocated by policy makers and providers along with a greater homogenization of standard practices to ensure high quality care is delivered to patients and their families.
In summary, our systematic review suggests that HMV likely provides quality of life benefit and reduced hospitalizations in patients with CRF secondary to NMD, RTD, and OHS. However, small sample sizes, heterogeneity in study design, and variable methodological quality weaken these inferences. With the preferential and proactive implementation of noninvasive over invasive ventilation, the utilization of HMV is likely to expand considerably. Future investigations are clearly needed to better understand the optimal methods for providing care for HMV patients, along with associated caregiver concerns and health economic implications.
Home mechanical ventilation
Noninvasive ventilation
Tracheostomy ventilation
Chronic respiratory failure
Bilevel positive airway pressure
Ventilator assisted individual
Chronic obstructive pulmonary disease
Neuromuscular disorders
Restrictive thoracic disease
Obesity hypoventilation syndrome
Health related quality of life
Family caregiver
Short Form-36
Sickness Impact Profile
Severe Respiratory Insufficiency questionnaire
Randomized control trial.
Sean M. Bagshaw holds a Canada Research Chair in
The authors declare that no potential competing interests exist with any companies/organizations whose products or services may be discussed in this paper.
Sean M. Bagshaw and Erika J. MacIntyre had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Erika J. MacIntyre contributed to the study design, literature search, retrieval of studies, data extraction, study quality assessment, and drafting of the paper. Leyla Asadi contributed to the retrieval of studies and study quality assessment. Doug A. Mckim contributed to the study design and drafting of the paper. Sean M. Bagshaw contributed to the study design, data extraction, study coordination, and drafting of the paper.
Regina Landeck, a certified member of the Association of Translators and Interpreters of Alberta, provided translational services for German articles to English. Elizabeth Dennet, an experienced librarian with the University of Alberta, provided assistance with development of the search strategy and the literature search. Dr. Bagshaw is supported by Canada Research Chair in Critical Care Nephrology and an Independent Investigator Award from Alberta Innovates-Health Solutions.