Section 6: Home ventilation in children with chronic lung diseases

Figures & data

Table 1. Summary of studies evaluating outcomes in children with bronchopulmonary dysplasia and related chronic lung disease on HMV.

Author (Year)	Study population /# of patients	Study type	Aim of study	Outcomes	Comments
El-Masri (2015)^1	N = 1 (out of 48 children with MRSA sepsis) on T + V	Retrospective cohort study	Describe outcomes of children with MRSA sepsis.	Patient survived
Reiterer (2015)^2	N = 1 on T + V (primary pulmonary lymphangiectasis and related CLD)	Case report	Describe outcome	Patient surviving as of 3 years, 7 months of age
Com (2013)^3	N = 91, 26 with CLD on T + V	Retrospective cohort study	Describe outcomes of T + V in children	Long-term outcomes of survival, decannulation/ventilator independence, and cognitive achievement were highest in the group with acquired chronic lung disease (100% survival), BPD without CNS insult (92% survival, 79% ventilator independence) than those with BPD with CNS insult (62% survival, 0% ventilator independence)
Cristea (2015)^4	N = 94 with BPD on T + V (overlap with cohort below)	Retrospective cohort study	Effect of living in a lower income neighborhood on mortality in BPD patients on T + V	15 patients died Median time to liberation from ventilation: 23–26 months Median time to decannulation: 34–39 months	Children from lower-income neighborhoods had a higher mortality rate. At least one death was due to accidental tracheostomy decannulation
Cristea (2013)^5	N = 102 with BPD	Retrospective cohort study	Program evaluation	81% alive, 19% deceased 67.6% weaned off ventilator (97.1% weaned before 5 years old) 58.8% decannulated (96.7% before 6 years old) Increase in rate of PPV for BPD from 1.23 per 100 000 live births in 1984 to 4.77 per 100 000 live births in 2010	No deaths were reported due to ventilator dysfunction, but the cause of 8/14 deaths was unknown.
Hsia (2012)^6	N = 139; 21 at home (remainder in “respiratory ward”), N = 10 with pulmonary disease (CLD, lung hypoplasia)	Retrospective cohort study	Examine status of children on long-term mechanical ventilation	(N = 21 at home, 19 invasive, 2 NIV, 6 with 24-hour support)
Downes (2007)^7	N = 1008; 454 with CLD	Retrospective cohort study	Program evaluation	44% long-term survival with continued ventilation, 34% weaned from ventilation, 18% mortality; ventilation rate for CLD increased from 36%–53%
Ramsey (2013)^8	N = 62; 38 with BPD on T + V	Retrospective chart review	Identify factors influencing wean from HMV	Weight gain (z-score) predicted decrease in weaning severity index score
Rane (2014)^9	N = 31 with BPD on T + V	Retrospective chart review	Comparison of early vs. late tracheostomy in neonates	Need for HMV significantly associated with gestational age, higher respiratory severity scores and late tracheostomy; shorter duration of ventilation in early tracheostomy group
Tofil (2013)^10	N = 15; 3 with CLD on T + V	Post intervention prospective survey	Evaluate simulator delivered caregiver education	Simulator training improved parents’ confidence
Aghamohammadi (2012)^11	N = 166; 29% with CLD	Retrospective cohort study	Program evaluation	71.5% discharged to home and 24.8% discharged to chronic care facility; 3.6% died before discharge
Kun (2012)^12	N = 109, 66 with CLD	Retrospective cohort study	Understand factors associated with re-admission	Incidence reason for re-admission; non-elective re-admission in 40%, half of those within 3 months of discharge	A change in medical management <1 week before discharge was associated with non-elective re-admission
Sovtic (2012)^13	N = 29, 4 with CF all treated with NIV (palliative treatment)	Retrospective cohort study	Program evaluation	2 CF patients died of progression of their disease 2 CF patients stopped NIV due to mask intolerance all 4 CF patients required O2 with NIV	HMV is feasible in developing countries
Reiter (2011)^14	N = 54, 4 with BPD, 3 restrictive lung disease, 1 with PAP, 1 with CF and CDH	Retrospective analysis using questionnaires/ interviews	Investigate incidence and safety of HMV	5/14 children with respiratory disease suffered an emergency situation, 2 severe enough to call for rescue 1 death due to disconnection of ventilator with pulse oximetry turned off	low incidence of severe complications independent of diagnosis, mode of ventilation interface and nursing coverage
Edwards (2010)^15	N = 228 T + V; 120 with CLD (BPD, pulmonary hypoplasia, congenital heart disease, recurrent pneumonitis)	Retrospective cohort study	Program evaluation	29.1% weaned; 51.7% alive on T + V; 19.2% died; CLD as reason for ventilation is independently associated with earlier weaning (HR =7.38; 95% CI 3.0–18.2, p < 0.001); median age of discharge on T + V = 8 months
Tibballs (2010)^16	N = 168; 8 with CLD, pulmonary hypoplasia	Retrospective cohort study	Program evaluation	2 recovered 2 stable on ventilation 1 transferred to other institution 3 died	Multi-disciplinary approach to early discharge with trained parents and laypersons is effective and economical
Paiva (2009)^17	N = 50 with NIV, 2 with lung disease	Prospective cohort study	Validation of combined SpO2/PtcCO2 probe	42% of patients were hypercapneic during sleep; nocturnal hypercapnia is observed in 1/3 of children treated at home with nocturnal NIV that is not associated with symptoms of sleep-disordered breathing, nocturnal desaturation or daytime hypercapnia on blood gases	Non-invasive monitoring of CO2 is possible and this probe is reliable in children
Oktem (2008)^18	N = 34, 21 with CLD and/or airway problems	Retrospective cohort study	Program evaluation	Higher rate of NIV use in younger age children than previously reported; 1 patient died of CVID end-stage lung disease
Downes (2007)^7	N = 77; 37% with CLD	Retrospective cohort study	Program evaluation	Advantages: child is at home with parents and has improved social and neurodevelopment, less healthcare costs, fewer infections Disadvantages: increased parental stress and fatigue, unanticipated expenses, negative impact on other children in the family
Gowans (2007)^19	N = 77; 19 with CLD (BPD and other diseases)	Retrospective comparative cross-sectional study	Assess prevalence of HMV use	Prevalence of HMV did not change much over time (9 children in 1996 and 9 in 2004) 15 were weaned off HMV Progression of underlying disease was most common cause of death
Graham (2007)^20	N = 197 (up to age 21), 138 cared for at home, only 14 with BPD, 12 with “other” CLD	Cross sectional survey	Program evaluation	3 fold overall increase in prevalence of children ventilated over 15 years with higher proportion of children with neuro/neuromuscular disease (vs CLD) in more recent years
Zaidi (2005)^21	N = 1 (severe BPD and PH on T + V and iv epoprostenol)	Case report	Effect of epoprostenol in child with BPD	Weaned from both ventilator and vasodilator after age 5
Edwards (2005)^22	N = 39 on 24 hour T + V; 12 with BPD	Retrospective cohort study	Program evaluation	1 child with BPD died at home. Death was not secondary to failure of ventilator. All children >1.2 years old have been decannulated.
Appierto (2002)^23	34 children on T + V; 3 (6.5%) with BPD	Retrospective cohort study	Program evaluation	Discharged between 5 and18 months of age  - 1 died after 7 months at home from medical deterioration - 2 improved and were decannulated and still alive after >4 years  - caregivers expressed satisfaction after initial 6 months of anxieties
Schweitzer (2002)^24	N = 16;2 with CLD (1 on T + V, 1 on NIV)	Retrospective cohort	Program evaluation	No deaths
Kamm (2001)^25	N = 32, 1 of which had “other” lung disease	Cross sectional survey	Identify and establish family history of children HMV	CCHS and NMD far outnumbered those children with “other” diagnosis
Nakamura (2000)^26	N = 57, 13 with CLD	Prospective cohort study	Prevalence of latex allergy in children on HMV	4/13 patients with CLD had positive RAST tests for latex, though overall latex sensitivity for all 57 patients was 29.8% (17 patients) (prevalence in general population is 0.1–1%) length of mechanical ventilation and underlying disorder do not correlate with frequency of latex allergy
Jardine (1999)^27	N = 136, 6 with BPD, 8 “other”	Cross sectional survey	Identify and establish family history and vent needs	Number of children requiring support has increased substantially Majority of children ventilated lived at home and were able to attend mainstream schools
Dhillon (1996)^28	N = 82, 4 with BPD, 11 “miscellaneous”	Cross sectional survey	Outcomes of children on chronic ventilation	45 of 82 received ventilation at home, remainder in PICU, intermediate care unit or general community hospital setting
Sakakihara (1996)^29	N = 434; 19 with CLD	Retrospective cohort study	Program evaluation	Average duration of HMV: 3.9 years
Goldberg (1994)^30	N =1 with BPD	Case series	Describe healthcare costs and outcomes of children on HMV	Significantly decreased cost of care Decannulation by age 3
Wheeler (2014)^31	N = 100; 50 with CLD, 50 with CHD on T + V	Retrospective cohort study	Program evaluation	98% survival in CLD group vs 74% in CHD group (p = 0.01), 6.6 re-admissions days/patient in CLD group vs 25.9 days/patient in CHD group (p = 0.01), 96% decannulation rate in CLD vs 63% in CHD (p = 0.0001), mean decannulation age 19.5–21.3 months (CLD and CHD)	Overall morbidity concluded to be higher in children with CHD and T + V HMV, versus children with CLD alone.
Wheeler (1994)^32	N = 55; 16 with CLD (most with BPD on T + V)	Retrospective cohort study	Program evaluation	95% survival 71% weaned off ventilator in <1 year of home use 0.8 respiratory hospitalizations/year
Nelson (1996)^33	N = 68, though included patients up to age 29; 7 with BPD, 19 with “other”	Retrospective cohort study	Program evaluation of stressors/complications of home care	Younger children had greatest numbers of hospitalizations and required longest initial hospital stay 3 patients died of ventilator malfunction/disconnection 1 patient with BPD was weaned from ventilator no correlation of death or weaning with any parameter measured
Fields (1991)^34	N = 23 ventilator-dependent children, 8 of whom had primary respiratory disease (6 had BPD, 1 with interstitial pneumonia, 1 with tracheomalacia)	Prospective descriptive study	Program review of community-based care management as alternative to hospital-based	Outcomes of community-based care similar to hospital-based 2 children with BPD, 1 with tracheomalacia, and 1 with IP became technology-independent by 33 months; no deaths in those with primary respiratory disease, all 5 deaths occurred in neurologic dysfunction group (overall mortality rate 18%)
Robinson (1990)^35	N = 10; 3 with CLD on T + V (2 = BPD, 1 = other)	National retrospective survey	Prevalence and outcomes of children on HMV	Increase prevalence of children on long-term ventilation over time
Rodriguez (1990)^36	N = 35 with BPD; 5 on T + V;	Retrospective cohort study	Program evaluation	Reduced healthcare cost to third-party payers
Schreiner (1987)^37	N = 19 with BPD	Retrospective cohort study	Program evaluation	Increased prevalence over time of CLD among children discharged on home T + V; no deaths so home T + V is safe and weaning is possible in many by age 1 year; 4 children with restrictive lung disease not weaned
Burr (1983)^38	N = 6; 1 with CLD on T + V	Case series	Describe outcomes of children on HMV	HMV is safe and results in > $166,000 USD reduction in healthcare costs per year; negative aspects include loss of privacy, disruption of sleeping patterns and schedules of families, limitation on social activities of parents/siblings

BPD = bronchopulmonary dysplasia; T + V = tracheostomy + ventilator; MRSA = Methicillin Resistant Staphylococcus Aureus; NIV = noninvasive positive pressure ventilation; CLD = chronic lung disease; CF = cystic fibrosis; PCD = primary ciliary dyskinesia; CNS = central nervous system; USA = United States of America; vs. = versus; BO = bronchiolitis obliterans; IP = interstitial pneumonia; PPV = positive pressure ventilation; PAP = pulmonary alveolar proteinosis; CDH = congenital diaphragmatic hernia; HR = hazard ratio; CI = confidence interval; CVID = common variable immunodeficiency; PICU = pediatric intensive care unit; CCHS = congenital central hypoventilation syndrome; NMD = neuromuscular disorder; USD = US dollars; CHD = congenital heart disease.

Table 2. Summary of studies evaluating outcomes in children with cystic fibrosis on HMV.

Authors (Year)	Study population/# of patients	Study type	Aim of study	Outcomes	Comments
Mallory (2014)^39	N = 150; CF children <10 years old, post lung transplantation	Retrospective cohort study	Determine outcomes post transplant	6 patients were on NIV at the time of lung transplantation and all survived	Evidence for NIV as a bridge to transplant in children
Collins (2011)^40	N = 23 CF pediatric patients using NIV	Cross sectional survey	Establish factor for NIV initial and extent of use	Median age of NIV initiation was 14 years Reasons for initiation of NIV included acute exacerbation, bridge to transplant and adjunct to physiotherapy	1 report of pneumothorax
Busa (2010)^41	N = 52, 6 with CF	Pilot study of mask contamination	Investigate hygiene of nasal mask	Masks of 6 patients with CF using NIV were tested, one found contaminated with S. aureus, rest were sterile or grew polymicrobial flora
Ottonello (2007)^42	Total N = 20, 1 with CF NIV, 1 with pulmonary hypertension on T + V	Retrospective cohort study	Program evaluation	Ventilation initiated electively after polysomnography showing hypoventilation Both required <24 hour support Overall home costs were lower than hospital costs
Fauroux (2008)^43	N = 2078 CF patients, pediatric only	Cross sectional survey	Evaluation of NIV use in CF	Most common indications for starting were respiratory exacerbations and stable persistent diurnal hypercapnia
Fauroux (2005)^44	N = 40, 10 pediatric CF patients	Cross sectional study	Quantify side effects of NIV	Global facial flattening was less common in children with CF (OR 18, 95% CI 1.6–200, p = 0.007) on NIV but skin injury more common
Fauroux (2004)^45	N = 10 with CF (5 already on home NIV)	Prospective trial	Comparison of invasive vs noninvasive methods to initiate NIV	Clinical noninvasive approach to initiating NIV is as effective as a more invasive technique
Fauroux (2003)^46	N = 102 on NIV; 17 with VF	Cross sectional national survey	Determine prevalence of HMV use	NIV used successfully in CF patients ages 6–18
Fauroux (2001)^47	N = 8 CF patients with PaO2 < 60, PaCO2 > 45	Randomized crossover trial	Comparison of 2 ventilators/modes	NIV sessions of 20 min awake improved blood gases, increased tidal volume, reduced respiratory effort in both modes
Gozal (1997)^48	N = 6, 3 were pediatric patients with CF	Randomized cross-over trial	Compare respiratory and sleep benefits of NIV or low-flow oxygen	NIV as effective as supplemental oxygen in improving REM and NREM sleep-related hypoxemia, though NIV improved alveolar ventilation as well 4/6 patients preferred oxygen to NIV despite morning headache in 2 patients
Padman (1994)^49	N = 15 patients age 4–21 years, 4 with CF	Retrospective case review	Evaluate NIV use in severe CF	4 patients with CF survived to lung transplantation

CF = cystic fibrosis; NIV = noninvasive positive pressure ventilation; T + V = tracheostomy + ventilator; UK = United Kingdom; REM = rapid eye movement sleep; NREM = non-rapid eye movement sleep.

Table 3. Summary of studies evaluating outcomes in children with bronchopulmonary dysplasia, cystic fibrosis or other causes of chronic lung disease on HMV.

Authors (Year)	Study population/# of patients	Study type	Aim of study	Outcomes	Comments
Nasilowski (2015)^50	N = 187 children (total study N = 928) from 9 centers across Poland	Retrospective cohort study	Program evaluation	Increase in rate of HMV use among children between 2000 and 2010 (7 in 2000 to 187 in 2010) and overall (children and adults) increase over time of 21% in proportion of patients with lung disease (CF, CLD, other) rather than neuromuscular disease as indication for HMV
Amin (2013)^51	N = 257; 10 with BPD (4 on NIV, 6 on T + V); 14 CF/PCD on NIV	Retrospective cohort study	Program evaluation	Overall mortality rate = 0.73%/year
Racca (2011)^52	N = 378, 20 with CLD (CF, BPD, other)	Cross sectional multi-center national survey	Assess prevalence of HMV use and patient characteristics	Invasive ventilation correlated with younger age; 81% of those invasively ventilated spent >12 hours on ventilator
Edwards (2005)^22	N = 160, 2 with CF, 5 with bronchiectasis, 2 with BPD	Retrospective cohort study	Program evaluation	Chronic lung disease specific outcomes not highlighted
Fauroux (1995)^53	N = 158 on home vent; 8 with CF, 18 with BPD, 1 with ARDS, 1 with pulmonary fibrosis	Cross sectional survey	Program evaluation	Most children attended school Overall good prognosis (total of 9 deaths)
Canlas-Yamsuan, (1993)^54	N = 22, 8 of whom had primary pulmonary disease	Retrospective cohort study	Program evaluation	4/8 have died from progression of underlying disease (CF, chronic interstitial fibrosis, asphyxiating thoracic dystrophy); 2 patients with BPD were weaned; no effect of type of family, home location, age at start of mechanical ventilation
Goldberg (1984)^55	N = 18; 2 with CLD on home T + V (1 with post-radiation fibrosis and 1 with pulmonary hypoplasia)	Case series	Describe healthcare costs and outcomes of children on HMV	Home T + V allows improvement in medical condition and psychosocial development; 70% initial cost savings; child with pulmonary fibrosis died; child with pulmonary hypoplasia alive and well at 3.5 years; attends school
Wallis (2011)^56	N = 933; 18 with CLD (9 on T + V, 9 on NIV), 5 with CF on NIV, 14 with BPD (9 on T + V, 5 on NIV)	Assess prevalence of HMV use and patient characteristics	Cross sectional multi-center national survey	Overall increase in prevalence of HMV among children (141children in 1998 to 933 children in 2007)	Steepest increase in rate of HMV is among children with neuromuscular diseases

BPD = bronchopulmonary dysplasia; CF = cystic fibrosis; NIV = noninvasive positive pressure ventilation; T + V = tracheostomy plus ventilator; UK = United Kingdom; ARDS = acute respiratory distress syndrome.

El-Masri K, Diaz F. Winkler MK community associated Staphylococcal Aureus sepsis in previously healthy children. J Investig Med. 2015;63(2):451.

 Google Scholar

Reiterer F, Grossauer K, Pfleger A, et al. Severe primary pulmonary lymphangiectasis in a premature infant: management and follow up to early childhood. Pediatr Int. 2015;57(1):166–169.

 Google Scholar

Com G, Kuo DZ, Bauer ML, et al. Outcomes of children treated with tracheostomy and positive-pressure ventilation at home. Clin Pediatr (Phila). 2013;52(1):54–61.

 Google Scholar

Cristea AI, Ackerman VL, Davis SD, et al. Median household income: association with mortality in children on chronic ventilation at home secondary to bronchopulmonary dysplasia. Pediatr Allergy Immunol Pulmonol. 2015;28(1):41–46.

 PubMed Web of Science ®Google Scholar

Cristea AI, Carroll AE, Davis SD, Swigonski NL, Ackerman VL. Outcomes of children with severe bronchopulmonary dysplasia who were ventilator dependent at home. Pediatrics. 2013;132(3):e727–e734.

 Web of Science ®Google Scholar

Hsia SH, Lin JJ, Huang IA, Wu CT. Outcome of long-term mechanical ventilation support in children. Pediatr Neonatol. 2012;53(5):304–308.

 Google Scholar

Downes JJ, Boroughs DS, Dougherty J, Parra M. A statewide program for home care of children with chronic respiratory failure. Caring. 2007;26(9):16–18. 20, 22–23 passim.

 Google Scholar

Ramsey AM. Factors affecting weaning from chronic home mechanical ventilation in children under the age of four years. Chicago IL: Chest (MEETING ABSTRACT); 2013.

 Google Scholar

Rane S, Bathula S, Thomas RL, Natarajan G. Outcomes of tracheostomy in the neonatal intensive care unit: is there an optimal time? J Mater Fetal Neonatal Med. 2014;27(12):1257–1261.

 PubMed Web of Science ®Google Scholar

Tofil NM, Rutledge C, Zinkan JL, et al. Ventilator caregiver education through the use of high-fidelity pediatric simulators: a pilot study. Clin Pediatr (Phila). 2013;52(11):1038–1043.

 Google Scholar

Aghamohammadi S. Predictors of outcome in the pediatric home mechanical ventilator population. Critical Care Congress of the Society of Critical Care Medicine (MEETING ABSTRACT), San Juan, Puerto Rico; 2012.

 Google Scholar

Kun SS, Edwards JD, Ward SL, Keens TG. Hospital readmissions for newly discharged pediatric home mechanical ventilation patients. Pediatr Pulmonol. 2012;47(4):409–414.

 Google Scholar

Sovtic A, Minic P, Vukcevic M, et al. Home mechanical ventilation in children is feasible in developing countries. Pediatr Int. 2012;54(5):676–681.

 Google Scholar

Reiter K, Pernath N, Pagel P, et al. Risk factors for morbidity and mortality in pediatric home mechanical ventilation. Clin Pediatr (Phila). 2011;50(3):237

 Google Scholar

Edwards JD, Kun SS, Keens TG. Outcomes and causes of death in children on home mechanical ventilation via tracheostomy: an institutional and literature review. J Pediatr. 2010;157(6):955–959.

 Google Scholar

Tibballs J, Henning RD. Noninvasive ventilatory strategies in the management of a newborn infant and three children with congenital central hypoventilation syndrome. Pediatr Pulmonol. 2003;36(6):544–548. Dec

 PubMed Web of Science ®Google Scholar

Paiva R, Krivec U, Aubertin G, et al. Carbon dioxide monitoring during long-term noninvasive respiratory support in children. Intens Care Med. 2009;35(6):1068–1074.

 Google Scholar

Oktem S, Ersu R, Uyan ZS, et al. Home ventilation for children with chronic respiratory failure in Istanbul. Respiration. 2008;76(1):76–81.

 Google Scholar

Gowans M, Keenan HT, Bratton SL. The population prevalence of children receiving invasive home ventilation in Utah. Pediatr Pulmonol. 2007;42(3):231–236.

 Google Scholar

Graham RJ, Fleegler EW, Robinson WM. Chronic ventilator need in the community: a 2005 pediatric census of Massachusetts. Pediatrics. 2007;119(6):e1280–e1287. Jun

 Google Scholar

Zaidi AN, Dettorre MD, Ceneviva GD, Thomas NJ. Epoprostenol and home mechanical ventilation for pulmonary hypertension associated with chronic lung disease. Pediatr Pulmonol. 2005;40(3):265–269.

 Google Scholar

Edwards EA, Hsiao K, Nixon GM. Paediatric home ventilatory support: the Auckland experience. J Paediatr Child Health. 2005;41(12):652–658.

 Google Scholar

Appierto L, Cori M, Bianchi R, et al. Home care for chronic respiratory failure in children: 15 years experience. Paediatr Anaesth. 2002;12(4):345–350.

 PubMedGoogle Scholar

Schweitzer C, Camoin-Schweitzer MC, Beltramo F, et al. Domociliary assisted ventilation in children. Rev Pneumol Clin. 2002;58(3):139–144.

 Google Scholar

Kamm M, Burger R, Rimensberger P, et al. Survey of children supported by long-term mechanical ventilation in Switzerland. Swiss Med Wkly. 2001;131(19–20):261–266.

 Google Scholar

Nakamura CT, Ferdman RM, Keens TG, et al. Latex allergy in children on home mechanical ventilation. Chest. 2000;118(4):1000–1003.

 Google Scholar

Jardine E, O'Toole M, Paton JY, Wallis C. Current status of long term ventilation of children in the United Kingdom: questionnaire survey. BMJ. 1999;318(7179):295–299.

 PubMed Web of Science ®Google Scholar

Dhillon J, Frewen T, Singh N, et al. Chronic mechanical ventilation-dependent children in Canada. Paediatr Child Health. 1996;1(2):111–116.

 Google Scholar

Sakakihara Y, Yamanaka T, Kajii M, et al. Long-term ventilator-assisted children in Japan: a national survey. Acta Paediatr Jpn. 1996;38(2):137–142.

 Google Scholar

Goldberg AI. Technology assessment and support of life-sustaining devices in home care. The home care physician perspective. Chest. 1994;105(5):1448–1453.

 Google Scholar

Wheeler W, Han BK, White W, Overman A, Lu Y. Hospital readmission rates and clinical outcomes of children with chronic respiratory failure due to chronic lung disease or congenital heart disease. Paper presented at: Chest 2014; Austin, TX.

 Google Scholar

Wheeler WB, Maguire EL, Kurachek SC, et al. Chronic respiratory failure of infancy and childhood: clinical outcomes based on underlying etiology. Pediatr Pulmonol. 1994;17(1):1–5.

 PubMedGoogle Scholar

Nelson VS, Carroll JC, Hurvitz EA, et al. Home mechanical ventilation of children. Dev Med Child Neurol. 1996;38(8):704–715.

 Google Scholar

Fields AI, Rosenblatt A, Pollack MM, Kaufman J. Home care cost-effectiveness for respiratory technology-dependent children. Am J Dis Child. 1991;145(7):729–733.

 Google Scholar

Robinson RO. Ventilator dependency in the United Kingdom. Arch Dis Child. 1990;65(11):1235–1236.

 Google Scholar

Rodriguez AM, McCutcheon L, Simpser M. Home management of the infant with complex BPD: an alternative to long-term hospitalization. Int Pediatr. 1990;5(4):350–354.

 Google Scholar

Schreiner MD, Donar ME, Kettrick RG. Pediatric home mechanical ventilation. Pediatr Clin North Am. 1987;34(1):47–60.

 PubMedGoogle Scholar

Burr B, Guyer B, Todres ID, Abrahams B, Chiodo T. Home care for children on respirators. N Engl J Med. 1983;309(21):1319–1323.

 Google Scholar

Mallory GB, Mp E, Gazzaneo MC, Crews N. Retrospective review of CF lung transplant recipients aged less than 10 years at an active US pediatric lung transplant centre. Paper presented at North American Cystic Fibrosis Conference 2014; Atlanta, GA.

 Google Scholar

Collins N, Gupta A, Wright A, et al. Survey of the use of non-invasive positive pressure ventilation in UK and Australasian children with cystic fibrosis. Thorax. 2011;66(6):538–539.

 Google Scholar

Busa T, Stremler-Le Bel N, Bosdure E, et al. Hygiene of nasal masks used at home for non-invasive ventilation in children. J Hosp Infect. 2010;76(2):187–188.

 Google Scholar

Ottonello G, Ferrari I, Pirroddi IMG, et al. Home mechanical ventilation in children: retrospective survey of a pediatric population. Pediatr Int. 2007;49(6):801–805.

 Google Scholar

Fauroux B, Le Roux E, Ravilly S, Bellis G, Clement A. Long-term noninvasive ventilation in patients with cystic fibrosis. Respiration. 2008;76(2):168–174.

 Google Scholar

Fauroux B, Lavis JF, Nicot F, et al. Facial side effects during noninvasive positive pressure ventilation in children. Intens Care Med. 2005;31(7):965–969.

 PubMed Web of Science ®Google Scholar

Fauroux B, Nicot F, Essouri S, et al. Setting of noninvasive pressure support in young patients with cystic fibrosis. Eur Respir J. 2004;24(4):624–630.

 Google Scholar

Fauroux B, Boffa C, Desguerre I, Estournet B, Trang H. Long-term noninvasive mechanical ventilation for children at home: a national survey. Pediatr Pulmonol. 2003;35(2):119–125.

 Google Scholar

Fauroux B, Pigeot J, Polkey MI, et al. In vivo physiologic comparison of two ventilators used for domiciliary ventilation in children with cystic fibrosis. Crit Care Med. 2001;29(11):2097–2105.

 Google Scholar

Gozal D. Nocturnal ventilatory support in patients with cystic fibrosis: comparison with supplemental oxygen. Eur Respir J. 1997;10(9):1999–2003.

 PubMed Web of Science ®Google Scholar

Padman R, Lawless S, Von Nessen S. Use of BiPAP by nasal mask in the treatment of respiratory insufficiency in pediatric patients: preliminary investigation. Pediatr Pulmonol. 1994;17(2):119–123.

 Google Scholar

Nasiłowski J, Wachulski M, Trznadel W, et al. The evolution of home mechanical ventilation in Poland between 2000 and 2010. Respir Care. 2015;60(4):577–585.

 Google Scholar

Amin R, Sayal P, Syed F, et al. Pediatric long-term home mechanical ventilation: twenty years of follow-up from one Canadian center. Pediatr Pulmonol. 2014;49(8):816–824

 Web of Science ®Google Scholar

Racca F, Bonati M, Del Sorbo L, et al. Invasive and non-invasive long-term mechanical ventilation in Italian children. Minerva Anestesiol. 2011;77(9):892–901. Sep

 Google Scholar

Fauroux B, Sardet A, Foret D. Home treatment for chronic respiratory failure in children: a prospective study. Eur Respir J. 1995;8(12):2062–2066.

 Google Scholar

Canlas-Yamsuan M, Sanchez I, Kesselman M, et al. Morbidity and mortality patterns of ventilator-dependent children in a home care program. Clin Pediatr (Phila). 1993;32(12):706–713.

 PubMedGoogle Scholar

Goldberg AI, Faure EA, Vaughn CJ, et al. Home care for life-supported persons: an approach to program development. J Pediatr. 1984;104(5):785–795.

 Google Scholar

Wallis C, Paton JY, Beaton S, et al. Children on long-term ventilatory support: 10 years of progress. Arch Dis Child. 2011;96(11):998–1002.

 PubMed Web of Science ®Google Scholar