Long-term Home Mechanical Ventilation Research Articles

Causes and management of urinary system problems in children on long-term home invasive mechanical ventilation.

Many ventilator-dependent children have comorbid conditions including urinary tract disorders. We aimed to present a focused review of the literature describing the causes and management of urinary system problems in children with long-term home mechanical ventilation. We performed a literature search in PubMed/MEDLINE, Scopus, and Web of Science with keywords "children," "home mechanical ventilation," "urinary system," "urinary tract," "neurogenic bladder," "clean intermittent catheterization," "urinary tract infection," "urolithiasis," and "acute kidney injury." We included original articles, reviews, guidelines, and case reports published in English. Ventilator-dependent children may have neurogenic bladder/bowel dysfunction which renders them prone to urinary tract infection, high bladder pressure, vesicoureteral reflux, hydronephrosis, and renal dysfunction. These children require bladder catheterization, medications affecting parasympathetic/sympathetic nervous systems, or surgical procedures to prevent urinary infections, and to maintain continence and renal functions. However, bladder catheterization or surgical procedures like augmentation cystoplasty may also be complicated with urinary infections, urolithiasis, or urethral strictures. Urolithiasis frequency is also increased due to immobilization-related hypercalciuria, hypocitraturia caused by antiepileptic drugs, urinary stasis, and urinary infections. On the other hand, mechanical ventilation can impair renal function by reduction of cardiac output, redistribution of intrarenal blood flow and stimulation of sympathetic and hormonal pathways. Children requiring long-term invasive home mechanical ventilation may have other comorbid conditions, including urinary system diseases, which become manifest as these patients are being kept alive due to the advances in ventilation strategies. These children must be carefully observed for urological complications and managed accordingly to prevent kidney injury.

Arrangement of residence before hospital discharge for children on home-invasive mechanical ventilation.

Children on long-term home mechanical ventilation are a growing population due to clinical and technological advances and the benefit for the child's quality of life. Invasive home ventilation is one of the most complex therapies offered in the home setting, requiring adequate home environment and appropriate equipment and supplies before discharge.The transition from hospital to home represents a vulnerable period that can be facilitated with an established transition plan with multidisciplinary team involvement. Readiness for home care is achieved when the patient is stable and has been transitioned from a critical care ventilator to a home mechanical ventilator. In parallel, comprehensive competency-based training regarding the knowledge and skills needed to help families use the equipment confidently and safely. Before discharge, families should be counseled on an adequate home environment to ensure a safe transition. The residence arrangement may include physical space modifications, verifying electrical installation, or moving to another home. Durable medical equipment and supplies must be ordered, and community healthcare support arranged. Parents should receive practical advice on setting up the equipment at home and on preventive measures to minimize complications related to tracheostomy and ventilator dependence, including regular maintenance and replacement of necessary equipment.Given the overall impact of invasive ventilation on home life, a structured home care action package is essential to alleviate the burdens involved.

Long-term health-related quality of life in patients on home mechanical ventilation

BackgroundIt is fundamental to optimize and retain health-related quality of life (HRQoL) in the long term in patients with home mechanical ventilation (HMV). Therefore, this study aimed to evaluate the evolution of the HRQoL in patients already established on HMV across a period of 5 years and whether the HRQoL is associated with mortality.MethodsThis was a 5-year longitudinal cohort study conducted in an Outpatient Ventilation Clinic. Consecutive patients on HMV for at least 30 days responded to the Severe Respiratory Insufficiency (SRI) questionnaire at inclusion and again at 5 years.ResultsA total of 104 patients were included (male 56.7%, median age 69 [P25;P75] [61;77] years). Almost half of the patients had COPD (49.0%). Patients were on HMV for a median of 43.5 [22;85.5] months, with overall good adherence (median 8 [6;9] daily hours). Fifty-seven (54.8%) patients were alive at 5 years. In surviving patients, the only difference with statistical significance was in the attendant symptoms and sleep subscale, with patients scoring 7.1 [-4.5;25] points higher in the final questionnaire (p = 0.002). Survivors had significantly better scores in the SRI at inclusion than deceased patients (median 59.6 [49.2;71.7] vs 48.7 [38.4;63.2]; p = 0.004).ConclusionsThese results shows that HRQoL remains stable in surviving patients with HMV at five years. It also suggests that SRI can be of important prognostic value and help predict the terminal phase of the disease course in patients with long-term HMV.

Home Mechanical Ventilation in Children: Experience of Paediatric Pulmonology Divisions in Istanbul

Advances in technology in recent years increased the long-term survival rate of patients with chronic respiratory failure. As a result, the number of patients receiving long-term home mechanical ventilation (LTHV) support is increasing worldwide. The most important step in the management of patients with home mechanical ventilation (HMV) is to ensure adequate oxygenation and ventilation. Successful care at home requires the presence of trained caregivers and accessibility to medical devices and a care team. The aims of this study were, to describe the characteristics of children on LTHV in Istanbul, to compare the patients receiving non-invasive ventilation (NIV) and invasive ventilation, and to evaluate the frequency and risk factors of hospital admission in this population.

Pain in amyotrophic lateral sclerosis: a narrative review.

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative condition characterized by loss of motor neurons, resulting in motor weakness of the limbs and/or bulbar muscles. Pain is a prevalent but neglected symptom of ALS, and it has a significant negative impact on the quality of life of patients and their caregivers. This review outlines the epidemiology, clinical characteristics, underlying mechanisms, and management strategies of pain in ALS to improve clinical practice and patient outcomes related to pain. Pain is a prevalent symptom among patients with ALS, with a variable reported prevalence. It may occur at any stage of the disease and can involve any part of the body without a specific pattern. Primary pain includes neuropathic pain and pain from spasticity or cramps, while secondary pain is mainly nociceptive, occurring with the progression of muscle weakness and atrophy, prolonged immobility causing degenerative changes in joints and connective tissue, and long-term home mechanical ventilation. Prior to treatment, the exact patterns and causes of pain must first be identified, and the treatment should be tailored to each patient. Treatment options can be classified into pharmacological treatments, including nonsteroidal anti-inflammatory drugs, antiepileptic drugs, drugs for cramps or spasticity, and opioid; and nonpharmacological treatments, including positioning, splints, joint injections, and physical therapy. The development of standardized and specific assessment tools for pain-specific to ALS is required, as are further studies on treatments to reduce pain, diminish suffering, and improve the quality of life of patients with ALS.

A qualitative study of patient and carer experiences with home respiratory therapies: Long-term oxygen therapy and home mechanical ventilation

Studies exploring the experience of patients receiving home respiratory therapies (HRT), such as long-term oxygen therapy (LTOT) and home mechanical ventilation (HMV), are still limited. This study focused on patients’ and carers’ experience with LTOT and HMV. An exploratory, cross-sectional qualitative study, using semi-structured focus groups, was carried out with 18 patients receiving HRT (median 71y, 78% male, 56% on both LTOT and HMV) and 6 carers (median age 67y, 67% female). Three focus groups were conducted in three regions of Portugal. Thematic analysis was performed by two independent researchers. Patients’ and carers’ experience was reflected in seven major topics, linked to specific time points and settings of the treatment: Initial symptoms/circumstances (n = 41), Prescription (n = 232), Implementation (n = 184), Carer involvement (n = 34), Quality of life impact (n = 301), Health care support/navigability (n = 173) and Suggestions (n = 14). Our findings demonstrate a general good perception of the HRT by patients and carers recognizing a significative quality of life impact improvement, while identifying specific points where improvements in healthcare are needed, particularly about navigability issues, articulation between the hospital, primary care and homecare teams, especially regardingprescriptionrenewal. This knowledge is crucial to promote a long-term HRT adherence and to optimize HRT delivery in line with patients’ experience, needs, and values. Moreover, these key points can inform the development of a specific patient-reported experience measure (PREM) for patients on HRT, which is not currently available, and foster a more integrated respiratory care model.

Validation of the Hungarian version of the SRI Questionnaire

BackgroundHome mechanical ventilation is a reliable treatment for patients suffering from chronic respiratory failure, improving survival and quality of life. Prevalence has been increasing worldwide as a result of evolving technical possibilities, telemedicine and improving national guidelines. Projects to establish a national guideline and registry for patients treated with home mechanical ventilation are currently under way in Hungary and our aim was to validate a quality of life questionnaire suited for evaluation and follow up in this specific patient group. The Severe Respiratory Insufficiency Questionnaire (SRI) is a quality of life tool designed to evaluate patients receiving home mechanical ventilation and has been validated both in patient groups receiving invasive and noninvasive ventilation.MethodsThe Hungarian version of the SRI was created using the translation-backtranslation method, which was then tested for validity, viability and reliability in a cohort involving patients from three centers, receiving long-term home mechanical ventilation for chronic respiratory failure through an invasive or noninvasive interface. Patient data was collected (demographic data, lung function test, arterial blood gas, ventilation settings) and quality of life was measured with the previously validated SF-36 and newly created Hungarian SRI Questionnaires at two time points.ResultsOne hundred four patients receiving home mechanical ventilation were enrolled. The time to complete the SRI Questionnaire was 8.6 (±3.1) minutes, 69.2% questionnaires were self-administered. Exploratory factor analysis explained 73.8% of the variance of the questionnaire, but resulted in 13 scales. We found correlations between the SRI subscale scores to corresponding scales of the previously validated general quality of life survey SF-36. The Cronbach alpha coefficient was 0.928 for the Summary Scale of the SRI Questionnaire, proving high internal consistency. Reproducibility was high for most scales, resulting in a high overall correlation for the summary score (0.877, p < 0.001).ConclusionsThe Hungarian version of the SRI Questionnaire is a viable, valid, reliable and reproduceable quality of life tool applicable for patients treated with home mechanical ventilation.

The Site of Oxygen Delivery Into a Home Ventilator Affects Recorded Volumes.

Long-term home mechanical ventilation is increasingly used by patients with chronic respiratory failure. Storage of medical data in the cloud is expanding, and ventilation can be monitored remotely. The aim of this bench study was to determine whether tidal volume (VT) can be affected by the location of supplemental oxygen placement. We tested 4 home ventilators in a bench test using a dual-chamber test lung to test the addition of supplemental oxygen placement via a connector in the circuit (ie, front intake port) versus via the manufacturer's rear intake port, with different oxygen supply flows of 2, 4, 6, and 8 L/min. We compared the effectively delivered VT as measured with a pneumotachograph (ie, measured VT) versus the VT reported by each home ventilator (ie, monitored VT). For all of the home ventilators, the monitored VT and measured VT were comparable when the rear oxygen intake was used, regardless of oxygen flow. However, when the front oxygen intake was used, the monitored VT as measured by the ventilators was significantly lower than the measured VT, with the greatest difference reaching 29% for the highest oxygen flow tested (8 L/min). The monitored VT may be inaccurate if oxygen is added with a connector in the circuit, which may have consequences on both the individual level and collective level (ie, big data analysis). Physicians who analyze data from home ventilators should be aware of the site of oxygen supplementation and promote use of only the rear oxygen intake.

A transition program to adult health services for teenagers receiving long-term home mechanical ventilation: A longitudinal qualitative study.

Adolescents receiving long-term home mechanical ventilation (HMV) who survive into adulthood must transition to adult health care services. Lack of transition readiness is reported to result in poor health outcomes. The objective of this study is to explore longitudinally the pediatric-to-adult health care transition experience involving a transition program for adolescents receiving HMV including transition readiness, barriers, facilitators, and modifiable features. A prospective qualitative longitudinal interview study of adolescent and family caregiver dyads recruited through a pediatric-to-adult HMV transition program jointly established by two collaborating health centers: The Hospital for Sick Children and West Park Healthcare Centre in Toronto, Canada. Eligible dyads were interviewed at three time points: pretransition, transition, and 12-months posttransition. Interviews were transcribed verbatim and analyzed using directed content analysis methods. Ventilator-assisted adolescents (VAAs) and caregiver participants perceived a lack of transition readiness in their ability to manage health communication and coordination across multiple adult providers. Transition facilitators included early transition discussion, opportunities for VAAs to speak directly with HMV providers during appointments, receipt of print informational materials regarding adult services, and a joint pediatric-adult team handover meeting. Modifiable transition barriers included lack of other specialist referrals, insufficient information about adult homecare service funding, and limited involvement of family doctors. Unresolved transition barriers resulted in perceptions of service fragmentation. Although the pediatric-to-adult HMV transition program conferred benefits service fragmentation was perceived. Transition barriers may be overcome through early planning and staged transition with all specialists, community providers, and the family and adolescent working in collaboration.

The spontaneous breathing trial is of low predictive value regarding spontaneous breathing ability in subjects with prolonged, unsuccessful weaning.

BackgroundThe spontaneous breathing trial (SBT) is a well-established diagnostic test for predicting extubation failure in intubated intensive care unit (ICU) patients. However, the SBT has not been evaluated in a specific cohort of tracheostomized patients in whom weaning is prolonged and ultimately unsuccessful.ObjectiveThe aim of the trial was to investigate the relevance of SBT failure criteria in chronic respiratory failure subjects undergoing long-term invasive home mechanical ventilation following tracheostomy and weaning failure.MethodsMeasurement of all established failure criteria including pneumotachygraphical assessment of the rapid shallow breathing index (RSBI) took place during an SBT. The decision to continue spontaneous breathing was based on failure criteria as well as the subjective willingness of the patient.ResultsFifteen subjects with a median age of 58 years (interquartile range [IQR] 44–74) were studied; 10 with COPD, 4 with neuromuscular diseases and 1 with both. Twelve subjects met the SBT failure criteria within 30 min, but one third of these subjects were still able to continue with spontaneous breathing. In contrast, 3 subjects could not be weaned despite the SBT being successful. An increased RSBI was the most frequently observed SBT failure criterion (57% of all SBT). However, the SBT varied substantially in individual subjects who were able to sustain spontaneous breathing, despite having reached the cut-off for SBT failure.ConclusionThe SBT was of low predictive value regarding spontaneous breathing ability in chronic respiratory failure subjects with prolonged, unsuccessful weaning.

Clinical evidence for respiratory insufficiency type II predicts weaning failure in long-term ventilated, tracheotomised patients: a retrospective analysis

BackgroundPatients who require a prolonged weaning process comprise a highly heterogeneous group of patients amongst whom the outcome differs significantly. The present study aimed to identify the factors that predict whether the outcome for prolonged weaning will be successful or unsuccessful.MethodsData from tracheotomised patients who underwent prolonged weaning on a specialised weaning unit were assessed retrospectively via an electronic and paper-bound patient chart. Factors for weaning success were analysed by univariate and multivariate analyses.ResultsOut of the 124 patients examined, 48.4% were successfully weaned (n = 60). Univariate analysis revealed that long-term home mechanical ventilation prior to current weaning episode; time between intubation and the first spontaneous breathing trial (SBT); time between intubation and the first SBT of less than 30 days; lower PaCO2 prior to, and at the end of, the first SBT; and lower pH values at the end of the first SBT were predictors for successful weaning. Following multivariate analysis, the absence of home mechanical ventilation prior to admission, a maximum time period of 30 days between intubation and the first SBT, and a non-hypercapnic PaCO2 value at the end of the first SBT were predictive of successful weaning.ConclusionsThe current analysis demonstrates that the evidence for respiratory insufficiency type II provided by clinical findings serves as a predictor of weaning failure.

Gender differences in patients starting long-term home mechanical ventilation due to obesity hypoventilation syndrome

Background and objectives: Obesity hypoventilation syndrome (OHS) is often diagnosed late. The aim of this study was to analyse gender differences at initiation of long-term mechanical ventilation ...

Respiratory home care: take the pulmonary specialist out of the hospital!

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Gender differences in patients starting long-term home mechanical ventilation due to obesity hypoventilation syndrome

Background and objectivesObesity hypoventilation syndrome (OHS) is often diagnosed late. The aim of this study was to analyse gender differences at initiation of long-term mechanical ventilation (LTMV) in patients with (OHS), to analyse gender differences in treatment effect and to study how the prescription of LTMV due to OHS has changed over time. MethodsData on patients on LTMV due to OHS between 1996 and 2014 were obtained from Swedevox, a nationwide health quality registry of patients on LTMV in Sweden. ResultsWhen starting LTMV, women were generally older (age 64.4 ± 11.2 vs. 60.1 ± 12.1 years, p < 0.001), more obese (BMI 43.0 ± 8.2 vs. 41.5 ± 7.9 kg/m2, p < 0.001), more hypoxic (PaO2 7.6 ± 1.5 vs. 7.9 ± 1.6 kPa, p = 0.001), had more hypercapnia (PaCO2 7.2 ± 1.3 vs. 6.9 ± 1.3 kPa, p = 0.001), had higher base excess (6.9 ± 4.1 vs. 5.8 ± 4.7 kPa, p < 0.001) and more frequently started LTMV in a non-elective situation (43.2% vs. 37.5%, p = 0.026) than men. Improvement of arterial blood gas values or in age-adjusted mortality at one-year follow-up did not differ. During the study period, the age of patients at the initiation of LTMV rose by 3.4 years/decade (P = 0.001) in women and with 1.9 years/decade (P = 0.048) in men but there were no significant changes in BMI (P = 0.425). ConclusionsDiagnosis of OHS is more delayed in women and as a consequence the disease is more advanced when diagnosed. In spite of this, there is no gender difference in survival rate in patients with OHS treated with LTMV. More and older patients with OHS nowadays gain access to LTMV.

Prolonged weaning: From the intensive care unit to home

Weaning is the process of withdrawing mechanical ventilation which starts with the first spontaneous breathing trial (SBT). Based on the degree of difficulty and duration, weaning is classified as simple, difficult and prolonged. Prolonged weaning, which includes patients who fail 3 SBTs or are still on mechanical ventilation 7 days after the first SBT, affects a relatively small fraction of mechanically ventilated ICU patients but these, however, requires disproportionate resources. There are several potential causes which can lead to prolonged weaning. It is nonetheless important to understand the problem from the point of view of each individual patient in order to adopt appropriate treatment and define precise prognosis. An otherwise stable patient who remains on mechanical ventilation will be considered for transfer to a specialized weaning unit (SWU). Though there is not a precise definition, SWU can be considered as highly specialized and protected environments for patients requiring mechanical ventilation despite resolution of the acute disorder. Proper staffing, well defined short-term and long-term goals, attention to psychological and social problems represent key determinants of SWU success. Some patients cannot be weaned, either partly or entirely, and may require long-term home mechanical ventilation. In these cases the logistics relating to caregivers and the equipment must be carefully considered and addressed.

Prolonged weaning: From the intensive care unit to home

Weaning is the process of withdrawing mechanical ventilation which starts with the first spontaneous breathing trial (SBT). Based on the degree of difficulty and duration, weaning is classified as simple, difficult and prolonged. Prolonged weaning, which includes patients who fail 3 SBTs or are still on mechanical ventilation 7 days after the first SBT, affects a relatively small fraction of mechanically ventilated ICU patients but these, however, requires disproportionate resources. There are several potential causes which can lead to prolonged weaning. It is nonetheless important to understand the problem from the point of view of each individual patient in order to adopt appropriate treatment and define precise prognosis. An otherwise stable patient who remains on mechanical ventilation will be considered for transfer to a specialized weaning unit (SWU). Though there is not a precise definition, SWU can be considered as highly specialized and protected environments for patients requiring mechanical ventilation despite resolution of the acute disorder. Proper staffing, well defined short-term and long-term goals, attention to psychological and social problems represent key determinants of SWU success. Some patients cannot be weaned, either partly or entirely, and may require long-term home mechanical ventilation. In these cases the logistics relating to caregivers and the equipment must be carefully considered and addressed.

Pediatric long-term home mechanical ventilation: Twenty years of follow-up from one Canadian center

Canadian longitudinal data from a pediatric domiciliary long-term mechanical ventilation (LTMV) program is lacking. Our aim was to report on the clinical characteristics and trends of children followed in one of Canada's pediatric home ventilation programs over the past 20 years. A retrospective chart review was conducted on patients receiving long-term domociliary mechanical ventilation between January 1, 1991 and December 31, 2011 in a single center. Domiciliary long-term mechanical ventilation was defined as the daily use of invasive mechanical ventilation (IMV) or noninvasive positive pressure ventilation (NiPPV) for at least 3 months, in the users' home or in a long-term residential facility. Between 1991 and 2011, a total of 379 children were identified (313 [83%] with noninvasive ventilation). The median age at initiation was 9.6 years (interquartile range [IQR] 2.9-13.9), the median duration of ventilation was 2.2 years (IQR 0.8-4.9) and 53% were male. Ninety-nine percent of children were cared for at home. The reason for ventilation was "musculoskeletal" in origin for the majority of children. The number of children receiving long-term mechanical ventilation at home increased from 2 in 1991 to 156 children as of December 2011. There was a twofold increase in the number of invasive ventilation initiations in the second 10 years, n = 45 (2001-2011) as compared to the first 10 years, n = 21 (1991-2000). However, there was more than a fivefold increase in the number of noninvasive initiations in the first 10 years, n = 50 (1991-2000) as compared to the second 10 years, n = 263 (2001-2011). The largest growth was in the 13-18 years age group. There were 55 (15%) mortalities over the study period. In summary, our 20-year retrospective study has shown that there has been an exponential growth in the number of children receiving domiciliary LTMV with the majority of children having favorable outcomes. Our study represents a step towards developing a Canadian registry to design and implement programmatic change for this medically complex population to ensure best practice for these children as well as their families.

Choosing a ventilator for home mechanical ventilation

<h3>Educational aims</h3> To discuss the basic principles of ventilator function To help the reader to recognise the different features of a home care ventilator To outline the advantages and drawbacks of the different home care ventilators To provide some recommendations to facilitate the clinical management of patients on home care ventilator <h3>Summary</h3> Mechanical ventilation, applied either invasively through a tracheotomy tube or noninvasively <i>via</i> a mask, is increasingly used for long-term management of many forms of severe chronic respiratory failure in the home setting. In recent years, the quality of the ventilators for long-term home mechanical ventilation has improved considerably and, concomitantly, the number of machines available has also increased. This broader range of commercially available machines is clearly an advantage; however, it makes the choice of the optimal device for a specific patient more difficult. The aim of the present article is to provide useful information to help and guide the choice of device for long-term mechanical ventilation in the home setting.

Long-Term Home Mechanical Ventilation in the United States

Patients requiring prolonged mechanical ventilation are rapidly increasing in number. Improved ICU care has resulted in many patients surviving acute respiratory failure to require prolonged mechanical ventilation during convalescence. Also, mechanical ventilation is increasingly used as a therapeutic option for patients with symptomatic chronic hypoventilation, with an increased effort to predict nocturnal hypoventilation to initiate ventilation earlier. There are continued efforts by ventilator manufacturers to improve home ventilators. These factors point to a likely increase in the number of patients receiving home mechanical ventilation in the United States. Unfortunately, there are no comprehensive databases or national registry of home ventilator patients-therefore the number of home ventilator patients is unknown. There are real challenges to providing mechanical ventilation in the home, which include caregiver training, adequacy of respiratory care, and reimbursement.

Long‐term home ventilation of children in Italy: A national survey

Improved technology, as well as professional and parental awareness, enable many ventilator-dependent children to live at home. However, the profile of this growing population, the quality and adequacy of home care, and patients' needs still require thorough assessment. To define the characteristics of Italian children receiving long-term home mechanical ventilation (HMV) in Italy. A detailed questionnaire was sent to 302 National Health Service hospitals potentially involved in the care of HVM in children (aged <17 years). Information was collected on patient characteristics, type of ventilation, and home respiratory care. A total of 362 HMV children was identified. The prevalence was 4.2 per 100,000 (95% CI: 3.8-4.6), median age was 8 years (interquartile range 4-14), median age at starting mechanical ventilation was 4 years (1-11), and 56% were male. The most frequent diagnostic categories were neuromuscular disorders (49%), lung and upper respiratory tract diseases (18%), hypoxic (ischemic) encephalopathy (13%), and abnormal ventilation control (12%). Medical professionals with nurses (for 62% of children) and physiotherapists (20%) participated in the patients' discharge from hospital, though parents were the primary care giver, and in 47% of cases, the sole care giver. Invasive ventilation was used in 41% and was significantly related to young age, southern regional residence, longer time spent under mechanical ventilation, neuromuscular disorders, or hypoxic (ischemic) encephalopathy. Care and technical assistance of long-term HMV children need assessment, planning, and resources. A wide variability in pattern of HMV was found throughout Italy. An Italian national ventilation program, as well as a national registry, could be useful in improving the care of these often critically ill children.