Use Of Home Mechanical Ventilation Research Articles

The Role of Cognition, Affective Symptoms, and Apathy in Treatment Adherence with Noninvasive Home Mechanical Ventilation in Myotonic Dystrophy.

Chronic respiratory failure often occurs in myotonic dystrophy type 1 (DM1) and can be treated with noninvasive home mechanical ventilation (HMV). Treatment adherence with HMV is often suboptimal in patients with DM1, but the reasons for that are not well understood. The aim of this exploratory study was to gain insight in the prevalence of mild cognitive impairment, affective symptoms, and apathy and to investigate their role in HMV treatment adherence in DM1. The Montreal Cognitive Assessment (MoCA), the Hospital Anxiety and Depression Scale (HADS), and the Apathy Evaluation Scale (AES) were used to assess cognition, affective symptoms, and apathy in DM1 patients that use HMV. Patients with low treatment adherence (average daily use HMV <5 h or <80% of the days) were compared with patients with high treatment adherence (average daily use of HMV≥5 h and ≥80% of the days). Sixty patients were included. Abnormal scores were found in 40% of the total group for the MoCA, in 72-77% for the AES, and in 18% for HADS depression. There was no difference between the high treatment adherence group (n = 39) and the low treatment adherence group (n = 21) for the MoCA, AES, and HADS depression. The HADS anxiety was abnormal in 30% of the total group, and was significantly higher in the low treatment adherence group (p = 0.012). Logistic regression analysis revealed that a higher age and a higher BMI were associated with a greater chance of high treatment adherence. This exploratory study showed that cognitive impairment and apathy are frequently present in DM1 patients that use HMV, but they are not associated with treatment adherence. Feelings of anxiety were associated with low treatment adherence. Higher age and higher BMI were associated with high treatment adherence with HMV.

End of life decision making when home mechanical ventilation is used to sustain breathing in Motor Neurone Disease: patient and family perspectives

BackgroundMotor Neurone Disease (MND) leads to muscle weakening, affecting movement, speech, and breathing. Home mechanical ventilation, particularly non-invasive ventilation (NIV), is used to alleviate symptoms and support breathing in people living with MND. While home mechanical ventilation can alleviate symptoms and improve survival, it does not slow the progression of MND. This study addresses gaps in understanding end-of-life decision-making in those dependent on home mechanical ventilation, considering the perspectives of patients, family members, and bereaved families.MethodsA UK-wide qualitative study using flexible interviews to explore the experiences of people living with MND (n = 16), their family members (n = 10), and bereaved family members (n = 36) about the use of home mechanical ventilation at the end of life.ResultsSome participants expressed a reluctance to discuss end-of-life decisions, often framed as a desire to “live for the day” due to the considerable uncertainty faced by those with MND. Participants who avoided end-of-life discussions often engaged in ‘selective decision-making’ related to personal planning, involving practical and emotional preparations. Many faced challenges in hypothesising about future decisions given the unpredictability of the disease, opting to make ‘timely decisions’ as and when needed. For those who became dependent on ventilation and did not want to discuss end of life, decisions were often ‘defaulted’ to others, especially once capacity was lost. ‘Proactive decisions’, including advance care planning and withdrawal of treatment, were found to empower some patients, providing a sense of control over the timing of their death. A significant proportion lacked a clear understanding of the dying process and available options.ConclusionsThe study highlights the complexity and evolution of decision-making, often influenced by the dynamic and uncertain nature of MND. The study emphasises the need for a nuanced understanding of decision-making in the context of MND.

Role of respiratory characteristics in treatment adherence with noninvasive home mechanical ventilation in myotonic dystrophy type 1, a retrospective study

Chronic respiratory insufficiency is common in patients with myotonic dystrophy type 1 (DM1) and can be treated with noninvasive home mechanical ventilation (HMV). HMV is not always tolerated well resulting in low treatment adherence. We aimed to analyze if baseline respiratory characteristics such as pulmonary function, level of pCO2 and presence of sleep apnea are associated with HMV treatment adherence in DM1 patients. Pulmonary function testing, polysomnography and blood gas measurement data of DM1 patients were retrospectively collected. Initiation of HMV and treatment adherence after one year was documented. Patients with low treatment adherence (average daily use of HMV <5 h) were grouped with patients that discontinued HMV and compared with patients with high treatment adherence (average daily use of HMV >5 h). HMV was initiated in 101 patients. After one year, 58 patients had low treatment adherence. There were no differences between the low and high treatment adherence group regarding the respiratory characteristics. None of the included predictors (gender, age, body mass index, cytosine-thymine-guanine repeat length, FVC, daytime pCO2, bicarbonate, nighttime pCO2, nighttime base excess, apnea-hypopnea index and mean saturation during sleep) was able to significantly predict high treatment adherence. In conclusion, the respiratory characteristics are not associated with treatment adherence with HMV in DM1 patients and cannot be used to identify patients at risk for low HMV treatment adherence.

Can addressing family education improve outcomes of patients under home invasive mechanical ventilation? A randomized controlled clinical trial

ABSTRACT The use of home mechanical ventilation is increasingly developing. The present study aimed to examine the effect of a family-centered training program on patients under home invasive mechanical ventilation. A total of 60 adult patients under invasive mechanical ventilation were selected and randomly allocated into two groups. The supportive home care program consisting of six training sessions using a teach-back technique and follow up training at home. The rate of hospital readmission and mortality in the intervention group was significantly lower than the control group(P = .02 and P = .03, respectively). Moreover, the home caregivers’ level of knowledge in the intervention group was significantly higher than that of the control group(P = 0/000). In addition, implementing the intervention effectively increased home caregivers’ ability of functional skills. Therefore, comprehensive preparation of the patient and family before discharge and coherent support and continuity of care after discharge with the effective presence of nurses.

How to improve the quality of care for people on home mechanical ventilation from the perspective of healthcare professionals: a qualitative study

BackgroundThe rapid increase in the use of home mechanical ventilation (HMV) for people with chronic respiratory failure poses extreme challenges for the healthcare system. People on HMV have complex care needs and require support from an interprofessional team. In Germany, HMV is criticised for inadequate quality standards, particularly in outpatient intensive care practice. The objective of this study was to describe the quality of care for people on outpatient HMV in Germany, Bavaria and provide recommendations for improvement from the perspective of healthcare professionals (HCPs).MethodsSemi-structured qualitative telephone interviews with HCPs (i.e., nurses, equipment providers, therapists, and physicians) were analysed using the framework method. The quality framework of Health Improvement Scotland (HIS), which aims to improve the quality of person-centred care, was used to build a deductive analysis matrix. The framework includes the three key areas: (1) Outcomes and impact, (2) Service delivery, and (3) Vision and leadership. The domains (meta-codes) and quality indicators (sub-codes) of the quality framework were used for deductive coding.ResultsOverall, 87 HCPs (51 female, mean age of 44.3 years, mean professional experience in HMV of 9.4 years) were interviewed (mean duration of 31 min). There was a complex interaction between the existing health care system (Outcomes and impact, 955 meaning units), the delivery of outpatient intensive care (Service delivery, 939 meaning units), and improvement-focused leadership (Vision and leadership, 70 meaning units) that influenced the quality of care for people on HMV. The main barriers were an acceleration in transition management, a neglect of weaning potential, a shortage of qualified professionals and missing quality criteria. The central recommendations for promoting person-centred care were training and supervision of staff and an inspiring leadership. An integrated care structure supporting medical home visits and outpatient rehabilitation should be developed.ConclusionThis study describes a heterogeneous and partly deficient care situation for people on HMV, but demonstrates that high quality care is possible if person-centred care is successfully implemented in all areas of service provision. The recommendations of this study could inform the development of a person-centred integrated care structure for people on HMV.

Cross-sectional survey on home mechanical ventilator use: major deficiencies in a home care system in South Korea.

BackgroundDespite the increased use of home mechanical ventilation (HMV), data on home care services for HMV users in Asian countries are scarce. This study investigated the current status of HMV use in the Seoul metropolitan area.MethodsThis cross-sectional study involved three university-affiliated hospitals. Subjects who were receiving HMV at home for >3 months were included, and door-to-door visits were done to collect data (e.g., on devices, caregivers, and healthcare service use) from the subjects or their families.ResultsAmong the 140 individuals who were initially screened, 38 adults and 26 children were finally enrolled; the duration of HMV use was 14.5 (8.8–37.5) months and 20.5 (7.0–28.0) months, respectively. Tracheostomy ventilation was performed in 36.8% of the adults and 61.5% of the children, and life-support ventilator in 55.3% and 96.2%, respectively. Regarding ancillary devices, 42.1% of the adults and 80.8% of the children had an oxygen monitoring device, while only one member of each group had a cough assist device. Among those with a tracheostomy, 64.3% of adults and 81.3% of children had an AMBU-bag. Reliance on a family member for care was determined in 65.8% of adults and 88.5% of children, but a home visit by a hospital nurse during the previous year occurred in only 26.3% of the adults and 3.8% of the children. Emergency incidents at home occurred in 39.5% of the adults and 50.0% of the children, with dyspnea being the most common cause. Out-of-pocket expenses tended to be higher in the tracheostomy (vs. non-tracheostomy) group and in children (vs. adults).ConclusionsOur study highlights the challenges faced by adults and children dependent on HMV, and their families. There is an urgent need for nationwide standardization of care for patients receiving HMV at home.

Home mechanical ventilation: back to basics.

Over recent decades, the use of home mechanical ventilation (HMV) has steadily increased worldwide, with varying prevalence in different countries. The key indication for HMV is chronic respiratory failure with alveolar hypoventilation (e.g., neuromuscular and chest wall disease, obstructive airway diseases, and obesity-related respiratory failure). Most modern home ventilators are pressure-targeted and have sophisticated modes, alarms, and graphics, thereby facilitating optimization of the ventilator settings. However, different ventilators have different algorithms for tidal volume estimation and leak compensation, and there are also several different circuit configurations. Hence, a basic understanding of the fundamentals of HMV is of paramount importance to healthcare workers taking care of patients with HMV. When choosing a home ventilator, they should take into account many factors, including the current condition and prognosis of the primary disease, the patient’s daily performance status, time (hr/day) needed for ventilator support, family support, and financial costs. In this review, to help readers understand the basic concepts of HMV use, we describe the indications for HMV and the factors that influence successful delivery, including interface, circuits, ventilator accessories, and the ventilator itself.

Home Mechanical Ventilation Use in South Korea Based on National Health Insurance Service Data.

In Asian countries, nationwide data on patients undergoing home mechanical ventilation are limited. This study investigated the prevalence and primary indications for home mechanical ventilation use in South Korea by using nationwide registry data from the National Health Insurance Service. The study period included a total of 4,785 subjects on home mechanical ventilation (mean ± SD age, 56.0 ± 23.9 y; females, 40.1%). The estimated overall prevalence of home mechanical ventilation use in South Korea was 9.3 per 100,000, with a prevalence of 6.3 per 100,000 among children (ages < 15 y). The most common primary diagnoses were neuromuscular diseases (42.0%) and lung and/or airway diseases (27.7%). The prevalence of lung and/or airway and cerebrovascular diseases as the primary diagnosis increased with age (r = 0.310, P < .001; and r = 0.156, P < .001, respectively). Noninvasive ventilation was used by 37.2% of all the subjects, with the highest prevalence in those with neuromuscular diseases (54.4%) or chest wall diseases (53.4%). Noninvasive ventilation use was lowest among subjects with brain lesions. Home mechanical ventilation was most commonly prescribed by internists (41.3% of cases), followed by rehabilitation and neurology physicians. These data will aid in planning the optimal health-care system for users of home mechanical ventilation locally and will allow for comparison of home mechanical ventilation use rates among countries.

Analysis of a Pediatric Home Mechanical Ventilator Population.

The population of children requiring home mechanical ventilation has evolved over the years and has grown to include a variety of diagnoses and needs that have led to changes in the care of this unique population. The purpose of this study was to provide a descriptive analysis of pediatric patients requiring home mechanical ventilation after hospitalization and how the evolution of this technology has impacted their care. A retrospective, observational, longitudinal analysis of 164 children enrolled in a university-affiliated home mechanical ventilation program over 26 years was performed. Data included each child's primary diagnosis, date of tracheostomy placement, duration of mechanical ventilation during hospitalization that consisted of home mechanical ventilator initiation, total length of pediatric ICU stay, ventilator settings at time of discharge from pediatric ICU, and disposition (home, facility, or died). Univariate, bivariate, and regression analysis was used as appropriate. The most common diagnosis requiring the use of home mechanical ventilation was neuromuscular disease (53%), followed by chronic pulmonary disease (29%). The median length of stay in the pediatric ICU decreased significantly after the implementation of a ventilator ward (70 d [30-142] vs 36 d [18-67], P = .02). The distribution of subjects upon discharge was home (71%), skilled nursing facility (24%), and died (4%), with an increase in the proportion of subjects discharged on PEEP and those going to nursing facilities over time (P = 0.02). The evolution of home mechanical ventilation has allowed earlier transition out of the pediatric ICU and with increasing disposition to skilled nursing facilities over time. There has also been a change in ventilator management, including increased use of PEEP upon discharge, possibly driven by changes in ventilators and in-patient practice patterns.

Quality of life in home-ventilated children and their families.

Perceived QOL by children with HMV and their families is lower than that of healthy children. Parents are happy to care for their children at home, even though it negatively affects family life. What is Known: • The use of home mechanical ventilation (HMV) in children has increased over the last years. • Normal family functioning is usually disrupted by HMV. What is New: • The aim of HMV is to provide a lifestyle similar to that of healthy children, but perceived quality of life by these patients and their parents is low. • Most of the families caring for children on HMV agree that support and resources provided by national health institutions is insufficient.

Home Mechanical Ventilation: An Overview.

Prevalence studies have shown heterogeneous use of home mechanical ventilation in different conditions, with a marked increase in uptake especially in users of noninvasive ventilation. Although randomized controlled trials have examined noninvasive ventilation in acute exacerbations of chronic obstructive pulmonary disease, for weaning from invasive ventilation and for postextubation respiratory failure, the evidence base for long-term noninvasive ventilation and comparisons with invasive ventilation are less well developed. The combination of noninvasive ventilation and cough-assist devices has reduced the indications for tracheotomy ventilation in some situations (e.g., Duchenne muscular dystrophy, spinal muscular atrophy, myopathies, and amyotrophic lateral sclerosis) and has also prolonged survival. Several excellent overviews have been written on the history of home mechanical ventilation and its evolution from negative pressure to positive pressure techniques, including a systematic review of outcomes. This review, instead, will cover recent trials, trends in the field, outcomes, and safety. Because the greatest growth has been in home noninvasive ventilation, this will be the main focus of this article.

Home mechanical ventilation in childhood-onset hereditary neuromuscular diseases: 13 years' experience at a single center in Korea.

IntroductionChildren with hereditary neuromuscular diseases (NMDs) are at a high risk of morbidity and mortality related to respiratory failure. The use of home mechanical ventilation (HMV) has saved the lives of many children with NMD but, due to a lack of studies, dependable guidelines are not available. We drew upon our experience to compare the various underlying NMDs and to evaluate HMV with regard to respiratory morbidity, the proper indications and timing for its use, and to develop a policy to improve the quality of home noninvasive ventilation (NIV).MethodsWe retrospectively analyzed the medical records of 57 children with childhood-onset hereditary NMDs in whom HMV was initiated between January 2000 and May 2013 at Seoul National University Children's Hospital. The degree of respiratory morbidity was estimated by the frequency and duration of hospitalizations caused by respiratory distress.ResultsThe most common NMD was spinal muscular atrophy (SMA, n = 33). Emergent mechanical ventilation was initiated in 44% of the patients before the confirmed diagnosis, and the indicators of pre-HMV respiratory morbidity (e.g., extubation trials, hypoxia, hospitalizations, and intensive care unit stay) were greater in these patients than in others. The proportion of post-HMV hospitalizations (range, 0.00−0.52; median, 0.01) was lower than that of pre-HMV hospitalizations (0.02−1.00; 0.99) (P < 0.001). Eight patients were able to maintain home NIV. The main causes of NIV failure were air leakage and a large amount of airway secretions.ConclusionsThe application of HMV helped reduce respiratory morbidity in children with childhood-onset hereditary NMD. Patients with SMA type I can benefit from an early diagnosis and the timely application of HMV. The choice between invasive and noninvasive HMV should be based on the patient’s age and NIV trial tolerance. Systematic follow-up guidelines provided by a multidisciplinary team are needed.

Enzyme Replacement Therapy Improves Respiratory Outcomes in Patients with Late-Onset Type II Glycogenosis and High Ventilator Dependency

Type II glycogenosis (GSDII) is a rare and often fatal neuromuscular disorder caused by acid alpha-glucosidase deficiency. Although alglucosidase alfa enzyme replacement therapy (ERT) significantly improves outcomes in subjects with the infantile form, its efficacy in patients with the late-onset one is not entirely clear. The long-term efficacy of ERT in late-onset GSGII complicated by severe pulmonary impairment causing high mechanical ventilation dependency was investigated in this study. The long-term clinical efficacy of ERT was assessed in eight late-onset GSDII patients using home mechanical ventilation (HMV) by comparing their outcomes with those of six historical control patients (GSDII patients) who had received HMV alone. The number of hospitalizations due to pulmonary exacerbations and of hours of daily use of HMV were considered the study's primary efficacy endpoints. The treatment group showed an increased tendency toward shorter follow-up compared to the control group (35.8±29.2 vs. 52.6±8.55months; p=0.04). At the end of the study period, the daily use of HMV (12.5±7.6 vs. 19±14.3h; p=0.004) and the hospitalization rate [incidence rate ratio=0.43 (95% confidence interval 0.18-0.93); p=0.03] were significantly lower in the patients receiving ERT. The differences in the forced vital capacity absolute value and percentage change from baseline were not significantly different in the two groups. ERT reduces ventilator dependency in late-onset GSDII patients and the need for hospitalization due to respiratory exacerbations.

Withdrawal of Invasive Home Mechanical Ventilation in Patients with Advanced Amyotrophic Lateral Sclerosis: Ten Years of Danish Experience

Due to the growing use of home mechanical ventilation in amyotrophic lateral sclerosis (ALS), physicians are increasingly confronted with patients seeking discontinuation of therapy. Yet there are few systematic investigations of the withdrawal of invasive home mechanical ventilation (IHMV). This article aims to describe the medical and patient-related aspects of terminating IHMV in patients with advanced stage ALS. A retrospective, descriptive study was made of all ALS patients cared for at our center from 2002 to 2009 who decided to withdraw their consent for IHMV. All 12 ALS patients (4 females) received continuous IHMV. They had a median age of 61 years (range, 39-69 years). In all cases advance directives for end of life care, particularly concerning withdrawal of treatment, were discussed before the initiation of IHMV. The median time from initiation of IHMV to the decision to terminate treatment was 22 months (range, 1-35 months). The reasons for requests were for all patients a general loss of "meaning in life." Deep sedation was achieved with high dose morphine and diazepam before disconnecting the ventilator. Patients' requests for the withdrawal of IHMV in advanced stage ALS was related to a perceived "loss of meaning in life." Termination of treatment was performed under deep sedation as a medically, legally, and ethically justified procedure.

Discharge planning and management for patients with chronic respiratory failure using home mechanical ventilation

Educational aims To identify some of the clinical issues associated with the safe and effective care of the patient using home ventilatory support. To recognise those patients and clinical situations where problems related to home ventilation are more likely to occur. To assist in developing discharge plans to improve quality of care for patients using long-term home ventilation. Summary The use of home mechanical ventilation (HMV) to manage chronic respiratory failure (CRF) is becoming increasingly prevalent, and is usually delivered by means of a mask interface and pressure preset device. Although the transition from initial acclimatisation and prescription of ventilation to long-term home use is fairly straightforward for many patients with respiratory failure, issues such as nutrition, progressive ventilatory dependence and psychosocial problems can arise. Anticipating these problems and identifying resources and procedures that can reduce the negative impact of complex care carried out in the home is important for the long-term success and safety of HMV.

Home mechanical ventilation: who cares about how patients die?

Home mechanical ventilation (HMV) is increasingly being used to treat chronic hypercapnic respiratory failure arising from different aetiologies, such as restrictive thoracic disorders, neuromuscular disorders (NMD), obesity hypoventilation syndrome and chronic obstructive pulmonary disease (COPD) 1–3. The prognosis of patients receiving HMV depends on both the type and state of disease upon commencement of HMV, with the worst outcome reported in patients with COPD, as well as those with rapidly progressive NMD such as amyotrophic lateral sclerosis (ALS) 3–6. However, in contrast, the survival benefits provided by HMV in individuals with restrictive thoracic disorders or stable, slowly progressive NMD are overwhelming 3–6. Importantly, HMV has also been shown to strongly improve health-related quality of life (HRQoL) in patients with varying disorders including COPD and NMD 7; this improvement was revealed via the application of the Severe Respiratory Insufficiency Questionnaire 8, 9, an instrument specifically developed and validated for patients with HMV use. In addition, enhancements in HRQoL were also reported in several previous trials that used various instruments to assess HRQoL 3–5. Thus, HMV has become the treatment of choice for patients with chronic hypercapnic respiratory failure, which is a sign of major progress in medicine. Nevertheless, the clinical status of all these patients will eventually deteriorate, ultimately leading to death. This scenario is characterised by the more frequent occurrence of threatening symptoms, most likely dyspnoea, and possibly problems related to ventilation, such as more alarm announcements or a greater dependence on daily ventilation. This can occur in the weeks or even months prior to death. Another important factor is the role of family members, who are often involved in the daily management of HMV patients; this is undoubtedly a huge challenge. Of note, spending …

Six minute walking distance in kyphoscoliosis patients with chronic respiratory failure

Aim: To evaluate kyphoscoliosis patients with chronic hypercapnic respiratory failure (CHRF) using the six minute walk test (6MWT) distance (6MWD) and cardio-pulmonary function tests. Method: This prospective cross-sectional study was carried out in a tertiary training and research hospital in Turkey. Kyphoscoliosis patients with CHRF on home mechanical ventilation (HMV) followed in a respiratory intensive care unit (RICU) out-patient clinic were enrolled. Patients’ demographics were recorded as well as transthoracic echocardiography (ECHO), 6MWD, spirometry, arterial blood gas (ABG) values and high resolution chest computed tomography. 6MWT results were compared with other parameters. Results: Thirty four patients with kyphoscoliosis and chronic respiratory insufficiency admitted to our outpatient clinic were included in the study but 25 (17 M) patients underwent 6MWT (8 patients walked with oxygen supplement due to PaO2 &lt; 60 mm Hg). The mean 6MWD was 274.4 ± 76.2 (median 270) m and median 6MWD predicted rate was 43.7% (inter quartile ratio, IQR, 37.6% to 47.7%). Median HMV use was 3 years (IQR 2-4). 6MWD predicted rate, body mass index (BMI), HMV duration were similar in male and female patients. 6MWD correlated well with age, BMI, dyspnea score for baseline 6MWT (r: - 0.59, p &lt; 0.002, r: - 0.58, p &lt; 0.003, r: - 0.55, p &lt; 0.005 respectively) but modestly with forced expiratory volume in one second, pulse rate for baseline 6MWT, pulse saturation rate, fatigue and dyspnea score at end of 6MWT (r: - 0.44, p &lt; 0.048; r: 0.44, p &lt; 0.027; r: - 0.43, p &lt; 0.031; r: - 0.42, p &lt; 0.036; r: - 0.42, p &lt; 0.034 respectively). 6MWD predicted rate was only correlated with dyspnea score at baseline (r: - 0.46, p &lt; 0.022). The systolic pulmonary arterial pressure (PAPs) in 6 (24%) cases was more than 40mmHg, in whom mean PaO2/FiO2 was 301.4 ± 55.4 compared to 280.9 ± 50.2 in those with normal PAPs (p &gt; 0.40). Conclusion: The 6MWT is an easy way to evaluate physical performance limitation in kyphoscoliosis patients with chronic hypercapnic respiratory failure using home mechanical ventilation. Nearly 275 m was the mean distance walked in the 6MWT, but rather than distance in meters, the 6MWD predicted rate according to gender and body mass index equation might be a better way for deciding about physical performance of these patients. Dyspnea score at baseline before the 6MWT may be the most important point that affects 6MWD in this patient population.

Six minute walking distance in kyphoscoliosis patients with chronic respiratory failure

AimTo evaluate kyphoscoliosis patients with chronic hypercapnic respiratory failure (CHRF) using the six minute walk test (6MWT) distance (6MWD) and cardio-pulmonary function tests.MethodThis prospective cross-sectional study was carried out in a tertiary training and research hospital in Turkey. Kyphoscoliosis patients with CHRF on home mechanical ventilation (HMV) followed in a respiratory intensive care unit (RICU) out-patient clinic were enrolled. Patients' demographics were recorded as well as transthoracic echocardiography (ECHO), 6MWD, spirometry, arterial blood gas (ABG) values and high resolution chest computed tomography. 6MWT results were compared with other parameters.ResultsThirty four patients with kyphoscoliosis and chronic respiratory insufficiency admitted to our outpatient clinic were included in the study but 25 (17 M) patients underwent 6MWT (8 patients walked with oxygen supplement due to PaO2 < 60 mm Hg). The mean 6MWD was 274.4 ± 76.2 (median 270) m and median 6MWD predicted rate was 43.7% (inter quartile ratio, IQR, 37.6% to 47.7%). Median HMV use was 3 years (IQR 2-4). 6MWD predicted rate, body mass index (BMI), HMV duration were similar in male and female patients. 6MWD correlated well with age, BMI, dyspnea score for baseline 6MWT (r: - 0.59, p < 0.002, r: - 0.58, p < 0.003, r: - 0.55, p < 0.005 respectively) but modestly with forced expiratory volume in one second, pulse rate for baseline 6MWT, pulse saturation rate, fatigue and dyspnea score at end of 6MWT (r: - 0.44, p < 0.048; r: 0.44, p < 0.027; r: - 0.43, p < 0.031; r: - 0.42, p < 0.036; r: - 0.42, p < 0.034 respectively). 6MWD predicted rate was only correlated with dyspnea score at baseline (r: - 0.46, p < 0.022). The systolic pulmonary arterial pressure (PAPs) in 6 (24%) cases was more than 40 mmHg, in whom mean PaO2/FiO2 was 301.4 ± 55.4 compared to 280.9 ± 50.2 in those with normal PAPs (p > 0.40).ConclusionThe 6MWT is an easy way to evaluate physical performance limitation in kyphoscoliosis patients with chronic hypercapnic respiratory failure using home mechanical ventilation. Nearly 275 m was the mean distance walked in the 6MWT, but rather than distance in meters, the 6MWD predicted rate according to gender and body mass index equation might be a better way for deciding about physical performance of these patients. Dyspnea score at baseline before the 6MWT may be the most important point that affects 6MWD in this patient population.

Home Ventilation for Children with Chronic Respiratory Failure in Istanbul

Background: The number of children on home mechanical ventilation (HMV) has increased markedly in Europe and North America but little is known about the HMV use and outcomes in children in Turkey. Objective: To review clinical conditions and outcome of children who were discharged from the hospital on respiratory support. Methods: Thirty-four patients assessed at the Marmara University Hospital in Istanbul who had been receiving ventilatory support at home for more than 3 months were included in the study. Results: Thirty-four patients with a median age of 5.1 years were discharged home with ventilatory support. HMV was started in 2001 at our institution and the number of children treated has increased substantially since then (2001: n = 1, 2002: n = 3, 2003: n = 3, 2004: n = 2, 2005: n = 14, 2006: n = 11).Ventilatory support was started at a median age of 1.8 years and continued for 13 months. Eleven (32.4%) patients received invasive mechanical ventilation via tracheostomy and 23 (67.6%) patients received noninvasive mechanical ventilation. Sixteen children (47.1%) were on noninvasive mechanical ventilation via nasal mask while 7 (20.6%) used a face mask. Seven (20.6%) patients received ventilatory support for 24 h and 27 (79.4%) patients were supported only during sleep. Twenty-four (70.6%) children received supplemental oxygen in addition to ventilatory support. Three patients successfully came off ventilatory support; 11 patients died during follow-up. None of the patients had home nursing and there were no life-threatening complications. Conclusions: A rapidly rising trend of HMV use in chronic respiratory failure (CRF) has been observed in this study. HMV can be safely applied in selected children with CRF with close monitoring and proper follow-up in developing countries despite the lack of home nursing.

When should home mechanical ventilation be started in patients with different neuromuscular disorders?

Current international consensus guidelines identify a number of indicators for the establishment of home mechanical ventilation (HMV) for patients with neuromuscular diseases but do not address the possible clinical differences between each of the underlying disorders. This study assessed the differences in the physiological parameters of patients with neuromuscular disease commenced on HMV for the treatment of symptomatic chronic hypercapnic respiratory failure. Patients commenced on HMV for the treatment of symptomatic chronic hypercapnic respiratory failure over a 9-year period were studied. Physiological parameters at the time of referral for HMV, impact of HMV and survival were analysed. The study recruited 66 patients with neuromuscular disease. Thirty-one patients had rapidly progressive disease: amyotrophic lateral sclerosis (ALS, n = 19), Duchenne muscular dystrophy (DMD, n = 12) and 35 patients had slowly progressive disease. Mean FVC at HMV onset was 40.3 +/- 17.5% predicted in all patients, but was >50% predicted in eight patients (12%). ALS patients were more hypercapnic (P = 0.03) and more hypoxaemic (P < 0.001), but had better FEV(1) at HMV onset, compared with DMD patients (P = 0.005). Maximal inspiratory mouth occlusion pressure (PImax) was 3.0 +/- 1.6 kPa in all patients, but values were lower compared with international consensus guidelines (5.88 kPa). Median survival in DMD, slowly progressive diseases and ALS was 132, 82 and 16 months, respectively (P < 0.001). Blood gases and lung function parameters vary substantially between patients with differing underlying neuromuscular disorders when commenced on HMV for the treatment of symptomatic chronic hypercapnic respiratory failure. In contrast, PImax is equally reduced in all patients and more severely reduced compared with consensus guidelines. The specific underlying neuromuscular disease has a major impact on outcome. Specific selection criteria are needed for the use of HMV in the different diseases that comprise neuromuscular disorders.