Randomised crossover trial of telemonitoring in chronic respiratory patients (TeleCRAFT trial).

DESIGN: Randomised crossover trial with 6 months of standard best practice clinical care (control group) and 6 months with the addition of telemonitoring. PARTICIPANTS: 68 patients with chronic lung disease (38 with COPD; 30 with chronic respiratory failure due to other causes), who had a hospital admission for an exacerbation within 6 months of randomisation and either used long-term oxygen therapy or had an arterial oxygen saturation (SpO2) of <90% on air during the previous admission. Individuals received telemonitoring (second-generation system) via broadband link to a hospital-based care team. OUTCOME MEASURES: Primary outcome measure was time to first hospital admission for an acute exacerbation. Secondary outcome measures were hospital admissions, general practitioner (GP) consultations and home visits by nurses, quality of life measured by EuroQol-5D and hospital anxiety and depression (HAD) scale, and self-efficacy score (Stanford). RESULTS: Median (IQR) number of days to first admission showed no difference between the two groups­77 (114) telemonitoring, 77.5 (61) control ( p=0.189). Hospital admission rate at 6 months increased (0.63 telemonitoring vs 0.32 control p=0.026). Home visits increased during telemonitoring; GP consultations were unchanged. Self-efficacy fell, while HAD depression score improved marginally during telemonitoring. CONCLUSIONS: Telemonitoring added to standard care did not alter time to next acute hospital admission, increased hospital admissions and home visits overall, and did not improve quality of life in chronic respiratory patients. TRIAL REGISTRATION NUMBER: NCT02180919 (ClinicalTrials.gov).

Analysis of home support and ventilator malfunction in 1,211 ventilator-dependent patients.

Risk management is an important aspect of home ventilation (HV). We examined the nature of calls to a home support helpline to identify patient/equipment problems and strategies to minimise risk for patients, healthcare teams and manufacturers. From 1,211 adult and paediatric patients with neuromuscular disease, chronic obstructive pulmonary disease or chest wall disease receiving HV, all calls to a dedicated respiratory support telephone hotline between January 1, 2006 and June 30, 2006 were analysed. 1,199 patients received noninvasive ventilation, 12 tracheostomy ventilation; 149 had two ventilators for 24-h ventilator dependency. There was a mean of 528 daytime calls per month and 14 calls a month at night. Following 188 calls, a home visit was performed; these identified a technical problem that could either be solved in the patient's home in 64% or required replacement or new parts in 22% of cases. In 25 calls in which no mechanical fault was identified, 13 patients were either found to be unwell or required hospital admission. Patients using HV have a substantial requirement for assistance, with most technical problems being resolved simply. Where no fault can be found during an equipment check, the patient themselves may be unwell and should receive early clinical evaluation. The patient may have mistaken clinical deterioration for an equipment problem.

Lives on the line? Ethics and practicalities of duty of care in pandemics and disasters.

Pandemics and acute emergencies raise pressing medical, ethical and organisational challenges. These include global governance, priority setting, triaging of patients, allocation of scarce resources and restricting individual liberty in the interests of public health. We will focus particularly on an issue of direct relevance to all respiratory team members, i.e. what is the duty of the healthcare worker to continue working in the face of personal risk, and draw lessons from guidelines, ethical considerations, past pandemics and evolving experience with H1N1 swine influenza.

Autotitrating versus standard noninvasive ventilation: a randomised crossover trial.

The aim of the present study was to compare the efficacy of automatic titration of noninvasive ventilation (NIV) with conventional NIV in stable neuromuscular and chest wall disorder patients established on long-term ventilatory support. In total, 20 neuromuscular and chest wall disease patients with nocturnal hypoventilation treated with long-term NIV completed a randomised crossover trial comparing two noninvasive pressure support ventilators: a standard bilevel ventilator (VPAP III) and a novel autotitrating bilevel ventilator (AutoVPAP). Baseline physiological measurements, overnight polysomnography and Holter monitoring were repeated at the end of each 1-month treatment period. Nocturnal oxygenation was comparable between the autotitrating device and standard ventilator, as were sleep efficiency, arousals and heart rate variability. However, there was a small significant increase in mean overnight transcutaneous carbon dioxide tension (median (interquartile range) 7.2 (6.7-7.7) versus 6.7 (6.1-7.0) kPa) and a decrease in percentage stage 1 sleep (mean+/-sd 16+/-9 versus 19+/-10%) on autotitrating NIV compared with conventional NIV. Autotitrating noninvasive ventilation using AutoVPAP produced comparable control of nocturnal oxygenation to standard nonivasive ventilation, without compromising sleep quality in stable neuromuscular and chest wall disease patients requiring long-term ventilatory support for nocturnal hypoventilation.

Congenital myasthenic syndromes in childhood: diagnostic and management challenges.

The Congenital Myasthenic Syndromes (CMS), a group of heterogeneous genetic disorders of neuromuscular transmission, are often misdiagnosed as congenital muscular dystrophy (CMD) or myopathies and present particular management problems. We present our experience of 46 children with CMS, referred to us between 1992-2007 with provisional diagnoses of congenital myopathy (22/46), CMS or limb-girdle myasthenia (9/46), central hypotonia or neurometabolic disease (5/46), myasthenia gravis (4/46), limb-girdle or congenital muscular dystrophy (4/46) and SMA (2/46). Diagnosis was often considerably delayed (up to 18y4 m), despite the early symptoms in most cases. Diagnostic clues in the neonates were feeding difficulties (29/46), hypotonia with or without limb weakness (21/46), ptosis (19/46), respiratory insufficiency (12/46), contractures (4/46) and stridor (6/46). Twenty-five children had delayed motor milestones. Fatigability developed in 43 and a variable degree of ptosis was eventually present in 40. Over the period of the study, the mainstay of EMG diagnosis evolved from repetitive nerve stimulation to stimulation single fibre EMG. The patients were studied by several different operators. 66 EMGs were performed in 40 children, 29 showed a neuromuscular junction abnormality, 7 were myopathic, 2 had possible neurogenic changes and 28 were normal or inconclusive. A repetitive CMAP was detected in only one of seven children with a COLQ mutation and neither of the two children with Slow Channel Syndrome mutations. Mutations have been identified so far in 32/46 children: 10 RAPSN, 7 COLQ, 6 CHRNE, 7 DOK7, 1 CHRNA1 and 1 CHAT. 24 of 25 muscle biopsies showed myopathic changes with fibre size variation; 14 had type-1 fibre predominance. Three cases showed small type-1 fibres resembling fibre type disproportion, and four showed core-like lesions. No specific myopathic features were associated with any of the genes. Twenty children responded to Pyridostigmine treatment alone, 11 to Pyridostigmine with either 3, 4 DAP or Ephedrine and five to Ephedrine alone. Twenty one children required acute or chronic respiratory support, with tracheostomy in 4 and nocturnal or emergency non-invasive ventilation in 9. Eight children had gastrostomy. Another 11 were underweight for height indicative of failure to thrive and required dietetic input. A high index of clinical suspicion, repeat EMG by an experienced electromyographer and, if necessary, a therapeutic trial of Pyridostigmine facilitates the diagnosis of CMS with subsequent molecular genetic confirmation. This guides rational therapy and multidisciplinary management, which may be crucial for survival, particularly in pedigrees where previous deaths have occurred in infancy.

A high prevalence of sleep disordered breathing in men with mild symptomatic chronic heart failure due to left ventricular systolic dysfunction.

BACKGROUND: Sleep disordered breathing (SDB) is common in severe chronic heart failure (CHF) and is associated with increased morbidity and mortality. The prevalence of SDB in mild symptomatic CHF is unknown. AIM: The aim of this study was to determine the prevalence and characteristics of SDB in male patients with NYHA class II symptoms of CHF. METHODS AND RESULTS: 55 male patients with mild symptomatic CHF underwent assessment of quality of life, echocardiography, cardiopulmonary exercise, chemoreflex testing and polysomnography. 53% of the patients had SDB. 38% had central sleep apnoea (CSA) and 15% had obstructive sleep apnoea. SDB patients had steeper VE/VCO(2) slope [median (inter-quartile range) 31.1 (28-37) vs. 28.1 (27-30) respectively; p=0.04], enhanced chemoreflexes to carbon dioxide during wakefulness [mean+/-sd: 2.4+/-1.6 vs. 1.5+/-0.7 %VE Max/mmHg CO(2) respectively; p=0.03], and significantly higher levels of brain natriuretic peptide and endothelin-1 compared to patients without SDB. No differences in left ventricular ejection fraction, percent predicted peak oxygen uptake, or symptoms of SDB were observed. CONCLUSIONS: A high prevalence of SDB was found in men with mild symptomatic CHF. Patients with SDB could not be differentiated by symptoms or by routine cardiac assessment making clinical diagnosis of SDB in CHF difficult.

Impact of age on breathing and resistive pressure in people with and without sleep apnea.

We investigated the effect of age on breathing and total pulmonary resistance (RL) during sleep by studying elderly (>65 yr) and young (25-38 yr) people without sleep apnea (EN and YN, respectively) matched for body mass index (BMI). To determine the impact of sleep apnea on age-related changes in breathing, we studied elderly and young apneic patients (EA and YA, respectively) matched for apnea and BMI. In all groups (n = 11), breathing during periods of stable sleep was analyzed to evaluate the intrinsic variability of respiratory control mechanisms. In the absence of sleep apnea, the variability of the breathing was similar in the elderly and young [mean (+/- SD) coefficient of variation (CV) of tidal volume (VT); wake: EN 21.0 +/- 14.9%, YN 14.7 +/- 5.5%; sleep: EN 14.0 +/- 6.0%; YN 11.5 +/- 6.4%]. In patients with sleep apnea, breathing during stable sleep was more irregular, but there were no age-related differences (CV of VT; wake: EA 22.0 +/- 11.6%, YA 16.7 +/- 11.3%; sleep: EA 32.8 +/- 24.9%, YA 25.2 +/- 16.3%). In addition, EN tended to have a higher RL (n = 6, RL midinspiration, wake: EN 7.1 +/- 3.0; YN 9.1 +/- 6.4 cmH(2)O. l(-1). s, sleep: EN 17.5 +/- 11.7; YN 9.8 +/- 2.0 cmH(2)O. l(-1). s). We conclude that aging per se does not contribute to the intrinsic variability of respiratory control mechanisms, although there may be a lower probability of finding elderly people without respiratory instability.

Lung volume reduction surgery in a ventilated patient with severe pulmonary emphysema.

There are a limited number of reports in the literature cocerning lung volume reduction surgery in patients receiving mechanical ventilation. We present a case in which a ventilator-dependent patient with apparent endstage pulmonary emphysema underwent lung volume reduction with a successful outcome. Although the role of this procedure for selected nonventilated patients has been widely discussed its use in ventilated patients is still not clearly defined. We show that lung volume reduction surgery may facilitate ventilatory weaning in such cases and improve functional status.

Sleep apnoea and daytime function in the elderly--what is the impact of arousal frequency?

Arousals from sleep result in hyperventilation and hypocapnia that can lead to sleep apnoea. We have investigated whether sleep apnoea in the elderly is associated with more arousals compared with younger people. Additionally, the impact of arousals on daytime symptoms was noted. Four groups (n = 11) of elderly (> 65 years) and young (< 39 years) apnoeic (EA and YA), and age-matched non-apnoeics (EN and YN) were studied. The arousal index (AI) and apnoea/hypopnoea index were determined from polysomnography. Sleepiness (Epworth Sleepiness Scale) and Quality of life (QoL, SF-36) were assessed. The mean (SD) AI was: EN 23.1 (7.6), EA 46.5 (8.8), YN 13.2 (6.6), YA 38.5 (12.1) events/h. AI was higher in the elderly (P = 0.002) and in apnoeics (P = 0.001); however, the increase in AI associated with sleep apnoea was not age dependent (P = 0.73). The influence of sleep apnoea on sleepiness was similar in both age groups. YA but not EA reported reduced physical functioning (P = 0.04), vitality (P = 0.007) and general health (P = 0.04) compared to non-apnoeics. We conclude that (1) the effect of sleep apnoea on arousal is no greater in the elderly compared to the young (2) despite similar levels of sleepiness, elderly apneoics perceive a reduced loss of QoL compared to younger patients.

Nasal ventilation in progressive neuromuscular disease: experience in adults and adolescents.

A nihilistic approach to ventilatory support in progressive neuromuscular disorders is no longer acceptable. Noninvasive ventilation may improve survival and quality of life in some patients. In other situations, worthwhile palliation of symptoms of nocturnal hypoventilation and dyspnoea may be achieved. Noninvasive ventilation combined with cough assistance may obviate the need for tracheostomy ventilation, or can be employed as part of a staged approach. Individual titration of ventilatory support to meet the needs of the individual and family is vital. The participation of the patient, family and carers in decision-making is crucial and advanced directives are likely to be used increasingly.

Nasal intermittent positive pressure ventilation in neuromuscular and chest wall disease.

The short- and long-term physiological, functional and economic benefits of NIPPV in patients with chronic chest wall and stable neuromuscular disease have been confirmed and its advantages over non-invasive negative pressure ventilation outlined. Current research should help provide guidelines for patient selection and the most appropriate use of NIPPV in progressive neuromuscular disorders.

Weaning difficulties.

All units providing ventilatory support will experience patients with weaning difficulties. The factors which contribute to weaning problems include the pathophysiology of the illness, the extent of underlying chronic disease, and equipment, physician-related and organisational issues. In this article ways to resolve these problems are outlined.

Nocturnal ventilation in neuromuscular disease--when and how?

While much evidence has been gained over the last decade to support the use of non-invasive ventilation (NIV) in a series of acute and chronic applications, some issues still need to be addressed, particularly regarding the timing of initiation of NIV and the best methods of implementation. This article discusses the role of NIV, if any, in preventing respiratory decline, as a physiotherapy adjunct, and in perioperative care for adults and children with neuromuscular disease.

Non invasive ventilation.

Non-invasive ventilation refers to the technique of providing ventilatory support to a patient without an endo/orotracheal airway. It is a promising and rapidly upcoming new technique and is being used as first line therapy in a wide variety of conditions causing respiratory failure. The major indications for its use include respiratory failure due to a variety of causes (chest wall abnormalities, neuromuscular disease, COPD), weaning and stabilization of cardio-respiratory status before and after surgery. Patients who are candidates for this modality usually have a hypercapnic respiratory failure but are able to protect the airway and cooperate with treatment. The biggest advantage of the technique is its simplicity and avoidance of complications of intubation like trauma, infection and delayed complications like tracheal stenosis. Patient comfort is significantly improved and important functions like speech, swallowing and cough are preserved. Several purpose built ventilators are available for use including pressure preset and volume present machines, each of which have their own advantages and disadvantages in clinical practice. A range of patient interfaces is available. The initiation of non-invasive ventilation is much easier as compared to invasive ventilation and can be done for most patients in an intermediary care unit thereby cutting down treatment costs and saving precious intensive care beds. Titration of ventilatory parameters can usually be done using simple tests like oxymetry and blood gases. Several technique related problems like skin pressure sores, nasal symptoms and abdominal distension can be managed with simple measures. Non invasive ventilation has got a special and evolving role in management of COPD, both in acute exacerbations and chronic respiratory failure. In short, the advantages of this form of ventilation are numerous and physicians must familiarize themselves with this new technique, facilities for which should be available in all hospitals admitting patients with respiratory failure.

Risk management protocol for gastrostomy and jejunostomy insertion in ventilator dependent infants.

Gastrostomy, gastrojejunostomy and anti-reflux surgery in infants and children who are chronically ventilator dependent are associated with significant risk of morbidity and mortality. We report outcomes of 22 high risk children who underwent these procedures at our centre. Pre-operative investigations included: overnight oxygen and carbon dioxide monitoring and subsequent optimisation of ventilatory support, echocardiography, video fluoroscopy, and assessment of gastroesophageal reflux. We carried out 24 procedures under general anaesthesia. Twenty-one children used ventilatory support pre-operatively. Median age of first surgical procedure was 18 months (range 3-180). Supplementary feeding was commenced in 20 children prior to procedure, median age 9 months (1-31). Median PICU length of stay was 1 (1-8) days. No children died in the post-operative period. Extubation was possible within 24h in 87% of cases. Complications included; atelectasis (n=2), ileus (n=2), abdominal distension (n=4) and loose stools (n=1). We conclude that, in this high risk cohort of ventilator dependent children with predominantly neuromuscular disorders, with careful assessment, operative intervention can be carried out under general anaesthesia, with the child being extubated early back onto their routine ventilatory support and aggressive airway clearance. Additionally this protocol can minimise post-operative complications and is associated with a good outcome in the majority.

Outcome of goal-directed non-invasive ventilation and mechanical insufflation/exsufflation in spinal muscular atrophy type I.

BACKGROUND: There are widely discrepant views on the respiratory management of infants with spinal muscular atrophy (SMA) type I. Typically, management is palliative. DESIGN: A descriptive study of interventions and investigations is reported that were offered to a cohort of 13 children with SMA type I referred to our centre. Interventions and investigations included sleep studies, provision of non-invasive positive pressure ventilation (NIPPV) for ventilatory support/dependency and for physiotherapy and the use of mechanical insufflation/exsufflation (MI-E). RESULTS: NIPPV was provided for the following indications: continuous positive airways pressure flow driver dependency (n=3), nocturnal hypoventilation (n=3), to enable successful extubation (n=2), in anticipation of respiratory decompensation (n=3), and oxygen dependency/decompensation (n=2). NIPPV and MI-E were used for successful protocol-led extubations (n=9) but not non protocol-led successes (n=3). NIPPV was essential for discharge home in patients with ventilatory dependency (n=7) and was used for palliation of respiratory symptoms (n=4). Chest wall shape improved with NIPPV. The parents of children who died (n=5) were positive about the use of these techniques. CONCLUSION: NIPPV can be used to facilitate discharge home, and MI-E is helpful in this group. This symptom and goal-directed approach can be used to inform medical decision making and to help parents make informed choices about the appropriateness of respiratory interventions in SMA type I.