A5059873278- Respiratory Support and Mechanisms
- Obstructive Sleep Apnea Research
- Neuroscience of respiration and sleep
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Neurogenetic and Muscular Disorders Research
- Airway Management and Intubation Techniques
- Tracheal and airway disorders
- Neonatal Respiratory Health Research
- Family and Patient Care in Intensive Care Units
- Intensive Care Unit Cognitive Disorders
- Muscle Physiology and Disorders
- Long-Term Effects of COVID-19
- Palliative Care and End-of-Life Issues
- Childhood Cancer Survivors' Quality of Life
- Mechanical Circulatory Support Devices
- Cardiac Arrest and Resuscitation
- Dysphagia Assessment and Management
- Pleural and Pulmonary Diseases
- Cardiovascular and Diving-Related Complications
- Myasthenia Gravis and Thymoma
- Delphi Technique in Research
- Sleep and related disorders
- Amyotrophic Lateral Sclerosis Research
- Congenital Anomalies and Fetal Surgery
- Ethics in medical practice
Imperial College London
2003-2024
Harefield Hospital
2012-2024
Guy's and St Thomas' NHS Foundation Trust
2009-2024
Royal Brompton & Harefield NHS Foundation Trust
2014-2023
European Respiratory Society
2007-2023
Royal Brompton Hospital
2013-2022
The Recovery Center
2022
European Medicines Agency
2021
St. Vincent's University Hospital
2021
University College Dublin
2021
Spinal muscular atrophy is a neurodegenerative disease that requires multidisciplinary medical care. Recent progress in the understanding of molecular pathogenesis spinal and advances technology have not been matched by similar developments care for patients. Variations practice coupled with differences family resources values resulted variable clinical outcomes are likely to compromise valid measure treatment effects during trials. The International Standard Care Committee Muscular Atrophy...
This is the second half of a two-part document updating standard care recommendations for spinal muscular atrophy published in 2007. part includes updated on pulmonary management and acute issues, topics that have emerged last few years such as other organ involvement severe forms role medications. Ethical issues choice palliative versus supportive are also addressed. These becoming increasingly relevant given recent clinical trials prospect commercially available therapies will likely...
Long term non-invasive ventilation (NIV) reduces morbidity and mortality in patients with neuromuscular chest wall disease hypercapnic ventilatory failure, but preventive use has not produced benefit normocapnic Duchenne muscular dystrophy. Individuals nocturnal hypercapnia daytime normocapnia were randomised to a control group or NIV examine whether hypoventilation is valid indication for NIV.Forty eight congenital aged 7-51 years vital capacity<50% predicted underwent overnight respiratory...
<h3>BACKGROUND</h3> Respiratory failure is the commonest cause of death in patients with Duchenne muscular dystrophy (DMD). Life expectancy less than one year once diurnal hypercapnia develops. This study examines effects nasal intermittent positive pressure ventilation (NIPPV) on survival symptomatic established ventilatory failure. <h3>METHODS</h3> Nocturnal NIPPV was applied 23 consecutive DMD mean (SD) age 20.3 (3.4) years who presented and nocturnal hypercapnia. <h3>RESULTS</h3> One...
Adults and children with neuromuscular disease exhibit weak cough are susceptible to recurrent chest infections, a major cause of morbidity mortality. Mechanical insufflation/exsufflation may improve efficacy by increasing peak flow. It was hypothesised that mechanical would produce greater increase in flow than other modes augmentation. The acceptability these interventions also compared. Twenty-two patients aged 10–56 yrs (median 21 yrs) 19 age-matched controls were studied. Spirometry...
Nasal intermittent positive pressure ventilation (NIPPV) is a new technique which has rapidly supplanted other non-invasive methods of over the last 5-10 years. Data on its effectiveness are limited.The outcome long term domiciliary NIPPV been analysed in 180 patients with hypercapnic respiratory failure predominantly due to chest wall restriction, neuromuscular disorders, or chronic obstructive lung disease. One hundred and thirty eight were started electively, 42 following an acute...
< Acknowledgement of stress factors and mental health issues, requirement for wider support through organizations, practical focused individual care are needed patients their parents or carers.[D] <Routine pain evaluation should be part standard clinical assessment in all children young people with neuromuscular disorders.[D] Attention to the physical impact chronic illness vigilant symptom management is required.[D] Dyspnoea a subjective feeling, which can respond non-drug measures...
<h3>Importance</h3> Continuous positive airway pressure (CPAP) and high-flow nasal oxygen (HFNO) have been recommended for acute hypoxemic respiratory failure in patients with COVID-19. Uncertainty exists regarding the effectiveness safety of these noninvasive strategies. <h3>Objective</h3> To determine whether either CPAP or HFNO, compared conventional therapy, improves clinical outcomes hospitalized COVID-19–related failure. <h3>Design, Setting, Participants</h3> A parallel group,...
<h3>Introduction</h3> Automatic titration modes of non-invasive ventilation, including average volume assured pressure support (AVAPS), are hybrid technologies that target a set by automated adjustment (PS). These could offer potential advantages over fixed level PS, in particular, patients who super obese. <h3>Methods</h3> Consecutive with obesity hypoventilation syndrome were enrolled two-centre prospective single-blind randomised controlled trial AVAPS versus fixed-level PS using strict...
Hospitalised patients with coronavirus disease 2019 (COVID-19) as a result of SARS-CoV-2 infection have high mortality rate and frequently require noninvasive respiratory support or invasive ventilation. Optimising standardising management through evidence-based guidelines may improve quality care therefore patient outcomes.A task force from the European Respiratory Society endorsed by Chinese Thoracic identified priority interventions (pharmacological non-pharmacological) for initial...
Congenital muscular dystrophies are a group of rare neuromuscular disorders with wide spectrum clinical phenotypes. Recent advances in understanding the molecular pathogenesis congenital dystrophy have enabled better diagnosis. However, medical care for patients remains very diverse. Advances many areas technology not been adopted practice. The International Standard Care Committee Muscular Dystrophy was established to identify current issues, review literature evidence-based practice, and...
<h3>Background</h3> There are widely discrepant views on the respiratory management of infants with spinal muscular atrophy (SMA) type I. Typically, is palliative. <h3>Design</h3> A descriptive study interventions and investigations reported that were offered to a cohort 13 children SMA I referred our centre. <h3>Interventions investigations</h3> included sleep studies, provision non-invasive positive pressure ventilation (NIPPV) for ventilatory support/dependency physiotherapy use...
Central sleep apnoea/Cheyne-Stokes respiration (CSA/CSR) is a risk factor for increased mortality and morbidity in heart failure (HF). Adaptive servo-ventilation (ASV) non-invasive ventilation modality the treatment of CSA/CSR patients with HF.SERVE-HF multinational, multicentre, randomized, parallel trial designed to assess effects addition ASV (PaceWave, AutoSet CS; ResMed) optimal medical management compared alone (control group) symptomatic chronic HF, LVEF ≤45%, predominant CSA. The...
Background The number of children receiving domiciliary ventilatory support has grown over the last few decades driven largely by introduction and widening applications non-invasive ventilation. Ventilatory may be used with intention increasing survival, or to facilitate discharge home and/or palliate symptoms. However, outcome this intervention transitioning adult care as a consequence longer survival is not yet clear. Methods In retrospective cohort study, we analysed in (<17 years)...
<h3>Objective</h3> To assess the impact of home telemonitoring on health service use and quality life in patients with severe chronic lung disease. <h3>Design</h3> Randomised crossover trial 6 months standard best practice clinical care (control group) addition telemonitoring. <h3>Participants</h3> 68 disease (38 COPD; 30 respiratory failure due to other causes), who had a hospital admission for an exacerbation within randomisation either used long-term oxygen therapy or arterial saturation...
Background Many people are dying from coronavirus disease 2019 (COVID-19), but consensus guidance on palliative care in COVID-19 is lacking. This new life-threatening has put healthcare systems under pressure, with the increased need of provided to many patients by clinicians who have limited prior experience this field. Therefore, we aimed make recommendations for using Convergence Opinion Recommendations and Evidence (CORE) process. Methods We invited 90 international experts complete an...
BACKGROUND: Domiciliary assisted ventilation, using negative or positive pressure devices, is an effective treatment for respiratory failure due to chest wall deformity and neuromuscular disease. Negative ventilators have been used with some success in patients chronic obstructive lung disease hospital, but attempts continue at home disappointing. This study evaluates the practicalities of nasal intermittent ventilation effect on sleep quality life. METHODS AND RESULTS: Twelve hypercapnic...
BACKGROUND: The non-invasive technique of nasal intermittent positive pressure ventilation (NIPPV) has an established role in providing domiciliary nocturnal ventilatory support patients with chest wall disorders, neuromuscular disease, and chronic obstructive lung disease. NIPPV was used to simplify management assist the return spontaneous breathing respiratory insufficiency who had failed wean from conventional (IPPV). METHODS: A trial carried out 22 weaning difficulties. Nine disorders or...