Domiciliary Ventilation Research Articles

Domiciliary ventilation in chronic obstructive pulmonary disease: where are we?

This quality improvement project aimed to reduce the 9no show9 rate in a paediatric neurology clinic in Qatar. No show, in outpatient clinics, is defined as patients who fail to attend their scheduled clinic appointments. It is one of the targets for improving quality of care. It leads to longer waiting times for patients to be seen in outpatient clinics, and the result is patients missing their important appointments. It also results in a waste of the clinic resources, and physician and other healthcare practitioners9 time. This study was undertaken as part of the CCITP (clinical care improvement training programme). A project team was assembled with coaching support. The department chairman and the appointment system personnel were involved. Baseline and ongoing measures were collected and charted. The baseline no-show rate was identified as 49%. Following three intervention PDSAs, mainly addressing communication and appointment flexibility, the post intervention no-show rate dropped to 18% and was sustained below the target of 25% for two years. Better communication and appointment flexibility can significantly reduce the no-show rate in outpatient clinics.

The effect of a time delay on the measurement of capillary blood gases

Patients using domiciliary nasal ventilation, or long-term oxygen, require regular assessment which could be carried out in the home. Blood gas analysis may be regarded as an essential part of the assessment. The present study investigated the effect of a 1-h time delay on the measurement of capillary blood gases. Four samples of arterialized earlobe blood were collected from 15 outpatients. One sample was analysed immediately, the other three were stored on crushed ice and analysed at intervals of 30, 45, and 60 min post-collection. In order to examine any range effect, a wide range of PaO 2 values were examined. The delay, at all levels, resulted in minor changes in the measurement of PaO 2, which would be unlikely to alter clinical management. The technique might be used for the reliable assessment of patients in the home.

Outcome of domiciliary nasal intermittent positive pressure ventilation in restrictive and obstructive disorders.

Nasal intermittent positive pressure ventilation (NIPPV) is a new technique which has rapidly supplanted other non-invasive methods of ventilation over the last 5-10 years. Data on its effectiveness are limited. The outcome of long term domiciliary NIPPV has 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 patients were started on NIPPV electively, and 42 following an acute hypercapnic exacerbation. Outcome measures were survival (five year probability of continuing NIPPV), pulmonary function, and health status. A crossover study from negative pressure ventilation to NIPPV was carried out in a subgroup of patients. Five year acturial probability of continuing NIPPV for individuals with early onset scoliosis (n = 47), previous poliomyelitis (n = 30), following tuberculous lung disease (n = 20), general neuromuscular disorders (n = 29), and chronic obstructive pulmonary disease (n = 33) was 79% (95% CI 66 to 92), 100%, 94% (95% CI 83 to 100), 81% (95% CI 61 to 100), 43% (95% CI 6 to 80), respectively. Most of the patients with bronchiectasis died within two years. One year after starting NIPPV electively the mean (SD) PaO2 compared with the pretreatment value was +1.8 (1.9) kPa, mean PaCO2 -1.4 (1.3) kPa in patients with extrapulmonary restrictive disorders, and PaO2 +0.8 (1.0) kPa, PaCO2 -0.9 (0.8) kPa in patients with obstructive lung disease. Arterial blood gas tensions improved in patients transferred from negative pressure ventilation to NIPPV. Health status was ranked highest in patients with early onset scoliosis, previous poliomyelitis, and following tuberculous lung disease. In the group as a whole health perception was comparable to outpatients with other chronic disorders. The long term outcome of domiciliary NIPPV in patients with chronic respiratory failure due to scoliosis, previous poliomyelitis, and chest wall and pulmonary disease secondary to tuberculosis is encouraging. The results of NIPPV in patients with COPD and progressive neuromuscular disorders show benefit in some subgroups. The outcome in end stage bronchiectasis is poor.

Management of mitochondrial disease on an intensive care unit

Patients with mitochondrial disease may present to the Intensive Care Unit (ICU) with a variety of neurological and general medical disorders. Eleven patients were admitted to a neurological ICU between 1970 and 1992 because of respiratory insufficiency, status epilepticus and/or metabolic encephalopathy associated with mitochondrial disease. Respiratory impairment occurred in eight patients and was associated with nocturnal hypoventilation due to respiratory muscle weakness, aspiration due to bulbar weakness and abnormalities of central control leading to a reduced CO2 drive, irregular respiratory patterns and sleep apnoea. Seven patients received continuous respiratory support during the acute illness; three were subsequently weaned to domiciliary ventilation, and four died. Five patients had stroke-like episodes, which in two were recurrent. Four patients developed tonic-clonic grand mal epilepsy associated with myoclonic fits (2 patients), absences (2), focal fits (1) and status epilepticus (2). Encephalopathy was associated with recurrent lactic acidosis (2 patients), cardiac failure (2), hyponatraemia (2), renal abnormalities (3) and complete heart block (1). Although rare, mitochondrial disease should be considered in any patient with unexplained respiratory failure, intractable epilepsy, lactic acidosis or recurrent stroke.

Motor neurone disease-a study of prevalence and disability

We conducted a point prevalence study of motor neurone disease in the counties of South Glamorgan, Mid Glamorgan and Gwent, whose combined population is estimated at 1,394,400. A total of 56 patients were identified, giving a point prevalence for motor neurone disease in this area of 4.02/100,000 on 22/06/92. These cases were reclassified according to the World Federation of Neurology criteria for amyotrophic lateral sclerosis following a further clinical assessment, when disability was also evaluated (in 49 cases). Seven patients were reclassified on review of their medical records alone. Overall, 38 were classified as either definite or probable cases, which gave a point prevalence of 2.73/100,000. Of these patients, 94% (46/49) were living at home, 43% (21/49) scored 15 or less on the Barthel index, 65% (32/49) had some degree of mobility impairment, and 20% (10/49) were wheelchair-dependent. Swallowing speed was reduced (< 10 ml/s) in 67% (33/49), with three requiring feeding through a percutaneous gastrostomy tube. Vital capacity was < 70% of predicted value for age, sex and height in 67% (29/43). One patient was receiving domiciliary ventilation. These results give a quantitative indication of the dependency in a population of patients with motor neurone disease.

Long term non-invasive domiciliary assisted ventilation for respiratory failure following thoracoplasty.

Ventilatory failure is a well recognised complication of patients who have had a thoracoplasty for tuberculosis, but there are few data regarding the value of long term non-invasive assisted ventilation in this situation. Thirty two patients who had had a thoracoplasty 20-46 years previously and who had developed respiratory failure were treated with nocturnal cuirass assisted ventilation or nasal positive pressure ventilation. Their survival and changes in arterial blood gases, nocturnal oximetry, and pulmonary function tests were assessed. The actuarial survival rates at one, three, five, and seven years after starting treatment were 91%, 74%, 64%, and 55%, respectively. Only seven of the 13 deaths were directly attributable to chronic respiratory or cardiac failure. The arterial PO2, PCO2, mean nocturnal oxygen saturation, vital capacity, and maximal inspiratory and expiratory pressures had all improved at the time of the initial post-treatment assessment (mean 12 days after starting treatment), but no subsequent improvements were seen after up to 48 months of follow up. Neither survival nor physiological improvements were correlated with the patients' age, the interval since thoracoplasty, or the pretreatment arterial blood gas tensions or results of pulmonary function tests. These results show that, even when ventilatory failure has developed, the prognosis with non-invasive assisted ventilation is good and the physiological abnormalities can be partially reversed. Patients who develop respiratory failure after a thoracoplasty should be considered for this type of long term domiciliary treatment.

Nasal masks for domiciliary positive pressure ventilation: patient usage and complications.

Nasal mask discomfort is a major factor in compliance with treatment by nasal intermittent positive pressure ventilation (NIPPV) and nasal continuous positive airway pressure (CPAP). A study of skin complications resulting from mask usage, with particular reference to predisposing factors, was carried out in 66 patients by means of a postal questionnaire. An effective means of managing ulceration at the nasal bridge while continuing therapy is described. Some disruption of treatment due solely to mask discomfort was experienced by 35 patients (53%), consisting of broken skin or open sores in 11 cases (17%). CONCLUSIONS-Although complications resulting from nasal mask usage are common, early identification of patients at risk of developing such complications and appropriate intervention will result in improved patient compliance.

DIAPHRAGMATIC PARALYSIS IN MOTOR NEURONE DISEASE: USE OF NON‐INVASIVE INVESTIGATIVE AND THERAPEUTIC TECHNIQUES

SUMMARY Two cases of bilateral diaphragmatic weakness are described in which the condition was the presenting feature of motor neurone disease. Inspiratory muscle strength was assessed by a non‐invasive technique involving measurements of pressures generated within the mouth. One patient with severe inspiratory muscle weakness is being treated with domiciliary nasal ventilation and has returned to a good‐quality life. The other patient with less severe weakness has thus far required no ventilatory support.

Recommendations concerning the costing and management for patients requiring domiciliary ventilation.

Reinstallation back in their own homes of severely disabled people who are ventilator dependent is increasingly becoming a viable trend. Such a development calls for detailed and imaginative planning and negotiations with several agencies. More than a decade of experience in Southport has highlighted the need for early and comprehensive negotiations to ensure that these profoundly disabled people are not denied a quality of life to which they are entitled and which advances in technology has made possible.

The Swedish modification of the tracheostomy tube to permit speech.

Over the last 20 years the Respiratory Unit at Danderyd Hospital, Sweden has treated and rehabilitated more than 90 patients on domiciliary ventilation, the majority of whom require night-time ventilation. Sixteen patients (11 men and 5 women) with high spinal cord lesions have been treated. Of these 16 patients, 9 are using diaphragmatic pacing. Every patient has a customised tube. If the patient can use diaphragmatic pacing the tube, as a rule, is single fenestrated. In patients using the ventilator full time, the tube is made to fit snugly in the trachea. When such a tube fits 'as a bullet in its tube' there is no need for a cuff. The patient has adequate ventilation and the small leak around the tube also permits good speech function. Sometimes a PEEP-valve is used to get a better voice.

Respiratory involvement in primary muscle disorders: assessment and management

Eighty-four patients with primary disorders of muscle were referred for assessment of respiratory insufficiency between 1978 and 1991. The eventual diagnoses were: 'limb girdle syndromes' (18 patients), adult onset acid maltase deficiency (14), dystrophia myotonica (13), inflammatory disorders (10), congenital myopathies (nine), rigid spine syndromes (five), dystrophies (Duchenne (six), facioscapulohumeral (four), Becker (one)) and miscellaneous (four). Presentations were often insidious, with progressive nocturnal hypoventilation culminating in respiratory failure or arrest, recurrent respiratory tract infections, or obstructive sleep apnoea. Respiratory symptoms developed relatively early in patients with acid maltase deficiency and inflammatory disorders but parallelled the development of limb weakness in limb girdle, myotonic and congenital syndromes. Sixty-six patients received respiratory support for a median of 5 years (1-34 years) using various techniques of negative and positive pressure ventilation. Fourteen patients received short-term support for an episode of respiratory failure before being weaned, 51 required nocturnal domiciliary ventilation and one was dependent on continuous domiciliary support. Tracheostomy was performed in 32 patients and used for domiciliary nocturnal ventilation in 25. Twenty-eight patients have subsequently died.

Southport experience with domiciliary ventilation.

Experience of the Southport Spinal Injuries Centre in reestablishing ventilator dependent patients back in their own homes has confirmed the view that such level of disability need not prevent people from returning to the community and start living again. Convincing the carers in the community and abolishing the traditional boundaries between hospital and community appear to be the fundamental prerequisites. Increased use of advanced assistive technology will enable these disabled people to have more acceptable control of their lives.

The Swedish experience in the organisation of domiciliary ventilation for patients with a high spinal cord injury.

The Swedish experience in the organisation of domiciliary ventilation for patients with a high spinal cord injury

The French organisation of mechanical ventilation at home for neuromuscular diseases

In this paper there is an outline of the organisation and the results of domiciliary ventilation for patients with neuromuscular diseases where there are serious respiratory problems, in relation to special facilities that have been made available in France. The clinical and economic benefits are stated, in particular concerning the situation in the greater Paris area.

Domiciliary nocturnal nasal intermittent positive pressure ventilation in hypercapnic respiratory failure due to chronic obstructive lung disease: effects on sleep and quality of life.

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 pressure ventilators have been used with some success in patients with chronic obstructive lung disease in hospital, but attempts to continue treatment at home have been disappointing. This study evaluates the practicalities of nasal intermittent positive pressure ventilation at home in patients with chronic obstructive lung disease and the effect on sleep and quality of life. Twelve patients with chronic obstructive lung disease and hypercapnic respiratory failure received nasal intermittent positive pressure ventilation at home during sleep. At six months eight were continuing with the ventilation. One patient had died and three had withdrawn because they were unable to sleep with the equipment. Full polysomnography performed during ventilation in patients continuing treatment at six months showed an increase in mean PaO2 of 11% (+2% to +23%) and lower mean transcutaneous carbon dioxide tensions (by -2.7 (-1.3 to -5.1) kPa) overnight compared with spontaneous breathing before the start of nasal intermittent positive pressure ventilation. Total sleep time and sleep efficiency changed during ventilation by +72.5 (+21 to +204) minutes and +5% (-3% to +30%) respectively; sleep architecture and the number of arousals were unchanged. Quality of life did not change but was no worse during ventilation. At one year seven patients were still using the ventilator and PaCO2 and bicarbonate ion concentration during the day had improved further by comparison with the values at six months (change from baseline -1.7 (-2.1 to -0.6) kPa, p less than 0.05, and -6.3 (-11.9 to -4) mmol/l, p less than 0.05). Nasal intermittent positive pressure ventilation can be used effectively at home during sleep in selected patients with chronic obstructive lung disease. Its future place in management can be established only by formal comparison with long term oxygen therapy.

Respiratory involvement in multiple sclerosis.

Respiratory complications occur in advanced multiple sclerosis (MS) but may also complicate acute relapses earlier in the disease. We present 19 patients with MS who developed respiratory complications at a mean of 5.9 (range 1-12) yrs after the onset of neurological symptoms. Fourteen patients developed severe respiratory insufficiency presenting with a combination of reduced forced vital capacity (FVC), hypoxaemia or hypercapnia (12 patients) and respiratory arrest (four patients). Two patients presented with apneustic breathing, one with paroxysmal hyperventilation, one with obstructive sleep apnoea and one with bulbar weakness leading to aspiration pneumonia. Respiratory muscle weakness was a major factor in 14 patients (predominantly diaphragm involvement in six), bulbar weakness in seven patients, impaired voluntary control in three and impaired automatic control in three. Twelve patients received mechanical respiratory support of whom seven have subsequently died. The methods of support used were intermittent positive pressure ventilation (nine patients), iron lung (three), cuirass (two) and rocking bed (one). Six patients were maintained on respiratory support until they died after intervals varying from 24 h to 6 yrs (mean 17.7 mths). Five patients received temporary ventilation for between 6 d and 42 d: of these four remain alive at up to 4 yrs and one died after 16 yrs. One patient remains on domiciliary nasal intermittent positive pressure ventilation (IPPV) after 1 yr.

Domiciliary nocturnal nasal intermittent positive pressure ventilation in COPD: mechanisms underlying changes in arterial blood gas tensions

The improvement in arterial blood gas tensions following assisted ventilation in chronic obstructive pulmonary disease (COPD) has usually been attributed to the relief of incipient or established respiratory muscle fatigue. The contribution of changes in the load placed upon and the drive to the respiratory muscle pump have not been evaluated. We have investigated the contribution of changes in respiratory muscle strength, the ventilatory response to CO2 and ventilatory function to changes in arterial blood gas tensions in eight patients with severe COPD completing six months domiciliary nasal intermittent positive pressure ventilation. Six patients showed a reduction and two an increase in arterial carbon dioxide tension (PaCO2), median (range) for eight patients, -0.9 kPa (-1.5 to +0.4) (p less than 0.05) and seven showed an improvement in arterial oxygen tension (PaO2), +0.7 kPa (-0.4 to +1.7) (p less than 0.05) during daytime spontaneous breathing. The reduction in PaCO2 was not related to increased inspiratory muscle strength but was correlated with a decrease in gas trapping (Spearman rank correlation coefficient (r(S)) 0.85, p less than 0.05) and in the residual volume (r(s) 0.78, p less than 0.05), suggesting reduced small airway obstruction and, therefore, a reduction in load. The change in PaCO2 also correlated with the increase in ventilation at an end-tidal CO2 of 8 kPa during rebreathing (r(s) -0.76, p less than 0.05) suggesting improved chemosensitivity to CO2. Our data do not support the hypothesis that improvements were due to the relief of muscle fatigue. We suggest that the contribution of changes in load and central drive warrant further investigation.

Occult respiratory failure as a cause of neuropsychiatric symptoms in chest wall deformity and neuromuscular disease

Nocturnal hypoventilation occurs in patients with severe kyphoscoliosis (1) and neuromuscular disorders (2,3) and may lead to the development of daytime symptoms and cardio-respiratory failure. The onset is generally insidious and delay in diagnosis common. We report three patients presenting with primarily neuropsychiatric symptoms, in whom an underlying respiratory abnormality was not immediately apparent. All were returned to normal health by domiciliary nocturnal nasal intermittent-positive pressure ventilation (NIPPV).

Assisted ventilation 6. Non-invasive and domiciliary ventilation: positive pressure techniques.

Assisted ventilation 6. Non-invasive and domiciliary ventilation: positive pressure techniques.

Assisted ventilation. 5. Non-invasive and domiciliary ventilation: negative pressure techniques.

Assisted ventilation. 5. Non-invasive and domiciliary ventilation: negative pressure techniques.