Long-term Nasal Intermittent Positive Pressure Ventilation in Advanced Duchenne's Muscular Dystrophy

Vianello, Andrea; Bevilacqua, Michela; Salvador, Vittorino; Cardaioli, Claudio; Vincenti, E

doi:10.1378/chest.105.2.445

Cited by 177 publications

(80 citation statements)

References 10 publications

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“…However, the long-term effects of NIV have been explored by comparing clinical course and pulmonary function in five hypercapnic patients who received NIV and a control group of five patients who did not receive ventilatory support [24]. Over a 2-yr period, all of the subjects receiving NIV survived, whereas four of five of the control subjects died (mean survival 9.7 months).…”

Section: Duchenne Muscular Dystrophymentioning

confidence: 99%

Home ventilation: Table 1—

Simonds¹

2003

Eur Respir J

127

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Home ventilation is a growth area. Rapid expansion during the 1990s was stimulated by the development of noninvasive ventilation (NIV)viaa mask and the recognition that an increased number of patient groups can benefit. Although patients receiving NIV in the home outnumber those receiving invasive ventilationviatracheostomy, there is substantial variation in practice between European countries.Evidence that individuals who develop ventilatory failure as a consequence of chest wall disease or stable neuromuscular disease such as old poliomyelitis benefit from nocturnal NIV is overwhelming. Patients with progressive neuromuscular disease such as Duchenne muscular dystrophy and amyotrophic lateral sclerosis can also derive prolongation of life, palliation of symptoms and an improvement in quality of life.Home ventilation in chronic obstructive pulmonary disease (COPD) patients remains controversial. Multicentric randomised controlled trials of long-term oxygen therapy (LTOT)versusNIV plus LTOT in COPD have produced mixed results, although certain subgroups,e.g.those with recurrent infective exacerbations requiring short-term NIV, patients aged >65 yrs, and those with uncontrolled hypercapnia on LTOT or symptomatic nocturnal hypoventilation, may benefit.At the other end of the age spectrum, children as young as a few months can be successfully treated with noninvasive ventilation. Most work on paediatric home ventilation centres on children with congenital neuromuscular disease. Pressure preset bilevel ventilators are now the dominant form of ventilator in adults and children. Discharge planning is vital for the home ventilator patient and a sensible risk management strategy should be in place.

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Section: Duchenne Muscular Dystrophymentioning

confidence: 99%

Home ventilation: Table 1—

Simonds¹

2003

Eur Respir J

127

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“…The mean survival following the development of diurnal hypercapnia in DMD is 9.7 months if ventilatory support is not provided [47]. A Consensus recommendation [13] is that NPPV should be initiated in symptomatic DMD patients with a daytime Pa,CO 2 w6.0 kPa.…”

Section: Progressive Neuromuscular Diseasementioning

confidence: 99%

Noninvasive ventilation for chest wall and neuromuscular disorders

Shneerson¹,

Simonds²

2002

Eur Respir J

164

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Neuromuscular and chest wall disorders are individually uncommon but together form an important group of conditions that can lead to chronic ventilatory failure. This is best recognised in scoliosis, kyphosis, following a thoracoplasty, in muscular dystrophies, such as Duchenne muscular dystrophy (DMD), and myotonic dystrophy, after poliomyelitis and with motor neurone disease (amyotrophic lateral sclerosis).If bulbar function is impaired, tracheostomy ventilation may be required, but in other situations, noninvasive ventilation is preferable. Positive pressure techniques using nasal and face masks are usually the first choice, but negative pressure ventilation is an alternative.There are no randomised-controlled trials regarding the indications for initiating noninvasive ventilation, but this is usually provided if there are symptoms due to nocturnal hypoventilation or right heart failure in the presence of a raised carbon dioxide tension in arterial blood (Pa,CO2) either at night or, more usually, in the daytime as well. There is no evidence that “prophylactic” ventilatory support is of benefit if this is provided before ventilatory failure has appeared. Careful selection of patients is required, especially in the presence of progressive neuromuscular disorders such as DMD and motor neurone disease.There are no randomised-controlled trials concerning the outcome of noninvasive ventilation in these conditions, but studies have shown an improved quality of life, physical activity and haemodynamics, normalisation of blood gases and slight improvement in other physiological measures, such as the vital capacity and maximal mouth pressures. Survival in chest wall disorders is ∼90% at 1 yr and 80% at 5 yrs, and similar figures have been obtained in nonprogressive neuromuscular conditions. If, however, the underlying disorder is deteriorating, particularly if it involves the bulbar muscles, it may limit survival despite the provision of adequate noninvasive ventilatory support.

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“…The effectiveness of nocturnal ventilatory support in reversing daytime respiratory failure and improving daytime function has been demonstrated by many groups [1][2][3][4][5]. The improvements both in physical [6] and psychological [7] functioning have enabled many to return to an active lifestyle and full employment [6,8,9].…”

mentioning

confidence: 99%

“…In initial reports, ventilatory support was provided either invasively via a tracheostomy [8], or noninvasively using one of the negative pressure devices [6,9]. More recently, it has been found that home positive pressure ventilation could be achieved effectively and simply using noninvasive nasal mask ventilation [1][2][3][4][5]. Although there is now an extensive literature demonstrating the improved daytime function when patients receive long-term nocturnal ventilation, there are few data on how such treatment affects spontaneous breathing in sleep in these patients.…”

mentioning

confidence: 99%

Effects of long-term nocturnal nasal ventilation on spontaneous breathing during sleep in neuromuscular and chest wall disorders

Piper¹,

Ce²

1996

European Respiratory Journal

110

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Nocturnal nasal intermittent positive pressure ventilation (NIPPV) is an effective means of normalizing awake blood gases in patients with respiratory insufficiency due to neuromuscular or chest wall dysfunction. However, little attention has been paid to the effects of long-term ventilation on spontaneous breathing during sleep in such patients. The purpose of this study was to determine whether spontaneous breathing during sleep improved after long-term nasal ventilation.Fourteen patients with documented nocturnal respiratory failure, who had been treated with nocturnal NIPPV for at least 6 months, were reviewed with an all night polysomnograph on a night without ventilatory support. The severity of nocturnal desaturation both in non-rapid eye movement (NREM) and rapid eye movement (REM) sleep without nocturnal ventilation was compared to desaturation measured during the initial diagnostic study.Spontaneous daytime blood gas values (mean±SD) were significantly improved at follow-up compared to values obtained prior to nasal ventilation: arterial oxygen tension (Pa,O 2 ): 7.5±1.2 to 10.2±1.3 kPa (p<0.005); arterial carbon dioxide tension (Pa,CO 2 ) 8.2±1.6 to 6.4±0.7 kPa (p<0.001). Significant improvements in inspiratory muscle strength were also observed with maximal inspiratory pressure (PI,max) increasing from a baseline value of 41±18 to 65±26% predicted measured prior to the night of ventilation withdrawal (p<0.003). Spontaneous breathing during sleep after long-term treatment was markedly improved although still abnormal. During NREM sleep without ventilatory support, oxygen desaturation was significantly less severe compared to the initial study (arterial oxygen saturation (Sa,O 2 ) 88±4 vs 78±8%; p<0.001). Minimum Sa,O 2 during REM sleep similarly improved from a mean value of 49±14% during the diagnostic night to 73±10% at review follow-up (p<0.001). In 12 patients, transcutaneous carbon dioxide was measured continuously during sleep on both occasions and demonstrated significantly less CO 2 retention during follow-up compared to control studies both in NREM (p<0.003) and REM sleep states (p<0.004).Long-term nocturnal ventilation produces improved respiratory drive both asleep and awake and improved arousal responses to abnormal blood gases.

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Long-term Nasal Intermittent Positive Pressure Ventilation in Advanced Duchenne's Muscular Dystrophy

Cited by 177 publications

References 10 publications

Home ventilation: Table 1—

Home ventilation: Table 1—

Noninvasive ventilation for chest wall and neuromuscular disorders

Effects of long-term nocturnal nasal ventilation on spontaneous breathing during sleep in neuromuscular and chest wall disorders

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