Long-term Ventilation for Patients With Duchenne Muscular Dystrophy

Gibson, Barbara E.

doi:10.1378/chest.119.3.940

Cited by 72 publications

(18 citation statements)

References 15 publications

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“…Importantly, the healthcare workers consistently underestimated the quality of life of DMD patients. Unfortunately, information on the impact of NIV in DMD does not yet seem to have filtered through to all centres who manage such patients, some of whom do not discuss the option of mechanical ventilation with patients because of a misperception that it results in a poor quality of life [28].…”

Section: Duchenne Muscular Dystrophymentioning

confidence: 99%

Home ventilation: Table 1—

Simonds¹

2003

Eur Respir J

125

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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

125

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“…By using the DMD-FNS during clinic visits, health care professionals may be in a better position to understand each family's unique priority needs and strengths, instead of having to second guess what they might be or be influenced by clinician's personal bias [Gibson, 2001;Kinali et al, 2007]. The revised DMD-FNS that was used in this study incorporates topics specific to the care of individuals with DMD, and therefore may serve as an ideal prompt for parents to discuss these issues during their child's regular clinic visits.…”

Section: Discussionmentioning

confidence: 99%

Psychosocial Support Needs of Families of Boys with Duchenne Muscular Dystrophy

Mah¹,

Biggar²

2012

Neuromuscular Disorders

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“…This dataset did not include information about quality of life, so increases in age at death do not necessarily equate with improvements in quality of life. 29 Interpretation was complicated by the coding of MDs as a group in the ICD system, and by the lack of age-, race-, and sex-specific prevalence and age at death estimates for MDs. The sample sizes for female and black decedents limited the power of some race-or sex-specific analyses, especially when the need to dichotomize year of death could have masked small gradual changes.…”

Section: Figurementioning

confidence: 99%