Symptoms, clinical and physiological findings motivating home mechanical ventilation in patients with neuromuscular diseases

Title

Symptoms, clinical and physiological findings motivating home mechanical ventilation in patients with neuromuscular diseases

Creator

Laub M; Berg S; Midgren B

Publisher

Journal of Rehabilitation Medicine

Date

2006

Subject

PedPal Lit

Description

Objective: To clarify the relationship between symptoms, clinical signs and physiological abnormalities that were motivating the initiation of home mechanical ventilation in patients suffering from neuromuscular diseases.Methods: From The Swedish Home Mechanical Ventilation Register we identified 352 patients with neuromuscular diseases and we looked at circumstances (acute vs elective) and clinical motives for starting ventilatory support.Results: Home mechanical ventilation was commenced electively in 268 patients (76%) and among these daytime sleepiness was the most common motive, being reported in 56% of the patients. In the 24 children with spinal muscular atrophy, however, 96% started ventilation electively and cough insufficiency was the most common motive. The patients were moderately hypercapnic (PaCO2: 7.0 kPa, SD 1.3). None of the clinical motives were related to the PaCO2 level. Average PaO2 was above 8 kPa in all groups, but lowest in the patients with post-polio and dystrophia myotonica. Mean vital capacity was close to 40% of predicted, but significantly lower in the Duchenne patients (26% of predicted).Conclusion: Daytime sleepiness was the most common clinical symptom motivating home mechanical ventilation in this group of patients with chronic hypercapnic respiratory insufficiency secondary to neuro/myopathies. Respiratory function testing is therefore suggested to be included in the diagnostic work up of daytime sleepiness in these patients.
2006

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Type

Journal Article

Citation List Month

Backlog

Citation

Laub M; Berg S; Midgren B, “Symptoms, clinical and physiological findings motivating home mechanical ventilation in patients with neuromuscular diseases,” Pediatric Palliative Care Library, accessed February 23, 2024, https://pedpalascnetlibrary.omeka.net/items/show/13594.