Long-term mechanical ventilation equipment for neuromuscular patients: meeting the expectations of patients and prescribers.

BACKGROUND: To maximize the likelihood of successful long-term mechanical ventilation (MV) in patients with neuromuscular diseases, ventilator characteristics and settings must be chosen carefully, taking into account both medical requisites and the patient's preference and comfort. OBJECTIVES: To evaluate patients' knowledge about and comfort with their long-term MV; to compare patients' and prescribers' opinions and expectations regarding long-term MV; and to compare the equipment used by the patients to the prescribers' current MV prescription. METHODS: Neuromuscular patients receiving long-term MV, and home MV prescribers in Belgium and France were asked to respond to a questionnaire survey specifically developed for the study. RESULTS: Completed questionnaires were collected from 209 patients (mean age 35.4 ± 15.9 y, range 3-86 y), ventilated since 11 ± 17 year, and 45 MV prescribers. One hundred sixty-three (78%) patients correctly designated their MV mode as a volume or pressure controlled mode. When an inspiratory trigger was available, 92% of the patients were able to use it, but only 69% were satisfied. Prescribers were more prone than patients to use new technologies such as an emergency-release system for the noninvasive interface (1-10 visual analog scale score 9.2 ± 1.5 vs 6.8 ± 3.3, P < .001), a humidification system (8.6 ± 1.4 vs 7.8 ± 2.6, P = .02), a contactor for providing larger inspiratory volumes (8.4 ± 1.7 vs 6.0 ± 3.0, P = .009), a built-in cough assistance mode (9.2 ± 1.4 vs 5.5 ± 3.5, P < .001), new options to improve speech, or new MV modes such as a volume-targeted pressure control. CONCLUSIONS: The opinions of patients and prescribers differed about the ideal home ventilator. Patients were less prone to use new technologies, mainly because of a lack of information, underlining the need for regular MV update in patients receiving long-term MV.

ALS with respiratory onset: clinical features and effects of non-invasive ventilation on the prognosis.

Respiratory muscle involvement is one of the main prognostic factors in amyotrophic lateral sclerosis (ALS). Acute respiratory failure is sometimes the first manifestation of the disease, although onset can be more insidious. In the present retrospective study, it was proposed to review the clinical features and to assess the effects of non-invasive ventilation (NIV) on the prognosis of patients with respiratory onset, which was taken to be present when the first symptoms of muscular weakness were dyspnoea at exertion, dyspnoea at rest, or orthopnoea. Respiratory onset ALS is uncommon, since it accounts for less than 3% of ALS cases. ALS with respiratory onset has some common clinical features: male predominance, frequent camptocormia or dropped head, frequent widespread fasciculations, limb mobility fairly well preserved and significant weight loss in the early stages. ALS patients with respiratory onset still have a poor prognosis compared with those with bulbar or spinal forms. NIV should be proposed promptly because it improves the symptoms, general state of health and survival time. Efforts should be made to inform general practitioners and chest physicians and remind them that neuromuscular respiratory insufficiency may be attributable to this particular form of ALS.

Enteral and parenteral nutrition in the later stages of ALS: an observational study.

Current practice guidelines state that enteral feeding should be proposed and provided whenever ALS patients show significant dysphagia and/or weight loss. Percutaneous endoscopic gastrostomy (PEG) is usually available at ALS centres, but this method is not indicated in the case of patients with severe respiratory impairment. We undertook a study to assess the feasibility of applying home parenteral nutrition (PN) to ALS patients with respiratory insufficiency, to assess the complications and survival times after the procedure. PN was provided, via an implanted port, to 30 ALS patients with respiratory insufficiency requiring nutritional support. Nutritional and neurological follow-up were carried out. The results were compared with those obtained on a group of 35 patients who underwent PEG, who were subdivided into patients with respiratory insufficiency and those without respiratory insufficiency at the time of the PEG. Post-procedure survival of PEG patients with respiratory insufficiency was similar to those of the PN group. The results of the study suggest that PN is a safe and effective method, which improves the patients' nutritional status and stabilizes their weight in most cases. Home PN could be applied to patients with advanced ALS and poor respiratory function as an alternative to enteral feeding.