Introduction: Diagnostic-therapeutics strategies adopted towards Neuromuscular disease (NMD) and amyothrophic lateral sclerosis (ALS) by 247 Respiratory Units (RU) are unknown. Materials and methods: An investigation by web to collect geographical distribution, patients' number, clinical and diagnostic aspects, follow-up, prognosis, rehabilitation management of the acute episodes and end of life decisions were provided. Results: The percentage of answer was 30,76%. The geographical distribution of RU was homogeneous on the national territory. 50% and the 28% of the RU resulted of high experience, having respectively followed up over than 20 patients with NMD or 40 ALS. Catarrhal encumbrance is the main symptom in the ALS. Vital capacity, arterial blood gases and night-time oxymetry were used as diagnostic parameters in both the diseases. Worsening of the blood gases, necessity to perform NMV or tracheostomy were recognized as prognostic factors. Home MV and programs devoted to the management of the cough were used by almost all centres. In the 18% and 10% of the cases for NMD and ALS respectively tracheostomy has not been counselled. ALS patients receive more frequently programs of care structured during the serious acute episodes. Over the 50% of the pneumologists interviewed declare not to use a structured approach. Respiratory Units use hospital offices and hospital admission for long time follow-up. The frequency of the controls is individualized. Only in the 30% of the cases a protocol directed to interview and knowledge the anticipated and shared directives for end of life decisions is used. A multidisciplinary approach is referred in the 80% of the cases mainly involving neurologists; sectional effectiveness, poor communication and experience in the field are factors limiting the staff interaction. Conclusions: Italian pulmonologists seem able to answer to care requests for MNM. Treatment of acute episode, cough assistance, palliative programs and different approaches according to specific illnesses need further development.
|Translated title of the contribution||Survey on neurological and pneumological integrate care in neuromuscular diseases and in amyotrophic lateral sclerosis|
|Number of pages||7|
|Journal||Rassegna di Patologia dell'Apparato Respiratorio|
|Publication status||Published - Dec 2011|
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Pulmonary and Respiratory Medicine