Neuromuscular disorders encompass a wide category of illnesses with a significant burden on the patient, family, and society. Telehome monitoring presents an alternative for closer follow-up, by ensuring timely transmission of data and prompting medical support before deterioration occurs. We used telehome monitoring devices for amyotrophic lateral sclerosis patients with respiratory failure in a clinic. This instrument directly acts as a digital recorder of parameters received from an external instrument, such as the bi-level ventilator, and contains an internal modem for direct connection to the Internet, thereby allowing bidirectional flexibility in collecting data and parameter changes whenever needed. The use and acceptance of the system was investigated with patients and all therapists involved. The instrument was found to be user-friendly and effective by the researchers, being practical for both patients and therapists. Therefore, it can be integrated into a routine home care application based on a large respiratory rehabilitation center. The device was extensively tested, and in an ongoing randomized controlled study it is being investigated whether this management strategy is able to hasten the ventilator adaptation process and to diminish the overall utilization of health services, with concurrent economic viability. We conclude that home telemonitoring represents a positive contribution to the management of chronic patients and raised awareness of it should be considered in a near future. This portable telemonitoring device provides an opportune approach to better understand and recognize a ventilatory pattern through long-term ventilation monitoring in the home environment.