Abstract
Since 1980 we have engaged in developing the cuirass respirator (CR) apparatus to improve the management of respiratory failure in patients with progressive muscular dystrophy of terminal stage, and also used it clinically. This apparatus consists of two parts, ventilator system and body jaket. The former was designed to regulate air pressure, the ratio of expiration to inspiration and respiratory rate to meet various conditions of patients, and the latter was made to keep airtightness and comfortability. Next we examined the efficacy and the safety of this apparatus by two types of pilot tests, animal experiments and short-period application to the patients, before its clinical use. The optimal condition was observed under the negative pressure of -10 to -15cm H2O in this apparatus.
Seven cases, in which there is a case of application for longer than 4 years long, have been under good control by CR apparatus of our 20 cases (DMD: 16 cases, LG: 4 cases). We were obliged to perform tracheostomy in 7 cases in the course of CR application (3; died), nine patiennts died of cardio-pulmonary failure or pneumonia. We began to apply the CR apparatus when the level of blood gas Paco2 increased more than that of Pao2 (60 Torr). First it was used for preventing nocturnal dyspnea, and then even during daytime according to the blood gas level of the patients. We mentioned the application and management of our CR apparatus as above.
The DMD patients must wear the CR apparatus for a long period during their lives, so it is necessary for us not only to keep their respiration in good condition but also to elevate their quality of lives. This study suggests that it is useful to use the CR apparatus as first application instead of conventional tracheostomy in the treatment of respiratory failure of PMD patients.
