1/1/2004

“Jason” is a 15 year old male with a non-diagnosable and atypical neuromuscular weakness syndrome. Tests for Duchenne and Becker type muscular dystrophy proved negative. Jason is of normal intelligence but has had significant weakness resulting in full time use of a wheelchair and several recent hospitalizations for respiratory illnesses. He uses oxygen continuously. Most recently the need for ventilatory assistance resulted in the prescription of a Bipap Synchrony with a nasal interface. Despite multiple attempts to customize the settings and the interface Jason has not tolerated the Synchrony. On his most recent clinic visit he appeared more fatigued. An arterial blood gas showed his pH to be 7.33 mmHg and his pCO2 to be 69 mmHg. These values were a clear indication of the need for nocturnal ventilatory support and quite possibly continuous ventilatory support. A tracheostomy was discussed. Jason and his mother decided that having a trach was not an option and Terry Respiratory in Houston was consulted.

After a careful evaluation it was determined that Jason was not tolerating the Bipap and current interface. He complained that the “air” was not sufficient and exhaling proved difficult if not impossible at times. He would ask that the supplemental oxygen be increased so that he could “feel” more air. Jason appeared fatigued and an increased work of breathing noticed. At this time he was using supplemental O2 at 3 lpm via nasal cannula. An LTV 950 was selected and a Breeze sleepgear system with nasal pillows was implemented. The pressure (with assist control) mode was set using a starting pressure of 14 cmH2O, a breath rate of 16, and an inspiratory time of 0.8 secs. Leak compensation was set to “on” and we introduced the ventilator to Jason. Almost immediately we received a “thumbs up” from Jason along with a big smile. In about two hours we finally settled at a pressure of 20 cmH2H. Good bilateral chest excursion was obvious and overall comfort was noted. The PEEP was removed.

Over a two week period, supplemental oxygen was discontinued during ventilation and the daily length of need for ventilation was reduced to 12 hours. Obvious lung volume recruitment was evidenced by chest radiograph. The latest pCO2 was within normal limits.

Jason was spared complications of a tracheostomy for now. The noninvasive means of ventilating him via the LTV 950 in the pressure mode proved successful. The continuous flow of air and removal of the peep was essential to his tolerance. Careful selection of the proper mask interface was also a key factor. The LTV 950 has been in use for over 90 days now with excellent compliance and no complaints. The pulmonary physician agrees to continue this modality as long as possible. According to Jason that will be forever!