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Biomedical subjects

John R Bach

Publications and source records attributed to John R Bach.

24 records · Page 2Linked to original sources
PubMed2002

Spinal muscular atrophy type 1: management and outcomes.

Our objectives were to describe survival, hospitalization, speech, and outcomes related to respirator needs for spinal muscular atrophy type 1 (SMA1) patients, using noninvasive or tracheostomy ventilation. From 65 SMA patients referred to our clinic since 1996, we chose 56 SMA1 patients who developed respiratory failure before age 2 years. Patients either had tracheostomy tubes (group A), or used noninvasive ventilation and assisted coughing; a previously reported extubation protocol (group B) was used as needed. Sixteen patients underwent tracheostomy at 10.8 +/- 5.0 months of age, 33 were in group B, and 7 others died without life-support interventions. Compared to group B, group A patients had fewer hospitalizations until age 3 years, but more after age 5, and 15 of 16 lost all spontaneous breathing tolerance posttracheostomy and could not speak. One group A patient died at 16 months of age, and the others were 73.8 +/- 57 months of age (the oldest was 19 years old). Two group B patients died at 6 and 13 months, respectively, whereas the other 31 were 41.8 +/- 26.0 months (and up to 8.3 years) old. Three of 31 in group B required high-span positive inspiratory pressure plus positive end-expiratory pressure (PIP + PEEP) continuously with minimal tolerance for breathing on their own, and 4 could not communicate verbally.In conclusion, SMA type 1 children can survive beyond 2 years of age when offered tracheostomy or noninvasive respiratory support. The latter is associated with fewer hospitalizations after age 5 years, freedom from daytime ventilator use, and the ability to speak.

Adolescent↗
PubMed2002

Continuous noninvasive ventilation for patients with neuromuscular disease and spinal cord injury.

Patients with a variety of neuromuscular diseases including quadraplegia due to high spinal cord lesions can be managed with full-time noninvasive ventilation instead of intermittent positive pressure ventilation (IPPV) via a tracheostomy. This approach is not suitable for patients with severe bulbar involvement. To be successful with full-time noninvasive IPPV, the ventilator user must realize three goals. First, respiratory system compliance should be optimized and maintained by frequent full inflations delivered by stacking breaths from a volume-cycled ventilator or by insufflating air at adequate pressures using a mechanical insufflator-exsufflator. Second, normal levels of alveolar ventilation are sustained using a variety of noninvasive approaches, including nasal, oronasal, or mouthpiece interfaces. Third, patients must be taught to use techniques and devices to enhance cough, particularly the mechanical insufflator-exsufflator. When these goals are met, noninvasive NPPV can offer patients greater comfort, simpler and more convenient ventilator use, and reduced rates of infections, complications, and hospitalizations as compared with tracheostomy IPPV.

Journal Article↗
PubMed2002

Duchenne muscular dystrophy: prolongation of life by noninvasive ventilation and mechanically assisted coughing.

OBJECTIVE: To quantitate prolongation of survival for patients with Duchenne muscular dystrophy with the use of noninvasive intermittent positive-pressure ventilation (IPPV) with and without access to a protocol involving mechanically assisted coughing. DESIGN: In this retrospective review of all patients with Duchenne muscular dystrophy visiting a neuromuscular disease clinic, patients were trained to use mouth piece and nasal IPPV and mechanically assisted coughing to maintain oxyhemoglobin saturation >94% (protocol). Survival was considered prolonged when noninvasive IPPV was required full time. RESULTS: Ninety-one of 125 patients used noninvasive IPPV part time for 1.9 +/- 1.3 yr, and 51 went on to require it full time for 6.3 +/- 4.6 yr. Of the 31 noninvasive IPPV users who died without access to the protocol, 20 died from respiratory causes and seven died from cardiac causes. None of the 34 full-time noninvasive IPPV users with access to the protocol underwent tracheotomy or died from respiratory complications during a period of 5.4 +/- 4.0 yr, whereas three died from heart failure. Five patients with no breathing tolerance were extubated or decannulated to continuous noninvasive IPPV. CONCLUSIONS: Noninvasive respiratory aids can prolong survival and permit extubation or decannulation of patients with Duchenne muscular dystrophy with no breathing tolerance.

Adolescent↗
PubMed2002

Mechanical insufflation-exsufflation: pressure, volume, and flow relationships and the adequacy of the manufacturer's guidelines.

OBJECTIVE: Pulmonary complications of neuromuscular disease can be averted by increasing peak cough flows with the use of a forced exsufflation device. The purpose of this study was to examine the pressure, volume, and flow relationships for a range of settings generated by this device, and compare them with clinically efficacious values and the manufacturer's guidelines. METHODS: The In-exsufflator was connected to a standard lung model. The resulting forced deflation volumes, flows, and pressures were averaged over 10 cycles at each setting. RESULTS: The set insufflation pressures significantly correlated with the generated insufflation pressures and volumes and the exsufflation volumes and flows. Increasing set insufflation time significantly increased generated insufflation pressures, flows, and volumes and exsufflation volumes. Increasing set exsufflation time did not significantly increase generated exsufflation flows. At set pressures of 40 to -40 cm H2O, insufflation time of 3 sec, and exsufflation time of 2 sec, the exsufflation flow was 4.09 l/sec. A plateau insufflation volume of 3.8 l was reached after 4.9 sec of insufflation. CONCLUSIONS: In-exsufflator performance was very consistent. Its clinical effectiveness can be explained by its generation of exsufflation flows >2.7 l/sec. Increasing insufflation times more than exsufflation times is more important for optimal function. Current manufacturer use guidelines may not yield optimal exsufflation flows.

Cough↗
PubMed2002

Traumatic tetraplegia: noninvasive respiratory management in the acute setting.

A 15-yr-old patient with high-level spinal cord injury developed ventilatory failure 24 hr after hospital admission and required continuous ventilatory support. Although he lost all ventilator-free breathing tolerance, he was managed by receiving noninvasive intermittent positive-pressure ventilation rather than intermittent positive-pressure ventilation via an endotracheal intubation. Cooperative, uncomplicated, acutely injured patients with spinal cord injury who develop ventilatory failure are candidates to use noninvasive intermittent positive-pressure ventilation to avoid intubation.

Acute Disease↗