Use of Bilevel Positive Airway Pressure (BIPAP) in End-Stage Patients with Cystic Fibrosis Awaiting Lung Transplantation

Abstract

Nine consecutive end-stage patients with cystic fibrosis (CF) awaiting lung transplantation were admitted to the pediatric intensive care unit (PICU) in respiratory decompensation. They all received noninvasive bilevel positive airway pressure (BIPAP) support and were evaluated to determine whether or not it improved their oxygenation and provided them with long-term respiratory stability. BIPAP was applied to all patients after a brief period of assessment of their respiratory status. Inspiratory and expiratory positive airway pressures (IPAP, EPAP) were initially set at 8 and 4 cm H2O respectively. IPAP was increased by increments of 2 cm H2O and EPAP was increased by 1 cm H2O increments until respiratory comfort was achieved and substantiated by noninvasive monitoring. Patients were observed in the PICU for 48 to 72 hours and then discharged to home with instructions to apply BIPAP during night sleep and whenever subjectively required. Regular follow-up visits were scheduled through the hospital-based CF clinic. The patients' final IPAP and EPAP settings ranged from 14 to 18 cm H2O and 4 to 8 cm H2O, respectively. All nine patients showed a marked improvement in their respiratory status with nocturnal use of BIPAP at the time of discharge from the PICU. Their oxygen requirement dropped from a mean of 4.6 +/- 1.1 L/min to 2.3 +/- 1.5 L/min (P < 0.05). Their mean respiratory rate decreased from 34 +/- 4 to 28 +/- 5 breaths per minute (P < 0.05). The oxygen saturation of hemoglobin measured by pulse oximetry, significantly increased from a mean of 80% +/- 15% to 91% +/- 5% (P < 0.05). The patients have been followed up for a period of 2 to 43 months and have all tolerated the use of home nocturnal BIPAP without any reported discomfort. Six patients underwent successful lung transplantation after having utilized nocturnal BIPAP for 2, 6, 14, 15, 26, and 43 months, respectively. Three patients have utilized home BIPAP support for 2, 3, and 19 months, respectively, and continue to await lung transplantation. An acute development of refractory respiratory failure resulted in the demise of the remaining three patients after having utilized BIPAP for 3, 6, and 10 months, respectively. The authors conclude that BIPAP therapy improves the respiratory status of decompensating end-stage CF patients. It is well tolerated for long-term home use and provides an extended period of respiratory comfort and stability for CF patients awaiting lung transplantation.

Effects of lung transplantation on inpatient end of life care in cystic fibrosis

“…Patients who have survived previous illnesses involving the use of invasive or noninvasive mechanical ventilation may, in particular, be more willing to undergo such treatments. During the time period reviewed, non-invasive ventilation has been described as a therapy and possible bridging mechanism to lung transplant in patients with CF and acute respiratory failure [18,19], but this strategy was not utilized in our population as all patients in this category were too ill for this therapy to be effective. In our analysis, no obvious change in level of care over the period of time studied was apparent.…”

Noninvasive ventilation in cystic fibrosis

“…Bilevel positive pressure ventilation (PPV) has been used with success in nine adult patients with end-stage lung disease [23]. All patients survived the intensive care unit (ICU) and six underwent successful lung transplantation.…”

“…In this condition, upper airway obstruction and/or work of breathing induced by intrinsic PEEP are prevented by expiratory positive airway pressure, and thus PS can be triggered easily by the patient. This ventilatory mode has been used in patients with CF [23,40].…”

Lung transplantation for cystic fibrosis: Current concepts and one center's experience

“…1), and shortterm outcomes for patients with various indications for lung transplantation have been shown to be similar between mechanically ventilated and non-mechanically ventilated patients undergoing lung transplant at other centers [21][22][23]. Non-invasive positive pressure ventilation has been used as a "bridge" to transplant in patients with severe respiratory insufficiency as a means to try and avoid invasive ventilation and improve oxygenation both acutely and on a chronic basis [24][25][26][27], and we have used non-invasive ventilation (BIPAP) to support many of our patients until they could undergo transplantation.…”