Noninvasive ventilation in cystic fibrosis patients with acute or chronic respiratory failure

Abstract

The experience of using noninvasive ventilation (NIV) in 113 adult cystic fibrosis (CF) patients with chronic respiratory failure, during episodes of acute deterioration in respiratory function is reported.The patients aged 15-44 yrs were divided into three groups. Group A consisted of 65 patients (median forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) 0.7/1.4 L) who were on a lung transplant waiting list. Group B consisted of 25 patients (median FEV1/FVC 0.7/1.4 L) who were being evaluated for lung transplantation. Group C consisted of 23 patients (median FEV1/FVC 0.6/1.2 L) who were not being considered for lung transplantation.The mean duration of NIV support for groups A, B and C was 61 (range: 1-600) days, 53 (1-279) days and 45 (0.5-379) days respectively. Twenty-three patients in group A subsequently received lung transplantation and 12 of these patients had a median survival of 39 months postsurgery. Thirty-nine patients died and three awaited transplantation. Five patients in group B received a transplant four of whom survived; thirteen patients died and seven awaited transplantation. Twenty patients in group C died.Noninvasive ventilation improved hypoxia but failed to correct hypercapnia in these cystic fibrosis patients. Noninvasive ventilation is useful in the treatment of acute episodes of respiratory failure in cystic fibrosis patients with end-stage lung disease who have been accepted, or are being evaluated, for lung transplantation. For these patients, there is a possibility of prolonging life if they are successfully treated for their acute episode of respiratory failure until transplantation. In this group, treatment is not merely prolonging the process of dying.

El trasplante pulmonar en la fibrosis quística y la influencia de la circulación extracorpórea

“…Los autores concluyen que la VMNI es un tratamiento útil en los episodios de insuficiencia respiratoria. 257 Meachery y cols en su serie de 176 pacientes FQ que recibieron un TP, no encuentran una mayor mortalidad en los pacientes portadores de VMNI (p = 0,89). 70 Al igual que las publicaciones previas, nuestra serie no encuentra un aumento de mortalidad en los pacientes que requirieron VMNI previa al TP (p = 1,00).…”

Indications, patient selection and timing of referral for lung transplantation

“…Status in hospital or in intensive care units (ICUs) and invasive mechanical ventilation are associated with less favourable 1-and 5-yr outcomes [5], although this may apply to a lesser extent to patients with CF [17]. Outcome data from patients on noninvasive ventilation are scant but this appears to be a preferable bridge to transplant where available [18,19].…”

Long-Term Noninvasive Ventilation in Patients with Cystic Fibrosis

“…Indeed, a beneficial effect of long-term NPPV on gas exchange and lung function, survival or quality of life has not been demonstrated [10, 11]. Some studies did not observe a significant decrease in hypercapnia [6, 12]. Long-term randomized controlled trials on NPPV in patients with CF are difficult to implement because of the variability and unpredictability of the clinical course in patients with severe lung disease and also because of ethical issues.…”