The E ffects of N oninvasive V entilation in P atients with C ystic F ibrosis
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The Effects of Noninvasive Ventilation in Patients with Cystic Fibrosis
Senay RE, Uyan ZS, Oktem S,
Karadag B, Ersu R, Karakoc F, Dagli E
Marmara University School of Medicine,
Department of Pediatrics, Istanbul, Turkey
• Electrolyte transport dysfunction exists in the lungs, gastrointestinal system, hepatobiliary system, genitourinary system
• The most important prognostic factor is the involvement of lung pathology, it causes for 90% of the mortality
• Infection, inflammation and sequestration leads to progressive airway ostruction and bronchiectasis and causes for permanent destruction.
Pediatr Pulmonol Suppl 1996;13:245
Cystic Fibrosis (CF)
Noninvasive Ventilation(NIV)
• Alveolar ventilation without the use of endotracheal tube or invasive techniques
• BiPAP (Bilevel Positive Airway Pressure) is the most common type used
Am Rev Respir Dis 1993;147:1050-1055
Use of NIV in patients with CF
• NIV can be used as a complementary airway clearance techniques especially in patients with difficulty of extracting sputum
• Patients with mild-heavy general status benefit more
from NIV+oxygen than oxygen alone when used during sleep at night
Cochrane Database of Systematic Reviews 2007, Issue 4
• However there is still no clear guidelines for determining the use of NIV
NIV- Oxygenation in patients with CF
• Increases alveolar ventilation - During awake period, - During sleep period, - During acute exacerbations maintains gas exchange
• Increases respiratory muscle performance
• Increases exercise tolerance
Eur Respir Mon 2006; 35: 127-138
Objective
• We aimed to determine the effects of NIV in acute and chronic respiratory dysfunction in patients with CF
Methods
• Use of NIV was explored in 180 patients with CF followed in our department
• Parameters of 15 patients with use of NIV was studied
• Demographic data, clinical and laboratory parameters before and after the use of NIV was analyzed
Statistical analysis
• SPSS 11.0 statistical package program was used for the analysis of demographic data, clinical and laboratory parameters before and after the use of NIV
• Independent-T test was used to compare the measurements before and after the application of NIV
• p<0.05 was accepted as statistically significant
Demographic Data of Patientsn=15
Current age (year) 10 ± 5
Age at diagnosis (year) 2 ± 3
Age at the start of our follow up period (year)
5 ± 5
Age when NIV was started (year) 8 ± 4
Mean follow up period (year) 6 ± 5
Gender M / F 11 / 4
BMI 16 ± 4
Clinical parametersPatients who needed MV before NİV (%)
27
Patients who were aplied NIV with nasal way (%)
93
Patients with Pseudomonas (%) 87
Patients with PHT (%) 21
Patients with bronchiechtasis (%) 85
Patients requiring oxygen with NIV (%) 80
Invılvement of GIS pathology (%) 53
Patients with ABPA (%) 6
Endication for NIV
34%
33%
33%
Hypoxemia +respiratoryinsufficiency
Atelectasis
Hypoxemia+CO2 retention
Application of NIV
Mean rate of hospitalization before NIV 3 ± 0,7
Mean duration of NIV(year) 3 ± 2,0
Mean number of exacerbations before NIV
8 ± 3,8
Mean rate of hospitalization after NIV 2 ± 1,2
Laboratory Parameters Before and After NIV
(n=15) Before NIV After NIV p value
Hospitalization rate
3 ± 0,7 2 ± 1,2 >0.05
FEV1 37,1 ± 16,4 31,3 ± 4,9 >0.05
FEF (25-75) 35,6 ± 25 15,9 ± 12,7 >0.05
SaO2 86,7 ± 4,3 94,2 ± 2,8 <0,001
pH 7,36 ± 0,04 7,41 ± 0,03 0,007
CO2 53,1 ± 12,4 46,4 ± 4,05 0,047
HCO3 29,4 ± 3,5 28,6 ± 3,05 >0.05
Last State of Patients
46%
7%
47% Discharged withoutNIV
Discharged with NIV
Exitus
Conclusion
• NIV improves the blood gas parameters and decreases the hospitalization rate with acute exacerbations in CF with chronic respiratory dysfunction
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