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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