Reduction in respiratory exacerbation rate in patients with severe bilateral cerebral palsy following daily PEP-mask therapy: a retrospective study.

BACKGROUND: Respiratory complications caused by the inability to protect the upper airways and ineffective cough represent a major cause of morbidity and mortality in patients with cerebral palsy (CP). Even though the application of positive end-expiratory pressure (PEP) through a face mask has gained large popularity as a technique to prevent bronchial mucous encumbrance, its long-term effects on clinical course and respiratory function in individuals with CP have not been investigated. AIM: The aim of this study is to investigate whether regular application of PEP through a face mask can improve clinical status and respiratory function in patients with severe CP. DESIGN: Observational, retrospective cohort study. SETTING: The outpatient rehabilitation unit of the IRCCS E. Medea Rehabilitation Hospital in Conegliano, Italy. POPULATION: CP outpatients admitted to the unit between January 1st, 2006 and December 31st, 2018. METHODS: All the medical records of the enrolled patients were collected and reviewed. All patients underwent multidisciplinary respiratory evaluation at T0 (immediately before the beginning of PEP-use) and T1 (12 months after). The evaluation assessed respiratory infections history (number of exacerbations per year), blood gas analysis, measurement of airway resistance through the interrupter technique. RESULTS: Twenty-one patients affected with CP (mean age 9.19+/-5.56 years, range 3-23 years, 8 females) were included. All patients had more than 3 infections per year (mean 4.81+/-1.17) in the year prior to treatment (T0). At T1 mean number of infections was 1.57+/-0.81); 17 patients (80%) reported less than three infections; two patients (10%) reported zero infections, two patients (10%) reported three infections. Blood gas analysis and airway resistance values did not show a significant difference at T0 and T1. CONCLUSIONS: Daily PEP-mask therapy reduces frequency of respiratory exacerbations in patients with severe bilateral CP. CLINICAL REHABILITATION IMPACT: PEP-mask is a valuable rehabilitative tool in severe CP patients with frequent respiratory exacerbations.