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OUTCOME OF MECHANICALLY VENTILATED INFANTS WITH CYSTIC FIBROSIS (CP) AND RESPIRATORY FAILURE

Previous studies have documented a 75-85% mortality rate in infants (≤12 mos.) with CF who initially presented with respiratory failure requiring mechanical ventilation (RFMV). Five infants with CF and RFMV as their initial presentation were cared for between the years 1980-86 at Children's Hospital of Wisconsin. Clinical presentation of each case (age, physical exam, ABGs, CXR findings, tracheal cultures, days ventilated) was similar to previously reported studies of infants with CF and RFMV. The purpose of this study was to compare the outcome of these infants with age matched CF infants who never had RFMV.Three age matched controls with CF and born between the years 1980-86 were selected at random for each case. Most recent Schwachman's scores (SS) were obtained independently by two investigators. Interobserver differences in SS were not significant. All study cases were alive from 1-6 years after the episode of RFMV. Their most recent mean SS was 82.0 (range 79-95). In comparison, the recent mean SS of the controls was 78.0 (range 50-95). There was no difference between group SS (t=0.54, NS).We conclude that the outcome of infants with CF whose initial presentation is RFMV is not as grave as once thought, if indicated, infants with CF and respiratory failure should be ventilated.