Previous studies have documented a ...

Previous studies have documented a 75 to 80 percent mortality rate in infants (< 12 months) with cystic fibrosis who current with RF/MV. We investigated the circulating outcome of these patients by the agency of comparing the outcome of five infants with cystic fibrosis and RF/MV between the years 1980 and 1986 with that of age-matched superintend subjects with cystic fibrosis and no respiratory failure. fresh Shwachman scores and the number of hospitalizations during the studious mood period were used to assess issue All study cases were alive single in kind to six years after the episode of RF/MV Their mean Shwachman score (83 [range: 70 to 95]) did not differ from the novel mean Shwachman score (78 [range: 50 to 95]) of the direct group. Number of hospitalizations did not differ between disposes We conclude that the issue of infants with cystic fibrosis whose initial presentation is RF/MV is not as grave as formerly thought. If indicated, infants with cystic fibrosis and respiratory failure should be ventilated.

(Chest 1989; 96:136-38)

Cystic fibrosis is the principally common genetric disease that shortens the lifespan of affected caucasians. Patients with cystic fibrosis and relentless pulmonary disease die younger than those with les strict involvement and infants with RF/MV are reported to have an on the same level worse long-term prognosis.[1-3] In common series, five of six infants with cystic fibrosis and RF/MV died within six month of ventilatory support.[2] We lately reviewed our experience with infants who had the disease and RF/MV to determine if their result differed from infants who had the disease and did not require mechanical ventilation for respiratory failure in their first year of life.

Methods

We reviewed charts of all cystic fibrosis patients cared for at Children's Hospital of Wisconsin who had an episode of RF/MV in infancy (<1 year). Respiratory failure was defined according to a respiratory rate greater than 65 breaths by minute, an arterial pH value of <725; [PaO.sub.2], <60 (while upon at least 0.40 [FIo.sub.2]); or [PaCO.sub.2], >60 mm Hg; and an abnormal chest radiograph as interpreted at a pediatric radiologist. Infants intubated for surgical actions were excluded from the close attention group. Children with cystic fibrosis and RF/MV were ventilated to normalize their arterial line gases ([PaCO.sub.2] [35 to 45 mm Hg]; [PaO.sub.2] [80 to 100 mm Hg]; pH [>735]) All ventilated infants were treated with similar supportive care which included chest physiotherapy, antibiotics, enteral and parenteral nutritional support, and bronchodilators when indicated. Steroids were not used.

We recorded the following data for each patient: age, physical examination, arterial house gas values, chest radiograph findings, tracheal aspirate improvements and days ventilated.

issue of ventilated patients was compared with that of cystic fibrosis infants who were not at all mechanically ventilated for respiratory failure during their first year of life. Three repress subjects born within 18 month of their respective case were selecteded at random for each ventilated infant. Shwachman scores were determined from data recorded in the chart for ventilated and curb infants during their most fresh clinic visit record. Shwachman scores were determined for bridles at a similar age as their matched cases. The Schwachman scoring classification is an objective measurement of the clinical status of cystic fibrosis patients.[4] Patients with scores of 90 to 100 are classified as "excellent" 80 to 89 as "good" 70 to 79 as "fair," and 50 to 69 thinks a "poor" clinical status. The number of hospitalizations for complications of cystic fibrosis that occurr during the cogitation period also were recorded.

Scores were determined independently from two investigators who were unaware of the patients' dispose status. Paired t proof analysis was performed to document inter-observer difference. Wilcoxon rank any amount test was used to determine if there was a significant difference between assemblage Shwachman scores. Significant was defined as p<005

Results

From 1980 to 1986 three females and sum of two units males, ages one day to five month unfolded RF/MV. It was the first hospitalization for four abroad of five infants, and cystic fibrosis was diagnosed in all of them during the hospitalization. individual patient had been admitted and discharged common week earlier with a diagnosis of bronchiolitis. He was readmitted with similar symptoms. In individual infant, cystic fibrosis was diagnosed prior to initiation of mechanical ventilation (one month antique [Table]). The rest were diagnosed while being ventilated. Prior to presentation none of the infants had a history of intermittent pneumonias or chronic illness.

Clinical and arterial house gas values at admission [TABULAR DATA OMITTED] for each patient are summarized in Table 1 single in kind infant presented at birth with asphyxia, meconium aspiration and bilateral pneumothoraces requiring chest tube placement. Three of five readyed with a clinical picture consistent with bronchiolitis which l to their respiratory failure. All three had a two-to three-week history of cough and wheezing. The final infant lay opened a severe nosocomial pneumonia that required mechanical ventilation. Mean admission arterial line gas levels for the consideration infants were: pH, 7.12; [PaCO.sub.2], 70 mm Hg; and [PaO.sub.2], 50 mm Hg All five patients had abnormal chest radiographs with atelectasis, lobar or perihilar infiltrates, and/or hyperinflation usually involving the pair lung fields. Tracheal aspiration improvements grew enteric Gram-negative rods,[4] Pseudomonas sp[3] Staphylococcus aureus,[2] or Haemophilus influenzae.[1] Each patient received appropriate parenteral antibiotics. The mean time for ventilatory support was seven days (range, 3 to 15 days). All patients were weaned, extubated and discharged. brace to six years later, all patients in the investigation group were still alive.