Part 3 CF Predictions

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Within each age group, the median value for the FE[V.sub.1] percent

 predicted in each age group at each site was calculated, and the

sites were

 ranked from lowest to highest according to this median value. The

sites then

 were divided into quartiles so that those with the highest values

(upper

 quartile) could be compared with those having the lowest values

(lower

 quartile). For comparisons of practices and interventions between the

quartiles,

 which are markedly affected by disease severity, patients within each

quartile

 were pooled and stratified by disease severity based on FE[V.sub.1]

(ie, < 40%

 of predicted, 40 to 69% of predicted, 70 to 99% of predicted, and

[greater than

 or equal to] 100% of predicted). This allowed the sites in the upper

and lower

 quartiles to be compared based on their practices within groups of

patients with

 similar lung health.

    Statistical Methods

    For each possible pair of the three age groups, the

consistency of site

 rankings between the two age groups was assessed using Spearman rank

correlation

 coefficients. Statistical comparison of upper quartile sites with

lower quartile

 sites were done using Mantel-Haenszel tests for dichotomous variables

and

 Wilcoxon rank sum tests for counts of events or durations of therapy,

with

 stratification by FE[V.sub.1] (ie, < 40% of predicted, 40 to 69% of

predicted,

 70 to 99% of predicted, and [greater than or equal to] 100% of

predicted) so

 that practices were compared within patients of a similar severity

level

 Stratified descriptive statistics included either proportions for

dichotomous

 variables, means for counts of events or medians for duration of

therapy The

 quartiles also were compared globally on the overall mean FE[V.sub.1]

percent

 predicted and weight for age percentile using t tests.

    RESULTS

    Of the 194 participating sites in the ESCF, 132 with 8,125

patients met the

 eligibility criteria for this analysis. For the 6 to 12-year-old age

group, 112

 sites with 3,361 patients (mean, 30 patients per site; SD, 19

patients per site)

 were eligible. For the adolescent age group (13 to 17 years of age),

84 sites

 with 1,734 patients (mean, 21 patients per site; SD, 11 patients per

site) were

 eligible. For the adult group ([greater than or equal to] 18 years of

age), 103

 sites with 3,030 patients (mean, 29 patients per site; SD, 24

patients per site)

 were eligible. The observation that a consistent trend of monitoring

and

 intervention was observed across the four quartiles (ie, that the

middle

 quartiles fell between the upper and lower extremes) allowed the

subsequent

 analysis to be restricted to the upper and lower quartiles.

    For sites that qualified with at least two age groups, the

rankings of sites

 tended to be consistent across the age groups with Spearman rank

correlation

 coefficients of 0.30 for ages 6 to 12 years and 13 to 17 years (p =

0.007; 80

 patients), 0.29 for ages 6 to 12 years and [greater than or equal to]

18 years

 (p 0.007; 84 patients), and 0.17 for ages 13 to 17 years and [greater

than or

 equal to] 18 years (p = 0.17; 69 patients). Considering each pair of

age groups,

 very few sites (six or fewer) were in the upper quartile in one age

group and

 the lower quartile in the other age group.

    The observed differences between upper and lower quartile

sites in disease

 severity, as characterized by FE[V.sub.1] percent predicted, were

substantial,

 ranging from 15 to 23% of age points (Table 1).

    Comparing patients within each of the severity groups, the

monitoring of

 events (ie, number of visits, spirometry testing, and cultures)

Occurred more

 frequently at upper quartile sites (Table 2). These trends were

highly

 statistically significant in each age group. The greatest differences

in the

 number of cultures were seen in the youngest age group, which had 40

to 77% more

 cultures performed at upper quartile sites than at lower quartile

sites.

    Certain microorganisms were reported more frequently at

upper quartile sites

 (Table 3). Compared with the lower quartile sites, there was a higher

incidence

 of patients with at least one culture positive for Pseudomonas

aeruginosa and

 for at least one strain of this organism that was reported as

" multiply

 resistant. " Also, compared with the lower quartile sites, there were

more

 patients with at least one culture positive for Burkholderia cepacia,

and for

 Stenotrophomonas maltophilia at the upper quartile sites.

    Some therapies were used more frequently at the upper

quartile sites (Table

 4). Upper quartile sites administered oral corticosteroids and

inhaled cromolyn

 or nedocromil more frequently than did lower quartile sites

consistently across

 all age and disease-severity groups. Very large differences were seen

in the use

 of inhaled cromolyn and nedocromil, ranging from 10 to 42% (p <

0.001). Oral

 nonquinolone antibiotics also were used more frequently in upper

quartile sites.

 Prophylactic inhaled antibiotics (ie, aminoglycoside and quinolones)

were used

 more frequently in young pediatric patients. Among other routine

therapies,

 substantial and significant differences were observed only in adults.

In this

 age group, upper quartile sites administered more inhaled

bronchodilators, more

 inhaled corticosteroids, and more dornase alfa than lower quartile

sites (Table

 4). No differences were seen in the use of pancreatic enzymes and

airway

 clearance techniques, which were used by > 90% of patients in almost

all age and

 disease-severity groups. No attempts were made to quantify airway

clearance

 techniques.

    For patients who were < 18 years of age, upper quartile

sites tended to

 report more frequent treatments of exacerbations with IV antibiotics

(Table 5).

 These differences were proportionately largest in the relatively

healthy

 patients, particularly in the age group of 13 to 17 years. Upper

quartile sites

 also tended to treat patients for longer periods, regardless of

disease

 severity. This trend was particularly strong in adult patients, for

whom the

 increased duration was highly significant (p < 0.001). Upper quartile

sites also

 reported more frequent treatments of exacerbations with inhaled

antibiotics in

 patients < 18 years, with differences once again proportionately

largest in the

 relatively healthy patients. In contrast, in patients < 18 years of

age the

 reported use of oral quinolones for the treatment of exacerbations

tended to be

 greater in lower quartile sites, except in the youngest, sickest

patients (6 to

 12 years of age, < 40% predicted FE[V.sub.1]). In adults, there was

no

 significant difference between upper and lower quartile sites for the

use of

 inhaled antibiotics or oral quinolones. Because of less accurate

documentation

 of stop dates for inhaled antibiotics and oral quinolones, the

duration of

 treatment could not be reliably assessed.

Becki

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