Although adherence to the treatment regimen is largely responsible for the extended life expectancy of children with cystic fibrosis, nonadherence remains a significant problem for patients. Factors such as age, gender, intellect, complexity of treatment regimen, and patient-provider communication are importantly related to adherence. Additionally, psychiatric comorbidity, poor self-esteem, poor family support, and child and family stress factors all negatively impact treatment adherence. Rates of adherence to treatment for cystic fibrosis vary greatly depending on which components of the regimen are measured and which method is used to assess adherence. In one study, 50% of adolescents with cystic fibrosis indicated that they were doing less than 50% of their prescribed therapies, and 30% indicated that they were doing none (DiGirol-amo et al. 1997). In a multimethod assessment of adherence in children with cystic fibrosis, the overall mean rate of adherence for treatment, based on refill history, daily phone diary data, and electronic monitoring, was demonstrated to be below 50% (Modi et al. 2006).

Rates of adherence for chest physiotherapy for children with cystic fibrosis are estimated to be 40%-47% (Passero et al. 1981; Quittner et al. 2000). Even when hospitalized and in a structured setting, 35% of teens showed significant nonadher-ence to ordered chest physiotherapy regimens (Cza-jkowski and Koocher 1986). Rates of adherence are lowest for dietary recommendations and have been estimated to be 16%-20% (Anthony et al. 1999; Passero et al. 1981; Stark et al. 1995). Recombinant human dornase alfa is a mucolytic agent administered through a nebulizer, which takes about 15 minutes. In children and adolescents, one study found that the overall mean adherence rate to dor-nase alfa was 66% (Zindani et al. 2006). Another study found general adherence rates to range from 57% to 90%, with adherence to pancreatic enzymes as low as 27%-43% (Modi et al. 2006).

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