| Description |
The objective of this body of research was to (1) survey current self-management skills and treatment knowledge in patients with phenylketonuria (PKU) and (2) evaluate self-efficacy, patient activation, dietary adherence, and blood phenylalanine (Phe) levels in individuals with PKU before and after a 6-month motivational interviewing (MI) intervention. Patients with PKU aged 7-30 years (n=43) completed one of three age-specific self-management questionnaires during standard metabolic clinic appointments and were invited to participate. Separately, patients with PKU aged 7-35 years (n=31) participated in a 6-month intervention including phone-based MI, goal setting, and a monthly summary. Participants completed stage of change (SOC), self-efficacy, patient activation, and food frequency questionnaires online and obtained monthly blood Phe samples. Demographic data were collected from the electronic medical record. We considered p<0.01 as significant when analyzing aim 1 and p<0.05 as significant when analyzing aim 2. Participation in self-management behaviors and treatment knowledge did not increase with age for most measures (p>0.01 for all) with only approximately half of adults meeting clinical recommendations. Higher self-management skills and treatment knowledge were not associated with lower blood Phe levels (R2=0.249, p=0.025), which may reflect the small sample size. Most participants were in the action/maintenance SOC for PKU treatment behaviors during the 6-month MI intervention. Self-efficacy significantly increased from baseline (7.4±1.9) to month 6 (8.6±1.3) among adolescent and adult participants (p=0.002). There was no increase in patient activation (p=0.10) or dietary adherence, though most (n=28/31) reported adherence to medical formula at baseline. Pre-intervention blood Phe slope for all participants (β=0.71) was not significantly different from the intervention slope (β=0.26, p=0.13). Higher baseline SOC (β=-61.6, p=0.005) and increasing self-efficacy (β=-64.5, p<0.001) were associated with a decreased blood Phe slope. Self-management skills were lower than clinically recommended and did not consistently increase with age. We demonstrated a significant increase in self-efficacy with MI and, in turn, higher self-efficacy was associated with a reduction in blood Phe levels. Our results suggest strategies to support self-management and increase motivation for behavior change are necessary. Phone-based MI is feasible with a PKU population has potential to increase confidence to engage in self-management behaviors. |
