BARRIERS TO ADHERENCE
Current research indicates that pediatric patients have better rates of adherence than do adolescent and adult patients.
5, 11 According to the 2003 Haemophilia A Practice Patterns Survey, adherence is highest among infants and children and lowest among adolescents and young adults (Table 1).11
Low rates of adherence among the adolescent and young adult cohort have been examined by other investigators as well. Hacker and colleagues conducted a telephone survey on prophylaxis adherence among 38 participating subjects (or their parents) with hemophilia A or B.4 Issues covered in the survey included decision to initiate prophylaxis; adherence self-assessment; challenges, barriers, and facilitators of prophylaxis; and perceived value of therapy. Investigators' criteria for self-rated adherence were based on percentage of prescribed infusions administered, with excellent and good adherence requiring 76% to 100% and 51% to 75% of prescribed infusions, respectively. Based on these criteria, the study team found that only a little more than half the patients aged 1 to 18 years had self-reported rates of adherence described as excellent.
Lack of Knowledge
In 1999, reporting on the perceptions of transplant clinicians regarding patient compliance, Hathaway and colleagues noted that poor knowledge was a determinant of noncompliance.12 Several years later, Lindvall et al noted a similar association in the hemophilia population.13 He and his team conducted a prospective multicenter study to determine adherence to prophylactic treatment among 108 hemophilia patients aged 13 to 25 years. Investigators found that an overwhelming majority of patients possessed a high degree of self-knowledge about disease type and severity (96% and 99%, respectively), with a correspondingly high percentage of patients (68%) treating bleeds immediately. However, it was also found that 41% of the study population did not follow primary prophylaxis as prescribed. Investigators positively correlated adherence with a high degree of knowledge about hemophilia.
A follow-up study by Lindvall and investigators in an older patient cohort (>25 years) showed a similar correlation between self-knowledge and adherence.2 Among 413 survey respondents, 96% were aware of hemophilia type, and 93% were aware of disease severity. Correspondingly, only 15 patients interrupted treatment for less than 1 month, and 25 patients on prophylaxis for a median of 16 years decided on their own to stop completely.
Adolescence and Transitioning
The transition to adulthood can be especially challenging for adolescents with disabilities.14 This is often the time when hemophilia patients move toward greater independence and begin to self-infuse, assuming responsibility for much of their care.10 A lack of adolescent-focused education can foster nonadherence to treatment at this time, with a shift in focus from parent (the object of much of the early education, beginning at time of diagnosis) to child. Providers need to educate patients in a developmentally appropriate fashion through all stages of the life cycle and especially through adolescence, bearing in mind the issues confronted by adolescents and the potential impact of these issues on adherence.14 During adolescence, personal identities are developed and relationships outside the family are built.14 There is a tendency to move away from authority figures, including parents, and focus on self.15 Although adolescents begin to engage in abstract reasoning and problem solving, they are still oriented in the present and give little thought to the long-term consequences of their actions.15
The Lindvall studies point to the importance of education as a promoter of adherence. In Lindvall's investigations, surveyed patients believed that HTCs provided the best education/information about hemophilia, leading the study team to conclude that given the value placed on education, HTC staff should provide patients with an opportunity to understand the nature of the disease, risks of complications, benefits of treatment, and importance of self-care.2, 13 Participants viewed the HTC nurse, in particular, as essential to their education about the disease. As such, nurses should meet with patients to discuss hemophilia type, severity, benefits of regular treatment, breakthrough bleeds, physical activities, and work and/or school issues.2