Self-management skills in adolescents with chronic rheumatic disease: A cross-sectional survey

Children with special health care needs (CSHCN), which include children with chronic rheumatologic conditions, typically enter the health care system as passive participants, with parents assuming responsibility for the majority of their health care-related tasks. However, as pediatric patients age into adolescence and young adulthood, there is an expected shift in the burden of responsibility from parent to young adult. Young adults must learn to complete such tasks as scheduling appointments, communicating with providers, securing insurance coverage, and taking medications as directed. Prior studies have shown that CSHCN are often unprepared to make this transition [1, 2], leaving them at risk for ongoing dependence on their families and delayed achievement of developmental milestones [3]. While the American Academy of Pediatrics has recommended transition planning for all CSHCN since the 1980s, only 41% of CSHCN receive transition-planning services [4]. Among young people with juvenile idiopathic arthritis (JIA), studies have shown high rates of unsuccessful transfer to adult care [5]. Many studies have assessed the challenges that CSHCN face during transition, but evidence-based recommendations to improve transitional care for CSHCN are lacking, and even less is known about the optimal management of rheumatologic disease-specific issues related to transitioning.

Self-management has been defined as an "individual's ability to manage the symptoms, treatment, physical and psychosocial consequences and lifestyle changes inherent in living with a chronic condition" [6]. The development of self-management skills is a critical part of the transition to adulthood for adolescents with a diagnosis of lifelong chronic illness, and failure to develop these skills can prevent successful transition to adult care. Difficulties with self-management in late adolescence and young adulthood may be exacerbated by the transition to new providers and changes in insurance. In most of the United States, public health plans such as Medicaid, SCHIP and the federal Title V program facilitate access to comprehensive health care for CSHCN through age 18 or 21, at which point patients typically "age out" of coverage [7]. Following loss of insurance patients often experience gaps in care, with lapses in doctor visits and medication adherence placing them at risk for increased disease activity and adverse health outcomes [8]. Among patients with rheumatic diseases such as systemic lupus erythematosus and juvenile idiopathic arthritis, ongoing disease activity increases the risk of permanent damage and functional limitation [9]. The problem of poor outcomes in the transition period has been demonstrated in multiple populations [1, 10].

Little is known about the self-management skills of adolescents with chronic rheumatic disease, though a recent study showed improved disease knowledge and decreased pain among subjects receiving an internet-based intervention designed to improve self-management in JIA [11]. It is unclear which health care tasks adolescents are completing independently and which tasks they feel unable to complete. While prevalence and causes of medication non-adherence are also not well understood, non-adherence has been associated with poorer health perceptions, self-esteem, mental health, family cohesion, and social functioning [12]. It is unclear whether health-related self-management skills are improving with increasing age, as patients prepare to transfer to adult rheumatology care. Therefore, this cross-sectional study examined self-reported adherence and health-care-related behaviors, as a first step toward assessing health-related self-management among adolescents with rheumatic disease prior to transfer to adult-centered care.

For the secondary outcome measure - medication adherence - 54% reported perfect adherence, 40% reported 1-2 missed doses per week, and 6% reported missing >3 doses per week. Reasons for non-adherence included forgetfulness (54%), running out of medication (10%), and intentionally skipping doses (10%).

Mean score on the Exercise of Self-Care Agency Scale was 73.2 (SD 16.9), as compared to 89.5 (SD 19.4) for a published report of high school students (P<0.001) [17]. There was a trend towards lower levels of self-care agency among subjects with higher self-reported adherence (P = 0.07). According to the Brief IPQ, most subjects believe medications can effectively treat their illness (7.2) and that they have moderate understanding of their illness (6.7). There were no significant associations between illness perception or other predictors and self-reported adherence.

Our findings provide insight into self-management proficiency and medication adherence among adolescents with chronic rheumatic disease. While this cohort showed good progress towards achieving independence in disease-management tasks in several domains, deficits were evident in other areas. Furthermore, older patients did not demonstrate an increase in proficiency in several areas as compared to their younger peers. Lack of improvement in self-management independence with increasing age suggests that adolescents are not mastering certain skills critical to self-management of chronic illness. Tasks that are most challenging for young patients involve interaction with adult-oriented systems and organizational skills, such as calling a medical office to schedule appointments, keeping a calendar, and maintaining a personal medical file.

While interventions to improve self-management have been shown to improve health outcomes and quality of life in both adults and children [18], these programs are not widely implemented. Data from several studies suggests that patient education interventions for CSHCN can be effective in improving health outcomes and decreasing loss to follow-up in the transition period [19]. Like many institutions, UCSF lacks a formal transition program for adolescents with chronic illness. Patients are expected to master disease self-management skills through parental coaching at home and physician encouragement as a part of routine care. However, our data suggest that this approach is not sufficient to achieve independence in all self-management skills in these older adolescents who are approaching transition.

Approximately half of our patients report imperfect adherence to medications, which is similar to other reported adherence rates among adolescents with chronic illness. The most common reason provided for missing medications was forgetfulness, a finding that has been reported previously [20]. Few subjects admit to intentionally skipping doses of medication, though subjects may be hesitant to disclose intentional non-adherence. Understanding the cause of medication non-adherence is important because while medication reminders may be effective for patients who forget to take their medications, they are unlikely to improve adherence if patients actively choose not to take their medications. Automated medication reminders have been studied extensively among adults with chronic disease, with some evidence of success [21].

With regard to predictors of self-reported medication adherence, accurately reporting the medication names and dosing regimens did not correlate with adherence. Interestingly, knowledge of medication purpose fell at two extremes, with the majority of patients demonstrating either very good understanding of the indications for their medications, or very poor understanding. It may be that some subjects have not been sufficiently engaged in their medical care or have not been willing to engage, and therefore lack understanding of the purposes of their medications. Other patients with pediatric-onset disease may have not be aware of the purpose of medications that they have taken since early childhood, when information about medications was directed towards the parent rather than the patient.

This study has several important limitations. Our results are derived from English-speaking patients at a single U.S. center and thus may not be generalizable to all practices. However, since UCSF is a Title V referral site, this study does draw from a diverse patient population representing Northern California. While subjects were recruited consecutively, our population may be biased towards more adherent patients, since those who did not appear for their appointments did not have the opportunity to participate in the study. Data on adherence and self-care practices were obtained via self-report, which is simple and cost-effective but may be subject to recall bias and social desirability bias [22]. In addition, the adherence measures used were developed by the authors and not tested prior to use. Our sample size was also small; however, rheumatic diseases in children are relatively rare. Finally, quantitative analysis may not be able to fully and accurately describe complex behavior around adherence.

Transition to adulthood is an exciting and a challenging time, when adolescents and young adults must complete many educational, social, and vocational tasks. For adolescents with a diagnosis of lifelong chronic illness, mastering disease self-management is a critical part of this transition. This study suggests that the current standard of care is inadequate to prepare adolescents to successfully manage their disease in adulthood. Further work is needed to assess deficits in self-care skills in a broader population of adolescents with rheumatic disease to ensure generalizability of the current findings, which can then be applied to the development of targeted self-care improvement programs. Interventions to improve self-management should be integrated into standardized transition readiness procedures that address both the self-management and health care systems aspects of transition to adult care, and ultimately lead to improved health outcomes.