When a child is first diagnosed with a chronic illness , it is often a shock to the family; and in many cases diagnosis is experienced as traumatic, especially if the caregiver or child fears the child may die or be seriously harmed. High rates of post-traumatic stress symptoms have been reported in parents of children diagnosed with cancer (Kazak et al. 1998) and type 1 diabetes (Cline et al. 2011; Landolt et al. 2002). Less severely, about one in three children develop a diagnosable adjustment disorder at diagnosis (Cameron et al. 2007).

For most children with a chronic illness and their families, the shock dissipates but a sense of chronic stress remains. As we noted in the Introduction, many illnesses require complex and intrusive daily management; others, such as asthma and sickle cell disease, have recurrent (and sometimes unpredictable) symptom flares; and still others (e.g., cystic fibrosis) result in substantial functional limitations and disability.

The recognition that a chronic illness becomes a chronic stressor to which the child and family adapt is at the core of stress and coping models of illness adjustment (e.g., Thompson and Gustafson 1996; Wallander and Varni 1998). Also important is the idea that the condition requires “continual readjustment” and “repeatedly interfere[s] with the adequate performance of ordinary role-related activities” (Wallander and Varni 1998). Adjustment requires the child and family to manage emotional responses, consider social implications, and martial resources both within and outside the family for managing the disease and maintaining (or returning) to “life as normal” as far as possible. It also requires children and families to “change and reprioritize … goals in order to incorporate new goals related to [illness] management” (Schwartz and Drotar 2006).

Children and their families draw on their resources to cope with illness. In Wallander and Varni’s (1998) model, these so-called resilience factors include a person’s competence and skills, family environment, social support, practical resources, and “stress-processing factors such as cognitive appraisal and coping strategies.” At the same time, pre-existing or co-existing risk factors interfere with or complicate children’s and families’ abilities to cope and manage the illness. Important risk factors include disease-related disability, reduced ability to complete activities of daily living, and psychosocial stressors (Wallander and Varni 1998). An important contribution of stress and coping models has been to identify modifiable risk factors that can be targeted for intervention.

More recent models of coping with chronic illness have focused on the dimension of control (Compas et al. 2012). Primary control (or active coping) refers to efforts to change the source of stress or one’s reactions to it, whereas secondary control refers to efforts to accommodate to the stressor. Disengagement or passive coping refers to avoidance or lack of any coping attempts (Rudolph et al. 1995). Not surprisingly, disengagement or avoidance coping has been associated with poorer adherence (e.g., Reid et al. 1994).

In a recent review, Compas et al. (2012) noted that secondary coping has the strongest support in terms of child adjustment to illness, and they suggested that the uncontrollable nature of many illnesses requires adaptation rather than active attempts to control the disease. This finding is consistent with the burnout often seen among children and youth who attempt to maintain tight illness control and sheds some light on why children and youth with better adherence sometimes have worse psychosocial adjustment. It may also shed light on a point made over a decade ago by La Greca and Bearman (2001): “What may appear to be ‘nonadherence’ to a health care professional may actually be the family’s way of adapting the regimen to accommodate the child’s quality of life.” In other words, poor adherence when an illness is especially difficult to control may sometimes be the most effective coping strategy, at least in terms of maximizing immediate quality of life. The fact that adherence and quality of life are often at odds may be the most challenging aspect of maintaining good illness control. As noted by Schwartz and Drotar (2006) in discussing a hypothetical youth with diabetes, “if prioritizing and working towards her health-related goals compromises her ability to pursue and achieve other personally salient goals, then she may feel that her [chronic health condition] is affecting her quality of life and adaptation.”

Managing a chronic illness requires a new set of knowledge and skills to carry out health behaviors correctly and consistently. As noted by many authors (e.g., Adams et al. 2004; Hood et al. 2009), medical regimens have become increasingly complex, and often stretch the abilities of patients and their families. After diagnosis, physicians and other healthcare providers focus on patient and family education, although there is some question of whether enough time is spent for families to truly learn and understand the condition and its treatment for many illnesses (Turner et al. 2009), especially as diagnosis is often a time of such high stress, which can limit parents’ ability to actively engage in learning.

Health literacy has been defined as “the degree to which individuals have the capacity to obtain, process, and understand basic health information and services needed to make appropriate health decisions” (US Department of Health and Human Services 2000). Recent reviews of health literacy in the pediatric domain (Abrams et al. 2009; DeWalt and Hink 2009; Yin et al. 2009) suggest the following main points:

Regarding the last point above, a recent meta-analysis of interventions for pediatric nonadherence reported negligible-to-small effect sizes for education-only interventions (d = 0.16, 95 % CI = 0.10–0.22; Kahana et al. 2008). Education is an important component of interventions for adherence, however. A second meta-analysis (Graves et al. 2010) found that interventions that combined educational with behavioral approaches had more potent effects on health outcomes (d = 0.74, 95 % CI = 0.55–0.94) than either type of approach alone (d = 0.16, 95 % CI = 0.02–0.30). Moreover, Graves et. found that educational approaches resulted in better long-term health outcomes on follow-up. Taken together, these findings support the notion that education is necessary but not sufficient for adherence to medical recommendations (DeWalt and Hink 2009).

It is also important to recognize that knowledge is different from the ability to use that knowledge successfully. For example, caregivers in the National Cooperative Inner-City Asthma Study demonstrated good knowledge of asthma (M = 84 % correct answers on an asthma information quiz), but when presented with hypothetical problem situations most offered at least one solution that was “potentially dangerous or maladaptive” (Wade et al. 1997). Interventions that focus on teaching illness-specific problem-solving skills (e.g., Grey and Berry 2004) are likely to be more effective than interventions focused on increasing knowledge.

Many factors play into families’ understanding of disease and illness. Healthcare providers are used to taking for granted the empirical basis for most of what they do—clinical guidelines and best practice recommendations are based, to the degree possible, on the best available scientific evidence. Many laypeople do not think this way, however. It must be acknowledged that there is a lot of distrust of medicine and “Big Pharma.” For example, a recent Pew poll found that only one quarter of U.S. adults have a lot of confidence that new medicines have been carefully tested before being made available to the public, half had “some” confidence, while the last quarter had little to no confidence (http://​www.​pewforum.​org/​2013/​08/​06/​chapter-4-views-about-todays-medical-treatments-and-advances/​). Many people use alternative therapies despite the lack of an empirical basis. A National Science Foundation survey from 2001 found that 88 % of respondents agreed that “there are some good ways of treating sickness that medical science does not recognize” (Science and Technology: Public Attitudes and Public Understanding; http://​www.​nsf.​gov/​statistics/​seind02/​c7/​c7s5.​htm#c7s5l2a), and all indications are that use of alternative therapies has only increased since that time.

Adjustment and coping attempts and adherence all rely on the child and family’s beliefs about the illness, its controllability, treatment, and their own capabilities. According to Helman (1981) in a classic article :

Faced with an episode of ill-health, patients try to explain what has happened, why it has happened and decide what to do about it. The shaping of the illness and the behavior of the patient—and of those around him—will depend on the answers to six questions:

How the questions are answered, and the behavior that follows, constitutes a ’folk model of illness’.

In other words, patients (and their families) will attempt to come to an understanding of what the illness is and what it means to them.

The Health Belief Model (HBM; e.g., Janz and Becker 1984) posits that people’s adherence will be influenced by their beliefs that the illness poses a true threat to their health, that the treatment is effective and its benefits outweigh its costs, and they are capable of doing what they need to do to manage the illness. The HBM has a substantial amount of empirical support in the adult literature and has been one of the most influential theories of health-related behavior. But to the degree that pediatric adherence results from an interaction between the child and his/her caregivers, the question arises, whose health beliefs should be considered (La Greca and Mackey 2009)? This question is especially important as child and parent health beliefs are not always correlated (Charron-Prochownik et al. 1993).

It should also be kept in mind that most children have multiple caregivers, not all of whom may agree about the meaning of the illness or importance of treatment. For example, we often hear anecdotal reports of multigenerational families in which a grandparent undermines the parent’s attempts to manage a child’s illness by expressing doubt about the need for the prescribed treatment, or a preference for a more traditional alternative medicine approach .

Indeed, few studies have examined both child and parent health beliefs and their relation to adherence within a single study. Bush and Iannotti (1990) adapted the health belief model for children (the Children’s Health Belief Model) and used the model to predict children’s (age 8–14 years) expected medicine use for common (acute) health problems. They first examined child health-belief predictors and then repeated the analysis entering caregiver variables, thus accounting for the effect of caregiver beliefs. Surprisingly, caregiver beliefs accounted only for a small (although statistically significant) amount of additional variance, although it should be noted that the outcome was expected medication use, not actual use. (They could not measure actual use because they used a sample of children without chronic illness requiring regular medication management.) It seems plausible if not likely that parents’ beliefs would have a much stronger effect on whether medicines are actually taken or not.

Studies of youth with type 1 diabetes have generally shown positive effects of youth health-beliefs on adherence (although see Urquhart et al. 2002). Skinner and colleagues have consistently found relations between perceived treatment effectiveness and better diabetes self-care (diet, exercise, blood glucose monitoring, and insulin administration; Skinner and Hampson 1998; Skinner and Hampson 2001; Skinner et al. 2002). Perceived threat of diabetes has also been found to be associated with better adherence (Skinner et al. 2002), but possibly only when the costs of following the diabetes regimen are seen as low (Bond et al. 1992). Interestingly, Bond et al. found that metabolic control was worst when perceived threat and perceived cost were both high, suggesting that perceived threat may be a risk factor for poor illness control when youth struggle with management tasks. Studies of youth with asthma have also generally shown positive effects of health-beliefs in the expected directions (Buston and Wood 2000; Rich et al. 2002; Zebracki and Drotar 2004).

Many of the studies examining health beliefs in children have methodological limitations (Haller et al. 2008), especially regarding differences in measurement of the relevant constructs (Rapoff 2010). A promising measure of youth health beliefs is the Beliefs About Medication Scale (BAMS; Riekert and Drotar 2002), a 59-item scale that assesses a number of important constructs of the HBM: Perceived Threat (severity and susceptibility), Positive Outcome Expectancy (i.e., benefits), Negative Outcome Expectancy (i.e., barriers), and Intent. In the validation study of 133 adolescents with asthma, HIV, or inflammatory bowel disease, the BAMS accounted for 22 % of the variance in self-reported medication adherence. Three subscales were positively correlated with adherence and the fourth approached significance. A shorter version of the scale has also been developed to assess caregiver beliefs (Naar-King et al. 2006) and presumably could be re-adapted for use with children.

Health beliefs , as measured by the constructs of the HBM, may not be good predictors of adherence or illness control for minorities, although few studies have examined this directly. Patino et al (Patino et al. 2005) found no relation between health beliefs and adherence or glycemic control in a sample of youth with a relatively high proportion of minorities (Black and Hispanic youth). However, they did find that perceived susceptibility to diabetes was much higher and perceived severity of the illness was lower compared to the findings reported by Bond et al. (1992), suggesting that this sample saw themselves as more vulnerable but perceived the consequences of diabetes as less bad.

Overall, research findings indicate that both parent and youth health beliefs have an effect on children’s adherence. Studies are needed that examine the concordance between parent and child health-beliefs and their effect on illness management. In line with this, a recent study by Herge et al.(2012) found that higher concurrent youth and parent self-efficacy for diabetes was associated with better adherence . Better understanding of health beliefs may open up new avenues for intervention, although to date interventions that have changed health beliefs have had minimal impact on adherence behavior (Strecher and Rosenstock 1997).

Health beliefs strongly influence the goals people set for themselves (or for their children, or for their patients), and goals in turn drive intentions, or the plans and effort expended in the pursuit of goal attainment (Ajzen 1991, 1996). Intentions are seen as proximal indicators of a person’s readiness to perform a behavior (Ajzen 2005) and have been shown to account for 20–30 % of the variance in health behavior in adults (Gibbons 2008).

Goal-setting is often the first step in developing a plan for behavior change. For example, a physician will set glycemic goals for a child with newly diagnosed diabetes, and an overweight youth will set weight-related goals for himself. More proximal behavioral goals in these examples might be determining the number of glucose checks the first child performs, and setting up a walking schedule and dietary targets for the second child.

However, in line with the theme of this book, goal-setting is complicated by involving multiple actors. Goal discrepancies between the child, parent(s), and healthcare providers are common. Children and their parents often have competing goals—parents tend to be more focused on illness management, whereas children focus on immediate quality of life, such as their social lives, school performance, and extracurricular activities. Health-related goal setting may place pressure on a child to alter her standards for herself in other areas of her life, such as “not having to be the best soccer player; not having to get As on every test” (Schwartz and Drotar 2006). As one can imagine, this sort of reorientation of goals and standards can entail a significant sense of loss for the child. Goal-discrepancies between parents also occur.

Children and families also may have different goals from healthcare providers. In general, healthcare providers see disease management as the primary goal, whereas families will often prioritize goals “to maintain normalcy and enhance well-being” in the family (Rehm and Franck 2000).

Some examples of differing goals between patients, parents, and healthcare providers include:

Competing views on the relative importance of different goals often result in misunderstandings and conflict—between the child and parent, or between the healthcare provider and the child or family (Schwartz and Drotar 2006), even if the provider is not aware of the conflict. As Schwartz and Drotar note, and as we discuss later in this volume, “discrepancies among invested parties will likely be minimized when there is collaboration and agreement about what goals are important and how to achieve them.” Even when agreement is not achieved, simply improving communication among the relevant parties can greatly increase chances for adherence success (DiMatteo 2000).

It is also important to recognize that people often have competing goals themselves, requiring them to prioritize goals, and patients will often prioritize non-health-related goals over health-related goals. Thus “understanding and respecting patients’ non-health-related goals” (Schwartz and Drotar 2006) is necessary for providers who wish to best help their patients with adherence. If providers are unaware of these competing motivations, they will be unable to discuss pros and cons with their patients. Using a motivational interviewing-style approach (see Chap. 4) may prove especially helpful here, as providers can point out the youth’s own competing goals and highlight the discrepancy between them, which has been shown to help motivate behavior change. On the other hand, simply telling patients what they should do is very likely to backfire, as we discuss later in this chapter.

Attaining health-related and adherence goals requires self-control—i.e., inhibiting an impulse to engage in some desirable behavior (such as eating a restricted food) in the interest of a goal whose benefits may be far in the future. The human animal is simply not wired to do this. Evolution predisposes us to “eat now because there may be famine tomorrow.” Greater uncertainty of receiving the long-term benefit contributes to this tendency to favor immediate reward (Mischel and Ayduk 2004).

Nonetheless, people are often able to delay gratification and work toward goals that are far in the future. Self-regulation refers to a person’s attempts to control impulses and adapt immediate behavior in the interest of attaining a long-term goal or goals (de Ridder and de Wit 2006). Models of self-regulation focus on two linked processes: setting goals, and then striving to achieve them. Successful self-regulation depends on multiple factors including having goals that are personally meaningful, a belief in one’s ability to attain the goal (termed self-efficacy; Bandura 1997), and the skills necessary to problem-solve difficulties, overcome barriers, and cope with frustration and other emotional responses.

One may ask whether it is self-regulation when it is the parent who is largely in control of health management. We believe the answer is yes, as in these instances the child has to conform—to self-regulate—in response to the parent’s wishes or demands. When a child is unable to do this, significant behavior problems result, often requiring intervention by a behavioral specialist. Still, as we will see, the issue of control is critically important to self-regulation in illness management, and we will return to this issue later in this section.