Perinatal

Attachment, the reciprocal bonding relationship between the infant and the parent that evolves based on the mutual connection of their dyad, is a primary task of this developmental stage. The parent’s and the infant’s mental representations and behaviors are involved in the attachment process. The parent-child relationship is a critical context for the developing infant, and the quality of attachment predicts important child psychosocial factors such as emotional and behavioral regulation, social skills, and the ability to cope with stress. Parental distress can have negative implications for attachment, particularly when coupled with parental depression and anxiety.

The attachment period often coincides with the time of CF diagnosis. The diagnostic process is emotionally challenging. Even before a definitive CF diagnosis, parents of infants with positive CF screens during NBS report high levels of depressive and anxious symptoms, frequently accompanied by hypervigilence such that parents misinterpret common newborn behaviors as evidence of CF. Negative emotions such as worry, sadness, and guilt caused by the hereditary component of CF are common. Worse coping with a positive screen has been found when parents were not knowledgeable about the NBS process, had little knowledge about CF, and were experiencing more general adjustment issues related to the newborn period (significant sleep deprivation, adjusting to role of new parent). In addition, parents’ distress was reported to be amplified when the medical team communicated the results of the screen in an impersonal fashion and before the infant was old enough to undergo a sweat test (thus extending the waiting period). Once a CF diagnosis is confirmed, parents typically continue to experience fluctuating states of psychological distress and normalcy as they take on the role of medical parent, and may be particularly vulnerable to depressive symptoms when their infants are still only a few months old. Glasscoe and colleagues studied the mental health of parents of infants with CF and reported that mothers and fathers of infants less than 9 months old had higher relative risk of scoring at or more than the clinical cut-off for mild depression (on the Beck Depression Inventory) compared with parents of age-matched healthy controls (relative risk 2.6 and 2.26, for mothers and fathers, respectively). No increased risk was found in parents of older infants.

Despite this risk of heightened distress in parents during the initial attachment period, there do not seem to be higher rates of maladaptive attachments between infants with CF and their parents, again highlighting the resiliency of families with CF. However, when maladaptive attachment does occur, it has been found to have a negative effect on the health status of infants with CF. Infants with CF who have problematic attachment styles have been found to have significantly poorer nutritional status and lower body mass index (BMI, calculated as weight in kilograms divided by the square of height in meters). Apart from the positive findings related to general attachment patterns, other research has found differences between families with CF and healthy controls in specific parent and child behaviors. For example, one study using direct observation to explore differences in interpersonal behaviors of children with CF between the ages of 12 and 24 months and their parents versus healthy dyads found parents of children with CF showed more controlling, serious, and less encouraging behaviors, whereas their children displayed more whining and less responsivity to parent behaviors. Thus, even in the earliest stages of development, the toll of CF may begin to influence family interactions.

Early childhood: preschool

During the preschool period, developmental issues related to expanding language skills, cognitive development in areas such as understanding causality and assessment of ability to control environment, and emotional/behavioral regulation development may be of particular relevance to the experience of CF. Developing language skills helps the child process and express their experiences so that parents and health care providers can have a better understanding of the child’s cognitive and emotional events. Although much research has tied children’s causal attribution to Piagetian stages, with young children attributing illness to magical or superstitious factors, recent research suggests that preschool children may have a more sophisticated understanding of illness which also incorporates biologic factors. In the one study that looked directly at causal attributions in a CF sample, more than half of the 4- to 6-year-old children with CF knew that they had been born with the illness and only 1 of the 17 preschool children studied stated they were being punished with their CF. However, few knew what the various treatments were for. The preschool child is also still developing accuracy in understanding which events can or cannot be controlled, which has significant implications for the young child with CF who may be in a myriad of stressful environmental situations beyond their control such as medical procedures and treatments. The young child with CF may exhibit extreme behavioral reactions to attempt to escape these procedures, and may develop heightened aversion for future medical events.

The interface of behavioral self-regulation and CF-related tasks is particularly noteworthy in this age range, when preschool children are already contending with increasing awareness of behavioral limits, whereas parents are developing strategies to enhance compliance. Even when children have mild disease and low levels of symptoms, treatment burden is significant: daily nutrition goals, enzyme supplementation, and ACT are recommended to preserve growth and lung health. The increased opportunities for parent/child conflict may alter parenting strategies such that parents may become either more authoritative or harsh (particularly for health-related behaviors deemed essential for survival) or more permissive as a result of parenting resources stretched thin by the chronic illness, a choose-your-battles approach, or overprotection. These additive behavioral demands associated with CF treatment can challenge the emotional resources of children and parents and can have negative implications for child and parent mental health, general family functioning, and health-related outcomes. There is little research exploring general psychosocial functioning in young children with CF. However, Ward and colleagues recently published the results of a study comparing parent-reported behavioral functioning of preschool children with CF with normative data. Although increases in child internalizing or externalizing mental health issues were not found, nearly half of the children with CF were found to have moderate to large sleep and/or eating problems, and 40% of the children had poor compliance with ACT. Thus, although not appearing to have significant psychological impairment, these preschool children with CF did struggle with health-related processes and behaviors.

Problematic mealtime behaviors are a particularly salient health-related concern for children with CF because they are at high risk for poor growth as a result of chronic lung infection and dietary fat malabsorption. Nutritional status, in turn, is related to lung functioning, health complications, and morbidity in CF. Although nutritional guidelines for children with CF recommend eating between 110% and 200% of the DRI, studies of infants, toddlers, and preschool children report typical consumption of only 100% of DRI, with fewer than one-quarter of the children achieving the minimum recommendation for children with CF. As might be expected given the sense of urgency parents feel and the higher demands placed on the children, mealtimes with preschool children with CF have been shown to be more problematic than in healthy families. Children with CF show a higher frequency of behaviors that interfere with eating such as crying and whining, delaying meals by talking, spitting out food, and leaving the table. At the same time, parents of children with CF engage in behaviors such as increased coercion, commands, physical prompts, and actual feeding of preschool children compared with a healthy sample. Parents often cite mealtime as a particular concern, and report thinking that they have little control over their children’s eating patterns.

Although CF-related eating concerns persist throughout development, assessment and intervention during early childhood may change the trajectory of maladaptive behaviors and interactions. In general, behavioral problems in children in this age range are best treated through behavior modification. A recent randomized clinical trial of behavioral modification involving toddlers and preschool children with CF found an increase in average daily caloric intake of approximately 850 kcal, corresponding to 120% of DRI. This finding was significantly better than a treatment-as-usual control condition in which the children’s caloric intake declined during the same period. The gains found in the behavioral treatment group were maintained at 3- and 12-month follow-up. The intervention consisted of 6 weekly individual sessions during which parents received nutrition counseling and training in behavioral modification principles. Specific behavioral techniques highlighted included active praising of desired eating behaviors, ignoring noneating behaviors, setting time limits for meals, using successive approximation to determine weekly goals, and providing tangible reinforcement for goal achievement.

The management of developmental and treatment-related concerns for the preschool child may have negative implications for broader family interaction patterns as well as the adjustment of other family members. For example, Spieth and colleagues compared mealtimes of families of a preschool child with CF with those of families of children without CF. Relative to the healthy control families, the families with a child with CF had greater deficits in family functioning in areas such as communication, emotional expression, interpersonal involvement, and appropriate behavioral expectations and enforcement. Greater marital role strain and decreased home recreation time have also been reported in families caring for a preschool child with CF. There is also evidence that parents of preschool children with CF are at risk for problematic psychosocial functioning. For example, in the study by Ward and colleagues, parents of preschool children with CF reported increased levels of depression (33%), anxiety (16%), and stress (34%).

Siblings in the household may also be affected. Research across chronic illnesses has shown that the presence of a child with chronic illness in the family has a negative effect on siblings, particularly in the areas of peer activities and psychological functioning. Although sibling psychosocial functioning has not been studied in CF, research has examined differences in how parents treat the child with CF compared with a healthy sibling. A study comparing parenting practices toward sibling dyads in which the younger sibling had CF versus sibling dyads of 2 healthy children found greater discrepancy in patterns of differential treatment in the families with CF. Mothers spent more time with the younger sibling who had CF in mealtimes and playtimes. In addition, mothers rated the quality of their time with their ill child as extremely positive, but reported as much negative time as positive time during interactions with their well child despite no evidence that these children had worse behaviors. The strong emotional reactions of having a child with a life-limiting illness may promote more positive feelings toward that child and more negative parenting relationships with well children. Beyond the effects this differential treatment may have on the sibling’s adjustment, the quality of the sibling relationship may be negatively affected, which may have long-term social implications for both children.

Early childhood: preschool

During the preschool period, developmental issues related to expanding language skills, cognitive development in areas such as understanding causality and assessment of ability to control environment, and emotional/behavioral regulation development may be of particular relevance to the experience of CF. Developing language skills helps the child process and express their experiences so that parents and health care providers can have a better understanding of the child’s cognitive and emotional events. Although much research has tied children’s causal attribution to Piagetian stages, with young children attributing illness to magical or superstitious factors, recent research suggests that preschool children may have a more sophisticated understanding of illness which also incorporates biologic factors. In the one study that looked directly at causal attributions in a CF sample, more than half of the 4- to 6-year-old children with CF knew that they had been born with the illness and only 1 of the 17 preschool children studied stated they were being punished with their CF. However, few knew what the various treatments were for. The preschool child is also still developing accuracy in understanding which events can or cannot be controlled, which has significant implications for the young child with CF who may be in a myriad of stressful environmental situations beyond their control such as medical procedures and treatments. The young child with CF may exhibit extreme behavioral reactions to attempt to escape these procedures, and may develop heightened aversion for future medical events.

The interface of behavioral self-regulation and CF-related tasks is particularly noteworthy in this age range, when preschool children are already contending with increasing awareness of behavioral limits, whereas parents are developing strategies to enhance compliance. Even when children have mild disease and low levels of symptoms, treatment burden is significant: daily nutrition goals, enzyme supplementation, and ACT are recommended to preserve growth and lung health. The increased opportunities for parent/child conflict may alter parenting strategies such that parents may become either more authoritative or harsh (particularly for health-related behaviors deemed essential for survival) or more permissive as a result of parenting resources stretched thin by the chronic illness, a choose-your-battles approach, or overprotection. These additive behavioral demands associated with CF treatment can challenge the emotional resources of children and parents and can have negative implications for child and parent mental health, general family functioning, and health-related outcomes. There is little research exploring general psychosocial functioning in young children with CF. However, Ward and colleagues recently published the results of a study comparing parent-reported behavioral functioning of preschool children with CF with normative data. Although increases in child internalizing or externalizing mental health issues were not found, nearly half of the children with CF were found to have moderate to large sleep and/or eating problems, and 40% of the children had poor compliance with ACT. Thus, although not appearing to have significant psychological impairment, these preschool children with CF did struggle with health-related processes and behaviors.

Problematic mealtime behaviors are a particularly salient health-related concern for children with CF because they are at high risk for poor growth as a result of chronic lung infection and dietary fat malabsorption. Nutritional status, in turn, is related to lung functioning, health complications, and morbidity in CF. Although nutritional guidelines for children with CF recommend eating between 110% and 200% of the DRI, studies of infants, toddlers, and preschool children report typical consumption of only 100% of DRI, with fewer than one-quarter of the children achieving the minimum recommendation for children with CF. As might be expected given the sense of urgency parents feel and the higher demands placed on the children, mealtimes with preschool children with CF have been shown to be more problematic than in healthy families. Children with CF show a higher frequency of behaviors that interfere with eating such as crying and whining, delaying meals by talking, spitting out food, and leaving the table. At the same time, parents of children with CF engage in behaviors such as increased coercion, commands, physical prompts, and actual feeding of preschool children compared with a healthy sample. Parents often cite mealtime as a particular concern, and report thinking that they have little control over their children’s eating patterns.

Although CF-related eating concerns persist throughout development, assessment and intervention during early childhood may change the trajectory of maladaptive behaviors and interactions. In general, behavioral problems in children in this age range are best treated through behavior modification. A recent randomized clinical trial of behavioral modification involving toddlers and preschool children with CF found an increase in average daily caloric intake of approximately 850 kcal, corresponding to 120% of DRI. This finding was significantly better than a treatment-as-usual control condition in which the children’s caloric intake declined during the same period. The gains found in the behavioral treatment group were maintained at 3- and 12-month follow-up. The intervention consisted of 6 weekly individual sessions during which parents received nutrition counseling and training in behavioral modification principles. Specific behavioral techniques highlighted included active praising of desired eating behaviors, ignoring noneating behaviors, setting time limits for meals, using successive approximation to determine weekly goals, and providing tangible reinforcement for goal achievement.

The management of developmental and treatment-related concerns for the preschool child may have negative implications for broader family interaction patterns as well as the adjustment of other family members. For example, Spieth and colleagues compared mealtimes of families of a preschool child with CF with those of families of children without CF. Relative to the healthy control families, the families with a child with CF had greater deficits in family functioning in areas such as communication, emotional expression, interpersonal involvement, and appropriate behavioral expectations and enforcement. Greater marital role strain and decreased home recreation time have also been reported in families caring for a preschool child with CF. There is also evidence that parents of preschool children with CF are at risk for problematic psychosocial functioning. For example, in the study by Ward and colleagues, parents of preschool children with CF reported increased levels of depression (33%), anxiety (16%), and stress (34%).

Siblings in the household may also be affected. Research across chronic illnesses has shown that the presence of a child with chronic illness in the family has a negative effect on siblings, particularly in the areas of peer activities and psychological functioning. Although sibling psychosocial functioning has not been studied in CF, research has examined differences in how parents treat the child with CF compared with a healthy sibling. A study comparing parenting practices toward sibling dyads in which the younger sibling had CF versus sibling dyads of 2 healthy children found greater discrepancy in patterns of differential treatment in the families with CF. Mothers spent more time with the younger sibling who had CF in mealtimes and playtimes. In addition, mothers rated the quality of their time with their ill child as extremely positive, but reported as much negative time as positive time during interactions with their well child despite no evidence that these children had worse behaviors. The strong emotional reactions of having a child with a life-limiting illness may promote more positive feelings toward that child and more negative parenting relationships with well children. Beyond the effects this differential treatment may have on the sibling’s adjustment, the quality of the sibling relationship may be negatively affected, which may have long-term social implications for both children.

School age

The school-age period is characterized by evolving cognitive skills and an increased emphasis on peer relationships. As children’s cognitive and language skills expand, they are able to communicate better about their own beliefs and expectations related to their illness. This development may facilitate effective psychoeducation that targets misinformation and addresses unfounded fears or concerns. It is essential that children’s sense of ownership and control of their chronic illness be encouraged during this period to develop skills and self-efficacy related to self-management and collaboration throughout the life span. The opportunity to develop child self-care skills seems to be missed frequently. Savage and Callery observed family-provider interactions during CF clinic visits, and found that most school-age children were marginalized by staff, felt bored, and perceived the interaction to be only between their parent and the provider. In addition, children’s own health priorities were not addressed. For example, diet consultations were focused toward the parent and emphasized weight gain, whereas children reported that their primary desired outcome for increased nutrition was more energy. Children are more interested in adopting healthier behaviors if these behaviors are framed toward outcomes personally desired and meaningful to school-age children.

Although cognitive changes can affect children’s relationships with health providers, peer relationships are a core catalyst for the psychosocial development of the school-age child. Children’s identity and sense of competence begin to be developed largely through a process of comparison with their peers. For the child with CF, this peer comparison process may highlight their CF-related differences (eg, frequent coughing, taking enzymes with meals, more fatigue, school absences). Children who were diagnosed at birth or in early childhood may, in effect, undergo a second diagnostic period during which they realize at a deeper level the effect of CF on multiple aspects of their life. School-age children with CF describe significant concerns related to being different than their peers, with a high premium on appearing normal. Coping with negative peer reactions such as teasing or overprotection in response to the visible manifestations of CF is reported to be one of the most stressful daily events for children with CF. Even with supportive peers, children seek to diminish the emphasis placed on their illness. Children are often unsuccessful at hiding the more visible aspects of the disease, and may be setting themselves up for worse peer issues because providing the CF context for atypical behaviors may minimize negative peer perceptions. Keeping illness status a secret can also negatively affect the ability to develop and maintain intimate friendships.

Support from peers who share the same medical illness has often been promoted to circumvent the potential for stigmatization, decrease the sense of alienation, and increase sharing of adaptive strategies to maintain quality of life. Before the mid-1990s, children with CF were encouraged to attend CF-related summer camps and other venues for peer support, and although empirical evidence is lacking, research with other chronic illnesses suggests that these experiences were beneficial for increasing adjustment. However, in the mid-1980s CF medical communities became cognizant of Burkholderia cepacia , a particularly treatment-resistant constellation of bacteria that can be contracted from environmental sources and person-to-person transmission, and that often corresponds with rapid decline in lung functioning for patients with CF. It was soon determined that CF summer camps were the setting for epidemic spread of this bacteria, and these programs were closed. Other treatment-resistant bacteria have since been identified, and evidence of epidemic spread has been found within outpatient CF clinics as well as on inpatient hospital wards. In reaction, CF centers have implemented strict segregation policies (eg, isolating children with CF from each other during clinics), and have strongly recommended that patients with CF should not interact with each other. There is little research into the emotional consequence of the stricter infection-control policies. However, some studies suggest that parents and children are in agreement with the policies because of the health benefits incurred by segregation but also identify costs such as decreased social support, feeling alienated, and missing friends with CF. Thus, children and adolescents with CF, although having similar challenges with healthy peers as do other children with chronic illnesses, are not afforded the benefits of support from those peers with CF who would most closely understand their experiences.

The additive psychosocial challenges incurred by CF during this age period seem to increase the risk of psychological distress in these children, although there is some disagreement in the literature. In a study using semistructured diagnostic interviews, 60% of children with CF met criteria for a psychological diagnosis, with an anxiety diagnosis present in 35%. Externalizing diagnoses were also present, with a prevalence of 22.5% for oppositional defiant disorder and 12.5% for conduct disorder. Depression rates have been found to range from 2% to 9% in some studies to 33% in more recent studies. Poorer adjustment in this age group has been related to higher levels of stress, lower self-efficacy, increased monitoring for CF-related cues, and less internal health locus of control. Other cognitive factors from the general chronic illness literature that may be relevant are illness uncertainty (the degree to which children experience confusion related to illness status and course) and parental perception of child vulnerability. The life-limiting nature of CF, growth problems, and significant respiratory symptoms may render parents of children with CF particularly susceptible to viewing their child as fragile. The communication of this perception (either overtly or subtly) coupled with the unpredictable course of CF may negatively affect children’s developing self-concept because they may learn to perceive themselves as lacking the skills needed to manage disease-related factors.

Behavioral and cognitive-behavioral interventions are generally considered to be first-line treatments for depression and anxiety in the general population of school-age children. There is a dearth of literature applying these evidence-based treatment protocols to enhance adjustment in the CF population. However, 2 recent studies did integrate cognitive-behavioral features such as problem-solving, anticipatory guidance, behavioral modeling, and relaxation training into their interventions. In one study, children 8 to 12 years old with CF were randomly assigned to either a control group or to a Building CF Life Skills intervention that targeted problem-solving and social skills. The intervention was delivered in 1 home visit and 1 small-group intervention. Loneliness and perceived effect of CF were improved relative to control immediately after intervention as well as at the 9-month follow-up. The use of a group intervention was particularly noteworthy in this study given the usual strategy of isolating children with CF from each other for infection-control purposes. Here, any children with drug-resistant infections were in separate clinic rooms connected by a real-time video camera link, which gave the children a rare opportunity to form a live CF peer group. Another study used a CD-ROM program to increase CF-related knowledge and expand the coping repertoire of 10- to 17-year-old children. Results showed that children randomly assigned to the CD-ROM condition had greater disease-related knowledge and generated more coping strategies to hypothetical challenging situations when compared with a wait-list control.

In addition to challenges related to psychosocial functioning, health-related behaviors may become increasingly compromised as children grow older. Treatment burden is high during this period, with preteen children spending more than an hour per day completing treatment-related tasks. The time-intensive nature of disease management may increasingly interfere with school- and peer-related demands of childhood. A recent study used diary data assessment methods to document ACT adherence in school-age children and found only 51% of the recommended ACT were completed, and of those completed, 64% lasted the recommended duration. In this same study, diary data showed that adherence to enzymes and nebulized medications was less than 50%. Several barriers to adherence have been identified, such as lack of knowledge of CF in general and of individual treatment recommendations, disagreement with provider recommendations, complexity of treatment, psychological issues (on the part of the parent and the child), forgetting, oppositional behaviors, difficulty with time management, and child’s low level of disease symptoms.

Research targeting improved adherence to CF treatment has reported the short-term benefits of behavioral interventions for school-age children with CF. In a recent multisite randomized clinical trial comparing a behavioral versus nutrition-only intervention, children in the behavioral treatment group had nearly double the daily caloric intake than did the children in the nutrition group after the 9-week treatment. Between-group differences disappeared at the 2-year follow-up, at which point all the children maintained approximately 120% DRI. Analysis of the nutrition intervention suggests that this treatment included important behavioral components such as weekly monitoring of intake, successive approximation toward goals, and individualized goal plans, suggesting that behavioral approaches, whether implemented in strictly psychological interventions or integrated into the work of other disciplines, are successful at improving health behavior for school-age children. Single-subject studies have documented the efficacy of behavioral interventions for increasing at-home exercise and ACT. A randomized clinical trial investigating a self-administered education program based on the cognitive-behavioral and social learning models was shown to be effective in improving adherence to prescribed aerosol treatment in this age group, with an improvement in ACT only on the days that the child was feeling unwell.

There are few studies examining the effect of CF on the family of the school-age child. However, a recent study of a clinic sample of parents of children in this age range revealed increased rates of anxiety and depression in mothers and fathers, with rates of anxiety approaching 50%. Overall, however, parents reported a positive quality of life. Correlates of better parent adjustment have been shown to include less self-blame, less avoidant coping, and more social support for parents, family cohesion, and parental hope for positive outcomes for their children. These correlates seem to differ between mothers and fathers. As might be predicted, parents are particularly concerned with and affected by their child’s worsening disease status and possibility of death. Systematic interventions directly targeting the mental health of CF parents are sparse, despite the relationship between child and parental distress. However, one study examined the benefit of massage therapy for school-age children with CF and their parents. Compared with a group that only read together, child and parent anxiety were improved in the massage group, as was child peak air flow.