Internet Editor’s Note: This article is dedicated to my sister, Samantha Grosse, who lived with Cystic Fibrosis and passed away in 2009 at the age of 22.
Cystic Fibrosis (CF) is the most common progressive, chronic, life-threatening genetic disorder. However, it is still rare. There are around 30,000 cases diagnosed in the United States (Ernst et al., 2011; Kerem et al., 2005). There is no known cure for CF, however, it is common for patients with CF to live into their 30’s. CF causes mutations in cells that produce mucus and affect digestion, associated with the cystic fibrosis transmembrane conductance regulator gene (CFTR gene; Ernst et al., 2011). Bodily functions affected by CF are the lungs, pancreas, and digestive enzymes, which are vital for adequate absorption of nutrients. These areas become dysregulated and can cause malnutrition. Complications can later lead to increased rates of infertility, liver disease, CF-related diabetes (CFRD), respiratory failure, the contraction of more serious viruses from compromised immune functioning, shortened lifespan, and/or eventually the need of a lung transplant. These complications have many implications aside from early mortality, such as the illness burden of living with a second chronic illness, frequent hospitalizations throughout the life span, and end-stage lung disease/end of life considerations. Since it is genetic, both parents must be carriers of the CFTR gene for the child to potentially be a carrier or have the disease itself. Diagnosis of the disease normally happens during a newborn screening immediately after birth or even prenatally, as most people with CF are diagnosed before the age of two. Testing can also be done through sweat tests and genetic testing. Most effective treatment outcomes are seen to be in earliest intervention.
Factors Affecting Outcomes
There are many factors that will affect the prognosis of the disease, and it is dependent on the person’s unique individual case. Factors that affect these outcomes include age of diagnosis, severity of case of diagnosis, access to care, caregiver factors, and treatment adherence, among others (Ernst et al., 2011). It is important to note that the treatment burden of CF is significant, even in more mild cases with low levels of symptomology. Despite recent scientific advances, the average life expectancy is still quite short, being around 37-years-old. Due to these complex and differing factors, treatment varies for patients with CF. Treatment plans are invasive and extensive, and on average take two to four hours a day in total to complete as interdisciplinary care is often required. Mortality with CF typically occurs due to complications related to pulmonary infections, and females are at higher risk (Ernst et al., 2011; Kerem et al., 2005).
The intense treatments, as well as mortality factors that come with this disease, can make it difficult for patients and their families to cope at times. However, according to Ernst et al. (2011), “despite the enormous treatment burden and shortened mortality, individuals with CF and their families have been shown to be tremendously resilient, with most parents reporting a high quality of life”. Health psychologists working with this population should be aware and responsive to the impact CF has on the child’s development, as well as the impact on the gradual physical deterioration and how that impacts the patient’s psychological needs and quality of life (Kerem et al., 2005). The role of a health psychologist is imperative consequently.
Anxiety and depression are the two most common psychiatric comorbid diagnoses in CF, and they are found at higher rates in the CF population than the general population (Cruz et al., 2009). Anxiety disorders (i.e., panic disorders, phobias, obsessive-compulsive disorder, separation anxiety disorder, and generalized anxiety disorder) and depressive disorders, are both related to poorer quality of life and disease outcomes in CF adolescents. However, depression seems to be the most detrimental to CF patients (Cruz et al., 2009; Smith et al., 2010). Depression is correlated with lower treatment adherence. One of the most worrisome findings is that comorbid depression is related to worse health outcomes for patients with CF, related to younger age of death (Cruz et al., 2009). Interestingly, there is also a higher reported diagnosis of CFRD when the CF patient has symptoms of an anxiety disorder (Cruz et al., 2009). This highlights the effect psychiatric disorders can have on the progressing prognosis of the disease. It has also been found that child depression scores were significantly and positively associated with lower rates of airway clearance adherence (Smith et al., 2010).
Another well-studied area is the risk for children with CF to have distorted eating patterns and poor nutritional adherence. Simon and colleagues (2011) explored children with CF’s body satisfaction in relation to nutritional adherence and health-related quality of life. The results demonstrated that 24% of the children were not adherent to caloric goals. These youths typically consumed caloric levels that were recommended for a healthy child, and not to the heightened caloric level suggested to CF patients. Due to this, over 40% of patients were found to not be currently obtaining their required minimum fat intake. A troubling finding is that over 44% of females in the study were found to be inconsistent with their treatment goals due to desiring a smaller body size or weight despite being already under the recommended body mass index weight for their height and weight group, compared to only 8% of males with CF in the study. Additionally, the females found to be treatment inconsistent due to their distorted body image thoughts had very low health-related quality of life scores. It is possible that some of the youth with CF with distorted eating and body image thoughts would qualify for eating disorder diagnoses. This highlights the importance of clinicians working with this population to be informed in eating disorder treatment.
Infancy and Attachment
Parents with infants who tested positive for CF screenings report high levels of depressive and anxiety symptomology (Ernst et al., 2011; Quittner et al., 2014; Smith et al., 2010). The attachment period for the infant and the parent could be affected; however, many show normal attachments despite the various disruptions that occur. For children who develop insecure attachments with their caregivers, it has been shown to have a negative impact on their physical health, such as lower body mass index scores and poorer nutritional levels (Ernst et al., 2011). Additionally, even with children who have healthy attachment patterns with parents, CF still seems to play a role in the influence of the familial interactions, showing that parents are more likely to be controlling, serious, and less engaging than parents of healthy children’s counterparts. Around 40% of parents who have children with CF have shown to use blaming themselves for their child’s illness as their main “coping strategy” (Ernst et al., 2011).
In the next stage of early childhood, developmental issues of children with CF stem from cognitive development, language skills, and emotional and behavioral regulation. Youths with CF at this age may have more extreme behavioral reactions to situations than their healthy peers. They may also show more aversion to medical treatments such as surgeries. Conflict between parent and child seems to be enhanced during this age period. This could be due to many of the treatment demands, which can lead many CF parents to take one of two extremes; an authoritarian approach or a permissive approach (Ernst et al., 2011). Two factors might emerge at this age; significant eating problems related to the diagnosis and less cohesive family mealtimes. The combination of these two factors can interact with familial functioning and further communication and emotional deficits might begin to arise. In this developmental period, parents of children with CF also begin to exhibit increased levels of depression and anxiety than in other groups (Ward et al., 2009). Parents are also more likely to spend less time with their healthy child/children and rate their interactions with their healthy children as much less pleasant despite significant behavioral problems not being present with that child/children. This, in turn, can negatively impact the child with CF’s relationship to their sibling(s) (Quittner & Opipari, 1994).
School-age developmental considerations are related to the child taking more control of their chronic illness and beginning to learn how to care for themselves at higher levels, which is related to better outcomes. Commonly seen at doctor’s visits, both health care providers and parents see the medical interaction as solely between the two parties, which leads to boredom and feeling marginalized in the patient (Ernst et al., 2011). In this age group, more uncertainty is sparked compound with CF, and around 60% of children in this developmental stage meet criteria for a psychological diagnosis. Many report feeling lonely (Cruz et al., 2009). Treatment burden seems to heighten in this preteen period, with children typically spending more than an hour a day on treatment. This can be a heavy task on top of the demands of school and peer life. Treatment adherence in this period seems to go down significantly, with only around 51% of preteens completing all of their required treatments (Modi et al., 2006).
Adolescents represent the next developmental group, where peer time begins to outnumber time spent with family. In addition to this, it is typical for CF patient’s health to get substantially worse in adolescence with more intense pulmonary burdens. More severe anxiety symptomology, smoking due to peer pressure, and disturbed eating attitudes might become more common (Ernst et al., 2011). This can create many conflicts for the adolescents with CF, as their peers begin to gain more independence than ever, they may be experiencing more reliance and hospitalizations, which separates and secludes them from their peers further. Gender differences with patents of CF begin to become more apparent in this age group, with females faring much worse in terms of survival rates and risk of pulmonary infection in adolescence, and girls having a greater influence of self-perception in peer life and disturbed eating attitudes (Ernst et al., 2011). Another concern is the degradation of treatment adherence, which steadily worsens from preteens into adolescence, with 30% of patients not doing their treatments at all (Digirolamo, Quittner, Ackerman, & Stevens, 1997).
Important Considerations for Intervention
Other familial factors
Interestingly, when parents are more compliant with the treatment plan and nutritional plan for the child, it is related to higher marital satisfaction among caregivers and lower rates of parental stress. Opposing to this, low levels of parent-child synchrony, as well as low levels of two-party parental agreement relate to child treatment compliance, which will, in turn, lead to worse health outcomes for the child (Eddy et al., 1998). Although CF does not inherently in and of itself cause family dysfunction at higher rates than healthy samples, it does intrinsically change the family unit and can cause strains on marriages (Gotz & Gotz, 2000).
It is apparent that one of the largest barriers to health, longevity, and treatment of CF seems to lie in difficulty with treatment adherence among CF patients. Reluctance or poor treatment adherence is related to a wealth of complications, including but not limited to; greater psychological difficulties and distress, higher rates of psychiatric diagnosis, familial discourse, and health complications (Smith & Woods, 2007). Another factor related to adherence is that there has been found to be very large gaps in the disease-related knowledge for both children with the disease and caretakers (Modi & Quittner, 2006). Additionally, treatment beliefs and knowledge about the importance of specific treatments in adolescents is important. Positive beliefs the patients held about their need for treatment relative to the side effects was correlated to treatment adherence. These findings show how beliefs and cognition can impact treatment outcomes and the role that locus of control may play on the compliance and adherence to treatment (Bucks et al., 2009). This provides hope for the future of psychological health-related interventions and provides insight into how treatment adherence can be implemented.
Intervention considerations concerning treatment adherence state that patients with more effective coping skills have better treatment adherence (Abbott, Dodd, Gee, & Webb, 2001). Additionally, patients who scored higher in optimism and hopefulness had better treatment adherence. Those who used distraction as a coping skill were more likely to be partially adherent while those with avoidant strategies were more likely to be non-adherent to treatment (Abbott et al., 2001). Physicians rarely ask parents or children about treatment adherence, potentially because of time constraints, lack of knowledge, or comfort levels regarding these issues. Additionally, most interventions to treatment adherence focus on education to parents and patients. Although this is important, it is clear that interventions should also encompass perceived barriers being experienced by caretakers and patients themselves, as these may differ by individual. Communication between physician and patients/caregivers should also be improved, and health care providers should be asking more about treatment adherence. With a complex disease like CF, treatments may frequently change, and patients and caregivers would benefit from a thorough explanation of the treatment plan with its requirements and benefits. Ultimately, adequate relationships with their specialist reduces confusion and miscommunication (Modi & Quittner, 2006).
Other psychological interventions
Treatment adherence seems to be one of the most frequent targets of psychological intervention for CF patients. It is clear that targeting treatment adherence could not only improve the wellbeing and quality of life of CF patients but also as a preventative factor of worsening health/development of comorbid diagnoses. The patient’s education needs to be assessed, as more knowledge of the treatment plan and diagnosis can create a higher internal locus of control, which can improve treatment adherence (Smith & Wood, 2007). Intervention should focus on preparing youth for adult life with CF, particularly their big transition from a pediatric hospital to an adult hospital, which typically happens around age 19. This is done effectively by preparation and collaboration of care providers. Unfortunately, transition-related concerns are not typically addressed until late teen years, and many CF patients are unprepared and uncertain of how to make this transition or what it will mean. Intervention to target this should happen in early teenage years, so by the time the very big adjustment arrives, the patient and their family feel ready (Ernst et al., 2011).
At all ages of development, particularly in the younger years, intervention is critically important on addressing psychoeducational barriers to children, such as: why they need to do their treatments, psychoeducation on the disease, social skills training, nutritional and eating related concerns, among others. Across the board, screening for psychiatric comorbidities must be increased. Behavioral and cognitive behavioral therapy and motivational interviewing techniques have seen to be most efficacious (Ernst et al., 2011). Although CBT may be more time consuming than taking psychotropic medications, the long-term outcomes have shown to be more effective (Cruz et al., 2009). Other empirically supported treatments that health psychologists may provide to patients living with CF include; long-term management of the disease, psychosocial functioning interventions, working on independence, gene pre-test education and psychotherapy for family members, and other behavioral interventions (Glasscoe & Quittner, 2008).
Since CF has been historically seen to affect mostly European and European-Americans, ethnic minorities living with CF seem to be left out of much of the research to date. Not only are they commonly underrepresented in the literature; they are also commonly underrepresented in pharmaceutical drug trials for CF (Mcgarry & Mccolley, 2016). Further, clinician and physician perceptions and bias play into this inequity. It is shown that physicians view ethnic minorities with CF are extremely rare and uncommon, when although it is more common for European-Americans to have CF, it is not extremely rare for ethnic minorities to have the disease (Spencer, Venkataraman, & Weller, 1993). Aside from viewing it as less common than it really is, ethnic minorities are also more likely to be diagnosed at older ages, which can significantly impede on the quality of life of the patient both in the present time and in later life (Spencer et al., 1993).
Further, low-income individuals living with CF are a population that might greatly affect outcomes of living with the disease. Being in a low socioeconomic status influences many factors of a patient’s life and quality of life living with the disease. There is a strong correlation between income and mortality rates with CF (O’Connor et al., 2003).
It is clear that there are many needs and factors that health psychologists must consider when working with CF patients and their families. All of these components work together to make CF a multifaceted diagnosis with many elements and layers to consider. Beyond the diagnosis and treatment management, lung transplant seems to be an inevitable process that many with CF will end up seeking towards the later part of their lives that opens up the door for aid from health psychologists as well. Despite the limitations in current research, CF as a disease is more manageable now than ever before. Although it is a rare genetic disease, the multifaceted nature of the complications due to CF make it a very important disease for health psychologists working in pediatrics to be familiar with and comfortable working with. It is imperative that not only are they comfortable working with considerations related to the patients, medication adherence, and transplant, but also with CF patients’ families and developmental issues many children with CF may have. Knowing of the mind-body connection in health, now more than ever before, makes it apparent that the role of the health psychologist is critical. It is important not only for the mental health and transitions in patients’ lives but also for their prognosis and long-term health outcomes.
Cite This Article
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