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Abstract

Bilateral lung transplantation remains a treatment for end-stage cystic fibrosis (CF) lung disease when there is no further medical or surgical treatment available. The aim of lung transplantation is to improve patient life expectancy. The consensus view that lung transplantation extends life in children has been challenged. However, challengers have faced alternative explanations of their finding and it is largely accepted that lung transplantation in the adolescent patient population is an important treatment modality. The goal of this article is to address these concerns and assist with developing creative approaches to this known challenging subset of patients. The main focus of this article will be to discuss the looming question: what existing evidence or best practice insights could improve bilateral lung transplantation outcomes for adolescent patients with end-stage CF?

Keywords

lung transplant pediatric lung transplant adolescent cystic fibrosis adherence adult transition

Introduction

Bilateral lung transplantation remains a treatment for end-stage cystic fibrosis (CF) lung disease when there is no further medical or surgical treatment available [Faro et al. 2007]. The aim of lung transplantation is to improve patient life expectancy. The consensus view that lung transplantation extends life in children has been challenged [Aurora et al. 1999; Liou et al. 2007]. However, challengers have faced alternative explanations for their findings and it is largely accepted that lung transplantation in the adolescent patient population is an important treatment modality [Sweet et al. 2008]. The goal of this article is to address these concerns and assist with developing creative approaches to this known challenging subset of patients. The main focus of this article will be to discuss the looming question: what existing evidence or best practice insights could improve bilateral lung transplantation outcomes for adolescent patients with end stage CF?

Identifying the population and pertinent factors

Pediatric lung transplant statistics

CF is the leading indication for bilateral lung transplantation in children (aged 6–11) and adolescents (aged 12–17) accounting for 56% and 72% of all transplants, respectively [Benden et al. 2011]. Survival for pediatric lung transplant continues to be similar to that of the adult population. The half-life of adult recipients was 5.4 years compared with 5.5 years in pediatric recipients. Statistical analysis of recipient survival when analysis was performed in different age groups reveals a significantly better survival of recipients aged 1–11 years compared with recipients aged 12–17 years, 7.3 years versus 4.7 years, respectively [Benden et al. 2011]. It appears that below average outcomes from bilateral lung transplantation may be associated with both CF diagnosis and adolescent age, which represent the largest population and illness etiology in this age group. When adolescent recipients with CF were compared with other adolescent groups receiving bilateral lung transplant, such as pulmonary hypertension, CF recipients reveal a trend for greater median survival [UNOS, 2011].

An institutional study which was performed at the University Hospital of Zurich examined comparisons between a 5-year survival post-bilateral lung transplants for patients versus a calculated 5-year survival without lung transplantation. Using a predictive year survivorship model, they demonstrated that performing a lung transplant at an experienced center can offer true survival benefit for patients with end-stage CF disease. Furthermore, pediatric age had no negative impact on survival in this cohort. Despite some centers reporting excellent survival in their CF patient populations post-transplant, there is nevertheless a trend in the ISHLT data set that suggests a decreased survival in the patient population between 12 and 17 years of age. There are many factors that are believed to contribute to this observed difference in the survival of the adolescent aged patient when compared with younger patients and the adult population.

Defining the adolescent patient

Adolescence is defined as the period of physical and psychological development from the onset of puberty to maturity. Chronologically this generally encompasses children between the ages of 12 and young adulthood. Adolescence marks the beginning of the development of more complex thinking processes including abstract thinking, the ability to reason from known principles, the ability to consider many points of view according to varying criteria, and the ability to consider the process of thinking [Abbott et al. 2001]. In times of extreme stress, such as when experiencing complex medical care and chronic disease, adolescents may have difficulty with complex thoughts. The adolescent may be unable to systematically use decision-making capabilities which could therefore impact future care. Many of the areas of concern that will face this select age group include but are not limited to: adherence; depression, anxiety and adjustment disorders; system support; and transition to adult centers.

Defining CF in the adolescent period

CF disease in the adolescent patient can be a very challenging chronic illness. Lung function as measured by forced expiratory volume in 1 second (FEV₁) is a measure that predictably declines with age in CF patients [Konstan et al. 2007]. The severity of respiratory disease and the scope and impact of extrapulmonary disease manifestations (gastroesophageal reflux, cirrhosis, diabetes, nephropathy, bone density disorders and depression) may emerge or peak in adolescence [Braun and Merlo, 2011]. An estimated 20% of adolescents with CF develop CF-related diabetes [Moran et al. 2010]. These comorbidities associated with CF can contribute to the complexity of care in patients experiencing severe end-stage lung disease. While defining the mechanism awaits further research, many adolescent CF patients experience deterioration of their general health status that contributes to worsening pulmonary function. This includes impaired general fitness [Philpott et al. 2010], poor nutrition [Nasr and Drury, 2008], and sleep disturbances [Naqvi et al. 2008].

Psychosocial aspects

The effects of psychosocial phenomena on CF patients may be particularly pronounced in adolescents. Indeed, whether cause or effect, psychosocial functioning deteriorates in parallel with age (as does lung function) [Pfeffer et al. 2003]. The CF patients perceived quality of life and physiological status correlate with one another, inducing anxiety and depression [Havermans et al. 2008]. Compared with other age groups adolescent CF patients score significantly lower in the emotional state, social, body image, and respiratory domains of the Revised Cystic Fibrosis Questionnaire (CFQ-R) [Hegarty et al. 2009]. Adolescent depression is commonplace, but is even more prevalent in those with chronic disease [Bennett, 1994]. Depression in children with CF places patients at greater risk for nonadherence to medical management [Smith et al. 2010]. Depression may be the most common and troublesome comorbidity in adolescent CF patients [Quittner et al. 2008]. This comorbidity increases with age and is correlated with active wait listing for transplantation. Rates vary more with severity of illness than other variables, and regardless of the patient’s pre-existing support system [Wray and Radley-Smith, 2006], meaning, even with an excellent family and social support system depression is more closely related to severity of illness than an outstanding support system [McCubbin et al. 2001]. Treatment of depression is an important aspect of pretransplant care, and mental health treatment should be provided. Transplantation is not curative for anxiety, adjustment or depressive disorders and there can be reoccurrence posttransplant. Such disorders, while they can still be present, generally improve posttransplant [Wray and Radley-Smith, 2004].

Adherence

Adherence to complex medical protocols is problematic in the adolescent age group [Bender, 2006], particularly in the setting of solid organ transplant recipients [Wells and Faro, 2006]. Several factors have been shown to influence the likelihood of successful adherence to complex medical regimens. Gender disparity among adolescent patients has significant influence on patient outcomes. Studies have shown that adolescent female patients with a chronic disease are less adherent than their male counterparts [Patterson et al. 2008]. This has been demonstrated in the CF population specifically. Studies have found that females have a worse prognosis than males with CF. The median survival age for females with CF is approximately three years less than for males, according to Demko and colleagues and Sims and coworkers [Demko et al. 1995; Sims et al. 2005]. This finding was attributed to early acquisition of Pseudomonas aeruginosa which resulted in worse survival, poor chest radiograph scores, and pulmonary function tests than patients without these bacteria. According to Stallings and colleagues, poor nutrition, growth status, and delayed menarche were associated with poor pulmonary function in females with CF [Stallings et al. 2005]. Milla and colleagues Sims and coworkers performed a survival analysis on a cohort of CF patients to determine the development of diabetes on survival. They found female subjects with CF-related diabetes have a poorer prognosis compared with male subjects with CF, and female subjects with CF without diabetes [Milla et al. 2005; Sims et al. 2005]. The etiology of this sex difference was not clear. Finally, Patterson and colleagues sent a questionnaire (LCFQ) to patients ages 10–21, both males and females with CF, comparing perceptions of disease-related strains, living with CF, and factors related to treatment feelings and behavior [Patterson et al. 2008]. They found that CF appears to have a greater emotional impact on adolescent females when compared with adolescent males, which may contribute to poorer pulmonary function among females with CF during adolescent years.

With regards to lung transplantation, the gender ratio of patients waitlisted for lung transplantation is approximately equivalent. Based on current data from the Organ Procurement and Transplantation Network, there are currently 46 patients between the ages 11 of 17 actively waitlisted for bilateral lung transplantation in the United States. Of these waitlisted lung transplant patients, half are female [UNOS, 2011]. However, more female patients actually undergo transplantation [HRSA, 2012]. When reviewing the total number of lung transplantations performed nationally between 1 January 1988 and 30 April 2012 of patients aged 11–17, the amount of female transplantations is double the number of male transplantations [Scientific Registry of Transplant Recipients, 2010].

Gender aside, adherence concerns persist suggesting poor compliance with recommended supportive care. In both male and female adolescent patients, it is clear that adherence with physiotherapy and nutrition declines as patients enter adolescence and as their disease progresses [Arias Llorente et al. 2008]. This association is clear, but the causality is not as unequivocal. Perception of disease status is known to influence adherence, so the finding that adolescents with CF underestimate the severity of their illness is important [Britto et al. 2004]. Belief in the importance of treatment also declines in adolescence exacerbating nonadherence [Bucks et al. 2009]. In two studies of adherence barriers based on age, attitude issues, and oppositional behaviors were both less prominent in younger children, than in adolescents [Dziuban et al. 2010; Modi and Quittner, 2006]. This pattern of adherence does not always change posttransplant where adolescents in other solid organ populations struggle with medical regimen adherence whether intentionally or unintentionally [Wray and Radley-Smith, 2006].

Quittner conducted a randomized, controlled trial to improve adherence in adolescents with CF. The aim of this study was to evaluate the effectiveness of a behavioral intervention called the Comprehensive Adherence Program (CAP), when compared with standard care with regards to medication adherence. Results suggest that adolescents have significant gaps in knowledge of disease management and treatment skills [Quittner, 2011]. Koocher and colleagues [Koocher et al. 1990] suggested that nonadherence falls into two groups that are based on patient’s knowledge and beliefs: inadequate knowledge (unintentional nonadherence) and education nonadherence (intentional nonadherence) [Koocher et al. 1990]. When asked, 40% of participants reported that they would like to know more about how some of their CF treatments work [Koocher et al. 1990], suggesting that most of these participants are in the unintentional nonadherence group.

Dziuban and colleagues examined barriers to adherence in CF patients and found that adolescent males aged 12–20 were more likely to agree that it is acceptable to miss doses if they are made up with extra doses later. Patients who perceived themselves to be less healthy agreed more to statements expressing feelings of limited freedom, nonsympathetic medical providers, and difficulty adhering during times of decreased symptoms [Dziuban et al. 2010].

Abbott and colleagues found that CF treatment adherence is treatment specific, reporting adherence rates of 46% to vitamins, 53% to physiotherapy, 74% to exercise, and 83% to enzymes [Abbott et al. 1994]. Other studies have looked at coping with CF and implications for adherence. These studies found that those who were adherent scored high on the optimistic acceptance scale and hopefulness scale, and that the degree of adherence to treatments was influenced by personal styles of coping. This indicates that the identification of effective coping strategies to aid both long-term psychological and clinical well-being might improve adherence [Abbott et al. 2001].

Early work examining the psychosocial impact of chronic illness in children took what has been described as the ‘deficit–centered’ approach, which assumes that the child would inevitably show emotional and behavioral deficits [Drotar, 1978]. Even with this perspective the literature is far from clear regarding the extent to which children show maladjustment. There are also many other issues of concern in addition to psychosocial function, but many of these are poorly studied, partly because there is no obvious standardized instrument available [Eiser, 1997].

Pediatric Transplant in the CF patient is reserved for patients with end-stage lung disease, who often fall into the adolescent age group. While this clearly represents a daunting challenge, the existing body of research provides many clues for approaches that may be effective in improving outcomes in this population group.

Age-related differences

Age and disease processes interact in a complex manner. The concept of life course epidemiology provides a conceptual roadmap through this maze [Ben-Shlomo and Kuh, 2002]. In congenital chronic disease the adolescent may manifest a critical or sensitive stage in the disease process and the cumulative effects of the disease process physiologically. There are also other factors such as biological and psychosocial consequences that lead to delayed growth and puberty. Finally, there is a range of psychosocial developmental impairments affecting health status, response to treatment, and participation in treatment [Suris et al. 2004]. These phenomena not only disproportionately exist in the chronically ill adolescent population, but may also concurrently peak and act synergistically. Parents and scholars have both long understood the special vulnerability of this age, with or without disease [Abraham et al. 1999]. Among sick adolescents, adaptation to disease and treatment coincide with adaptation to adult life, perhaps most challengingly within the family [Fisher and Weihs, 2000]. Timing has yet another clinical implication. In lifelong chronic disease management without cure, disease can have a deleterious effect not only on the patient’s medical status but on the availability of economic and psychosocial resources. There is evidence that parental supervision is lost, without replacement, possibly more as a result of role fatigue than a failure to modify parenting styles [Modi et al. 2008]. Financial resources may also be strained. Over the period 2001–2007, strictly outpatient costs for CF care outpaced the consumer price index by 20:1 [Briesacher et al. 2011]. Just when community resources and support are most critically needed, the development of such networks outside the family are most impaired, delayed, or willfully precluded: 17% of adolescent CF patients refuse to disclose their diagnosis to others [Barker et al. 2011].

Lung transplantation process

Transplant is principally timed by demand and organ availability, but is not a curative modality. Thus, age, particularly developmental age, and timing loom as challenges or opportunities in the quest for improved transplant success. The lung transplantation process represents a period of extreme uncertainty and discontinuity for patients and families. Improving these could have measurable outcome benefits, but there is a paucity of evidence to support this conclusion.

One study directly addressed the issues of uncertainty and discontinuity, finding that a telemedicine intervention did improve satisfaction with care and body image perceptions among patients awaiting transplant, but did not improve scores for quality of life such as anxiety depression, or use of outpatient services [Wilkinson et al. 2008]. Other research has shown that psychosocial problems are generally improved by transplantation [Reiss et al. 2005]. On the other hand, established patterns of noncompliance tend to persist posttransplant [Wray and Radley-Smith, 2006].

For adolescents with CF, transplantation provides hope and the promise of extending their lives. This does not, however, come without a cost: transplant, while hoping to provide an improved life expectancy with an enhanced quality of life, exchanges one complex medical condition for another, and represents a dramatic change in their lives. Speculatively, ‘system readiness’ (for uncertainty and discontinuity) and its match to ‘patient readiness’, once specified and operationalized, may stand shoulder to shoulder with other lung transplantation eligibility criteria.

Michaud and colleagues reviewed and organized strategies shown to optimize medical professional responsiveness to the chronically ill adolescent [Michaud et al. 2004]. They emphasize that direct patient communications appropriate to the development stage and a contractual approach to adherence are especially necessary, yet are often absent in practice. Messages must be constructed and delivered in ways that are mindful of the fact that each ‘packet’ can have intended or unintended social, biological and health behavior effects [Cummings et al. 2011]. A major aim of communicating with adolescent CF patients should be acceptance of disease as this has been shown to reduce depression, improve adherence, and social function [McLaughlin et al. 2008].

Many of the challenges of adolescent CF patients can be attributed to maladaptation in the family [Annunziato et al. 2007]. Yet of the 176 studies identified by Berge and colleagues over a 20-year period many had serious methodological limitations [Berge et al. 2007]. A recurrent theme is facilitating parental involvement by helping them adapt to the new dynamic introduced by adolescence. By self-report, adolescents want an educational approach, involving trust and a transfer of responsibility [Bregnballe et al. 2011]. Because many adolescents with CF experiment with risky behaviors, life coaching should displace some of the time parents spend on other matters [Michaud et al. 2004]. The natural adolescent quest for independence, and the psychosocial and somatic benefits derived ought to be programmed rather than presumed. Key elements of self-management include the promotion of health education, communication skills, decision-making and problem-solving skills, and self-care in the context of meaningful social support [Sawyer and Aroni, 2005]. Self-management skills are integral to both optimal medication adherence and the achievement of independence necessary for a successful health care transition [McDonagh and Hackett, 2008; Watson, 2005]. Tools are available to assess readiness and guide progression [Baker and Denyes, 2008]. And, yes, ‘there is an app for that’ to appeal to the tech-savvy teen [Cummings et al. 2011].

Pediatric to adult medical transition management

Transition conventionally refers to processes occurring between pediatric and adult medical care. Lung transplantation may compound some of the standard difficulties inherent in the transition process [Annunziato et al. 2007; McLaughlin et al. 2008; Vandenbranden et al. 2012]. A recent review [Rosen, 2004] highlighted the following predictors of transition success: flexible timing, self-care readiness, information and coordination, joint oversight, family engagement, continuity of primary care. While some may be limited by the special circumstances of transplant, none appear to be categorically precluded. Pediatric transplant centers must effectively interface with their adult counterparts. The transition of adolescents with chronic disease to an adult medical center is a challenge [McLaughlin et al. 2008; Reiss et al. 2005]. An unsuccessful transition from a pediatric center to an adult one could significantly affect a patient’s short-term and long-term health. The risk of failure of this process in solid organ transplantation could be detrimental with either decreased graft survival or overall patient survival. In the liver transplant population, tacrolimus levels were used as a surrogate marker to monitor patient adherence. Lower drug levels were observed in the transition cohort compared with patients in the pediatric and adult populations that were not transitioned, with lower levels observed in patients with decreased adherence [Annunziato et al. 2007]. Transplanting adolescents at centers that are prepared to comprehensively care for this patient population is also important.

The Bell consensus panel is a report discussing adolescent transitioning to adult medical care which is specific to solid organ transplantation [Bell et al. 2008]. The panel’s practical tips include: having a current, portable synopsis of status and treatments; sessions devoted to adolescent problem solving and goal-setting; a role conversion course for parents which steps them down from ‘CEO’ of care to ‘consultant’; and a program that steps adolescents up towards adult integration. The latter recommendation is notable for addressing the impending loss of the supportive role of school, the practical needs associated with evolving independence, and the psychosocial benefits of anticipating an adult life. Some additional suggestions included in the panel’s suggestions include transition clinics, a year of follow up by the pretransition or pretransplant provider, and an independent evaluation of transition or transplant outcomes.

The Children’s Hospital of Philadelphia: Transitioning in Cystic Fibrosis

The ability to transitions adolescent transplant recipients to adult programs is paramount to long-term outcomes. The concept of an ongoing quality initiative for improving the transition process from pediatric transplant patients to the adult transplant center is important. One of the initial steps is informing families that at the age of 14, the adolescent patients, will be seen alone for part of the clinic visit. This is done to enhance their knowledge of CF and transplantation, discuss high-risk behaviors, and help them to gain more independence in their care. Discussion of transition should be an ongoing topic at each of these visits. Each patient should be treated individually and transition times may vary. However, the goal is for all patients to meet the adult transplant center physician prior to official transition. There is significant importance in achieving continuity of care at this period of adjustment. Since implementing this practice at the author’s center there has been a dramatic increase in transition rate, nearly doubling patients that have been transition comparing 2010 with 2011. This change reveals a positive trend and reflects a more strategic practice of transition which includes monthly meetings with the adult CF/transplant care team as well as providing tours of the adult CF center/transplant center, and the patient meeting the adult care team prior to their first posttransition visit. At the present further work to develop a tool to survey our adolescent patient’s pretransition and posttransition will hopefully provide greater insight how to further improve the process.

Discussion

This article sought to integrate evidence-based best practice into an approach to caring for this challenging adolescent patient population. Certain key features that characterize this age group include but are not limited to: adherence; depression, anxiety and adjustment disorders; system support; and transition to adult centers. The authors believe a systematic approach needs to be developed with the special risks of adolescent transplant prospects in view. In order to optimize an individualized approach to each patient, we strongly encourage referring physicians to disclose details regarding adherence and other psychosocial factors that may influence care. Throughout the pretransplant process, a multidisciplinary team addresses both the patient and their family members’ needs. The team is selected and specially trained to work with patients and families with chronic disease. The transition plan has two main aspects it is contractual, between team and patient–family, and the care plan is viewed as dynamic, allowing flexibility and adaptations based on ever-changing circumstances. Transplant referral centers should be trained to respond to the challenges posed by adolescent patients. Success derives from a system always open to innovation.

Summary

This article serves to support pediatric lung transplantation within this challenging patient population, while in the same regard appreciating and sustaining the hypothesis that being an adolescent patient, per se, is not the risk factor, but the chronological summit of a wave of medical and psychosocial challenges. Considered as a discontinuity to which patient and family have to adapt, the transplantation process itself may stir up that wave even further. Some of these challenges are coincidental to chronological and developmental age, while others are agonized or initiated by phenomena unique to this life stage. Deconstructing the wave supports the view that adolescent transplant candidates require special focus, strategic planning for successful outcomes, and targeted interventions to calm down the wave.

Footnotes

Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Conflict of interest statement

The authors declare no conflicts of interest in preparing this article.

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Transplanting the adolescent cystic fibrosis patient: can we do it?

Abstract

Keywords

Introduction

Identifying the population and pertinent factors

Pediatric lung transplant statistics

Defining the adolescent patient

Defining CF in the adolescent period

Psychosocial aspects

Adherence

Age-related differences

Lung transplantation process

Pediatric to adult medical transition management

The Children’s Hospital of Philadelphia: Transitioning in Cystic Fibrosis

Discussion

Summary

Footnotes

Funding

Conflict of interest statement

References