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Since the beginning of United Network of Organ Sharing data collection in 1987, a total of 8,333 pediatric patients have received a heart transplant in the United States. Because these patients now have longer graft success with improved care and immunosuppression, many of them are entering adolescence and young adulthood. Primary care pediatric nurse practitioners need to be alert to the prevalence of noncompliance with treatment in heart transplant patients, which continues to be highest in adolescence. Low compliance in adolescence increases morbidity, contributes to decreasing quality of life, and is the leading reason for graft failure and mortality in this age group. This article will review common barriers to treatment adherence in the adolescent heart transplant patient, discuss the role of the primary care pediatric nurse practitioner in preventing noncompliance, and review strategies that the primary care pediatric nurse practitioner can implement to improve compliance in this patient population.
). In total, since October 1, 1987 (the beginning of United Network of Organ Sharing data collection), 8,333 pediatric patients have received a heart transplant in the United States (
). Thus, in the United States, there is the likelihood that a pediatric nurse practitioner may encounter a heart transplant recipient in practice. Because these patients now have longer graft success with improved care and immunosuppression (77.93% survival at 3 years after transplantation and 70.38% survival at 5 years after transplantation [
]), many of these patients are entering adolescence and young adulthood. During this delicate time between childhood and adulthood, these patients have to make the challenging transition to self-care. Noncompliance with treatment continues to be highest in adolescent heart transplant recipients and is the leading reason for graft failure and mortality in this age group (
In the United States, there is the likelihood that a pediatric nurse practitioner may encounter a heart transplant recipient in practice.
This article will serve to aid the primary care pediatric nurse practitioner (PNP-PC) in encouraging compliance in adolescent heart transplant patients and facilitating the transition to self-care and self-management by discussing the barriers to compliance and management strategies and by providing resources to improve outcomes.
Prevalence
Currently in the United States, there are 331 patients under 18 years of age who are listed as waiting for a heart transplant. Additionally, the survival rate under the age of 18 years according to the
reported nonadherence rates for adolescents as high as 65%. Although health consequences of nonadherence in all solid organ transplant recipients are significant, patients with cardiac transplants are especially vulnerable to a catastrophic event due to rejection (
studied Organ Procurement Transplant Network data from 1999 through 2007; they identified 2,070 pediatric heart transplant recipients and the evaluated the impact of medication noncompliance on survival in these patients. Oliva et al. found that nonadherence in this population was highest (18.3%) among adolescents and that among patients with more than two reported incidences of nonadherence and subsequent rejection, there was a 56% mortality rate within 2 years. These findings serve to underline the grave importance of this topic and highlight the overwhelming incidence of poor compliance in this adolescent patient population.
Pathophysiology
Reason for Transplantation
Understanding why a patient received his or her transplant is an important part of care as a provider.
Cardiomyopathy
Patients receive transplants for diagnoses of both hypertrophic cardiomyopathy and dilated cardiomyopathy, with the most common indication for pediatric heart transplantation overall being dilated cardiomyopathy (
). Both long- and short-term outcomes after transplantation for cardiomyopathy patients are impressive, with 1- and 10-year survival rates of 90% to 95% and 60% to 80%, respectively (
Congenital heart disease continues to be the most common reason for cardiac transplantation in infants but has decreased as reparative and palliative surgical interventions have evolved (
The Registry of the International Society for Heart and Lung Transplantation: Sixteenth Official Pediatric Heart Transplantation Report—2013; Focus Theme: Age.
The Journal of Heart and Lung Transplantation.2013; 32: 979-988
Retransplantation is considered for patients with allograft failure due to acute rejection less than 6 months after transplantation, severe coronary allograft vasculopathy or moderate to severe systolic or diastolic dysfunction in the absence of rejection if there are no contraindications to retransplantation present such as a history of significant nonadherence (
There are multimodal ways in which rejection can damage the heart and affect morbidity and mortality. Cellular rejection is mediated by T lymphocytes that are mounted against the allograft tissue in the recipient and found in the cardiac muscle upon endomyocardial biopsy (
). Antibody-mediated rejection is facilitated by B lymphocytes, which produce antibodies against the allograft called donor-specific antibodies, and typically occurs soon after transplantation but can occur later (
The Registry of the International Society for Heart and Lung Transplantation: Sixteenth Official Pediatric Heart Transplantation Report—2013; Focus Theme: Age.
The Journal of Heart and Lung Transplantation.2013; 32: 979-988
). PNP-PCs must remember to always assess for the signs of cardiac failure (fluid retention, fatigue on exertion, chest pain) in patients, especially if they are nonadherent, because although most early rejection is asymptomatic, these patients can show signs of early hemodynamic compromise (
For the purpose of this article, the terms nonadherence and noncompliance will be used interchangeably. Different organizations have differing definitions of adherence/compliance with medical treatment, which are open to interpretation by reporting centers and thus may affect the reported rates of nonadherence (
defines adherence as the “degree to which the person's behavior corresponds with the agreed recommendations from the healthcare provider” (p. 3), which encourages inclusion of all aspects of nonadherence including medication noncompliance, missed appointments, missed blood draws, procedures, lifestyle modifications, and diet. This definition is important in the sense that all types of nonadherence can affect the health of the patient and the success of his or her graft, not solely medication adherence.
Nonadherence to or noncompliance with medical recommendations in the pediatric heart transplant recipient can encompass many aspects. The most common form of nonadherence in the adolescent patient population is medication nonadherence (
). Nonadherence to medication is typically measured by timed medication trough levels, which are drawn either during an appointment or at an outside laboratory facility (
However, these patients may also be nonadherent to appointments, procedures, routine laboratory work, lifestyle modifications, and diet, all of which have negative repercussions on health and the success of the graft (
). Ultimately, the definitions available are vague and open to interpretation, and a consistent definition of adherence to medical recommendations is needed (
There are numerous barriers that affect the adolescent heart transplant recipient's adherence with his/her medications and medical regimen. Understanding these obstacles will help the PNP-PC aid the teen in overcoming those barriers.
Socioeconomic Status
Lower socioeconomic status (SES) has been linked to poorer outcomes both for those on the waiting list for a transplant and after transplantation in the pediatric population (
). There has long been a correlation between lower SES status and poorer health outcomes reasons including medication cost, access to health care, living situation, cost of transportation to appointments, and low health literacy. Children of lower SES have a greater risk of graft loss after the first year (
). Inability to obtain or pay for medications, inability to pay for parking at follow-up visits, lack of transportation, or a lack of a stable home will all add to the likelihood of nonadherence to medications and treatment regimens. Social work and case management should be involved in all transplantation patients; however, it is clear that additional attention needs to be paid to those with low SES status because of the increased risk of graft compromise.
Family Environment and Support
Having a supportive and functioning family environment is key to the success of the adolescent transplant recipient. Impaired family function and compromised child psychological function have been associated with higher rates of nonadherence (
). Adherence and care of the transplanted organ must be seen as a family issue, and attention to the family function, dynamics, and relationships is paramount (
). Family discord can lead to psychosocial problems in these teens, which, for these patients, can spiral into nonadherence. The PNP-PC can help adherence in these patients by encouraging poorly functioning families to seek out counseling.
Risk-Seeking Behaviors
Adolescence is a time of rebellion, confusion, ambiguity, and risk-taking behaviors for any teenager, let alone one with a complex medical condition. Puberty and hormones have been hypothesized to be responsible for the increase in risk seeking behaviors; however,
suggest that asynchrony in the maturation of the brain structures and function may be linked to the influences of puberty on development and risk taking.
Medication nonadherence can be a different challenge in adolescents who have a history of complex congenital heart disease, especially those who have been restricted and hospitalized frequently throughout their lives and now feel a newfound sense of freedom (
). Feeling full of energy and healthy for the first time can enhance the feeling of invincibility that is pervasive throughout adolescence, encouraging risk-taking behaviors such as drugs, alcohol, and nonadherence to medications or follow-up visits. Adolescents struggle with the realization that they traded a life-threatening illness for a chronic condition instead of the desired pristine health. These patients now feel physically better yet continue to require daily medications and regular doctor appointments and are now immunosuppressed (
Adolescents struggle with the realization that they traded a life-threatening illness for a chronic condition instead of the desired pristine health.
Adolescents continue to have inferior long-term outcomes from heart transplantation compared with younger recipients, which is believed to be partly related to nonadherence to medications (
). Risk-seeking behaviors in this age group are not simply manifestations of teen angst but dangerous, graft-threatening behaviors.
Body Image Struggles
Adolescence is a time when teenagers seek to accept their changing body image and struggle with burgeoning sexuality. For the adolescent organ transplant recipient, the physiologic manifestations of his or her immunosuppressive medications are of vital importance. Hirsutism, gingival hyperplasia, weight gain, and short stature are all adverse effects of the posttransplantation immunosuppressive regimen and can cause adolescents to become nonadherent with medications (
). Teens may either not understand the risk of being nonadherent with their medications or, at the time, may weigh their physical appearances as being more important than medication adherence. The feeling of looking different is in direct conflict with the adolescent desire for normalcy and fitting in with their peers (
Additionally, antihypertensive medications, which are often used in heart transplant recipients, may cause sexual adverse effects (namely, impotence), and these can be perceived as devastating to the male adolescent (
Being open to discussing the issues with patients and working to ameliorate their concerns regarding their appearances or sexual functions will help adolescents feel a sense of control over changing that which is concerning to them. Hair removal techniques, approved acne medications, exercise suggestions, and referrals to a psychologist are ways in which the primary care provider can provide aid to teenage patients (
Adolescent brains are not the same as adult brains because they are not yet entirely mature. The prefrontal cortex does not fully mature until the age of 25 years (
). Adolescents are notably prone to nonadherence with medical prescriptions and medical advice, which is due in part to the developmentally appropriate questioning of authority and need to make their own decisions (
). Executive functioning is a neuropsychological term that includes many of the more advanced cognitive skills such as organization, planning, and problem solving (
showed that adolescents and young adult solid organ transplant recipients have significantly greater levels of dysfunction in total executive functioning and metacognitive abilities compared with the normal reference scores of healthy peers. Caring for themselves as young adults will require organization and planning skills to maintain their complex medical regimens, and thus, deficits in overall executive functioning can introduce significant challenges (
). The PNP-PC may consider neurocognitive testing if the deficits in executive functioning are becoming a barrier to the patient's adherence.
Transitioning to Adulthood and Independence
A common theme that has emerged is the understanding that the teens can and need to take responsibility for medication regimens. However, this shift to self-care can be a stressor in the parent–adolescent relationship as parents struggle with trusting their adolescents to manage their medications autonomously (
). Parents must be counseled at the start of preadolescence to begin to allow teens to take more ownership of their medications and medical management. Family therapy or counseling can help smooth this transition and enhance family function.
Parents must be counseled at the start of preadolescence to begin to allow teens to take more ownership of their medications and medical management.
Confusion can arise surrounding who is now responsible for medication adherence between the adolescent and parent (
). A slow, gradual change from parent-directed medical management to adolescent self-management is ideal yet can be fraught with difficulties. This evolution of care can be intimidating and frightening to both parents and adolescents. However, a child who is given or who takes on none of the responsibility for his or her care until adolescence is more likely to see the regimen as something imposed by adults and is far more likely to resist, rebel, or become nonadherent (
showed that teens who readily accepted ownership (and the parents who permitted them to do so) and incorporated their medication regimen into daily life were more successful. Starting the transition to self-care must begin in early adolescence; however, if the patient is pushed into fully caring for himself/herself before the brain is mature with well-developed executive functioning, he or she could be set up for failure. A delicate balance is needed with extensive support for both the teen and his/her family throughout this time.
Improving Adherence
Nonadherence is a problem for documented adolescent heart transplant recipients (
found that teenagers wanted more occasions to network socially with other adolescents who had gone through transplantation in their usability testing of the first three modules of their Internet program “Teens Taking Charge: Managing My Transplant Online.” All 21 adolescent transplantation patients who participated in this testing enjoyed the videos of other patients discussing their experiences (
). Online or in-person support groups conducted specifically for adolescents who have undergone transplantation should be coordinated by all transplantation centers to provide a built-in support network for these patients. PNP-PCs should encourage patients and families to participate in such opportunities and aid them in seeking programs out when needed.
Smart Phone Use
With the increasing popularity of smartphones, it is not surprising that 75% of teens use text messaging, with most older adolescents sending more than 100 text messages a day (
). Because of the popularity of smart phones among the adolescent population, applications that send text reminders to take medications at a specified time are gaining popularity (
Text message reminder systems and mobile health applications have been shown to improve medication compliance among adolescents who have undergone solid organ transplantation (
). For a more comprehensive list of apps, programs, and Web sites to help adolescents and their parents manage complex medical problems in the age of technology, see Table 1, Table 2.
Organ Transplants: A Survival Guide for the Entire Family (It Happened to Me) by Tina P. Schwartz
A book with real-life stories of teens whose parents, siblings, or other family members are transplant recipients, as well as teens who have received transplants themselves.
found that teens preferred the anonymity of online communication to face-to-face support groups. The PNP-PC should assess the preferred method of communication and note that some teens may prefer e-mail, online chat rooms, or online education.
Group Visits
Seeing multiple patients with the same chronic disease condition at the same time in a group setting (with separate private physical examinations) is a method that has recently been investigated in this specific patient population. Group visits can be conducted monthly, bimonthly, or biannually depending on the needs of the specific group of patients. As an example, each visit could begin with individual laboratory work and physical examinations and then be followed by a 1-hour group session led by a nurse practitioner or physician for a period of open discussion or education. Although previous studies had shown that the group visit method can successfully be applied to primary care pediatrics and a plethora of other chronic disease conditions, to our knowledge, no studies have been done on the pediatric heart transplantation population (
recently showed that this method can effectively be applied to the pediatric heart transplantation population with a high level of patient and family satisfaction, increased face time with providers, and exceptional retention of the content discussed.
Improved Care Transition
Care transition in this patient population can refer to two different transitions; the transition to an adult health care provider and the transition to self-management.
Transitioning from a pediatric provider to an adult provider
This transition is difficult and is a period that can exacerbate or trigger nonadherence. Pediatric patients become comfortable with their providers as a safety net and may have been followed by their transplant team and PNP-PC since diagnosis (which may have been in infancy).
showed that having an organized clinical process and transition readiness assessments throughout late adolescence helped set the patients up for success. Although this transition is inevitable and necessary, it must be done slowly and gradually to ensure that a patient feels comfortable with his or her new medical team. When a patient is closely followed during this transition and guided through this change, the transition is more likely to be smooth and controlled, with minimal to no nonadherence (
Adolescents must begin to learn to be the primary manager of their complex medical regimen. The transition to self-management is an active, daily, oscillating process in which teens begin to assume control over their health and well-being, including chronic disease management (
). Pre-teens and adolescents can be encouraged to take ownership of their transplant by carrying a personalized informational wallet card containing information about their transplant (refer to Figure 1). Adolescent patients' level of executive functioning (planning and organization) may be impaired (
). The processes governed by executive function are not limited to taking medications, but also entail timing of refills, communication with insurance companies, scheduling of surveillance biopsies, and maintaining prescribed laboratory schedules. An adolescent patient should be monitored during transition at first and have multiple resources to contact if he or she has questions.
Role of the PNP-PC
Primary care providers must not only understand why adherence problems exist in this patient population but must play a significant role in improving compliance in these adolescents as the established medical provider and longstanding care coordinator. The PNP-PC must address all aspects of non-adherence in order to be comprehensive in their care of these patients (see Figure 2).
Clinical
Care coordination and follow-ups
Maintaining an open line of communication with a patient's transplantation team and other members of the care team is key to helping a patient and family manage what is typically a plethora of appointments, follow-ups, and laboratory draws. Making sure to set aside time with a family at every annual visit to discuss and lay out a patient's follow-up schedule with all of their specialists is a way the PNP-PC can minimize confusion, stress, and ultimately nonadherence. Helping organize a schedule of appointments, offering support, and identifying any potential barriers to compliance is part of creating a medical home and aiding in care coordination.
supported the idea that developmental maturity was directly related to adherence among adolescent and young adult heart transplant recipients. Overall, there was a sharp dichotomy between the good adherers and the poor adherers along the themes of developmental age and relationships (
). Maturity is an ongoing process that must be continually reassessed, and the education of a specific patient must be tailored appropriately.
PNP-PCs can aid families by performing developmental and maturity assessments not only to help guide the transition to self-management but to identify those patients at risk for nonadherence during the transition because of their developmental status. Health care providers can gain valuable information about their adolescent and young adult patients' neurocognitive functions and developmental statuses by assessing executive functioning. This can direct the guidance given to families and patients about transitioning adolescents to self-care and the appropriate levels of responsibility these patients should be able to assume.
Screening for depression and substance abuse
Depression in adolescent cardiac transplantation patients is a real concern. A chronic illness can lead to anger, sadness, and frustration, all of which can have negative effects on medication adherence (
American Academy of Pediatrics, Committee on Practice and Ambulatory Medicine & Bright Futures Periodicity Schedule Workgroup 2016 Recommendations for preventive pediatric health care.
if not at every visit. Early identification and appropriate mental health referrals and care are key in preventing depression-related nonadherence in this population.
Implementing group visits
The group visit method can be suggested to the patient's transplantation office. Additionally, if the PNP-PC has more than one adolescent transplant patient in his or her practice, this method could be used for primary care visits.
Education of the Patient and Family
Before transplantation
Transplant education, including the importance of adherence, must begin before transplantation, during the listing process. Understandable and comprehensive educational material should be provided to the family and patient during the listing process. Special care should be taken to ensure that both family and patient (if age appropriate) understand the lifelong commitment that is required after receiving an organ transplant. Transplantation centers may have their own educational information, and many centers in the United States and Canada use a comprehensive educational booklet from the Pediatric Heart Transplant Study (
). Primary care providers should discuss the commitment and evaluate the level of understanding of a patient and his or her family, if possible, before a transplantation occurs.
After transplantation
Immediately after transplantation is a time for healing. However, it is important at this stage that the transplantation team have significant continued education for the patient and family on the medications and follow-up processes before discharge from the hospital. Seeing a patient for the first primary care appointment after transplantation is the optimal time to have a detailed discussions about the medication, follow-up schedule, lifestyle recommendations, etc.
Continuing and age appropriate
Typically, much of the effort for education is focused on the pretransplantation and immediate posttransplantation period (
). However, continued age-appropriate education as the patient grows and matures should take place before the outside stressor of the adolescent needing to transition to self-care and adult providers (
). A family-centered educational program, consisting of videos and written material to be reviewed as a family at home, can significantly increase the knowledge level (
). A mature understanding of the purpose of their transplant, why medication adherence and timing is imperative, and how to meld their medical needs into their lives is key in lowering the level of nonadherence in teen heart transplant recipients.
Research
Much of the research performed in this area focuses on kidney or liver transplant recipients. More research is needed on nonadherence in heart transplant recipients specifically (
). Additionally, there is a scarcity of research on interventions to increase adherence in this age group, which is where research should be directed on this topic (
). Cell phone applications and use of smartphones in helping to aid compliance in this specific population needs continued development and research into their ease of usability and effectiveness in reducing nonadherence.
Conclusion
Noncompliance in teenage heart transplant recipients continues to be the leading reason for graft failure in this age group; adolescent nonadherence outpaces that of any other age group (
). By incorporating patient education, screening tools and group visits and by providing age-appropriate resources for our patients, PNP-PCs can use this knowledge to help reduce the hurdles adolescents encounter in living with this chronic condition. An individualized approach to each patient, taking into consideration their maturity level, need for education and executive functioning, as well as their likelihood to respond positively to mobile health initiatives or support groups, can easily be implemented into the practice setting. This multifaceted approach can enhance the practitioner's ability to engage patients and work collaboratively toward the goal of adolescent adherence and overall improved quality of life.
Figure 1Sample of a wallet card teens can personalize.
The Registry of the International Society for Heart and Lung Transplantation: Sixteenth Official Pediatric Heart Transplantation Report—2013; Focus Theme: Age.
The Journal of Heart and Lung Transplantation.2013; 32: 979-988
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