The Case

A 21-year-old woman with a history of Marfan syndrome complicated by aortic root dilation presented to the emergency department with abdominal pain and was found to be pregnant. This was her second pregnancy, as she had had a therapeutic abortion 4 years previously. At that time, the patient was being followed by a pediatrician and a pediatric cardiologist. They had advised her of the high-risk nature of the pregnancy, including a 10% chance that she could have an aortic rupture during pregnancy. She decided to terminate the pregnancy and was advised that she should undergo surgical repair of her aortic root in the near future. Shortly thereafter, she turned 18 years old and was referred to an adult primary care physician and cardiologist for continued care. The patient never saw her new physicians and was never scheduled for the recommended cardiac surgery procedure. In fact, over the next 3 years she did not receive regular follow-up care at all, although she remained relatively healthy. She also never received pre-conception counseling or contraception, despite the risk to her health should she become pregnant.

Upon learning of the current pregnancy, the patient decided against another abortion. She was evaluated by adult cardiology and cardiac surgery, and she was advised to undergo an aortic root repair during her second trimester in order to minimize potential harm to the fetus while preserving her own health. Understandably, this decision caused significant anxiety for the patient and her family. She did undergo the procedure without complications and proceeded to have a healthy child at full term.

The Commentary

Nationally, approximately 20% of children under 18 years old, or an estimated 12.8 million children, have a chronic medical condition.(1) Each year, more than 750,000 of these children reach the age of majority (generally considered age 18–19).(2) This population includes children with chronic conditions including asthma, autism, congenital heart disease, cancer survivorship, cystic fibrosis, developmental delay, diabetes, and spina bifida. Medical advancements over the last 50 years have led to a significant increase in the lifespan of many children who would previously have died in childhood. However, without long-term management to ensure health and wellness, young adults with chronic medical conditions can experience serious, even catastrophic consequences, as with the patient in this vignette who required urgent surgery.(3)

In order to reduce the high morbidity and address the developmental needs of patients in adolescence and early adulthood, young adults with chronic conditions need to be formally transitioned from pediatric to adult-focused care.(4) This health care transition is formally defined by Blum as "the purposeful, planned movement of adolescents and young adults with chronic physical and medical conditions from child-centered to adult-oriented health-care systems."(5) An important distinction is that transition is not transfer of care. The transfer from pediatric to adult care is, indeed, a point in the transition process, but it takes years of preparation for young adult patients to become fully independent in their medical care. In this vignette, there were failures of two processes: the process of transitioning the patient to prepare her for the future as an adult with congenital heart disease, and the process of transfer to ensure that the woman's care would be monitored to minimize poor outcomes.

This clinical vignette focuses on Marfan syndrome and congenital heart disease. Both the Working Group on research in adult congenital heart disease and the American Heart Association have generated recommendations for management and monitoring of adults with congenital heart disease.(6,7) Various medical societies have advocated that the transfer-of-care process start at age 12–14, and that the transfer process include the identification of the receiving provider as well as communication between pediatric and adult health care teams prior to transfer.(5,8) Applying these recommendations to this vignette, the discussion—regarding planning for future outcomes (such as understanding management for aortic dilation) and family planning—should have been initiated early in the life of the patient. The need for surgical repair was discussed, but there was no follow-up on prevention or steps to take in the event that the patient became pregnant. This patient needed her transfer of care planned and coordinated with the receiving adult provider in order to ensure that the recommended cardiac monitoring was achieved. While the recommendations made by various disease-specific organizations have disease-specific concerns and guidelines (Table), the elements of transition across disease conditions are not dissimilar. These include measures to:

• Ensure the patients have knowledge and understanding of their disease.
• Make sure the patients know how to manage their disease and understand future contingencies for which they need to prepare. This knowledge includes developing self-advocacy skills, learning to interact with adult health care providers, maintaining insurance, and planning on how the disease will affect future family planning, schooling, and vocational activities. For those with developmental disabilities, future planning will include designating who will be primarily responsible for the patient's health care needs. These activities can be operationalized through the development of a written transition plan.
• Evaluate patients' transition preparedness. This can be done by assessing their understanding of their diseases and their own self-efficacy in disease management, which can be facilitated through the use of available transition tools.(9,10)
• Prepare patients and their parents for the differences between pediatric and adult health care (in terms of expectations from patients as well as their providers). This preparation includes discussion of the changing role of the parents during the transfer process. Activities such as developing "medical passports" (11,12) or a "portable medical summary" (13) can be helpful in this area. For those patients whose parents will need to be their primary caretakers, legal processes for power of attorney or medical proxy need to be discussed.
• Ensure that the disease care and preparation during the transfer process are actively coordinated and discussed between the referring pediatric provider and the receiving adult provider.
• Make sure transfer to adult care is completed. Clinics need to develop systems to monitor and follow up (either via case management or transition navigator) on failures to transfer. The goal is to attain a seamless transfer of care and proper follow-up with accountability by the medical teams.

A unique challenge to this type of transition of care is the cultural dissimilarity and resource differences between pediatric and adult systems. In pediatrics, the parents are the focus in the patient decision-making process, whereas in the adult heath care world, the decision-making is focused on the patient, and not necessarily the family. This shift in focus can be daunting for young adults; indeed, many are not prepared for this shift in responsibility. In addition, pediatric specialty- or primary care-based medical homes tend to be better resourced, which allows for extensive multidisciplinary care in a singular location, a situation that is often not mirrored in the adult care system. Consequently, young adults must be much more active in their own case management when they transfer to the adult care system. This change emphasizes the need for including pediatric patients early in decision-making and ensuring they know how to handle their own care management to ensure a smooth transfer of care.

Quality improvement in transitions starts with the understanding that adolescents and young adults with chronic disease need to acquire general life skills as well as disease-management skills. Many medical societies have made disease-specific recommendations readily available (Table).(4) For example, programs have initiated transition navigation programs, education programs, and shared care model systems.(14-16) Success of these programs has been measured by improved biometric markers, screening, better follow-up rates, and decreased acute or chronic disease complications.(17) In addition, national agencies, such as Got Transition (13), have materials for providers, families, and youth that can assist in the transition-of-care quality improvement process.(9) Clinics have employed mechanisms to improve transition-of-care processes through the use of Plan-Do-Study-Act (PDSA) cycles.(18,19)

PDSA cycles can be used to implement and monitor processes of care. For example, if a clinic wished to address the issue of transfer in this vignette, the clinic could have a follow-up call built in to the scheduling (Plan), then call the patient in 3 months after the last visit (Do). This follow-up would have alerted the pediatrician or pediatric cardiologist that no follow-up appointment had been made (which would indicate a failure of transfer). The clinic could then have started tracking those who did and did not transfer within a set time period (Study); this tracking, in turn, would help identify barriers to transfer, such as receipt of disease instruction, receipt of transition planning, or provider access. This data could then help the clinic decide what other steps were needed, such as passport generation (Act), which then leads back to planning on implementation and repeating the PDSA cycle as part of continuous quality improvement. Outcomes for transition, such as transition readiness scores (e.g., Transition Readiness Assessment Questionnaire or TRAQ [10]), transfer status, disease-specific health outcomes, and perceived quality of life can have been measured as part of the transition quality improvement process.(4)

The ultimate responsibility of transfer ideally would be a shared responsibility between pediatric and adult providers. The responsibilities of the pediatric program are to prepare, provide summary information, and to seek acceptance by a qualified and available adult provider. The adult provider ideally would assist with patient access needs (adult programs and insurance navigation) and inform the pediatric referring provider of completion of the first visit. Using quality improvement techniques to implement clinical improvements in care in transitions would likely prevent the case scenario described in this vignette.

Take-Home Points

• The process of preparing or transitioning a youth with a chronic condition for the eventual transfer to adult health care can take years. It is critical that patients learn how to care for their condition while learning how to navigate the complexities of the health care system over the entirety of their lifespan.
• Many youth may not understand that a surgical repair of a congenital anomaly or that a past treatment (such as with chemotherapy) requires maintenance to sustain continued health and wellness.
• Ensuring continuity of care is critical; part of this is accomplished by helping patients to understand the importance of regular health care visits. Transfer completion happens with a successful visit with the adult provider—not with the identification of an adult provider.
• A variety of quality improvement techniques and tools are available to help aid clinics and hospitals in creating more seamless transitions from the pediatric to the adult health care world.

Megumi J. Okumura, MD, MAS Assistant Professor Division of General Pediatrics Division of General Internal Medicine University of California, San Francisco

Roberta G. Williams, MD Professor and Past Chair Department of Pediatrics Keck School of Medicine University of Southern California Children's Hospital Los Angeles

References

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4. McPheeters M, Davis AM, Taylor JL, Brown RF, Potter SA, Epstein RA. Transition Care for Children With Special Health Needs. Rockville, MD: Agency for Healthcare Research and Quality; June 2014. AHRQ Publication No. 14-EHC027-EF. [Available at]

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8. American Academy of Pediatrics; American Academy of Family Physicians; American College of Physicians-American Society of Internal Medicine. A consensus statement on health care transitions for young adults with special health care needs. Pediatrics. 2002;110(6 Pt 2):1304-1306. [go to PubMed]

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10. Sawicki GS, Lukens-Bull K, Yin X, et al. Measuring the transition readiness of youth with special healthcare needs: validation of the TRAQ—Transition Readiness Assessment Questionnaire. J Pediatr Psychol. 2011;36:160-171. [go to PubMed]

11. von Scheven E. Transitioning Adolescent Patients (TAP) from Pediatric to Adult Care; 2012. [Available at]

12. MyHealth Passport. Toronto, Canada: The Hospital for Sick Children. [Available at]

13. Got Transition. Washington, DC: The National Alliance to Advance Adolescent Health. [Available at]

14. Kipps S, Bahu T, Ong K, et al. Current methods of transfer of young people with Type 1 diabetes to adult services. Diabet Med. 2002;19:649-654. [go to PubMed]

15. Okumura MJ, Ong T, Dawson D, et al. Improving transition from paediatric to adult cystic fibrosis care: programme implementation and evaluation. BMJ Qual Saf. 2014;(suppl 1):i64-i72. [go to PubMed]

16. Van Walleghem N, Macdonald CA, Dean HJ. Evaluation of a systems navigator model for transition from pediatric to adult care for young adults with type 1 diabetes. Diabetes Care. 2008;31:1529-1530. [go to PubMed]

17. Langley GJ, Moen R, Nolan KM, Nolan TW, Norman CL, Provost LP. The Improvement Guide: A Practical Approach to Enhancing Organizational Performance. 2nd ed. San Francisco, CA: Jossey-Bass; 2009. ISBN: 9780470192412.

18. Science of Improvement: Testing Changes. Cambridge, MA: Institute for Healthcare Improvement; 2015. [Available at]

19. Langley GL. The improvement guide: a practical approach to enhancing organizational performance. 2nd ed. San Francisco, CA: Jossey-Bass; 2009.

Table

Table. Consensus Statements for Transition Care.(4)