APHON/ASPHO Joint Position Paper
The transition of Patients with Sickle Cell Disease from Pediatric to Adult Health Care
Health care transition involves the process of changing from a pediatric-focused to an adult-focused model of care with or without transfer from a pediatric to an adult provider. As the professional voice of pediatric hematology/oncology healthcare practice, the Association of Pediatric Hematology/Oncology Nurses (ASPHON) and the American Society of Pediatric Hematology/Oncology (ASPHO) recommend the following principles: that the process of transition from pediatric to adult care begin early and be presented as part of the natural process of becoming an adult; that patients, providers, and families are all involved in creating a transition plan and assessing transition preparedness annually; that the transfer of care involves direct communication between the pediatric team and the accepting adult providers; and that adult services focus on helping young adults integrate into the adult model of care.
Sickle cell disease (SCD) refers to a group of inherited blood disorders affecting approximately 100,000 individuals in the United States and millions worldwide (Sedrak & Kondamudi, 2020; Wastnedge et al., 2018). Complications of SCD result from hemolysis and vaso-occlusion which cause acute and chronic multisystem complications leading to significant morbidity and mortality (National Heart, Lung, and Blood Institute [NHLBI], 2017). With improved medical interventions such as universal newborn screening, prophylactic antibiotics, targeted immunizations, and optimization of hydroxyurea (Lanzkron et al., 2013), childhood mortality has declined and over 90% of children with SCD in the US now survive to adulthood (Quinn et al., 2010). Despite these advances, there is little improvement in adult outcomes (Lanzkron et al., 2013).
The time of transition between pediatric and adult care is a high-risk period with increased rates of acute care utilization, lack of access to preventative care, and risk of early mortality (Blinder et al., 2013; Hemker et al., 2011). Factors contributing to these poor outcomes are systems factors that include the lack of continuity of care, disease-specific comorbidities such as cognitive impairment due to cerebrovascular complications, and psychosocial vulnerabilities inherent to adolescent development (Crosby et al., 2015). It is imperative that systems caring for adolescents and young adults (AYA) with SCD provide structured transition support for this vulnerable population.
In this position paper, we address the transition of care from a pediatric-oriented healthcare system to an adult-oriented health care system with or without a change in provider. Transition support goes beyond coordination of transfer between different locations or levels of care; it encompasses self-management support and assistance with reaching educational and vocational milestones. Help may also be needed to avoid loss of insurance coverage and promoting access to specialty care for young adults (Sawicki et al., 2017).
Adolescents and young adults with SCD may face an additional barrier to transition due to cerebrovascular complications of the disease which can include stroke, silent cerebral infarctions, and cognitive impairment (Strouse, 2016). In some cases, patients may require decision making support from a third party through guardianship or a power of attorney (American Academy of Pediatrics [AAP], American Academy of Family Physicians [AAFP], American College Physicians [ACP], & Transitions Clinical Report Authoring Group [TCRAG], 2011). It is incumbent upon the pediatric team to introduce a dialogue about transition early and identify and address any barriers prior to the transfer of care.
Health care transition is defined as a ‘purposeful, planned’ process with a goal of maximizing ‘lifelong functioning and potential through the provision of high quality, developmentally appropriate healthcare services that continue uninterrupted as the individual moves from adolescence to adulthood’ (AAP et al., 2011; Sable et al., 2011). While transfer from pediatric to adult providers is often a component of transition, the process must start well before transfer and continue until the young adult is well established in an adult-focused system of care (DeBaun & Telfair, 2012; Saulsberry et al., 2019). Early transition preparation assists with reducing gaps in care by ensuring that evidence-based routine health care maintenance continues uninterrupted.
Despite recognizing the importance of transition as part of the continuum of healthcare for AYA with SCD, a paucity of established, effective strategies exist. Barriers to transition support include limited staff training, lack of identified personnel responsible for transition, and financial limitations (Lebensburger et al., 2012). Professional organizations such as APHON and ASPHO, which represent providers caring for these AYA patients, should advocate for better reimbursement for transition planning and services.
Limited availability of adult providers with expertise in SCD is another barrier to a smooth transition (Lebensburger, 2015). Pediatric providers should identify specialists or interested generalists in their area. Adult primary care providers can use resources such as the NHLBI’s evidence-based guidelines or take advantage of regional learning consortiums to remain up to date on current treatment recommendations.
It is the position of APHON and ASPHO that programs caring for AYA with SCD must provide transition preparation focused on helping patients become independent in managing their disease and integrating into an adult-focused model of care. The Center for Health Care Transition Improvement proposes using six core elements of transition as a framework to guide best practices. These include the following: developing a health care transition policy, identifying transitioning youth and maintaining a transition registry, assessing and tracking transition readiness, addressing healthcare transition needs with the youth and family, ensuring direct communication between pediatric and adult providers during any transfer of care, and continuing contact with the young adult to ensure successful completion of transition (Got Transition, 2014).
- Transition Policy
- Formal discussion about transition should begin at age 12 (or when developmentally ready) and include both the patient and their family (Porter et al., 2014).
- The practice or institution should have a clear transition policy that is accessible to providers, patients and families (e.g., posted on the practice website, in exam rooms, or mailed to families)
- Whether or not transfer of care will occur, the transition discussions should include details about changes in privacy when the patient turns 18 and any shift to an adult model of care (e.g., expectation that appointments will be scheduled by patients not their parents) (AAP et al., 2011).
- The transition planning team should be multidisciplinary and include physicians, advanced practice providers, nurses, psychologists and social workers from both pediatric and adult care settings (Jordan et al., 2013).
- Pediatric providers should allow patients and caregivers to express feelings and concerns around transition (e.g., fears about transferring to a new provider) and include family members in the transition plan.
- Tracking and Monitoring
- Every patient should have an individualized transition plan documented by age 14 and updated annually (AAP, 2015).
- Each institution should have a way to identify and track transition aged patients and ensure that planning is done and documented. Integration into the electronic medical record can help improve transition planning (Sharma et al., 2018).
- Transition Readiness
- Patient’s should have an assessment of their readiness for transition performed regularly using either a generic or a SCD specific tool (Treadwell et al., 2015). Use of these tools can guide patients specific transition planning and determine the appropriate timing of transfer of care.
- Patients should have neuropsychological evaluations done to identify deficits that may impact transition readiness (Daly et al., 2011).
- Transition Planning
- Consensus statements recommend that transition preparation should focus on improved patient-provider communication and increased patient responsibility for disease self-management (AAP et al., 2011).
- Transition preparation should be addressed annually and include medical topics (SCD complications, disease inheritance) as well as educational and vocational topics.
- Pediatric providers should directly address insurance issues and any changes in insurance coverage due to age.
- Nursing staff can facilitate transition by assessing preparedness, resources, relationships and responsibilities as part of the individual transition plan (Fegran et al., 2014).
- Providers caring for patients with SCD can normalize the transition process by helping families understand that transition to an adult-focused system of care is a normal part of development (Mahan et al., 2017).
- Practices should initiate an ‘adult’ model of care around the adolescent period by seeing patients alone for part of the visit, directing questions to the adolescent during the visits, and as they get close to age 18 encouraging patients to call for appointments and medication refills.
- Transfer of Care
- Identify adult providers in your area with expertise or interest in SCD.
- If transition includes a transfer of care, have the family meet their adult provider prior to transfer as this may improve access and adherence to adult care (Bloom et al., 2012; Hankins et al., 2012).
- Consider staggering transfer so patients are not changing primary care and subspeciality providers at the same time.
- Transfer should occur when the patient, family and providers agree that the patient is ready to take on an adult role in their care. If the hospital has a set age for transfer, work to develop a policy exception for patients with cognitive delay or other special needs.
- Transfer should be planned; avoid abruptly transferring patients such as during an unplanned hospital admission or pregnancy.
- Pediatric providers should communicate directly with the adult provider and send a written medical summary that includes medical history, an individualized pain management plan, and baseline labs.
- Transfer Completion
- Transfer is not complete until the patient has been seen at least once in the adult providers office, which should happen within 3 months of leaving pediatric care.
- Pediatric providers should contact AYA to confirm attendance at the first adult visit and follow-up with the adult practice to confirm completion of transfer (AAP et al., 2011). Consultation can be provided as needed.
- Pediatric and adult teams should continue to evaluate and adjust their transition process. This evaluation should include the perspective of patients, families and all involved providers.
All AYA patients with SCD should receive transition preparation to help them maneuver from pediatric- to adult-focused health care. This allows for greater independence and the development of self-management skills whether or not they transfer providers. Transition education before, during and after transfer of care may assist in reducing morbidity and the high rate of mortality from SCD complications. Transition readiness should be assessed regularly as the move from pediatric- to adult-focused care is a maturational process that occurs over time rather than through a single event. Pediatric and adult care teams should work together and continuously evaluate their transition processes.
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