Sickle cell disease (SCD) in adolescents and young adults (AYA): Transition from pediatric to adult care

INTRODUCTION — Pediatric to adult life transition is a critical time requiring continued support to ensure continuity of care. There is a clear need for a structured process of transition from pediatric to adult care for youth with sickle cell disease (SCD) that is not based on age but rather on social and emotional development and achievement of milestones in disease-management knowledge and skills.

This topic describes standards, our approach to planning and carrying out health care transition in SCD, and how national guidelines and frameworks can guide this process.

Related issues are discussed in separate topic reviews:

●SCD management – (See "Sickle cell disease in infancy and childhood: Routine health care maintenance and anticipatory guidance" and "Overview of the management and prognosis of sickle cell disease".)

●Confidentiality and consent in adolescent medical care – (See "Confidentiality in adolescent health care" and "Consent in adolescent health care".)

DEFINITIONS — Health care transition is defined as "the purposeful, planned movement of adolescents and young adults (AYA) with chronic physical and medical conditions from child-centered to adult-oriented health care systems" [1].

Health care transition comprises three phases [2]:

●Transition preparation

●Transfer

●Adult care integration

Transfer comprises the act of changing from a pediatric to an adult-centered care model, while transition refers to the entire period of preparation, transfer, and integration into adult care.

Guidelines and standards are discussed below. (See 'National guidelines and frameworks for health care transition' below.)

HEALTH CARE TRANSITION AS A VULNERABLE TIME

Morbidity and mortality risks — Survival for children with SCD has substantially improved in middle- and high-income countries (HICs) in recent decades [3,4]; survival is also improving in low-income countries [5]. This improvement in survival translates into a change in the disease paradigm, which today can be considered a chronic disease in which most children in middle- and high-income countries are expected to survive to adulthood.

However, as adolescents and young adults (AYAs) with SCD transition from pediatric to adult care, the stakes for their health and even survival are high. The timing of this transition often coincides with other life and health changes that contribute to their vulnerability, including:

●Multiple comorbidities develop, including worse mental health problems, and an increase in disease severity ensue [6-10].

●Mortality rises following adult care transfer [3,11-14]. Lifespan is shortened by approximately 20 to 40 years compared with race-matched counterparts [15,16].

●AYAs learn to assume responsibility for their own care.

●Use of emergency department and inpatient resources increase and may exacerbate disease complications.

●Systems of inequities intensify, including structural and interpersonally mediated racism and stigmatization [17].

Increased costs and acute care utilization — In large administrative claims analyses using Medicaid data and the Nationwide Readmissions Database, including children through adults, complications and health care costs increased significantly in AYAs, while the use of preventive therapies such as hydroxyurea prescription and transfusions decreased after age 18 [11,18].

●An examination of administrative data over a nine-year period showed that costs increased for AYAs with SCD (ages 18 to 21 years) compared with younger adolescents (ages 13 to 17 years) hospitalized in pediatric settings [19].

●In an analysis of a different administrative dataset, 1189 adolescents ages 16 to 18 years experienced an average of 3.9 severe vaso-occlusive pain episodes, and 725 patients (61 percent) had chronic complications during one year of follow-up, including pulmonary disease in 369 (31 percent) [9]. Health care utilization was high, with 2.0 hospital admissions, 3.4 emergency department visits, and 5.0 office visits.

●An analysis from the Nationwide Inpatient Sample database of 37,344,532 hospital encounters of patients with and without SCD, ages 16 to 24 years during the period from 2003 to 2017, hospitalizations increased with age [20]. With increasing age, AYA with SCD were more likely to die during their hospitalization than individuals without SCD.

SCD complications rise during the AYA period — Certain morbidities of SCD become more likely as individuals age. As a result, during the transition to adult care, increased monitoring for these conditions, along with patient education and possibly interventions to prevent or mitigate their progression, should occur. (See 'Health care transition interventions' below.)

Complications that worsen in emerging adults and deserve attention include the following; these are discussed in more detail in the linked topic reviews:

●Strokes and cognitive dysfunction – The incidence of ischemic stroke peaks within the first few years of life, and ischemic lesions continue to accrue as patients age. AYAs are more likely to experience hemorrhagic stroke. (See "Overview of the clinical manifestations of sickle cell disease", section on 'Neurologic complications' and "Prevention of stroke (initial or recurrent) in sickle cell disease", section on 'Individuals with silent cerebral infarctions'.)

●Kidney disease and hypertension – (See "Sickle cell disease effects on the kidney".)

●Heart disease – (See "Overview of the clinical manifestations of sickle cell disease", section on 'Cardiac complications'.)

●Pulmonary hypertension – (See "Pulmonary hypertension associated with sickle cell disease".)

●Avascular necrosis of joints – (See "Acute and chronic bone complications of sickle cell disease".)

●Chronic pain – (See "Disease-modifying therapies to prevent pain and other complications of sickle cell disease", section on 'Chronic pain'.)

●Transfusional iron overload – (See "Transfusion in sickle cell disease: Management of complications including iron overload", section on 'Excessive iron stores'.)

●Depression and depressive symptoms – (See "Overview of the clinical manifestations of sickle cell disease", section on 'Psychosocial issues'.)

A general approach to screening that includes testing in AYAs is also presented separately. (See "Overview of the management and prognosis of sickle cell disease", section on 'Routine evaluations and treatments'.)

NATIONAL GUIDELINES AND FRAMEWORKS FOR HEALTH CARE TRANSITION — National initiatives have been formed to guide the implementation and monitoring of health care transition programs.

There are frameworks for health care transition with overlapping elements.

●The standards set by the Association of Maternal and Child Health Programs are particularly useful for providers in adult health care settings as they prepare to receive and sustain the AYA in their practice. (See 'Association of Maternal and Child Health Programs' below.)

●Got Transition emphasizes the importance of monitoring and tracking progress at the population level. (See 'Got Transition' below.)

●The SICKLE recommendations are aimed at settings of any income level (low, middle, or high) and focus on individual-level variables that lend to successful transition from pediatric to adult life and care. (See 'SICKLE' below.)

Providers can use elements from each of the frameworks; they should be clear about how transition policies and practices align with each framework.

Association of Maternal and Child Health Programs — Transition to adulthood is one of the domains of the standards for systems of care for children and youth with special health care needs established by the Association of Maternal and Child Health Programs to improve their health quality and outcomes (AMCHP Standards v2.0) [21]. These are divided into four standards for pediatric care and four standards for adult health care settings.

●Pediatric health care setting

•Transition policy – Each institution should develop a policy for transition. Key components include informing patients and families/caregivers about differences related to privacy and consent and the age at which these changes apply (typically age 18 years). The policy should be developed with input from patients and family members/caregivers. Generally, discussions of the policy begin when the patient is approximately age 12 to 14 years, and the policy is regularly reviewed as part of ongoing care.

•Transition readiness – A standardized, scorable tool is used to determine youth's understanding of self-care and use of health care, initially administered with youth and caregivers at age 12 to 14 years and periodically reassessed by the pediatric provider.

•Transfer of care – Clinical documents should be available to the youth's new adult provider, including:

-Cover letter

-Final transition readiness assessment

-Updated plan of care

-Updated medical summary and emergency care plan

-If needed, legal documents, condition fact sheet, and additional provider records

•Transfer completion – Ensure communication with the adult provider, confirming transfer and offering pediatric consultation assistance as needed. The AYA/caregiver should be contacted to confirm transfer of care and elicit feedback on their experience with the transition process.

●Adult health care setting

•AYA transition and care policy – This is a policy/statement about the practice's approach to accepting and partnering with AYAs, developed with patient input and shared and discussed with the AYA at their first visit and regularly reviewed as part of ongoing care.

•Orientation to/integration into adult practice – Identify and list adult providers interested in caring for new AYA patients and develop a process for welcoming and orienting AYAs into the adult providers' practices. The system should document if the adult providers received the transfer documents.

•Initial visit – An initial visit plan includes:

-Addressing the AYA's concerns about transfer

-Clarifying adult approach to care

-Conducting a self-care assessment

-Reviewing the AYA's health priorities as part of a current plan of care

-Updating and sharing a medical summary and an emergency care plan

•Ongoing care – Communicate with pediatric practices regarding:

-Confirmation of the transfer into the adult practice

-Communication about when pediatric consultation is needed

-Assistance with connections to adult specialists and other support services

-Support for ongoing development of self-care skills and care management

-Feedback from the AYA about their experience with their health care

Got Transition — The National Alliance to Advance Adolescent Health, in partnership with the Maternal and Child Health Bureau, created the Got Transition/Center for Health Care Transition Improvement (https://gottransition.org/). Got Transition has defined the basic components of health care transition support through the development of the Six Core Elements of Health Care Transition. The Six Core Elements of Health Care Transition serve as a framework for new and existing programs and comprise (table 1):

●Creating a transition policy

●Tracking and monitoring progress

●Assessing transition readiness

●Planning for adult care

●Transferring to adult care

●Integration into adult care

The Six Core Elements have been tested and customized to different care settings. Guidance on how to implement these core elements can be found at https://www.gottransition.org/6ce/?how-to-implement [22]. Additionally, they are used as metrics for accreditation of a Patient-Centered Medical home [23].

SICKLE — The SICKLE recommendations are a list of six strategies and considerations that were outlined to guide the development of a transition plan for young people with SCD in 2020 [24]. They are aimed at settings of any income level (low, middle, or high). The SICKLE recommendations include the following, which create the acronym SICKLE:

●Skills transfer – Teaching the necessary transition skills (pain management, booking of appointments)

●Increasing self-efficacy – Increasing a patient's ability to manage their own disease

●Coordination of transition – Support by a trained community health worker or nurse navigator

●Knowledge transfer – Disease education; development of curriculum or handbook can facilitate this education

●Linking to adult services

●Evaluating readiness

SICKLE recommends mapping of all facilities offering specialist SCD care to facilitate referral to the nearest adult-centered service for patients in care at centers that offer pediatric care exclusively.

DEFINING A SUCCESSFUL TRANSITION — There is no established metric that defines successful health care transition in SCD. However, efforts at identifying quality metrics for pediatric-to-adult health care transition have produced a comprehensive framework for measurement that includes 54 measures grouped into four concepts [25]:

●Structure (infrastructure and capacities for health care transition)

●Process (clinician activities geared towards transition preparation)

●Outcome (care utilization, cost)

●Mediator (factors that facilitate or hinder the transition process, such as care coordination and patient characteristics)

While not yet empirically tested, this comprehensive framework can serve as a guide for developing measures to assess the quality of health care transition services.

Elements borrowed from other chronic conditions and measures derived from a survey of SCD experts can also inform indicators of successful transition [26]. These also include patient-level and program-level indicators [27,28].

Patient-level indicators

●Disease status – Measures of the patient's disease severity include chronic pain and end-organ dysfunction.

●Patient-centered outcomes [29]:

•Health-related quality of life and satisfaction with transition

•Social functioning

•Achieving important life milestones (postsecondary education, marriage, child rearing [if desired])

•Health literacy and disease-specific knowledge

•Self-efficacy (participation in preventive care, medication adherence, ability to call and schedule appointments)

•Satisfaction with transition

•Trust between adult providers and patients

AYAs with SCD have expressed that successful transition was characterized by increasing independence in the management of their disease with fading parental control [30].

The Center for Health Care Transition Improvement recommends direct assessment of the perspectives of young adults about the transition process, wherein they provide ratings of how prepared they felt for transition once transferred as well as ratings of how well elements of the transition process were executed within both the pediatric and adult health care realms [31]. A survey instrument is available online for making these assessments.

●Transition readiness – Transition readiness based on a self-management skills checklist (SMSC) or a transition intervention program-readiness for transition (TIP-RFT) assessment [32,33]. (See 'Readiness' below.)

●Health care utilization – Completion of a first visit to the adult provider within six months of leaving pediatric care, retention in adult care 12 and 24 months later, and frequency of emergency department or inpatient visits.

Program-level indicators — Program-level indicators address implementation of transition activities at the population level, such as in an outpatient SCD clinic.

As national standards for health care transition are incorporated into a transition program, metrics for their implementation should be used and monitored. (See 'National guidelines and frameworks for health care transition' above.)

As an example, if the Six Core Elements of Health Care Transition are chosen (table 1), guides to implement them are available and can be adopted using quality improvement methods [34]. Process measurement tools to quantify progress in implementing the Six Core Elements of Health Care Transition are also available [35]. (See 'Six core elements of health care transition' below.)

BARRIERS TO A SMOOTH TRANSITION

Limited number of capable providers for adults with SCD — A major challenge to the smooth transition from pediatric to adult care in SCD is the limited availability of knowledgeable adult-oriented providers and the resulting limited access to quality health care [36-39]. These may be hematologists or primary care providers who are willing to take on the complexities of SCD care.

●Unique requirements – The systems of care into which the AYAs are transitioned must be sensitive and responsive to the unique needs and challenges of AYAs with SCD. Care is complex because a number of prevalent adult SCD comorbid conditions begin in childhood; these include silent cerebral infarcts, avascular necrosis of joints, asthma, chronic sickle lung disease, and chronic kidney disease leading to kidney failure [40]. Without transition support and assured continuity of care, these conditions very likely may worsen and may seriously impact functional ability. (See 'SCD complications rise during the AYA period' above.)

Finding knowledgeable providers who can manage adults with chronic conditions that were previously deemed pediatric-only conditions has been shown to be problematic [2,39,41]. Accessing quality health care is particularly challenging for youth with SCD as they transition to adult health care. Pediatric SCD specialists reported that the inability to find an adult provider to whom they could transfer their patients was the most significant issue they faced in the transition process [37].

Providers do not merely need to be knowledgeable about medical aspects of SCD care; they also need to be capable of establishing a relationship of two-way trust with the patient, especially related to pain and its management.

•Pain is the hallmark manifestation of SCD, yet AYAs with SCD, who are primarily Black/African American in the United States, are often questioned by health care providers about their reports of pain and are labeled as drug seeking, manipulative, or malingering [42]. (See "Evaluation of acute pain in sickle cell disease", section on 'Clinical assessment of pain'.)

•Clinicians may be hesitant to prescribe or administer sufficient amounts of pain medications to manage severe pain episodes, creating major dissatisfaction among patients [43]. (See "Acute vaso-occlusive pain management in sickle cell disease".)

●SCD pain management and site of care – One of the most frequently cited barriers to accessing optimal care reported by AYAs in transition and adults with SCD is negative interactions with emergency department and primary care providers [42,44-47]. A hematology/oncology infusion center or dedicated day hospital care is preferred for acute, uncomplicated vaso-occlusive pain in individuals with SCD. (See "Acute vaso-occlusive pain management in sickle cell disease", section on 'Day hospital/infusion center'.)

This is supported by the following observations:

•Emergency department providers have been found to exhibit more negative attitudes towards patients with SCD compared with other providers, and emergency department providers with the highest levels of negative attitudes towards patients with SCD were less amenable to adhering to recommended pain-management guidelines [48].

•A study that compared pain management of individuals with SCD in the pediatric versus the adult emergency department at the same institution found significant differences in acute pain management, including longer wait until first analgesia and lower equianalgesic dose of the first opioid [49].

•In a prospective cohort study, the mean time to first dose of parenteral pain medication was 70 minutes shorter for adult patients with SCD in an infusion center when compared to those treated in the emergency department [50].

●Cycle of mistrust – As a cycle of mistrust develops between AYAs with SCD and providers, AYAs may be prone to non-adherence, self-discharge from the hospital, and avoidance of the health care system altogether, all of which can lead to serious health risks [45,46,51-53].

Additional evidence for the negative impact of these barriers to care include:

•In a 2021 report, pain-related injustice appraisals were associated with worse pain, greater pain catastrophizing, functional disability, and emotional distress for Black/African American youth with SCD [54].

•In Ghana, young adults described interpersonal and institutional stigma in relation to their pain experience, with detrimental consequences for their health-related quality of life [55].

Adults with SCD who are exposed to these persistent negative interactions with providers may experience a higher pain burden [52]. (See "Acute vaso-occlusive pain management in sickle cell disease", section on 'Provider misperceptions that interfere with the assessment'.)

Low transition readiness — AYAs with SCD report low readiness for transition [41,44]. In a survey about transition completed by 70 patients ages 14 to 20 years, the anticipated difficulty of transition was high, and mean readiness scores were low, with the greatest deficiencies in prior thought, knowledge, and interest [56]. (See 'Readiness' below.)

Other findings from this survey included [56]:

●Younger age (≤16 years) was associated with less knowledge and interest.

●Higher disease severity (≥3 painful episodes per year) was associated with lower interest but greater anticipated difficulty.

For AYAs with SCD transitioning from pediatric to adult care in the United States and United Kingdom, surveys conducted during or after the transition process have identified the following fears and concerns, which are common for patients and their families/caregivers [27,57,58]:

●Fear of leaving the health care provider with whom they were already familiar

●Fear that adult care providers might not understand their needs

●Mistrust in the medical setting

●Worries about costs of care and how they will be paid

●Feeling uninformed about and unprepared for the process

●Frustration if the transition is based upon age rather than readiness or needs

●Difficulties with employers and with keeping insurance

●Difficulties accessing follow-up care in the adult medical system

●Anticipation of stressful inter-personal relationships

●Family/caregiver concern about their AYA's ability to take responsibility for self-care and to cope successfully in the adult health care system

In a 2022 study of AYAs with SCD, barriers to care included lack of knowledge of self-care; inadequate caregiver support and competing life demands; limited provider knowledge, provider inexperience, poor provider-patient relationships, and lack of provider appreciation of the patients' SCD knowledge. Socio-environmental/institutional barriers included limited access to transportation, underinsurance, poor care coordination, and limited availability of SCD care providers/clinics [59]. (See 'Limited number of capable providers for adults with SCD' above.)

AYA interviewed in a qualitative study described similar themes, with additional concerns expressed about stigma [60].

As in highly resourced countries, adolescents and their families/caregivers in low- and middle-income countries may lack necessary knowledge and skills to be adequately prepared for transition; in settings where disease-related mortality in childhood has been very high, family members and caregivers may withhold information about SCD from their children [61].

Means of improving readiness are described below.

Cognitive disability and depression — Decreased cognitive functioning in SCD, whether associated with overt stroke, cerebral infarcts, or no previous neurocognitive findings, can negatively impact educational attainment and employment [62-64]. (See "Overview of the clinical manifestations of sickle cell disease", section on 'Neurologic complications' and "Prevention of stroke (initial or recurrent) in sickle cell disease", section on 'Individuals with silent cerebral infarctions'.)

●Cognitive disability – Reduced cognitive function can affect health literacy, coping, and resilience [65]. Problems with memory and executive function may be particularly frustrating for AYAs with SCD and their providers as the AYAs fail to problem solve, organize, and plan adequately for adherence with appointment keeping and other aspects of their medical regimen.

Lower full-scale intelligence quotient (IQ) and younger age are associated with inadequate health literacy [66]. In addition, AYA with lower IQ and lower verbal comprehension took longer to attend their first adult-based clinic visit, and those with attention difficulties were less likely to remain in the adult clinic system [67]. These results highlight the need to recognize this subgroup of AYAs as being at even higher risk of poor transition outcomes [68].

Deficits in processing speed, working memory, and perceptual reasoning are also associated with unemployment among adults with SCD [63]. In a 2023 study involving >2000 participants enrolled in the Sickle Cell Disease Implementation Consortium Registry, unemployment was associated with increased pain frequency [69]. Therefore, identifying deficits and referring for rehabilitative services is needed to maximize the chances of a smooth transition to adult, independent living. This practice is also endorsed by the American Society of Hematology guidelines for cerebrovascular disease in SCD [70].

●Depression – AYAs with SCD are also at risk for depression or depressive symptoms, which can further contribute to lower cognitive functioning [71]. Additionally, there is a link between chronic pain and depression in adolescents and adults with SCD, and both depression and chronic and recurrent pain are associated with functional impairments in performance at school and work, difficulties with social interactions, and increased use of health care resources [72-74]. For youth and adults with SCD, the chronic pain-depression burden can be severe [45,73,75,76].

Annual depression screening is recommended beginning at age 12 years so that early treatment can commence, given that a diagnosis of major depressive disorder in adolescence is strongly associated with recurrent depression in adulthood [77]. In lower resource settings, depression screening is equally critical, although access to psychological and psychiatric care is more limited [78]. (See "Screening tests in children and adolescents", section on 'Depression and suicide risk screening' and "Screening for depression in adults".)

PREDICTORS OF A SMOOTH HEALTH CARE TRANSITION

Self-efficacy — Self-efficacy is an individual's confidence in their ability to achieve desired outcomes, such as SCD self-management [79].

●In a 2016 multisite study and a 2015 review of the literature, AYAs with higher self-efficacy consistently expressed that they were better prepared for transition [80,81]. Most studies that evaluated clinical outcomes found correlations of better self-efficacy with better outcomes, although in total, only a few studies assessed this correlation. AYAs with higher self-efficacy reported fewer physical and psychologic symptoms, less stress, and improved outcomes, including decreased pain frequency, increased number of outpatient health care visits maintained after transition, and better quality of life.

●In another study from 2015, adults with SCD who cited several barriers to obtaining care in the adult health care system once transitioned nevertheless reported better self-efficacy as they improved in their self-care and autonomy [44].

Readiness — Transition readiness refers to indicators that AYAs with SCD and their support system (eg, parents, caregivers, and health care providers) can begin, continue, and finish the transition and ultimate transfer process from pediatric to adult-oriented health care [82].

Clinical algorithms and toolkits developed to support successful transition stress the importance of ongoing assessment of transition readiness so that AYAs are guided into adulthood in a structured process [41,83,84]. Transition-readiness assessment tools may be generic or disease-specific [32,33,85-87]. In either case, they should have adequate psychometric properties to allow for reliable and valid assessment.

Modifiable transition readiness factors include the following [88,89]:

●Disease-specific knowledge (eg, knowledge of disease pathophysiology, inheritance, and complications)

●Self-management (eg, medication adherence, knowledge of how to schedule medical visits and obtain medication refills) and independent living skills

●Beliefs or expectations

●Goals and motivation

●Communication skills

●Emotional status

Assessment of transition readiness must be multidimensional, evaluating not only knowledge and skills in relation to disease management but also where the individual stands in relation to developmental goals of adolescence. These goals include achievement of autonomy, establishment of supportive relationships, development of a comfortable body image and sense of self-esteem, adjustment to sexual maturation, and preparation for future goals including occupational readiness, social development, and reproductive and family planning. Assessment of emotional adjustment to the transition process and development of self-efficacy are also important.

Studies that have gathered perspectives of AYAs after the transition from pediatric to adult care have identified several facilitators of health care transition. Addressing fear of the unknown has been identified as important and can be mitigated by meeting the adult provider prior to transfer and being well-informed about what to expect [30]. AYAs also recommended gradually taking over disease management and starting the process of transition early. In a 2017 survey of 19 AYAs with SCD (ages 18 to 30), those who had completed the transition to adult care reported a history of primarily negative experiences in the adult health care system, particularly in emergency departments (EDs) [90]. They also confirmed the importance of self-efficacy, adequate knowledge, developmental maturity, acceptance, and motivation as important for transition.

As noted above, lack of readiness is a major barrier to a smooth transition. (See 'Barriers to a smooth transition' above.)

Cognitive function — Cognitive dysfunction, including attention deficit, is common in SCD, often progresses with age, and can negatively impact the transition process, as discussed above. (See 'Cognitive disability and depression' above.)

By the same token, relative strengths in domains of cognition and attention are associated with successful transition. Performance on a measure of attention at around the age of 17 years predicted later attendance at the first adult health care visit within six months of the last pediatric health care appointment [68,91].

Adherence to care plan — The transition from pediatric to adult care is a high-risk period for nonadherence, which can contribute to poor health outcomes [88,92].

In a 2009 study that combined qualitative and quantitative methods, adolescents cited having competing activities and forgetting as key barriers to adherence to clinic appointments [93]. There was a complex relationship between health status and clinic attendance, wherein some adolescents who did not feel well were less likely to attend clinic visits, while for others, feeling well was a barrier to attendance.

Efforts to increase disease-specific knowledge have been shown to improve clinic attendance while reducing acute emergency department visits and hospitalizations [94]. Structural factors such as reduced access to insurance and transportation may also impact adherence, as those living farther away from the treatment center were less likely to complete a first visit with the adult provider [95].

Virtual visits through telemedicine and mobile health interventions may have high acceptability and accessibility to adolescents with SCD and thereby may prove to be effective in promoting adherence and self-management [96-99]. (See "Telemedicine for adults".)

Parental/caregiver and community support — Parental/caregiver support is important to adherence but must be balanced with the need to transfer responsibilities for disease management to AYAs.

During the transition process, parents/primary caregivers should be coached to fade involvement at medical visits but still supervise medications and health care until the youth is able to seek out acute and preventive medical care as independently as possible. Siblings have also emphasized the importance of self-management and medication adherence to successful transition for youth with SCD [88]. We recognize that relatives and extended families remain an integral source of support for many adults with SCD, but they must learn to balance their advocacy role with their role in supporting the AYA's development of self-advocacy skills.

There is growing interest in complementing family, caregiver, and provider support with community health workers or patient navigators who empower AYAs with SCD, support their adherence, and link them to health and community services [100-104].

Care coordination — There are several opportunities for care coordination that may improve adherence and help promote a smooth transition to adult care:

●Medical care – A gap between pediatric and adult care >6 months and lack of treatment with disease-modifying therapies (eg, hydroxyurea, monthly blood transfusions) are risk factors for nonadherence with subsequent adult visits [95,105,106].

●Insurance – Patients on public insurance are less likely to complete a first visit with an adult provider post-pediatric care; this is a potential area for intervention [107].

●Familiarity with sites of care – AYAs and families/caregivers express anxiety about the transition process, even with pediatric/adult medicine colocation models, as care in the ED and inpatient services often must take place in community facilities. To alleviate this anxiety, it can be helpful to offer tours of partner community facilities [108]. It is important for SCD specialists to cultivate relationships with primary care providers willing to comanage patients and with other adult medicine specialists, where available. (See 'Overarching principles' below.)

●Patient-centered medical home – There is evidence that AYAs (and adults) receive higher-quality care when it is delivered within a patient-centered medical home (PCMH). As an example, in a cohort of over 20,000 AYAs in the general population, those who received care in a PCMH were more likely to receive preventive care, immunizations, cancer screenings, and screening for sexually transmitted infections [109].

AYAs cared for in the PCMH receive family-centered care from providers who know them well and are better able to anticipate their needs, including transition planning. This is especially important because SCD is a complex medical condition that requires age-specific preventive strategies and ongoing assessment of milestones in relation to medical care, social development, and disease-specific knowledge [40]. Also, as noted above, lack of trust from clinicians who are not familiar with the patient may further degrade care. (See 'Limited number of capable providers for adults with SCD' above.)

HEALTH CARE TRANSITION INTERVENTIONS

Overarching principles

●Transition to adult care must consider all aspects of the AYA's life, including employment, education, home life, and community [40].

●Transition interventions should be flexible to accommodate the needs of AYAs with serious cognitive challenges or multiple severe SCD complications, yet be structured and systematic [110].

●Transition to adult care is a multidisciplinary effort. Ideally, health care transition teams should have clinicians (eg, clinicians, advanced practice providers), nurse educators, social workers, and psychologists. Teams that include transition coordinators, academic liaisons, and community/peer navigators are better positioned to provide holistic and comprehensive transition services.

●Transition preparations should begin early, between ages 12 and 14 years (table 2) [111].

●The chronologic age of transfer to adult care should match the age at which the individual is ready to transfer to adult care.

Six core elements of health care transition — Approaches to transition are organized using the Six Core Elements of Health Care Transition pathway (table 1) (see 'National guidelines and frameworks for health care transition' above):

●Transition policy – Transition policies provide information about the process and should be discussed with youth with SCD and their families/caregivers between 12 and 14 years of age.

Key information to be provided includes the following:

•Details specific to the institutional transition process, including clearly stating the age when youth are typically transferred to an adult provider

•Differences in the culture of adult versus pediatric medicine and the preparation process that the youth will receive prior to beginning interactions with the adult provider

•Role of self-advocacy in health care (as well as other areas such as school and employment)

•Insurance issues

•Differences related to privacy and consent starting at the age of 18 years

Other resources for providing this information, such as seminars and online tools, are listed below. (See 'Resources for patients and families/caregivers' below.)

●Tracking and monitoring – Between ages 14 and 18, progress in acquiring the knowledge and skills necessary for health care transition can be tracked using a checklist, registry, or database that captures the benchmarks in achieving transition readiness, as well as the outcomes that are used by the program in defining a smooth transition for their population. Ideally, databases and checklists are integrated with electronic health records.

●Transition readiness – Different assessment tools have been developed; some are generic, such as:

•The transition readiness assessment questionnaire (TRAQ) [87]

•The TRxANSITION Index [112]

•The STARx Questionnaire [113]

Some are SCD specific, such as:

•The Transition Intervention Program-Readiness for Transition (TIP-RFT) [33]

•The self-management skills checklist [32]

•The American Society of Hematology toolkit [114]

Tools that examine health literacy or the ability to complete personal medical history are also used and can be complementary to the transition readiness assessments [115].

The frequency of assessments varies according to the needs of the patient, but yearly or every-other-year assessments are recommended, so adjustments and individualization of the transition planning can be made.

●Transition planning – SCD-specific transition planning ideally integrates cognitive assessment as part of the transition program, and parents/caregivers and providers must assess the youth's ability to make future independent decisions and function autonomously [40,65].

Education milestones related to SCD include both health care knowledge:

•SCD phenotype

•SCD pain plan

•Preventive measures for SCD complications

•Current and future primary health care team

•Medications and allergies

•The most important components of medical history

Health care skills are also required, including [40]:

•Ability to implement pain plan

•Ability to request refills and take medications as instructed

•Ability to schedule appointments and articulate the most important components of the medical history

•Informed reproductive decision-making

•Understanding of health insurance specific benefits and expected out-of-pocket costs

As with other adolescents, safe-sex practices should be discussed, with further consideration of the implications of a partner's sickle cell status (eg, presence of SCD, sickle cell trait, or beta thalassemia), which may have implications for reproductive counseling [116]. (See "Sexual development and sexuality in children and adolescents".)

Existing tools to support transition planning include use of music therapy, individual and group psychoeducation sessions, and online health self-management and educational applications [65,116-121]. Additionally, handouts that are customized to the youth's areas of deficit have been shown to improve knowledge and skills in the areas of independent living (eg, laundry, housekeeping) and health care [122]. Specific coaching in communication and self-advocacy skills is also helpful [123].

●Transfer to adult care – Transfer from a pediatric to adult-centered care should ideally occur within two months (or at least within six months) from the last pediatric visit, according to expert opinion [26].

The transfer should only occur once an appropriate adult provider has been identified; therefore, coordinated communication between pediatric and adult providers (primary care, SCD, and other specialists) needs to take place. (See 'Care coordination' above.)

Transfer of medical records to the next provider should occur in a timely manner, and the pediatric provider should remain available for questions from the adult provider.

A medical summary listing all prior and current morbidities and treatments (prepared by the pediatric provider) should be shared with the adult team. Resources for these documents are available on several websites. (See 'Resources for providers' below.)

The transfer summary should include:

•An action plan for pain control and prevention (individualized for each patient).

•A summary of evaluation for SCD comorbidities should be completed and include the following areas: ophthalmology, dental medicine, pulmonary, cardiac, kidney function, screening for stroke risk with transcranial Doppler ultrasound and, in some cases, brain magnetic resonance imaging (MRI) for silent cerebral infarcts, with follow-up cognitive testing if needed, or cognitive testing if academic achievement is problematic [40].

•Information on educational progress (a marker for cognitive functioning).

•Immunization status with a list of the recommended future immunization schedule (during the adult years). (See "Overview of the management and prognosis of sickle cell disease", section on 'Immunizations'.)

Having AYAs meet the adult provider prior to leaving pediatric care and incorporating a transition navigator are both successful strategies to ensure successful transfer completion [102,108]. In some programs, pediatric and adult teams meet together to review medical, psychologic, education, and social needs of patients in advance of the first adult clinic visit. These activities can facilitate the transfer process but have not been formally tested.

Additional information about routine evaluations and treatments is presented in more detail separately. (See "Sickle cell disease in infancy and childhood: Routine health care maintenance and anticipatory guidance", section on 'Older adolescents' and "Overview of the management and prognosis of sickle cell disease", section on 'Routine evaluations and treatments'.)

●Integration into adult care – The completion of transition typically occurs between the ages 18 and 26 years. During this period, a confirmation of transfer should take place. Additionally, feedback related to the satisfaction with the transition process should be elicited from the patients and their families/caregivers. Their feedback should be incorporated into the transition activities for continued quality improvement of the transition program. Long-term retention in adult care is an important metric of health care transition. Risk factors for poor adult care retention are not yet identified in SCD and have implications for care continuity and maintenance of health and quality of life.

In some programs, pediatric and adult teams involved in SCD care are co-located in the same building, which facilitates communication. Co-located pediatric and adult care in the first few years of adult care has been shown to prevent the expected spike in acute care utilization among AYAs with SCD [94].

Once the AYA is transferred, questions to consider in judging the efficacy of the process include:

•Is the AYA in the care of competent health care providers in an organized system of care?

•Are they competent to partner with their health care providers and/or do they have supports in place to assist them? Considerations regarding the role of caretakers should be given to those who need conservatorship.

•Are they receiving preventive and SCD-specific health care consistent with clinical guidelines and best practices? Is their clinical course stable?

•Are they well-functioning and realizing their potential?

Examples of programs that have successfully implemented all the Six Core Elements of Health Care Transition and have reported program and patient-level outcomes are available [124,125].

Areas of continued uncertainty — Additional areas that are being explored include:

●Optimal ways to deliver transition guidance (eg, community health worker, peer-mentoring, health care team, online resources).

●Standardized outcome measures for transition that could be used to evaluate best practices and define success.

●Alternative models for health care delivery (other than fee-for-service) such as value-based initiatives that support the required components of a health care transition, such as records transfer and disease education.

Resources for patients and families/caregivers — There are several resources available for patients and families/caregivers to prepare for the transition to adult care. As examples:

●Workshops – The Sickle Cell Disease Foundation, a community-based organization (CBO) in California, has offered annual transition workshops that consisted of a mix of didactic and interactive sessions promoting knowledge and skills important for optimal functioning in adult health care (eg, self-advocacy in the ED, understanding of preventive therapies for SCD) as well as in general adult life (eg, sexual health, financial health). Virtual participation in workshops and support groups has been evaluated in other settings, with demonstrated feasibility and acceptability [126]. We encourage contacting a CBO within each community to identify resources for transitioning patients.

●Online tools and other electronic communication – Internet-based interventions, social media, and other online communication tools are increasingly used to enhance patient engagement in the health care process, and these can result in improved health outcomes such as reduced hospital readmission rates, enhanced quality of life, and decreased mortality [127]. Text messaging and mobile phone application-based interventions (mobile phone apps) have demonstrated positive impressions, feasibility, acceptability, and some evidence for efficacy in improving adherence for youth with chronic health conditions [99,128-130].

●Internet-based references – The use of resources developed by professional societies with accurate medical information should be encouraged. Examples include the following:

•Healthychildren.org from the American Academy of Pediatrics (AAP) has general information about nutrition, fitness, emotional wellness, home life, and common issues, presented in a developmentally appropriate framework.

•The Sickle Cell Transition E-Learning Program (STEP) for teens with SCD from the St. Jude Children's Research Hospital is a six-module online program with a series of videos with interactive quizzes to teach teens about SCD.

•Health Smart: Teens with Sickle Cell Disease Moving from Pediatric to Adult Care is an online brochure from the St. Jude Children's Research Hospital that lists information and answers to common questions directed at an AYA audience.

•Got Transition is a website created via a cooperative agreement between the Maternal and Child Health Bureau and the National Alliance to Advance Adolescent Health, both in the United States; it provides general tools for every step of the transition process from pediatric to adult medical care.

•Taking Charge of Your Health and Healthcare from the Centers for Disease Control and Prevention (CDC) raises awareness about the transition process in SCD, provides tips to prepare for the process, and offers additional resources for managing transition.

Resources for providers — There are several resources available for medical providers to improve the transition process from pediatric to adult care for youth with SCD.

●The American Society of Hematology (ASH) has an initiative on care transitions [131]. Resources include a toolkit developed to minimize gaps in care and help youth improve the management of their own health care. The toolkit contains forms that were developed as part of a broader initiative led by the American College of Physicians.

●ASH Clinical Practice Guidelines on SCD.

●The National Institute for Children's Health Quality (NICHQ) has online information related to several aspects of SCD care. As examples:

•Improving care for SCD pain in the ED [132]

•Improving access to SCD care and use of hydroxyurea [133]

●A comprehensive summary of age-appropriate information related to medical, social, and academic milestones in SCD care was created by Working to Improve Sickle Cell Healthcare (WISCH), a network of comprehensive sickle cell centers, sickle cell community-based organizations, youth with SCD, and primary care providers. Several resources are available, including a "Compendium of Tools and Resources for SCD" from 2021.

●Information on High Value Care in SCD from the American College of Physicians Pediatric-To-Adult Care Transitions Toolkit.

●Provider-facing interfaces in the iManage mobile phone application that provide guidelines, algorithms, and methods for communication with local SCD specialists [134].

●General initiatives related to the pediatric-to-adult care transition are available from various organizations:

•The 2018 joint report on action steps for the pediatric-to-adult care transition, created by the AAP, the American Academy of Family Physicians (AAFP), and the ACP-American Society of Internal Medicine [2]. (See 'Overarching principles' above.)

•The "Six Core Elements of Health Care Transition" (table 1) developed by the Center for Health Care Transition Improvement (also called "Got Transition"), which can be customized for different practice settings [83]. These tools allow flexibility depending on whether the youth is transferring to an adult provider, with guidelines for pediatric family medicine and medicine-pediatrics providers or transitioning to an adult approach without changing providers.

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Sickle cell disease and thalassemias".)

PATIENT PERSPECTIVE TOPIC — Patient perspectives are provided for selected disorders to help clinicians better understand the patient experience and patient concerns. These narratives may offer insights into patient values and preferences not included in other UpToDate topics. (See "Patient perspective: Sickle cell disease".)

SUMMARY AND RECOMMENDATIONS

●Importance – The transition period from pediatric to adult care for youth with sickle cell disease (SCD; as with other chronic conditions) is important as they develop the knowledge, skills, and motivation to actively participate in their health care. The stakes for health and even survival are high for individuals with SCD, who often experience an increase in the use of health care resources as well as a sharp increase in mortality during the AYA years. In addition, the incidence of certain disease complications rises as individuals with SCD age. (See 'Health care transition as a vulnerable time' above.)

●Indicators of success – An optimal transition from pediatric to adult SCD care is one in which the youth adheres to therapy, continues along a stable disease trajectory, participates in preventive care and health maintenance, and achieves appropriate developmental milestones. Other indicators of success include good communication, self-efficacy, and trust with the adult provider. (See 'Defining a successful transition' above.)

●Barriers to success – A major barrier to the smooth transition from pediatric to adult care in SCD is the limited availability of knowledgeable adult-oriented providers (and hence, limited access to quality health care). Other barriers also exist, including lack of readiness, cognitive dysfunction (eg, due to stroke or silent cerebral infarcts), and depression (eg, due to chronic pain). (See 'Barriers to a smooth transition' above and "Prevention of stroke (initial or recurrent) in sickle cell disease", section on 'Individuals with silent cerebral infarctions'.)

●Predictors of success – The success of the transition depends on the patient's self-efficacy, emotional and intellectual readiness, adherence to the care plan, support from the parents/family/caregivers, and a patient-centered approach to care. (See 'Predictors of a smooth health care transition' above.)

●Stages of transition – Transition is a multidimensional process that must begin early and occur in stages that include providing information about the process, assessing readiness, coordinating communication between pediatric and adult providers, completing age-appropriate medical assessments and interventions, preparing a medical summary and an action plan for pain control and prevention (table 2), and introducing the adult provider. The table (table 1) summarizes the Six Core Elements of Health Care Transition. (See 'Overarching principles' above.)

●Age and readiness – Most youth are transitioned to adult care between 18 and 21 years of age; however, transition readiness is determined by the individual's social and emotional development and achievement of milestones in disease management, not by a prespecified age. (See 'Readiness' above.)

●Resources – A number of outstanding online resources are available for patients, families, caregivers, and providers. The Center for Health Care Transition Improvement recommends direct assessment of the perspectives of young adults with regard to the transition process. A survey instrument is available online for making these assessments. (See 'Resources for patients and families/caregivers' above and 'Resources for providers' above.)

ACKNOWLEDGMENT — UpToDate gratefully acknowledges Stanley L Schrier, MD (deceased), who contributed as Section Editor on earlier versions of this topic review and was a founding Editor-in-Chief for UpToDate in Hematology.