Transition of the Chronically Ill Youth With Hemoglobinopathy to Adult Health Care: An Integrative Review of the Literature

Method of Review 

A comprehensive exhaustive literature search on the transition to adult health care was conducted by using MEDLINE, Cumulative Index of Nursing and Allied Health Literature (CINAHL), PsycINFO, UpToDate, the Agency for Healthcare Research and Quality (AHRQ), MD Consult, Cochrane Database of Systemic Reviews, ProQuest, National Guideline Clearinghouse, Web of Science, and several organizational Web sites such as the American Academy of Pediatrics (AAP), the American Heart Association, and Sigma Theta Tau International. The search limitations included children to young adult age bracket, the English language, and publication dates from 1980 to 2007. The search strategies also included searching the names of authors who had published more than one article on transition to adult health care. The search terms and phrases utilized were: adolescent, young adult, chronically ill youth and hemoglobinopathy, pediatric-oriented care, adult-oriented care, children and special health care needs, transition program, transfer program, continuity of care, transitional strategy, transitional barriers, transition to adult care, and transfer to adult care.

Initially, the inclusion criteria consisted of transition search terms, age range, and the English language, with the only exclusion criteria being middle to older age individuals. However, after sorting through a plethora of nonempirical literature published on chronically ill youth's transition to adult health care, the exclusion criteria were broadened to include commentaries, editorials, and case studies on transition. The reference lists of each research study were perused to identify other related studies. A hand search of nursing, medical, and psychological journals, books, position papers, and conference proceedings was performed until the references became repetitive in the literature.

Historical Aspects 

Expert panels, publications, conferences, and policy statements have discussed facilitating an organized, coordinated transition for young people with chronic conditions from the child to the adult health care system (Blum, 1995, McGrab and Millar, 1989, Rosen, 1995). In 1984, the United States Surgeon General co-hosted a national invitational conference with the Under Secretary of Education, focusing on the needs of older adolescents with chronic and disabling conditions. This conference provided the first national focus on health care transition issues (McGrab & Millar). The Society for Adolescent Medicine addressed the problem of transition in a position paper delineating recommendations for transition funding, research on outcomes of transition programs, investigation of multiple transition models, and education concerning transition issues among health care trainees and providers (Blum et al., 1993). The American Academy of Pediatrics, 1996, American Academy of Pediatrics published policy statements describing the importance of the health care provider role to educate and advocate for youth and family in promoting transition to adult health care.

Since the 1980s, a limited number of empirical studies have been published on transition of the CIY to adult care, especially for youth with hemoglobinopathies. To compile a comprehensive review of the transition literature, it was necessary to supplement the paucity of hemoglobinopathy studies with transition studies on a variety of chronic illnesses (e.g., cystic fibrosis [CF], diabetes, and disabilities). The literature review identified 17 empirical studies that met the inclusion criteria, with only five of the studies focusing on hemoglobinopathies (Table 1, Table 2, Table 3). All of the transition studies were published in peer-reviewed journals. The studies are listed in Table 1, Table 2, Table 3, which are divided into five areas: author and date, design and purpose, subjects, key findings, and clinical recommendations. In reviewing the 17 studies, three main transitional obstacles consistently emerged from the literature: (a) five studies focused on the lack of transitional support (Table 1); (b) six studies focused on limited transitional programs (Table 2); and (c) the remaining six studies focused on transitional resistance toward adult health care (Table 3).

Table 1. Transitional obstacle: Lack of transition support

	Author/date	Design/purpose	Subjects	Key findings	Clinical recommendation
	Scal, P. (2002)	Survey design: mailed investigator- developed survey to nominated health care providers by families of patients (e.g., CF, epilepsy, others); purpose of this study was to describe transition services provided by primary care physicians	13 of 36 nominated health care providers completed the 53 forced-response items and 8 open-ended items on the survey (survey reviewed by 6 researchers prior to distribution)	Summarized responses on transition were: fear/ambivalence; transition is a process; focus on youth future; family physician view; transition as part of care; identify adult provider; lack of institutional support (no planning time/no personnel) and community resources	Recommend individualized care to transition; view transition as a continuum; start early and work as a team; identify and train adult providers to care for chronically ill youth; obtain institutional support for transition services (planning time/resources/ personnel (most significant/study); seek community resources (school/employer)
	Scal, P., & Ireland, M. (2005)	Cross-sectional design using a random digit-dial method to generate sample; purpose was to determine from surveyed parents if the health care provider addressed transition from child to adult health care for youth with special health needs	Data from 4332 out of 4426 youth 14 to 17 years old from 2000-2001 National Survey of Children with Special Health Care Needs via 3 questions: (a) Have providers talked about health care needs changing when adult? (b) Has a plan that addressed changing needs been developed with provider? (c) Has provider discussed transfer to adult health care?	50% of parents reported discussion of transition issues with doctor; HCT was discussed comprehensively with 16% of youth; condition complexity, older age, White race, high-quality relationship with provider significantly addressed HCT issues/ bivariate analysis; to date, no outcome studies on transition interventions in literature to substantiate the value HCT services	Discuss transition process early with youth and family; work as a team with adult provider, youth, and family; establish transitional plan with the team; facilitate HCT services (insurance coverage, employment/school guidance)
	Reiss, J. G., Gibson, R. W., & Walker, L. R. (2005)	Qualitative design; purpose was to address the need for exploratory research on HCT and documentation of the transition experience; it is widely known that HCT is often unsuccessful, few empirical data have been collected to help understand and explain the difficulties	143 participants in 34 focus groups almost evenly distributed: youth/young adults (49), family members (44), and health care providers (50);ages ranged from 13-35 y with a variety of chronic illnesses (e.g., asthma, cardiac, cancer); focus groups and interviews conducted separately using a standard protocol	Two factors with significant effect were: cognitive ability of patient and progressive nature of the disability; stages of transition: envisioning a future; age of responsibility; age of transition; allow for developmental delays; age of transition is completed by age 21 y; transitional barriers: aging out of treatment, insurance/funding, availability of care, practice differences	Incorporate cognitive/developmental readiness in transition preparation to adult care; avoid automatic transfer to adult care (pregnancy/substance abuse/criminal activity); discuss subcultures (pediatric vs. adult medicine) with youth and family; allow youth to verbalize concerns about transfer; institute therapeutic termination of pediatric relationships as part of the transition process; notify social worker and/or financial counselor to aid with continuation of insurance/funding for health care of youth
	Wojciechowski, E., Hutig, A., & Dorn, L. (2002)	Nonexperimental descriptive design used to collect historical data; purpose of study was to obtain database to understand natural history when AYA with SCD make the transition from PCC to ACC	18/35 participants ages 18-24 y were chosen from 3 Midwestern hospitals; using Compliance Questionnaire (Cronbach's α = 0.56) and Self-Efficacy Scale for Managing SCD (α = 0.84), the correlation between dependent variables (uninterrupted care, compliance, self-efficacy, independence) and independent variables (preparation, gender, pediatric patient care) was questionable	AYAs with chronic illness are transferred to ACC abruptly with little preparation or opportunity to practice managing own care; follow-up ceases once AYA leave PCC; transfer is often initiated because of the AYAs' age or pregnancy rather than on youth's readiness; many AYAs remain on public insurance and have jobs; lack of communication between PCC and ACC practitioners	Transfer to adult care based on readiness (educational/developmentally); increase communication between adult and pediatric providers; verbally communicate between providers regarding transfer information; provide a medical summary for the adult provider
	Hauser, E., & Dorn, L. L. (1999)	Exploratory nonexperimental design; purpose was to develop framework to guide the transition of SCD patients from PCC to ACC and to provide knowledge useful to nurses working with SCD adolescents and those with other chronic illnesses	Purposive sample from 4 SCD pediatric centers in Midwestern city comprised 3 groups: 22 adolescents age 13-21 y, 22 parents/ caregivers with mean age of 45.7 y, and 8 practitioners who completed open-ended questions /interview via audiotape and notes	Focus group indicated the concerns, expectation, and needs of the parents and adolescents were parallel (e.g., lack of transition preparation, unfamiliar provider); transition framework recommended and formed based on focus group results With abrupt transfer, adolescent tend not to keep follow-up appointments with new adult provider and may return to pediatrics or ED for care	Avoid abrupt transfer to adult health care if possible; establish a transition program based on research; provide and evaluate transition framework; introduce adolescent and parents to adult practice; empower the adolescent with knowledge of SCD and practice decision-making skills

ACC, Adult-centered care; AYA, adolescent/young adults; CF, cystic fibrosis; ED, emergency department; HCT, health care transition; PCC, pediatric-centered care; SCD, sickle cell disease.

Table 2. Transitional Obstacle: Limited Transitional Programs

	Author/date	Design/purpose	Sample	Key findings	Clinical recommendation
	Reid, G. J., et al. (2004)	Cross-section study from entire cohort; purpose was to determine the percent of young adults with complex CHDs who successfully transferred from pediatric to adult care and examine correlates of successful transfer; successful transfer defined as keeping 1 adult appointment of any type (e.g., clinic, surgical, test)	234/360 eligible youth aged 19-21 y with complex CHDs from a pediatric tertiary center database completed questionnaire; of the total cohort, 170/360 youth successfully transferred to adult care	Based on multivariate analysis, successful transfer was significantly related to more pediatric surgeries, older age, and recommendation from pediatric cardiologist; first study to document the % of youth with chronic illness who successfully transferred to adult care in a timely manner; no formal transition program in place other than transfer of record; transition process needs to start before patients are transferred	Establish a transition program well before transfer of the youth begins; provide a medical summary for the adult provider
	Por, J., et al. (2004)	Survey design used to collect descriptive data; purpose was to explore the views/needs of HCP regarding transition of care for adolescents with chronic illness to adult services	Data collected by 120 anonymized mailed investigator-developed questionnaires to HCP from the same hospital; 40 of 120 questionnaires were returned; content validity was established by expert panel and piloted with 5 subjects to test questionnaire	Maturity rather than age is main criteria for assessing youth readiness for transfer to adult care; content analysis identified: anxiety/fears/concerns of transition to adult care, concerns about paying for adult services, time to meet adult provider; support need for transition program, but only 10% of their youth with chronic conditions were adequately prepared for transition to adult services	Provide multidisciplinary approach to formulate a clear written transfer plan; establish a transition program utilizing a consistent provider; orient youth/family /provider to adult clinic before transfer (meet with adult provider team); discuss insurance/medical funds for adult care within transfer program
	Fiorentino, L., Datta, D., et al. (1998)	Mixed study design; purpose was to examine the assumption that improve transitional process reflects improved outcomes for YDY and carers; transition experience of youth was compared with legislative practice guidelines of good transition practice	87/206 individuals aged 16-25 y and/or carers agreed to participate; YDY were from several registries and had cerebral palsy, spina bifida, juvenile arthritis, and other diagnoses; hypotheses focus: (a) Handover to adult services differ between schools; (b) experiences of YDY depend severity of disability; (c) quality of handover process affect perceptions of service received	An ethnographic qualitative analysis of the interview material and quantitative data were analyzed using SPSS; YDY handover experience to adult services related to: type of school and severity of disability; perception of service (unexpected transfers cause distress/anger); usual handover: 16 y; YDY leave pediatrician and medical care declines	Establishment of transition program to include: identification of all YDY early and formulate plan; maintain a flexible age for transition; train adult providers in developmental care of YDY
	Lotsein, D. S., McPherson, M., Strickland, B., & Newacheck, P. W. (2005)	Descriptive study using nation-wide random telephone survey; purpose was to describe current planning practices of health care providers nation-wide for youth with special heath care needs from the perspective of parents and guardians	Of the 373,000 youth in all 50 states, the final sample included 38,865 CSHCN; Questionnaire created by Maternal Child Health Bureau and used from Oct. 2000-Apr. 2003; 3 transition questions were added after the study started so 5533/13885 youth aged 13-17 y and parents were asked transition questions	In the first national Survey of CSHCN, the overall rates of transition planning are relatively low, particularly for youth from ethnic minorities; a multivariate logistic-regression model found that older age with medical home were more likely to receive transition planning; pediatric providers wanted to know the “right” age to transfer	Formulate transition plan encompassing shift to adult health care; identify when the youth is physically, medically, and socially prepared for transition; discussion of transition with providers, youth, and family prior to transfer; discuss change in health care needs as an adult
	Anie, K., Telfair, J., & Transition Study Working Group (2005)	Cross-sectional survey design employed; purpose is to identify similarities in 2 countries with different health services; study predicted self-efficacy and self-management skills in adolescents are influenced by age, educational level, and gender	224 SCD adolescents from SCD centers in United States and 1 center from United Kingdom were surveyed; validated SCD Psychological Symptom Scale/ SCDEQ/SCDARS were used	Using analysis of variance, no significant differences between the 2 countries; using the hierarchical regression on SCDEQ and factor analysis of SCDARS, analysis indicated that older adolescents with higher educational level tend to report more physical and psychological symptoms related to SCD based on SCDPYS	Provide SCD knowledge and promote self-care skills in the SCD adolescent; orient the youth and family to adult care system prior to transfer
	Telfair, J., Alexander, L., Loosier, P., Alleman-Velez, P., & Summons, J. (2004)	Cross-sectional, multi-format survey in form of confidential mailed questionnaires to 3 sample populations of HCP of SCD patients; purpose is to focus on the opinions and insights of HCP from multiple disciplines regarding the transition to adult care from pediatric care of adolescents with SCD ages 12 years and older	227 pediatric and adult medical psychosocial HCPs sampled in 3 waves: (a) sample of HCPs at 2 sickle cell programs; (b) sample of private providers likely caring for SCD patients; (c) national sample of all SCD care providers Investigator-developed provider-version Sickle Cell Transfer Questionnaire(P-SCTQ) α = 0.80 score	89% of respondents indicated a need for systematic transition; providers significantly believed a systematic transition program provides more support in meeting youth needs; good transition program includes comprehensive/clear goals and education/ discussion/support/ HCP; based on bivariate and comparative content analyses, 70% of adult HCPs expect youth to attend clinic with parent, 67% adult HCPs believed the opposite	Establish comprehensive clear transition goals; recognize transition youth readiness and start preparation of youth for transition; educate the HCP

CHD, Congenital heart defects; CSHCN, children with special health care needs; HCP, health care provider; SCD, sickle cell disease; SCDEQ, SCD Efficacy questionnaire; SCDARS, SCD Readiness Scale; YDY, young disabled youth.

Table 3. Transitional obstacle: Transition resistance to adult health care

	Author/date	Design/purpose	Sample	Method	Key findings/clinical recommendation
	Telfair, J., Myers, J., & Drezner, S. (1994)	Cross-sectional survey; purpose was to determine the issues, concerns, and expectations of adolescents, young adults, and primary caretakers with regard to transfer to adult care	Volunteer participants included 36 adolescents (13-19 yo), 60 young adults (21-30 yo), and 25 adolescent primary caretakers from 2 pediatric and adult sickle cell programs; respondents either completed 67-item mailed SCTQ or used the interview method with the SCTQ	Bivariate statistics used to examine that 44% of adolescents, 50% of young adults, and 68% of caretakers had concerns: adolescents/young adults were concerned about payment of medical care and treatment by adult providers; all groups were concerned about teens leaving pediatric care; caregivers were concerned about being excluded from medical decisions	Start transition process when youth is developmentally ready; establish transfer program; encourage youth, parents', and providers' verbal participation in the establishment of a transfer program (express concerns/expectations); listen and demonstrate respect for opinions, concerns of the youth; allow youth to participate in decision making during the transition process
	Fiorentino, L. L., Phillips, D., Walker, A., & Hall, D. (1998)	Descriptive study using a semi-structured interview; purpose was to describe the procedures in place to facilitate transition from pediatric services to adult services	Utilized the Sheffield Case Register updated every 3-5 y that identified young people with physical and learning disabilities. between the ages of 16-24 y; compiled a database of 159 youth and carers, of which 50 were interviewed	Data from precoded questions and from postcoded open-ended questions were analyzed using SPSS statistical package; none of the young people experienced a smooth transition from pediatric to adult care; transition program consisted of: impersonal formal letter through the post and pediatrician informed youth at final annual meeting to transfer to 1 of 3 hospitals	Prepare youth for transition; establish a specific transition policy for pediatric/specialty clinics; establish a formal transition program; teach youth the skills needed to function in adult clinic; encourage advance practice nurses to act as a facilitator of youth's transition to adult care; develop links between adult and pediatric clinics; evaluate transition programs
	Christian, B. J., D'Auria, J. P., & Fox, L. C. (1999)	Exploratory, qualitative pilot study; purpose was to explore the meaning of chronic illness experience for adolescents with DM in relation to taking responsibility for their own care	Purposeful sampling; 4 adolescents ages 14-18 y diagnosed with DM at least 4 y were recruited from a pediatric diabetes center; a retrospective semi-structured interview to explore the meaning of self-responsibility for diabetes care	3 themes marked the process: making DM fit within life activities, being ready /willing to transition, having a safety net of friends; acceptance of self- responsibility varied based on cognitive and emotional readiness; this study indicated cognitive readiness and willingness to learn knowledge of disease/problem-solving skills was identified as early as age 12 y	Incorporate youth developmental needs and readiness into the transition preparation; provide opportunities to practice independence guided by a consistent provider (e.g., advance practice nurse)
	Boyle, M., Farukhi, Z., & Nosky, M. (2001)	Descriptive design; purpose was to identify concerns and expectations of parent and patients prior to/after transitions then develop strategies for the future needs of patients/family members; develop database of patient concerns and patient profile to formulate a customized transition protocol	52 CF patients (age 18-63 y) and 38 parents who volunteered to complete an open-ended survey 3 months pretransition and 60 participants completed post-transition interview 8-12 mo	Investigator-developed survey revealed areas of concerns, which were leaving caregivers, meeting new team, and exposure to infection; a significant lower level of concern was noted in all areas of concerns with a prior introduction to adult CF team in post-transition group	Transition process needs to be implemented between the ages of 15-25 y; transition education should include youth, parent, and providers; develop a transition program to adult care; introduce youth to adult team prior to transfer; incorporate the concerns of the youth/families into a customized transition protocol
	Soanes, C., & Timmons, S. (2004)	Qualitative design; purpose was to examine the attitudes of young people with chronic illness who were facing transition	Purposive sample of 7 adolescents (aged 14-17 y) were interviewed; the chronic illnesses of the adolescents consisted of 4 with diabetes, 2 with chronic renal failure, and 1 with cancer	Data collected via semi-structured interviews; content analysis revealed 5 themes: flexibility, informality/formality, comfort/familiarity, ready/prepared, support	Prepare youth for transition by assessing readiness, then disseminate transition information (diagnosis/medication/preventive actions); establish a Buddy System with a previously transitioned youth; schedule and informal day with adult provider; employ a flexible, supportive provider to direct the transition program
	Lam, P., Fitzgerald, B., & Sawyer, S. (2005)	Retrospective chart review; purpose was to explore the extent to which disease complexity and failure of transition planning may contribute to young adults' health care who continue to be based at a children's hospital	Sample consisted of 247 individuals; 57% were 18-20 yo, 35% were 20-25 yo, and 8% were 25-45 yo; 68% admitted to surgical unit (e.g., orthopedic, neuro, surgical); 52% admitted to adolescent unit (cardiac endocrine and others); 27% had more than 1 admit, and 17% were admitted the following year	Significant increase in admission over 10- year period and 3/6 of the surgical units had significant increase in admissions; both disease complexity and failure of transition planning contribute to the increase admissions to hospital; need greater focus on support of youth with chronic illness during transition to adult care using transition planning	Focus of transfer age to adult care expects to occur at completion of high school; establish a transition plan; provide continuous education of the youth regarding diagnosis, medications, and preventive measures

CF, Cystic fibrosis; DM, diabetes mellitus; SCTQ, Sickle Cell Transfer Questionnaire.

Transitional Obstacle: Lack of Transitional Support 

Lack of transitional support was described in the following five studies that focused on the lack of available resources to support transitional programs for the CIY. Scal (2002) surveyed 13 out of 36 health care providers who were nominated by CIY's caretakers. After receiving a mailed investigator-developed survey, the health care providers identified lack of transitional support (e.g., no time for transition planning, no resources, and no personnel) and difficulty in finding or communicating with an adult subspecialist medical provider as factors that interfered with the transfer of the CIY (e.g., CF and epilepsy) to adult health care (Scal). Scal's study identified other factors such as lack of time to address transition issues, lack of additional training for the health care provider in relation to adolescent developmental and emotional issues, lack of utilization of transition services (e.g., insurance coverage, employment and/ or school guidance) and limited community based resources, which was a factor supported in another study by Scal & Ireland (2005).

The lack of transitional support was identified in a cross-sectional study using a random digit-dial method. The study surveyed a sample of 4332 out of 4426 eligible parents of youth aged 14 to 17 years obtained from 2000-2001 National Survey of Children with Special Heath Care Needs (Scal & Ireland, 2005). The Health Care Transition (HCT) survey asked the parents if the health care provider (HCP) addressed transition issues during their routine visit with the chronically ill adolescent (see Table 1 for listed questions). Bivariate analysis examined the relationship between factors (e.g., age, gender, and disease severity) and parents' response scores that ranged from 0 to 3 (negative to positive response) from the HCT survey. Older age, White race, greater number of health care needs, and higher quality of parent-provider relationship were associated with significantly higher scores on the HCT survey. Overall, 50% of the parents reported that transition to adult health care was mentioned by the HCP, but only 30% had developed transitional plans. Focusing on the lack of transition support, this study considered health care transition to be suboptimal in that only 16 % of CIY had health care transition comprehensively addressed by the HCP (Scal & Ireland).

Reiss, Gibson, and Walker (2005) focused on lack of transition support by interviewing 143 participants about their experiences with transition services and health care systems. The participants were distributed into focus groups consisting of 49 youth with chronic illnesses (e.g., asthma, cardiac, and cancer), 44 family members, and 50 health care providers. Reiss and colleagues reported similar factors that contributed to the lack of transition support, which was described in a study by Scal (2002). Reiss and colleagues reported factors such as availability of care, insurance issues, and lack of funding with the automatic discharge to adult care because of age or undesirable behaviors (e.g., pregnancy and criminal activity). The researchers stressed the need for further exploratory research on health care transition and the need for documentation of the transition experience (Reiss et al.). The 2002 study by Wojciechowski, Hurtig, and Dorn identified lack of transitional support from the questionnaire responses of 18 participants aged 18 to 24 years with SCD. Pediatric and Adult Site Review Forms (PASR), SCD Compliance Questionnaire (Cronbach's α = 0.56), and SCD Self-Efficacy Scale (Cronbach's α = 0.84) questionnaires were used. Supporting each other's findings, these studies identified the following: automatic discharge from pediatrics (because of events such as age ≥18 years, pregnancy, criminal activity, or other behaviors), lack of continuation of funding and insurance, lack of practitioner training and experience with disabled youth, and the lack of transition preparation as contributing to the lack of transitional support (Reiss et al.; Scal; Scal & Ireland, 2005; Wojciechowski et al.).

Hauser and Dorn (1999) also focused on the lack of transitional support. The pilot study consisted of a purposive sample divided into three focus groups comprising 22 youth with a SCD aged 13 to 21 years, 22 parents, and 8 health care providers. Hauser and Dorn's content analysis identified the following themes: unfamiliar adult provider, lack of pre-transition education, and lack of preparation in assuming an adult role, which has been supported by other studies (Reiss et al., 2005, Scal, 2002, Scal and Ireland, 2005, Wojciechowski et al., 2002). Hauser and Dorn developed a draft framework as part of the pilot study. The framework, developed to guide a successful transition program for youth with SCD to adult care, has not been empirically evaluated. Of the five studies reviewed, two studies focused on the CIY with hemoglobinopathy. All five studies reported that transition programs are necessary to promote the successful transfer of chronically ill youth to adult care.

Transitional Obstacle: Limited Transitional Programs 

The following six studies focused on the limited number of transitional programs for CIY and the necessity for these programs. Out of the six studies reviewed on the limited number of transition programs, two focused on CIY with hemoglobinopathy. However, the majority of studies focused on a variety of other chronic illnesses (Table 2). The first cross-sectional study published on transition programs documented that 47% (170 out of 360) of youth aged 19 to 21 years with congenital heart defects successfully transferred to adult care in a timely manner (Reid et al., 2004). Yet, the measurement of successful transfer for the study was limited to keeping one appointment in the adult clinic or any adult-related test. Using an investigator-developed questionnaire, another study on transition programs evaluated the views of 40 health care professionals regarding transitional preparation for adolescents with a chronic condition (e. g., CF, asthma, and diabetes) to adult health care (Por et al., 2004). Based on the content analysis, the providers supported the need for transition programs, but revealed that only 10% of their adolescents with chronic conditions were prepared adequately for transitional care (Por et al.). Both of these studies supported the need for transitional planning and transition programs.

Another study supporting the need to plan for transitional programs was reported by Fiorentino, Datta, and colleagues (1998). An interview was conducted of 87 out of 206 eligible disabled youth (e.g., youth with cerebral palsy, spina bifida, and juvenile arthritis) and/or their caregivers from the registry of organizations such as Cerebral Palsy Association (Fiorentino, Datta et al.). Using content analysis and a statistical software program for analysis, the study compared the legislative guidelines for good transition practice to the youth's transition experience to adult health care. The disabled youths' perceptions of the transition process were correlated with the type of school the youth attended. The severe learning disabled youth in a special school usually had a transition plan, whereas the mild or moderately disabled youth in a mainstream school did not have a transition statement or plan. Therefore, the more severe physically disabled youth were more apt to be transferred to an adult specialist, which speaks to the quality of the transition experience. In contrast, the mild or moderate physically disabled youth usually remained with their general practitioner (Fiorentino, Datta et al.). Fiorentino, Datta, and associates concluded that transitional practices did not correlate with the legislature's transition guidelines and that problems exist in the transition process, which subsequently interfere with the transition of the disabled youth to adult health care.

The 2005 study by Lotsein, McPherson, Strickland, and Newacheck added transition questions to an existing national survey. Using a random-digital dial questionnaire created by an expert panel associated with the Maternal Child Health Bureau, 5533 out of 13,885 youth aged 13 to 17 years with special health needs and their parents answered transition questions. All analyses were conducted by using a statistical software program by the Stata Corporation. The study reported that transitional planning with a medical transition goal was performed in only one of seven youth with special health care needs, with even lower results in youth from ethnic minorities. Lotsein and colleagues noted that 20% of youth with a medical home met the medical transition goal compared with the 11 % of youth without a medical home. The medical home is defined as an approach to providing continuous and comprehensive pediatric care from infancy to adulthood (AAP, 2002). Not only have studies focused on the youth and families' transitional needs and the concerns of the health care providers, but they also have stressed the need for transitional planning. Clearly obstacles exist that impede the transfer from pediatric to adult care setting, which include the limited number of evaluated transitional programs.

Transitional programs are considered necessary as reported by a cross-sectional study of 224 adolescents with SCD who were selected from Sickle Cell Centers in United States and a center in the United Kingdom (Anie, Telfair & Sickle Cell Disease Transition Study Working Group [SCDTSWG], 2005). The focus of the study was to predict that self-efficacy and self-management skills in adolescents were influenced by age, educational level, and gender. Validated disease specific instruments (Sickle Cell Disease Psychological Symptoms Scale [SCDPYS], Sickle Cell Disease Efficacy Questionnaire [SCDEQ], and SCD Adolescent Readiness Scale [SCDARS]) were used in the study (Anie et al.). The instruments were used to assess the adolescents' perceptions of physical and psychological symptoms, self-efficacy, and self-management skills that were correlated with demographic factors (Anie et al.). Using analysis of variance, there were no significant differences between the two countries on the demographic factors (Anie et al.). Using hierarchical regression analysis on SCDEQ and factor analysis of SCDARS, both analyses indicated that older adolescents with higher educational level tend to report more physical and psychological symptoms related to SCD according to SCDPYS results (Anie et al.). The researchers of this study recognized the importance of demographic factors as a part of the transition program. They also recommended the need for further qualitative research to explore the transitional views of the CIY and their parents.

In another cross-sectional survey, the majority of the providers (89%) of youth with hemoglobinopathy supported the need for a transition programs (Telfair, Alexander, Loosier, Alleman-Velez, & Simmons, 2004). Two hundred twenty-seven randomly sampled health care providers of youth with SCD from two medical centers and from the National SCD Center (NSCDC) database were surveyed using an investigator-developed questionnaire: Sickle Cell Transfer Questionnaire-provider version (P-SCTQ). After two Delphi rounds reviews, a pilot draft of P-SCTQ was developed and tested by 10 providers (Telfair, Alexander, et al.). Using the bivariate and comparative content analyses, the results of the P-SCTQ denoted that majority of providers (70%) serving both adolescent and adult populations expected the patient to be seen with the parent or guardian. Conversely, 67% of adult-only providers expected the patient to be seen without the parents present (Telfair, Alexander, et al.). To diminish these inconsistencies among adult providers' expectations, the literature supports the need for transition programs that incorporate the expectations of the not only the provider but also the parent and CIY.

Transitional Obstacle: Transitional Resistance to Adult Health Care 

Transitional resistance was reflected in six studies that focused on the concerns of youth, parents, and health care providers regarding the transfer of the CIY to adult health care. Of these six studies, only one study focused on the CIY with hemoglobinopathy. Telfair, Myers, and Drezner (1994) used a cross-sectional survey to determine issues, concerns, and expectations of the 36 adolescents with SCD in pediatric care, 60 young adults with SCD, and 25 primary caretakers and providers. The investigator-developed Sickle Cell Transfer Questionnaire (SCTQ) was mailed to providers of two major medical centers (Telfair, Myers, et al.). The providers either administered the questionnaire using the interview method or mailed the questionnaire to study participants. Using the bivariate analysis, the study reported that the caretakers and youth were primarily concerned about how they would pay for the medical care, how they would be treated in the adult setting, and how to manage their mixed feelings about leaving the pediatric provider (Telfair, Myers, et al.).

Fiorentino, Phillips, Walker, and Hall (1998) also recognized mixed feelings of the disabled youth and their caregivers about their transfer to adult care in a descriptive study. Using a pre-existing database, a survey of 50 out of 159 physically disabled youth aged 16 to 24 years and their caregivers was conducted (Fiorentino, Phillips, et al.). The content analysis noted that the participants felt angry and distressed about unexpected transfers and none of the participants reported a smooth transfer to adult services because of lack of transitional preparation (Fiorentino, Phillips, et al.). Christian, D'Auria, and Fox (1999) supported study results from Fiorentino, Philips, and colleagues regarding the need to prepare the CIY based on readiness for the transfer to adult health care. A retrospective review of a purposeful sample of four teens with diabetes identified that cognitive readiness can begin as early as 12 years in the preparation for transition to adult care (Christian et al.).

Boyle, Farukhi, and Nosky (2001) also focused on transition preparation of youth with CF to adult care. The researchers surveyed 52 youth with CF and 38 parents prior to transition to adult care (pre-transition group) and 60 participants 8 to 12 months after transition to adult care (post-transition group). Comparison of survey responses between the two groups demonstrated significantly lower levels of concerns in all areas for participants who had met the adult team prior to their transition to adult health care (Boyle et al.). The investigator-developed survey revealed the areas of concerns were: leaving health care providers, meeting a new team, and the infection control policies in adult programs (Boyle et al.). The investigator-developed survey was based on the review of previous CF studies and tested by CF experts and four CF patients. In all these studies, the parents, youth, and providers surveyed had a positive attitude toward the establishment of a transition program as long as the program was based on their concerns.

Concerns about facing transition to adult care were shared by seven 12- to 17-year-old chronically ill youths (e.g., with diabetes, chronic renal failure, cancer) in a qualitative study (Soanes & Timmons, 2004). Content analysis of the interview transcripts revealed concerns regarding the importance of a familiar face, an informal adult atmosphere to promote communication, and a gradual individual/ flexible transition process with adequate preparation from supportive providers (Christian et al., 1999; Soanes & Timmons). In these studies, transitional resistance was identified by the concerns regarding leaving the health care provider, meeting a new provider, and lack of adequate transition preparation.

A retrospective chart review of 247 chronically ill young adults exhibited transitional resistance by reporting a significant increase in admissions of these adults to pediatric hospitals (e.g., to endocrine, oncology, and neurosurgery services) during a 10-year period (Lam, Fitzgerald, & Sawyer, 2005). Almost half (49%) of the adults admitted to the hospital had severe complications of their illness and had been transferred previously to an adult care provider or had a documented transition plan (Lam et al.). Regardless of whether the CIY had a documented transition plan or had been previously transferred to adult health care, many of the CIY continued to return to pediatric care. Resistance toward transition to adult heath care is an area that needs further research, especially in the CIY with hemoglobinopathy.

Discussion 

Of the 17 empirical transition studies reviewed, only five studies focused on CIY with hemoglobinopathy. Therefore, this comprehensive literature review was supplemented with other studies that focused on CIY with a variety of chronic illnesses (e.g., diabetes, CF, and neurologic disabilities). Also, no studies addressed similarities and differences in the transitional experience among various cultures (e.g., Asian, Latino, and Native American). Most of the studies were conducted in the United States and the United Kingdom, with the remainder in Canada and Australia. The majority of studies reviewed were quantitative or mixed studies utilizing investigator-constructed scales and surveys or validated questionnaires (e.g., the P-SCTQ scale). The transitional obstacles identified in the literature included the lack of transition support, limited transitional programs, and transitional resistance to adult health care. Clinical recommendations from the studies are organized in the following section according to the related transitional obstacle.

Limitations 

The limitations of this integrated review are based on the limits of the individual studies reviewed. Small sample size, purposive samples, no mention of validity or reliability on some of the investigator-developed instruments, the need to replicate the study on a larger scale, retrospective and cross-sectional data, and nonrandom samples were some of the limitations. The use of nonspecific sampling strategies, that the majority of providers interviewed were pediatric providers, that ethnic minorities were underrepresented, and the lack of sample characteristics in some studies (education, illness severity, gender, types of chronic illnesses) also limited study comparisons.

Summary 

There has been little qualitative exploration of the transition experience of CIY published in the literature (Britto et al., 2007). Telfair, Ehiri, Loosier, and Baskin (2004) reported that published empirical studies highlight the most rudimentary issues of transition, especially because the CIY's point of view remains rare. Of the 17 studies reviewed, the majority were quantitative and mixed studies that were not able to discuss the youth's transitional experience to adult health care in depth. Further research is needed to gain understanding of the transition experience of young adults in order to contribute to the establishment of transition programs. It is evident in this review of literature that there is limited research in the area of transition and chronic illness, especially among CIY with hemoglobinopathy. This empirical review contributes significant knowledge to the transition literature by identifying three transitional obstacles that emerged from the literature. The obstacles were categorized as lack of transition support, limited transition programs, and transitional resistance to adult health care with associated clinical recommendations. Most of the clinical recommendations were based on speculation and experience rather than empirical evaluation, which are areas for needed research. Therefore, to promote successful transition to adult care, further research is needed to explore the transition experience of the CIY (especially with hemoglobinopathy) and to evaluate transition programs based on clinical outcomes.