Adult Care In Sickle Cell Disease Research Articles

Patient, Caregiver, and Provider Perceptions of Quality Care for Inpatient Sickle Cell Disease Care: A Scoping Review of Qualitative Literature

Background: The experiences of patients with sickle cell disease (SCD) who receive care in the outpatient and emergency department settings have been well-explored, resulting in the implementation of guidelines ensuring that patients with SCD receive high-quality care in those healthcare settings. However, there remains limited guidance on what constitutes high quality inpatient SCD care despite most patients with SCD of all ages having at least one hospitalization per year. Our overall goal is to use mixed methods scoping review methodology to identify potential inpatient SCD quality of care indicators. The goal of this study is to identify qualitative research examining inpatient SCD care, focusing on the experiences of patients with SCD, their caregivers, and their healthcare providers that should inform inpatient SCD care quality of care indicators. Methods: We search seven databases including CINAHL, Embase, PsycINFO, PubMed, Scopus, Web of Science, and World Health Organization for relevant articles using a search algorithm developed by a research librarian. Using an online collaborative software designed for conducting literature reviews, a team of independent reviewers screened the titles and abstracts of 9,938 articles for inclusion, with two reviewers screening each article. Articles were selected for inclusion if they were qualitative in nature and related to the experiences of patients (or their family members/providers) with SCD while admitted to an inpatient service. Articles were excluded if they related to the care of patients with SCD solely within the ED, or if they related to the care of pregnant patients with SCD. We resolved discrepancies by discussion among reviewers, with final decisions on inclusion made by the senior author. We completed full text review of included articles with manual data extraction (Table). We plan to next conduct a thematic analysis by organizing all identified themes from the included articles into sub-themes, which will then be categorized into parent themes as defined by the Donabedian Model. Decisions for clusters of sub-themes will be reached by team consensus. Results: Initial literature search retrieved 20,819 studies with removal of 10,906 duplicates (Figure). Of the remaining 9,913 studies that underwent title and abstract review, we identified 186 for inclusion in full text review. Fourty-three studies were included in data extraction, of which only 13 were recent (published between 2013-2023). The majority of studies explored patient perspectives on inpatient care of SCD (n=31), whereas a smaller number of studies included caregiver (n=12) and healthcare provider (n=10) perspectives. Several studies explored multiple stakeholder perspectives (n=8). Of the studies that explored healthcare provider perspectives, both nurses (n=7) and physicians (n=9) were represented. Only two studies included social workers in their study population, and none included case managers or pharmacists. Of the studies that explored caregiver perspectives, a majority focused on caregivers of children with SCD (n=10), while fewer focused on caregivers of adults with SCD (n=4). The majority of studies addressed adult SCD care only (n=24), while 11 studies included only pediatric inpatient SCD care. 8 studies included both pediatric and adult patient populations. The most common study locations were the United States (n=25) and the United Kingdom (n=11). Other study locations included Brazil (n=3), Bahrain (n=2), Uganda (n=2), Canada (n=2), Cameroon (n=1), and Jamaica (n=1). Some studies included multiple countries. Conclusions: Existing qualitative literature about inpatient SCD care is concentrated on the patient experience. Studies regarding healthcare providers' perspectives primarily included nursing and physician perspectives with a notable absence or underrepresentation of other interdisciplinary team members. It is notable that while African countries have the highest global burden of SCD, only a small number of qualitative studies examined inpatient SCD care in those countries. In next steps, we will use deductive thematic analysis of extracted qualitative data to identify potential structural, process, and outcome measures for inpatient SCD care from patient, caregiver, and healthcare provider perspectives.

Changing Trends in Sickle Cell Disease-Related Mortality in the United States over Four Decades

Introduction: Sickle cell disease (SCD) causes significant morbidity and early mortality. The Cooperative Study of Sickle Cell Disease estimated median life expectancy for individuals with SCD at 42 years for males and 48 years for females in the early 1990s. However, this calculation was made prior to now standards-of-care in SCD, including routine transcranial doppler screening, pneumococcal prophylaxis, and the introduction of the primary disease-modifying therapy hydroxyurea and the widespread use of chronic blood transfusion. Over the last thirty years, with expanded access to disease modifying therapies, increased evidence exists for the preservation of organ function and less frequent acute vaso-occlusive pain. It remains unclear whether the introduction of these therapies has affected overall SCD-related mortality or the specific causes of death of those with SCD in the United States, particularly in the last decade when the use of hydroxyurea has expanded most significantly. Methods: We used the US mortality statistics for 1979-2020 available publicly through the CDC WONDER database (http://wonder.cdc.gov), which is a compilation of county, state, and national-level mortality statistics derived from death certificates, including age category at death, sex, race, ethnicity, year of death, underlying cause of death, and additional contributing causes using ICD-9 codes for 1979-1998 (282.6 sickle cell anemia- all SCD genotypes were included under one code in ICD-9) and ICD-10 codes for 1999-2020 (D57.0 sickle cell anemia with crisis; D57.1 sickle cell anemia without crisis; D57.2 double heterozygous sickling disorders; D57.8 other sickle cell disorders). We searched the SCD ICD-10 codes listed as underlying cause of death by five-year age groups for Black individuals and all ethnicities, (from Hamideh and Alvarez, Pediatric Blood and Cancer 2013) and compared the crude death rates overall and for each age group to those of 1999-2009. We also searched using the SCD ICD-10 codes included in “the multiple causes of death” to determine the 15 most common ICD categories of death in each 10-year period. Results: For the period 2010-2020, 5272 Black (Hispanic & non-Hispanic) individuals died in the United States with the underlying cause of death listed as one of the ICD-10 SCD codes (see Figure 1A for a map of the crude mortality rate distribution). The crude mortality rate was 1.1 per 100,000 Black individuals, which was significantly lower than the period 1999-2009 (crude rate 1.2 per 100,000, p < 0.0001) (Figure 1B). Of note, similar to prior trends, there is a significant increase in mortality rate in the 15-19 year age group versus 20-24 years (0.9 per 100,000 to 1.4 per 100,000, p < 0.0001) corresponding with the age of transition to adult centers. The mean age at death for all genotypes for 2010-2020 was 43 years (females: 44 years, males: 41 years) versus 39 years for 1999-2009 (females: 40 years, males: 38 years). For the period 2010-2020, 61% of cases listed SCD as the primary cause of death without specificity or proximal cause; for the other 39% of cases when an underlying cause of death was noted, cardiovascular disease was the most common (28%), followed by accidents (7%), cerebrovascular disease (7%), malignancy (6%), septicemia (4.8%), and renal disease (3.8%). The fifteen most common causes of death accounted for 90% of underlying causes of death listed in the SCD population. Conclusion: The sickle cell disease-related mortality rate has significantly declined over the last 40 years, including in the period 1999-2020, corresponding with the introduction of disease modifying therapies, improved SCD care, and decreased infant mortality. This overall improvement, however, provides contrast to the enduring sharp increase in mortality in the young adult years. This demonstrates the critical need to improve the transition process from pediatric to adult care and to emphasize the need for additional support for young adults after they transition to adult SCD care.

Family planning needs of young adults with sickle cell disease.

Sexual and reproductive healthcare standards for adolescents and young adults with sickle cell disease (SCD) are not established. A total of 50 young adults entering adult SCD care completed a Family Planning Survey assessing sexual and reproductive health needs from March 2019 to July 2020. Clinical data were abstracted from respondents' electronic medical records. Linear and logistic regression was applied to explore associations between clinical characteristics and survey results. Few respondents (8%) wished to be pregnant in the coming year, and 46% answered yes to at least one of four needs assessment questions. Those who were not employed full time were more likely to endorse needing help with getting sickle cell trait testing for a partner (ORadj=9.59, p-value=0.05). Contraceptive use was associated with having an obstetrician-gynecologist (OR=6.8, p-value=0.01). Young adults with SCD entering adult care have diverse reproductive health needs, highlighting opportunities to provide multidisciplinary, SCD-specific reproductive healthcare.

Transition Readiness Assessment of Adolescents with Sickle Cell Disease and Their Caretakers at the Sickle Cell Clinic in Kumasi, Ghana

Background: Sickle Cell Disease (SCD) is associated with high child mortality in sub-Saharan Africa (SSA). Improved medical services within an established SCD program in Kumasi, Ghana have focused on enhanced newborn screening and comprehensive pediatric SCD care (Ohene-Frempong et al., 2008). These efforts have improved SCD survival and increased the need for adolescents to transition to adult care. We sought to understand patient and caretaker perspectives on transition to adult care for adolescents with SCD visiting the Komfo Anokye Teaching Hospital Sickle Cell Clinic (KATH SCC) in Kumasi, Ghana, where the transition occurs by age 15 years.Methods: Structured interviews were conducted with adolescents aged 12-15 years and adults who accompanied them to KATH SCC. Interviews queried 4 areas that are relevant for assessing transition readiness in the U.S.: SCD medical knowledge, symptom self-management, psychosocial impact, transition preparation. Interview questions were adapted from two surveys that assessed transition readiness of adolescents with SCD in the U.S. (Telfair et al., 2004; Treadwell et al., 2016) and modified to fit the Ghanaian context. Interviews were conducted in English or Twi (the prevalent local language), per participant preference. Responses were transcribed during the interview, and later translated into English, as needed, by one interviewer (MKS) for descriptive statistics and qualitative analysis.Results: In total, 46 children (mean age 13 years) and 46 accompanying adults were consented (or assented) and interviewed. Most participants selected Twi for interviews. Adults were either parents (70%) or close relatives, with mean age 39.8 years (range 18-70). SCD type was determined by clinic records: 59% of children interviewed had HbSS, 35% HbSC, and 6% had no SCD type recorded. Most children and their caretakers had some knowledge about SCD and management: 61% of children knew that SCD is a blood disorder, while 74% knew that SCD is an inherited disorder. Majority of children knew what to do to ease pain in crisis (61%), where to go for care (85%), what care is helpful (72%), and could engage with the hospital team independently (87%). Gaps in knowledge were evident for up to one third of children for each question. Their responses were corroborated by at least 46% of adults. Significantly fewer children (39%) knew their SCD type compared to the adults (63%) (p=0.02). Children who reported knowing how to handle a pain crisis used a combination of analgesics, folic acid, penicillin, and a local ointment, but did not understand that folic acid and penicillin are maintenance medications that do not relieve painful episodes. Children appeared more concerned than adults about their current health and social stigmatization than about future health-related challenges. Seventy-six percent of children worried about having SCD, 28% felt like burdens to family, 37% told their friends about having SCD, but 96% had plans for their future. Adults had different perspectives of disease burden, where only 48% and 6% believed their child worried and felt like a burden, respectively. Few children (35%) and adults (37%), were aware of the requirement to switch to adult care by age 15, but most (72% or 74%) were willing to transition when asked to do so.Conclusions: To the best of our knowledge, this is the first study of perspectives about care transition for adolescents with SCD and their caregivers in SSA. Our clinic-based assessment at a single site suggests that the youth and their adult caregivers are informed about SCD but lack information relevant to adequate medical care, are concerned about peer stigmatization and were unaware of the need to transition. Challenges to successful transition in the country may include the relatively young age of transition and gaps in knowledge needed for successful self-management. Future efforts to improve transition to adult SCD care may need to include education of adolescents and families about the disease, self-management, and transition and address sense of self-burden to families and stigmatization among peers. Generalizability of our findings and practical methods to address needs for SCD transition within Africa remain to be tested.Acknowledgments: Support to MKS by David E. Rogers Student Fellowship Award from New York Academy of Medicine and IFAP Global Program of Columbia University. We thank KATH SCC staff for facilitating this research. DisclosuresNo relevant conflicts of interest to declare.

Transition Experiences of Adolescents and Young Adults (AYA) with Sickle Cell Disease at the Ghana Institute of Clinical Genetics (GICG), Korle Bu in Accra, Ghana

Background: Sickle Cell Disease (SCD) is a significant global health problem. Previously considered an illness restricted to childhood especially in low-resource areas, more patients are surviving into adulthood. Expanding SCD services in Ghana has improved survival.(Ohene-Frempong K., Pediatrics 2008) Thus, the period of transitioning from pediatric to adult SCD care is important. This period presents challenges that transition preparation could help address.(Quinn C., 2010) We sought to understand past transition experiences of adolescents and young adults (AYAs) at the Ghana Institute of Clinical Genetics (GICG), an adult SCD clinic. We hypothesized that receiving prior transition preparation would positively influence participants' transition experiences to the adult clinic. Methods: Structured interviews were conducted with AYAs ages 13 to 22 years receiving routine SCD care at GICG. Participants were asked to recall transition preparation on the pediatric side, emotions, concerns and overall satisfaction with transition experience, and current perception of SCD self-care abilities. Questions used in the structured interviews were informed by existing SCD transition surveys and focus groups and modified to be culturally appropriate for Ghana.(Telfair J., 1994, Sobota A., 2015) Interviews were conducted in English or Twi per participant preference. Responses were transcribed and translated into English, as needed, for summary and comparative statistics. Results: One hundred participants were consented, interviewed and included in the analysis, mean age 17.9±2.9 years and 65% female. The majority had HbSS (77%), 30% HbSC and 3% not readily available. Average age of first visit at the adult clinic was 14.4±2.8 years. Only 29% of participants indicated receiving some type of transition preparation. Of these, 52% visited the adult clinic with pediatric staff, 34% participated in a transition program on the pediatric side, and 24% had a visit with the pediatrician without parents present. Prior to their actual transition, participants in the transition preparation group were more likely to report having had positive emotions (happy, comfortable) about needing to transition to adult care than those without transition preparation (55% vs 32%, p = 0.03). No other statistically significant differences were found between the two groups' responses to concerns prior to transition, challenges faced after they started attending the adult clinic, and overall satisfaction with their transition experience. Overall, 85% of participants reported having initial challenges at GICG upon transition, including not knowing the staff on the adult side and being unfamiliar with speaking to doctors on their own. Despite those difficulties, (61%) reported positive emotions after their first adult visit, and 64% reported feeling satisfied with their overall transition experience because they became familiar with the staff and experienced short wait times at visits. Over half of participants were very confident in their SCD self-care abilities. Only 25% were very confident about dealing with the frustrations of having SCD and 62% did not feel comfortable telling their peers they have SCD due to stigma. Most participants (92%) believed their health would improve in the future. Conclusions: As reported elsewhere, transition preparation among Ghanaian AYAs at our single study site is highly variable. Most study participants reported no transition preparations prior to adult care. Regardless of preparation received, participants had concerns and challenges with transitioning. Similar to our previous study of younger patients prior to transition, concerns about peer stigmatization persisted in this sample of AYA patients. (Kwarteng-Siaw M., 2017) Despite this, our study shows a modest but significant benefit in emotions of AYAs receiving transition preparation for adult SCD care. These findings emphasize the importance of transition services in SCD care in Ghana. Generalizability of our findings and practical methods to address needs for SCD transition within Africa remain to be tested. Disclosures No relevant conflicts of interest to declare.

Emergency Department Encounters, Hospitalizations and ED Reliance Among Medicaid Eligible Patients with Sickle Cell Disease in North Carolina

Introduction: Sickle cell disease (SCD) is a complex disease for which pain is the hallmark. Pain from vaso-occlusive episodes is the number one reason for ED visits and hospital admissions. This paper reports Medicaid claims data from NC for individuals with SCD, including: 1) ED encounters and re-encounters within 7, 14 and 30 days; 2) hospitalization and re-hospitalization within 7, 14 and 30 days; and 3) ED reliance (EDR) score. Methods: We examined Medicaid claims data from for patients with a diagnosis of SCD (ICD 9 CM codes: 282.6x, ICD 10 CM codes: D57.0x, D57.1, D57.2x, D57.4x, D57.8x) in North Carolina. Data is reported for a cohort of 2,790 patients with a diagnosis of SCD, age 1 to 65+ and enrolled at least 11 months in NC Medicaid between March 1, 2016 and February 28, 2017. ED re-encounters and re-hospitalizations within 7, 14 and 30 days were identified using the time between the date of service listed on the ED or hospital claim and the next date of service in the subsequent claim. Individual ED Reliance (EDR) score was calculated as the total number of ED encounters divided by the total ambulatory visits (outpatient + ED encounters) per enrollee, (ambulatory visits reported elsewhere). Similar to Kroner et al, an EDR of >0.33 was considered a high score. Inpatient claims were identified using a category of service code indicating hospitalization. Results: The participants in the sample (n=2790) were majority female (57.92%), lived in metropolitan areas (77.63%) and had a mean age of 23.05 years old (SD=16.06). Of the 9,075 total ED encounters, 69.86% of the total sample had an ED encounter during the 12-month study period. There was a mean of 3.25 (SD=7.38) and median of 1 (IQR = 0 - 3) ED encounters per patient for the sample. Those who were 18-30 years old had the highest mean and median ED encounters per patient (4.98, SD= 9.34 and 2, IQR 1 to 5). The 31-45 year old group had the second most, with 4.82 (SD= 11.03) total ED encounters. The percentage of the sample with an ED re-encounter within 7, 14, and 30 days was also highest among the 18-30 year old group (29.17%, 33.98% and 40.89%) followed by those 31-45 years old (23.71%, 28.49%, and 34.80%), respectively. The 31-45 age group had the second most hospitalizations/patient and re-hospitalizations. The mean EDR was highest among 18-30 year old patients (0.35) and 46.48% of this age group had an EDR of 0.33 or greater. In the 31-45 year-old age group, the mean EDR was 0.28 and 35.18% had an EDR of 0.33 or greater. The overall sample had a mean of 1.30 (SD= 2.75) hospitalizations/patient. The 18-30 year old age group also had the highest mean total hospitalizations (2.08, SD= 3.72) and mean re-hospitalizations within 7 (0.16; SD=0.77), 14 (0.41; SD=1.68), and 30 (0.82; SD=2.79) days. The 31-45 age group had the second most hospitalizations/patient and re-hospitalizations (Table 1). Conclusions: Overall, increasing age coincided with increased ED and inpatient utilization, as well as with the period of transition from pediatric to adult SCD care. Furthermore, high EDR was most prevalent in the 18-30 age group. Our study further supports the need for increased focus on acute care utilization in the 18-45 year-old age group and considerations for improved care transition interventions. Disclosures Tanabe: NIH: Research Funding; AHRQ: Research Funding. Shah:Novartis: Consultancy, Research Funding, Speakers Bureau; Alexion: Speakers Bureau; GBT: Research Funding.

Feasibility of implementing mobile technology-delivered mental health treatment in routine adult sickle cell disease care.

Sickle cell disease (SCD) is a severe hemoglobinopathy characterized by acute and chronic pain. Sufferers of the disease, most of whom are underrepresented minorities, are at increased risk for mental health disorders. The purpose of this study is to test the acceptability and implementation of a computerized cognitive behavioral therapy (cCBT) intervention, Beating the Blues, to improve depression, anxiety, and pain in patients with SCD. Adults with SCD and significant symptoms of depression (Patient Health Questionnaire [PHQ-9] score ≥ 10) or anxiety (Generalized Anxiety Disorder Scale [GAD-7] score ≥ 10) were eligible to participate and be randomized to either receive eight sessions of cCBT with care coach support or treatment as usual. Participants reported daily pain and mood symptoms using a mobile diary app. Depression, anxiety, and pain symptoms were assessed at 1, 3, and 6 months. Thirty patients were enrolled: 18 to cCBT, and 12 to control. The cCBT intervention was feasible to implement in clinical settings and acceptable to participants. Patients in the cCBT arm reported a marginally greater decrease in depression at 6 months (-3.82, SE = 1.30) than those in the control group (-0.50, SE = 1.60; p = .06). There were no significant effects of treatment on anxiety; however, cCBT was associated with improved daily pain reported via a mobile diary app (p = .014). cCBT, delivered via mobile device, is a feasible strategy to provide mental health care to adults living with SCD. cCBT was acceptable to the target population; was able to be implemented in real-world, nonideal conditions; and has the potential to improve patient-reported outcomes.

Trajectories of Sickle Cell Disease Severity during Transition to Adult Care

BackgroundAdolescents and young adults (AYAs) with sickle cell disease (SCD) are at increased risk for disease complications and mortality, particularly during transition to adult care. Little is known about the disease course that might be contributing to the increase in morbidity and mortality during transition. This study provides a longitudinal description of the clinical characteristics and disease severity among AYAs with SCD during transition to adult care.MethodsWe conducted a longitudinal analysis of the electronic health records of 339 AYAs (ages 12-27 years) with SCD (97% black, 56% male, 69% hemoglobin SS) who received care at a comprehensive SCD center in the Southeast US between 1989 and 2015. Measures included sociodemographics, transfer, complications, comorbidities, and severity of SCD (using a modification of the pediatric SCD severity index).We used group-based trajectory modeling to identify subgroups of AYAs with distinct severity trajectories. AYAs who were in care for at least 10 years were included (n=133). We used chi-square and unpaired student t-test to explore subgroup differences among (1) AYAs who died compared to AYAs who were still alive during the study; (2) AYAs who were eligible to transfer (i.e., ≥ 19 years) (n=293) and transferred compared to AYAs who were eligible to transfer and did not transfer; and (3) AYAs with different severity trajectories. Statistical significance was set at p=0.05 and 95% CI.ResultsCommon complications among AYAs (n=339) included vaso-occlusive crises (80%), acute chest syndrome (41%), chronic pain (35%), and cerebral infarcts (22%). Comorbidities included depression (19%) and anxiety (14%). Most AYAs who were eligible to transfer transferred (n=220, 75%) at a mean age of 19 years (SD=1.3). Fourteen AYAs died, 10 (71%) after transfer at a mean of 7.3 years (SD=3.1) from transfer.SCD Severity Trajectories (Figure 1): Group-based trajectory modeling identified both stable and increasing severity trajectory groups: stable-low (n=31, 23%), stable-medium (n= 61, 46%), stable-high (n=6, 5%), low-increasing (n=13, 10%), and medium-increasing (n= 22,17%) severity trajectory groups. Most AYAs (74%) had stable severity over time, whereas 26% had increased severity with increasing age.Subgroup Differences in Care and Mortality: AYAs who died (n=14) spent less time in pediatric care (mean=4.6 years, 95%CI 3.7-5.6), however, had a higher mean number of pediatric annual visits (mean=9.3 visits; 95%CI 6.3-12.3) compared to those who were alive (n=324, mean=6 years in pediatric care, 95%CI 5.7-6.1; mean number of pediatric annual visits=5.4, 95%CI 4.8-6.1). There were no differences in demographics, SCD complications, or morbidity.Subgroup Differences in Transfer to Adult Care (Tables 1 and 2): Compared to AYAs who did not transfer, AYAs who transferred were older and lived closer to the SCD clinic. AYAs who transferred were at higher risk for SCD complications and comorbidities. They were more likely to be receiving hydroxyurea. They had higher mean annual pediatric SCD clinic visits, especially during older adolescence, and were in pediatric care for a longer duration.Subgroup Differences in Disease Severity (Tables 3 and 4). Compared to AYAs in stable severity trajectory groups (Figure 1: Groups 1, 3, and 5), AYAs in unstable disease severity trajectory groups (Figure 1: Groups 2 and 4) were more likely to be married, older, and lived closer to the SCD clinic. AYAs in unstable groups were also at higher risk for SCD complications and comorbidities. They were less likely to be receiving treatments and had less pediatric SCD clinic visits by age 18. However, they were more likely to transfer to adult care, have had more adult SCD visits overall, and remained longer in adult SCD care after transfer.ConclusionsWhereas most AYAs with SCD had stable disease severity, nearly a quarter had increasing disease severity, over time. AYAs with increasing severity lived closer to the SCD clinic, were more likely to transfer to adult care, and demonstrated higher and longer use of adult SCD care compared to AYAs with stable disease severity. Genotype was not associated with disease severity trajectory groups, underscoring the importance of clinical care for AYAs, over time. [Display omitted] DisclosuresKayle:Department of Health and Human Services, Administration for Community Living, NIDILRR Advanced Rehabilitation Research Training Health and Function Grant #90AR5019 (PI Heinemann): Other: post-doctoral fellowship; Department of Health and Human Services, Administration for Community Living, NIDILRR Advanced Rehabilitation Research Training Health and Function Grant # 90ARHF0003 (PI Heinemann): Other: Postdoctoral fellowship. Tanabe:Duke University: Employment; NIH and AHRQ: Research Funding; Alliant Health: Consultancy. Maslow:The Crohn's and Colitis Foundation and the American Gastroenterological Association, in collaboration with Pfizer, Inc: Research Funding. Holl:Before Brands, Inc: Research Funding; NIH: Research Funding; Branstad Family Foundation: Research Funding; AHRQ: Research Funding. Shah:Novartis: Research Funding, Speakers Bureau.

Transition to adult care in sickle cell disease: A longitudinal study of clinical characteristics and disease severity

Sickle cell disease (SCD) is a chronic blood disorder in which mortality has increased for adolescents and young adults (AYA). A longitudinal analysis of medical records was conducted to describe the clinical course among AYAs (ages 12-27 years) during transition to adult care. Measures included sociodemographic, complications, SCD severity (modified pediatric SCD severity index), comorbidities, and transfer. Group-based trajectory modeling (GBTM) to identify subgroups with distinct severity trajectories and chi-square and unpaired Student t test to explore subgroup differences were used. Overall, 339 AYAs (97% black, 56% male, 69% hemoglobin SS) had 10848 clinic, 3840 hospital, and 3152 emergency department visits. Complications included vaso-occlusive crises (80%) and acute chest syndrome (41%). Comorbidities included depression (19%) and anxiety (14%). Most AYAs transferred to adult care (n=220) at 19 years. Fourteen AYAs died, 10 within seven years from transfer. GBTM identified both stable and increasing severity trajectory groups: stable-low (n=31, 23%), stable-medium (n=61, 46%), stable-high (n=6, 4.5%), low-increasing (n=13, 10%), and medium-increasing (n=22, 17%). AYAs with increasing severity (25%) were older, lived closer to the clinic, and had higher risk for SCD complications and comorbidities. They had fewer pediatric clinic visits; however, they were more likely to transfer and remain longer in adult SCD care. Whereas most AYAs had stable severity, nearly a quarter had increasing severity, over time. AYAs with increasing severity had more complications, were more likely to transfer to adult care, and demonstrated higher and longer adult SCD care utilization compared with AYAs with stable severity.

Defining "Community" from the Perspectives of Individuals with Sickle Cell Disease in Rural Georgia.

Individuals with sickle cell disease (SCD) often struggle to transition from pediatric to adult-centered medical environments. One probable cause is that many transition programs do not focus on what happens when patients leave the medical environment and return to their communities. Little is known about how individuals with SCD define community. Therefore, we worked with health care providers at four rural Georgia SCD outreach clinics to conduct qualitative interviews with 21 individuals who had transitioned to adult SCD care. The biopsychosocial ecological model was the framework for the study. Findings indicated that individuals with SCD describe five aspects of community that span across the family, community, and society-levels of the biopsychosocial ecological model: 1) immediate family, friends, and social circle; 2) relationships with medical providers; 3) geographic community/neighbors; 4) church/spiritual support; and 5) society. Interventions designed to improve SCD in rural communities may be most effective if they include not only support from family but also enhanced neighborhood supports and links between adults with SCD and relevant community organizations.

Using Qualitative Perspectives of Adolescents with Sickle Cell Disease and Caregivers to Develop Healthcare Transition Programming.

Youth with sickle cell disease (SCD) are living longer, requiring transition from pediatric to adult health care. Transition programs have been created to improve transition readiness and help patients take responsibility for their health. The aim of this study was to explore the usefulness of current transition materials and identify unmet transition needs from the perspective of adolescents with sickle cell disease (SCD) and caregivers to refine transition programming and interventions. Focus groups were conducted with 14 adolescents with SCD (Mean age = 14.6 years, SD = 1.9) and 20 caregivers (Mean age = 43.2 years, SD = 9.3) to gather perspectives about transition to adult care, current transition program materials and recommendations for future programming. Four themes emerged: (a) transition skills and knowledge needs, (b) change in health care responsibility, (c) concerns with adult SCD care, and (d) useful transition readiness strategies and resources. The findings of this study were used to develop Web based educational modules, experiential transition skills learning, and an adolescent and caregiver hematology support group. Findings highlight the need to conduct periodic readiness assessments, provide opportunities and scaffolding to learn skills based on readiness level, and help build social support networks to encourage and facilitate learning.

Identification of risk factors for an unsuccessful transition from pediatric to adult sickle cell disease care

A successful transition from pediatric to adult sickle cell disease (SCD) care is paramount to continued improvements in survival. In order to enhance transition success, our pediatric SCD transition process was modified to include combined adult and pediatric provider clinics that incorporated participation by our local SCD community-based organization. All children ages 16 and over participated in this newly-formed transition program. After 5 years of implementation of the modified SCD transition program, we retrospectively studied clinical and non-clinical risk factors for an unsuccessful transition. Risk factor categories studied included patient demographics, transition clinic attendance, and disease severity. Thirty-two percent of patients did not transition successfully. Demographic factors such as gender, race, and type of insurance did not influence transition outcome, although travel distance to the adult SCD center was an identifiable risk factor for an unsuccessful transition. While transition clinic attendance rate did not affect transition outcomes, older age at first modified combined transition clinic visit was a significant risk factor for lack of transition. Patients with clinical markers of milder disease severity (SC and Sβ(+) genotypes and no chronic transfusion therapy) were at higher risk for an unsuccessful transition than patients with severe disease. We have identified several risk factors for lack of transition success which will allow us to modify our transition efforts going forward to capture this highest risk subset.

An Evidence-Based Approach to the Treatment of Adults with Sickle Cell Disease

The application of evidence-based medicine to the management of adults with sickle cell disease (SCD) is currently primarily driven by clinical expertise and patient preference, as there is a paucity of randomized controlled trial (RCT) data to guide decision-making. A summary of SCD management principles in the areas of health care maintenance, transfusion therapy, treatment and prevention of painful episodes, acute chest syndrome, stroke, renal disease, contraception and pregnancy, and priapism is predominantly based on the authors' interpretation of available observational studies as well as the opinions of experts in SCD. RCTs impacting current practices address use of hydroxyurea to prevent painful episodes and acute chest syndrome, intensity of pre-operative transfusion, transfusion during pregnancy, and angiotensin-converting enzyme inhibitor therapy for proteinuria, but most issues in adult SCD care have not been rigorously studied and management may not be appropriately extrapolated from pediatric data. While challenging clinical problems need to be addressed by RCTs, there is also the need for development of practice guidelines using formal methodological strategies. This brief review is not a substitute for the process but provides a literature-based approach to making treatment decisions when caring for adults with SCD.