Development of quality indicators for transition from pediatric to adult care in sickle cell disease: A modified Delphi survey of adult providers

Abstract Background: The transition from the pediatric setting to adult care is a challenge for many adolescents with chronic disease. Patients with sickle cell disease (SCD) represent a unique cohort as the timing of psychosocial development of adolescence often coincides with worsening end organ damage. Previously, we used the Adolescent Autonomy Checklist (AAC) modified to include SCD specific tasks that patients with SCD need to practice in order to transition to adult healthcare and independent living. This study sought to use the AAC to measure the effects of skill based educational handouts on improving self-management and transition readiness in adolescents with SCD. Methods: This was a single center, retrospective study approved by the Washington University Institutional Review Board. Inclusion criteria were patients with SCD, age 13-21 years, and completion of pre and post assessments. As standard care, patients from a pediatric hematology clinic completed the AAC-SCD. The AAC-SCD assesses skill level in twelve domains (Table). The tool includes 100 items, and users check "can do already" or "needs practice" for each item. After review with the coordinator, participants were given skill-based handouts based on up to five noted deficits. Patients completed the AAC-SCD at the subsequent clinic visit. In addition to baseline and follow up AAC-SCD data, medical and demographic data were collected via chart abstraction. All data were entered into SPSS for statistical analysis, including descriptives, paired sample T-tests, and bivariate Pearson's correlations. Results: A total of 61 patients completed baseline and follow up. Of those participants, 49.2% were female. The mean age was 15.4 (+ 2.2) years. The genotypic distribution was as follows: 67.2% HbSS, 19.7% HbSC, 3.3% HbS-beta-thal+ and 9.8% HbS-beta-thal0. The majority of patients received healthcare coverage via Medicaid (52.5%), private insurance (45.6%) and 1.6% had no insurance coverage. Twenty-five patients (42.0 %) had a history of stroke or silent cerebral infarct and 34 (55.7%) were currently taking or were previously prescribed hydroxyurea. Formal academic support (IEP or 504 Plan) was reported for 20 (32.8%) of patients. At baseline, patients needed the most help with skills in the kitchen, housekeeping, personal care and leisure. Statistically significant improvements (p< 0.05) occurred in skills related to laundry, housekeeping, healthcare, sexual development and living arrangements. Modest sized and statistically significant correlation between the receipt of the educational handouts and decreased number of items marked "needs help" occurred in the areas of money management (r=-0.27, p=0.044), vocational skills (r=-0.27, p=0.046;) and laundry (r=0.32, p=0.015). A post hoc analysis by age groups 13-15 (n= 34),16-18 (n=24) and 19-21 (n=3) showed a decreased amount of items marked "needs help" in the areas of sexual development for both 13-15 year olds (r=0.42, p=0.024) and 16-18 year olds (r=0.93, p=0.001) as well. Conclusion: Transition skills improved over time among adolescents with SCD. While we cannot say for certain if gains in knowledge occur with age as development progresses or if a formal transition program can be credited, providing educational materials on transition related skills within a clinic setting was associated with significant improvements in three of the domains. Our preliminary data offers insight into what skill deficits may be most amenable to educational interventions based on age group. As is the case with medical management, the development of a multimodal intervention is needed to prepare adolescents with SCD to transition to adult care and independent living. Clinic based education is a simple intervention that could be one component of future approaches to transition. Disclosures No relevant conflicts of interest to declare.

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Attention difficulties are associated with lower engagement in adult care amongst youth with sickle cell disease

British Journal of Haematology ◽

10.1111/bjh.16421 ◽

2020 ◽

Vol 189 (1) ◽

Author(s):

Jeanelle S. Ali ◽

Frank Andrasik ◽

Kristoffer S. Berlin ◽

Jerlym Porter ◽

Jane Hankins ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Cell Disease ◽

Adult Care ◽

Attention Difficulties

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Rigorous and practical quality indicators in sickle cell disease care

Hematology ◽

10.1182/asheducation-2017.1.418 ◽

2017 ◽

Vol 2017 (1) ◽

pp. 418-422 ◽

Cited By ~ 1

Author(s):

Suzette O. Oyeku ◽

Elissa Z. Faro

Keyword(s):

Sickle Cell Disease ◽

Environmental Factors ◽

Quality Indicators ◽

Sickle Cell ◽

Strong Evidence ◽

Age Groups ◽

Current Status ◽

Cell Disease ◽

Stakeholder Groups

Abstract In recent years, several sickle cell–specific quality indicators have been developed using rigorous approaches. A review of the history and current status of the development of sickle cell–specific indicators highlights opportunities for future refinement. Despite efforts at alignment, lack of strong evidence hinders the adoption of current quality indicators across stakeholder groups. There are many directions in which to take the current existing quality indicators, including expanding to different age groups, aims of care such as safety and equity, and better understanding of contextual and environmental factors.

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Outcome of Transitioning Pediatric Patients with Sickle Cell Disease to Adult Programs.

Blood ◽

10.1182/blood.v104.11.3743.3743 ◽

2004 ◽

Vol 104 (11) ◽

pp. 3743-3743 ◽

Cited By ~ 3

Author(s):

Samir K. Ballas ◽

Carlton Dampier

Keyword(s):

Chronic Pain ◽

Young Adults ◽

Pain Management ◽

Sickle Cell Disease ◽

Medical Care ◽

Sickle Cell ◽

Adolescents And Young Adults ◽

Cell Disease ◽

Adult Care ◽

Female Ratio

The transition of medical care of patients with sickle cell disease (SCD) from pediatric to adult providers represents a milestone in their lives. Major concerns among adolescents and young adults about transition include taking responsibility for self, making own decisions, cost of medical care, fear of suboptimal pain management, and reluctance to leave known providers. In this study we present our experience in the process of transition to adult care and its outcome over the last ten years. Adolescents and young adults were given information about the nature of medical care provided by adult internists and hematologists. The sickle cell programs available in the city were described. Moreover, site visits to the hospitals where adult care was to be provided were arranged. During these visits, adolescents and young adults had the chance to meet the hematologist and other potential providers and ask questions, visit the emergency room, the clinic, and the sickle day unit if applicable. Patients were empowered to choose the program to which they wished to be transitioned. During the last 10 years, 90 adolescents and young adults (See Table) with SCD (Sickle Cell Anemia [SS], Hemoglobin SC Disease, and Sickle Thalassemia [ST]) were transitioned to the adult sickle cell program of Thomas Jefferson University. Age of transition varied between 18 and 25 years. Eighteen patients (20%) died. Age at death was 24.9 ± 2.95 years and the male/female ratio was 10:8. Complications of sickle cell disease after transition included leg ulcers, stroke, avascular necrosis, anxiety, depression, and priapism. Nineteen patients (10 males, 9 females) were employed. Twenty-nine (32%) patients developed chronic pain syndrome and its sequelae. Many patients failed to achieve their childhood goals. The data show that a significant number of patients die within 10 years after transition. The quality of life of survivors is suboptimal and drifts into issues of chronic pain management in the adult environment. Identifying these issues may provide predictors that identify children at risk to have undesirable outcomes after transition. Aggressive management and refining the process of transition should improve the outcome after transition. Distribution of the Transitioned Patients SS SC ST Total Male 31 8 4 43 Female 34 8 5 47 Total 65 16 9 90

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Web-Based Technology to Improve Disease Knowledge Among Adolescents With Sickle Cell Disease: Pilot Study (Preprint)

10.2196/preprints.15093 ◽

2019 ◽

Author(s):

Anjelica C Saulsberry ◽

Jason R Hodges ◽

Audrey Cole ◽

Jerlym S Porter ◽

Jane Hankins

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Participation Rate ◽

Self Management ◽

Cell Disease ◽

Adult Care ◽

Web Based ◽

Disease Knowledge ◽

Transition Readiness ◽

One Step

BACKGROUND Advancements in treatment have contributed to increased survivorship among children with sickle cell disease (SCD). Increased transition readiness, encompassing disease knowledge and self-management skills before transfer to adult care, is necessary to ensure optimal health outcomes. The Sickle Cell Transition E-Learning Program (STEP) is a public, Web-based, 6-module tool designed to increase transition readiness for youth with SCD. OBJECTIVE The objective of our study was to investigate the participation rate of youth with SCD in STEP and its association with transition readiness. METHODS This was a single-center, Institution Review Board–approved, retrospective cohort review. A total of 183 youths with SCD, aged between 12 and 15 years, were offered STEP as an adjunct to in-clinic disease education sessions. Participation rate (number of patients who used at least one STEP module divided by those approached) was calculated. The association among the number of STEP modules completed, disease knowledge, and self-management was explored. RESULTS Overall, 53 of the 183 approached adolescents completed at least one STEP module, yielding a participation rate in STEP of 29.0%. Of the 53 participants, 37 and 39 adolescents had disease knowledge and self-management confidence rating available, respectively. A positive correlation (<italic>r</italic>=0.47) was found between the number of STEP modules completed and disease knowledge scores (<italic>P</italic>=.003). No association was found between the number of modules completed and self-management confidence ratings. Disease knowledge scores were significantly higher among participants who completed ≥3 STEP modules compared with those who completed <3 STEP modules (<italic>U</italic>=149.00; <italic>P</italic>=.007). CONCLUSIONS Improvement in disease knowledge in adolescence is critical to ensure the youth’s ability to self-care during the period of transition to adult care. Despite low participation, the cumulative exposure to the STEP program suggested greater promotion of disease knowledge among adolescents with SCD before transfer to adult care.

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A Patient-Centered Approach to Gathering Information on Social Determinants of Health from Patients with Sickle Cell Disease in Preparation for Their Transition to Adult Care

Blood ◽

10.1182/blood-2021-147963 ◽

2021 ◽

Vol 138 (Supplement 1) ◽

pp. 4943-4943

Author(s):

Katherine EC Smith ◽

Jessica R Cox ◽

Rosalind L Haynes ◽

Nicole Ivette Bohnker ◽

Kay L Saving

Keyword(s):

Sickle Cell Disease ◽

Community Health ◽

Social Determinants Of Health ◽

Sickle Cell ◽

Social Determinants ◽

Determinants Of Health ◽

Transition To Adult Care ◽

Cell Disease ◽

Adult Care ◽

Patient Centered

Abstract Background: Transition from pediatric to adult care has been a recent focus of sickle cell programs across the country due to patients with sickle cell disease (SCD) to living longer. 1 Transition programs for other chronic conditions focus largely on patient knowledge of the disease, treatments, and appointment compliance but for patients with SCD, social determinants of health (SDOH) must also be considered when guiding patients toward adult care. 2 Historically, lack of acknowledgement of the importance of relationships and trust building when addressing SDOH has led to barriers in patient responsiveness. 3 To facilitate this aspect of transition, our recently hired Community Health Worker (CHW) developed a Community Health Transitional Questionnaire (CHTQ) and conducted a Quality Improvement (QI) project to address SDOH in a patient-centered manner during transition to adult care visits at the clinic. Aims: Refine the CHTQ by gathering the "voice of the customer" to provide a more patient-centered approach to transition to adult care for patients with SCD. Complete the CHTQ with 70% of transition-aged (12-25 years) patients by 7/1/21. Methods: The CHTQ consists of 32 questions about SDOH in 8 categories (Food, Housing, Money/Finances, Transportation, Education/Work, Family and Other Support, Stress Prior to/After COVID, and Sleep). Thirty pediatric sickle cell patients were identified as being within the established transitional age group. A series of Plan-Do-Study-Act (PDSA) cycles were used to refine the CHTQ. Through patient/caregiver feedback, small increments of change were used to establish a CHTQ that would improve patient care and be accepted by the patients/caregivers. PDSA #1: During regularly scheduled appointments, two patients and their caregivers met with the CHW who described the purpose of the CHTQ and then asked the patient to privately complete the CHTQ. PDSA #2: Because of feedback received from PDSA #1, the CHW allowed the next two patients to have caregivers present while answering the CHTQ, but explained that ideally the patient should be able to answer the CHTQ without input from their caregiver before transition to an adult environment. PDSA #3: Because of the feedback received from PDSA #2, the CHW accompanied the clinic RN to the exam room and worked the CHTQ into the nursing check-in questions in an informal manner making the questions seem more routine. Results: After 3 PDSA cycles, 17 additional patients/caregivers completed the CHTQ without issues or concerns. A total of 22/30 (73%) CHTQs met the goal of completion by 7/1/21. One additional CHTQ has been completed since. Using the information from the CHTQ, the team has been able to better address individualized SDOH for each patient. For example, one area of concern identified was transportation with 9/23 (39%) patients expressing concerns getting to & from appointments and/or school/work. Transportation needs were then discussed with the clinic social worker who is now working with those patients, giving them tools to arrange transportation. Conclusion For SCD patients, it appears imperative that a relationship be established prior to inquires about SDOH. Using an informal patient-centered approach with the CHTQ was more likely to gain patient trust and allowed the CHW to obtain information needed to assist the patient with SDOH issues. Once SDOH issues were identified through the CHTQ, the CHW was able to find patient-driven, individualized resources addressing those concerns. References DeBaun, M.R. & Telfair, J. (2012). Transition and Sickle Cell Disease. PEDIATRICS, 130(5), 926-935 https://doi.org/10.1542/peds.2011-3049 Andermann, A. (2016). Taking action on the social determinants of health in clinical practice: a framework for health professionals. Canadian Medical Association Journal, 188(17-18), E474-E483. https://doi.org/10.1503/cmaj.160177 Cheney, C., Pecci, A., & Porter, S (2020, May). Social Determinants of Health: Lead or Partner. HealthLeaders. https://www.healthleadersmedia.com/clinical-care/social-determinants-health-lead-or-partner-0 Disclosures No relevant conflicts of interest to declare.

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Impact of a paediatric-adult care transition programme on the health status of patients with sickle cell disease: study protocol for a randomised controlled trial (the DREPADO trial)

10.21203/rs.2.16608/v1 ◽

2019 ◽

Author(s):

Delphine Hoegy ◽

Nathalie Bleyzac ◽

Alexandra Gauthier-Vasserot ◽

Giovanna Cannas ◽

Angélique Denis ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Coping Skills ◽

Morbidity And Mortality ◽

Care Transition ◽

Cell Disease ◽

Adult Care ◽

Biopsychosocial Approach ◽

Adult Transition ◽

And Coping

Abstract Background: Thanks to the advancements in medical care, a majority of sickle cell disease patients worldwide live beyond 18 years of age, and therefore patients initially followed in paediatric departments are then transferred to adult departments. This paediatric-adult care transition is a period with an increased risk of discontinuity of care and so morbidity and mortality. During this period, the patient will have to manage new interlocutors and places of care, and personal issues related to the period of adolescence. To take into consideration all these aspects, an interesting approach is to refer to the patient as a whole system as presented in biopsychosocial approach. The aim of this trial is to evaluate the impact of the proposed biopsychosocial paediatric-adult transition programme. Methods: The DREPADO study is a multicentre randomised control trial comparing a control group ( Arm A ) versus an interventional group with paediatric-adult transition programme based on a biopsychosocial approach ( Arm B ). To be included, patients should suffer of SS, SC or Sβ-form of sickle cell disease and aged between 16 and 17 years. The randomisation in a 1:1 ratio assigns the Arm A or B . The primary outcome is the number of hospital admissions and emergency in the index hospital for complications, in the 2-years after the first consultation in the adult department of care. Secondary outcomes consider the quality of life, but also included coping skills such as self-efficacy feeling and disease knowledge. To provide patient and parent knowledge and coping skills, the transition program is composed of 3 axis : educational, psychological and social, conducted in individual and group.Discussion: By providing self-care knowledge and coping skills related to SCD and therapeutics, helping patient's empowerment related to pain management and emotions and facilitating the relationship to oneself, others and care in the Arm B of DREPADO study, we believe that the morbidity and mortality of patients with SCD may be reduced after the proposed paediatric-adult transition programme. Trial registration: ClinicalTrials.gov, ID: NCT03786549; registered on 17 th December 2018; https://clinicaltrials.gov/.

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Supplemental Material for Understanding Barriers to Transition From Pediatric to Adult Care Among Young Adults With Sickle Cell Disease to Develop a Transition Mentor Program

Clinical Practice in Pediatric Psychology ◽

10.1037/cpp0000372.supp ◽

2020 ◽

Keyword(s):

Young Adults ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Mentor Program ◽

Cell Disease ◽

Adult Care

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