Sickle Cell Disease Access-to-Care Summit 2018

Electronic version available at GBT.com.

Sponsored by Global Blood Therapeutics, Inc Sickle Cell Disease Access-to-Care Summit 2018 1 M-UNB-US-00050 V1.0 Table of Contents 2 Abbreviations

3 Introduction

6 Enhancing Care Within the Institution: Establishing a Sickle Cell Disease Clinic and/or Day Hospital for Comprehensive Care Management

11 Utilizing Existing Care Infrastructure: Leveraging Available Capacity by Partnering with Non-sickle cell disease Stakeholders

16 Transitioning Adolescent Care: Structuring Transition Between Pediatric and Adult Care Settings

21 Expanding Care Outside the Institution: Building a Community Outreach Model to Extend the Reach of Care

27 References

Abbreviations CBO=community-based organization DH=day hospital ECHO=Extension for Community Healthcare Outcomes ED=emergency department HCPs=healthcare providers NBS=newborn screening PCP=primary care provider SMART=Specific, Measurable, Achievable, Relevant, and Time-bound US=United States

2 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 this context of institutional and systemic racism,1 “Of all the forms of inequality, people living with the disease face a system of injustice in health care is the parallel, fragmented care2 that rests on an unwieldy most shocking and inhumane.” infrastructure and a healthcare environment riddled - Dr. Martin Luther King, Jr. with injustice. A Steering Committee of eight stakeholders from Introduction academia and business (Figure 1) formulated the The Sickle Cell Disease Access-to-Care Summit, program and set the objectives which were to: held on September 12, 2018 brought together  Identify and discuss existing access-to-care committed stakeholders from across the United models used successfully to improve healthcare for States to explore ways of improving the lives of both adults and children with sickle cell disease people living with the disease who labor under a  Create draft roadmaps of successful access-to- myriad of burdens, blockades and beliefs that have care models by involving stakeholders from across weighed upon African Americans for four centuries. the healthcare spectrum Moreover, as a chronic illness sickle cell disease  Develop implementation roadmaps suitable for brings additional hardships and challenges that are dissemination through existing channels to local unbounded by race, religion or national origin. In and regional access-to-care initiatives

Figure 1. Steering Committee Members

Biree Andemariam, MD Payal Desai, MD Associate Professor, Department of Medicine Director, Sickle Cell Program Director, New England Sickle Cell Institute Director, Sickle Cell Research and Connecticut Bleeding Disorders Center Assistant Professor of Internal Medicine University of Connecticut Division of Hematology Farmington, CT The Ohio State University Columbus, OH Andrew Campbell, MD Director, Comprehensive Sickle Cell Program John J. Strouse, MD, PhD Children’s National Medical Center Director of the Adult Sickle Cell Program Associate Professor of Pediatrics Associate Professor of Medicine and Pediatrics George Washington University School of Duke University School of Medicine Medicine and Health Sciences Durham, NC Division of Hematology Washington, DC Julie Kanter, MD Kim Smith-Whitley, MD Director, Adult Sickle Cell Program Director, Comprehensive Sickle Cell Center at University of Alabama at Birmingham Children’s Hospital of Philadelphia Birmingham, Alabama Clinical Director, Division of Hematology Associate Professor of Pediatrics Perelman School of Medicine at the University of Pennsylvania James R. Eckman, MD Philadelphia, PA Professor Emeritus, Hematology & Oncology Emory University School of Medicine Atlanta, Georgia

Kenneth Bridges, MD Vice President, Medical Affairs and Principle Medical Director Global Blood Therapeutics South San Francisco, CA Founder and Former Director, Joint Center for Sickle Cell and Thalassemic Disorders Brigham & Women’s and Massachusetts General Hospitals Boston, MA

Sickle Cell Disease Access-to-Care Summit 2018 3 M-UNB-US-00050 V1.0 Access-to-Care challenges were addressed under programs and institutions built on a funding infra- four overarching headings: structure that includes private, local, state, and  Expanding care within the institution: establishing federal sources. The focus of healthcare often is a sickle cell disease clinic and/or day hospital for short-term with problems addressed most often on comprehensive management a year-to-year basis, an approach best suited to  Utilizing existing care infrastructure: leveraging acute issues of limited duration. Acquiring adequate available institutional capacity by partnering with healthcare can be challenging for people who are stakeholders outside the sickle cell disease arena deft within the system and well-resourced. For  Transitioning adolescent care: structuring transition those who are poor and marginalized the problem is between pediatric and adult care facilities daunting. For poor, marginalized people with chronic  Expanding care outside the institution: building illness the mountain is almost insurmountable. outreach models to extend the range of care into the community and beyond Sickle cell disease is a complex, chronic illness affecting approximately 100,000 Americans (Figure Participating stakeholders included patient advocates, 2). The multifaceted disorder evolves over decades health care professionals (HCPs), hospital admin- in character and manifestations. Except for the istrators, as well as representatives of professional limited option of bone marrow transplantation, no medical societies and government healthcare agen- cure exists. The problems are further exacerbated cies. The Steering Committee created draft roadmaps by the fact that most physicians are unfamiliar and/ to kindle group discussions, the output of which or uncomfortable with sickle cell disease manage- is distilled into the guidance material below. The ment.3,4 Episodes of acute vaso-occlusive pain resulting implementation roadmaps are freely avail- are the hallmark of sickle cell disease and over able to interested stakeholders for use and adaptation the course of a year these drive most patients into to individual circumstances. an emergency department (ED) on one or more The US healthcare system is a patchwork of occasions.5,6 Unfortunately, the ED is an option of

Figure 2. Sickle Cell Disease in the US

SCD in births: Number of individuals with SCD out of Black or African 15 every 36 American births ~100,000 AND in Hispanic every American 1 16,300 births

Sickle cell trait Life expectancy for those with sickle cell disease is million reduced by 1 carry sickle cell trait

in Black or African 1 13 American births ~30 years

4 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 last resort for many who struggle with sickle cell Importantly, reductions in hospitalizations and length disease7 due to that fact that many ED providers of stay by DHs can substantially reduce the cost of harbor negative attitudes about people with the care for patients with sickle cell disease.15 By chan- disease8 who consequently often experience inade- neling patients into an alternate management setting, quate disrespectful and even humiliating treatment. DHs potentially can free ED facilities and personnel The sad sequela for patients suffering with excruci- to focus on problems they handle best, such as ating pain commonly are longer waits than people major trauma or acute myocardial infarction.19 9 with other pain issues and frustrating delays in Drs. Andemariam and Eckman summarize the 10 analgesic administration. When treatment finally implementation roadmap for establishing DHs and/ arrives, relief often is minimal and fleeting due to or sickle cell disease treatment clinics. Starting 11 suboptimal dosing despite evidence that applying such programs often involves tapping into other guidelines for higher opioid dosing improves pain departments at an institution whose resources can 12 outcomes and decreases hospitalizations. be shared. Comprehensive Cancer Centers are an Adding to the misery, the whirlwind created by example where synergy possibly exists, particularly the US opioid crisis has pulled people with sickle in the hematologic malignancy sections where staff cell disease into its violent vortex.13 Policy makers are familiar with issues involving the blood-forming have responded to the debacle by implementing elements. Drs. Desai and Strouse address this guidelines for opioid analgesic administration that approach to expanding access-to-care for people are restrictive and sometimes draconian. Patients with sickle cell disease. suffering with sickle cell disease commonly require Sickle cell disease is a chronic disorder that results opioid analgesics to manage the severe pain from a single amino acid substitution in the hemo- resulting from compromised blood flow through the globin molecule. Clinical manifestations begin in microcirculation. Some need very high doses of infancy and extend into adulthood. The life-saving these medications. However, opioid use by patients intervention of childhood prophylactic penicillin with sickle cell disease often is more moderate was the catalyst for sickle cell disease newborn than daily opioid doses for patients with other pain screening programs which currently exist in each 14 syndromes. Nevertheless, people with sickle cell state. Efficient screening technology combined with disease frequently are stereotyped as drug-seeking effective treatment created a moral imperative to malcontents in a confrontational setting where implement sustained and comprehensive care for appropriate treatment of legitimate pain symptom- children with sickle cell disease. Although the lifespan atology frequently is sidelined. for patients with sickle cell disease lags the average Day Hospitals (DHs) are an alternative approach for African Americans, survival has increased beyond to treatment that avoids many of the shortcomings that reported in the Cooperative Study of Sickle Cell 20 of ED management. A dedicated facility staffed by Disease, with improved childhood survival contrib- HCPs familiar both with the clinical manifestations uting significantly to the better numbers. of sickle cell disease and its treatment provides a Young adults generally transition from pediatric to stable setting for comprehensive management of a adult healthcare systems between the ages of 18 complex disorder. Intensive treatment of vaso-oc- and 25 years. Unfortunately, the dearth of benign clusive pain in DHs consistently reduces the rate hematology providers has created a crisis in the of hospital admission for uncomplicated episodes management of adults with sickle cell disease.21 relative to the ED.15,16 Moreover, DHs can evaluate The problem is exacerbated by infrastructure gaps uncomplicated vaso-occlusive pain crises and begin common in the adult world. The integrated, multidis- analgesics much more rapidly than occurs in the ciplinary approach to disease management that is ED.17 Consequently, patient satisfaction is greater a bedrock in pediatrics sadly is a rarity for adults.22 with DH treatment leading many patients to seek Children and parents come to see the pediatric these facilities in lieu of the ED.18 In fact, patients clinic as a haven that at times serves nearly as a often manage pain at home while waiting for DH second home. Young adults are understandably facilities to open rather than seeking ED treatment. anxious when considering where they will receive

Sickle Cell Disease Access-to-Care Summit 2018 5 M-UNB-US-00050 V1.0 treatment in the adult world and by whom.23 Most Enhancing Care Within the Institution: patients endeavor to remain in the pediatric environ- Establishing a Sickle Cell Disease ment for as long as possible. Unfortunately, delay Clinic and/or Day Hospital for 24 places patients at risk of having no transition at all. Comprehensive Care Management The issue of pediatric to adult transition for patients Unpredictable episodes of severe acute pain in with sickle cell disease is complex with multiple childhood that evolve later in life into chronic, often components that include the number of patients debilitating pain syndromes are the sine que non of served and the proximity of adult and pediatric sickle cell disease. Episodes of pain become more facilities. Drs. Campbell and Smith-Whitley address frequent in older youth and young adults resulting this central challenge in access to care. Importantly, in frequent ED visits and recurrent hospitalizations. even geographic location within the country can This section of the monograph defines the rationale 25 influence the outcome of transition. The road to for DH treatment, outlines preparations, discusses adult care sadly has more potholes for patients in identification of resources, guides planning for some regions of the country. implementation, and defines metrics that justify the Geography plays another unfortunate and even program and support quality improvement (Figure 3). broader negative role in access-to-care for people Rationale for the Establishment of a with sickle cell disease. In 2010, 55 percent of black Dedicated Unit for Care of Individuals with Americans lived in the South, and 105 Southern Sickle Cell Disease counties had a black population of 50 percent or Suboptimal care in EDs for individuals with sickle 26 higher. Rural healthcare has traditionally lagged cell disease is one of the most common complaints that available in urban areas. The picture has by patients and families. The challenge is exacer- changed dramatically over the past decade due to bated by those ED providers who harbor negative a general transformation of health care financing, attitudes towards individuals with sickle cell disease8 the introduction of new technologies, and the clus- often labeling them as “drug seeking” or referring to tering of health services into systems and networks. them by the offensive term, “sickler”.33 In addition, Despite these changes, resources for rural health communication between ED personnel, the primary 27 systems remain relatively insufficient. Many rural care physicians and sickle cell specialist is often communities continue to experience shortages of suboptimal. Adding to the challenge, new regulatory physicians, and the proportion of rural hospitals guidelines in response to the opioid epidemic have under financial stress is much greater than that of prompted many EDs and inpatient providers to restrict urban hospitals. follow-up opioid prescriptions given at discharge, For people with sickle cell disease living in rural further compromising treatment of pain episodes. areas the challenges are greatly amplified. In addition to providing expert care, DHs create a Utilization of services is directly related to socio- supportive environment that fosters trust between economic conditions that patients face and clinic patients and providers. The first event in the distance.28 Patients with sickle cell disease in creation of a DH is the emergence of a cham- rural South Carolina without access to compre- pion to lead the program; second, identification of hensive care, for instance had a higher rate of resources; third, creation of a plan of action and acute care utilization and readmission.29 Not timeline; and finally, definition of metrics for ongoing surprisingly, patient dissatisfaction often is high assessment and quality improvement. due to long wait times for care that is seen as suboptimal.30 Telemedicine31 and tele-mentoring32 Taking the First Step: Identify a Champion are new approaches that harness technology to The first and most important step in developing extend sickle cell disease management expertise a dedicated acute care unit for individuals with from urban centers into rural regions. Dr. Kanter sickle cell disease is the emergence of a champion addresses this vexing problem in care access for for patients with sickle cell disease at the health- people with sickle cell disease. care facility. This most often is a physician with

6 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 Figure 3. Roadmap for Creating a Sickle Cell Disease Clinic and/or Day Hospital

Preparation  Issues to Address  Baseline Data Needs  Major Milestones to and Sources Define  Barriers to Anticipate  Develop Timeline

Recruitment/  Champions  Potential Funding  Space Resources Sources  Stakeholders  Staff Requirements

Action  Identify and plan space  Continuous program Planning assessment and  Develop operating restructuring procedures  Recruit and train appropriate personnel Access Metrics  Redevelop/refine action  Care pathways plan as needed

Metrics/  Number of patients  Inpatient days Measurement  Number of acute visits  Readmissions  Number of health  Patient satisfaction maintenance visits  Patient quality of life  Clinical data  Costs  Number of admissions an interest in sickle cell disease but may be any political attention to ED care or a preventable nega- committed HCP. In some cases, the initial champion tive outcome for a patient. These opportunities can is a patient or parent who demands improvement often be used as leverage to facilitate the establish- in the emergency care of individuals with sickle ment of an acute care center. cell disease by the healthcare facility. In Atlanta, the 12-hour, 7 day-a-week ED-dedicated sickle Develop a Business Plan cell disease unit at Grady Memorial Hospital was Preparation begins with determining feasibility of championed by a parent who lead the parent/patient the program by developing a business plan. The group and reached out to the Dr. James Eckman, initial step is identifying the nature and extent of Director of the Adult Sickle Cell Program, to orga- services that will be provided in the dedicated nize the development of the new unit. Another acute care area. This must include the types of example is the University of Connecticut Health services that will be available, hours of operation, System where Dr. Biree Andemariam championed as well as procedures for smooth interactions with 18 the Sickle Cell Program and DH. the ED, inpatient areas, laboratory, blood bank and radiology. Existing resources should be identified Join Hands with the Community including staff and space. Ideally, planning for optimal treatment for sickle pain crises and other acute care issues should begin The current population served should be estimated immediately in every health system or community including numbers of patients registered in the with significant numbers of patients with sickle cell existing sickle cell program, seen in the ED, disease. Pressure from patients and their families admitted to the inpatient services, and seen in other can prod the health system to action, often after outpatient clinics. Estimates of growth potential complaints to the administration bring media or local include opportunity for increased referrals from the

Sickle Cell Disease Access-to-Care Summit 2018 7 M-UNB-US-00050 V1.0 community, numbers of pediatric patients approaching Health system administration usually leads overall transition, and estimates of regional population size plans for allocation of space, financial oversite, and travel distances. Often, regional and statewide administrative structure, and assessment of hospital consortia and departments of health maintain outcomes. The sickle cell champion, patients and utilization data that can inform population estimates. caregivers, impacted healthcare providers, nursing, Partnering with local community-based organizations social services, pharmacy, and laboratory services (CBOs) can also serve as a referral resource for develop the personnel needs, general methods of patients not in active comprehensive care. operation, patient flow, and outcome quality metrics. Financial data to plan feasibility includes an esti- An action plan outlines detailed planning for the mate of current costs, charges and reimbursement acute care facility. Hours of operation and numbers for emergency department, outpatient, and inpatient of beds required will determine if the facility will be services. Baseline data should identify insurance integrated into an infusion center or observation types including Medicaid, Medicare, commercial facility in the emergency department or become a insurance, and percentages of uninsured in the free-standing clinic. Other areas that may support existing patient population. Potential cost improve- such clinics include ED observation units, bone ment can be estimated from projected decrease in marrow transplant clinics, and transfusion units. admission rates, reduced length of stay, preven- Details of space and allocation will be required to tion of unnecessary diagnostic and therapeutic determine patient flow, integration into the sickle cell interventions, and increased hydroxyurea use and continuity clinic, transfers to and from the emergency adherence to therapy. department, procedures for transport to laboratory and radiology facilities, and admission protocols. Get Your Plan Approved Procedures, protocols, and storage for medications, Once data are generated in these preparatory activ- opioids, intravenous fluids, blood products and ities, a meeting of all impacted stakeholders should infusion/patient-controlled analgesia pumps need determine if the project will go forward. Advocacy to be defined. The location should be accessible for by patients and caregivers, community sickle cell patients and families with adequate waiting rooms organizations, advocacy groups, funding entities, and facilities for families and other care givers. managed care organizations, and politicians can help make the project a reality. Health system Personnel requirements include the core physicians administration, the sickle champion, representatives and physician extenders, nurses, clerical support, of nursing, pharmacy, radiology, laboratory, the and medical assistants who will staff the acute care emergency department, and inpatient services need facility. Support staff enable providers to focus fully to approve both moving forward and the scope of on patient care thereby improving continuity, maxi- the acute care facility. mizing efficiency, and reducing provider burnout. Personnel need to be identified to provide social Identify Resources, Recruit Stakeholders, support, psychological interventions, and education. and Develop an Action Plan Ideally, a dedicated social worker should be hired, Approval of the concept of a dedicated acute care preferably one with a mental health therapy exper- facility such as a day hospital initiates detailed plan- tise and familiarity with the psychosocial stressors ning. A formal planning group of stakeholders must endured by many patients. be established that includes health system adminis- tration, the sickle cell champion, patients/caregivers/ Secure and Maintain Funding CBOs, impacted healthcare providers, nursing, A detailed financial plan should be co-developed by social services, pharmacy, laboratory services and the administration and the sickle cell champion. The information technology. This group will determine basis for the financial projections is a cost-saving required space needs, personnel resources, time- model based on existing resources. Financial plans line for planning and implementation, finances and often are justified by reducing losses, an important cost accounting, as well as interactions between consideration to financial administrators to which other health system services. clinicians must be attuned. Sickle cell disease

8 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 programs often are money-losing cost centers of treatment intervention for acute pain episodes because some segments of the population are unin- and other complications can attract funding from sured or underinsured leading to inadequate levels pharmaceutical companies that can indirectly of reimbursement. For example, inpatient stays that support the patient management infrastructure.34 exceed diagnosis-related group reimbursement produce profound inpatient losses. Unlike inpatient Identify and Train Personnel stays, outpatient costs are not bundled so that Personnel requirements of sickle cell disease outpatient losses are lower relative to the inpatient acute care units are challenging. The basic team setting. Sickle cell disease programs are often essential to evaluation and treatment of acute pain justified by reducing losses rather than increasing episodes are the physician, physician extenders and profits. These are systemic constraints that must be nurses. Key ancillary support staff includes medical understood and accepted by the system’s financial assistants, social workers, mental health special- leadership. Even modest improvements in average ists, counselors, chaplains, and care managers. length of stay can translate into large cost savings Personnel resources for data collection and anal- provided the resources on the outpatient side are ysis will facilitate ongoing evaluations of quality sufficiently robust to prevent admission in the first improvement. Once staffing needs are outlined, an place, reduce the likelihood of a readmission within environmental scan can define the scope of respon- 30 days, or to insure proper post-discharge clinical sibility and job descriptions. follow-up including assessment of social contrib- The recruited and trained personnel form the core utors to long lengths of stay (e.g., lack of stable team in the unit. Recruiting within the health system housing). Availability of efficient acute care can will facilitate training because such individuals are facilitate earlier discharge and reduce readmissions, already familiar with operations. Key to a successful thus providing other indirect cost savings. program is developing and maintaining a team Billing and reimbursement from third-party payers with empathy towards individuals with sickle cell should be maximized. Cost centers need to reflect disease and their caregivers who also are invested the total financial profile of the sickle cell patient in improving overall care for people living with population including revenue from pharmacy, labo- sickle cell disease. A medical village must also be ratory, and imaging services in addition to direct identified to provide needs for specialty support billing for encounters. Apheresis has the potential and ancillary services. Additional champions within to bring in substantial revenue. Consider embed- this village will ideally come from the other service ding apheresis into the new center or, if apheresis lines essential for the operation of the sickle cell is “owned” by another service such as transfusion unit such as the ED, hospitalist groups, hematology/ medicine, be certain to include that downstream oncology, nursing, transfusion medicine, obstetrics revenue in the cost center. and pharmacy.

Additional external funding sources have been Develop Operating Procedures exploited by several sickle cell disease programs to and Individualized Care Plans support special sickle cell treatment facilities. Direct Operating procedures must be developed for admin- state legislative support can be a funding source istration, nursing services, care protocols, and record obtained by advocacy with support of legislators like keeping. Procedures for transferring patients to the the Legislative Black Caucus. Private foundations ED and inpatient services must also be codified. may also support demonstration projects focused Protocols for management of medications, opioids, on improving healthcare for orphan diseases. Other intravenous fluids, as well as environmental issues philanthropic sources to leverage include grateful should be outlined in an operation manual along with patient families and private donations. The health assignment of responsibilities. General patient care system’s business development staff and legislative protocols are needed for all patients cared for in the liaisons should endeavor to identify and secure new unit. These care pathways summarize the general avenues of financial support. The unique ability of medical needs and opioid management issues sickle cell disease care programs to conduct studies unique to patients with sickle cell disease.

Sickle Cell Disease Access-to-Care Summit 2018 9 M-UNB-US-00050 V1.0 Ideally, these care pathways should be individual- ciency, and support ongoing quality improvement. ized into care plans for each patient treated in the Baseline data can be used to justify initiating unit. Plans should outline specific medical problems program services based on projections of utilization and ongoing approaches to management. Plans and future growth. Data collected during operation should cover management of acute pain episodes, can document financial impact, improvement in chronic pain, transfusions, and other ongoing care, and patient satisfaction. medical issues. Care plans must be multidisciplinary Baseline data includes number of patients in the with input from the medical team, social service, sickle cell clinic and health system, as well as the pain management specialists, and psychology. number utilizing the ED and living in the region Specific individual protocols for opioid use with served. Current insurance profiles of the popula- appropriate monitoring are needed to address tion and distances from the center are important. management of acute and chronic pain. Ongoing Data are needed on current numbers of clinic visits, communications with ED staff along with updates ED visits, admissions, average length of stay, and or changes to plans are vital to maintaining an 30-day readmission rates. Important baseline effective program. clinical data include time to treatment and admis- A mechanism should exist that makes the care sion rates in the ED, visit rates per patient, the rate plan available throughout the health system and at which health maintenance visits to the clinic are especially in the ED, inpatient units, and primary kept, adherence to treatment, and hydroxyurea care clinics. These should be reviewed regularly utilization. Surveys of patient satisfaction, quality of and modified as the clinical situation changes. life, social functioning, and economic status should Specific protocols are necessary for periods of be collected early in implementation. increased frequency of pain episodes, pregnancies, and medical complications. The care plan should As the program develops, these parameters be highlighted in the communications tab of the should be regularly reevaluated along with ongoing electronic health record and should include contact program costs and revenues. Morbidity and information for the primary provider/champion. mortality rates in the population should also be documented. There should be ongoing assessment A medical community for each patient is developed of referral sources of new patients including self-re- that includes all the providers required to address ferrals, transition from pediatrics, consultations and primary care, chronic disease management, and referrals from the community, primary care, and specialty needs for individuals with sickle cell other specialists. disease. Even if existing providers are not well- versed in the management of sickle cell disease, These data can justify ongoing support for the ongoing clear communication and education can program by the health system. The data are also quickly enhance their level of comfort. Often, simply used for ongoing quality assessment and improve- reassuring the collaborating providers that they ment in all aspects of operation. Experience in will not be required to coordinate chronic pain and the Grady Health System in Atlanta, Georgia and opioid prescription management makes them more University of Connecticut Health System in the receptive to serving as engaged practitioners upon Greater Hartford region of Connecticut document whom the sickle cell program can count. the critical importance of such activities in justifying, maintaining and expanding those programs. Such Follow Metrics to Refine Action Plan data also document and support ongoing quality and Maintain Institutional Support improvement in services. Experience with managing dedicated acute care facilities for sickle cell disease demonstrates the Conclusions importance of collecting metrics in planning, imple- Day hospitals utilizing appropriate approaches menting, managing, and improving care. Careful to managing sickle pain episodes reduce time to thought is required in developing data management pain control, rate of hospitalization, length of stay, activities to document the impact of the program, and overall costs in adults.15,35-37 Experiences from justify maintenance and expansion, improve effi- Atlanta and Hartford demonstrate that integrating

10 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 such services into comprehensive care requires a community settings. They excel in health mainte- champion who initiates early advocacy and prepa- nance but generally are ill-suited to manage the ration; recruits and organizes adequate resources; acute or emergency issues that are integral to develops an action plan and timeline; and identifies the care of people with sickle cell disease. Health appropriate metrics and collection strategies. Care maintenance is valuable, however, in preventing pathways to assure rapid initiation of appropriate some of the co-morbidities that trigger acute issues pain management are critical to improving patient for patients with sickle cell disease. Moreover, PCPs outcomes and reducing hospitalization rates. at FQHCs are well-positioned for surveillance and Individualized patient-specific comprehensive care long-term care of issues such as hypertension, plans reduce emergent outpatient episodes, admis- diabetes and other important chronic conditions not sion rate, length of hospitalization, and readmission connected to sickle cell disease. Federally Qualified rates. Ongoing collection of quantitative and qualita- Health Centers therefore can be important partners tive patient reported data is critical for justifying the in care delivery for these patients. program and for cycles of quality improvement that enhance both patient outcomes and program cost Preparation and Program Construction effectiveness. Money, space, and staffing commonly are a triple barrier to the creation of facilities to address the Utilizing Existing Care Infrastructure: healthcare needs of people with sickle cell disease. Less obvious than lack of space and people are Leveraging Available Capacity by inertia and acceptance of the status quo. More often Partnering with Non-sickle cell disease than malice, belief in the adequacy of existing care Stakeholders derives from a failure to appreciate the challenges Comprehensive care for people living with sickle cell faced by patients with sickle cell disease and the disease has important infrastructure requirements degree to which “standard” practice fails to provide including, but not limited to, a venue for outpatient quality care. Institutional change is difficult and and inpatient care along with DH/infusion/acute care requires careful planning and coordination. treatment facilities as alternatives to general ED Leveraging existing infrastructure first requires management. These facilities are manned by key assessment of where within the institution surplus personnel including hematologists and advanced capacity might exist and the degree to which that practice providers who coordinate with other capacity is compatible with the care requirements providers such as nurses, social workers and of patients with sickle cell disease. The programs pharmacists in the delivery of quality care. within medical centers most frequently compatible An earlier section of this monograph covers plan- with management of sickle cell disease are compre- ning for a day hospital and/or acute care treatment hensive cancer centers (Figure 4). Oncologists facility for patients with sickle cell disease. The (sometimes) and hematologist/oncologists (often) expense of creating new facilities for a small patient receive training in management of benign hemato- group with specialized needs can, however, be logic conditions including sickle cell disease. Most daunting. In the case of sickle cell disease, some often, such training is not reinforced in subsequent institutions have addressed the problem success- oncology practice, where hematologic disorders fully by leveraging available capacity within the overwhelmingly are malignancies. However, a existing medical center infrastructure, such as foundation exists that can support high-quality cancer or hemophilia treatment centers. Table 1 care when sickle cell treatment is added to existing highlights the pros and cons of these approaches to oncology programs. expand care for patients with sickle cell disease. Bone marrow suppression and associated cytope- Another intriguing approach currently being nias are primary adverse effects of chemotherapy, explored is collaboration with Federally Qualified meaning that red blood cells, neutrophils and plate- Health Centers for the delivery of quality care to lets are part of the daily dialogue of allied healthcare patients with sickle cell disease. Federally Qualified personnel in oncology centers. Although the focus Health Centers generally are in easily accessible of laboratory interpretation differs for patients with

Sickle Cell Disease Access-to-Care Summit 2018 11 M-UNB-US-00050 V1.0 Table 1. Partnering with Existing Infrastructure to Expand Care for Patients with Sickle Cell Disease

Partner Pros Cons

 Common at medical centers  High cost value to patient-treatment  Familiarity with blood-related issues chairs    Pain management expertise Scheduled rather than episodic or acute chair use  Infusion facilities Comprehensive  Transfusion expertise Cancer Center  Pharmacy availability  Laboratory testing  Patient-treatment chairs  Psychosocial support services

 Familiarity with blood-related issue  High cost value to patient-treatment  Infusion facilities chairs    Pharmacy availability Uncommon at medical centers  Lack of pain management expertise Hemophilia Treatment  Laboratory testing Center  Patient-treatment chairs  Management of episodic/acute events  Psychosocial support services

 Community location  Lack of episodic/acute clinical  Low infrastructure and treatment management expertise cost  Lack of pain management expertise Federally-Qualified  Orientation toward health  Unfamiliarity with blood-related Health Center maintenance disorders  Lack of patient-treatment chairs  Lack of infusion facilities sickle cell disease, advanced practice providers exists since sickle cell disease treatment programs and nurses in oncology centers are facile with the are not profitable. The argument that quality sickle elements of blood panels. Moreover, transfusion, a cell disease care reduces losses associated with common intervention for sickle cell disease, is an inte- management of these patients might resonate with gral part of patient management in oncology centers. the leadership. In the end, however, the case rests on the humanitarian value of doing the right thing. Another model of infrastructure sharing involves hemophilia treatment centers. As a benign hemato- In addition, issues related to regulations and char- logic condition, hemophilia is included in the same ters are important in considering cancer centers or subspeciality as sickle cell disease. Pain manage- hemophilia centers as sites of sickle cell disease ment commonly is a point of overlap between sickle care. The charters of comprehensive cancer cell disease and hemophilia due to recurrent bleeds centers maintain that the programs exist to treat cancer patients, which is an understandable stipu- into large joints with the clotting disorder. lation to ensure that funds directed toward cancer A key challenge to leveraging infrastructure care and research are used for that purpose. resources in comprehensive cancer centers or Flexibility exists in these guidelines, but the leader- hemophilia treatment centers is convincing lead- ship of the cancer center must be willing to use it. ership to address and remove barriers to sickle Similarly, an important aspect of funding for hemo- cell disease care (Table 2). No financial incentive philia treatment centers involves use of the 340B

12 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 Drug Discount Program in which drug manufacturers provide of the 340B program is to allow covered outpatient drugs to eligible health care organizations and entities to stretch scarce federal resources covered entities at significantly reduced prices. The intent as far as possible to reach more eligible patients and provide more comprehen- sive services. Hemophilia treatment with Figure 4. Overlap of Resource Requirements for Patient Care biologic agents is the centerpiece of hemophilia programs and naturally their operations are aimed at patients with hemophilia. Some flexibility exists but willingness to use it is the key. As noted in the Introduction, pain manage- ment is central to the care of people with SCD Cancer Hemophilia sickle cell disease. Pain control is also a Patient Patient Patient common issue for patients with cancer. Opioid administration and management Treatment Chairs    are not intimidating for cancer center Infusion facilities    personnel even when drug doses substan- tially exceed those used in routine patient Blood Bank    care. Nevertheless, oncology staff are not immune to prejudices around opioid use    Pharmacy and misuse that often stigmatize patients with sickle cell disease. Training and Pain management    education are needed to address the false Radiology    belief that high opioid requirements are de facto evidence of addiction. Management   acute/episodic Program Start-up events Convincing medical center leadership of the feasibility of a sickle cell clinical program requires data and a business Table 2. Leveraging Available Health Center Resources plan (Table 2). An outline of the nature and for Sickle Cell Disease Care: Overcoming Barriers scope of the planned services is the first  Business plan step. New programs most often focus on  Stakeholder interest acute care services with routine clinic care  Financial losses associated with as a subsequent or ancillary component. management of sickle cell disease Once the scope of the operation is fixed, Issues to   Data on sickle cell patient management staffing requirements can be determined. Address at the parent institution Usually, a physician-champion drives  Charters on patients served at compre- hensive cancer or hemophilia centers the creation of services. However, the  Convincing leadership of value of covering program cannot run without support from patients with sickle cell disease other physicians, advanced practice providers, nurses, and administration.  Alignment of departments within the A plan for identifying and hiring new institution staff or reassigning existing staff is vital.  Coordination with the emergency Furthermore, training to create both Barriers to department Anticipate competence and confidence for providers  Lack of education/knowledge about sickle cell disease new to sickle cell disease care is essential to success. Plans to integrate ancillary  Staff hiring or reassignment services including laboratory, blood bank,

Sickle Cell Disease Access-to-Care Summit 2018 13 M-UNB-US-00050 V1.0 and radiology are important to program operation. emergency department, outpatient, and inpatient Fortunately, most such services are integral to services. Baseline data should identify insurance cancer centers and hemophilia programs abrogating types including Medicaid, Medicare, commercial the need for extensive restructuring. insurance, and uninsured percentages in the current A crucial difference between cancer care and patient population. Potential cost improvement can acute management of sickle cell disease is the be estimated from projected decreased rate of ED ratio of planned versus urgent infusion visits. Most visits and hospitalizations, reduced length of stay, treatment chairs in cancer infusion suites are filled prevention of unnecessary diagnostic and thera- with patients who are scheduled days or weeks in peutic interventions, and increased hydroxyurea use advance. In contrast, acute sickle cell pain episodes and adherence to therapy. are sporadic and unpredictable, creating a problem Once data are generated in these preparatory when planning availability of treatment chairs. activities, the impacted stakeholders should meet Chairs may be empty on some days while on others the number of patients exceeds capacity. The latter to determine how the project will proceed (Table 3). issue requires coordination for rerouting patients to Advocacy by patients and caregivers, community the ED. Empty chairs highlight a key financial issue sickle cell organizations, funding entities, managed that overhangs the arrangement. Chemotherapy care organizations, and politicians can help make infusions and other cancer treatments fill chairs with the project a reality. Buy-in to the acute care facility patients whose treatment is profitable for the center. should come from health system administrators Each chair occupied by a patient with sickle cell and the sickle cell disease champion, along with disease is however a potential net loss for the insti- representatives of nursing, pharmacy, radiology, tution. Frank discussion is needed around the issue laboratory, ED, and inpatient services. of profits and losses associated with reassignment of resources. Table 3. Leveraging Available Health Center The nature and extent of services that will be Resources for Sickle Cell Disease Care: provided in the dedicated acute care area must Key Partners also be defined. Considerations include the types  Medical Staff of services that will be available, hours of operation, Direct Care  Nursing Staff as well as procedures for smooth interactions with  Emergency Department the ED, inpatient areas, laboratory, blood bank and radiology. Existing resources should be identified  Social Services including staff and space.  Pharmacy Care Support The active sickle cell patient population should  Laboratory Services be estimated including numbers of patients seen  Blood Bank in the ED, admitted to the inpatient services, and  Pain Management seen in outpatient clinics. Estimates of growth potential should include the likelihood of increased  Health System referrals from the community, the number of pedi-  Hospital atric patients approaching transition, estimates Administration  Department Chairs of regional population size and travel distances. - Medicine Often, regional and statewide hospital consortia - Oncology/ Hematology and departments of health maintain utilization - Nursing data that can inform population estimates. Partnering with local CBOs can also serve to  Political leaders  Community-based estimate the number of patients not currently in External comprehensive care. organizations  Patients/families Financial data to plan feasibility includes estimating  Funding entities costs, charges and reimbursement for current

14 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 Program Execution Data capture for patients treated in the new center Although adequate planning expedites program is vital. A database beyond the standard electronic execution, the process is far from simple. The medical record can capture information needed to personnel needed to care for the new patients with demonstrate program success. Programs without sickle cell disease come from reassignment of such benchmarks will be severely handicapped in existing personnel or new hires. Reassignment taps measuring outcomes. A patient database could also people familiar with operations within the institution provide anonymized information to national sickle and can be challenging due to potential shortages cell disease registries that are under construction. created for existing oncology or hemophilia patients. Data sharing contributes to the long-term improve- Planning and careful coordination help avoid ment in patient care both locally and nationally. impasses that can sabotage a new effort before it begins. New hires circumvent this issue. Both Metrics/Measurement however will need training in management of people Table 4 lists some of the parameters that can be with sickle cell disease. The depth and breadth of assessed to determine the success of the sickle the training will vary but cultural sensitivity should cell treatment program. Others are possible but be an integral component. An advanced practice should fit the SMART criteria: Specific, Measurable, provider is the hub of most sickle cell disease treat- Achievable, Relevant and Time-bound. Essential ment programs and should be in the first wave assessments without SMART characteristics are of recruitment. patient and staff satisfaction. Continual review and adjustment will improve the quality of the program. Care pathways are essential to the proper manage- ment of patients with sickle cell disease. A generic Conclusions pain management protocol should be established Integrating a care program specific to sickle cell as a template to be personalized for each patient. disease into an existing system can markedly A personalized treatment plan can avoid frustration improve care for people living with the condition. An for both the patient and care personnel. Operating innovative/creative approach is sometimes needed to procedures should be codified to cover the gamut of issues around acute management of people with sickle cell disease including Table 4. Leveraging Available Health Center Resources for Sickle Cell Disease Care: Success Metrics and Measures fluid administration, transfusion, manage- ment of fever, and radiographic investigation. Metrics/Measurement

The quality and severity of vaso-occlusive  Decreased time to first dose pain varies greatly for patients with sickle cell  Improvement in pain disease along with the analgesics to which Quality of treatment  Decreased admissions they respond. Unfortunately, opioids are at  Decreased readmissions and return the epicenter of a public health emergency in ED visits the United States. These drugs are essential   for pain management by many patients with Increased visits to day Access to acute hospital/infusion center sickle cell disease and most handle the medi- treatment  Increased observation visits cations without misuse or abuse. However, patients with sickle cell disease are caught in  Immunizations the whirlwind around opioid management and Continuity of care  Disease-modifying therapy restriction. Clear documentation of patient  Decreased hospitalization rate history and needs, along with an individual- ized management that is adjusted based on  Complaints Patient Satisfaction response and can minimize pain (both phys-  Surveys ical and psychological), is essential. Pain  Reduced cost to institution for sickle specialists should participate in manage- Costs cell patient care ment whenever possible.

Sickle Cell Disease Access-to-Care Summit 2018 15 M-UNB-US-00050 V1.0 integrate into existing infrastructure (e.g., integration mortality has shifted to adults, making the tran- into an oncology program with specifically allocated sition to adult-focused care a high-risk period for chairs for patients with sickle cell disease). With any death.40,41 As patients transition from childhood to new service, champions/advocates are essential to adulthood, chronic comorbidities increase including convert a good plan into a real solution. Patients are chronic pulmonary disease, nephropathy, and persuasive advocates, giving voice to difficult issues cardiovascular complications. A coordinated that they face. When embarking on such an endeavor, effort around care transition can mitigate the a perfect plan should not impede implementation of dire health consequences suffered as patients a good one—take what is available and implement it. cross the threshold between the pediatric and Most importantly, approach adversity with patience, adult worlds. Recognition of the challenges faced using each challenge as opportunity to educate and by adolescents and young adults is the first step improve the sickle cell program. toward solutions.

Overarching Issues Specific to Transitioning Adolescent Care: Pediatric/Adult Transition Structuring Transition Between When considering transition from pediatric- to Pediatric and Adult Care Settings adult-based sickle cell disease care, the meaning A transition program that facilitates the move from of the word “transition” must be clear. Transition is pediatric to adult care for adolescents and young an orderly, coordinated shift in patient care from adults with sickle cell disease is critical to optimal a pediatric program to an adult program. A shift long-term health outcomes. Indeed, mortality of in patient care that is not orderly and coordinated patients with sickle cell disease older than 18 is a transfer, not a transition (Figure 5). Transfers years increases by 2- to 3-fold relative to younger generally result in poor patient care compared patients.38,39 The rate of acute care encounters (ED to transition with a key factor being the difficulty visits and inpatient stays) also increases markedly patients face in crossing the care chasm without in patients aged 18–30 years relative to younger guidance or assistance. Because sickle cell disease cohorts.6 Because most children born with sickle is a life-long chronic illness whose characteristics cell disease in high-resource countries survive to change between childhood and adulthood, care adulthood, the primary impact of morbidity and continuity is essential. Management and prophylaxis

Figure 5. Sickle Cell Disease Pediatric to Adult Transition

eiatric rasitio Ault Care Care

raser

Care Casm

16 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 programs developed during early care should be guidelines must be developed. Patients, families, carried forward into the adult setting as appropriate. and pediatric providers develop a special bond Anticipation of issues that emerge in adulthood is an over years, derived from care that often stretches important component of transition. from infancy. Relinquishing that care to strangers of unproven mettle can be trying for all. No substitute Limited guidance exists on transitioning patient care exists for unrushed time during which patients, from pediatric to adult settings for people with sickle families and adult providers develop familiarity cell disease. Adult-focused programs are commonly and trust. driven by a single provider which contrasts with pedi- atric programs that often have more resources, a Barriers Unique to or High Profile in Transition broad team (e.g., nurses, social workers) and usually Transition occurs during, and is complicated by, are more “nurturing” for patients. Furthermore, one of life’s greatest challenges: adolescence. Even pediatric providers have a well-rounded percep- for unaffected children, the physical and emotional tion of patients with sickle cell disease due to changes wrought by adolescence can smash extensive clinical contact around issues such as childhood tranquility into painfully jagged fragments. penicillin prophylaxis and vaccinations which create For adolescents struggling with sickle cell disease a rounded and holistic view of patients. In contrast, that often is morphing in character and intensity, the institutional experience with sickle cell disease for teenage years can be a nightmare. Adding transi- adult providers often is based on a small sub-pop- tion to this chaotic climate can approach cruel and ulation of patients with high ED utilization and unusual punishment for patient and family. Patience frequent inpatient stays, fueling a perception of high and resolve by healthcare providers are vital to navi- opioid use, noncompliance, and high readmission gating these rocky rapids. rates. Data are key to creating an accurate picture and changing institutional perceptions of young adult A key consideration in pediatric to adult transition patients with sickle cell disease. For instance, at the is the age at which the issue should be addressed Children’s Hospital of Philadelphia, of 179 patients with patients and families. Initial discussion should with sickle cell disease between the ages of 18 and be part of visits during pre-adolescent years. 22 years, only 8 patients (4.5%) had more than 10 Social workers from oth pediatric and adult teams hospital admissions per year and only 2 patients determine “readiness” (which may not always be (1.1%) spent greater than 50% of days on docu- patient-dependent: the adult care providers also mented outpatient opioids. need to be ready for the new patient). Transition should be carefully thought out and paced. Poor communication between pediatric- and adult-focused programs, CBOs, and patients/fami- The optimal age is not well defined, and no clear lies is a frequent issue that complicates transition age-dependent triggers exist for transition. However, planning and execution. Poor understanding of tools are available to help clinicians assess patient coping and support needs of young adults as they readiness for transition.43-47 Often, the transition age move through transition is another obstacle. In is payor driven but remains highly variable, with no addition, patients often have only a vague under- standardization. A common and unfortunate result is standing of transition as well as limited insight into complexity and confusion for patients and families. their basic medical history.42 A “bridge” is needed Medicaid programs in some states such as Texas to allow patients to experience and understand the require a transition plan, but this is not a uniform new adult medical care model. Communication and mandate across states. education are needed for pediatric patients to make Patient separation from their pediatric provider the transition to adult-focused care. and lack of preparedness of the adult healthcare Essential structural components to transition system are major obstacles to successful transi- include a pediatric care program, an adult care tion.48 Brain injury and neurocognitive impairment program, and a bridge between the two (Figure 5). are important comorbidities in sickle cell disease Commencement and completion of the transition producing developmental challenges that can process is variable meaning that institution-specific also complicate transition readiness. Suboptimal

Sickle Cell Disease Access-to-Care Summit 2018 17 M-UNB-US-00050 V1.0 adult care programs, including a lack of adult care personnel ideally are multi-level providers (i.e., providers, a deficit in provider readiness (only about medicine/pediatrics), educators (disease self-man- one third of general internists feel comfortable agement, educational modules), family medicine, being the primary provider for patients with sickle mental health, vocational/occupational specialists, cell disease49), and a dearth of nurse coordinators, sexual reproductive health specialist, and genetics psychologists, patient navigators/coordinators, make counselor. Although this comprehensive list is transition unattractive to patients and parents. Adult daunting, programs usually begin with a minimal subspecialty providers such as cardiologists and provider contingent and build out as success grows. nephrologists must also be considered as part of transition planning. Transition Resource Planning: Specific to Adult Program Partners Consequent anxiety for patients and families as On the adult side of the transition bridge, solicita- well as patient depression are significant concerns. tion and recruitment of adult providers to care for Social support therefore is an important compo- transitioning patients is paramount. Providers who nent of transition success. Other barriers include actively care for sickle cell disease patients are a the lack of systematic approaches or processes to good starting place. Hematology/oncology programs track patients through transition, lack of a standard emphasize hematologic malignancies. However, definition of successful transition, lack of third-party such programs often have a provider who focuses payer care/case management, and lack of knowl- on benign hematology who may be open to adding edge around support for patients who fail transition sickle cell disease to the treatment portfolio. A pilot readiness but are adults. In addition, many patients initiative involving a small number of patients can be need coaching on personal accountability and skills useful to resolve bumps before a large patient influx. for navigating the healthcare system. Another pivotal step is reaching agreement with Transition Resource Planning: Pediatric adult providers on key requirements for transition. and Adult Programs A phased startup approach using age- or disease- Figure 6 highlights the conceptual framework of based prioritization is a reasonable first step. For transition and the key personnel and other factors example, priority might be given to transitioning that must be integrated into planning. Facilitators adult patients in the pediatric program or patients are vital to success and include program cham- with milder disease, keeping complex patients on pions, hospital administration (chief medical officers the pediatric side while the adult providers become are particularly important to program administrative more comfortable with management of sickle cell ownership), medical staff (including psychiatry/ disease. Tiered efforts build confidence of patients psychology), nursing staff as well as patients and and providers, as well as program supporters their caregivers. Table 5 lists these and other and champions. important stakeholders including community advo- The transfer of key patient information at handoff cacy groups (foundations, churches/faith-based is also essential to transition. In this age of early organizations), third-party payers as well as public hydroxyurea treatment, some patients with sickle cell or political champions. Input from stakeholders is disease have pediatric courses that are barely distin- key to creating a shared vision of success. guishable from unaffected children. Unfortunately, Resource planning is vital to success when building others have major challenges including strokes, a sickle cell disease transition program. This acute chest syndrome and frequent vaso-occlusive includes a business plan, a program and services pain crises. Chronic transfusion treatment with its plan, plans for addressing personnel requirements attendant comorbidities is a common consequence as well as a needs assessment for space. Staff of these clinical challenges. The adult providers in requirements include dedicated medical providers, the transition program must receive key information advanced practice providers, social workers, patient from the pediatric medical record, such as data navigators, care coordinators/managers, and a related to chronic exchange transfusion, as well as support mechanism for patients (e.g., community management plans to avoid potentially dangerous health worker, peer mentor, etc.). Other potential treatment lapses.

18 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 Figure 6. Conceptual Framework of Key Patient and Institutional Factors Influencing the Development of a Transition Initiative

Patient Characteristics Facilitators  Patient diagnoses  Close physical proximity of  Complexity and severity of adult and pediatric care illness  Parent and patient advocates  Duration of illness  Institutional (leadership)  Cognitive and functional status priority for transitions    Demographics Healthcare provider group priority for transitions Short-Term Outcomes  Family education/health    Patient, family, provider literacy Healthcare provider group champions for transitions experience  Pressure from payers  Timely transition to adult services  Less inconsistency (ie, back- and-forth between Internal Medicine and Pediatrics Barriers services)  Disconnected medicine/pedi-  Fewer hospitalizations atric divisions Institutional Context  Fewer ED visits  Conflicting priorities for transi-   Physician training and tion goal among stakeholders  Fewer medical errors experience (departments, provider types,  Physician scope of practice patients, etc.) (internal medicine-pediatrics,  Distinct resources in pedi- family medicine-pediatrics, atric/adult hospital settings inpatient/outpatient practice) (eg, child life, social work,  Adolescent inpatient unit etc.)  Organizational structure,  Lack of knowledge about eg, free-standing children’s transition hospital  Different motivators for inpa- tient vs. outpatient providers (ie, limited inpatient bed availability at hospital vs. desire for PCP/Subspecialist to continue providing care)

Transition Initiation from “overprotective” behaviors derived from diffi- Creation of an institutional policy around the age culty letting go. Pediatric providers may also request for transition initiation is the first step in building a that parents wait in lobby until the end of exams/ program. As noted earlier, an optimal age for tran- visits to allow individual, confidential discussions sition from pediatric to adult sickle cell disease care and HEADSS assessment (Home, Education, is not well-defined. Discussions of transition with Activities/employment, Drugs, Suicidality, and Sex) patients and families should start early on before Payer coverage issues often are also linked to patient the process truly begins to avoid the perception age (e.g., 18 years) placing additional constraints on of an abrupt cutoff of care. Patients and families care transition. In any case, patients and families who commonly view late adolescence as a time to are forewarned about transition can better cope with consider transition. Many clinicians feel however the potential stress inherent to the process. that initial discussion of transition at around age 14 years of age is appropriate. An early start on the An educational program around transition is one transition pathway allows patients to make deci- way to forewarn and forearm patients and families. sions and advocate for themselves, as well as take Transition to the adult care world is scary and responsibility for medication adherence and other mysterious for all patients and those with sickle aspects of care. Moreover, parents can be weaned cell disease are no exception. Transition can be

Sickle Cell Disease Access-to-Care Summit 2018 19 M-UNB-US-00050 V1.0 Table 5. Resource Planning and Recruitment for Pediatric to Adult Transition

Scoping Your Solution: Recruitment/Resource Planning

 Patient and family first and foremost  Hospital team of peers, others doing transition  Divisional leadership Stakeholders  Hospital administration  College-bound patients—college campuses, office of disability on college campuses  3rd-party payers

 Someone you need in a specific role to help you accomplish a specific task  CBOs Champions  Political leaders  Public figures

 Patient insurance, comprehensive care Potential Funding  3rd-party or hospital funding Sources  Private donations/foundations/philanthropy  Grants—federal or private

 Nurse/nurse practitioner  Social Worker Staff Requirements  Care coordinator  Support mechanism for patients—community health worker, peer mentor, etc.

 Space, approval for the space  Education materials for transition readiness Other  Transition process tracking resources  Other infrastructure laden with anxiety and angst. An education program families includes the location of the adult facility that commences before transition can smooth the and detailed instructions on how to reach the sickle process. An emphasis on positive features of tran- cell disease clinic. Clinic phone numbers should be sition, such as the child becoming a big person like provided along with details on information services their siblings, relatives and friends sends a positive at the adult facility. Check-in calls from the adult and hopeful message. Even today, patients with clinic should occur prior to the first visit and follow sickle cell disease at times are told they will not live up calls should be made if the visit is missed. long. Preparation for transition is a clear message to Patients can be lost for months or years due to the contrary. failures at the first hand-over visit, sometimes with disastrous consequences. Ideally, the initial discussion of transition should be in the pediatric facility with the prospective adult Transition Execution providers attending as guests. New faces seen in a A transition advisory committee composed of familiar environment eases stress for patients and healthcare providers, patients/families, and advo- families while allowing the adult providers to see cates can give useful insight into challenges and how patients and families interact with their long- successes of transition. In addition, a real-time standing healthcare team. This introduction should tracking program is helpful for monitoring individual occur months or years before the patient’s first visit patients, opening the possibility of midcourse to the adult facility. Vital information for patients and correction to the transition process.

20 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 Patient data collection, both at baseline and over Conclusions the course of transition, is vital to assess program Successful transition from pediatric- to adult-based success. Careful planning is required to create a care is critical to maintain continuity and quality of data management program that documents the care for patients with sickle cell disease. Important impact of the transition effort, justifies maintenance challenges include when and how to institute tran- and expansion, improves efficiency, and supports sition, lack of adult providers, poor communication ongoing quality improvement. Baseline data can between pediatric and adult sickle cell disease care be used to justify initiating the program while data programs, and emotional attachment of patients/ collection during operation of the transition program families to their providers in the pediatric setting. documents financial impact, improvement in care, Essential structural components to transition include and patient satisfaction. a pediatric care program, an adult care program, A database beyond the standard electronic medical and a bridge between the two. The transition record should be created to capture informa- process is variable and therefore institution-specific tion needed to demonstrate program success. guidelines must be developed. Important compo- Aggregate data along with trends and statistical nents of the transition program include stakeholders considerations are powerful tools for analysis of (e.g., patients/family, HCPs, administration, and program activities and execution. payers), funding sources, staff (e.g., nurse/nurse practitioner, social worker, and care coordinator), Success Metrics physical space for the program, educational mate- Measuring important components and outcomes rials for transition readiness, and transition process related to transition is important for stakeholder tracking resources. The program’s success should assessment of program success and to highlight be assessed by key metrics including patient/ needed adjustments. Table 6 lists some important family feedback, ED visits, hospitalizations/re-hos- measures including first visit to the adult clinic, pitalizations, treatment adherence, and cost. A return visits to the pediatric clinic post-transition, robust transition program can positively influence a ED visits at either adult or pediatric facilities and patient’s clinical course for years and even decades. hospitalizations at either facility. Other important measures include the number of patients tran- Expanding Care Outside the Institution: sitioned or lost during transition, continuation of scheduled transfusions, continuation of mainte- Building a Community Outreach Model nance medications and use of opioid analgesics. to Extend the Reach of Care Satisfaction reports from patients and staff are also Healthcare providers with specialized knowledge, important metrics. experience, and insight are critical to the manage-

Table 6. Pediatric to Adult Transition: Assessment of Program Success

Individual Patient Measures Other Measures Pediatric Encounters Adult Encounters

 ED visits  First clinic visit  Number of patients transitioned  Hospitalizations  ED visits  Patient loss during transition  Clinic visits  Hospitalizations  Maintenance of transfusion schedule  Opioid use  Patient satisfaction  Staff satisfaction  Program costs  Deaths

Sickle Cell Disease Access-to-Care Summit 2018 21 M-UNB-US-00050 V1.0 ment of complex, chronic disorders. Experience (Extension for Community Healthcare Outcomes) and insight are developed only with frequent patient program,56,57 uses a case-based teaching approach interactions. The challenge is magnified for rare with additional didactics provided. Multiple remote disorders, which can range from ultra-orphan condi- providers can interact with the expert through tions like paroxysmal nocturnal hemoglobinuria to group discussion around individual patients. This “more common” orphan disorders like sickle cell disease. Not surprisingly, specialists advanced in the care of rare disorders are Figure 7. Approaches to Extend Sickle Cell Disease Care usually found at medical centers in large Beyond the Institution urban areas. To enhance care outside of the institution, it is necessary to build a path to ele-cosultatio share both the specialized knowledge about the disease as well as the experience with patient management. Despite new therapies and improved childhood survival, sickle cell disease still limits life expectancy through a myriad of SCD ert D or D atiet chronic complications.50-52 Care provided by specialists familiar with sickle cell disease elemeicie management is critical if patients are to benefit optimally from current and evolving therapies. Many people living with the disease reside in urban areas such as New York and Chicago where specialists are often available. However, for those living in rural areas, most often in Southern states, atiet the search for knowledgeable providers who deliver quality, evidence-based care 28,53 often is daunting. Routine travel of SCD ert several hours to reach sickle cell treatment specialists often strains the social and economic wherewithal of people who may be socio-economically challenged, adding to D or D the health disparities of their disease.54,55 Fortunately, evolving technology is being u Soke harnessed to address and mitigate the healthcare chasm between people in urban and rural regions of the country. Figure 7 shows three ways to decentralize sickle cell disease expertise to benefit people in outlying, underserved areas. One is atiet tele-mentoring in which the expert holds online video conferences with providers (typically physicians, advanced practice SCD ert providers, nurses and social workers) who then deliver patient care at remote loca- tions. In this model, the expert is an adviser and does not provide direct medical care. Aace Care ractitioer Instead, the model, based on the ECHO

22 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 tele-mentoring mimics subspecialty rounds at in rural regions. However, before creation of (SC)2, medical centers, providing additional educational the three main academic centers in the state had opportunities. With this approach however, the sickle cell disease pediatric programs, but none of quality of patient care still depends on the skill and them had programs for affected adults. Adults with knowledge of the local provider. The expertise of the disease thus were underserved throughout the sickle cell disease specialists often is intangible and state, often seeing primary care doctors, commu- subtle insights can be lost in translation. nity oncologists or only obtaining care from the ED In contrast, telemedicine involves interaction of the regardless of whether they lived in the city or in the expert with both the local provider and the patient. country. Effective redress required a program that As Figure 7 illustrates, this more dynamic interac- fully assessed the situation and was designed to tion allows the expert to evaluate the patient directly reach all areas. and possibly uncover disease-related issues that the local healthcare provider may have missed. Preparation Real-time interactions amplify effectiveness in There are several issues to address and barriers complex conditions like sickle cell disease where to anticipate when expanding sickle cell disease multiple organ systems are affected and interact. care outside of the main institution (Table 7). Telemedicine usually requires a presenter (often Data are key to any new endeavor including the a nurse or medical assistant) where the patient is creation of a program to extend quality healthcare located to facilitate the use of the equipment. into rural America. Data can define the need and identify resources to address that need. As clear, Another way in which telemedicine can be used is unequivocal facts, data can be the rallying point for within a hub-and-spoke approach, where the expert supporters, champions and allies. is credentialed at the outlying facilities, provides medical care, and supervises mid-level practi- Understanding sickle cell disease demographics tioners. The hub-and-spoke model can have greater within a state is a key first step in program planning. reach than telemedicine alone by expanding care In the US, Sickle Cell Disease Newborn Screening to outlying facilities where an advanced practice (NBS) exists in all states and is key to under- provider can give hands-on care on a daily basis, whereas telemedicine clinics are usually more Table 7. Extending Sickle Cell Disease Care infrequent. An important caveat is that the hub-and- Beyond the Institution: Overcoming Barriers spoke model places greater responsibility on the expert who becomes a real provider through a  Patients live far from academic virtual connection. center (therefore, no care, ED didn’t know what to do, etc.) Building a program to extend healthcare is daunting. Issues to  No insurance or local SCD Clarity around strategy and diligence concerning Address specialists tactics are vital. Identification of local or regional  Finding affected individuals stakeholders, collaboration with ancillary care  Finding interested stakeholders teams and other approaches that broaden access are crucial to building an effective outreach system.  Alignment between different systems of care Other factors that can influence a program’s viability  include political or community champions, newborn Alignment within institution screening programs and associated follow-up  No coordination of care system activity, as well as private and government-spon-  Credentialing process Barriers to  Funding sored third-party payment programs. Anticipate   2 Lack of education/knowledge The South Carolina Sickle Cell Network (SC) , about disease a hub-and-spoke model, provides insight into  Engaging payers, politicians successful program development. Newborn  Data screening data showed that a substantial number of  Hiring staff people with sickle cell disease in South Carolina live

Sickle Cell Disease Access-to-Care Summit 2018 23 M-UNB-US-00050 V1.0 standing patient proximity to urban centers.58 NBS fied “hot spots” of need that could be targeted for programs are administered by state-designated intervention (Figure 8). Following the identification entities meaning that information can be acquired of local hospitals that frequently evaluated patients only through individual state programs. Currently, with sickle cell disease in the emergency depart- there is no national NBS database and many ments and inpatient units, specific hospital-based states expunge the data every few years. There data could be obtained. are no national registries for sickle cell disease at Rallying stakeholders to the cause of improved care present and data surveillance has only been done for people with sickle cell disease should be an early in California and Georgia through CDC-sponsored action in the care expansion effort. Table 8 high- grant projects. Thus, it can be a challenge to char- lights stakeholders key to the mission of providing acterize and quantify the local populations. quality care for people with the disease. Community In South Carolina, a needs assessment was hospitals are important to people who live far from performed to address questions around adult urban hubs and academic centers. These hospitals access to care through payer information gath- often are criticized for poor care while simultane- ered from the South Carolina Revenue and Fiscal ously shouldering the high cost of care associated Affairs Office, which keeps health statistics.29 with inexpert management, creating an environ- Statewide administrative data provided key infor- ment unpleasant both to patients and providers. mation on acute care utilization during a defined High costs are also an issue for third-party payers. 12-month period (2012) and was shared with state Bringing sickle cell disease management expertise and regional service providers, managed care into rural communities is in the interest of commu- companies, and policy makers to identify and nity hospitals and payers as well as patients. highlight gaps in care and inform policy around Political champions and CBOs within a state can improvements.29 Information on ED visits and hospi- powerfully influence healthcare both by opening talizations through patient-based uniform billing data access to key decision-makers and by raising were analyzed for acute care utilization and 30-day the level of public awareness. Social media is a readmission rates were stratified by patient age, major new force that shapes awareness and public region, and expected payer. These data identi- opinion and is a vehicle with which many younger

Figure 8. Extending Care for Sickle Cell Disease in South Carolina

PEE DEE 541 patients in 1 yr SA Georgetown Memorial: >250 patients/yr UPSTATE 350 patients in 1 yr DADS GHS: 500 patients/ 5 yrs D

MIDLANDS 613 patients in 1 yr Palmetto Health: 1033 pts/5 yrs C

LOWCOUNTRY Beaufort Hospital 800 patients in 1 yr. 160 patients in 1 yr MUSC: 2300 patients/ 300 patients/5 yrs 5 yrs

24 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 Table 8. Extending Sickle Cell Disease Care is also important. South Carolina chose a hub-and- Beyond the Institution: Key Stakeholders spokes care delivery model, with Medical University Stakeholders Role in Achieving Outcome of South Carolina as the hub of sickle cell disease expertise. The spokes provided a local medical home Community  Care clinic locations and advanced practice provider-supported clinics, Hospitals  In-kind funding support using Telehealth support as needed. The goal was to have sickle cell disease care clinics at each spoke  Provide funding support Third-Party with small “day hospital” care facilities that supported Payers  Endorse and support medica- tion needs laboratory services, intravenous fluids, pain medica- tions, transfusions, and hospital follow-up. State  Newborn Screening Program Facilitators, including supporters within the hub Department of  Funding (in some states) Health Local hospital, are crucial to establishing a treatment   Political Leaders Establish a state plan network. In South Carolina, important support came from the state-run telehealth program, Duke  Telehealth support Endowment (funding), and CBOs. Individuals with Academic  IT support (ECHO, website, Center(s) registry) sickle cell disease and their families also served on a community advisory board to give life and voice  Personnel to patient needs that might otherwise have been  Case managment abstract facts and dry statistics. Nothing substitutes Community-  Advocacy for the human face of illness. Based Organizations  Patient engagement Government programs are another potential pillar  Provider education to support expanded care for people with sickle cell disease. Medicaid is the insurance network for many people with the disease. Some Medicaid people, including those with sickle cell disease programs have health maintenance organization are facile. Alliances and publicity can help blunt that can be recruited to improve care. In addi- the impact of people who harbor negative or even tion, Departments of Health through programs for frankly hostile attitudes toward those living with Children with Special Healthcare Needs can be 8,59,60 sickle cell disease. important allies. The South Carolina needs assessment confirmed that many individuals with sickle cell disease lived in Recruitment/Resources rural areas and often relied on community hospitals The next phase of network creation is site recruit- without expertise in sickle cell disease. These data ment and determination of resource needs. demonstrated very high rates of acute care use and Important site considerations include location readmission (Figure 8). A discrepancy often exists and whether the site can be payer-directed or between pediatric and adult health outcomes in region-directed. Space is a premium in most sickle cell disease and is due in part to the paucity of medical facilities, including those in rural regions. Carving out areas dedicated to treatment of sickle providers willing and able to treat adults living with the cell disease requires patience and negotiating skill. disease.61,62 The issue is magnified for people in rural Identifying allies in the local institutions is vital, areas far from academic centers where transportation including ED medical directors, nursing staff and options are limited.53 Routine travel of several hours pharmacists. At the hub hospital, administrators to reach urban specialists is not sustainable. and program directors can facilitate program devel- Initial efforts through pilot projects often can garner opment. More broadly, medical societies (e.g., the more early support than a full-blown networking American Society of Hematology and the American effort. Starter programs allow the probing of barriers Society of Pediatric Hematology/Oncology) and including local credentialing for the expert and hiring Medicaid medical directors can be useful allies. of local staff. Choosing the best networking program Support can possibly be garnered through state

Sickle Cell Disease Access-to-Care Summit 2018 25 M-UNB-US-00050 V1.0 departments of public health, hospital in-kind funds, nidus for a statewide patient registry. Standardized foundations or third-party payers. Demonstration data templates and storage procedures which are projects by the Health Resources and Services key components needed for information sharing Administration of the US Department of Health and across a network are also requirements for a Human Services has supported networks, as well. registry. Programs start slowly due to builds around credentialing, identification of space, hiring staff Staff requirements for the program are extensive, and assigning responsibilities. Lessons learned with and includes nurses, pharmacists, primary care early adopter spokes can be applied to those that providers, and information technology support come onboard later. (Table 9). Establishment of a stakeholder advisory board is another important component of care Metrics/Measurement extension. Advisory board stakeholders include Program success and continuation ultimately rest individuals with sickle cell disease and their families, on data. Pre-specified metrics and milestones key care providers, third-party payers, state govern- therefore are vital to long-term program viability. ment and Department of Health and Environmental As outlined in Table 10, important metrics include Control members, and CBO members. the number of spokes/clinics established, patient Action Planning access (who and how many patients are seen and how often) as well as the capacity of the various The next steps in expanding care outside the spokes/clinics (what can be done at those loca- institution involve program implementation and tions). Data on use of ED versus day hospital for measurement of success. Data specific to individual acute care and 30-day readmission rate gauges hospitals are needed. Each spoke is unique in its the impact of care programs on patients as well as patient and staff composition, operational struc- ture and relationship to the hub. Data from multiple spokes integrated with that from the hub can form a Table 10. Extending Sickle Cell Disease Care Beyond the Institution: Success Metrics and Measures Table 9. Extending Sickle Cell Disease Care Beyond the Institution: Staff Requirements Measure Metric

 Nurse Practitioner Number of  ED/acute care use functioning   Telemedicine Nurse/public health nurse care clinics  Social worker (SPOKES)  Cost  Human services coordinator Disease-  Hydroxyurea  Case manager modifying  Transfusion  Patient coordinator for spokes therapy  PCP (ideal) Education  Echo attendance  Family medicine resident rotations  Symposium attendance  Ideal mental health services  Physical therapy Patient  New referrals of previously unaf-  Palliative care engagement filiated patients and care  Pharmacy  Individualized pain plans coordination  Pain medicine expert/anesthesiologist  IT support (comes with telehealth?) Community  Creating new educational engagement symposiums  EMR/data  Working with CBO to establish  Quality improvement person case management  Administrator – policy/procedure/data  Lab/imaging Registry  Start-up  Data coordinator  Enrolled patients

26 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0 costs to institutions. Establishment of rational opioid References management policies across various spokes (same  1. Nelson SC, Hackman HW. Race matters: perceptions of race and racism in prescribing policies at each location) is another a sickle cell center. Pediatr Blood Cancer. 2013;60(3):451-454.  2. Adams-Graves P, Bronte-Jordan L. Recent treatment guidelines important metric of care quality. Implementation of for managing adult patients with sickle cell disease: challenges in access to care, social issues, and adherence. Expert Rev Hematol. preventative programs such as prophylactic peni- 2016;9(6):541-552.  3. Whiteman LN, Haywood C, Jr., Lanzkron S, et al. Primary Care Providers’ cillin and disease-modifying therapies also reflect Comfort Levels in Caring for Patients with Sickle Cell Disease. South Med J. program success. Finally, overall patient assess- 2015;108(9):531-536.  4. Mainous AG, 3rd, Tanner RJ, Harle CA, et al. Attitudes toward Management ment of the program is vital; if they don’t like it, of Sickle Cell Disease and Its Complications: A National Survey of Academic Family Physicians. Anemia. 2015;2015:853835. they won’t use it. Patient-reported outcome tools  5. Lanzkron S, Little J, Field J, et al. Increased acute care utilization in are valuable in this regard as are tools that assess a prospective cohort of adults with sickle cell disease. Blood Adv. 2018;2(18):2412-2417. depression, an underrecognized issue for people  6. Brousseau DC, Owens PL, Mosso AL, Panepinto JA, Steiner CA. Acute care utilization and rehospitalizations for sickle cell disease. JAMA. with sickle cell disease. 2010;303(13):1288-1294.  7. Evensen CT, Treadwell MJ, Keller S, et al. 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Patterns of opioid use in sickle cell disease. sion costs is paramount. Sickle cell disease care Am J Hematol. 2016;91(11):1102-1106. programs do not make money for institutions. 15. Benjamin LJ, Swinson GI, Nagel RL. Sickle cell anemia day hospital: an approach for the management of uncomplicated painful crises. Blood. However, cost reduction and cost avoidance can 2000;95(4):1130-1136. 16. Raphael JL, Kamdar A, Wang T, et al. Day hospital versus inpatient positively affect the institutional balance sheet. management of uncomplicated vaso-occlusive crises in children with sickle Clear documentation of baseline and subsequent cell disease. Pediatr Blood Cancer. 2008;51(3):398-401. 17. Binding A, Ward R, Phua C, et al. An innovative short-stay health care program costs is essential. model for treatment of uncomplicated vaso-occlusive crisis in adult sickle cell disease patients in Canada to reduce emergency department utilization. Cjem. 2019;21(1):55-62. Conclusions 18. Andemariam B, Jones S. Development of a New Adult Sickle Cell Disease Center Within an Academic Cancer Center: Impact on Hospital Programs to expand care to needy patients with Utilization Patterns and Care Quality. J Racial Ethn Health Disparities. 2016;3(1):176-182. sickle cell disease are born from compassion 19. Lanzkron S, Carroll CP, Haywood C, Jr. The burden of emergency depart- but can die from lack of data to support that ment use for sickle-cell disease: an analysis of the national emergency department sample database. Am J Hematol. 2010;85(10):797-799. compassion. Unfortunately, the human value of 20. Elmariah H, Garrett ME, De Castro LM, et al. Factors associated with survival in a contemporary adult sickle cell disease cohort. Am J Hematol. an interaction between a caring physician and a 2014;89(5):530-535. patient cannot be measured. Therefore, scrupulous 21. Marshall AL, Jenkins S, Mikhael J, Gitlin SD. Determinants of hematol- ogy-oncology trainees’ postfellowship career pathways with a focus on record keeping is needed for other components of nonmalignant hematology. Blood Adv. 2018;2(4):361-369. 22. Larsen LS, Neverett SG, Larsen RF. Clinical nurse specialist as facilitator of that interaction that can be quantified. Patience and interdisciplinary collaborative program for adult sickle cell population. Clin persistence along with clarity and focus in planning Nurse Spec. 2001;15(1):15-22. 23. Porter JS, Wesley KM, Zhao MS, Rupff RJ, Hankins JS. Pediatric to Adult are essential to program development. Not enough Care Transition: Perspectives of Young Adults With Sickle Cell Disease. J Pediatr Psychol. 2017;42(9):1016-1027. can be said on the value of operational dexterity and 24. Andemariam B, Owarish-Gross J, Grady J, et al. Identification of risk factors flexibility. Final programs never reflect drawing board for an unsuccessful transition from pediatric to adult sickle cell disease care. Pediatr Blood Cancer. 2014;61(4):697-701. representations. Lastly, continuous engagement with 25. Anderson N, Eckman JR, Ballas SK. Beyond the transition of adolescents and young adults with sickle cell disease to adult care: Role of geography. patients and families, payers, as well as local and Am J Hematol. 2017;92(6):E110-e112. federal government allows continuous reassessment 26. United States Census Bureau. The Black Population: 2010. Available at: https://www.census.gov/prod/cen2010/briefs/c2010br-06.pdf. Accessed which contributes to long-term program success. April 24, 2019.

Sickle Cell Disease Access-to-Care Summit 2018 27 M-UNB-US-00050 V1.0 27. Ricketts TC. The changing nature of rural health care. Annu Rev Public 45. Treadwell M, Johnson S, Sisler I, et al. Development of a sickle cell Health. 2000;21:639-657. disease readiness for transition assessment. Int J Adolesc Med Health. 28. Haque A, Telfair J. Socioeconomic distress and health status: the urban- 2016;28(2):193-201. rural dichotomy of services utilization for people with sickle cell disorder in 46. Treadwell M, Johnson S, Sisler I, et al. Self-efficacy and readiness for tran- North Carolina. J Rural Health. 2000;16(1):43-55. sition from pediatric to adult care in sickle cell disease. Int J Adolesc Med 29. Schlenz AM, Boan AD, Lackland DT, Adams RJ, Kanter J. Needs Health. 2016;28(4):381-388. Assessment for Patients with Sickle Cell Disease in South Carolina, 2012. 47. Sobota A, Akinlonu A, Champigny M, et al. 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Am J Prev Med. 2016;51(1 Suppl 1):S39-47. 2010;115(17):3447-3452. 59. Brennan-Cook J, Bonnabeau E, Aponte R, Augustin C, Tanabe P. 41. McLaughlin JF, Ballas SK. High mortality among children with sickle cell Barriers to Care for Persons With Sickle Cell Disease: The Case anemia and overt stroke who discontinue blood transfusion after transition Manager’s Opportunity to Improve Patient Outcomes. Prof Case Manag. to an adult program. Transfusion. 2016;56(5):1014-1021. 2018;23(4):213-219. 42. Williams CP, Smith CH, Osborn K, et al. Patient-centered approach 60. Puri Singh A, Haywood C, Jr., Beach MC, et al. Improving Emergency to designing sickle cell transition education. J Pediatr Hematol Oncol. Providers’ Attitudes Toward Sickle Cell Patients in Pain. J Pain Symptom 2015;37(1):43-47. Manage. 2016;51(3):628-632.e623. 43. Sobota AE, Shah N, Mack JW. Development of quality indicators for tran- 61. Crosby LE, Quinn CT, Kalinyak KA. 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28 Sickle Cell Disease Access-to-Care Summit 2018 M-UNB-US-00050 V1.0