Abstract
Background: Sickle cell disease (SCD) is the most common inherited blood disorder in the United States (U.S.). Despite advances in the treatment for children with SCD, care for affected adults has not significantly improved and is often inaccessible. Difficulties with access to care for adults with SCD in the US is multifactorial and includes paucity of trained, specialized physicians, limited physical access to specialty centers, lack of health insurance/health care coverage, and lack of SCD-education by other medical providers. To address these issues, we have established a state-wide sickle cell disease network to improve access to care for affect adults in South Carolina. This network, (SC)2, Sickle Cell-South Carolina is working to enhance access to care and to examine ongoing barriers to care for adults with SCD. The "hub" of (SC)2 is located at the Medical University of South Carolina (MUSC); specific outside hospitals have been identified as "spokes" within the state to form the network. Clinics are either established or being established at each spoke and additional education is being given through tele-mentoring. Current knowledge of referral patterns for patients with SCD is limited. Information on the mechanism of referral and the coordination of the referral process will ultimately help to eliminate obstacles to appropriate and timely patient referrals and help develop patient engagement.
Methods: This is retrospective review of patients >18 years of age referred as "new patients" to the Lifespan Comprehensive Sickle Cell Center at MUSC from October 2015-July 2017. Patients were identified through a chart review of the (SC)2 database including referral notes, telephone calls, and initial patient visits. Data collected included the age of the individual, location (within the state) in which the individual resides, presence or absence of SCD, SCD phenotype, referral source, hematologic care within the prior two years, and the consistency of follow-up after the initial visit to the Sickle Cell Center.
Results: During this period, 130 new adult patients were referred to the Lifespan Sickle Cell Center at MUSC. Patients referred for care ranged in age from 18-67 (median 34.5 years) of age and 33% (34/104) were male. To be included in the results, these individuals could not have been seen be the Lifespan Sickle Cell Center within the prior three years so that patients transitioned within the center were not included. Of those patients referred during this period, 85% percent (110/130) were successfully seen at the Lifespan Sickle Cell Center for (at least) an initial visit. However, 40% of these individuals missed their first new patient visit at the Sickle Cell Center and required additional outreach in order to complete that first visit. Of those patients successfully seen for a new patient visit, 95% were confirmed to have SCD (6 individuals were diagnosed with sickle cell trait or did not have a hemoglobinopathy). Only 1/3 of the patients referred to the Sickle Cell Center had seen a hematologist in the last 2 years. The majority of the remaining affected patients had not seen a hematologist since they were children or had never seen a hematologist in their lifetime. Referral sources included primary care physicians, subspecialists, (SC)2 affiliated hospitals (spokes), non-affiliated emergency departments, community based organizations and also included family members and peers. Most of the new referrals were from outside of the Tricounty (local) area (75%) and region did not affect the likelihood of successfully establishing care.
Conclusion: The development of a statewide network (SC)2 is an effective initial strategy to facilitate referrals to a SCD center for adults with SCD. Physician-mediated engagement and outreach was key for introducing the (SC)2 network to the state and enabling referrals. When patients missed their initial new visit appointments, aggressive follow-up and trouble-shooting was key to ensuring a successful visit. Additional examination of patient-specific barriers at each phase of the referral process will be key to understanding discrepancies in follow-up rates and obstacles in the referral process. The process through which patients are referred to the center and arrive for their first visit are determinants in their future medical care. Enhanced implementation of this referral process is key to the success of the (SC)2 network.
Kanter: GBT: Research Funding; American Society of Hematology (Sickle Cell Disease Guideline Panel): Membership on an entity's Board of Directors or advisory committees; MUSC: Other: The site PI for sponsored research conducted at MUSC who receives funds from: Novartis, bluebird bio, GBT, Sancillo, Apopharma, Pfizer; Pfizer: Research Funding; Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding; NHLBI (sickle cell disease research advisory committee): Membership on an entity's Board of Directors or advisory committees, Research Funding; Apopharma: Research Funding; AstraZeneca: Membership on an entity's Board of Directors or advisory committees; Bluebird Bio: Membership on an entity's Board of Directors or advisory committees, Research Funding; Sancillo: Research Funding.
Author notes
Asterisk with author names denotes non-ASH members.
