expert roundtables

Improving Access to Care in Patients With Sickle Cell Disease

by Stella T. Chou, MD; Michael Rutledge DeBaun, MD, MPH; and Julie Kanter, MD

Overview

Patients with sickle cell disease (SCD) require regular follow-up throughout their entire lives. Unfortunately, many of these individuals do not have access to care or they lose access and drop out of care entirely. A recent and ongoing shift toward value-based preventive care may improve the access to and quality of care.

Q:

How does access to care affect the quality of care in SCD, and what are the opportunities for improvements in this area?

Michael Rutledge DeBaun, MD, MPH

Professor of Pediatrics and Medicine
Vice-Chair for Clinical and Translational Research
J.C. Peterson Endowed Chair in Pediatrics
Director, Vanderbilt-Meharry Center for Excellence in Sickle Cell Disease
Vanderbilt University Medical Center
Nashville, TN

“The management of SCD must evolve to recognize that SCD is now a chronic disease that all children with SCD are expected to live to and through adulthood. Successful models of health care delivery for other chronic diseases such as diabetes and cystic fibrosis have been developed over the course of more than 2 decades, and we can learn from them.”

Michael Rutledge DeBaun, MD, MPH

Patient access to care depends largely on who is paying for the medical care. Broadly, there are 3 buckets of payors: self-pay, private insurers, and public insurance (Medicaid or Medicare). Fortunately, children in the United States have a large safety net that provides them with medical care regardless of their family’s financial status. Almost every child is eligible for Medicaid. However, health care delivery becomes fragmented as adolescents become young adults.

Most adults with SCD do not have a primary care provider, yet many of the complications that we see in adults with SCD require co-management by a primary care provider. All of us in hematology have had training in primary care, but I would say that most hematologists are experts on the discipline of hematology and not the common primary challenges that occur in SCD. In contrast, primary care physicians are more adept at managing issues such as depression, chronic pain unrelated to SCD, or reproductive issues that are associated with chronic disease.

The management of SCD must evolve to recognize that SCD is now a chronic disease and that all children with SCD are expected to live to and through adulthood. Successful models of health care delivery for other chronic diseases such as diabetes and cystic fibrosis have been developed over the course of more than 2 decades, and we can learn from them. The work flow and team health care provider responsibilities are foreign to most SCD clinics. However, when implemented, these strategies allow for a coordinated care plan for each patient. In the chronic care model, nurses are devoted to educating individuals with SCD and family members about the disease, as well as providing anticipatory guidance and educational materials for self-education. An important step toward improving care would be to formally train and certify nurses who care for patients with SCD so that they can, in turn, empower families to provide chronic self-care.

Julie Kanter, MD

Associate Professor, Division of Hematology and Oncology
Director, Adult Sickle Cell Program
Codirector, Lifespan Comprehensive Sickle Cell Center
University of Alabama at Birmingham
Birmingham, AL

“The standard model of care should transition to a preventive model that leaves fewer patients unaffiliated with care and ensures that every patient has access to an SCD specialist, whether that care is provided directly from an SCD center or through a telehealth-based system or a primary care extender–based system."

Julie Kanter, MD

Enhancing access to care and looking at novel ways to do that, as well as the dissemination of standards and improving the implementation of care, are some of my main areas of research interest. Adults with SCD may have a primary care provider or an SCD specialist, but many do not have both. Additionally, many individuals who are treated by an SCD specialist as children fall out of pediatric care before they have the opportunity to receive education about transitioning from pediatric care to adult care.

Although the pediatric health care system is set up very well in the United States (for children with SCD), patients can still fall through the cracks. An estimated 25% of patients lost to follow-up are younger than 15 years of age, and often have relatively mild disease in childhood. Pediatric patients with milder SCD often develop more severe disease as adults, so it is important that engagement with this group improves.

Those with SCD are healthier when problems are prevented, and, until recently, treatment options that were proactive instead of simply reactive were limited. Now, clinicians need to consider cost of care and access to that care while providing optimal preventive care for everybody living with SCD. Additionally, we need new ways to bring care to patients who become unaffiliated with care or were never affiliated as adults. Such patients are frequently identified in Emergency Departments because they are experiencing pain or secondary problems, such as blindness caused by retinopathy, which might have been prevented if it had been identified earlier.

Unfortunately, individuals with SCD are not always treated regularly and do not always receive the necessary preventive care. The standard model of care should transition to a preventive model that leaves fewer patients unaffiliated with care and ensures that every patient has access to an SCD specialist, whether that care is provided directly from an SCD center or through a telehealth-based system or a primary care extender–based system. The recently founded National Alliance of Sickle Cell Centers is specifically geared toward increasing access to care and improving the assessment of the quality of care that is being provided. This type of effort can make a big difference in the lives of our patients with SCD.

Stella T. Chou, MD

Associate Professor of Pediatrics
Chief, Division of Transfusion Medicine
Children’s Hospital of Philadelphia
Perelman School of Medicine at the University of Pennsylvania
Philadelphia, PA

“. . . good communication between hematologists and primary care physicians is vital in any model of care for patients with SCD.”

Stella T. Chou, MD

I agree with the commentary from my colleagues and that the management of SCD needs to evolve toward becoming a value-based care system. The pillar of such a value-based system is good preventive care because that can decrease hospital admissions and the health care costs that are associated with poor preventive care.

In our setting, most of our patients are pediatric, and we are fortunate that they are eligible for health care coverage. We provide resources to help families navigate the payor landscape, and to ensure that all patients receive the necessary care by providing payors with information to show medical necessity when appropriate. Advocacy has a major role, and it takes a team of clinicians, social workers, and insurance specialists to coordinate the optimal care of patients with SCD.

We partner with primary care physicians to take care of our patients, especially in the pediatric setting. It is relatively easy to ascertain whether patients are seeing their primary care provider (vs having been lost to follow-up) by accessing their immunization records. We rely on their primary care physicians to provide necessary immunizations, as well as to monitor their growth, development, and well-being along with us, as hematologists.

In conclusion, I would simply reiterate the importance of prevention and integrated, value-based care. In addition, good communication between hematologists and primary care physicians is vital in any model of care for patients with SCD.

References

Black LV, Ezmigna D, Wallace‐Farquharson T, Wilkie DJ, Duckworth L. Feasibility and preliminary outcomes of an integrated pediatric sickle cell disease and pulmonary care clinic for children with sickle cell disease. Pediatr Blood Cancer. 2020;67(11):e28672. doi:10.1002/pbc.28672

Bodenheimer T, Wagner EH, Grumbach K. Improving primary care for patients with chronic illness: the chronic care model, part 2. JAMA. 2002;288(15):1909-1914. doi:10.1001/jama.288.15.1909

Crego N, Douglas C, Bonnabeau E, et al. Sickle-cell disease co-management, health care utilization, and hydroxyurea use. J Am Board Fam Med. 2020;33(1):91-105. doi:10.3122/jabfm.2020.01.190143

Kayle M, Valle J, Paulukonis S, et al. Impact of Medicaid expansion on access and healthcare among individuals with sickle cell disease. Pediatr Blood Cancer. 2020;67(5):e28152. doi:10.1002/pbc.28152

Kumar V, Chaudhary N, Achebe MM. Epidemiology and predictors of all-cause 30-day readmission in patients with sickle cell crisis. Sci Rep. 2020;10(1):2082. doi:10.1038/s41598-020-58934-3

Lee L, Smith-Whitley K, Banks S, Puckrein G. Reducing health care disparities in sickle cell disease: a review. Public Health Rep. 2019;134(6):599-607. doi:10.1177/0033354919881438

McClain BL, Ivy ZK, Bryant V, Rodeghier M, DeBaun MR. Improved guideline adherence with integrated sickle cell disease and asthma care. Am J Prev Med. 2016;51(suppl 1):S62-S68. doi:10.1016/j.amepre.2016.03.003

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