patient care perspectives

Sickle Cell Disease: Transitioning From Pediatric to Adult Care

by Michael Rutledge DeBaun, MD, MPH

Overview

The successful transition from pediatric to adult care involves overcoming a variety of challenges and barriers, some of which may be structural. Measures to integrate care and ensure consistency of care across the age spectrum, when possible, can empower families and help ease the transition from pediatric to adult care.

Expert Commentary

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

“We have attempted to remove barriers to successful transition by following the Chronic Care Model, which was developed by Edward Wagner, MD, MPH, and colleagues. In this model, pediatric and adult care providers have a seamless system in place and work together as a single team.”

Michael Rutledge DeBaun, MD, MPH

Several challenges related to the transition of care in sickle cell disease (SCD) are structural, including fragmented care and payor source. Pediatric care stops at a certain age, and adult care begins at another age. The threshold for transition is, unfortunately, rarely based on the maturation of the adolescent, but rather on the medical system by-laws and payor status. The typical forced transition, at a certain age, of medical care from a pediatric provider to an adult provider is not well suited for a disease such as SCD. For example, most of the chronic organ comorbidities in adults with SCD (eg, heart, lung, and kidney disease) have their subclinical manifestations in children, with progressive disease continuing to progress subclinically for decades. Thus, pediatricians and parents often comment about how well the child is doing without acknowledging that there is progressive heart, lung, and kidney disease that is not detectable.

Further, when patients reach the age for transition to adult care, they are frequently apprehensive about leaving the familiarity of their pediatric care provider and developing a new relationship with an adult care provider. Differences and distinct styles of care between pediatric and adult practices can hinder transition. Pediatric and adult care philosophies can even shift in terms of what would be considered evidence-based care in some instances, so these are actual barriers.

We have attempted to remove these barriers to successful transition by following the Chronic Care Model, which was developed by Edward Wagner, MD, MPH, and colleagues. In this model, pediatric and adult care providers have a seamless system in place and work together as a single team. We meet weekly to discuss patients who are ready to move on, so that the adult care provider has a better understanding of the patient’s needs and can provide continuity of care. We also have a family practice nurse practitioner who meets with adolescent patients in the pediatric setting and continues to comply with them as they transition to adult care. Our approach gives our families an added level of comfort and familiarity with the adult SCD providers. Our Center for Excellence utilizes the same protocol to screen for depression and end-organ disease in children and adults. Our strategy provides uniformity, expectations, prevention, and treatment. Importantly, we provide consistency of care across the age spectrum.

Another advantage of the Chronic Care Model is the ability to develop a relationship with our pediatric patients and their families outside the context of the office or the hospital. We routinely include family relationship–building events such as bowling, outings at the zoo, camps, and weekend retreats. The adult care provider also attends our weekend family retreat as the camp doctor, which gives the child with SCD and their family an opportunity to meet with the adult care provider in an informal setting. Also, after the formal transition to the adult service, our young adults can continue to meet with members of the pediatric SCD team in the outpatient setting. Our approach of having 1 SCD team across the life span reduces some of the anxiety that may arise during the transition period and improves communication between the pediatric and adult SCD teams. The families report a high level of satisfaction with this comprehensive type of care.

Most recently, with respect to COVID-19, the Chronic Care Model has been instrumental in the development of evolving strategies for the prevention of SARS-CoV-2, and it has helped us to implement an almost seamless transition to telemedicine, for both children and adults with SCD.

References

Andemariam B, Owarish-Gross J, Grady J, Boruchov D, Thrall RS, Hagstrom JN. Identification of risk factors for an unsuccessful transition from pediatric to adult sickle cell disease care. Pediatr Blood Cancer. 2014;61(4):697-701. doi:10.1002/pbc.24870

Artz N, Whelan C, Feehan S. Caring for the adult with sickle cell disease: results of a multidisciplinary pilot program. J Natl Med Assoc. 2010;102(11):1009-1016. doi:10.1016/s0027-9684(15)30727-6

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

Coleman K, Austin BT, Brach C, Wagner EH. Evidence on the Chronic Care Model in the new millennium. Health Aff (Millwood). 2009;28(1):75-85. doi:10.1377/hlthaff.28.1.75

Inusa BPD, Stewart CE, Mathurin-Charles S, et al. Paediatric to adult transition care for patients with sickle cell disease: a global perspective. Lancet Haematol. 2020;7(4):e329-e341. doi:10.1016/S2352-3026(20)30036-3

Renedo A, Miles S, Chakravorty S, et al. Not being heard: barriers to high quality unplanned hospital care during young people’s transition to adult services - evidence from ‘this sickle cell life’ research. BMC Health Serv Res. 2019;19(1):876. doi:10.1186/s12913-019-4726-5

Saulsberry AC, Porter JS, Hankins JS. A program of transition to adult care for sickle cell disease. Hematology Am Soc Hematol Educ Program. 2019;2019(1):496-504. doi:10.1182/hematology.2019000054

Stollon NB, Paine CW, Lucas MS, et al. Transitioning adolescents and young adults with sickle cell disease from pediatric to adult health care: provider perspectives. J Pediatr Hematol Oncol. 2015;37(8):577-583. doi:10.1097/MPH.0000000000000427

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