Because life expectancy has increased greatly in the past few decades for individuals living with sickle cell disease (SCD), transition to the adult healthcare setting has become a necessity to continue disease management. Transition for young adults with SCD is associated with declining health outcomes, including increased acute care use and mortality. Nurses can assist young adults with SCD who are at risk after transition by assessing the young adult’s ability to carry out disease self-management, facilitating the supportive role of the family, and recognizing young adults who may have difficulty accessing healthcare resources and providers.
AT A GLANCE
- Transition to adult health care for young adults with SCD poses many risks and burdens.
- Not all young adults with SCD are adequately prepared to care for their disease and independently engage with healthcare providers.
- To address barriers, nurses can assess the young adult’s readiness to independently perform disease self-management and access healthcare resources.
Sickle cell disease (SCD) is an autosomal recessive disorder that causes individuals to produce abnormal hemoglobin that can become sticky and crescent-shaped (National Heart, Lung, and Blood Institute [NHLBI], 2018). As a result, vaso-occlusion is common as the red blood cells stick to each other and blood vessel walls, leading to blocked blood flow, ischemia, and mild to severe pain (NHLBI, 2018). Over a lifetime, organ damage associated with vaso-occlusion can occur in the brain, lungs, kidneys, heart, skin, penis, and joints (NHLBI, 2018). Severe, life-threatening complications of SCD include splenic sequestration crises, infections, acute chest syndrome, aplastic crises, and strokes (NHLBI, 2018). Treatment for SCD is focused on reducing and addressing SCD complications as they develop (NHLBI, 2018).
Transition to Adult Health Care for Young Adults With SCD
Routine screening for SCD and advances in medical treatment have rapidly increased the life expectancy of those diagnosed with SCD from 14 years in 1973 to 40–60 years (Elmariah et al., 2014; Gardner et al., 2016). With more than 93% of children living with SCD reaching adulthood, an effective transition to adult health care provides continuity between pediatric and adult healthcare settings (Quinn et al., 2010). Transition processes ensure that young adults with SCD continue to receive treatment to manage SCD and receive preventive care to reduce long-term complications (Bryant et al., 2015). However, in the United States, young adults with SCD transitioning to the adult healthcare system face numerous barriers, and this time period is associated with increased morbidity and mortality (Blinder et al., 2013, 2015; Brousseau et al., 2010; McClish et al., 2017).
Challenges faced by young adults with SCD range from simple logistic issues (e.g., finding providers) to more complex challenges (e.g., lacking preparation to independently manage their SCD, discrimination). As individuals with SCD age, there are increasing rates of acute pain crises and healthcare use (Brousseau et al., 2010; Cronin et al., 2019; Pope et al., 2016). It is critical that young adults with SCD successfully establish a trusting relationship with an adult healthcare provider with the expertise to manage the long-term complications of SCD in adulthood. Even so, trouble identifying adult hematologists with SCD expertise is commonly reported by healthcare providers and individuals with SCD (Lunyera et al., 2017; Travis et al., 2019). In the United States, there is a lack of hematologists with SCD expertise, forcing young adults with SCD to turn to the emergency department for crisis management, leading to missed opportunities to prevent or mitigate severe SCD complications (Blinder et al., 2015; Lanzkron et al., 2018).
Transition for young adults living with SCD is not just a simple handoff of care between the pediatric and adult healthcare teams; rather, it is a process in which young adults assume their adult responsibilities for disease management and develop the knowledge and skills once held by their parents. Although young adults with SCD are legally adults, they may lack disease knowledge and decision-making experience, leading to a deficit in their capacity to perform self-advocacy and self-management necessary for maintaining continuity of care in the adult healthcare system. Their developmental age may further complicate their abilities to interact effectively with adult providers during appointments. In addition, young adults may have disruptions in their insurance coverage during the transition or lose coverage altogether, further reducing access to care (Bemrich-Stolz et al., 2015). Finally, they may grieve the loss of the previous relationship with their long-term healthcare providers and be unsure how to work with the new healthcare team that may approach SCD care differently (Bemrich-Stolz et al., 2015).
In the United States, a majority of individuals living with SCD are African American, and they may face discrimination when seeking medical care (NHBLI, 2018; Rizio et al., 2020). Individuals with SCD often become experts at managing their pain and are knowledgeable about medications and doses that have worked for them in the past (Reiss et al., 2005). Unfortunately, this knowledge and experience with their chronic disease can be perceived as drug-seeking behavior, particularly when they ask for specific therapies to treat their pain in emergency departments (Elander et al., 2003). Such negative encounters with adult healthcare providers may decrease the chances that young adults with SCD will continue to engage with the adult healthcare system to pursue routine SCD care (Jenerette et al., 2014; Mathur et al., 2016; Matthie et al., 2016). In addition, discrimination may encourage the development of mistrust toward adult healthcare providers, further preventing the formation of positive patient–provider relationships.
Nurses have a role in assessing the preparedness of young adults for transition. Formal transition programs could assist young adults to develop the knowledge and skills necessary for transition. Standard programs can teach self-management behaviors so that patients demonstrate desired outcomes (e.g., independence, medication adherence). It is important for nurses to assess self-management behaviors for all patients; however, for the young adult SCD population, there are additional factors to consider because of the complex complications, such as chronic pain, associated with SCD. Resources to assess transition readiness and tools to assist with continuity of care are available and can be used to identify young adults who are risk for complications and need assistance developing disease self-management skills (see Figure 1).
In the pediatric healthcare system, family is routinely included in the care of the child; nurses in adult health care can facilitate the development of self-management skills in young adults by engaging supportive family members. Although it should be expected that young adults take primary responsibility for their disease self-management, decision making, and communication with the healthcare team, family caregivers can provide support and assist the young adults to advocate for their own needs with their new adult healthcare providers (Sobota et al., 2015). Nurses could capitalize on family caregivers’ willingness to support their children as they enter the adult healthcare system and support family caregivers as they encourage their children’s independence in disease self-management.
Although it is common for youth to be transferred to adult health care based on age rather than developmental readiness, the young adult’s ability to access healthcare providers and resources remains the basis for this decision. Nurses can assess if young adults with SCD know how to do the following:
• Find appropriate providers.
• Know when and how to call their providers for follow-up.
• Understand how to obtain insurance.
• Comprehend how to perform basic skills, such as refilling prescriptions or scheduling appointments.
These skills may seem routine, but young adults with SCD may lack these abilities, and they may become a barrier to the young adults with SCD seeking routine health care.
Increasing numbers of children with SCD are now living to adulthood and will make the transition to adult health care. They will often face significant barriers to maintaining continuity of care. Young adults with SCD are legally adults who may be considered independent and able to manage their disease. However, nurses can recognize developmental age and determine that young adults with SCD may not be fully prepared for healthcare responsibilities. Nurses can assist in this transition by assessing and intervening to remove identified barriers that reduce the young adult’s ability to access care or perform self-management of health behaviors required for the prevention of SCD complications.
About the Author(s)
Maureen Varty, BSN, RN, is a PhD candidate and Lori L. Popejoy, PhD, RN, FAAN, is an associate dean for innovation and partnerships and an associate professor, both in the Sinclair School of Nursing at the University of Missouri–Columbia. The research reported in this article was supported by the National Institute of Nursing Research of the National Institutes of Health under Award Number F31NR018574. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. Varty can be reached at firstname.lastname@example.org, with copy to CJONEditor@ons.org.
Bemrich-Stolz, C.J., Halanych, J.H., Howard, T.H., Hilliard, L.M., & Lebensburger, J.D. (2015). Exploring adult care experiences and barriers to transition in adult patients with sickle cell disease. International Journal of Hematology and Therapy, 1(1), 1–5. https://doi.org/10.15436/2381-1404.15.003
Blinder, M.A., Duh, M.S., Sasane, M., Trahey, A., Paley, C., & Vekeman, F. (2015). Age-related emergency department reliance in patients with sickle cell disease. Journal of Emergency Medicine, 49(4), 513–522.e1. https://doi.org/10.1016/j.jemermed.2014.12.080
Blinder, M.A., Vekeman, F., Sasane, M., Trahey, A., Paley, C., & Duh, M.S. (2013). Age-related treatment patterns in sickle cell disease patients and the associated sickle cell complications and healthcare costs. Pediatric Blood and Cancer, 60(5), 828–835. https://doi.org/10.1002/pbc.24459
Brousseau, D.C., Owens, P.L., Mosso, A.L., Panepinto, J.A., & Steiner, C.A. (2010). Acute care utilization and rehospitalizations for sickle cell disease. JAMA, 303(13), 1288–1294. https://doi.org/10.1001/jama.2010.378
Bryant, R., Porter, J.S., & Sobota, A. (2015). APHON/ASPHO policy statement for the transition of patients with sickle cell disease from pediatric to adult health care. Journal of Pediatric Oncology Nursing, 32(6), 355–359. https://doi.org/10.1177/1043454215591954
Cronin, R.M., Hankins, J.S., Byrd, J., Pernell, B.M., Kassim, A., Adams-Graves, P., . . . Treadwell, M. (2019). Risk factors for hospitalizations and readmissions among individuals with sickle cell disease: Results of a U.S. survey study. Hematology, 24(1), 189–198. https://doi.org/10.1080/16078454.2018.1549801
Elander, J., Lusher, J., Bevan, D., & Telfer, P. (2003). Pain management and symptoms of substance dependence among patients with sickle cell disease. Social Science and Medicine, 57(9), 1683–1696. https://doi.org/10.1016/S0277-9536(02)00553-1
Elmariah, H., Garrett, M.E., De Castro, L.M., Jonassaint, J.C., Ataga, K.I., Eckman, J.R., . . . Telen, M.J. (2014). Factors associated with survival in a contemporary adult sickle cell disease cohort. American Journal of Hematology, 89(5), 530–535. https://doi.org/10.1002/ajh.23683
Gardner, K., Douiri, A., Drasar, E., Allman, M., Mwirigi, A., Awogbade, M., & Thein, S.L. (2016). Survival in adults with sickle cell disease in a high-income setting. Blood, 128(10), 1436–1438. https://doi.org/10.1182/blood-2016-05-716910
Jenerette, C.M., Brewer, C.A., & Ataga, K.I. (2014). Care seeking for pain in young adults with sickle cell disease. Pain Management Nursing, 15(1), 324–330. https://doi.org/10.1016/j.pmn.2012.10.007
Lanzkron, S., Sawicki, G.S., Hassell, K.L., Konstan, M.W., Liem, R.I., & McColley, S.A. (2018). Transition to adulthood and adult health care for patients with sickle cell disease or cystic fibrosis: Current practices and research priorities. Journal of Clinical and Translational Science, 2(5), 334–342. https://doi.org/10.1017/cts.2018.338
Lunyera, J., Jonassaint, C., Jonassaint, J., & Shah, N. (2017). Attitudes of primary care physicians toward sickle cell disease care, guidelines, and comanaging hydroxyurea with a specialist. Journal of Primary Care and Community Health, 8(1), 37–40. https://doi.org/10.1177/2150131916662969
Mathur, V.A., Kiley, K.B., Haywood, C.J., Bediako, S.M., Lanzkron, S., Carroll, C.P., . . . Campbell, C.M. (2016). Multiple levels of suffering: Discrimination in health-care settings is associated with enhanced laboratory pain sensitivity in sickle cell disease. Clinical Journal of Pain, 32(12), 1076–1085. https://doi.org/10.1097/ajp.0000000000000361
Matthie, N., Hamilton, J., Wells, D., & Jenerette, C. (2016). Perceptions of young adults with sickle cell disease concerning their disease experience. Journal of Advanced Nursing, 72(6), 1441–1451. https://doi.org/10.1111/jan.12760
McClish, D.K., Smith, W.R., Levenson, J.L., Aisiku, I.P., Roberts, J.D., Roseff, S.D., & Bovbjerg, V.E. (2017). Comorbidity, pain, utilization, and psychosocial outcomes in older versus younger sickle cell adults: The PiSCES Project. BioMed Research International, 17, 4070547. https://doi.org/10.1155/2017/4070547
National Heart, Lung, and Blood Institute. (2018). Sickle cell disease. U.S. Department of Health and Human Services, National Institutes of Health. https://www.nhlbi.nih.gov/health-topics/sickle-cell-disease
Pope, M., Albo, C., Kidwell, K.M., Xu, H., Bowman, L., Wells, L., . . . Kutlar, A. (2016). Evolution of chronic pain in sickle cell disease. Blood, 128(22), 1297. https://doi.org/10.1182/blood.V128.22.1297.1297
Quinn, C.T., Rogers, Z.R., McCavit, T.L., & Buchanan, G.R. (2010). Improved survival of children and adolescents with sickle cell disease. Blood, 115(17), 3447–3452. https://doi.org/10.1182/blood-2009-07-233700
Reiss, J.G., Gibson, R.W., & Walker, L.R. (2005). Health care transition: Youth, family, and provider perspectives. Pediatrics, 115(1), 112–120. https://doi.org/10.1542/peds.2004-1321
Rizio, A.A., Bhor, M., Lin, X., McCausland, K.L., White, M.K., Paulose, J., . . . Bronté-Hall, L. (2020). The relationship between frequency and severity of vaso-occlusive crises and health-related quality of life and work productivity in adults with sickle cell disease. Quality of Life Research, 29(6), 1533–1547. https://doi.org/10.1007/s11136-019-02412-5
Sobota, A.E., Umeh, E., & Mack, J.W. (2015). Young adult perspectives on a successful transition from pediatric to adult care in sickle cell disease. Journal of Hematology Research, 2(1), 17–24. https://doi.org/10.12974/2312-5411.2015.02.01.3
Travis, K., Wood, A., Yeh, P., Allahabadi, S., Chien, L.C., Curtis, S., . . . Sheehan, V. (2019). Pediatric to adult transition in sickle cell disease: Survey results from young adult patients. Acta Haematologica, 143(2), 163–175. https://doi.org/10.1159/000500258