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Optimizing adolescent transition to adult care for sickle cell disease.

Transitioning health care from a pediatric to an adult in a patient with sickle cell disease presents challenges. A program developed to assist with transition is described.

Children with sickle cell disease (SCD) are now ling into adulthood (Smith, Lewis, Whitworth, Gold, & Thornburg, 2011). Providing a seamless transition--a comprehensive, purposeful, planned move from one setting to another--from pediatric to adult health care is essential. Transitioning health care from a pediatric to an adult environment for young adults with a chronic disease presents unique challenges. Potential challenges include moving from family-centered care to patient-centered care, promoting social and financial independence, navigating a complex health care system, and addressing anxiety related to new health care providers (Baines, 2009).

Literature Review

A literature review was conducted of the last 7 years by using PubMed, Academic Search[TM] Premier, Centers for Disease Control and Prevention, Institute for Healthcare Improvement, National Institutes of Health, Cumulative Index to Nursing and Allied Health Literature, and Medline. The key terms used in the search were sickle cell anemia, sickle cell disease, transition, transition to adult care, and chronic illness transition.

Transitioning health care to an adult setting is a long process that needs to begin in the pediatric environment and involve the family. Current practice can be abrupt and devoid of preparation (Smith et al., 2011). Some apprehensions from young adults in moving to adult care include having inadequate information about transition, forming new relationships with an adult care team, and feeling anxious about becoming the responsible person for their health care (DeBaun & Telfair, 2012).

Additional transition challenges include limited access to adult providers with skills and/or interest in caring for patients with SCD, inadequate communication, and inconsistent follow up (Treadwell, Telfair, Gibson, Johnson, & Osunkwo, 2010).

Early discussions encouraging self-management and independence are essential for success in preparing young adults for transition (Kennedy & Sawyer, 2008). Managing sickle cell disease is complex and requires a multidisciplinary approach. When developing a young adult transition program, nurse leaders must incorporate education, self-management skills, psychosocial implications, relationship building with adult health care providers, and orientation to an adult hospital to promote the best possible outcomes (DeBaun & Telfair, 2012). Young adults with SCD transitioning care from a children's hospital to an adult academic hospital expressed concerns in understanding responsibilities and expectations, and in their ability to navigate as an adult.

SCD is an inherited chronic blood disorder that affects red blood cells in patients of primarily African descent. Under certain conditions, these red blood cells become sickle-shaped (crescent shaped) and have difficulty passing through small blood vessels (Lee, Askew, Walker, Stephen, & Robertson-Artwork, 2012). The most frequent clinical manifestation of the disease is recurrent episodes of acute pain in the abdomen, joints, chest, low back, and extremities (Pack-Mabien & Haynes, 2009). These episodes associated with SCD often require treatment with parenteral opioids in the emergency department (ED) and may result in inpatient hospitalization. Complications associated with SCD include pain, stroke, vaso-occlusive crises, delayed growth, lung and cardiac disease, and susceptibility to infections (Lee et al., 2012). SCD is managed with different treatment modalities entailing medication, diet, and chronic transfusion therapy, with the primary focus on health maintenance (DeBaun & Telfair, 2012).

The effects of chronic diseases in childhood are influenced by disease type and severity (Baines, 2009). External factors, such as health care access, psychosocial needs, and community understanding of the disease, play a vital role in the impact of a health issue during childhood (Rapley, & Davidson, 2010). Living with a chronic disease in childhood poses additional challenges, such as complex self-esteem issues, body image disturbances, and difficulty forming social relationships (Newland, 2008). Children with SCD often face additional stressors due to the stereotypical perceptions associated with this disease (Anie, Egunjobi, & Akinyanju, 2010). Some perceptions include miscommunication between patient and nurse, socioeconomic barriers, cultural disparity, and perception of care received. Under-treatment of symptoms may lead to a lack of trust of health care providers, resulting in pseudo-addictive patient behaviors (Wright & Adeosum, 2009).

Life expectancy for individuals with SCD is now into adulthood due to improvements in health care, such as newborn screening, antibiotic prophylaxis, and routine immunizations (Pack-Mabien & Haynes, 2009). Disease management for children with SCD has evolved from crisis management to anticipation, prevention, and early aggressive treatment (DeBaun & Telfair, 2012). Because of the increased life expectancy with SCD, new challenges now arise as affected children mature into adults. Parents play an integral role in navigating the health care system for children with a chronic disease. Few medical management expectations are placed on children in pediatric health care (Smith et al., 2011). As children develop into young adults, parents encounter challenges in having difficulty transitioning medical care responsibilities to their children (Rapley & Davidson, 2010). One challenge facing parents is fear of allowing the young adult to assume responsibility for self-managing his or her disease in an adult health care setting (Smith et al., 2011).

Improvement Needs

Young adults with sickle cell disease transitioning care from a children's hospital to an adult academic hospital expressed concerns during their first inpatient admission about understanding responsibilities and expectations, and being able to navigate an adult health care inpatient and outpatient setting. Approximately 20 young adults with SCD transition their care from a children's hospital to an adult academic hospital per year, and about 90% of them expressed these concerns to the inpatient nursing staff. To assist in preparation for transition to an adult health care setting, an interdisciplinary team from a 500-bed academic hospital partnered with staff from a children's hospital in developing a young adult transition program. The transition program focused on principles of self-management, and expectations and responsibilities as an adult.

Action Plan

An interdisciplinary team of direct-care nurses, a nurse educator, clinical nurse specialist, nurse manager, physicians, social workers, case managers, pharmacists, and emergency staff was formed to develop a program to assist transition for young adults with SCD from a children's hospital to an adult academic hospital. The focus of the transition program was to prepare young adults to accept responsibility for their health care needs and reinforce this independence to their parents. The goal of the interdisciplinary team was to provide a seamless transition of care.

One of the concerns expressed to direct-care nurses by the young adults during their first inpatient hospitalizations addressed differences in the emergency department, triage process, admission process, and layout of the hospital as a whole. One intervention developed by the interdisciplinary team was provision of ED and inpatient hospital tours to address this concern. This tour offered young adults with SCD and their families an opportunity to tour an adult academic hospital and meet providers. Young adult transition tours are offered monthly, prior to the time of their previously scheduled children's sickle cell clinic appointments.

The young adult transition tours are integrated into routine follow-up appointments at the children's sickle cell clinic. The tour starts in the ED as the typical entry point for hospital admissions and the site of initial care during a pain crisis associated with SCD. Young adults, parents, inpatient direct-care nurses, a nurse educator, clinical nurse specialist, nurse manager, physicians, social workers, case managers, pharmacists, emergency staff, and physicians from the adult and children's clinical team are present during the ED tour. The ED educator discusses what the young adults can expect from their arrival in the ED until either admission or discharge. The ED tour commences in the triage area, where young adults are shown the trauma bay as well as individual ED patient care rooms. The ED educator answers questions from the young adults and/or parents related to the department visit.

Next, the young adults and parents are escorted to the inpatient unit for likely admissions. Staff nurses on the inpatient adult unit provide a brief tour of the unit and its private patient rooms. An informational folder is provided that includes tips for communication, responsibilities of being in an adult hospital, hospital mission and vision, and commitment to pain management.

An adult multidisciplinary group meets with the young adults at the informational session to discuss functions of the adult hospital as well as expectations on admission. The adult multidisciplinary group consists of a physician, pharmacist, social worker, case manager, direct-care nurse, educator, clinical nurse specialist, and nurse manager. Representatives of the various disciplines share information about their roles and how they can assist the young adults during inpatient hospitalization. Time is allotted for questions and discussion of expressed concerns related to the management of sickle cell pain crisis during an inpatient hospital stay. Once the session is completed, the young adults and parents are accompanied back to the children's hospital for their follow-up appointments.


The interdisciplinary team continues to strengthen the program based on feedback received from young adults and their families, and members of the interdisciplinary team. Some modifications to the tour were made, including changing the time frame of the tour from 1 hour to 45 minutes, allowing more time for questions and answers, and explaining pain management strategies in greater detail. The adults' and children's interdisciplinary teams meet regularly to address possible barriers, scheduling, and how to transfer medical information between two health care systems.

Nursing Implications

Nurses play a pivotal role in young adult transition programs. Nurses provide education and guidance, and facilitate program development and implementation. Transition programs are essential for young adults with a chronic disease. Discussions regarding the transition of care to an adult health care environment are best when begun in the pediatric health care setting. Lack of discussion and implementation of transition programs may lead to improper use or potential gaps in ongoing treatment once young adults leave the children's environment (Baines, 2009).

This type of program can be adapted for other young adults with chronic diseases. This foundational framework was expanded to meet the needs of the patients. It is essential for nurses to understand development of young adults so they can tailor interaction and communication with them.


Patients and families stated the tours have impacted these young adults. The young adults indicated they had less apprehension about entering the adult health care system, and recognized their responsibilities as patients and their expectations during an inpatient stay.

The adult health care staff members involved in these tours value the opportunity to assist in the transition of young adults and provide a forum for building relationships with these patients. Summary statements from the interdisciplinary team include:

* Great opportunity to meet young adults and families

* Collaboration between inpatient and emergency department

* Partnering with colleagues from children's hospital

* Provide an exceptional service to patients

Transitioning care from a children's environment to an adult health care setting for young adults and families can be a time of apprehension. An interdisciplinary team approach is vital to the success of a transition program.


Anie, K.A., Egunjobi, EE., & Akinyanju, O.O. (2010). Psychosocial impact of sickle cell disorder: Perspectives from a Nigerian setting. Globalization and Health, 6, 2. doi:10.1186/1744-8603-6-2

Baines, J.M. (2009). Promoting better care: Transition from child to adult services. Nursing Standard, 23(19), 35-40.

DeBaun, M., & Telfair, J. (2012). Transition and sickle cell disease. The American Academy of Pediatrics, 130(5), 926-935.

Kennedy, A., & Sawyer, S. (2008). Transition from pediatric to adult services: Are we getting it right? Current Opinion in Pediatrics, 20(4), 403-409. doi:10.1097/ MOP.0b013e328305e128

Lee, L., Askew, R., Walker, J., Stephen, J., & Robertson-Artwork, A. (2012). Adults with sickle cell disease. Home Healthcare Nurse, 30(3), 173-183.

Newland, J. (2008). Factors influencing independence in adolescents with sickle cell disease. Journal of Child and Adolescent Psychiatric Nursing, 21(3), 177-185.

Pack-Mabien, A., & Haynes, J. (2009). A primary care provider's guide to preventative and acute care management of adults and children with sickle cell disease. Journal of the American Academy of Nurse Practitioners, 21(5), 250-257. doi:10.1111/j.1745-7599.2009.00401.x.

Rapley, P., & Davidson, P.M. (2010). Enough of the problem: A review of time for health care transition solutions for young adults with a chronic illness. Journal of Clinical Nursing, 19(3-4), 313-323. doi: 10.1111/j. 1365-2702.2009.03027.x

Smith, G., Lewis, V., Whitworth, E., Gold, D., & Thornburg, C. (2011). Growing up with sickle cell disease: A pilot study of a transition program for adolescents with sickle cell disease. Journal of Pediatric Hematology Oncology, 33(5), 379-382. doi: 10.1097/MPH.0b013e318211bb2e.

Treadwell, M., Telfair, J., Gibson, R.W., Johnson, S., & Osunkwo, I. (2010). Transition from pediatric to adult care in sickle cell disease: Establishing evidence-based practice and directions for research. American Journal of Hematology, 86(1), 116-120.

Wright, K., & Adeosum, O. (2009). Barriers to effective pain management in sickle cell disease. British Journal of Nursing, 18(3), 158-161.

Stephenie Cerns, MSN, RN, ACNS-BC, RN-BC, CHPN, is Clinical Nurse Specialist, Inpatient Medicine/Hematology-Oncology/Palliative Care Unit, Froedtert Hospital, Milwaukee, WI.

Colleen McCracken, BSN, RN, CMSRN, CHPN, is Staff RN/Educator, Inpatient Medicine/Hematology-Oncology/Palliative Care Unit, Froedtert Hospital, Milwaukee, WI.

Claire Rich, MS, RN, ACNS-BC, APNP, CMSRN, is Advanced Practice Nurse Practitioner, Regional Anesthesia and Acute Pain Service, Medical College of Wisconsin, Milwaukee, WI.
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Title Annotation:Clinical Practice
Author:Cerns, Stephenie; McCracken, Colleen; Rich, Claire
Publication:MedSurg Nursing
Geographic Code:1USA
Date:Jul 1, 2013
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