Growing Up With Sickle Cell Disease: Hospital Medicine and Health Care Transitions

The phase of health care transition, a stage when adolescents with diseases of childhood onset are making the change from receiving their health care from pediatric-trained providers to adult-trained providers, has long been known to be a period of increased vulnerability. Patients experience significant changes and challenges in their social, psychological, and medical lives. Many adolescents and young adults with chronic illness face increasing acute care needs and hospitalization during this time.¹  Patients with sickle cell disease (SCD) are additionally challenged with chronic pain that limits function and leads to stigma within both public and health care environments. Nearly all patients with SCD are of a minority race, are more likely to be from low socioeconomic backgrounds, struggle to complete their education and maintain employment and adequate health insurance, and experience systemic racism in medicine.^2,3  In the transition phase, adolescents and young adults with SCD must face all these challenges at the same time they are entering an adult health care system that has a shortage of providers who are trained to care for their disease process.

The current study by Fasipe et al⁴  highlights the dangers that the tenuous time of transition holds for patients with SCD, revealing increasing rates of hospitalization and mortality in the young adult time period. Specifically, this study uses data from the Nationwide Inpatient Sample over 15 years to establish trends in demographics and in-hospital mortality for patients with and without any form of SCD aged 16 to 24 years. The findings reveal that although hospitalizations increase with age for all patients, the odds of mortality for patients aged 22 to 24 years with SCD were nearly 3 times higher than the odds for those aged 16 to 18 years over the 15 years studied. This mortality trend was not appreciated in the non-SCD population. High health care use, particularly emergency department visits and hospitalizations, in adolescent and young adult patients with sickle cell disease has been demonstrated previously.^5,6  Unfortunately, patients with SCD report facing many barriers to adequate care when experiencing unplanned hospitalizations during the time of transition. They express that hospital staff does not listen to their concerns, that adult providers do not know enough about their disease, and that they feel out of place on the adult floors as a young adult.^7,8  In fact, patients note that their concerns often result in them electing to discharge against medical advice, a finding also noted in the current study.

Structured transition programs for patients with SCD can improve young adult outcomes, such as increasing patient knowledge and disease self-management, building trust and communication between pediatric and adult providers, and improving preparedness for the transfer to adult care.^9,10  However, many patients with SCD still are not supported by formal transition programs, resulting in an abrupt change to an adult provider that may lead to unsuccessful integration into adult practice with resulting patient attrition and loss to follow-up.¹¹  Adult providers may assume the transition process is complete after transfer of the patient with SCD into their care; instead, they should be hypervigilant to the ongoing progress of these patients into adulthood. There must be an acknowledgment of the unique needs of patients who have lived their lives with a chronic, debilitating disease to ensure they continue to receive high-quality care in both the inpatient and outpatient settings. Because of a lack of adult providers trained to care for SCD, the complexity and challenges of this disease may be inadequately managed by an adult primary care provider receiving the patient from a pediatric specialist. Adult providers who deliver care to patients with SCD must be comfortable managing complications of disease, including having knowledge of available therapies, chronic pain management, and the impact of psychosocial factors on each patient’s physical and mental well-being.

Although an increase in transition programs from pediatric to adult outpatient clinics has been observed in recent decades, there continues to be little focus on the transition concerns related to emergency department visits and acute hospitalizations. Age limitations within children’s hospitals and pediatric emergency departments often mean that acute care needs for patients with SCD result in the abrupt transfer to adult inpatient care when transition is not yet complete in the outpatient setting. This discordance in timing of transfer can impede or delay the overall transition process and result in confusion for the patient regarding their expectations and obligations as an adult in the health care setting. Issues such as loss of insurance, educational and vocational training, self-management, and communication or engagement with the health care system are often addressed in a reactive, rather than proactive, fashion. Deficiencies in these areas become particularly apparent during episodes of acute illness, when mounting psychosocial stressors place young adult patients at a disadvantage, making them less able to adequately assimilate health care instructions and use this information meaningfully in future encounters. It is therefore imperative that pediatric and adult sickle cell providers in both the inpatient and outpatient setting reconsider their roles in the transition of young adults with SCD. There must be an intentional effort conducted over the continuum of the patient’s care: not only in a single setting, not only in times of wellness, but through every health care encounter, ensuring that the patient has the greatest chance for success. This can best be achieved by increasing training of providers, formalizing transition programs, and using interdisciplinary teams working together with patients and families.¹² 

Hospital admission is not typically thought of as an opportunity for intervention in health care transitions, but many pediatric hospital medicine (PHM) providers agree that they should have an active role in providing these services.¹³  Although pediatric hematologists may more commonly care for the inpatient pediatric sickle cell population, PHM providers commonly care for adolescents and young adults with special health care needs, many of whom will face similar complications and barriers to transition. Hospital medicine providers are well versed in the process of transitioning patients because discharging a patient back into ambulatory care has many similarities to transitioning a patient from pediatric to adult health care. Both processes rely heavily on adequate communication between the provider and the patient and family and from one provider to another. Educating patients and families on their disease, preparing them to assume independent care, and ensuring communication with the provider receiving the patient after discharge are some of the mainstays of PHM practice. Using these same communication skills, pediatric inpatient providers can help prepare patients and families for the process of transitioning their care to adult providers. Advising adolescents and caregivers on the differences in the environment and expectations within the adult health care system, building skills of self-advocacy, and ensuring that patients can communicate their health care needs appropriately are skills that inpatient providers can continue to foster during an acute care hospitalization.¹⁴  Additionally, although many adult hospital medicine providers report frequent encounters with patients with diseases of childhood onset, they often report discomfort with managing those disease processes and uncertainty over whom to contact with care-related questions.^15,16  Pediatric inpatient providers can serve as a source of communication for their adult hospital medicine colleagues, particularly regarding patient populations that tend to be high users of inpatient services and may be well known to the inpatient pediatric team.

As pediatric inpatient providers, we should find it unacceptable that patients with SCD cared for in our facilities as children face the dire outcomes described in the current study by Fasipe et al⁴  as they age into young adulthood. Although patients with SCD may participate in structured outpatient transition processes, unplanned hospitalizations during the time of transition may abruptly place them in the care of adult providers before that process is complete. Adult providers may be unfamiliar with or uncomfortable caring for SCD or not use to managing young adults who may have not yet achieved health care independence. Young adult patients may find adult hospitals intimidating and less family centered than the pediatric inpatient setting. Pediatric inpatient providers are therefore uniquely poised to assist in health care transitions by contributing to the preparation of the patient and family while also serving as a resource to the adult providers who will be taking over their care. Successfully transitioning our patients into adult care is an essential aspect of our duty as pediatric clinicians, and PHM providers have an opportunity to play a vital role in the process.