Transition to adult care providers is a process that takes several years in the best of circumstances, and stakeholders (patients, families, and providers) still may face significant barriers for success, even with early discussion and planning. For YSHCN, specifically those with childhood neurosurgical issues, obstacles that are unique to this population have been identified in current literature (Vinchon et al. 2012; Rothstein and Li 2015; Rekate 2009).

First, the spectrum of neurosurgical disease in children is different from their adult counterparts. The clinical significance of certain brain insults, such as brain cysts and neurovascular disorders, tends to be more dramatic and requires neurosurgical intervention in pediatric patients. Additionally, CNS tumors of childhood, craniofacial deformities, and congenital spine disorders may present in young adulthood but are not commonly treated by adult providers versus their pediatric counterparts (Rothstein and Li 2015).

Hydrocephalus, which is one of the most common lifelong neurosurgical conditions that develop in early childhood, presents unique challenges. Although the exact number of persons currently with this condition is unknown, annually it is the diagnosis for 69,000 patients at discharge in the United States, of which half are children (Patwardhan and Nanda 2005). As a sole diagnosis, hydrocephalus in children accounts for nearly 40,000 admissions, upward of 433,000 inpatient days, and cumulative hospital charges of 2.0 billion US dollars each year (Simon et al. 2008). Based on current treatment patterns and numbers, it is projected that young adults (ages 18–35) will exceed 40,000 in the next two decades (Simon et al. 2009).

For the pediatric neurosurgeon, management of hydrocephalus and placement and revision of CSF shunts are significant components of practice (Rekate 2009). In general neurosurgery, adult-onset hydrocephalus is infrequent by comparison and is often acquired after trauma or brain tumor. With extensive experience, the pediatric neurosurgeon is adept at troubleshooting shunt issues that may have subtle or atypical symptoms commonly seen in childhood-onset disease.

With the advancement of neurosurgical procedures in the last 30–40 years, one of the greatest medical challenges today is caring for young adults with myelomeningocele (Rothstein and Li 2015; Rekate 2009). During childhood, health care for these patients is often coordinated and comprehensive. Many are followed in spina bifida clinics utilizing a multidisciplinary approach to patient and family care. The literature notes that once transferred to adult care, there is limited access to medical subspecialties, and care is reported as fragmented, resulting in untoward patient effects (Rothstein and Li 2015). Primary care providers are overwhelmed, complications are overlooked, and, as parent advocates age, there is decreased involvement in care (Camfield and Camfield 2011). Furthermore, the child with myelomeningocele who was functionally and medically stable through childhood and early adolescence can have significant deterioration of gait and walking, premature aging of the bones, and neuro-orthopedic issues such as kyphosis and scoliosis in young adulthood. There is increased morbidity and mortality in this population during or shortly after transition from tethered cord, syringomyelia, and failure of shunts and endoscopic third ventriculostomies (Rothstein and Li 2015). How much the lack of medical resources plays a part in this is unclear.

As mentioned earlier in this chapter, the survival rate for many of the pediatric CNS tumors diagnosed annually has been steadily increasing. For the survivors, latent effects of disease and treatment often become apparent years later in adolescence or young adulthood. Sequelae include secondary malignancies, fertility issues, seizures, cognitive disorders, and pituitary deficiencies (Rothstein and Li 2015). The ability for adult practitioners to recognize and appropriately manage these patients becomes challenging with little experience to guide them. Mental health concerns such as depression and anxiety are frequently under diagnosed in this young adult population. Potential reasons are decreased access to care, concern about medication interactions and adherence, and/or drug diversion. This is another contributor to a potential decline in health and overall quality of life for these emerging adults.

So what to do about transitioning these special children to adult neurosurgical practices? For some patients, they are fortunate in that their neurosurgeons have “double citizenship” and can take care of them cradle to grave. For many others, their pediatric practitioners must hand them off to adult providers due to regulations and resource limitations that restrict their scope of practice. A solution that is utilized by many pediatric neurosurgeons is to have privileges at institutions that provide care for patients older than 21 years so that continuity of neurosurgical care can be maintained (Rothstein and Li 2015).

From a professional standpoint, there are several organizations that are heavily involved in examining issues related to childhood-onset neurosurgical disorders. The section on neurological surgery of the AAP has been vocal in advocating for transition planning and defining strategies for this complex patient population. The Joint Section of Pediatric Neurological Surgery of the American Association of Neurological Surgeons (AANS) and Congress of Neurological Surgeons (CNS), which was established as a forum for sharing pediatric neurosurgical experiences and promotes education, research, and patient care standards, also has put the topic of transition high on their list of priorities. The American Society of Pediatric Neurosurgeons (ASPN) has recently updated their vision statement to include the reality of pediatric neurosurgical problems persisting into adulthood. The role these professional societies play in the support of viable care models is an important one. Although there is most likely not one care model that will fit all regions and situations, these organizations can establish guidelines that can be customized to individual neurosurgical practices (Rekate 2009).

Similar to their physician counterparts, nursing professionals will undoubtedly experience increased exposure to the growing number of youth and adults with special health-care needs. These complex patients are no longer limited to pediatric or subspecialty practices. Adult primary care, occupational medicine, and other various inpatient and outpatient environments of care will continue to see an influx of this population.

In response to this phenomenon, nursing curricula need to include education and clinical training that will prepare future providers to meet the needs of adults with childhood-onset neurological disease. Most often, nurses are in the position of having sustained contact with this patient population and can be the main facilitators of transition (Sharma et al. 2014). Important skills will include the ability to interact with patients who have cognitive and communication deficits and specific strategies to incorporate patient advocates during the patient encounter. Nurses who traditionally practice the “clinician-patient” model may need to learn how to incorporate an advocate while fostering autonomy and acceptance of the ASHCN. In addition, development of a transition nurse specialist role in primary care and hospital facilities that care for adolescents and young adults with special health-care needs can be helpful (Carroll 2015).

Experienced pediatric neuroscience nurses have the advantage of practicing under this model of care. Many of the common neurological and neurosurgical conditions start at birth or early childhood and are typically managed in primary care and subspecialty environments. Pediatric neuroscience nurses who care for the transitioning adult have experience in clinical assessment related to the neurosurgical condition, patient and family teaching, and developmental knowledge, all which are an asset to facilitating a plan of care. Referrals to community resources, coordination of care with other providers, and facilitation/implementation of therapies are well within the nurses’ scope of practice. The value of nursing support to any program that manages YASHCN cannot be understated.

With regard to advanced practice, nurses have been integral to many transition programs. Betz and Redcay (2003) described an innovative nursing model for a transition clinic led by family nurse practitioners and is based in part on the Creating Healthy Futures model used in the educational system. The clinic was unique in that it provided comprehensive services to adolescents and young adults and involved extensive prescreening, identification of unique needs, and follow-up to assure successful transition (Betz and Redcay 2003). Outlined in a later report, the role of the advanced practice registered nurse (APRN) as a transition services coordinator for the clinic was described, as well as the skills necessary to provide care and serve as a case manager and community liaison for this underserved population (Betz and Redcay 2005). Most transition clinic models reinforce the unique role of the nurse, clinical nurse specialist, or nurse practitioner as key to the success of the clinic and the confidence of the patient.

Certain limitations of APRN scope of practice have become an issue as the pediatric population ages. Pediatric nurse practitioners (PNPs) who have traditionally cared for the child with hydrocephalus and spina bifida or other chronic neurological disorders may be prohibited from doing so once the patient exceeds the age of 21. Depending on the State Board of Nursing Regulations, which vary from state to state, a PNP may be practicing outside her/his scope of practice by managing a 23-year-old. In another state, it may be within the scope if the patient has a chronic “pediatric onset” problem. It is vital that each APRN knows what the practice statement or policy is for the state she/he practices in.