To receive comprehensive care, adults with hydrocephalus need continuous access to expert surgical and medical providers. Recognition of surgical complications and access to surgical expertise are critical, because cerebrospinal fluid shunts can continue to fail in adulthood.12–14
Medical management for young adults with hydrocephalus must address monitoring for and treatment of adult conditions15
and facilitation of self-care (eg, medication knowledge, ability to discuss care with providers, insurance knowledge).16–20
For young adults with spina bifida, medical management must also address long-term follow-up for associated conditions (eg, epilepsy, cognitive delays, scoliosis, renal impairment, mobility issues)21
; prevention of secondary conditions (eg, urologic infections, renal calculi, pressure ulcers, osteomyelitis)22
; and facilitation of self-care (eg, bowel and bladder function).17–21
Vocational counseling is essential, because employment and financial independence are linked to well-being.16,23
Related life issues that the providers need to be cognizant of include autonomy,18
transportation, child care, insurability,16,23
psychological and/or behavioral services,23
assistance for advocate-parent(s),24
and end-of-life issues.18
Appropriate obstetric management can result in normal pregnancy and delivery for women with hydrocephalus.25–29
Other reproductive issues include genetic evaluation and counseling,28
contraception, parenting, and child care. Finally, patients' holistic well-being, including alternative therapies to address body, mind, and spirit, should be considered. Models of care for adolescents and young adults with hydrocephalus should address each of these health care needs.
Transition Care Models for Young Adults With Chronic Conditions
The goal of transition care for young adults with special health care needs is to maximize lifelong functioning and potential through the provision of high-quality, developmentally appropriate health care services that continue uninterrupted as the individual moves from adolescence to adulthood.4
It is patient centered, responsive, continuous, comprehensive, and coordinated4
; however, leaders of the health care system are struggling with this national priority,30
including who should provide transition care, how it should be delivered, and how to finance it.31–33
Several models of transition care for adults with specific pediatric medical conditions exist, including cystic fibrosis,20
congenital heart disease,34
type 1 diabetes,35
and rheumatic disease.36–38
We are unaware of any formal transition care models for young adults with hydrocephalus (Harold Rekate, MD, written personal communication, November 7, 2008). Proposed approaches to transition that help to promote self-management for young adults with chronic conditions and bridge the communication gap between pediatric and adult care providers and include appropriate timing and sufficient preparation for the process itself,39
incorporation of a medical home model,5,40,41
establishment of joint clinics between pediatric and adult providers,42
and electronic personal health records43–45
that include appropriate legal documentation.
Existing transition care models and approaches usually emphasize transition to care that is provided by adult surgical and medicine providers in adult medicine settings. Indeed, only 10% of pediatric neurosurgeons report providing care to their patients as they transition to adulthood (Harold Rekate, MD, personal communication, November 7, 2008). Given a lack of a systematic approach to hydrocephalus transition and adult care, we have termed the state of existing care for young adult with hydrocephalus the “ad hoc” approach.
Ad Hoc Care Model Fails Adults Who Live With Childhood-Onset Hydrocephalus
Ad hoc approaches to follow-up care for young adults most often result in inconsistent and fragmented care. Within 3 years after closing 1 pediatric multidisciplinary clinic, one half to two thirds of patients failed to maintain adequate follow-up.46
In the United Kingdom (where health care coverage is universal), two thirds of patients' shunt function went unassessed after age 16.13
Individuals with childhood-onset hydrocephalus and their parents share troubling anecdotes about the ad hoc approach.33
One 19-year-old woman with shunt failure was admitted to a psychiatric facility for mental status changes, and the mother of a 29-year-old man with recurrent shunt failure was prosecuted for Munchausen's syndrome by proxy. Inappropriate follow-up results in serious morbidity46
Adults without a designated provider to coordinate care often fail to maintain adequate follow-up care and subsequently endure severe and potentially preventable morbidity.
Ad Hoc Care Model Exists Because of Economic Challenges
The economic challenge of providing integrated health care for adults with chronic conditions is daunting.47
Whereas pediatric care for eligible individuals is supported by state and federal agencies through Medicaid, adult health care for adults with chronic conditions is often fragmented.33
Obtaining adequate private health insurance is challenging for adults with chronic conditions. Although individuals may qualify for Medicaid, the program has an uncertain future and is complicated by poor provider reimbursement and, therefore, a limited number of participating providers. As a result, health care options for adults with childhood-onset hydrocephalus are few, and although traditional integrated care is essential, it is rarely available.
Ad Hoc Care Models Fail Because Adult Health Care Providers Are not Trained to Care for Young Adults With Hydrocephalus
Health care for these individuals is increasingly delegated to adult surgical and medical practitioners. Historically, adult health care providers have not been or needed to be familiar with pediatric conditions such as hydrocephalus. From a surgical perspective, hydrocephalus that is diagnosed and treated in infancy is a very different disease from the type of hydrocephalus that begins in adulthood as a result of hemorrhage, infection, or tumor.48
The pathophysiology of the developing intracranial environment is significantly different from that with which adult neurosurgeons are familiar.
From a medical perspective, adults who live with childhood hydrocephalus have health care needs that are specific to the condition itself, as well as those that are common to all adults. Long-term management of these young adults' condition requires specific provider knowledge, skills, and experience with the underlying condition to render ongoing care and prevent unnecessary morbidity and mortality; however, current adult medicine training programs lack a dedicated curriculum that addresses conditions with pediatric onset. The vast majority of adult providers lack the specific knowledge, skills, and experience to provide adequate ongoing care and are ill prepared to manage an adult's childhood condition, in particular hydrocephalus.49
One exception lies in combined internal medicine and pediatric-trained practitioners. The scope and experience of dual-trained providers make them ideal to deliver long-term care for adults with chronic pediatric conditions.