The Transition Process: Initial Assessment and Development of a Treatment Plan

Fig. 1.1

Summary of the recommended steps for transition of care

The costs associated with non-transition of this population are both financial and human. Human costs include lost productivity of caregivers of persons with disabilities as well as lost productivity of the patients themselves. Caregivers of myelomeningocele patients reported a reduced productivity of 6.2–13.3 h per week [1]. Financial costs to the health care system, although poorly quantified, are substantial. These come in the form of increased costs of reactive versus preventative care, increased morbidity of disease, and increased utilization of costly emergency services. Estimates suggest that 34–47 % of admissions for adult myelomeningocele patients were for potentially preventable conditions, including urinary tract infections and wound problems. These estimates amount to approximately $364 M over a 2-year period [2, 3]. In an era of ever-expanding costs, focused preventative care of this population may provide reduced financial burden on the health care system and improved overall quality of life for patients.

The public health industry recognizes that lack of coordinated transition leads to substantially increased medical and societal costs and therefore has supported implementation of transitional programming in many public policies. The ACA has called transitional services as an “essential health home service” and the US Health and Human Services identified transition as one of six core performance outcomes necessary for comprehensive care of children with special needs. Despite a substantial amount of public health resources aimed at bolstering transition of care for emerging adults with special needs, it is estimated that only about 40 % of this patient group met the national standard for transition of care [4], a number that has shown essentially no improvement over the past 4 years [5]. As of 2005, 76 % of adults with chronic congenital anomalies did not have or could not identify a primary care doctor [6].

While the bulk of the burden of transition rests with the primary care provider, the urologist is not immune. The genitourinary tract is one of the systems most frequently affected by congenital defects. Such defects can be isolated (e.g., hypospadias) or involve multiple organ systems (e.g., myelodysplasia). In addition, patients with neurological conditions of childhood like cerebral palsy (CP) and neuromuscular diseases often have substantial urological comorbidity that is progressive with aging. For a small subset of this group of transitioning individuals (e.g., those born with exstrophy), the urologist is often the most knowledgeable care provider about their condition, anatomy, and long-term medical risks associated with the disorder. As such, the urologist may indeed serve an important role in facilitation of primary care needs for some of these individuals. An additional two subgroups that should be considered for transition are survivors of pediatric and pelvic cancers and renal transplant recipients. While these individuals may not have primary congenital urological issues, their urological needs in adult life may be very different from those in pediatric life, a long-term result of their childhood medical story, and they may have delayed acquisition of adult milestones of independence [7].

Traditionally, the pediatric urologist has served this diverse population throughout childhood and adulthood. Pediatric urologists are well equipped to do so with their knowledge and experience with congenital anatomy, their lifelong relationships with the patients and families, and focus on integrative care. However, as these patients now are surviving to adulthood with excellent health, issues of sexuality, postpubertal genital appearance and function, urinary and fecal incontinence, and fertility and pregnancy become increasingly important health and quality of life issues. Moreover, with aging and development of other medical comorbidities, patients with congenital anomalies experience typical age-related urological problems, such as BPH and prostate cancer, which are often complicated by their coexisting anomalies and prior operations. The field of urological congenitalism requires a knowledge base and skillset that pool the assets of both pediatric and adult urology (Fig. 1.2).

Fig. 1.2

(a) Graphic portrays relative importance of urological needs for a male patient with myelomeningocele throughout his lifespan. (b) The field of urological congenitalism combines the specialty skillset of pediatric and adult urology

While the approach to a typical adult urological patient is one problem: one solution, the patient with a congenital urological problem rarely presents in that manner. These patients have a lifelong history of treatment by other medical care providers and those experiences, both good and bad, often influence their expectations. Other limitations, including ambulation status, cognitive deficits related to lifelong hydrocephalus (for some), or nutritional challenges may make treatments more difficult or risky. Finally, these individuals may have variable dependence on other care providers at home and in the community, so it is critical to ensure that their input is part of the initial assessment. For some who have had a parent assist with self-care for their whole lives, the aging parent may be dealing with his/her own medical and physical constraints as well as the (often unspoken) distress about who will care for their child in the future.

As such, one should provide adequate time for careful consideration of the problem, patient education, and consideration of the patient’s and family’s needs (Fig. 1.3). Every evaluation should follow a step-wise approach. These include: (1) defining the patient’s baseline, (2) understanding the patient’s goals, resource constraints, executive and cognitive assets and deficits [8, 9], and social constraints, (3) characterizing the “new” or “worsening” complaint, (4) determining the appropriate diagnostic tests to define the problem, and (5) developing a menu of treatments that will address the concern and delineating the associated personal “costs” or risks of each. Oftentimes, development of a care plan may involve careful negotiation, a substantial amount of investment of time educating the patient about expectations, and communication with other medical providers.

Fig. 1.3

Summary of key components of initial evaluation of an adult with congenital urological needs

Step 1: Defining Baseline

While the population about which we write is heterogeneous, most will require a baseline assessment of urological function. This includes renal function, continence status/use of protective garments, urinary infections, sexual function and activity, fertility (past and present), and prior urological medical and surgical treatments. Assessment of renal function may be symptom based, require noninvasive evaluation like renal ultrasound and serum electrolytes, or may be more involved. For those with neurogenic bladder, whether related to spinal dysraphism on non-neurogenic bladder dysfunction related to posterior urethral valves or exstrophy complex, baseline function is ideally established using urodynamics or video urodynamics and then followed symptomatically and with periodic repeat studies every 2–5 years depending on baseline results. For patients with continent pouches who may have some baseline renal insufficiency, involvement with a nephrologist early is helpful to help manage complex metabolic challenges and secondary problems related to chronic acidosis and chronic kidney disease (CKD). It is now well recognized that renal insufficiency at all stages has substantial downstream effects, including increased systemic inflammatory markers and lipids, and results in higher all-cause mortality and cardiac mortality [10]. Slowed progression of CKD may not only prevent the burden and cost of renal replacement therapy (RRT), but may also prevent early death due to cardiac disease. Consideration of non-creatinine-based methods of estimation of GFR (iothalamate renal scan or cystatin C) may be warranted for patients for whom creatinine production is not normal; for example, patients who are non-weight-bearing, have short stature, or atypical body habitus.

Urinary continence and urine management with catheters often changes as a young person progresses through puberty, related to weight gain and other factors. Moreover, the social implications of incontinence may also change. This is particularly relevant for young individuals who are progressing from a home environment to a shared-living environment such as a college dorm. Understandably, compliance with self-catheterization may be difficult when a new living situation does not allow the individual to do this discreetly. Assistance with troubleshooting these challenges is relevant for some of our emerging adult patients.

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