Current Pediatric Reviews - Volume 8, Issue 2, 2012

Increasing prevalence of children with complex and chronic diseases has occurred in the last half century and will likely continue to occur. These children, their parental caregivers, their families and the health care system are challenged in providing seamless coordinated care that optimizes outcomes in an effective and efficient manner. This paper outlines some potential solutions including clarifying definitions of the population and evaluations of existing and evolving models of care such as the chronic care model, the primary care medical home and non-primary care practice based models, such as comprehensive clinics focused on a specific disease or population, comprehensive clinics focused on a non-categorical population (not focused on a single/limited set of disease(s)), interventions focused on home care; and, other models focusing on more limited types of interventions or policy change. Ongoing growth in impact of complex chronic disease of childhood demands increased attention across sectors of care as an immediate area of focus for child health policy.

Children with complex chronic conditions commonly face health issues related to feeding and nutrition. In this paper, we focus on feeding problems and undernutrition in children with neurologic disabilities. Children with neurologic disabilities often have growth problems related to nutritional and non-nutritional causes. The feeding problems and resulting undernutrition have diverse impacts on the child’s and family’s health, social well-being, and quality of life. Appropriate management of the child’s health issues and support of the family in this process is critical. A systems based perspective on the role of nutrition support programs in the management of children with complex chronic conditions is discussed. We review nutritional assessment and management including the use of technology assistive devices such as gastrostomy tubes and the evidence base around their effectiveness. We also discuss the management of gastroesophageal reflux disease and dysmotility.

This review focuses on the principles and practices of long-term mechanical ventilation in infants and children. The number of pediatric patients receiving chronic ventilation has been growing exponentially over the past decade. Chronic respiratory failure in children is highly variable and the ideal ventilation modality must be tailored to the needs of the individual patient and family. Despite the medical complexity involved in the care of these children, many are optimally managed in the home environment. Successful transition to home with a favorable outlook is aided by frequent follow up, a family centered approach and anticipatory guidance, all delivered by an experienced multidisciplinary team.

In parallel with the evolving recognition of children with medical complexity (CMC) as a specific and important population over the last decade, there has been a simultaneous increase in interest in healthcare quality. This article provides a theoretical overview of healthcare quality followed by a focused review of aspects of quality as they specifically pertain to the care of CMC. The importance of considering the full spectrum from the larger political and economic environment to the individual clinical microsystem is emphasized. Issues around measurement for the purpose of quantifying and improving healthcare quality are addressed, with a brief examination of emerging quality measures specifically for pediatrics and CMC. The Model for Improvement, a method for conducting formal quality improvement is described, and the distinction between this type of approach and traditional biomedical research is explained. A framework for addressing specific domains of healthcare quality is outlined, including safety, timeliness, efficacy, efficiency, equitability, and family-centredness. Challenges with delivering high quality healthcare specific to CMC are detailed, and corresponding specific interventions are described, drawing from an extensive literature review and from our own local experience. Areas of emphasis include barriers to access to appropriate care for CMC, the role of care coordination, hospital care of CMC (recognition of the deteriorating child, medication safety, and care of the child with technology dependence), and the burden of care for families of CMC. Gaps in current knowledge are addressed and recommendations for future research and practice are put forth.

The landscape of neonatal follow up is starting to shift. While there is continued emphasis on the traditional outcomes studied, including cerebral palsy, vision/hearing impairment, and cognitive impairment, there is a shift in focus to those outcomes which are much more prevalent and collectively can contribute to significant morbidity. The purpose of this review is to explore the outcomes of the preterm survivor, in the context of the principles of neurodevelopment. Approaching these outcomes from a developmental perspective provides insight into the challenges in early identification and management of these highly prevalent outcomes. In addition, we will explore the underlying brain injury associated with the preterm infant, particularly as it relates to the high prevalence low severity outcomes. We will also review the impact of this pattern in terms of clinical presentation and academic performance. Finally, we will look at the limitations within the medical system and community that potentially exacerbate the difficulties of the preterm survivor.

Increasing numbers of children living with chronic complex conditions also means an increase in the number of children with a potentially life-threatening illness. While curative and life-prolonging therapies continue to stretch the boundaries of what is possible, pediatric mortality remains highest among this group of children. In light of their medical fragility and unpredictable trajectories, children with chronic complex conditions are ideal candidates for palliative care. Integration of palliative care concurrently with curative or life-prolonging therapies results in improved outcomes and is widely endorsed as the most appropriate model of care for this population of children. This paper dispels common misconceptions about pediatric palliative care and outlines the benefits realized by children living with chronic complex conditions, and their families, when palliative care is introduced early in the course of their illness. Practical suggestions to facilitate difficult conversations and to manage challenging clinical situations are provided.

Significant improvements in the treatment of many pediatric malignancies have led to a growing population of long-term survivors of childhood cancer. Many of these survivors are at significant risk for late physical and psychosocial sequelae (“late effects”) as a result of their prior disease and its therapy. In some survivors (such as children treated for a brain tumor), late effects including endocrine dysfunction and neurocognitive challenges can develop during therapy and persist throughout life. In others (such as children and adolescents treated for Hodgkin’s lymphoma), late effects including congestive heart failure, pulmonary fibrosis and secondary breast cancers may not occur for many years, often once survivors have reached adulthood. During childhood, survivor care usually occurs at the pediatric cancer center, often in a specialized long-term follow-up clinic. However, adult survivors are usually cared for by primary care practitioners in their own communities. It is essential that the health care providers who will care for childhood cancer survivors as they age be aware of each survivor’s treatment exposures, long-term risks, and the surveillance strategies suggested for monitoring for these late effects. Strategies for effective transition from pediatric care and for ongoing communication between primary care practitioners and pediatric cancer centers need to be implemented to ensure that childhood cancer survivors receive appropriate care focused on their specific risks throughout their lifespan.

‘Complex severe obesity’ in children and adolescents can be defined as a BMI >95th percentile for their age and gender in addition to at least one significant obesity-related co-morbidity requiring specialty care, other co-existing chronic illness impacted by obesity, or a BMI ≥99th percentile for their age and gender. These children are at high risk of acute and long-term medical and psychosocial morbidities. Optimal management strategies for this group of patients are not well studied, although general principals include emphasis on a comprehensive family-based lifestyle intervention, while addressing medical and psychological aspects of obesity, as well as motivation to change behaviours. Further treatment options, such as bariatric surgery, may be necessary, but require a skilled interdisciplinary team and careful selection of candidates most likely to benefit from these procedures.

Children with complex illness present a challenge to our current system of care organization. This paper explores the policy issues raised by this new and specific population of pediatric patients, and suggests that effective system re-engineering must consider both the broader demographic and economic realities informing health system decision-making as well as the dominant health policy prerogatives of the health system writ large. The fundamentals of the policy process will be emphasized and a road map for change will be presented.