29 Children with Complex Chronic Conditions
Jori Bogetz, Christopher A. Collura, and Christy Torkildson
Introduction to Children with Complex Chronic Conditions
In the United States, advances in pediatric healthcare continue to reduce childhood mortality while allowing children with complex illness to live longer. Many of these children have special healthcare needs (CSHCN)—defined as those “at risk for a chronic physical, developmental, behavioral, or emotional condition requiring health related services beyond that required by children generally.”1 A subset of CSHCN with medically complex illness account for an increasing trend in healthcare utilization including hospital admissions, readmissions, prolonged length of stay, pediatric healthcare expenditure, and death.2,3,4,5
To better account for healthcare use by medically complex children and identify those at risk of death, Feudtner et al. defined complex chronic conditions (CCCs) as “any medical condition that can be reasonably expected to last at least 12 months (unless death intervenes) and to involve either several different organ systems or one organ system severely enough to require specialty pediatric care and probably some period of hospitalization in a tertiary care center.”6 This definition is widely accepted to best account for those children who commonly and recurrently receive care in intensive inpatient settings. To better delineate children with the most intensive healthcare needs among those with CCCs, the term “children with medical complexity (CMC)” is often used. While this terminology may best describe children with substantial caregiver needs, functional limitations, and healthcare service utilization, this chapter will use the umbrella term CCCs to describe the overarching population of CMC.
Children with CCCs and their families live with the lifelong joys of having a child with special needs and also the challenge of life-threatening and life-limiting illness. Specialty pediatric palliative care (PPC) teams partner with the child, family, and primary and subspecialty care teams to navigate a child’s chronic illness trajectory while balancing medical interventions intended to prolong life and restore function while helping the child and family thrive amid life-threatening illness. Increasingly, this includes interdisciplinary complex care teams that provide primary palliative care for patients and families. By centering values-based goals of care, treating physical and emotional pain, and supporting the psychosocial well-being of the child and family, PPC is an essential resource in the care for children with CCCs.7 Throughout the chapter, PPC will refer to the added layer of support provided by specialty pediatric palliative care teams.
Clinical Vignette (Part 1)
Charlie is 2-year-old boy with complex medical needs. He was born extremely premature at 23 weeks of gestation due to preterm labor. He experienced a very complicated neonatal intensive care course, including severe intraventricular hemorrhage, retinopathy of prematurity, and severe bronchopulmonary dysplasia requiring long-term ventilation via tracheostomy. He is assisted by a gastrostomy tube for enteral nutrition and had a ventriculoperitoneal shunt placed due to posthemorrhagic hydrocephalus. He was hospitalized at the children’s hospital for 6-months with the last several months of long-term inpatient care for chronic lung disease, while home nursing was secured to provide necessary support in the family’s house to optimize safe care of a child with chronic technology needs.
His parents, Sarah and Neal, understand that Charlie will likely have significant neurologic and physical disability from complications related to extreme prematurity. They remain hopeful that, with growth and development, he will not require lifelong invasive chronic mechanical ventilation. His family’s goals are to provide medical interventions that help Charlie experience joy and loving relationships with his family.
Illness Trajectories
Palliative care has used illness trajectory frameworks to help map prognostic time frames and anticipated needs of patients in order to best aid healthcare and social service support.8 The trajectory most commonly associated with children with CCCs is “long-term limitations with intermittent serious illness.” This trajectory, developed for adults with chronic conditions like heart failure and chronic obstructive pulmonary disease, details gradual functional decline with sudden and severe periods of deterioration and recovery toward a return to a diminished functional outcome. Acute episodes within this trajectory are often life-threatening and may result in death. For children with CCCs, multiple hospital admissions are strongly associated with decreased survival, yet uncertainty remains.9 Such data support the “long-term limitations with intermittent serious illness trajectory” for children with CCCs because recurrent acute episodes may eventually progress to a life-limiting outcome. Intensive care unit (ICU) admissions for children with CCCs are significantly associated with prolonged length of stay and mortality in the ICU.5 One cross-sectional survey of bereaved parents demonstrated that nearly two-thirds of children with life-threatening CCCs died in the hospital, with more than half dying in the ICU.10 Children with static encephalopathy, congenital and chromosomal disorders, and pulmonary disorders are at highest risk of in-hospital death.10
Using such illness trajectories may be helpful in understanding mortality risk for children with CCCs when recurrent hospitalizations for acute illness occur, but its application to the individual patient with CCCs likely oversimplifies an unpredictable and complicated course. Often recurrent admissions, especially to the ICU, involve multiple diagnostic and therapeutic interventions administered with restorative intent. In addition, for children with CCCs, their functional baseline may be unknown as the illness trajectory does not necessarily begin at the peak of function with subsequent gradual decline. Rather, for many children with CCCs with primarily neurologic, metabolic, congenital, or chromosomal disorders, or complications from premature birth, the “starting point” of the child’s disease—the neonatal ICU (NICU)—may encompass the sickest the patient will be for several years of life. Subsequent neurologic and physical development with multiple medical interventions with restorative intent may facilitate a new, higher functioning baseline (Figure 29.1).
Figure 29.1 Dynamic goals of care.
Unlike an adult patient with chronic illness who may follow an anticipated trajectory of gradual functional decline compounded by acute exacerbations toward eventual end of life, children with rare CCCs may not follow such a predictable course. Figure 29.1 illustrates that goals of care consultation (denoted by the red stars) by perinatal PPC teams may be indicated prior to birth if congenital or chromosomal disease is anticipated. The NICU course often presents a life-threatening hospitalization, and shared decision-making is indicated toward long-term medical interventions that may or may not allow for improved functional status, development, and growth for the infant. Following NICU discharge, acute life-threatening hospitalizations including intensive care utilization may pose greater need for PPC consultation over the age continuum, outlining family-centered and values-driven recommendations regarding further complex medicalization and technology needs. Again, such interventions may or may not have profound restorative effects that allow the child and family to thrive and are balanced against an unpredictable time frame that often leads to an eventual recurrent pattern of hospitalizations, functional decline, and end of life. The dynamic nature of the clinical course for children with CCCs illustrates the need for the close partnership with families that PPC can foster to support the assessment of benefits and burdens of proposed interventions. The long-term relationship between PPC and children with CCCs and their families recognizes the need to “re-goal” or reassess goals of care as the benefit-burden calculus changes over the course of a child’s illness trajectory—meaning what is weighed as beneficial at one point in time may be considered burdensome at another depending on the holistic consideration of the child’s life.11
Health Services Received by CCCs
Hospital data support a growing proportion of admissions in children with CCCs, and though these patients make-up only 10–22% of hospital admissions,2,3 such admissions account for a far greater proportion of intensive care utilization, total hospital days, and nearly half of all pediatric hospital charges.3,12 These data are driven most significantly by children with CCCs related to cardiovascular, congenital, neuromuscular, respiratory, and malignant disease. Readmissions for children with CCCs are influenced by polypharmacy and technology use.
Like hospital admissions for children with CCCs, technology use, including feeding tubes and tracheostomy along with long-term invasive and noninvasive mechanical ventilation, has increased in recent years. Children with CCCs account for the vast majority of gastrostomy tube placements, tracheostomies, and cerebral spinal fluid shunts in children.3,13 Such interventions present high resource utilization and need for hospitalization often occurring in the last year of life.14 Again, such findings speak to the uncertain nature of illness trajectories for children with CCCs. With declining functional status, it is paramount to identify that such decline may predict higher rates of hospitalization, intensive care utilization, and procedural and surgical interventions toward impending end of life and should be a trigger for PPC consultation.
PPC has been demonstrated to increase the frequency of establishing goals of care and advance care planning for children with life-threatening and life-limiting illness. This includes addressing resuscitation status and hospice involvement compared with patients who did not receive PPC consults.15
Clinical Vignette (Part 2)
Charlie has been hospitalized nine times since his initial discharge from the NICU for viral lower respiratory tract infections, new onset of seizures, ventriculoperitoneal shunt revision, and routine airway interventions. He has been diagnosed with epilepsy with multiple seizure types including infantile spasms and is maintained on three antiepileptic medications. He has a new diagnosis of spastic diplegia and is enrolled in the pediatric complex care clinic which specializes in the care of children with complex medical conditions and provides care coordination across the seven subspecialty providers who care for Charlie in the children’s medical center.
Mary and Neal worry that Charlie’s complex medical needs are causing pain and suffering. PPC is consulted to consider goals of care, symptom management, and support the family during this challenging course.
The Impact of CCCs on the Family
Long-standing research has consistently demonstrated the psychosocial impact that CCCs may have on children and families. Studies on the psychological adjustment of parents of children with CCCs have demonstrated increased rates of mental health treatment, depression, and other affective disorders, including disparate coping processes between mothers and fathers.16 Role conflicts significantly impact families of CCCs as parents and often siblings balance their family duties with also being an expert caregiver. Families must accommodate the technically challenging and substantial responsibilities of caring for a medically complex child, including teaching in-home and in-hospital healthcare professionals how best to care for their child while maintaining identities as a mother and father.17 Siblings of children with CCCs may also experience impaired psychological functioning including depression and anxiety and even impaired cognitive development—although they may also experience tremendous growth, responsibility, and pride.18 In addition to psychosocial challenges, families of children with CCCs often experience financial hardship. Many families report changes to employment, financial burdens related to the child’s healthcare costs, and difficulty in paying mortgages or utilities.19 Despite these challenges, families also find strength, resilience, joy, and love as a result of parenting a child with CCCs, and more research is needed to understand these from both parent and sibling perspectives.
As such, the care of children with CCCs must consider family-based outcomes through a comprehensive scope. This should not only consider the health and quality of life of the child, but also the function and resilience of the entire family.7 The role of PPC in the care of the family is consistently cited as an essential standard of care.20,21 Parental and family support from PPC over the trajectory of the child’s illness offers essential critical partnership in decision-making, coping, and identifying mechanisms to address financial stressors, access to mental health services, spiritual support, and counseling.
Transition to Adulthood
As children with CCCs live longer, there are mounting challenges in the transition into adulthood. Often children with CCCs continue living with genetic or congenital, neurologic, or childhood multisystem diseases traditionally cared for by pediatric experts. For many, chronological age alone drives a transition into adult care, and families may be asked to leave a long-standing supportive care structure for a new adult healthcare setting. Such a transition risks imparting a sense of abandonment, loss, and even fear with the loss of care coordination and family support integral in pediatric care.22 PPC can play a valuable role in facilitating a holistic transition by outlining the family’s goals of care with new providers while reviewing advance care planning. Depending on the child’s cognitive capacity, such a transition should support psychosocial development as much as clinical care with careful steps to empower the child to gradually gain ownership of her or his complex medical management when possible. Given the expertise by which PPC builds a trusted clinical relationship with families, PPC is essential to ensure a successful goals-driven transition.
In addition to the clinical and psychosocial transition into adulthood, there are additional legal considerations to ensure a seamless transition across the care continuum. For young adults with significant neurologic or psychomotor impairment who are dependent on their caregivers for medical decision-making and delivery of complex technology-assisted home care, legally appointed guardianship for parents is necessary to ensure the family maintains parental authority for medical and social decision-making for their child. Pediatric complex care programs in partnership with PPC should anticipate the need to legally secure guardianship at the age of 18 for the majority of young adults with severe cognitive disabilities. This planning should begin in early adolescence and requires integral knowledge of a community’s social and legal resources, potential out-of-home placement options, and advance care planning.
Unique Needs of Patients with CCCs and Their Families
This section explores the needs, challenges, and opportunities of caring for children with CCCs and their families. As previously noted, the population of children with CCCs has been increasing steadily with advances in technology and medical care. However, the ability to meet the varied needs of this vulnerable population has lagged behind.23 A 2016 National Academies Press Workshop that focused on quality and accessible care for children with disabilities and complex health needs summarized the challenges many of these children and their families face
Uneven public and professional awareness of their conditions and a research base marked by significant gaps have led to programs, practices, and policies that are inconsistent in quality and coverage. Parents often have to navigate and coordinate, largely on their own, a variety of social, medical, and educational support services, adding to the already daunting financial, logistical, and emotional challenges of raising children with special needs. The unmet needs of children with disabilities and complex medical and educational needs can cause great suffering for these children and for those who love and care for them.24
Home Care and Nursing Challenges
Children with CCCs typically require 24-hour care. Most of these children are dependent on two or more technologies, such as tube feeding and ventilatory support.25,26 Most, if not all, of this care falls to the family caregiver, most often the mother.26,27 Home nursing care is often prescribed to assist the children and families, offer some respite, and allow parents to attend to other children and other needs. Additionally, home nursing can provide education and support for the family to learn how to care for the varied needs of their child.
Home care can be provided in the home with a physicians’ order and third-party payer authorization, most commonly Medicaid.4 Home care can be visit or shift-based care. The care coordination and networks of care needed to support children with CCCs at home can be extensive, and community coalitions can help to strength collaborative ties between organizations.28 Challenges to home care include variability in available services across and within states. Additionally, home care agencies often face significant staffing shortages which impact the number of patients that can be served and the number of shifts that can be covered for any child.
Parents may need to be better prepared for some of these challenges, including the difficulty in filling home nursing hours and the potential for fluctuating levels of support at home over time. For nurses working in home care, there can be significant professional satisfaction, providing autonomy in practice, the opportunity to establish a close relationship with patient and family, and the ability to advocate for the patients’ needs. However, home care can also be challenging for a nurse who is used to working within a structured environment such as a hospital, where the professional providers are in the positions of authority. For many nurses, home care may be a completely new experience. Nurses in home care are guests in their patients’ home; they are not in charge. Add to this the needs of a child with CCCs, sibling support, parents/guardian needs, and the needs of other family members. There are additional challenges in providing care in a patients’ home such as isolation, boundary issues, limited resources, differences in socioeconomic status, and differences in living conditions (such as the number of members in a household). Additionally, practical needs include space for all the equipment, medications, and supplies needed for the care of the child, which can be significant.
The addition of community-based PPC services also increases opportunities for healthcare providers and services for children and their families. Unfortunately, community-based PPC is often not available, and access to services can be difficult. With the Affordable Care Act, children with CCCs nearing end of life can be supported by home hospice in addition to home nursing through the concurrent care provision—although there is substantial variability state to state, and access to services must be determined on a local level. Palliative and hospice care in the home is visit-based: it is different from home visit or shift-nursing care. This is a common source of misunderstanding when a family is first introduced to PPC or hospice care at home, as many expect shift-nursing care similar to what they received in the hospital. PPC is always provided by an interdisciplinary team, whereas home care can be provided by an interdisciplinary team if the physician orders discipline-specific therapies for the care of the patient. Unfortunately, all these factors contribute to more confusion and disconnects for the family.
It Takes a Community: School and Developmental Service Provisions
The primary goal for most families with children with CCCs is to provide as normal a lived experience as possible for their child; for many that includes going to school. School programs for children with special needs are not uncommon; however, the services provided may range from simply custodial to full integration with needed services such as physical therapy, occupational therapy, or speech therapy integrated into the special needs programs.
Grouped services are much more common for younger children in schools than they are for older children and adolescents, and families may have to try to maneuver between school and several different appointments and settings to get the services their child needs.29 A useful tool is care mapping. Antonelli and Lind define care mapping as, “a process which guides and supports the ability of families and care professionals to work together to achieve the best possible health outcomes.”30 This tool helps facilitate the many needs between “home care, healthcare, school and community.” There are “how-to” PDFs available for families and professionals; family information about care mapping can be found at http://www.childrenshospital.org/~/media/care-coordination/caremappingforfamilies21813.ashx?la=en&hash=DF739259501C48A1B099375D79D944F2D5478E2A. For professionals, information can be found at https://www.childrenshospital.org/~/media/care-coordination/caremappingforprofessionals21813.ashx?la=en
Isolation and the Loss of “Normal”
Despite the many appointments a child with CCCs has and the number of individuals needed to provide care for each child, having a child with significant medical needs can be isolating.31 Often the parenting role is focused on the medical needs of their child, determining signs of imminent decline or pain and discomfort. As noted previously, these children require around-the-clock care and technology to help them survive, which means several pieces of equipment to provide that technology. The practical challenge of trying to go anywhere, in addition to the multiple healthcare appointments, can be daunting and so going out of the home is often avoided. Added to that are the risk of infection for children who are often immunocompromised, which often also results in parents avoiding family gatherings or other social events, thus complicating an already isolating existence.32,33,34
Often with the birth of a child with CCCs or a diagnosis later on in childhood or adolescence, there is a sense of loss and grief.31,35,36 This loss includes thoughts of what might have been and the grief that comes with that loss. Amber Conn writes on her blog about the grief she feels since she heard of her daughters’ condition. With more than 1.7 million subscribers, her words hit home for many.
The moment we heard the words “Alexander disease” and “three-to-five-year life expectancy,” I began grieving. . . . I grieve for my child, who was at one point healthy, vibrant, and full of life. I grieve for the life we dreamed of having—watching our child play sports and having play dates like typical kids. (Conn, 2018, https://themighty.com/2018/09/grief-parenting-medically-complex-child/)
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