The Likely Impact of Congenital Zika Syndrome on Families: Considerations for Family Supports and Services

Medical Complexity and Lifelong Severity of Impact

The authors of a recent review concluded that CZS, at least in children with obvious symptoms at birth, differs from other congenital infections, with 5 distinct features: (1) “severe microcephaly with partially collapsed skull; (2) thin cerebral cortices with subcortical calcifications; (3) macular scarring and focal pigmentary retinal mottling; (4) congenital contractures; and (5) marked early hypertonia and symptoms of extrapyramidal involvement.”⁵ Infants with these characteristics of classic CZS will almost certainly have severe or profound cognitive and motor impairments. Other medical and developmental outcomes are becoming clear, involving multiple systems and including seizures, hydrocephaly, dysphagia, vision and hearing impairments, and sleep problems.^6–8

Longitudinal surveillance and natural history studies are needed to understand the full range of time-varying effects of CZS on outcomes such as growth, neurologic sequelae, cognitive and functional impairment, vision, hearing, motor development, communication, and social skills. We do not know whether or how many children will be able to walk, speak, use the toilet independently, or perform a range of other adaptive daily living skills. But even without such studies, it is safe to say that most children with CZS signs or symptoms at birth will have major impairments in comparison with typically developing children that will not be easily treated by surgery, medication, or therapy services. As “children with medical complexity,” infants and children with CZS will have substantial health care needs, severe chronic impairments, major functional limitations, and a high need for coordinated care.^9,10 Their families will face a lifetime of caregiving responsibility and the resulting direct and indirect economic burdens (eg, see Bailey et al¹¹ and Ouyang et al¹²). To address the multisystem involvement and functional limitations, they will require services from a variety of disciplines,¹³ including pediatric neurology, neurosurgery, ophthalmology, audiology, speech and hearing sciences, physical therapy, occupational therapy, special education, psychology, and social work.

Ideally, a service coordinator and a transdisciplinary team would serve as the central coordinating point for families. Those within disciplinary care organizations in some communities have demonstrated effective models for integrating medical and behavioral health services, often using a nurse or social worker as a care manager to serve as both the intermediary and the coordinator of services (eg, see Mastal et al¹⁴). But such services are not available in many communities, and the reality is that most families will need to play a major role in finding services, integrating information across multiple providers, and taking on professional roles in providing care throughout the day.¹⁰

Uncertainty About the Unfolding Consequences of CZS

Although we know much more about the consequences of ZIKV infection in pregnancy than we did even a year ago, much remains to be learned.⁶ The lack of longitudinal research means that neither the families of infants with obvious birth defects nor the professionals who serve them will be able to predict outcomes. The life expectancy of children with CZS is unknown, although the authors of initial reports suggest a fatality rate in Brazil as high as 8% to 10% in the early years.¹⁵ The extent to which this rate is due to a lack of services in certain areas of Brazil or whether this rate would be expected in other countries is unknown, but clearly the risk for infant death is a reality about which families will be greatly concerned. Caregivers will be living in a constant state of uncertainty about the emergence of new symptoms (such as seizures), their child’s future life, the possible death of their child, and their own lives as parents.

But only a small subset of infants exposed to prenatal ZIKV infection have obvious abnormalities at birth. For some families, unfortunately, the relief will be short-lived, because some exposed infants will rapidly begin to display abnormalities during the first months of life, as a result of continued ZIKV replication in the brain after birth.¹⁶ For example, the authors of a study of 13 infants with prenatal ZIKV infection without microcephaly at birth showed that all had brain abnormalities consistent with CZS.¹⁷ Surprisingly, head growth decelerated after birth, and by 5 months, 11 had microcephaly. The authors of another study reported ocular and neurologic findings in the absence of microcephaly in a 6-day-old infant.¹⁸

But the evidence to date suggests that most infants exposed to prenatal ZIKV infection will develop few, if any, obvious symptoms during the first year or 2 of life. A large unknown factor is the extent to which they are at risk for later-onset developmental delays, behavior problems, or other subtle impairments (eg, attention-deficit disorder, hyperactivity, learning disabilities). Only through large, longitudinal population cohort studies will it be possible to determine the full relationship between exposure and outcomes and identify factors (eg, genetics, environmental exposures) that contribute to variability in outcomes.¹⁹

The uncertainty that many parents inevitably will experience is analogous to the experience of those described as “patients in waiting,”^20,21 who are individuals with a diagnosis or risk factor that places them in a position of vulnerability, requiring ongoing surveillance to determine when and if symptoms will appear. For some parents, knowledge of prenatal ZIKV exposure could result in over-attribution of causality, prompting questions as to whether any health problem that emerges over time is the result of early ZIKV exposure or whether it is simply a normal pattern of illness. Furthermore, the fact that most people do not even know if they have had a ZIKV infection suggests that elevated anxiety may be possible for other women living in high-risk geographical regions. If they have children with mild learning or behavior problems, they may wonder whether they were unknowingly infected during pregnancy.

Limited Professional Knowledge and Services

Families of children with developmental disabilities in the United States and around the world experience challenges with limited health care and therapy services²² and often are in the position of having to advocate for more or more appropriate services for their children.²³ But in the case of CZS, most professionals will not have previous experience on which to draw. The Centers for Disease Control and Prevention, the American Academy of Pediatrics, and other professional associations have provided limited guidance for clinicians,^24–26 but this guidance continues to be updated, reflecting the rapidly changing knowledge base. Many parents will understandably be frustrated with the lack of knowledge and expertise, and some will find themselves traveling long distances to find a specialty clinic.

Providing Accurate and Understandable Information

Because CZS is a relatively recent phenomenon, information about natural history, likely outcomes, and appropriate treatments will be limited. Many professionals will never have seen or worked with a child with CZS; thus, both families and professionals will face challenges in getting accurate and up-to-date information. This information will likely come from multiple sources. Most families will be using the Internet and social media to learn as much as they can, and, of course, the quality and accuracy of this information will vary considerably. Professionals should help guide parents to relevant and trusted sources for information, building on parents’ interests as well as professional perceptions of the timely importance of new information as it relates to the family’s or child’s current circumstance. Unfortunately, much of the information likely to appear in the media or in professional literature will be focused on the unfolding nature of problems associated with ZIKV infection. Professionals will need to balance competing desires for simple yet comprehensive information, help maintain hope and optimism in the face of discouraging reports, and help parents access information in their primary language.

Of all the needs expressed by families of children with disabilities, the need for information consistently emerges at the top.³⁶ Families need information to understand their child’s special needs, talk knowledgeably with professionals, and find appropriate services. Accurate and useful information can empower families to engage as active and informed participants in partnerships with professionals.

Using Active Surveillance Strategies

Surveillance refers to the systematic and regular use of standardized screening or assessment tools, paired with clinical observations informed by knowledge of the condition and the range of possible secondary complications. Given the limited knowledge about the natural history of ZIKV during the early childhood years, ongoing surveillance of child development, behavior, and health must be a central part of family-centered services. In addition to professional assessments, parent observations and reports will be essential as part of ongoing assessment, providing important information about the functional consequences of CZS and a way to compare standardized clinic assessments with activities of daily living. With their reports, parents can provide critical information about symptoms, patient characteristics, and overall functioning and well-being.³⁷ These reports are essential for capturing information about functional adaptation and time-varying events, data almost impossible to collect in a clinic-based assessment.

Ideally, surveillance should be informed by clinical practice guidelines, which, in the case of CZS, will be constantly evolving. Hopefully, as recommended protocols evolve, health care professionals can share data so that, in the absence of formal studies, we can begin to build a more robust understanding of the primary and secondary consequences of CZS. Active surveillance can reassure parents that someone is paying attention to their child’s well-being, so that services or treatments will start promptly when needed. Engaging parents as active participants in surveillance is another way to empower families and to help ensure that treatments and services are aligned with family goals and priorities.

Providing Individualized Treatments

Children and families will need individualized treatments and services, and an interdisciplinary approach will almost certainly be called for. General interventions to support parenting and family functioning may need to be paired with more intensive services aimed at addressing specific needs. The timely provision of services will be important to maximize prevention and minimize secondary or tertiary consequences for children and families. Ideally, professionals should work to ensure that treatments or recommended practices meet family-identified goals, fit into family routines, and maximize community participation.

Two major areas of targeted interventions have been used to help support families of children with disabilities. The first type builds on the substantial literature in which authors have demonstrated the power of effective parenting, especially maternal responsivity, on the development and behavior of children with intellectual and developmental disabilities (eg, see Warren et al³⁹). The primary focus of these interventions is on enhancing parenting practices and improving responsivity, on the assumption that such programs will have a direct effect on children’s development, as well as on the secondary effects on family adaptation, by enhancing confidence in parenting and promoting children’s ability to participate in family routines and the activities of daily living. These programs are time intensive and require substantial family involvement, but for motivated parents, a robust evidence base reveals the efficacy of several parent training models.²⁷

The second area of targeted interventions is built on compelling evidence that stress is almost a universally experienced consequence of having a child with a disability,⁴⁰ especially for parents with children who have high levels of behavior problems or complex medical needs.^4,9 As such, the primary focus of these interventions is on reducing parent stress and enhancing quality of life. Major approaches include attempts to strengthen informal support networks, parent support groups, counseling to reduce marital strain, coping and stress management skills, and mindfulness training,^28,41 all of which have been demonstrated to have positive effects for some parents under some circumstances.

The question of whether 1 approach is preferable to another is not so relevant as is the question of which approach or combination of approaches is right for a particular family. The authors of recent reviews have demonstrated benefits of both approaches, highlighting the potential synergies between the 2 but also highlighting potential problems.^27,28 For example, a parent training program could have negative consequences for families already stressed by the demands of caring for a child with complex medical needs, adding a further burden of time and possible feelings of guilt if program recommendations are difficult to implement in the home. Likewise, the benefits of a stress management program might be limited in the absence of improvements in the child’s functional abilities. Crnic et al²⁷ argue that stress clearly interferes with parent training programs, and without addressing the stress parents feel, such programs will have only limited efficacy. These data reveal the need for an individualized approach, building on parent-identified priorities. Ongoing formative evaluation strategies are important to monitor effectiveness and receptivity and to adjust program focus or structure as needed.

Reviewers of telemedicine strategies have shown that they have great potential to relieve anxiety in families, provide a mechanism for sharing information as it becomes available, and reduce feelings of isolation.¹⁰ New technologies such as those found in mobile health applications with smartphones have been shown to have even greater promise, with the potential to reduce the frequency of clinic or office visits and enable direct services to a geographically dispersed patient pool. Applications based on versatile platforms allow 2-way sharing of information between parents and professionals.^42,43 Parents can provide self-reported or even sensor data, and professionals can deliver individualized guidance based on parent-reported problems. Ultimately, mobile health applications allow the possibility for flexibility in response to changing circumstances and integration of new technologies (eg, wearable sensors) and empirically based interventions (eg, stress reduction techniques, parent-child interaction strategies). These applications also have the potential to be used to monitor the effectiveness of interventions, connect individuals through social media and crowdsourcing, and share information and experiences bidirectionally with professionals.