Appreciating the Abilities of Children With Severe Neurologic Impairment Free

Maria was a 5-year-old girl with lissencephaly admitted for status epilepticus and benzodiazepine infusion to the PICU. As her medications were weaned, her family and the clinical team became misaligned about her baseline neurologic status. Specifically, the medical team thought that Maria was interacting in the way they expected for a child with her presentation and imaging studies, but her family felt that she was distant, uncomfortable, and ill.

Severe neurologic impairment (SNI) “describes a group of disorders of the central nervous system that arise in childhood, resulting in motor impairment, cognitive impairment, and medical complexity, where much assistance is required with activities of daily living. The impairment is permanent but can be progressive or static.”^1,2  The majority of children with SNI rely on their parent and family caregivers for direct care, including help with nutrition, bathing, mobility, interactions within their communities, and medical decision-making.¹  These caregivers know their child best, not only because of the time spent addressing their child’s care needs, but also because of the normative nature of the deep parent-child relationship that exists.³ 

Despite this, existing data suggest that parent and family caregivers can experience not feeling believed by clinicians about their child’s specific abilities and care needs.^3,4  This occurs especially in settings where long-term therapeutic relationships between clinicians and caregivers aren’t possible.³  For example, parents may have difficulty convincing hospital-based clinicians about the need for certain medications to be given together, or that feeds must be given a certain way for their child to have adequate seizure control and feeding tolerance respectively. Parents have also cited lack of a unifying diagnosis or ways their child doesn’t follow the “textbook” as aspects of clinician disbelief and misalignment.³ 

When disbelief occurs, it can result in the need for greater advocacy and vigilance on the part of parents and families on their child’s behalf.³  In studies, parents have shared that feeling unheard in regards to their perspectives on their child’s care needs is a major cause of poor communication and distrust with clinical teams.⁵  This, in turn, creates more stress and strain on the parent. Therefore, not only does disbelief impact the wellbeing of the child, but it also impacts parents and families who have a myriad of other stressors when their child is hospitalized.

Furthermore, known clinician biases about the quality-of-life of patients with SNI also likely contribute to lack of parents and families feeling believed.^6,7  Studies suggest that clinicians may under-acknowledge and underrecognize the added meaning, value, and joy children with SNI bring to their communities and to relationships within their families.^6,7  Clinicians may also underappreciate how much many children with SNI communicate without words about their likes, dislikes, and preferences. Therefore, to improve care for children with SNI, approaches to address families feeling believed need to be delineated and incorporated into clinical care.

So much about how the human brain works to process information and to communicate remains unknown. Historically, neuroscience has elevated the importance of the neocortex and the brain’s aptitude for logical thinking above its other abilities.^8,9  This is based on notions about the evolution of the brain along with the hypothesis that neocortical structures are what differentiate humans from other species in their ability for contextual thinking.¹⁰  Emotions, thought to be located in the limbic system, were once judged to be more primitive forms of human experience that needed to be controlled or modified.¹¹  Yet, recent research suggests that emotional and relational awareness serve a vital role in shaping human development, behavior, learning, and relationships.¹² 

In fact, humans are wired for emotional understanding in ways that extend beyond our abilities to reason. Much of human communication and learning is derived from our emotional and relational experiences.¹³  Although communication is often thought of as the words or gestures that people use to convey ideas, so much of communication occurs subcortically.¹⁴  For example, storytelling that engages human emotion to create strong memories has enabled humans to pass down information through generations. Similarly, understanding and connection are often gained through the arts, such as music and rhythm; light, color, painting; dancing, movement, and vibration.¹⁵  These mechanisms of experience that extend beyond direct verbal means of communication enable humans to experience deep relational and social connections. Moreover, there are large areas of the brain dedicated to recognizing faces, including those that interpret structure and symmetry and emotional expression. This attunement to other people is used from birth to develop attachment with caregivers and to establish important family ties. These connections also extend beyond preferences for familiar faces, to also include voices and smells of close caregivers. These sensory and emotionally oriented aspects of communication, learning, and relationship may be used among children with SNI, each in their own way, to enhance their connected experiences within the world and with caregivers.

As we learn more about the brain’s abilities, it is important to consider how this influences clinical care for children with SNI. Despite certain limitations, many children with SNI may have rich emotional and social connections, in part because of preserved neocortical functions that clinicians cannot understand. Clinicians may underrecognize this through their briefer interactions, but parents are often in tune with these abilities. Lack of clinician attention to a child with SNI’s development, such as their cognitive abilities, emotional attunement, communication skills, and social relationships may be a missed opportunity. Limited understanding of the neuroscience of brain development and adjustment to injury or malformation and limited verbal communication contribute to these challenges. This can profoundly influence clinician interactions and engagement around the child’s experience and with their parents and family caregivers. Instead, opportunities exist to start from a place where clinicians learn from the parent-child relationship to better integrate what family caregivers believe and know about their child. This may enhance an explicit separation of clinical versus value-based views when legitimate disagreement occurs, serving to achieve improved alignment. This can greatly impact the child’s clinical care – and can also enable further efforts that help children with SNI to build upon their individual strengths in terms of their emotional, social or relational, and cognitive skills.

Specific clinical interventions aimed at highlighting the parental perspective and the unique abilities of each child with SNI are needed. Use of photos,¹⁶  patient narratives,¹⁷  and family-centered rounds¹⁸  are all ways of emphasizing these aspects of care. When these child and family-centered interventions have been implemented, studies suggest that they are impactful in improving parent-clinician communication, sense of mutual trust and respect, and changing perceptions about quality-of-life.^17,19  These interventions can also help clinicians with their sense of purpose and perspective-taking in their care of children with SNI.²⁰  Other approaches at the individual or system and training levels that engage the parent and patient experience are shown in Table 1 and warrant further study.

Ultimately, it is imperative to consider parent and family caregivers’ perspectives about their child’s health and well being in health care. Advances in neuroscience continue to expand our understanding about the human brain and its functions. Caring for children with SNI and their families can provide an opportunity for clinicians to expand their attunement to the greater human experience. Engaging with children with SNI and their families in this way is likely to lead to better clinical care and may also accelerate important research that unpacks what remains unknown about the human brain, interconnectedness, and our shared humanity.

Maria’s parents were able to share videos and pictures of her at home with her family and at school with the PICU team. They also placed pictures at the bedside as visuals to help clinicians stay attuned to what they considered to be Maria’s baseline health. As clinicians rotated in the PICU, they were able to discuss the photos with Maria’s parents and address their concerns more fully when discussing treatment decisions. Maria’s parents expressed their appreciation for the way the clinical team involved them and Maria in her daily care. In the subsequent days, they diagnosed Maria with delirium that resulted from over 2 weeks in the PICU and the time she spent on a benzodiazepine infusion. The clinical team was able to provide orientation through light, sounds, and familiar objects at the bedside as well as medication management to ease Maria’s symptoms. Over the subsequent week, Maria returned to her baseline health and was able to smile, interact, and play in ways her family expected and recognized. Maria was able to then be discharged directly from the PICU to home.

We thank the many patients and families who have shared their perspectives with us.