Asset-Based Health Care for Children With Severe Neurologic Impairment

My son Colson began receiving care for suspected mitochondrial disease when he was 3 weeks old and was officially diagnosed via genetic testing at 4 months of age. Care for mitochondrial disease is supportive, and Colson’s disease progressed rapidly in his first year. His symptoms included chronic metabolic acidosis, severe hypotonia, failure to thrive, optic atrophy leading to blindness, neutropenia, early-stage chronic kidney disease, restrictive lung disease, and intractable epilepsy. Colson’s epilepsy initially manifested as treatment-resistant infantile spasms, leading his neurologist to refer our family to palliative care when he was 7 months old. At this time, the likelihood of Colson living with severe neurologic impairment (SNI) became devastatingly clear to me and his father. As his condition progressed and his care became increasingly complex, I identified 3 fundamental benefits of our relationships with many of Colson’s specialists that significantly shaped our experience of parenting him: (1) a consistent focus on his holistic baseline that accounted for both his medical status and quality of life; (2) early, frequent, and ongoing discussions of hope, which enabled our agency as parents and gave us permission to change what we hoped for over time; and (3) support to live in a meaningful present despite our significant grief over his condition and health trajectory. I’ve characterized the relationships we had with Colson’s clinicians as “asset-based.” This is a term borrowed broadly from asset-based community development strategies used in public health and other community-based interventions that focus on strengths and resources that exist, not just outstanding needs or problems.¹  Through an asset-based approach, Colson’s father and I were able to thoughtfully consider our son’s care throughout his life in ways that were meaningful and strengthening to us as his parents and ultimately beneficial for him.

Children with SNI have static or progressive central nervous system conditions that are permanent and result in medical complexity.²  The conditions associated with SNI often result in multispecialty care and frequent interactions with the health care system.²  Despite these frequent interactions, families report that they feel their child’s quality of life is often misunderstood by clinicians.³  This is exacerbated by the many clinical problems and coordination challenges that clinicians need to address when caring for children with SNI. Although these tasks are critically important, a sole focus on problems during health care interactions can leave families of children with SNI worried about how clinicians view their child, particularly when a child, like Colson, lives at the intense intersection of medical complexity and developmental disability. Existing data suggest that parents report this emphasis on problems adds to caregiver stress and strain because they need to provide greater advocacy and vigilance to relay the benefits and not only the burdens of their child’s care to clinicians.⁴  Instead, parents want their child’s clinical team to see their child as they do. This includes not only addressing problems as they arise, but also seeing what is bringing joy, meaning, and good days to their child and family and that adds context for the decisions they make for their child alongside clinicians.⁵ 

From a provider perspective, problem-focused care can be balanced with opportunities to proactively celebrate what is going well for children with SNI and their families. Although it is certainly the clinician’s duty to prepare families for the medical and developmental challenges that children with SNI often face, it is equally important that these concerns are shared in conjunction with the triumphs that are possible and the hopes that always exist, even as they change form.⁶  Building a shared, holistic understanding of a child’s baseline health and quality of life provides important points of consideration and reflection that can guide care for the child, even as that baseline may decline. Studies suggest that families want to share these aspects of their child’s life with clinicians so that there is alignment with the health care system that supports their family.^5,7 

In Colson’s case, my husband and I experienced peace as parents when we could move beyond the confines of his intense home-based care, provide him with a range of positive sensory experiences like baths and walks at the ocean, and visit new places as a family because these were important baseline indicators of Colson’s quality of life. I looked forward to updating his clinical teams during appointments about our adventures, and they were always willing to help us think about how to take these journeys safely. These opportunities to celebrate small milestones, or “inchstones,” were crucial.

We, as a parent and providers, encourage clinicians to be explicit about their approach to balancing honest clinical assessment with hope for the best quality of life for as long as possible. For example, clinicians might say to parents, “We will always share information in an honest way. Although our knowledge is informed by medical training and our experience with similar families, you are the expert in your child. We know that your child is so much more than their medical complexity; we encourage you to share with us all the parts of your child’s life that you feel are important.”

Although focusing on what can be celebrated may spread fears of providers giving too much unrealistic hope, this concept has increasingly been refuted in studies that suggest that hope is an adaptive and necessary part of coping.⁸ 

As Colson’s mother, I felt the role of hope in his care was 2-fold. For most of his life, hope provided a helpful counterbalance to the difficulty of his diagnosis and gave me and his father critical motivation to continue to provide complex, home-based care without any nursing support. Hopes shifted, but were almost always present. For example, when Colson received a gastrostomy tube at 14 months old, we hoped for him to develop skills to eat independently, even if just for pleasure. When intellectual disability and dysphagia made this impossible, we shifted hope to him being able to tolerate his tube-feedings and continue to gain healthy weight, which he did. In lieu of eating for pleasure, Colson learned to taste for pleasure, with bits of ice cream on his tongue and fruit-flavored lip balm. These were moments of enjoyment for him, and meaningful, nonmedical care for us as his parents. Second, toward the end of his life, the absence of hope for recovery to his early baseline was an important data point for us in considering care options. I felt strongly that his health was declining as his body grew bigger and his most difficult symptoms of seizures, vomiting, and airway clearance worsened, despite quality treatment. Colson’s food tolerance also decreased, and his limited expressive energy waned. I had a growing sense that the intensity of his required care was not sustainable or beneficial given the reality of his overall wellbeing. Realizing I was running out of hope for symptom improvement and caregiving stamina were important indicators that the time for comfort-focused end-of-life care was moving closer.

We, as a parent and providers, agree that putting what brings joy and enables hope and good days at the center of health care for children with SNI allows clinicians to align with parents and tap into additional sources of meaning and perspective-taking about the child. And, equally as important, its diminishment or absence gives clinicians and parents a relevant indicator of when shifts in care might be considered.

Families of children with SNI experience a range of stressors, with chief among them being grief.⁹  As Colson’s mother, I recall the moment we received a handout from the genetic counselor that said mitochondrial disease could be fatal as the worst moment of my life. My first and only child was just 3 weeks old, and I had to face the knowledge that I would likely outlive him. I was worried that my heartbreak, which felt so debilitating, would prevent me from caring for him the way I needed to. Over time, my husband and I learned to organize around a tenant of pediatric palliative care we obtained from Courageous Parents Network,¹⁰  a nonprofit organization that provides education and support to parents and clinicians caring for children with serious illness: “the better the before, the better the after.” We had many touchpoints with clinicians that reinforced the “better” aspects of Colson’s life and care, while simultaneously preparing us for the “after” that occurred when Colson died.

Outpatient clinic documentation that brought attention to positive experiences since Colson’s last visit, while also addressing his medical status, was one source of meaningful input for our family. His medical “home” in biochemical genetics provided comprehensive physical and social evaluation and related documentation every 6 months. His father and I looked forward to reading these visit summaries because they reinforced Colson’s relative stability at home and the positive outcomes of our caregiving. For example, his clinician noted things such as the delivery of Colson’s adaptive seating and that he felt comfortable in it; the completion of his first individualized education plan with the local school district; and Colson’s positive relationship with his grandparents. Identifying what was going well highlighted Colson’s strengths and those of our family and counterbalanced my grief about the limitations in Colson’s life.

I felt that even at points of decline in Colson’s health, clinicians helped frame our decisions as meaningful choices, rather than mandated interventions. His pulmonology team and I conducted a home-based nighttime bilevel positive airway pressure (BiPAP) trial when his sleep study indicated central and obstructive apneas. Colson didn’t tolerate the BiPAP and his father and I felt that forcing it detracted from his quality of life. Although this medical intervention was not the solution it could have been, it did help us, with his pulmonologist, prioritize other, less-invasive overnight options like positioning. As Colson’s mother, I felt validated when his pulmonology team took my concerns seriously. One of our priorities for his quality of life was a gentle sensory environment, and because he was blind, the BiPAP was very disorienting to him. Working with his team to consider alternate solutions gave me great comfort then and gives me even more comfort since his death as I grieve, knowing that I advocated for a solution that would work best for him, not just his symptoms.

As providers, we’ve observed that these opportunities for connection and meaning-making around treatment decisions can build trust with parents by demonstrating that clinicians are committed to “good” outcomes, not just managing what is “bad.” An asset-based approach can support parents in the grief that occurs throughout the illness trajectory for children with SNI associated with health setbacks as well as at end of life, knowing that the child was seen and cared for as their parents felt was best. It also can promote communication when clinicians share worries about an undesired outcome in conjunction with what can be hoped for by showing that they clearly see the child’s unique personality, quality of life, and times of flourishing. A list of recommendations is shared in Table 1.

Colson died of acute respiratory distress syndrome at the age of 4 years. As Colson's mother, I feel that our family’s asset-based relationship with clinicians helped us shape a carefully calibrated quality of life for Colson that balanced meaningful decision-making with the stark realities of his condition. In these relationships, his clinicians solicited and trusted my expertise about Colson’s needs, demonstrated knowledge about his medical status and their understanding of their role on his multispecialty team, and took time to know Colson as his own unique person. Establishing these relationships early in Colson’s disease allowed me and his father to shape a purposeful life for him, while examining the edges of its complexity and brevity.

The authors thank the many parents and families who have generously shared their experiences with us and the clinicians who provided expert, compassionate care for Colson. The authors also thank Blyth Lord, founder and executive director of Courageous Parents Network, for her review of this manuscript.