Because statistics don't tell the whole story: A call for comprehensive care for children with cancer

Abstract

Families facing a childhood cancer diagnosis are catapulted into crisis mode. Everyday family life is whisked away and replaced by frightening diagnoses and medical language, life-or-death decisions, intrusive emergency hospitalizations of unknown duration, a steady stream of hospital visits, medications and procedures that must be handled with perfection, expert opinions that must be acted upon in haste, negotiations with employers for extreme absenteeism, special arrangements with school principals and teachers during long periods of quarantine from other children and friends, and financial burdens that are felt years beyond that dreadful day of diagnosis. This is not a “new normal.” There is nothing “normal” about children being struck by serious illness. I know this well, from personal experience: my daughter, Alexandra, was diagnosed with medulloblastoma in 2011, just before we were to celebrate her fourth birthday. A childhood cancer diagnosis launches an unwanted journey with unrelenting potential for a bad ending. But there is a role for genuine hope in all aspects of the evolving experience. It begins with hope for cure or remission. If survival is possible, it can develop into hope for and a return to childhood, including reintegration into school. Yet many families come to learn that hope's constellations may change over time, particularly if disease progresses or recurs. In the face of such tragedy, with the horror of learning that death is certain, the hope may become one for perfect comfort to the end. No matter the course, the goal of providing care and comfort that supports a full life for all children with cancer must be supported and attainable. With cancer, grief begins at the time of diagnosis, when the family learns that their child is sick and will experience necessary harm in order to survive. In the event that a child emerges cured of cancer, he or she will likely face permanent and lifelong damage from treatment, ranging from mild to severe. Life will never be the same again, and the family's future shifts sharply from what was expected. When this grief and shift in expectations is addressed, it can be managed and the family can begin coping. As a cancer epidemiologist, I was better equipped than most parents to face down this crisis. But that realization frequently left me wondering: how did families with little medical knowledge or inflexible work schedules manage their fears and navigate the numerous daily unknowns? What is on the line is the life of your child. Worse yet, as we would be counseled in great detail, the cost of trying to save our young daughter would be certain and permanent cognitive devastation. Having to choose between treatment strategies that are terrible and terrible really presents no choice at all. Cancer in children presents a different illness experience from cancer in adults and triggers fundamentally different needs in families. Unfortunately, however, because of a lack of dedicated pediatric cancer research support, clinicians are regularly forced to recycle adult treatment approaches in pediatric cancer and, with good intentions to protect the families, they often do so without adequately acknowledging the significant and potentially fatal problems with this course of action. It is firmly established that children are diagnosed with cancer types that differ from those in adults, and even cancers that are the same behave differently in a child. Unlike adults, children's bodies are still growing and developing during illness and treatment; therefore, cancer treatments will affect them unequally over their life compared with adults. The long-term and lasting implications of cancer treatment on a child's physical, emotional, and cognitive functioning are increasingly evident.1, 2 Specialized medical care, and the research that guides it, must be tailored to address these unique circumstances, challenges, and family needs. We have seen successes for some cancers, but others remain incurable and untreatable. And no matter the prognosis or survival statistic, the literature is clear that preventable suffering is a part of the experience that interferes significantly with the quality of life of these children and their families.3 The accompanying article by Ward et al reporting statistics for childhood cancer incidence, mortality, and survival is an essential inventory to help evaluate our progress and shortfalls.4 Importantly, for the first time in over a decade,5 instead of presenting these figures in the aggregate, these more comprehensive data tease out several specific cancer types to capture a clearer picture of the actual childhood cancer landscape today. These figures demonstrate tremendous variation in survival and success rates across the different cancers affecting children. Moreover, while overall the cancer incidence numbers reported here may be considered comparatively small when measured against the much higher incidence among adults, cancer is still the leading cause of disease-related death (second only to unintentional injury in all causes of death) in children aged 5 years to 14 years.6 For families facing these unfortunate odds, characterizing childhood cancer as “rare” or survivable in “80%” of children is dismissive of the gravity of the impact on the young lives afflicted with cancer. Moreover, it fails to account for cancers that are terminal at the time of diagnosis or those that have widely varying behavior in terms of treatment response, and it fails to acknowledge that the state of the science for some childhood cancers has stagnated for decades. Finally, for some treatable cancers, the steep cost of survival is one of gross mental impairment, poor motor functioning, and highly compromised social well-being for decades of life after treatment. Associating aggregated information with the very unique cancers and circumstances of childhood cancer misrepresents the unyielding burden these children and their families must bear over their lifetimes. As a cancer professional, I understand why cancer clinicians present the information as they do: they want to keep hope for survival and a positive future at the forefront. But as a parent, I know that parents want information and want to feel fully informed. They do not want false hope. And research with parents has borne this out.3, 7, 8 There are many childhood cancers that carry a grim prognosis from the outset. For cancers that do exhibit survival probabilities in the “80%” range, the percentage fails to acknowledge that the overwhelming majority of these children will have significant, chronic, and life-altering side effects as a result of their cancer treatment. Clinicians often recognize and are inspired by the strength, resilience, and wisdom of children and parents facing life-threatening diseases such as cancer. Even very young children have remarkable insights and awareness about their condition. Although parents may not be experts in disease and medical decision-making, and may initially feel overwhelmed with what they do not know, they are the exquisite experts on their children and also the keepers of their family's needs and values. As such, parents deserve to be asked about their individual touchstones for ensuring their child's well-being and about their care goals and values for their child and the whole family. Ensuring that parents have a voice and shared role in the critical decisions is especially vital for helping families live with the fewest regrets. This focus on family engagement and support will require that we address the flaw inherent in today's health system that emphasizes treating disease without requiring concurrent palliative care to also support the patient and family. Fortunately, the American Cancer Society's advocacy affiliate, the American Cancer Society Cancer Action Network, has helped to lead efforts with multiple national partners to introduce and build support for quality-of-life legislative proposals in the US Congress and several statehouses as an important initial step in acknowledging that treating the pain, symptoms, and stress of serious illness, at any age and any stage, is as important as treating the disease.9 In addition, we must recognize that communication is central to the work clinicians accomplish. Good communication improves patient and family adjustment to illness, lessens pain and physical symptoms, increases adherence to treatment, and results in higher satisfaction with care.10 It also helps clinicians to enjoy and thrive in their work. To deliver truly person-centered and family-focused care, we must promote an oncology culture and associated academic training that emphasize clinical communication skills that equally balance honesty, empathy, and hope.11 As recommended in the Institute of Medicine's recent report,12 providing communication skills training through cancer programs is essential support for clinicians who aspire to provide optimal care for their patients, particularly among clinicians who already recognize and trust that the awareness of children and their families may be even more realistic and transparent than their own. Certainly many pediatric cancer clinicians are already strong communicators who practice palliative medicine and know well how to frame their conversations, especially the difficult ones, with parents. However, given the sensitive nature and life-changing content of their work, all cancer clinicians need training to be strong communicators who understand the benefits of palliative care. Childhood cancer requires a parent to helplessly stand by while bearing witness to a succession of physical and emotional insults. In the case of Alexandra's cancer and its treatment, she bore craniotomy scars, baldness, pallor, hydrocephaly-induced seizures, a cerebral shunt, severe mucositis, nutrition through a central line in her chest, chemotherapy-induced skin burns, disfigurement from steroids used for weigh