“We found a mass in her liver… it looks like metastatic cancer.”
As a pediatric resident, I received this call one night from a radiologist regarding a two-year-old patient our team had admitted earlier that day with a limp and refusal to bear weight. It was her second trip to the emergency department in a few days.
The first time the plain films of her hips and legs hadn’t shown anything concerning, so she was sent home to continue supportive care. When she returned still refusing to walk, she was admitted for further workup and imaging. The radiologist told me they had found a fracture in her femur, which made sense to me. But he said it also looked like she had a “lesion” at the site of the fracture. He explained that based on the abnormality, they had continued the scans upwards to include her abdomen and had found what looked like a large tumor in her liver.
The likelihood that this small child had a pathologic fracture of her femur secondary to a metastatic cancer was something that none of us had seriously considered while admitting her. A call like this can be surprising even in an adult patient, but in a two-year-old child who, as far as any of us knew, was healthy, this was shocking.
The mom and grandmother were waiting for me in her room to find out the results of the scan. I don’t remember how I went about breaking this news, although I suspect it did not follow the “breaking bad news” protocols I now know so well. I only remember that I sat down to talk, watching this little girl sleeping peacefully, knowing I needed to actually say the word “cancer” no matter how much I didn’t want to say this scary word that would change their lives forever. When I told the mom and grandmother that the scans were very concerning for cancer, I will never forget the grandmother looking at me and saying, disbelieving, “Children can get cancer?”
Children can get cancer. And so many other things that can limit their lives, although do not limit the meaning or impact of their lives. It isn’t fair, and it doesn’t make sense. But statistically, more than 50,000 children from birth through age 19 die in the United States each year. The majority of these deaths occur in infants from birth to age one year, with most of these deaths occurring in the first week of life. Pediatric hospice programs seek to reach out to these children and families, sometimes even before birth in prenatally diagnosed disease, to help prepare for whatever comes next, whether it is better or worse than expected.
As it turns out, realistic expectations for our children are incredibly difficult to determine. As important as it is making decisions and planning for the future, prognostication is complicated in children. Death in childhood is a relatively rare occurrence in this country, thank goodness, and the yndromes, congenital abnormalities and diseases from which children die are often rare. When we are dealing with diseases whose known population is in the hundreds worldwide — if even that — we make our best guess based on trajectory, available treatment options and family’s goals and go from there.
Even when we are involved prenatally with a family expecting a baby with a genetic diagnosis of Trisomy 18 or Trisomy 13, relatively “common” diagnoses on our team, the range we see prognostically is wide. We generally don’t know what to expect until the baby is born. Some of these babies die within a day of birth and never get to go home, while others thrive despite limitations and are discharged from our service after more than a year. While our expectations for quantity of life are often uncertain, our expectations for quality of life in hospice are clear.
How does pediatric hospice differ from adult hospice?
In practicing both, I find that while there are some important differences, there are more similarities. A prognosis of six months or less if the disease takes its normal course still applies for pediatric hospice eligibility. Determining the goals of care is vital, as these goals drive the plan. We still focus on impeccable symptom management and provide support for the entire family unit, including two years of bereavement support. Pediatric hospice teams include nurses, social workers, chaplains and physicians, all ideally with special training in pediatrics.
However, as any pediatrician will tell you, children are not just small adults and pediatric hospice is punctuated by many aspects that also make it unique. Pediatric hospice covers children from birth (and even prenatally in some programs) until age 21. In some cases, we serve children beyond the age of 21 who have chronic conditions of childhood that are mostly managed by pediatricians.
Children develop and process the world surrounding them vastly differently as they age. The five year old and the 15 year old on our team will understand their disease and prognosis at different levels and need different things than patients of a similar age difference on the adult side. The four-year-old child who asks “What happens when I die?” may be asking for very different information and for different reasons than the 12 year old or 20 year old who asks the exact same question. It is the pediatric team’s job to determine what answers the child is really seeking, and let them continue to guide the conversation.
Similarly, the child’s ability to be meaningfully involved in determining the course of their care is greatly influenced by their developmental stage. But what does this mean legally and ethically in the pediatric population? The 13 year old may have much more ability to make informed decisions about what care they do and do not want than a 5 year old, but no more legal right.
These gray areas of autonomy exist in adult medicine as well, but they are further complicated in pediatrics. What should happen when the child wants one thing and the parents want another? In addition, many of our patients have two legal decision makers, their parents, who do not always agree on what they feel is best for their child. Not to mention there are a great number of subspecialists who typically remain very involved in the child’s care throughout the course of their disease.
Beyond these more philosophical concerns, there are other practical differences between adult and pediatric hospice. In pediatrics there are few long-term care facilities available, and this is typically not an option families seek out. Also, many pediatric patients and their families have spent so much time in their hospital that it is a second home to them. Often they feel more comfortable and safe there than their own home. Dying at home is not, therefore, always preferred by parents or children as it is for most of our adult patients. We are also more likely to utilize medical technologies such as feeding tubes, home ventilators, tracheostomies, ports and other equipment in our pediatric patients.
The use of such artificial “life-sustaining” technology may seem at odds with traditional hospice philosophy. And until recently, parents and guardians have faced deciding between pursuing curative-intent treatment for their child’s underlying disease, or receiving hospice services while foregoing further disease-modifying therapy.
In 2010, President Obama signed the “Patient Protection and Affordable Care Act,” which changed this. This act, which is also referred to as “Concurrent Care for Children,” requires all state Medicaid programs to pay for curative treatment of the underlying disease as well as hospice services for children ages 21 and younger who qualify. Although it doesn’t fix everything, this law reduces a significant barrier to pediatric hospice services and opens up this resource to many more families.
The family of my two year old with newly diagnosed metastatic liver cancer met with many specialists and learned about her treatment options, limited as they were. There was no possibility of a cure. In the end, after much soul searching, they took her home with hospice.
Pediatric hospice is about working aggressively for the best quality of life – however a family defines this for their child. It isn’t that families who choose hospice are “giving up”– it is that their goals are to do whatever it takes to give their children good, comfortable days. They pursue time to make family memories, to cuddle, to live, to laugh, and yes, to cry.
At some point, the hospitalizations and tests won’t change the likely outcome for their child, but they do take up the time and space that may be used to create other memories. Hospice is about living in the moment and enjoying today. Our team has helped to arrange all sorts of things for patients and their families to remember – a first-ever family vacation to the beach, a trip to Hawaii, even a trip out of state on continuous life-sustaining IV medications for a patient who wants to visit friends.
And in between all the big celebrations, we celebrate the little things. We celebrate birthdays, even if they aren’t at the one-year mark. We celebrate a walk outside in the sun, a baby taking a bottle, siblings all curled up on the same bed together. On a journey that can be so isolating, our team seeks first and foremost to be present with families and to walk with them. We may not have the power to cure, but for today we can be present.
Laura Waddle, M.D. completed her fellowship training in Hospice and Palliative Care at Emory University. Prior to her fellowship, she did a combined residency in internal medicine and pediatrics. She serves as the pediatric hospice medical director for the dedicated pediatric team at Visiting Nurse Health System. In addition, she works as a palliative care physician at the Atlanta VA caring for veterans and teaching medical students and residents about palliative care and hospice.
