When 5-year-old Julianna Snow began having trouble breathing, her parents didn't rush her to the hospital.
Instead, they made her comfortable in her princess-themed bedroom and held her until she slipped away.
A promise kept.
It wasn't an unexpected death.
Julianna had Charcot-Marie-Tooth disease (CMT), an incurable neuromuscular disorder. The young girl had expressed her wish to die at home. Her parents and doctors gave her the care she needed while supporting her choice.
"She went after 18 beautiful months [in hospice]. She didn't go after a year of horrible hospitalizations," her mother told CNN.
At Arnold Palmer Hospital for Children in Orlando, Florida, Dr. Blaine B. Pitts, F.A.A.P., often encounters families with children who might die from their diseases.
"Vital conversations need to occur," he told Healthline. "We walk alongside the child and family, constantly reassessing goals of care and hope, to lift up the child's voice throughout their journey."
Pitts, medical director of Pediatric Palliative Care at the hospital, believes that if you miss out on these conversations, you risk ignoring the child's voice in what truly matters to them.
How much weight should a child's wishes carry?
According to Pitts, chronological age and level of maturity play a role, but there's another factor that is perhaps more critical. It's duration and severity of illness.
"A child who has had cancer for five years would know the burdens and benefits of pursuing additional therapies much more easily than a child who has been receiving disease-modifying therapies for only two weeks," said Pitts.
"There are other contextual and cultural factors that impact decision-making as well," he continued. "If parents don't agree with the child, we would recommend a family meeting, or multiple family meetings, to discuss goals of care." The family meeting would include asking questions such as:
Why is the child choosing one pathway and the parents are choosing another?
Are there aspects of unaddressed suffering (refractory pain) that are leading the child to choose their pathway?
Are there fears about end of life that are leading parents to choose their pathway?
"A vast majority of the time, the child is aware of their approaching end of life, which can lead to the child wanting to protect their family," Pitts said. "Without adequate palliative care services, the child may have unaddressed fears which may lead to increased suffering. Though the children do not have autonomy, we often give their voice more weight as the child approaches end of life. This is our only chance to get it right."
Pitts added that palliative care improves a child's quality of life for their remaining lifespan. The child may forego another round of chemotherapy in hope of gaining a better quality of life for the remaining days or weeks.
"At times, less is better," he said.
While Pitts said he supports the decision to stop treatment when it's in the child's best interest, refusing treatment for a curable disease is a completely different matter.
"We would advise against their decision and encourage them to reconsider medical therapy," he said. "If the child is a ward of the state, the legal system would need to give direction on end-of-life decisions."
The hospital ethics committee can also provide additional support and guidance through difficult cases.
Support for the family
When parents are at odds in end-of-life wishes, Pitts brings in extended members of the palliative care team.
That would include chaplains and social workers to optimize communication and to formulate a plan of care to meet everyone's wishes.
Signing a do not resuscitate order (DNR) for a child is a heart-wrenching decision. Pitts said some parents say they feel as if they're signing their child's death certificate.
It's more than some parents can bear.
"If a child chooses to go home, we would strongly recommend additional support in the home from a local hospice agency," said Pitts.
"We work closely alongside local hospices to provide additional support in the home," he added. "That includes anything from medications, equipment, spiritual care, or psychosocial bereavement support. The pediatric palliative care team may even perform home visits to meet child and family needs in a setting that meets the child's goals."
The role of hospice care
Dr. Stephen L. Brown, a board certified cancer specialist at Austin Cancer Centers, has transitioned many children and teens into hospice care.
In an interview with Healthline, Brown explained that hospice care has changed a lot in the past decade.
"Ten years ago, most people correlated hospice care to mean a 2- to 4-week life expectancy," said Brown. "Hospice has evolved into support that can be employed as much as six months to a year out."
Hospice care usually involves visits to the family home one or more times per week. Brown said this allows the family time to establish relationships with caretakers and nurses.
"Hospice is a support network. It helps get the family from active treatment to palliative care. I use hospice as the vehicle to start the dialogue about end of life," he added.
End of life is a difficult discussion at any age.
When broaching the topic, the question is, "Are children capable of processing it?"
According to Brown, the youngest patients may not be able to grasp it. In those cases, communication about prognosis usually goes through the parents.
"To me, it's a difficult conversation, but it's part of the job. You have to know how to approach and support people in a positive way," he said.
Parents can be understandably stressed and reluctant to accept that their child will not survive.
When that happens, Brown said the conversation must be redirected.
"We continue dialoguing. We want to make sure the parents are listening and are attuned to their child's wishes," he said. "Through the early induction of hospice, we're able to set parents' expectations more realistically in terms of life expectancy. Hospice gives us the window to communicate and address the issues."
He continued, "We may not have the ability to cure an individual. The question is, how long will they endure and suffer before you say no further curative treatment?"
In dealing with children and adolescents, communicating clearly and in down-to-earth terms is key.
"I just try to do the best job I can," said Brown.
Troy Slaten, a legal expert with Floyd, Skeren & Kelly, told Healthline that when physicians, parents, and a terminally ill child agree with the decision to stop treatment, there's no reason not to go along with it.
In an interview with Healthline, Slaten explained that it's best if more than one doctor offers an opinion. If everyone is in agreement and everything is well documented, he said there should be no problem for the doctors or hospital.
But there can be legal complications.
That would be the case if another family member, such as an aunt, uncle, or grandparent, disagrees with the decision.
"Anyone who doesn't believe the decision is in the best interest of the child could petition the court," said Slaten. "The court would then have to decide if a conservator should be appointed."
What happens when parents and child disagree?
It can put the doctors and the hospital in a difficult position.
When disagreements can't be resolved or when the hospital does not believe parents are acting in the best interest of the child, they have little choice but to turn to the courts for guidance.
"Basically, anyone under age 18 doesn't, on their own, have the ability to make those decisions," said Slaten. "Just like they can't enter into a contract, they can't decline medical treatment on their own."
A problem would occur in a case when parents want to take heroic measures, but the child doesn't want that.
For example, continuing a treatment such as chemotherapy even when there's an extremely low likelihood of success. Or if the child wants an advance directive or a DNR order and the parents refuse.
"In all states, the child can petition the court to appoint a guardian or conservator for the purpose of making medical decisions," said Slaten.
It would be up to the court to decide if a parent or an independent person is in a better position
to make medical or lifesaving decisions.
"It all comes down to what's in the best interest of the child," said Slaten.
By Ann Pietrangelo