Young and facing death, but having a say

Karly Koch, who has an immune disorder, goes over her end-of-life plans with her mother, Tammy, in Muncie, Ind.

Tumors had disfigured AshLeigh McHale’s features and spread to her organs. A year ago, AshLeigh, 17, flew from her home in Catoosa, Oklahoma, to the National Institutes of Health in Bethesda, Maryland, with a thread-thin hope of slowing her melanoma.

One morning, a social worker stopped by her hospital room. They began a conversation that would be inconceivable to most teenagers: If death approached and AshLeigh could no longer speak, what would she want those who surrounded her to know?

The social worker showed AshLeigh a new planning guide designed to help critically ill young patients express their preferences for their final days, and afterward.

If visitors arrived when AshLeigh was asleep, did she want to be woken? If they started crying, should they step outside or talk about their feelings with her?

What about life support? Funeral details? Who should inherit her computer? Or Bandit, her dachshund?

AshLeigh grabbed her blue and hot-pink pens, and began scribbling furiously.

When she died in July, she was at home as she had requested. Per her instructions, she was laid out for the funeral in her favorite jeans, cowgirl boots and the white shirt she had gotten for Christmas. Afterward, the family dined, as AshLeigh had directed, on steak fajitas and corn on the cob.

“I don’t know what I would have done if I’d had to make these decisions during our extreme grief,” said her mother, Ronda McHale. “But she did it all for me. Even though she got to where she couldn’t speak, AshLeigh had her say.”

A national push to have end-of-life discussions before a patient is too sick to participate has focused largely on older adults. When patients are younger than 18 and do not have legal decision-making authority, doctors have traditionally asked anguished parents to make advanced-care choices on their behalf.

More recently, providers have begun approaching teenagers and young adults directly, giving them a voice in these difficult decisions, though parents retain legal authority for underage patients.

“If you are one of the children for whom this matters, or one of their parents, this is a huge opportunity,” Dr. Chris Feudtner, a pediatric palliative care physician and ethicist at the Children’s Hospital of Philadelphia, said of these conversations.

But shifting from hushed talks with parents to conversations that include young patients has met some resistance. Many doctors lack training about how to raise these topics with teenagers. Until recently, most clinical teams believed that adolescents would not understand the implications of end-of-life planning and that they might be psychologically harmed by such talk.

Sometimes when providers do make the attempt, parents or patients may abruptly change the subject, fearful that by joining in, they are signaling that they have abandoned hope.

Yet research shows that avoiding these talks exacerbates the teenage patient’s fear and sense of isolation. In a 2012 survey of adolescent patients with HIV, 56 percent said that not being able to discuss their end-of-life preferences was “a fate worse than death.” In a 2013 study, adolescents and parents described such directed family talks as emotionally healing.

Karly Koch, a college student from Muncie, Ind., has been treated for many serious illnesses, including Stage 4 lymphoma, all related to a rare genetic immune disorder. Her older sister, Kelsey, died of the condition at 22.

Last spring, Karly, then 19, developed congestive heart failure. Her renal arteries were 90 percent blocked. As Karly lay in intensive care at the National Institutes of Health, a psychotherapist who had worked with the family for years approached her mother, Tammy, with the new planning guide.

“Do we talk about dying?” Koch recalled wondering. “Maybe Karly hasn’t thought about it. Do we put it in her head?”

“We had already buried a child and had to guess what she wanted,” she continued. “So we wanted Karly to have a voice.”

Karly’s reaction? “She said it wasn’t like we were telling her something she didn’t already know,” Koch said.

The guide used by Karly Koch and AshLeigh McHale is called “Voicing My Choices.” While there are end-of-life workbooks for young children and their parents, as well as planning guides for older adults, this is the first guide created for, and largely by, adolescent and young adult patients.

The intention was to create a way for them “to make choices about what nurtures, protects and affirms their remaining life and how they wish to be remembered,” said Lori Wiener, a social worker and principal investigator on the research that led to the planning guide.

In the two years since its introduction, more than 20,000 copies have been ordered by families and more than 70 medical centers from Aging With Dignity, the nonprofit that publishes it. “Voicing My Choices” has also been translated into Spanish, Italian, French and Slovak.

In straightforward language, the guide offers young patients check boxes for medical decisions like pain management. Another section asks about comfort. Favorite foods? Music? When visitors arrive, one option could be: “Please dress me, comb my hair and do whatever else is needed to help make me look like myself.”

What gives you strength or joy, the guide asks. What do you wish to be forgiven for? And who do you wish to forgive?

“These are the things that are important to know about me,” one list begins. AshLeigh, who would dance and sing down the aisles of Wal-Mart, wrote: “Fun-loving, courageous, smart, pretty wild and crazy.”

Devastating disease can leave anyone feeling powerless, so a means to assert some control can be therapeutic. For adolescents, who are exploring and defining identity, Feudtner said, “you can express who you are, what you are and what you care about.”

By offering young patients opportunities to write farewell letters, donate their bodies to research and create rituals for remembering them, the planning guide allays one of their greatest fears: that they are too young to leave a meaningful legacy.

And so the ability to do it can galvanize them. Lauren Weller Sidorowicz received a diagnosis of metastatic bone cancer at 18. Determined and outspoken, she joined a focus group of young patients at the NIH whose opinions led to the creation of the planning guide. Days before she died in 2011 at 26, Sidorowicz paged Wiener, frantic to include a final thought in a farewell letter.

To her grandmother, she wrote, “I hope there is potato salad in Heaven as good as yours.”

There are no standards for when and how to introduce a critically ill teenager to end-of-life planning; there are only intuition and experience. Many pediatric cancers have favorable prognoses, Feudtner said, and raising the topic prematurely may provoke anxiety and fear.

On July 25, Karly Koch had an experimental bone marrow transplant. Her family calls that date her “rebirthday.” With 12 medications a day and a surgical mask, she is out and about in Muncie.

Karly takes classes to become a physical therapy assistant. She is a youth leader at her church, where her boyfriend is also a member. She delights in “normal people” activities.

Her parents keep Karly’s copy of “Voicing My Choices” in their bedroom cabinet. “It isn’t gloomy to go through,” Karly said. “It’s kind of fun to get your feelings out there.”

“Now, looking at it,” she continued, “I think I’d like to add some things.”