We need to talk about death, say parents of kids with life-limiting illnesses
By Dr. Brian Goldman
Julie Drury always knew her fragile daughter Kate would die young. Still, she wonders why her child's doctors never spoke about it.
"I think they don't bring it up because it's not what they're supposed to be doing," says Julie in the living room of her home in Ottawa. "They're supposed to be fixing our kids and helping our kids and prolonging their lives."
Photos and other reminders of Kate are everywhere in the Drury home. It is here that Kate died on Nov. 30, 2015. She was just eight-years-old.
Julie is a charter member of One More Thing, a group formed in the Ottawa area, made up of parents of children who have extraordinary medical needs.
Typically, their kids are on a regimen of many pills. They require feeding tubes and machines to deliver oxygen and other medicines. Some need nearly round-the-clock care just to live at home — if they can leave the hospital for any length of time.
Their Facebook group, One More Thing is a stoical nod to the health professionals who keep telling them there's one more thing they should be doing for their children — so many "one-more-things" they've lost count.
'Medically fragile' kids
I first met Julie and five other members of One More Thing back in 2015. As experienced parents of children who are "medically fragile," they are adept at pointing out the shortcomings of our health-care system.
- WHITE COAT, BLACK ART: The One More Thing mom's club
Last year, the Ontario government named Julie as the inaugural head of the province's new Patient and Family Advisory Council. She's been hearing from patients and families to try and make the system become more responsive to their needs.
On the first anniversary of her death, Julie posted a searing account on her blog, Searching For Solid Footing.
It was around this time that Julie reached out to me to speak about those frantic final days and hours at Montreal's Sainte-Justine Hospital where Kate's doctors tried desperately to extend her life — an attitude that Julie now believes made it difficult for her, her partner Brian and Kate's older brother Jack to say goodbye.
Kate was born with a disease called SIFD, sideroblastic anemia with B-cell immune deficiency, periodic fevers and developmental delay. SIFD is so rare that Kate was the first person on the planet to be diagnosed.
Rare form of mitochondrial disease
SIFD is a mitochondrial disease. Mitochondria are found in most cells inside the body and essential for converting food calories into energy that powers everything from our hearts to our brains.
You fear showing emotion ... So I put a smile on, though I felt confused and scared and had an odd sense of dread, mixed with what I can only describe as hope.- Julie Drury
Like other children with SIFD, Kate was born deaf. She had cochlear implants.
SIFD also causes developmental delay, degeneration of the nervous system, seizures, plus damage to the major organs.
Kids affected need frequent blood transfusions and other treatments. Kate spent much of her eight short years in and out of Children's Hospital of Eastern Ontario (CHEO).
Only half the kids with SIFD make it past the age of four.
But there was one faint hope: a case report of a nine-month-old baby apparently cured of mitochondrial disease after receiving a bone-marrow transplant from a healthy matched donor.
Kate's brother Jack was a positive match.
In March 2015, Kate had the transplant at CHU Sainte-Justine in Montreal.
'I felt confused and scared'
Julie remembers that day with mixed emotions.
"The bone marrow transplant team had clearly done this time and again over the years," wrote Julie in her blog.
"The mood they set in the room was one of celebration and excitement. I tried to play along as we typically do as parents. You want to engage with your team and show you understand what is happening."
"You fear showing emotion, or the wrong emotion. So I put a smile on, though I felt confused and scared and had an odd sense of dread, mixed with what I can only describe as hope."
Kate developed graft versus host disease (GVHD) – a feared complication in which the transplanted bone marrow started attacking Kate's weakened body.
Throughout the summer of 2015, doctors at CHEO and CHU Sainte-Justine kept close watch on Kate, and tried various medications to keep the GVHD in check.
Julie says the pivot from trying to save Kate to watching her begin to die came suddenly and unexpectedly during a weekly checkup in Ottawa.
"I knew she wasn't doing very well," Julie recalls. "She was very fatigued. She was having difficulty walking. She was really struggling with her GVHD."
Julie and Brian took Kate to CHU Sainte-Justine, where she was admitted for tests.
"I think I knew on a really raw, intuitive, mommy, 'Spidey-sense' level that something was happening," Julie recalled.
She doesn't know if the doctors and nurses realized that Kate only had a short number of days to live. If they did, Julie says, they didn't discuss it with Brian or her, despite knowing the family well.
Julie believes that on some level, Kate knew she was dying.
"I think she knew too because she kept asking for home," says Julie.
'Kate's van': DNR (do not resuscitate) sign
Julie left Kate in the care of her partner Brian plus the doctors and nurses in Montreal, and hurried to Ottawa to make arrangements to bring Kate back. It meant organizing a lot of home care on very short notice and on a weekend.
"I've said this time and again. Nothing medical happens on a weekend," says Julie. "There is care that happens [but] it's the basic care for emergent situations. But if you're trying to get your child home with the support of a medical team, they're not around on a Saturday morning."
If she died en route, and we had to call an ambulance or a police, we wanted it to be clear that she should not be resuscitated.- Julie Drury
Before she and Brian could bring Kate home, Julie scrambled to arrange everything her daughter would need: oxygen, intravenous fluids and nutrition, a suction device to clear Kate's secretions plus intravenous morphine for pain.
Then she went back to Montreal to pick up Kate in the minivan the family dubbed "Kate's van." Julie says they put a DNR (do not resuscitate) sign in the dashboard of the vehicle.
With the help of Kate's long-standing palliative care doctor, friends, and many of the members of One More Thing, Julie and Brian managed to bring Kate home.
Julie recalls the doctor recommended treatment for Kate's congestion, more morphine to ease her breathing and stopped the intravenous fluids that had kept Kate from being dehydrated.
"He was delicate in his approach," she recalls.
Looking back, Julie thinks the palliative care doctor was hinting to her that Kate was dying.
"He's a wise person, and he loved our daughter too," says Julie. "I can't imagine the grief he was feeling. He'd known this little girl for six years."
'She was so intensely loved'
The day before she died, Kate passed into a coma from which she never regained consciousness.
"I came to the realization that night," Julie says. "Kate hadn't woken up. She was changing physically. Her morphine needs went up. My incredible son [Jack] was monitoring Kate's vitals constantly."
"I went to bed with her that night and cuddled her," Julie recalled. "I had this moment of talking to her and feeling incredible pain. And I thought, 'This is really happening.'"
Kate died on Nov. 30, 2015. Julie, Brian and their son Jack were at her side.
Preparing for a child's death
Now, two-and-a-half years following Kate's death, Julie is speaking out about what she thinks is an unmet need among parents of fragile kids like Kate: to help prepare them for the death of their child.
Death doulas exist. But I didn't know they exist. This [should be] part of the conversation.- Julie Drury
Some have suggested that parents and other loved ones could benefit from having the advice of a death doula or death midwife. A death doula is a person who assists in the dying process, much like a midwife or doula does with the birthing process.
"I was prepared for the birth of my children," says Julie. "I knew what was going to happen physically. I knew what my options were. I knew the nitty-gritty details."
She says doctors only advised her to follow Kate's lead and to keep her comfortable.
"Death doulas exist," says Julie. "But I didn't know they exist. This [should be] part of the conversation."
Julie's good friend and fellow member of One More Thing Kim McLeod agrees. Kim was present to support Julie as she talked about Kate's final illness.
"Stop creating an elephant in the room," says Kim. "For parents of kids with life-limiting illnesses, we get it. It's limited, and so we're going to enjoy our life as much as we can while we have it."
"It's a very hard conversation to ask about because it's so emotionally charged from our side," says Kim. "But we also want to know what death looks like."
As an ER physician, I have been on the medical side of some of those very difficult conversations. There are times when I must confess that I have not been as prepared as I should have been to speak plainly to patients and loved ones, and to just give them the space they need to feel what they feel in those heartbreaking moments.
As parents of fragile children, Julie, Kim and the other women who belong to One More Thing are good at identifying health care's shortcomings.
What I learned on this occasion is that health professionals like me underestimate the strength of parents like Julie and Kim.