Once in awhile, a story hits the news that is so heartwrenching and tragic that it seems rude and cold to ask questions – let alone challenge – elements of the story. Often, this is exactly the time that hard questions should be asked.
Right now, there is a life-and-death drama playing out at the Sick Children’s Hospital in Toronto. Kaylee Wallace, who is two months old, is receiving palliative care at the facility. On Tuesday, there was an attempt to harvest her organs under the “Donation after Cardiac Death” (DCD) or “Non-Heart Beating Donation” (NHBD) protocol. In this protocol, an individual’s organs are harvested soon after the heart has stopped beating. The organ harvesting is called off if too much time elapses between removal of a ventilator and stoppage of the heart.
On Tuesday, Kaylee continued to breathe after being taken off a ventilator. The problem was that she stayed awake – since her breathing only stops when she sleeps. (If you think you know a name for that condition, read on)
The following excerpts are from the story on Kaylee Wallace in the Globe and Mail, just one of many media outlets covering this story. A brief survey I did of various articles indicates this portrayal is fairly typical of the coverage.
The enduring life of Kaylee Wallace unleashed a torrent of emotion yesterday as her father vowed to continue his fight to donate the dying two-month-old’s heart.
A transplant attempt meant to save the life of a second girl failed last night after Kaylee, who has a rare condition that causes her organs to shut down in sleep, stayed awake when taken off life support.
A little later on, we get more information:
Mr. Wallace and the baby’s mother, Crystal Vitelli, took Kaylee home two weeks ago, believing their little girl was well enough to leave hospital. Shortly afterwards, they were back. Her rare condition, Joubert syndrome, has left her unable to breathe while asleep without a machine.
“She moves, she wakes up, she opens her eyes, but when she sleeps, she essentially dies,” Mr. Wallace said. “It’s hard watching her be a vibrant baby. When she goes to sleep, the machine does the rest.”
This information is a little more accurate. In fact, when the reporter writes that Kaylee’s condition that “causes her organs to shut down in sleep, in the second sentence of the article” it is nothing except the wildest of exaggerations. But it does help to build the image of an infant that is dying.
It’s her breathing that stops. And if you think you’ve heard a word for that, you’re right. It’s called apnea – and there’s not a single quote in a single story from anyone that uses the term. Apnea is a fairly common disorder, although found mostly in adults. Literally thousands of people with apnea in the U.S. and Canada use various devices to assist their breathing when sleeping.
Here’s where I ask one of my rude questions: Is the word apnea being avoided because people don’t associate it with “dying,” but recognize the term as covering a variety of treatable conditions?
And what about Joubert syndrome? Is it described as a terminal condition?
I didn’t know, so I checked. I checked Wikipedia, the National Institute of Neurological Disorders and Stroke (NINDS), the Joubert Syndrome Foundation & Related Cerebellar Disorders and the Cleveland Clinic. None of these sources describe Joubert syndrome as “terminal.”
Here’s a brief overview from NINDS:
Joubert syndrome is a rare brain malformation characterized by the absence or underdevelopment of the cerebellar vermis – an area of the brain that controls balance and coordination. The most common features of Joubert syndrome in infants include abnormally rapid breathing (hyperpnea), decreased muscle tone (hypotonia), jerky eye movements (oculomotor apraxia), mental retardation, and the inability to coordinate voluntary muscle movements (ataxia). Physical deformities may be present, such as extra fingers and toes (polydactyly), cleft lip or palate, and tongue abnormalities. Kidney and liver abnormalities can develop, and seizures may also occur.
Prognosis:
The prognosis for infants with Joubert syndrome depends on whether or not the cerebellar vermis is partially developed or entirely absent. Some children have a mild form of the disorder, with minimal motor disability and good mental development, while others may have severe motor disability and moderate mental retardation.
So what about that apnea? There’s a National Institutes of Health (NIH) document that shares some information about that:
Although some infants have died of apnea, episodic apnea generally improves with age and may completely disappear.
From where I sit, the hospital other professionals commenting on the story have a lot of explaining to do. Here’s my starting list of questions I want answered:
- How does this child’s disabilities differ from the majority of non-terminal children with the same condition?
- Was the possibility of treating the apnea ever seriously considered?
- Given the possiblity of improvement of apnea over time, why is ventilator assistance being withdrawn?
- Is this child really “dying” or is this a “quality of life” decision? Shouldn’t we try to be clear about which type of situation we’re talking about?
Meanwhile, there is another infant in the same hospital, named Lillian, who needs a heart. There are no guarantees that she’ll be the one to get Kaylee’s, if Kaylee’s death occurs in a way that makes harvesting possible.
But it makes it all the less likely there will be any serious questions asked about the seriously flawed coverage and public discussion of the drama playing out right now. –Stephen Drake