“My Nine-Year-Old Daughter Doesn’t Know She’s Dying”
When nine-year-old Emma Gray-Gonfalone talks about becoming a fashion designer one day, her mother doesn’t know what to say.
It’s not because Courtney Gray doesn’t support her young daughter’s ambition – she does. Rather, her reticence is because she knows something her daughter does not: that Emma probably won’t live long enough to achieve that dream.
Ten years ago, Gray was carefree – an American college student studying in Paris. In a fairytale twist, she met and married Frenchman Fabrice Gonfalone, and Gray quickly became pregnant.
Though newborn Emma was diagnosed with a heart murmur, she seemed otherwise healthy. But as Gray remembers, things began quickly deteriorating. “She wasn’t feeding properly, she wasn’t able to keep her temperature correct, and she had this mottled skin. A nurse finally started writing down everything, and she was like, ‘Something’s wrong’.”
A series of tests revealed unfavorable critical results, so doctors decided on an emergency transfer to another hospital in Paris.
As Gray tells it, one minute the new parents were happily watching their two-day-old baby, the next they were in the back of an ambulance, sirens blazing, speeding along the cobblestones of the Champs-Élysées, and watching Emma bounce around in an incubator. “It was a lovely experience,” Gray says with a wry smile.
The young family would spend the next 30 days in the NICU (Neonatal Intensive Care Unit).
While no parents want to see their baby unwell, the cultural differences made this time especially hard. “No one explained to us that we could spend the night. It was pretty clear that they did not want us there, so my daughter spent the first three nights by herself,” Gray recalls.
Eventually, Emma was diagnosed with Tetralogy of Fallot (TET), a rare but treatable congenital heart defect. Five and a half months later, she underwent surgery to repair it. But although the procedure was successful, it did not signal the positive outcome her parents were hoping for.
Months of persistent illness followed, until finally, in the week of Thanksgiving 2007, Emma was once again rushed to hospital. It was there that she was given her second diagnosis, the one that would come to define her life: pulmonary hypertension.
Known as ‘PH’, pulmonary hypertension is a condition where the pulmonary arteries (the blood vessels in the lungs) have higher blood pressure than normal, causing excessive strain on the heart.
“PH can occur in both adults and children, but for very different reasons,” says Dr. Venkataramana Vijay, a pediatric cardiothoracic surgeon at Mount Sinai Hospital Brooklyn Hospital in New York.
“For some children, the condition is completely reversible through minimal drug therapy,” he says. But those on the other end of the spectrum are not so lucky. “They may require a transplant.”
While many children with PH have normal life expectancy, it quickly became apparent that this would not be the case for Emma. (“I don’t think her doctors ever thought that she would make it to two,” adds Gray.)
A relentless series of consultations and treatments followed. But as the months turned into years, the family was not happy with the level of care Emma was receiving in Paris. Convinced that the U.S. held better options, the family – including Charlie, their newborn son – relocated to New York, and began a new phase in their daughter’s treatment.
Nine years on, and Emma has outlived those initial estimations.
Gray attributes her daughter’s endurance to her fighting spirit. “She’s tenacious. We have all said that if she wasn’t that way, she would not have survived this long.”
However, after a decade, two dozen medical procedures and hundreds of thousands of dollars in medical costs, Emma remains a medical mystery. Her doctors have admitted there will be no cure in her lifetime, and she is unlikely to live past her teenage years.
Despite this miserable prognosis, there is still much to be done each day to try to slow the relentless progression of her condition. Treatment includes about 19 doses of medication per day; some delivered orally, others by IV. She’s also never without her oxygen tank. “She’s tethered by her oxygen,” says Gray. “She’s basically on a leash all the time.”
This kind of intensive caregiving takes a toll on the whole family.
Friend Cristina Slattery has witnessed the incredible pressure both parents are under, and the different ways they cope. “Fabrice ran a marathon a few months ago, but in some ways, Courtney is going the opposite way in terms of self-care.”
Gray agrees that looking after her own health is hard. “Every year I have a campaign where I’m like, ‘Okay this is the year that I’m going to do something!’ and then Emma gets sick or whatever. I keep trying though.”
The situation is also difficult for eight-year-old Charlie. “On the one hand, he wants to be understanding and be a sweet brother and be helpful,” says Gray. “And on the other hand, he’s so resentful of the time and attention that she gets.”
Then there’s the other unavoidable toll when raising a terminally ill child: medical expenses. The early years in Paris were particularly hard – with all the co-pays and extra fees associated with Emma’s treatment, costs quickly spiraled.
In the U.S her medications are still expensive ($30,000 per month). The family has insurance through Fabrice’s job, and also receives Medicaid to cover additional costs.
Though others have little hope, the Gray-Gonfalone family is looking toward the future.
Emma is busy drawing, cooking, and making plans for her tenth birthday party. To her, there’s nothing unusual about her circumstances, and she can’t understand why others might be interested in her story. (When asked how it felt to have an article written about her, her reply was telling: “Ummm, I don’t know?”)
Like most kids her age, she also spends plenty of time dreaming about life as a ‘grown up’. “She’s heard the word ‘fatal’, but she doesn’t know what it means, and she’s never asked,” says Gray. “She really has no concept that she will not be getting married, she will not be graduating college and she will not be pursuing a career.”
Meanwhile, her parents are grappling with decisions about what’s next. The possibility of home schooling is being discussed, to ease Emma’s fatigue and improve her quality of life.
They’re also waiting to hear if she’s a candidate for a new type of transplant surgery. But even if it is an option, the decision is by no means straightforward.
“We could make the decision to do it, put her on the table, and lose her that day. Whereas we could’ve maybe had another six months, to a year, to a year-and-a-half. Or we put her on the table, and it seems to go well, then we lose her ten days later,” Gray’s voice trails off. “Or maybe everything is hunky-dory, and we have five more years. I mean, there are obviously no guarantees.”
Story Updated April 2023:
According to the Columbia University Irving Medical Center Website the family has since set up a fundraiser in Emma Gray-Gonfalone’s memory.
Over many years, our family has seen first-hand the incredible care and dedication that Dr. Erika Berman Rosenzweig, Director of the Pulmonary Hypertension Comprehensive Care Center at Columbia, provides to each and every one of her patients. For this reason, we have established the Emma Gray-Gonfalone Pulmonary Hypertension Award to propel her work to develop new diagnostic tools and treatments to transform outcomes for patients with this diagnosis.
We are so grateful for the support you have shown our family and for your generosity in considering a gift in Emma’s memory.
https://joinus.cuimc.columbia.edu/index.cfm?fuseaction=donorDrive.participant&participantID=1023
Interviews and sources:
Courtney Gray: Mother
Fabrice Gonfalone: Father
Emma Catherine Gray- Gonfalone: 10-year-old PH Patient
Cristina Slattery: Family friend and first lead to the story.
Tal Gross PHD: Associate professor at Columbia University. Public health and policy expert: Interviewed to offer expertise on European healthcare costs and reimbursements.
Dr. Venkataramana Vijay: Adult and pediatric cardiothoracic surgeon: Interviewed to offer expertise on pulmonary HTN and European reimbursements. He is not Emma’s specialist but is a board-certified MD and expert.