Like many 17-year-olds, Grace Fordyce dreams of travelling the world and attending university. One day, she’d like to be a nurse. But unlike most of her peers, just nine months ago, she wasn’t sure she’d be alive to achieve any of these things.
Living with Ehlers-Danlos syndrome (EDS) and rare abdominal vascular compressions syndrome (AVCS), last year Grace’s health deteriorated to the point she was unable to hold down any food or water and spent most of her life writhing in pain, taking copious amounts of opioids that did little to ease her misery.
AVCS is a group of conditions where the arteries and veins in the abdomen are compressed, restricting blood flow, and causing weakness, fatigue, weight loss, extreme pain and nausea, especially when eating or drinking.
EDS is an inherited condition affecting connective tissue, like joints, blood vessels and skin.
With her parents Keri and Dave by her side, Grace tells the Weekly she was told the only surgical option to improve her quality of life was in Germany and came with a $180,000 price tag.
The Auckland parents were worried it was “too risky and experimental”.
Instead, Keri relentlessly researched and trawled online support groups and, to her immense relief, discovered there were multiple other surgeons available around the world, some much more affordable and closer to home.
In November last year, Grace travelled to Canberra, Australia, for an operation to release the compressed veins and arteries, and remove nerves around the celiac artery.
All up, including pre-op tests and the private hospital stay, it cost around $35,000.
But what happened next was truly incredible. After years of living in agony, unable to eat without vomiting, Grace woke up pain-free, asking for sushi and ice cream.
“On day one, I was walking with a walker, on day five, I was eating fries and a hamburger, on day six, I went shopping with Mum and three weeks later, I went to the gym,” marvels the tenacious teen.
Perhaps most impressively, Grace went from being almost entirely bed-bound to completing a 71km hike in Queen Charlotte Sound just eight weeks following the operation.
Grace, Keri and Dave explain that they want to speak out about their experiences so that others struggling with diagnosis and treatment know their symptoms are very real – and that help is available.
“I feel incredible,” says Grace. “I have no pain, no nausea, no migraines, nothing. I’m a new person and I want other people to feel this good too. There are options out there, and medical professionals that will take you seriously and believe you.”
It marks a remarkable turnaround from a child who has always struggled with health problems, but battled even more to have them recognised.
Grace has lost count of the number of times she was told it was all in her head, that she was just experiencing panic attacks or that her weight loss and inability to hold down food meant she had an eating disorder.
She remembers being confused when, last year, she lost nine kilos within two weeks, despite being hungry all the time and eating hourly. She was also desperately struggling to breathe, with chest pain and spasms.
Grace was terrified by what was happening to her body and devastated when medical professionals didn’t take her seriously.
“I was a teenage girl with chest pain and trouble breathing, so it was labelled as a panic attack and I was vomiting, so it was thought I had an eating disorder,” she tells.
On one occasion, when she was hospitalised, the heart monitor readings were so extreme that doctors said it must be a technical malfunction. “I felt betrayed,” says Grace.
“I went to the hospital thinking they were supposed to help me. I was hooked up to all these machines and my heart kept setting them off with an extremely high heart rate, and my oxygen bottoming out like I was in critical condition. It happened three times in an hour and when they finally came in, they said the machine was just broken.”
Keri continues, “Grace had two oximeters [small devices placed on your finger to measure blood oxygen levels] on from home as well and took photos of all three machines saying the same thing. But they didn’t believe it was possible to be conscious with her oxygen so low.”
Watching their beloved daughter in so much pain, unable to attend school, hang out with friends or be a teenager, Keri and Dave were distraught.
“When you’re sitting with your daughter, who’s in excruciating pain, unable to eat or drink, chewing 200mg of Tramadol a day, what do you do?” questions Dave.
However, the close-knit family, including brother Will, 15, have always supported each other, no matter what.
Grace has had an extensive history of health complications since she was a young child, including a heart murmur, and a hole in her heart that was diagnosed at seven and repaired surgically when she was 10.
“Despite health issues, I was a happy and incredibly active kid,” recalls Grace.
“I played nearly every sport, even if I was the only girl on the team.”
At 13, things took a turn for the worse after Grace got her period for the first time, and started experiencing extreme pain, nausea and vomiting for three out of four weeks every month.
Then, when she was 16, after endometriosis surgery, a gynaecologist specialist asked if they’d ever considered that Grace might have EDS. At the time, Keri and Dave had already been on a waitlist to see an EDS specialist for Will for 18 months.
Keri explains that the wait time for an appointment was around four years, but after begging and pleading Grace’s case, she was given an emergency appointment and was diagnosed with EDS.
For the exhausted family, it was the first time they felt like someone really understood and believed them. From here, they were referred to gastroenterologist Dr Judy Huang, who, through extensive testing and a process of elimination, diagnosed Grace with AVCS.
There are four different kinds of compressions and Grace had three.
“I swear she actually saved us,” shares Keri gratefully. AVCS is considered incredibly rare in New Zealand. However, the Fordyce family says they believe many people are living without a diagnosis as it is a complicated and long process, with few specialists available.
“I honestly believe if we didn’t have private health insurance and Judy, I don’t know if I would be here now, if I would have survived,” shares Grace. It is possible for AVCS patients to slowly starve to death as their organs don’t get the vital nutrients needed to function.
Next came a vascular specialist, who Grace says advised her in July to book in for the expensive surgery in Germany.
Grace was desperate for a cure, but her parents strongly felt that Germany wasn’t the answer.
Surviving solely on dietary supplement shakes − which she would without fail vomit back up − and IV fluids, she pulled out of school. Her mental health suffered greatly and, she admits, at times she lost all hope.
Keri remembers, “She’d be rolling on the ground delirious with pain, not even able to keep water down. It was a vicious cycle as the more weight she lost, the worse the compressions got.”
Grace adds, “Then came all the research. I don’t think Mum slept at all.”
Keri says despite being told Germany was the only option, she discovered that AVCS surgeries were being carried out in Spain, the United Kingdom, Canada, America and Australia. She also learned that the surgery has been performed twice before in New Zealand.
They tried to have Grace’s case assessed by a NZ multi-disciplinary board, but Keri says they were told “the consensus of opinion within the meeting was poor”, and nothing more came of it.
Keri contacted around 30 surgeons globally. Some never replied and the high cost ruled out others, but in Australia’s Dr Gert Frahm-Jensen, they found a lifeline.
“The staff at the hospital and my surgical team were just amazing, so knowledgeable, so kind and caring, and they listened,” says Grace. “They took my voice into account and apart from Judy, these were some of the only people that took me seriously and I felt cared about.”
Reflecting on the November operation, she adds, “This has been life changing.”
Pain-free for the first time in years, Grace is excited about the future. She’s completing her final year of high school and planning a trip to Europe to visit her boyfriend in Italy, and then she wants to give back.
“I have always had pretty strong dreams, and wanted to be a nurse because nurses have always been the ones who made a difference to me in hospital and showed they cared,” explains Grace. “I want to do that for others.”
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