Expecting the unexpected has become part of everyday life for Auckland mum Dani Noyer. Her three-year-old son Iziyah has a rare blood condition called chronic granulomatous disorder (CGD).
“With CGD some of the white blood cells called neutrophils don’t work properly, so Iziyah struggles to fight off infections,” says Dani.
Ever since he was two months old he’s taken a long time to get over bugs, even with the help of antibiotics.
In January 2013 Iziyah had high fevers after a month of dealing with a tummy bug.
After repeated visits to their GP the doctor ordered an x-ray. It showed Iziyah had pneumonia and an abscess on his right lung which prompted immunologists to test for CGD.
It was an anxious two-day wait for Dani and Iziyah’s father Sia, before they were given the CGD result. Despite doctors’ suggestions to avoid looking for information on the internet, they spentthe time finding out everything they could about the condition, which Dani says helped prepare her for the bad news.
With Iziyah’s immune system unable to fight off the pneumonia even with the help of antibiotics, specialists decided he would need surgery to remove the lower right lobe of his lung.
“We were in hospital for a month before he started to improve. Sia was working full time to support us, and my mum helped a lot,” says Dani.
Dani and Sia were told Iziyah would need an allogeneic stem cell transplant to treat his CGD using matched stem cells from a donor in Germany.
“Iziyah’s eight month old sister Kaylee was too young to be his stem cell donor and because Iziyah has mixed Samoan, Dutch and New Zealand heritage finding a match in New Zealand didn’t prove possible,”says Dani.
“Our minds were exploding with information and the pros and cons of Iziyah having the transplant. However, we were told he wouldn’t survive the next infection, so there was only one option,” she adds.
In July 2013 Iziyah began chemotherapy to prepare for his transplant.
It was really hard for people to understand that Iziyah didn’t have cancer, especially when he started having chemotherapy.
She says the children’s journal she received from Leukaemia & Blood Cancer New Zealand helped her keep a record of Iziyah’s progress.
“It’s so easy to forget when there is so much coming at you. It was amazing to have a daily record in the journal during the transplant process to record everything we needed to know.”
At the back of the journal is a special sticker page which Dani used to record all of Iziyah’s treatment, hospital stays and procedures.
“He’s not a child with cancer, so he doesn’t get any beads. That’s why the stickers were so special for him. They mark everything he’s been through,” says Dani.
Dani, Iziyah and Kaylee spent two months in isolation together, while Iziyah went through his transplant. During this time Dani turned to other parents on the ward who were in a similar situation with their children.
It was easier to chat to other parents who knew exactly what you were talking about; it really helped get me through.
In September the family headed back home, which Dani says was daunting.
“It was scary going from 24-hour-care to me being the only person responsible for Iziyah. It was a big adjustment having to be a nurse, mum and housewife. It was a huge shock to the system.”
Along with CGD, Iziyah has another genetic condition called retinal dystrophy, which is causing his eyesight to deteriorate over time. He was first diagnosed when he was eight-months-old. Post-transplant Iziyah’s eyesight got worse leaving him blind in his left eye and with tunnel vision in his right eye.
“No one had seen this before. They thought after transplant his eye condition would stabilise, but it hasn’t,” says Dani.
“Doctors think that the retinal dystrophy might be linked to his CGD because it’s rare to have two genetic conditions,” she says.
“They’re not sure, so our ophthalmologist will be presenting Iziyah’s case at an Australasian conference later this year.”
By April 2014, the family had adjusted to a new normal, and Iziyah was having monthly appointments. Suddenly things began to go downhill.
“His platelet levels dropped from a normal range of about 200 to 30. They transfused platelets immediately, but then his levels dropped even further, like his body was eating his platelets.”
Iziyah was admitted to the paediatric intensive care unit at Starship Hospital with his platelet levels down to 10.
“They took a biopsy from his skin and gut and confirmed chronic graft versus host disease (GVHD) along with autoimmune thrombocytopenia, an autoimmune disease where his platelets were being attacked by his immune system.”
“He was reacting to every platelet infusion they gave him. He had an anaphylactic reaction where his heart rate was high and he was wheezing. They gave him adrenalin three days in a row,” says Dani.
Doctors also found that Iziyah’s haemoglobin levels were dropping, so they transfused red blood cells.
“He was screaming in pain and needed to be sedated, or he risked bursting a blood vessel in his brain. We tried whatever we could to calm him down, but he was so angry because of the steroids,” Dani adds.
Over the next four weeks he was given medication to help treat the GVHD and his platelet levels also improved.
By May the family was back home. To help give Iziyah a boost they decided to attend LBC’s Firefighter Sky Tower Stair Challenge event. They had previously met firefighters who planned on walking 300km from Waitangi to Auckland to support LBC.
To motivate them Iziyah decorated a helmet and the family created a badge for them to wear.
It was inspirational to know we had done something to help keep them motivated.
These days the family of four tries to take each day as it comes.
“The hardest part is the random little things that pop up unexpectedly, but it’s important to just accept it and go with it day by day. It’s also important to get as much support as possible, even if it feels like you don’t need it at the time, you will. It’s good to keep those support networks open,” says Dani.