The year that Brooke had. (The hidden costs of BMT)
By the age of seventeen, Brooke Hides was slipping out of school at lunch time.
Most teenagers spend their time going to school, doing homework, maybe going to a part time job and playing sport. If they wag school, it’s because the surf was good that day and sneaking in a wave or two seemed worth the risk of getting caught.
Not so for Brooke. When she was seventeen, she was leaving school at lunch time to walk down to the local pathology centre for blood tests before heading back to class for the rest of the afternoon.
Read Brooke’s story below:
Hi Jasmine. Thanks for taking the time to talk to me today about Brooke’s bone marrow transplant. I believe Brooke had Chronic Granulomatous Disease. What was it that first made you twig that something wasn’t right with Brooke? How did the disease present for her?
When Brooke was about three, she had an abscess on her elbow that just wouldn’t heal. We were back and forth to the GP, trying multiple ointments and antibiotics, but it just wouldn’t get better. Eventually she had to have it surgically removed, but the incision became badly infected, and it wouldn’t heal either. It did eventually get better, and nothing more was done. We just moved on. Looking back on it now, that was probably the first sign that something wasn’t right.
Then at about 6 years of age she developed a cough that wouldn’t stop. She was coughing and vomiting and went downhill fast. She was admitted to the John Hunter Children’s Hospital for treatment, and again nothing seemed to be working, and she got sicker and sicker, ending up with a bacterial infection that spread to her lungs, brain, liver, and spine. The bacteria looked something like TB. But where does a six-year-old girl in Newcastle who’s never travelled anywhere pick up TB? We were in hospital for about 3 months that time.
It was there at the John Hunter Children’s that a paediatric immunologist, Dr Rani Bhatia, suspected that there might be something more going on, and she did a neutrophil test, discovered that Brooke’s immune system wasn’t working properly, and the diagnosis of Chronic Granulomatous Disease (CGD) followed soon after that.
You’re a nurse, aren’t you Jasmine? Can you tell me a bit more about neutrophils?
Neutrophils are part of your immune system that protect you from infection. They are a type of white blood cell that engulfs and destroys pathogens, specifically bacteria and fungi. Neutrophils are a type of granulocyte: a cell that has granules of enzymes. It’s the enzymes in these cells that destroy the pathogens. In CGD, the neutrophils are able to find and engulf pathogens, but they’re faulty and are unable to do the next step of destroying or ‘consuming’ the pathogen and keeping you healthy. Instead, they engulf the pathogen, but they just die there, and then more come along and they also try and engulf and destroy, and then another and another, and ultimately this cluster of cells becomes a granuloma.
Sometimes these granulomas are visible as lumps on or under the skin like her first abscess, but often they’re internal and develop without anyone knowing they’re there until they cause a problem. They can develop in all sorts of vital organs like liver, lungs, brain, or in the digestive or reproductive systems. Brooke mainly suffered from these granulomas in her lungs, and her skin. Some were surgically removed and others were treated with high dose antibiotics, and some just never went away.
You say that as if it was just life as usual, but I’m guessing that’s not quite the case.
No, it’s not. Living with CGD isn’t “a normal life” by any stretch. Everyday Brooke risked exposure to bacteria and fungi, because we are surrounded by these pathogens. She took antibacterial and antifungal medications every single day to try and fight off an infection before it started. And each infection could be life-threatening. That’s probably the most obvious concern we had for Brooke and the one that directly impacted on her daily life. The hidden side of this disorder is the long-term effects of these infections. It’s like her body was chronically inflamed, and over time that can lead to all sorts of health issues.
Every time your body tries to fight an illness, it triggers all sorts of hormonal and chemical responses in your body. Under normal circumstances, your body fights the pathogen, and the high arousal of your immune system and other body systems settles down, and your body returns to a state of normal function. For people with CGD, there’s never that ‘settles down’ bit, leaving your body in a chronic state of ‘high alert’ all the time. Eventually that takes its toll.
So, Brooke’s specialists were recommending a bone marrow transplant?
Yep. A BMT is the only cure for CGD. We started thinking about that when Brooke was thirteen. Her response at the time was “no way, Mum!” She was getting on with her early teens and didn’t want to disrupt the friends, sports and life she had. We’ve always taken the approach that whatever course of action we took, Brooke had to be part of that decision, so we left things as they were. But by the time she was sixteen, she was beginning to look a little further into the future and realised the impact this disorder would have on her later life, and she decided to move forward with a bone marrow transplant.
How is that for you as not just a mum, but a nurse-mum, knowing what is involved in a bone marrow transplant?
A bone marrow transplant isn’t something you take on lightly! I’d never been involved with a bone marrow transplant in any way, not in my personal life nor in my nursing career. So of course, I knew it would be tough, but even with all my nursing education and everything I’ve seen as a nurse over the years, I still didn’t really understand just how tough until I had to watch my daughter go through it.
How do you do that? How do you line your child up for something that you know is going to be so hard.
Well, you just have to put your faith in the team and hope that it all works, I guess. It is tough, though, watching your child have treatment that you know is going to make them so sick as they go through it. But I think being a nurse prepared me for a lot of it. It enabled me to detach myself a bit when I had to, so I was able to be of some help to the nurses who were looking after her. I knew what things were just a minor symptom, I guess, and what was a change in Brooke that really did need to be addressed quickly.
Brooke was sixteen when she had her transplant?
She turned seventeen just before she had her transplant.
Seventeen? So, she must have been in year 11 or 12?
Year 12, her HSC year. Actually, she went through some fertility preservation treatment during the beginning of that year.
That’s another thing most people don’t think about, do they?
No, there are so many things with chemotherapy treatments that are hidden unless you’ve been through it yourself. The chemo treatment usually causes sterility. So while she was in year 12, she had hormone treatment and surgery to harvest eggs and store for her to use if and when she’s ready later on in life.
Most year 12 girls spend any spare time they have planning what they’re going to wear to the school formal.
Yeah. Not Brooke. Actually, she was leaving school at lunch time and going down the road to the pathology centre for blood tests before going back to school for the afternoon’s classes.
It was a tough year for her: she had an egg harvest in about April/May, and had a reaction to the follicle stimulation drugs. She ended up with hyper-stimulation syndrome and was in so much pain from it. She recovered from that, and then did her HSC trial exams. Fortunately, she did some accelerated courses, so she’d already completed her HSC requirements for some subjects. But still, she ended up having to apply to NESA (the NSW Education Standards Authority) for exemption from her final HSC exams because she was already in post bone-marrow-transplant isolation by the time the exam period came around.
That must be an unusual application!
Yes, the options on the form for missing an exam were things like “I had a car accident on the way to my exam” and the like. No box to tick for “I spent my two-week study period in a bone-marrow-conditioning-induced febrile hallucination”!
Such a tough year! How did she cope with it all?
She’s been so calm throughout it all. Once or twice, she got upset about the unfairness of it all: her friends finished their exams and went off to Schoolies for a couple of weeks of partying. She was awarded her HSC exam exemption and went off to a period of post-bone-marrow-transplant isolation. She’s been so strong, but I think having some strong friendships have really helped her cope.
And how is she now, Jasmine? What’s next for Brooke now that she’s out the other side of her transplant?
She’s doing really well. She’s not allowed to work or anything yet because she has to avoid infection risks like big crowds of people. She really wants to travel, so she’s studying an online travel course. She’s still taking Cyclosporine, a T-cell suppressant to keep her immune system dampened down a bit so that her new bone marrow has the best chance to succeed. Once she’s weaned off that, she can start the process of post-transplant re-immunisation, and slowly begin getting back out into the world. Her plans are to learn to surf and travel up the east coast of Australia. Then when she is re-immunised, her goal is to work in the ski fields of Canada and travel the world. She loves being active and adventurous!
It’s such a long journey, isn’t it? Not just the transplant itself, but all the lead up to it, and then the months of post-transplant isolation and potential complications. And the long journey reminds me: you live in the Newcastle area, but you were at Sydney Children’s Hospital for treatment. How did you manage that?
My husband decided to take some time off work. If we hadn’t been able to do that, it would have been really difficult to manage.
That’s a big financial hit, right at a time when you’ve got lots of increased expenses.
Yes, it is. We’re lucky that we have our own house and a mortgage that we could draw down on. I don’t know how other families that don’t have that would make it through. And we had grandparents who could come and help. They moved in here so they could help with day-to-day things, and my husband and I took it in turns to be in Sydney with Brooke. I took 8 weeks off work. After that I would be here in Newcastle for two weeks while my husband was in Sydney, then I’d go to Sydney and he’d come home here to be with our son, and we just kept going like that: two weeks here, two weeks there.
That’s got to take a toll.
Yes, it does. It’s tough on relationships, and it’s tough financially. If you’re not solid on both those fronts, I think things would fall apart.
I know you received some help from Arrow. Was that enough to be of benefit, given the size of the bills you must have been racking up?
Yes, it was so helpful! We mostly had assistance with grocery and fuel cards, and that was really needed, and we are so thankful for the support.
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