Amber's Mum Claire shares her story:
In 2006 when Amber was diagnosed with cystic fibrosis through newborn screening we were shocked. Like for most families when there is a cystic fibrosis diagnosis, there had been no previous indicators such as other known carriers or PWCF in the family. Although Amber was born small and slightly premature there were no red flags, she is pancreatic sufficient and therefore was growing as expected.
I remember it being very overwhelming in the beginning. Amber was our first child and I must admit I felt relief when I knew IVF was an option to help us avoid further heartbreak regarding another child with a positive diagnosis. IVF had a few challenges, but it was definitely the best path for our family and Amber has a brother, Isaac who is two and a half years younger than her.
Counselling has helped me over the years, for both CF related stuff and other life challenges. I definitely believe in the saying ‘a problem shared is a problem halved’. And my advice for others is, if you feel you could benefit from counselling don’t stop trying if you don’t relate to the first counsellor you meet – keep looking until you find someone you are comfortable with.
Having hope is so important and sometimes this is difficult with it being so hard to get new treatments into NZ. When Amber was younger, I thought life changing medicines would be here by the time Amber got to her teenage years, it is heart breaking that this is not the case, that there are so many hurdles.
In general Amber keeps very well. She has been hospitalised twice with pseudomonas – both times as a pre-schooler. Since then she has had pseudomonas pop up once again on a cough swab at age 11. In that time treatment changed a lot – it is a lot simpler to manage treatments for pseudomonas now versus 10 years ago.
Our biggest concern with Amber’s health over the past few years has been her lung function. Her FEV1 has always been quite low and this has been worrying. The medical team has been very supportive, and we have explored many options to try and find out why she has a low FEV1. As a result of various tests, we think this is just Amber’s norm and are no longer overly concerned. This could have very little to do with CF and may just come down to her body shape and size.
I feel that as parents we are advocates for our children and I have always followed my gut and asked questions. I believe the best thing is for us to have good relationships with Amber’s schools, medical team and CFNZ – for various reasons these are the relationships which will help us ensure Amber keeps as healthy as possible for as long as possible. There are other important relationships I think can help too and I believe knowledge is power.
The whole family, including extended family, is impacted when a loved one has cystic fibrosis. Our son used to see Amber getting lots of attention and gifts due to her having CF. I believe it was difficult for him to comprehend the other side of why she was getting these gifts and attention, until he was mid-late primary school. And when Amber needed a PICC line at age 3, a concerned family member panicked (after reading about a bad experience for a PWCF) and I had to deal with this as well as getting my own head around what Amber needed.
I hope parents of younger children realise physio gets easier – once PEP became an option for Amber, and as she got older and we could explain how important physio is for her, our life got easier and compliance got better. It also helps that our CF branch offers support with physio – through this, we have had regular contact with a lovely private physiotherapist and have had caregivers come in and help us a few times a week with Amber’s PEP. This has taken a lot of pressure off me, has been hugely beneficial for our family and improved physio compliance hugely.
The impression I have always had is that a lot of parents feel that a vest would change their lives. I think that as CF parents, ‘we’ pin our hopes on things like the vest but having owned one (again due to a well-meaning, concerned family member) I think there are better ways of spending the huge amount of money that a vest costs.
For me, other than the tragedy of people dying too young, one of the saddest things about cystic fibrosis is the risk of cross infection. I have always felt well supported by other parents of children with CF, having met them through conferences or at our local committee meetings. When Amber was younger, I wished she could meet with other PWCF and get this same support. Now she is older, social media is starting to help with these connections, but it is not as easy building a friendship having never met the person.
When Amber was a baby 13 years ago, the CF nurse told me that people with CF are special. She said she didn’t know if it was due to what they went through or whether it was nature’s way of balancing things out. These words have always stuck with me and I agree. I think their siblings are pretty amazing too – they go through a lot themselves and seem to be stronger and more empathetic because of this.