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This is Samara’s story about her EDS journey, as seem through her Mum’s (Carolyn’s) eyes:
“After a recent move from NZ, my husband and I discovered that we were going to be having another baby. There was a 9 year gap between our then youngest and the soon to be Samara. Following a bleed at 13 weeks, at 18 weeks we discovered that I had Grade IV placenta previa. I was told to take it easy, however I was told to prepare for my first c-section after 5 natural deliveries of 5 healthy daughters.
At 26 weeks I had a "significant bleed" and was transferred by ambulance from Nambour (regional QLD) to RBWH (a main Brisbane hospital). After a few days we were sent to Ronald MacDonald House and told we would not be going anywhere until after the baby was born. It was during this time that I got a phone call from my mother to say we had an answer for our floppy joints - Ehlers Danlos Syndrome. Not that I had much time or energy for looking into it that much. Due to my previous 2 girls being born at 35 weeks, it was decided to schedule a Caesar at 34 1/2 weeks.
At 31 weeks I began haemorrhaging, lost consciousness and was raced to the hospital. For reasons I will never know (but which became significant later), rather than delivering straight away, they left us 4 1/2 hours. But apart from me ending up with a collapsed lung, fluid build up in a Fallopian tube, and a blood transfusion, things seemed to be going fairly well for Samara, who was born weighing 1503gm. Initially the things I noticed with her were that she never ever cried. The other premmies all did. And every time she had a tube feed, her saturation levels would drop. Apart from that, all seemed well for a baby of her gestation.
At 4 weeks, they dropped a bombshell. She had Down Syndrome. I was completely thrown. They can see it in scans, yet 9 scans, and 4 weeks out in the world, and we had not seen this coming! I went through all the various grief stages, flipping from denial to acceptance and back again like a yoyo on steroids.
At 5 weeks she was diagnosed with laryngamalacia. At 11 weeks, she finally came home on NG feeds and thickened milk. I spent most of the next few months attempting to breastfeed, thickening EBM to try and feed with a Haberman, then tipping what she didn't drink down the sink and putting unthicken milk through the tube. Apart from that and countless admissions to hospital with pneumonia, I don't think I did much for the next year or so!
Because we are Kiwis, Samara was not a citizen at birth. This meant she could not access therapies, equipment etc. I won't bore you all with the fight we had over the next 5 years except to say we had to move out to Roma, in order to get a medical exemption on her Permanent Residency Visa. It was a long, hard and expensive battle, but one we eventually won.
Now, for the rare part. So far you are probably thinking Down Syndrome. That's not rare. But the thing with Samara is, she didn't stop there. At 2 she was diagnosed with a mild hearing loss (which has gotten worse, and is now moderate in one ear and severe in the other). At 4 she was diagnosed with ASD. Both of these are not a major surprise in Down Syndrome. But it was obvious there was more going on than that. At 5 she was diagnosed with Bronchiectasis. At 6 she was diagnosed with Ehlers Danlos Syndrome, and Cerebral Palsy - left hemiplegia (thanks to oxygen deprivation before birth). She also has a lobe in one of her lungs that never developed properly.
Samaras multiple diagnoses make her one of a kind. It's like she fits in many boxes, but she fits none of them very well! All the initial predictions of how well she would do have not come to pass, and I still struggle with that at times. Especially around other children with DS. But she is a happy little girl most of the time, who really makes our lives so much better.
At 10, Samara is attending special school, which she absolutely loves. She is mostly non verbal, but between the words she has, PECS, PODD, Pro Loquo, and Auslan, she usually can get across what she wants to say. Although she does get frustrated when we do not understand her.
She is incontinent, which freaks me out as she approaches puberty. It's hard enough cleaning dirty nappies now, let alone when there is hair to deal with too! I think I will stay here in denial a bit longer...
Samara is on a softened diet due to aspiration and choking risk, and has most of her fluids through her G-Tube. She only has one sister still at home, 19 year old Mahalia. The two of them are inseparable, and best friends. Mahalia is attending a musical theatre school, and as a result of that, Samara is a huge fan of musicals. Particularly Grease which Mahalia performed in last year, and which Samara had as a theme at her 10th birthday. She is also a big Emma Wiggle fan, and she loves superheroes especially Spider-Man.
As a family, we love travelling, and everywhere we go involves some sort of animal park, as Samara loves animals. Last year we went on a motorhome holiday up to Rockhampton, across to Carnavon, Down through Roma (where 2 sisters and her nephew still live), then back via the Western Downs Zoo. In January we went to Ballarat and Tasmania (and of course to the zoo in Tassie!) and last week we went to New Zealand for her big sister’s wedding (and yes, to a wildlife park where she got to see a real live Kiwi!). In December we are off to Fiji for 3 weeks - we were there 2 years ago and she loved it. The people were so friendly and so accommodating.
Because we did not have any funding until Samara was 5, I started a charity that year called Aroha Angels which helps families out with equipment loans when they cannot get or are waiting for funding. And a few months later I started Just Juniors Disability, specialising in paediatric disability equipment. Both of these take up most of our time, energy, money and house! But I wouldn't change it for the world. We get to meet so many amazing families. If it wasn't for dealing with government departments I would say we have the best job on earth!”