Our beautiful daughter, Scarlett Adelaide was born on the 26th of October 2007. At about 72hrs old, the doctor at the hospital noticed and told us ‘uummm, I don’t know how to say this, but there’s something really wrong with your child’. After a week of tests, tears and waiting, we were told she had adrenal insufficiency and given some brief information printed from the nurses computer about what this disease was and how to treat it. Further follow up with our new endocrinologist saw us get more information, and we got on with living and treating Scarlett as required. For 4 years, Scarlett saw her endo regularly and had regular blood tests, always showing she was healthy and growing well. Other than her adrenal insufficiency, she was a regular, bouncy, cheeky everyday kid.
On the 25th of September 2012, everything changed. Scarlett was at preschool, and her brother, Bastian was at school, and I was enjoying a ‘day off’ spending time with my best friend. At about 2 in the afternoon, I got a phone call from preschool that Scarlett had had a nap after lunch (very unusual) and had then woken up and vomited. I went straight down and picked her up. That afternoon and evening she drank fluids and could keep them and her medication down, no vomiting, and went to bed without dinner because she felt sick and not like eating. She was given a triple dose of hydrocortisone that night to help fight her illness. She woke at about 4am on Tuesday 26th of September, got out of bed, came into our bedroom and asked my husband, Stephen to come and fix her bed, because her doona had fallen off. She appeared to be her regular self, with no signs of illness, or what was about to happen.
It was school holidays, so I got a bit of a sleep in, and woke at about 8am. Scarlett was still in bed. I went in and could straight away tell she had a fever, so I gave her a quadruple dose of hydrocortisone, and watched to see if she could keep it down, before trying to give her medicine for her fever. I called Stephen at work and we discussed our plan, that if she didn’t perk up within half an hour, I would give her more hydrocort, or her emergency hydrocort injection and get her to hospital. I also told Stephen to get home from work in case we did need to go to hospital. Please forgive me; this is where the memory starts to get a bit foggy.
Scarlett kept the first lot of meds down, so 20 mins later; I tried to give her another quadruple dose of hydrocort. I noticed her teeth were clenched shut, but managed to squirt her medication into her mouth using a syringe. I got a cold wet towel to put on her face and neck. I checked her 10 mins later and she had vomited in her bed and I noticed her hand was twitching, I thought she was having a dream. I walked out of her room, looked back and saw her legs jumping. I went into the kitchen, got her emergency hyrdrocort shot ready. I got back into her room, gave her the shot and her whole body and face had started seizing. I ran, threw the rest of her shot and needle on the kitchen bench, and ran back into her; she was in a full body seizure and now turning blue. I grabbed her up in my arms, grabbed my phone and called 000.
The lady on the other end of the phone stayed with me until the ambulance arrived. She kept on asking if the seizure had stopped, I kept on saying no, she’s punching me and kicking and getting more and more blue, and is frothing at the mouth, but is still breathing, just very rapidly and is not conscious. I thought she was having a seizure because of her high temperature. Stephen arrived home about 5 mins before the ambulance arrived, I can remember screaming and swearing at him to give Scarlett the other half of the vial of her emergency injection, he’d never done it before and was a bit cautious, meanwhile I’m screaming “give her the F*ing shot, just jab it into her thigh and push it in!!” the lady on the other end of the phone told me the ambulance was close and should be here any minute. Bastian, our son watched the whole thing unfold and I don’t remember him even being around, but I’m sure he was sitting next to us on the lounge the whole time. It only took about just over an hour from when I first woke up, to the ambulance arriving, it happened so fast. I still don’t know how Stephen got home from work so fast, but I’m glad he did.
The ambulance arrived, straight away put an oxygen mask on Scarlett and went to work testing her pulse etc. I remember the man asking me if her pulse usually ran at over 200 bpm. Her temperature was 40.2*C. They tested her blood sugar, and found it was so low it wouldn’t register, so gave her sugar shots and anti-seizure meds. Stephen went in the ambulance with Scarlett, because I was still in my pjs and covered in vomit and saliva from holding Scarlett while we waited for it to arrive. I remember the ambulance calling for another team member to assist in the back on the way to the hospital. The ambulance left, and I thought the quickest way of contacting everyone was to just write it on Facebook, so I wrote a status and jumped in the shower, got Bastian ready and left to go down to the hospital.
I arrived at the children’s hospital and went straight to the emergency triage nurse and asked to be let in. She said Scarlett was in resuss bay number whatever and they were expecting me. The doors opened and there was a social worker waiting for me- I thought that was very strange, but went in with her anyway. I arrived to find Scarlett covered in doctors, tubes and wires. I don’t know why, but I really expected her to be sitting there eating a lemonade ice block like all our other ER visits. All I could say was oh my god, oh my god. The social worker asked me if it was alright if she took Bastian to a quiet room to do some activities, I had completely forgotten he was right next to me. I’m glad she was there to help out.
I was told by the doctors as I arrived that Scarlett had just been intubated and had required a general anaesthetic to stop the seizure, it had been going for about an hour without stopping. The doctors said that they thought the seizure had been caused by severe hypoglycaemia. The doctors could see I needed to do something and asked me to help by holding this, or doing that, and said that when Scarlett was stable, we would go for a CT scan and then up to intensive care. I still remember thinking really? Intensive care? Isn’t she just going to wake up in a few hours and be her regular self? Stephen and I just sat there waiting, trying not to get in the way. After a while, Stephen went to find Bastian, and I stayed with Scarlett.
We then went and had the scan; I remember them trying to manoeuvre Scarlett onto the table with all her machines and the oxygen she was attached to. Then we went up to the ICU. Scarlett got hooked up to more machines and put on a cool mat, which helped lowering her body temp. Once we were settled in and I knew she was in safe hands, I called Stephen and we met up and got some lunch. Scarlett went off to have an MRI scan. We then went back up to Scarlett. The doctors told us the scan had shown either sick, or dead neurones in the upper front part of her brain, damage caused by the seizure. They were also running every test imaginable to try and find out why she was sick in the first place, and already had her on antibiotics and anti-virus drugs to combat it. They have never found the actual initial cause of her illness, and it will forever be a mystery. I’m very nervous it could happen again, not knowing what it was in the first place.
For two days Scarlett seemed to be stable, fast asleep in a coma. At the end of day 2, we noticed she was starting to have seizure like movements in her face. By the next morning, they were getting even worse, and she was biting the inside of her mouth and almost bit the end of her tongue off. The doctors ordered another MRI. Late that afternoon, the doctors asked Stephen and I to follow them. We thought we were being taken to see the MRI results. Instead we were taken to a little room with comfy lounges and cushions, both of us thought; oh no this can’t be good. They sat us down and looked very serious and worried. They explained to us that the most recent scan showed significant swelling in her brain, caused by having such a huge seizure, and that her brain was swelling at such a rate, they didn’t know if she was going to live, because her brain stem was starting to be squashed out the bottom of her skull, and that is the part responsible for breathing and basic life functions. They also added, that they could see significant damage in the lower parts of her brain, because of the pressure in her skull, and that if she did survive, they weren’t sure of how she would return, and that she may be completely disabled in every way. The whole world stopped turning. There were just us and a million tears. I’ve never been in so much pain in all my life. It hurt to breathe.
The next few days are a blur of tears and beeping machines, watching the numbers swing from bad, to really bad, then bad again. The doctors decided the only way to try reduce the swelling was to try and dehydrate the cells in her brain. They literally pumped her full of pretty much sea water. She had another connection attached to monitor how much water was coming out, compared to how much was being pumped in, so they could see if they were actually dehydrating her brain cells. She now had so many machines and monitors that they ran out of room on poles around her bed, and they were now all sitting on her bed with her. All we could do was sit and wait, and tell Scarlett to be strong and hang on.
After 3 or 4 days, we could see signs that the swelling was subsiding, and that Scarlett was improving. After 10 days in the ICU, we were told that Scarlett was well enough to have her breathing tube removed and we could start to see if she would wake up. She tried to open her eyes, but they were glassy and looked straight through us. I remember saying to her, Scarlett, its Mummy talking, you got sick, and you’re now in the hospital. Her face moved into a crying face and she tried to use her voice to scream, she understood and tried to react- at least we knew her ears and hearing was alright.
About a week after we left the ICU and were on the ward, Scarlett started to get sick again, vomiting and getting lots of ulcers in her mouth. The doctors did blood tests and discovered she had very few white blood cells, and her red count was low too. We were terrified that this mystery illness was coming back again. The doctors decided to send Scarlett of for surgery to take some of her bone marrow to test for potential problems. The tests came back clear, nothing scary like leukaemia, and still no signs of illness. As it turns out, Scarlett was having a very rare reaction to one of the anti-seizure meds she was on, that stops your bone marrow effectively making cells, and her levels in her blood were back to normal within 4 or 5 days of stopping the medicine.
By about 2 weeks on the ward, we could see that Scarlett’s eye sight was returning, and she was responding by smiling when we spoke to her or played with toys for her, or she watched a DVD. The neurology team handed over to the rehab team somewhere in this period and we started working with therapists to see how she may recover. Ever so slowly she became more alert and responsive, and earned the nickname, Smiley Scarlett. While her cognitive function slowly started to return, her body function didn’t. She was a little smiling rag doll. After about 2 months in hospital, the rehab doctors told me that they were diagnosing Scarlett with quadriplegic cerebral palsy. They said that she fit all the criteria for the diagnosis, and that having a name for what has happened will allow us to receive more support and services once were out of the hospital. What this diagnosis means is that the parts of Scarlett’s brain responsible for movement have been damaged, she will have to learn all of even the most basic functions all over again, if her brain allows it to be done. The doctors haven’t given us a prognosis, because they aren’t sure themselves of her recovery and what will happen.
So, every day, we worked with Scarlett and the therapists to see what may come back. We were told that she would be a
ble to come home when the improvements started to slow down, and the therapists could organise all the equipment for us to bring her home. We finally came home on the 6th of December, after almost 3 months of living in-between hospital and home. Scarlett came home with a naso-gastro tube still in for her food and now on triple her old dose of hydrocortisone.
On the 1st of February, Scarlett went in to have surgery, to put in a gastronomy, which allows us to feed her directly into her stomach with a pump, and her NG tube was finally gone. Since having the NG tube taken out, Scarlett has started being able to eat pretty much what we eat, just in little soft pieces- we’ve even dropped out one of her feeds via the gastronomy since, and she eats dinner and lunch every day. She has also started “talking”, babbling like a small child does when learning to speak, and has some words we can recognise, hopefully her speech keeps improving; she is very frustrated at not being able to communicate. We have been working with our speech therapist to find the best site to mount a switch that will connect to a communication device for Scarlett to use. We also practice standing, sitting, hand moving and lots of other functional things every day, in hope that we can help rewire her brain, and we can get the physical functions back. We picked up Scarlett’s custom wheelchair on the 4th of April, and it’s made getting out with her so much easier, we have a little freedom now. Scarlett will be starting kindergarten in a special physical needs unit in a school an hour away from home, in a few weeks’ time. She is very excited about finally being able to start in ‘big school’.
So here we are, 6 months after the initial event, getting used to our new lives and doing our best to aid Scarlett’s recovery in any way we can. We have a long road ahead, but are trying just to take every day as it comes, we can see teeny tiny little steps forward all the time, be it a new noise she’s learned how to make, or seeing one of her hands move purposefully, trying so hard to function like they used to. She is still cheeky, vivacious, and full of life, with her fantastic sense of humour, and she never stops smiling or trying. Her determination and never quit attitude keeps us going too. Please forgive my lack of emotion writing this story, I’m not ready to explore that path yet, and my main objective in writing our story is to let other parents and carers of adrenal insufficient kids know what can potentially happen, we had no idea all this was possible, even most of our doctors have never seen this happen before. I wanted to get Scarlett’s story out in the world, so even if one person learns something from our experience, our pain and sadness will have at least helped another not go through what we have.
Scarlett’s mom just sent us these photos taken 4 years later. You’ll see this little girl has come a long way. Just like most kids technology is one of the things Scarlett enjoys.