“Within minutes Elora went limp in her mum’s arms and her heart rate hit 225 and she began to seize”.
“Mathew and Shona stood by helplessly watching as their daughter’s life was in the hands of the medical team…”
On the 22nd of December 2018 Elora began to show signs of what her parents thought was teething, irritable, mild temp 37-38 and not eating solids as much as a normal day. That evening Elora was put to sleep by her mummy just like every other night, by 10pm Elora woke and was given Panadol as she had a mild fever 37.1 (not typically classed as a fever) but mummy knew it was coming. Elora was difficult to put down but fell asleep on mum’s chest. At 1am Elora woke screaming, her mum checked her temp and it was 37.6. Neurofen was given and the children’s health line was contacted. After speaking with the nurses and health advisor, Shona was advised to take Elora to the doctors in the morning. They persisted and Elora went back to sleep but with great difficulty. At 4.30am Elora woke screaming again, her right eye was deviated and Shona panicked and took Elora straight to the hospital.
They were admitted, checked by the nurse and taken straight to the resuscitation room. Within minutes Elora went limp in her mum’s arms and her heart rate hit 225 and she began to seize. Doctors and nurses came in from everywhere and began to attempt to place cannula’s into Elora’s hands, feet and arms with many unsuccessful attempts. Finally they got one in and they began to treat Elora with seizure medication to begin to try and treat them. One was not enough and another line was attempted to be placed again and again with many failed attempts. Time went by and they finally got another access line and medication for bacteria and infections were started to treat all illnesses. Elora continued to seize for 5-7 minutes at a time and one after the other. This went on for 7 and a half hours before stabilising her. Once stabilised and the seizures were slowed down they ordered a CT scan. They took Elora away and when they came back Shona and Mat were informed Elora’s brain was swelling and had abnormalities. Elora was immediately transferred to Sydney Children’s Hospital. Once they arrived at Sydney Children’s Hospital Elora was taken straight to the ICU and Shona & Mat then met with multiple doctors, neurologist and nurses that began to continue to treat Elora and investigate why she had suddenly become so ill.
An MRI was performed. The MRI showed greater extensive damage was occurring more to the left than the right.
Elora laid there sleeping and Shona had not seen her eyes open in over 24hours, it’s now the 24th of December. Elora had blood tests, stool samples, urine tests and nasal swabs done to identify any infections or bacteria causing the brain swelling. That night Elora’s heart rate dropped below 60 and her oxygen dropped to 60 within seconds, the alarm was raised nurses and doctors surrounded Elora and began treatment. Multiple medications were given and they applied a manual oxygen mask to help bring back her heart rate and oxygen levels. Mathew and Shona stood by helplessly watching as their daughter’s life was in the hands of the medical team…
It was a close call. Once Elora was stable they ordered an emergency CT scan and Shona & Mat’s fears were coming true; Elora’s brain was becoming more affected and there was white matter damage all over her brain, left worse than right. They made the decision to incubate Elora and take over her breathing and oxygen intake to let Elora rest. They wheeled out the resuscitation machine and had this on standby. That night they took them into an empty room and let them stay bed side with Elora. Another MRI was performed and all of Elora’s brain was now becoming affected, areas less than others but still greater damage to the left then the right. After the MRI results were returned we had a meeting on the 25th of December (what was meant to be Elora’s first Christmas). They had a meeting with all the doctors and neurologist, they discussed Elora’s condition and told them that Elora had Acute Necrotizing Encephalopathy (rare brain disease) that is generally caused by a virus or infection. The virus triggers an immune reaction known as a cytokine storm. It is the immune system that attacks the brain, brain stem and sometimes the spinal cord. The exact reason this happens is unknown.
Upon discussion with the neurologist and doctors they told Shona & Mat that they did not believe that their daughter would make it through the next 48 hours and to be prepared to say goodbye. They believed the damage was going to continuously become worse and that her brain may soon tell her heart to stop beating. They asked them if they would want to resuscitate Elora if this happened, but strongly believed they shouldn’t. With strong will the Doctors were told that not resuscitating was not an option and not to give up on their daughter. The outcome did not matter as long as she was here with us. Shona & Mat could not bear the thought of losing their only child. The love they had brought together with hope and faith was that Elora would stay with them. The Doctors began to tell them the worst possible outcome; that if this was to happen their daughter may never wake, talk, eat, walk and that she wouldn’t have any emotional function and that she may only cough and sneeze if she ever wakes. Again they assured and enforced their words and their love for their child.
On the 26th Immunoglobulin treatment was infused and again on the 27th to continue to help Elora fight the infection and give her immune system the best chances of continuing to fight (antibodies taken from white cells in adults, helps people with immune deficiencies). Over the next six days EEGS were performed, on the 26th and 27th seizure activity was still current and they upped Elora’s medications higher to help control the seizures. On the 29th until the 31st there was no current seizure activity. Over the next few days Elora remained stable and sleeping. On the 31st of December they began to lower medications and provide less sedatives to assist Elora with waking and dropped the level of help she was given on the breathing machine to see if she would breath alone, with a rocky start Elora showed signs of withdrawals from the medications and was give another lot of medication to help control her withdrawals daily. Elora was initiating her own breathing at irregular times; it was an emotional and scary time as we did not yet know if she was able to breathe alone. Over these three days as they dropped the medications and breathing machine assistance Elora became responsive to initiating her own breathing more regularly and on the 31st they made the decision to remove the breathing tube on the 1st of January 2019 and allow Elora to breathe on her own. The day had come the 1st of January and it was time to remove the breathing tube, Mathew and I Stood beside as they removed Elora’s tube anxiously waiting to see if Elora was able to breathe on her own. As the tube was removed Elora opened her eyes! It was ever so quick and small but she was awake!
Elora was breathing on her own and required little help. Elora was in and out of consciousness for the following week. At first Elora was unable to move but would make groaning noises. Once she was on lower doses of medication and she was no longer on ketamine and morphine Elora was able to leave ICU and move to the Neuro ward to continue with her recovery. Then slowly Elora began to defy the odds of what they thought Elora would never do. Elora started to side play around the 9th of January and then began to roll not long after only on to her tummy. Each day Elora showed us things we never thought were possible yet. As Elora was a vibrant and advance developer she was unfortunately not able to crawl or sit assisted and play for long periods alone but we hoped in the near future and still do she will be able to do these things independently. Elora struggles to use the right side of her body but with time and dedication and motivation from Elora it is believed she will be able to use her right side. The weekend of the 19th-20th of January there was talk of Elora going home to continue her recovery journey as she was recovering well and showing very good signs and on the 24th of January we were discharged. Elora attends physio therapy and occupational therapy 6 times a week, swims 1-2 times a week, Paediatrician every 3 months , has follow up appointments at Randwick Sydney children’s Hospital every 6-8 weeks and currently seeing an eye specialist as Elora can currently not see properly from the left eye moving out towards the left. In the near future Elora will also need speech therapy eventually.
Over this past couple of weeks Elora has started to use both of her legs and holding her own body weight. Elora recently has started constraint therapy with her left arm and hand to help motivate her to use her right hand and arm. Elora this week started to also patch her good eye the right to help her strengthen to see if we can get her eye moving out to the left. For the first time Elora started to laugh on the 17th of February. There are new miles stones every corner we turn. Elora will continue all her therapies for years to come to help her achieve the best possible outcome of living a happy healthy life
Given everything this beautiful determined little girl has been through she continues to thrive and progress each and every day!
It is a long road ahead for Elora.
*Update * Elora has tested negative for the RanBP2 genetic mutation. However a few months after her 1st ANE episode, Elora had another episode of ANE associated with Influenza, her Doctors continue investigations. Elora is remarkable and continues to progress with her rehabilitation.