Hemi Hero: Shakaia
As I am sure you all know Life throws many challenges our way. We ask why and might not understand it at the time of going through the situation but I realise now that it is to make us stronger and better people. For many years I longed for a baby and my wish was granted on 16 July 2015. I am quite bias but I gave birth to the most perfect baby girl. She hardly cried, ate well and would sleep from about 6 hours straight per night when we brought her home. She was a real people person, loved everyone and would go to all. I was told over and over by family and friends that I needed to have another baby to see what it was really like to have a baby. In particular, I had some cousins who just wanted to pinch her to see if she would cry. Shakaia was never sick, teething was a breeze. However, in March 2016 our lives changed forever.
One day I noticed she was starting to get conjunctivitis. 2 hours later Shakaia endured her first seizure at her great Uncle’s wake. She was 8 months of age. Shakaia was admitted to Lady Cilento Childrens Hospital in Brisbane where she was monitored and sent home at about 10pm that night. We were told it was most likely a virus. The next morning around 5ish Shakaia endured her second seizure, same as the one she had the day before. A full body seizure where she lost consciousness went stiff, body convulsing and went blue around the mouth. Later we would find out that these were tonic-clonic / grand mal seizures. We were transferred to hospital for a second time by ambulance within less than a 24 hour period. This time we ended up at Prince Charles Hospital. Unfortunately, her mummy had to take over the spot light and started having panic attacks. I felt like everything was crashing in on me, I couldn’t breathe as we didn’t know what was happening to our perfect baby. (I am sure we have all been there with this feeling at some stage or more than once during our child’s journey but unfortunately this is normal). Eventually I got hold of myself (I had to for the sake of my daughter) and they wanted to monitor Shakaia for the next 24 hours.
She was admitted to a room and was good all day. No signs of anything out of the ordinary, just Shakaia being her happy little self. However, later that afternoon I put Shakaia to sleep and within 5 minutes of me putting her to sleep she had her third seizure. Unfortunately, they could not pull Shakaia out of the seizure and there was talk of putting her into an induced coma. There were many hospital staff working on Shakaia in her hospital room. They ended up transferring her to the emergency section so they had more room to work on her. It seemed nothing they did would pull her out of her unconsciousness state and she had to be resuscitated. More and more hospital staff would come to assist. My memory of this night is very vague but what I do remember with the knowledge I have now is that I think she was having seizure after seizure and they were trying all different medications to pull her out of it. This is where we learnt the name “Midazalon” which would eventually become her emergency drug. I believe that the medical team there had a fair idea that she was having seizures but at this stage we were still unaware of what was going on and out of our minds with worry.
Shakaia was transferred to Lady Cilento Children’s Hospital that night and was monitored over the next couple of days. She underwent many test including ct scan, MRI and EEG monitoring. I being like any parent was in denial. I truly believed that my baby girl only had a serious virus, that she would be okay however, the diagnosis was given to us on the Tuesday and that’s when I felt like my world had fallen apart. Shakaia was diagnosed with hemimegalencephaly (Hemi meaning one side, mega meaning large, encephalopathy meaning where the function or structure of the brain is affected). If you are unaware, as our kids do have different conditions, Hemimegalencephaly is a rare neurological condition (as all our kids conditions are) in which one half of the brain, or one side of the brain, grows abnormally larger than the other. The unusual and enlarged brain tissue causes frequent seizures. I don’t know about anyone else’s experience but this was not picked up in any of my pregnancy ultrasounds.
The paediatric and neurology team of Lady Cilento Childrens Hospital was brilliant. They sat with us and answered all our questions and were very compassionate. This is where Dr Stephen Malone would become a big part of our family. We just didn’t know it at that stage.
We were given training on how to deal with Shakaia’s seizures which was basically to put her on her side, to try to film the episodes on our phone, to call for an ambulance where we would have to go to the hospital each time Shakaia had a seizure until we were comfortable with coping with them on our own. We were also taught how to administer her emergency drug if ever needed then sent on our way home. This was a very daunting time for us.
Over the next couple of months, we did not get very much sleep. Shakaia’s cot was set up next to our bed and with every slight movement during the night we would be checking up on her. You see Shakaia’s seizures began happening when she was asleep. She would have the tonic-clonic seizures that would last roughly 1 minute or so.
Over the next couple of months we were in and out of the Gold Coast Hospital as we lived down the coast, her medication was increased and new medications added until they were comfortable with the dosage she was on and comfortable with us handling her seizures at home. Not long afterwards Shakaia started having seizures night and day. We would turn our backs just for a second and return to find her having a seizure. It was getting hard to manage from home.
I contacted my cousin over in Canada who is a brain scientist and has worked with epilepsy in adults. She didn’t sugarcoat anything for us and told us the truth that it is a known fact that medication does not work for Shakaia’s condition that the only thing that would help with the seizures would be an operation to remove parts of the brain. We were warned of this by the Lady Cilento Children’s Hospital but never in our wildest dreams did we think our daughter would have to endure this.
At this stage Shakaia was having up to 14 seizures per day, which doesn’t seem like a lot or even close to what she would end up having in one day. Sometimes she would come out of the seizures crying, other times she would want to go to sleep in our arms but most times she would just get back up and crawl away. We would let her do whatever she wanted to do as we followed her lead. It was like she had to see if she could still move.
We had to administer her emergency drug which was the midazolam. Josh and I both had the experience of giving this to her. My experience with this was on 17 May 2016 when she went unconscious. I thought she was asleep in her uncle’s arms however, after a short while I noticed she was limp and we could not wake her up. I phoned 000 who were sending an ambulance out however, they seemed to take too long so I told them I was going to administer the emergency drug. I sprayed the midazolam in each nostril and it was like she came back to life just like that, it was magic. Shakaia was taken to the Gold Coast Hospital and unbeknown to us she would not return home for a very long time.
Shakaia continued to have grand mal / tonic clonic seizures in hospital and we were to record each of them. They were increasing and she started having episodes of bilateral convulsive, blinking / staring and unresponsiveness seizures with her head moving from side to side. Her seizures were then lasting up to 1 hour or more. For hours one afternoon, we asked the nurse for a doctor to come to Shakaia’s room as something was not right. We were told that kids have seizures everyday, that she was fine. Eventually I asked another nurse to get someone and when she came and saw Shakaia, she rung the alarm bells and I thank her for listening. Shakaia ended up in the critical care unit that night being 19 May 2016 then transferred to Lady Cilento the next day.
“Ryans Rule” have you heard of this before, the 2 words that may save your child’s life. Please if you do not feel that something is right, then you scream, you rant, you do whatever you need to be heard as you know your child better than anyone else.
Shakaia was sent to the ward at the Lady Cilento Children’s Hospital however, upon arrival she became unconscious and the alarm bell was rung for a second time in a matter of a couple of days. (And this would not be the last time that alarm bell would ring for assistance in the upcoming weeks to help Shakaia). One by one medical staff were racing to her. The alarm kept going. I believe there was about 30 people standing around her bed. She was resuscitated again and ended up being transferred to ICU so she would have 24 hour nursing care. During the night Shakaia was intubated, ventilated and infused with her emergency drug together with a new medication.
Shakaia remained in ICU and continued to deteriorate. Ongoing EEG monitoring and other test were done. This is where Team Shakaia was formed and ongoing support started coming in for our baby girl. On 27 May 2016 we walked Shakaia to the anaesthetist and said our goodbyes where she would go onto the operating theatre to have a right frontal lobectomy (in layman’s terms the right frontal lobe of her brain was removed). During this time family and friends gathered down at Southbank to write messages for Shakaia on purple balloons representing epilepsy then released into the sky.
Shakaia’s surgeon, Dr Martin Wood was happy, the operation was a success until she haemorrhaged requiring a blood transfusion. 2 days later I picked up that Shakaia was seizing again. She would pull this face that we got use to and we as parents know best right! However, I had to scream at the ICU doctors and medical staff for them to listen to me as the EEG monitoring of the brain activity was not picking up any seizure activity. They thought I was overreacting. It turned out that we were picking Shakaia’s seizures up before the EEG was even picking them up. This is where Dr Malone gave me the nickname “hawk eyes”.
On 1 June 2016 Shakaia was prepped for her second surgery in which they went back in to tidy up and take more of what was done previously. A second blood transfusion was required following her surgery and more seizures were detected. Again, we had to argue the point of Shakaia having seizures post op.
On 10 June 2016 Shakaia went back into surgery for a 3rd time. It was intended that they would go in and take some more however, not long after saying goodbye to her we received a phone call from Shakaia’s neurologist saying they found activity on her motor skills section of the brain which would mean Shakaia would be left paralysed on her left side if they were to remove it. We were given 10 minutes to decide whether we authorised for this part of the brain to be removed. This was the hardest decision we ever had to make but at the end of the day Shakaia being with us and having some kind of quality of life meant that we had to agree to this. Although the next phone call we received was to say that they found even more activity on her temporal lobe. What’s the temporal lobe function we asked however, it was decided by the medical team that they would go back to the original plan to just tidy up what was done previously and to take a little more tissue around where the frontal lobe was. They had decided that once done they would take Shakaia in for a MRI and if more tissue was required to be removed then they would go straight back in. So Shakaia ended up having 2 operations on this day which lasted over 10 hours. This day we were pre-warned that a hemisphere disconnection may be required down the track. Josh and I was still in denial at this stage.
Shakaia seemed to recover well following these operations and we thought this was the end of the surgeries. Unfortunately, a few days later seizure activity was found to be jumping from the right side of the brain to the left. We were sat down and advised of a hemispherectomy disconnection of what remained of the right side of the brain. That weekend Josh and I googled and searched about hemispherectomy and what the long term affects would be. We found video’s on other boys and girls who had undergone the surgeries and how well they were doing. But as you all know there is not much information out there. We decided that this operation would be the best thing for Shakaia as we wanted her to have some kind of quality of life as possible. That Monday we told her neurologist our thoughts however, he had told us that it may not happen for a while, that we had to wait and observe. This scared us as how would we take Shakaia home the way she was, she was on so much medication, more than an adult dosage. Dr Malone would refer to it as “commercial dosages”. At this stage Shakaia was basically in a medical drug induced coma, she could not move except for her right leg which she would stick up in the air as to say “I am here” and try to pull the tubes out of her nose with her right hand. Sadly, I think her left side had already been affected by this stage as it was no longer moving. Shakaia mustn’t have liked hearing Dr Malone’s information and that night decided to take things into her own hands. She had a massive seizure and went unconscious. At this stage we couldn’t tell whether she was seizing or not. Her 5th operation being the hemispherectomy was set for 2 days time.
Shakaia’s 5th surgery was a success. We were warned that she may need a shunt by her surgeon as they had to go in so many times. We are so thankful this never happened. In the meantime, a drain was inserted at the time of the operation to drain all the fluid build up. This drain was inserted in too far causing Shakaia to have irregular heartbeat and flat line in front of our eyes. Thank god this was easy fixed.
In a matter of 5 weeks, Shakaia:-
• Was having all different types of seizures
• In one day Shakaia suffered just under 200 seizures
• Having seizures lasting 1 hour or longer
• Put on commercial dosages of medication
• In ICU for up to 1 month
• Had 5 brain operations, the last being a hemispherectomy
• Became paralysed on her left side of her body
• Lost her peripheral vision
• Only cuddles she could have was when her mummy was allowed to jump into bed with her but there was always tubes in between us. 5 weeks it took for her dad and mum to have proper cuddles with her again.
As a result of the hemispherectomy, amount of drugs and operations Shakaia had endured, as well as her age, Shakaia was basically a vegetable; she had to relearn how to do everything again from suckling to moving her neck. We had to move her in her bed including her neck and head. When she was born, she had the strongest neck but after her 5th operation there was no strength whatsoever. Soon after she went to rehab where she stayed another 4 weeks and had to learn how to hold her neck, sit with support, feeding was through a nasal gastric tube which I was trained to feed her through.
Shakaia spent her 1st birthday in hospital but was allowed out for a couple of hours so we had a party for her at Southbank with family and friends. This was the first time Shakaia was let out of hospital in 8 weeks.
The following week Shakaia returned home after 9 weeks in hospital. For the next 7 weeks I drove Shakaia up to Lady Cilento Hospital 3 days per week for 2 hourly therapy sessions. As the weeks went by Shakaia’s body strengthened with the help of her physio’s and OT’s, she learned how to eat soft foods again and to have a bottle with the help of her speech therapist. These sessions had started while she was in rehab but she had also received music therapy and dog therapy where dogs would come to her bedside.
We could not ask for a better rehabilitation or neurologist team for Shakaia at Lady Cilento Children’s Hospital. Don’t get us wrong we did have our problems, Shakaia was in hospital for so long but her main teams, we could not speak highly enough of.
As you could imagine this was a rollercoaster ride we were all on. Our strength came from Shakaia, she was so resilient. The number of times we had the medical staff come up to us to tell us that Shakaia was their favourite patient was huge. I would tell them to get in line!
Shakaia was lucky enough to meet Taylor Swift while in hospital. Yes, we were there when Taylor visited and she was so lovely and genuine. She spent a total of 4 hours at the hospital and went to most wards. She was so caring and so good with the sick children. I am so grateful to have met her. It gave the kid’s time in hospital just that little bit of excitement, joy and hope.
Shakaia, our warrior princess has defeated all odds and is doing exceptionally well. Her operations were a success.
When Shakaia returned home I looked into every option that the hospital had recommended me to from Disability Services, to employing a private physio which is about a 40 minute drive to a private OT which is close to an hour drive but at the end of the day I’ll drive Shakaia to wherever she needs to go to ensure that she is getting the best help. Her speech therapist would come to see Shakaia at home. She also attended a music therapy playgroup through Montrose as well as hydrotherapy. We were lucky enough to attend a baby bridges class which went for 6 weeks. I was in one room with the other parents talking about our experiences and other options suggested to us while Shakaia got to play with the other kids and have further physio and speech therapies (all these services being free through the government or Betterstart). Last year we were busy with therapies from 4 to 5 days per week. I was previously working from home however, Josh and I had decided for Shakaia to have the best kinds of success in life that I would give up work to focus on her. I know that this is not the option for all families, but it was for us and let me tell you it is hard whether you are a working family or not.
In the first 6 months following Shakaia’s release from hospital and the hospital rehab team, she had the NG Tube removed, learned to eat foods from purees to sandwiches, mashed veges, sushi, fruit, crackers / eating off mum and dad’s plate, to drinking from a sippy cup or straw cup & to drink water, Increased language / words from dad, mum, bub, yum to 50+ words, her Core Strengthened, she learned to sit on her own and to get from a laying down position to sitting on her own, commando crawl, bum shuffle, and starting to stand a little with help. With a lot of restraint work her Left Arm started to move from the shoulder. We still need to do a lot of restraint work here. Shakaia learned to clap again by clapping the top of her left hand with the right palm, she has started getting around the house exploring by bum shuffling / crawling mostly bum shuffling and getting into bags – pulling items out, opening and closing draws that she can reach and pulling out all items. She loves to play peek a boo, has learned to play such as posting games, Doing gestures & talk ie wave & say bye or hi and of course blow kisses.
This year we have continued with the physio, OT speech and hydro therapies. Shakaia can now kneel on her knees, she can stand at furniture with our support, stand on own with our support and move her left arm. She currently has a standing frame we put her in and we are looking at getting her a walking frame. Her language has improved and she is now repeating so many words. To look at her you wouldn’t know she has been through what she has been through. She is definitely a success story and a little miracle. The best news of all is that she did not lose her loving personality and we saw that come back as her medication was reduced after returning home. Shakaia’s medication intake was reduced to 5 morning and night when she left the hospital. She is no longer on any medication since February this year.
Prior to Shakaia ending up in hospital, she was crawling, and had started pulling herself up onto furniture. Shakaia is not yet pulling herself up on furniture but this is something we are working towards as well as walking. Therapy we realise will be part of her everyday life. Other challenges she will need to face is to learn what we take for granted by doing with 2 hands where she will have to learn to do with one hand from dressing herself to cooking. But you all know this. I actually received a message from my cousin today that she has looked into everything for Shakaia and she believes Shakaia will have a good life.
Shakaia amazes us every day, her ability to do what she can do with what she has been through is remarkable. You probably wouldn’t believe me but Shakaia is starting to have little tantrums and hitting things as well as her mummy and herself in frustration. This is something we are currently looking into as to whether there are any behavioural issues but I am hoping that she has entered the terrible two’s early.
I realise we are so lucky, even though Shakaia’s experience was so sudden and short, that some of your kids don’t receive their diagnosis for years and the pre surgical testing can take months. Shakaia couldn’t have all of her pre surgical testing as everything happened so quickly and she was too critical to be moved from one hospital to another for the testing.
In July this year, Shakaia will have her two year old review in which she will begin Botox injections to her left arm and calf. Please don’t get me wrong, this has not been an easy road and we still continue with everyday challenges which we in this hemi group all know.
And at the end of the day we did not really get the option to have this surgery but to see what we saw our daughter go through and to see how far she has come and how well she is doing today, to those families that have not yet gone down our road, I do promise you it does get easier. I cannot talk for the families that have had to endure watching their kids have a shunt and to those kids still having seizures as that is even harder work but at the end of the day not one of us would give up on any of our children and we will continue fighting for them and do what we need to do for them to have the best kind of life.
After meeting the other little kids and big kids at this year’s first hemispherectomy conference I realised which I think we all did, that we have some pretty special kids in this world. They have such a beautiful aura about them and are so loving so at the end of the day we are the special ones to have them in our lives.
I am happy to say that Shakaia has been close to 12 months seizure free. We have had a few scares but these were just clonis episodes and for someone who underwent 5 brain operations, then it’s understandable that her brain was going through so much with recovering. Her determination and strength outstands me. She had to remake new milestones and is now even meeting some milestones of her own age group. That is amazing for someone who had to relearn how to do everything again and is still learning how to redo some things.
We would not be able to get through this experience without not just our family, but our extended family, close friends and our hemi family.