Please note an abbreviated version of Ariel’s milestones can be found at the bottom of this page.

The Beginning 

It was February 2019, and we were in the middle of the normal flu season – nothing special. Each family member got sick with the flu, one after the other. First the parents and then the three boys.
The youngest, four year old Ariel, was the last one. He had a high fever for three days. It was almost impossible to lower the fever. On the fourth day, it was a Saturday, suddenly the fever was gone, and he felt much better. We thought the worst was over and soon we will be back to our usual lives.

It was around noon when we noticed that Ariel was walking with strange small steps. He almost did not lift his feet from the ground. When we asked him why he was walking like this, he did not answer. We realised that he seemed to have problems with his balance. He appreciated holding hands when walking. We thought that he might feel a bit dizzy because the flu weakened him so much.

Wrapped in a blanket he sat on the sofa when his brothers went upstairs to watch TV. I asked him if I should carry him upstairs so that he could join them. Normally there would be only one answer to this question, “yes”. But I had to ask several times and insist on getting an answer until he replied, “upstairs with blanket”. As it turned out later these where the last words we heard from him for a long time.

Ariel was completely exhausted from the flu – that’s what we thought. Even when he was sitting on the toilet, we had to hold him, because otherwise he would have fallen to the side. We put him to bed so that he could have a long sleep to recover and regain strength. If he didn’t feel better in
the morning we would go and see a doctor.
To be able to monitor him during the night he slept on a mattress next to our bed. I woke up around 4am because Ariel was so restless. When I checked him, I saw that he had wet the bed. This astonished me as he had not wet the bed for a long time. We made him fresh and took
him to our bed.
But we could not sleep, we did not have peace anymore. “Did he wet because he was not able to walk to the toilet? Did he need help from me to sit him up because he was not able to? What could be the reason for this? Can children get strokes?” I grabbed my mobile and started to look for answers. So did my husband. My research about strokes and children showed that there is no time to lose. But were the signs he showed really the ones of a stroke? It did not really match. My husband was more successful in his research. He read about ANE (a big thank you on this point to ANE International, who helped by making information about ANE available and discoverable on the internet). “No, it must not be ANE!” he thought and continued with his research. He did not tell me then what he had found because he did not want to frighten me.

It did not take long, and we could not stand the uncertainty anymore. We got dressed and threw a few clothes for the older brothers (9 and 6 years old) into a bag. Sunday morning at 6 o’clock we called my parents and informed them that we will drop the two brothers at their house in a few minutes because we must hurry to the hospital with Ariel. We took the kids directly from their beds to the car and we were off.

The Emergency Department
Half an hour later we arrived at the emergency department. We were not classified as urgent and had to wait initially. Even though it might have been only 10 minutes it felt like an eternity. Finally, we could go and see a doctor. He was very brief as it was just before the end of his shift. He too did not classify Ariel’s case as urgent or life-threatening, he only did a superficial examination and checked that it was not meningitis. He told us that in his opinion it is nothing neurological and as he is finishing his shift his colleague will come later and take a closer look on Ariel. We had to wait, and again it felt like an eternity. The young doctor came and took a closer look at Ariel. It did not take her very long until she came up with the suspicion that it is something neurological. As there was no neurological pediatrician in the hospital on this weekend, she decided to have Ariel transported to the children’s hospital. While we were waiting for the ambulance to pick us up, Ariel received some Isostar to drink with a drinking straw to avoid getting dehydrated. Yes, at that time he was still able to drink. Our “taxi” arrived, and I was allowed to accompany Ariel in the ambulance. My husband did follow us in our car. The doctor who came to pick us up ordered to drive with blue lights and siren. I was astonished about the sudden urgency. I still hadn’t grasped the seriousness of the situation. While my husband, with a bad feeling in his stomach, fought his way diagonal through the city of Zurich (of course he was not able to keep up with the ambulance), I could see how Ariel became more and more sleepy. I told him “It’s okay, have a rest”. Even though the doctor did have a good poker face I realised that she did not take it as a good of a sign as I did. She was afraid that he was drifting into a comatose state. She decided to call the hospital and have a shock room ready. A shock room is when a specialist team of all the different medical areas come together to see a patient and discuss and try to find out what the reason for the state is and what the next steps are.

At our arrival Ariel was brought to this room where everybody was already waiting. I had to wait outside. Wait again. My husband
arrived at the hospital and joined me.
After quite a while everybody came out of the room. We were informed that they are going to do a CT as a first step. They wanted to be sure there is no bleeding in his brain as Ariel had fallen down the stairs the day before. After the CT we were taken to a room in the emergency department. I was
glad we did not have to wait outside on the corridor as many others had to. Or was this a sign that it was more serious than “just a broken arm”? The CT was unremarkable. They continued with other tests. He tested positive for influenza A, which did not astonish or disturb us. It was just the flu.
They did a lumbar puncture to see if there was an active infection (virus or bacteria) going on in his brain. But there wasn’t any. This made it clear that steroids could be used. The doctor told us that he is going to monitor Ariel and if his state is getting worse Ariel would get a big dose of cortisone. It did not take long and he came back with the cortisone as he did not want to wait any longer. We learned that the standard procedure for such cases is to give a huge dose of steroids (cortisone) for 5 days.
This should help to decrease the inflammation which they assumed to be in his brain. I asked them if he must stay overnight. They told us that they have prepared a place in a room for him already and that I will be able to sleep in that room too, but he will stay on the emergency department until
evening as he can be better monitored here.
It was 7 p.m. when they came to relocate him. Not to the general unit but to the intensive care unit (ICU). I just got used to the thought that he had to stay overnight and now ICU! On the one hand it worried me but on the other hand it also meant that he will be in a place where there is no other
place in the hospital where he would have been monitored better.
For my husband it was time to go home and pick up Ariel’s brothers. It was not easy for him to leave Ariel, but his brothers needed some care too.

ICU
I stayed with Ariel in the hospital. We did not yet have a diagnosis. The doctors were searching in all directions to find out what’s the reason for his symptoms. An MRI was planned for Monday morning but then they decided to do it sooner, on Sunday evening. For the MRI Ariel needed a general
anaesthesia.

Intubated and sedated he came back from the MRI. The plan was to keep him sedated for the night so that his brain can have a better rest. The MRI showed that he had several smaller inflammations. The exact analysis of the MRI-pictures would be done on the following day.
I didn’t sleep much that night (they could give me a bed close to the ICU for one night). I spent a lot of time at Ariel’s bed, holding his hand and talking to him. I would have loved to stroke his head but there was no space left. Everywhere tubes, wires and machines. His forearms were bandaged
because of the venous catheters. Only his hands and legs were left where I could touch him. There were needles in his hair and scalp. They belonged to a “mini EEG”. Two big black badges were stuck to his forehead. They belonged to a new machine which was still in the testing phase. If I got it right,
it measured the oxygen saturation in the brain. There was a gastric tube through the nose and the tube for breathing through his mouth. A picture you are not going to forget quickly.

Monday – 1st day in the ICU
On this day the doctors started to treat Ariel with vitamins intravenously. This is because one possible reason for his symptoms was a metabolic disorder which results in a vitamin deficiency. The testing for this disease takes one to two weeks. (Ariel received the vitamins until this disease could be excluded). The doctors were still searching for a diagnosis. A lot of tests were done and to have better access to Ariel’s blood for the tests they did an arterial catheter and a central venous catheter.
To have a better idea of Ariel’s state, it was the plan to wake him up and extubate him if possible. As the reason for his brain inflammation was not yet clear, there were not many treatment options. Not that this would have changed after the diagnosis. But for the moment Ariel got the vitamins and his daily elephant dose of cortisone. No one knew if the cortisone was helping but they gave it anyway and hoped it helped. Beside this they did a so called “brain protection”. They do everything to avoid the brain being damaged. For example, keep the head in a higher position for less pressure and keep the body temperature as low as possible.
Even though they did stop the sedative medication on Monday at noon, Ariel did not wake up.
It is so hard to see your child like this. Not knowing because of what, not knowing what will be and from a medical point of view – nothing you can do to help him.

Tuesday – 2nd day in the ICU
During the night on Tuesday Ariel started to open his eyes from time to time but his eyes were still rolled up. He was holding my hand lightly. He was still on a ventilator but was able to breath on his own since he became a bit more awake. On that Tuesday afternoon we had a detailed conversation with his doctor in a separate room. The doctor explained that they had been able to narrow down to two possible reasons for his encephalitis. One was the metabolic disorder which would cause a vitamin deficiency and the other was ANE. You don’t want either of the two. The doctors continued to treat him with vitamins (intravenous) and cortisone. As ANE can have a genetic reason a DNA test was ordered. To get quicker results my husband and I had been tested too. It then still took about 5 weeks to get results. Further on we had been told that there is not much that they can say about the course of what was happening. They did not make any prognoses. The doctor continued, “what we can say for sure is that his brain is damaged”. Stop! What did the doctor say there? Ariel’s brain is damaged? It’s not “just” an inflammation in the brain, it also gets damaged by it? I felt like all the blood drained from my face and I had to assure myself that I’d heard correctly. But the doctor confirmed: the brain was damaged. What exactly, how strong and whether it is reversible or not, they could not say. “It will show once he wakes up from the coma – if ever he is going to wake up at all.”

This was the first hammer crashing down on me. The second was when the doctor explained that we have to be prepared that Ariel will have to spend several weeks in the hospital followed by several months of stationary rehab. I know it is totally naive, but somehow, I always thought that Ariel will be in hospital for three to four days and then everything will be okay again. It was just on Sunday when I thought that we might be able to take him home and now we were talking about such a long period! It would not have occurred to me that this could happen. Beside the sorrows for our son’s life and health we now had to deal with this logistic monster. How are we going to organise our life?

We decided that I will stay with Ariel while my husband had to go back to work and take care of our other kids. During the day they were at school, over lunch they were able to go to a school mate, neighbour or grandparents. On the weekends my husband and I changed, so that he could spend
time with Ariel and I could spend time with the two other boys and do the laundry etc.
The doctor himself told us that they have done what is possible. There is nothing else we or they could do but wait and pray. The sooner Ariel would wake up the better are his chances for recovery. And so we did wait and pray. I had to think about how Ariel was. His laughter, his singing and
jumping around. How he learned to ride a bicycle and he was learning to ski and almost able to swim. He so much loves to cuddle but I was not allowed to hold him (because of the breathing tube) and there was almost no part where I could touch him to comfort him. I would have liked to give him the comfort and security he needed. This was extremely hard.

We had to learn to take one day after the other and see what it would bring. And this for the following weeks and months. No prognosis; it can happen where there is everything or nothing and everything in between.

Wednesday – 3rd day in the ICU
Ariel was still sleeping a lot but he did open his eyes from time to time. On this day they tried to take him off the ventilator for the first time. It was a critical moment because they did not know if his throat muscles have enough tension that he is able to swallow and he will not aspire his saliva. It all
went well. Ariel was able to breath on his own and his swallowing reflex did work well! We got rid of the first machine. What a joyful day! We could see that Ariel felt much better now, that the breathing tube had been taken out of his throat. Before he had spastic legs (high tension in the
whole body) again and again, now he was lying much more relaxed. I think the spastic movements were his attempt to remove the breathing tube, but he was not able to control his body at all.
The nursing stuff suggested to hang up pictures of Ariel’s brothers and our dogs, as this usually helps the children, and it would also help them to get to know Ariel better. And they were right. Ariel loved to look at the pictures with the familiar faces.

Thursday – 4th day in the ICU
Another milestone has been reached. The “mini-EEG” and the monitoring of the oxygen saturation of the brain had been removed. Two more machines we got rid of! And finally, I was able to touch his head again. A full EEG was done. They wanted to be sure that he does not have seizures. Although seizures typically come along with ANE, Ariel has never had any. Hallelujah! On the same day Ariel could be moved to the Intermediate Care (IMC). A bit less intensive, intensive care unit. Even though it was only a few meters, and they were pushing his bed very slowly and softly it was
extremely exhausting for Ariel.
Ariel was in the IMC for about 6 days, making a little progress day by day. Once he tried to speak but only a voiceless “ph” came out of his mouth. When I asked him, if he tried to speak, a tear run down his cheek. When his brothers came to visit him, he became more active. And suddenly, things were possible, that he hadn’t done before.

General Unit
Finally, Ariel could be moved to the general unit. It was there where we received the result that it was not the metabolic disease. Consequently, Ariel’s diagnosis was ANE. You can get ANE with or without known genetic mutations. The diagnose was based on the MRI pictures which showed a very
characteristic inflammation pattern for ANE and the exclusion of the other possible reasons. Later when we were in rehab we received the results from the genetic testing. Ariel and my husband do carry the RANBP2 mutation.
In my mind I see the scenes how it all began again and again. What a horror! What a frightening feeling it must be when you realise that you slowly but steadily are losing control over your body!
Now it was all about regaining control over this body again. Step by step. Learn to turn, to sit up, to transport the food to the back of the mouth that you can swallow it. While we were waiting for a free place in the children’s rehab clinic Ariel had physio, occupational and speech therapy. In
the latter it was less about learning to speak and more about learning to eat and swallow properly so that the gastric tube could be removed. To open his mouth was the only controlled movement Ariel was able to do reliably. That’s why we used this movement as a communication tool. Now we could ask him yes or no questions and when he did open his mouth it meant “yes”, and no movement was “no”. If you think to say “no” he could have just shaken his head, I have to tell you this was too difficult for him to do.

 

 

Rehab
On March 5th , 2019, about 20 days after hospital admission we were lucky to move to the rehabilitation clinic. At that time, Ariel was able to turn from his back to his belly and back again. Sometimes he could sit up and he was able to crawl on all fours over two meters. He was able to grab a Duplo Lego brick, but it was too difficult to place it correctly on another one. We could feed him with pureed food, and he was able to drink enough thickened fluids that we could take out the gastric tube.
In the rehab Ariel got daily therapies (physio, occupational, speech and neuropsychological therapy and checks). All the therapies needed Ariel to be playing with something. Otherwise he did not cooperate well. He did not like it at all as soon as he realised that someone tries to find out (or to test) what he is able to do and where his limits are. But where he engaged himself 100% was in the animal supported therapy with dogs (occupational therapy) and horses (physiotherapy).
We are very grateful for this children’s rehab clinic with its various and modern therapy possibilities for the patient and the warm commitment of the therapists.
We spent three months there. On weekends I was allowed to take Ariel home. Luckily he was light enough to carry. We could carry him into the house and then carry the wheelchair which we could borrow from the rehab. If he had been heavier, we couldn’t have taken him home as our house is not accessible for wheelchairs.

 

If we have learned one thing during this time with ANE, it is that there is no other way than to take one day at a time. You are not able to make any plans for you or your son’s future. No one dared to make a prognosis as to which capabilities he will or won’t regain. We were only told that as soon
as he regains a capability, we know that he can regain it and not before. So we took one day after the other and celebrated every little bit of progress. (see also the overview of the milestones below).
The physical part is one thing. But there is also a psychological side of the story. The time in the rehab clinic was extremely challenging. Ariel is a fighter and has a cheerful nature. These two characteristics helped him well in this situation. Nevertheless it was a time filled with outbursts of
fury, frustration, grief, fight, lack of understanding, joy over little successes but above all a lot of homesickness. Ariel was desperately missing his home, the well known surrounding and especially his brothers. Evening after evening he cried himself to sleep even though I was there with him all the time.
Of course there were such and such days. To support him and absorb all this while I had to deal with very similar emotions was one of the most difficult things for me. The physical deficits caused by ANE were obvious. But that the encephalopathy threw him back also in his psychological/emotional development took me a long while to realise. He had almost no control over his emotions anymore.
One example from the rehab: You would think that a five-year-old boy would not have a problem to understand to use a play structure alternately with another child. He might not be happy about it, but he understands that now it’s the others child turn, and he must wait. Ariel was allowed to start and when it came to the other child’s turn, he went overboard, he completely lost his temper. It wasn’t the first time, and it wasn’t the last time for a long time. These outbursts accompanied us for at least one year after the rehab. With time they became less often and finally disappeared completely.
But to deal with these outbursts, to somehow stand and manage it did cost us (the parents) a lot. It emotionally totally drained us. You could not use us for at least two hours after such an outburst. I think you can’t imagine that if you haven’t experienced it.

After Rehab
After three month of rehab Ariel finally was allowed to go home. Actually, three months of rehab is not such a long time but for Ariel it was definitely long enough and he was relieved from the homesickness. The joy was great, however the return to our everyday life was not easy. Living in
the rehab clinic was like living in a bubble. If Ariel had an outburst there was no one who rolled their eyes or shook their head. Everybody knew that this was a special situation with special circumstances – not so at home. In the rehab I could concentrate completely on Ariel and on my
grieving and processing of the whole situation. I had the possibility to chat with other mothers in similar situations. At home the everyday life overtook me. My surroundings (not my family, but others) expected that I function and deliver normally. Now that we are home everything is back to
normal, and everything is good. The time of disease is finished. This also was clearly Ariel’s opinion.
“I’m at home again, I’m completely healthy again and everything will continue like before I got sick.”

But it wasn’t all well just because we were home. Ariel had to learn this. Again and again he was confronted with his deficits. He was able to walk but was still unstable on his legs. He was able to speak but not clearly. Strangers and other children did not understand him. His responsiveness
was delayed. He had no chance playing tag. And he could not stand to lose. Losing could lead immediately to an outburst. His brothers learned quickly to read the signs and react accordingly. But it was challenging and exhausting for his brothers too. They needed breaks from playing with him.
For a long time, his brothers were the only playmates for Ariel.
Of course, the therapies were much less than in the rehab but they still did determine our schedule. Ariel was not able to go back to his normal kindergarten. He and the teachers were overextended. He was moved to a curative education school (therapeutic pedagogy). This was hard for him, and he could not accept it for quite a while. He wanted to go back to his kindergarten. He wanted life to be as it was before. The phase of denial (when people asked, he said, he was not sick.) was followed by the phase of grieving and depression.

3.5 Year After ANE
Today (3.5 years after ANE), he has arrived in his new life. He has accepted that he sometimes can not do the same things as others do or needs help for certain things where others don’t. He has learned to appreciate his school and likes to go there. (His brothers would like to go there too!). He is
happy again! This is the most important thing to me.
Yes, he still has deficits. Balance, motor function, speech, orientation, mental deficits. You could say a little bit of everything. One tends quickly to treat him like a small boy because of his speech and physical deficits. But he is not that small anymore and his acquired brain injury does not make him stupid. He understands very well. He has acquired a great deal of knowledge about animals and plants while listening to science audio books for children. We can still see him making progress. In the rehab we were told that in the beginning the progress curve is steep and will flatten out with time. The curve is flattened. But it is encouraging to see that even after 3.5 years progress is still taking place.

The last word has not yet been spoken.

 

Week 3: Ariel pulls out the gastric tube himself. He can drink enough thickened fluids that the gastric tube does not need to be replaced. Ariel manages to pull himself into a kneeling position. And to sit up is getting better and better. To grab objects works already quite well with his left hand, with the right hand it is more difficult. Another EEG was done and showed an inconspicuous picture.

Week 4: moved to the rehab clinic. Ariel can sit up very quickly and pull himself into a kneeling position. In the physiotherapy he crawls on all fours. He grabs Duplo Lego bricks but is not yet able to put them together. He manages to pull himself into a standing position for the first time and tries first steps. He rehearses to enter the wheelchair on his own. He can drink with a drinking straw. We don’t need to thicken the fluids anymore.

Week 5: with some help Ariel can walk 8 meters. He speaks some small words again. He learns how do drive and steer the wheelchair.

Week 6: Ariel can walk further and further with help. He can overcome some steps of the stairs. He is throwing a ball for the first time again.

Week 7: Ariel can walk a few steps without help. He speaks more and more words. His view seems to be limited. He suffers from homesickness.

Week 8: While playing soccer with his brothers he stands up on his own, does two to three steps towards the ball and kicks it! Ariel starts with hippotherapy (horse supported physiotherapy). He can walk 8 steps without help.

Week 9: Ariel speaks more and more. He can do shorter distances by foot. We can visit the playground of the rehab without wheelchair. His comment on this: “now I’m healthy again, we can go home”.

Week 10-16: Ariel joins the therapies and makes further progresses. In the meantime, we are planning to leave the rehab and are trying to organise the therapies in our home town.

After 4 months: finally at home!