Acute Disseminated Encephalomyelitis (ADEM) Support Group

James's Story

Prior to his illness, James was an energetic, happy, confident, and very chatty toddler. His immunisations were all up to date, and apart from him contracting chicken pox at 12 months, he had never had any significant health problems. He was fully toilet trained both day and night, and had outgrown his daytime naps 6 months previously. At aged 2 years and 8 months, he came down with a virus, which led to the onset of ADEM. Below is James’s and our story. There is a lot of detail, but please take from it what you can.

On Tuesday 3rd August 2010, James contracted a virus, that left him lethargic and run down. We weren’t concerned as he was behaving as a typical child with a virus would – feverish, tired, and feeling very sad. We did notice that on a night-time, his temperature was incredibly high and he was sweating profusely in his sleep, so much so that we were changing his pyjama top at least once during the night. This continued for a couple of nights. We treated with Calpol during the day to keep his temp down, and let him sleep when he wanted to.

On Thursday 5th August, I got a call at work from my sister, who was looking after James and his brother, Harry at the time, to say James was very unwell. His temperature was high, and he was crying inconsolably. I left work and made a same day appointment with the doctor on my journey home, but when I got home 30 minutes later James had worsened. His temperature was even higher, he had lost control of his bladder, was unable to stop crying, and was presenting a pinprick rash on his right shoulder blade. I called NHS Direct where they went through their standard questions, such as “…is he responsive to you when you call his name…” – “No” was the answer as he was crying so much. “…does he have a pinprick rash anywhere on is body…” –“Yes”. They immediately dispatched a team of paramedics to come and check James over.

The paramedics arrived within 10 minutes and assessed James. Partly due to the fact that he was incredibly agitated and still crying, partly because his heart rate was deemed high, the paramedics called for an ambulance to take him into the local Accident & Emergency department at Good Hope hospital, Sutton Coldfield for further assessment. I wasn’t overly concerned at this stage as the paramedics are trained to deal with stressful situations in a calm manner. Alarms bells only started to ring when the paramedics called the ambulance and asked for “blue lights flashing on arrival”.

Once the ambulance team arrived, we took James into the ambulance, where the fantastic team of paramedics managed to calm him down and get his heart rate lower by buy wowing him with the on-board gadgets. They took us to A&E where we were immediately assessed by a triage nurse who gave James Calpol and ibuprofen. Mark (my husband) arrived within 30 minutes to see James playing in the specially adapted children’s A&E bay without a care in the world. An A&E doctor saw us shortly after, and asked us rather flippantly why we had come to A&E (it wasn’t us, the paramedics made that decision), and when we told her the story starting with us contacting NHS Direct, she rolled her eyes and said NHS Direct sends everyone in and we might as well not bother calling them. W were then packed off with some more ibuprofen and told that James had a virus, was likely to be ill for a few more days and to treat with Calpol and ibuprofen until he was better.

For the next few days, James continued to be okay during the day, but his temperature was spiking during the night (when the medication had worn off). He was also incredibly sleepy and got tired easily, falling asleep watching TV and even in Mark’s arms in church.

On Sunday 8th August, we were visiting family when we noticed that James’s behaviour was bizarre. He was always a very happy, easy to placate child, but on the Sunday, we was being incredibly naughty. Within an hour, we also noticed that his speech began to deteriorate, sounding like he was intoxicated, and his balance was a bit off as well.

We took him back to A&E, where again we were seen very quickly and given Calpol and ibuprofen, and all James’s symptoms disappeared. An A&E doctor again questioned why we had brought him in and told us that he still had a virus and would be okay in a few days.

We took James home, but he just wasn’t right. He lacked concentration, his fine motor skills had started to deteriorate and his speech was getting worse. He was unable to eat his dinner as swallowing seemed difficult and lost control of his bladder at the dinner table. We asked him to pop upstairs to get a change of clothes, and when Mark followed him up, he had fallen asleep (it was only a couple of minutes), this was around 5:30pm. We let him sleep and checked on him throughout the evening. I went up again at around 8:30pm to see he had wet the bed so I tried to rouse him to change his clothes. I stripped him off as he was really hot and laid him in our bed. At this stage I noticed he kept waking up and smiling at me, before drifting back off to sleep. Within a few minutes, he had another accident in our bed and his “waking up and smiling” progressed to him opening his eyes really wide (his pupils were enormous), his smile and become manic, and he was started to foam at the mouth.

Mark called NHS Direct again, who asked their standard questions and sent an ambulance straight out. The ambulance team saw this behaviour and agreed with us that it just wasn’t right (finally someone was listening!!!) and took us back to A&E at Good Hope hospital. Unfortunately, A&E on a Sunday night is a very busy place, and although it was around 9:30 when we arrived, we weren’t seen by a doctor until around 1am – James was asleep the whole time. They decided after a quick assessment that we should be transferred up to the paediatric ward to be further assessed, with a view to spending the night.

Once we got to Harvey Ward, James was assessed in great detail by a Junior Doctor. He was very thorough and asked for full details, before seeking advice from the on-call doctor who came in and asked the same questions again, and some more. Their initial thoughts were meningitis or possibly encephalitis, but the doctor didn’t deem it serious enough to perform an emergency MRI scan (which would have meant a transfer to another hospital and by now, it was a round 2:30am. One thing the doctor kept saying was “do you think his balance is off as it’s such a time of the morning, or because there is something wrong. I couldn’t give an answer, partly due to lack of sleep on my part and partly because I just didn’t know. He decided to take some bloods, insert a cannula into James’s right arm and prescribed IV antibiotics and anti-virals, whilst also requesting 2 hourly obs through what was left of the night before assessing again in the morning. At this point, James was actually quite chipper, laughing with the doctor whilst he was performing various fine motor skills tests on him so I was not overly concerned, though I knew something was wrong, it didn’t dawn on me how serious as he was still “James”.

We awoke in hospital at 8am and as James sat up, I noticed that although he was remarkably bright and breezy considering the lack of sleep and the strange night, he struggled to support himself and his head seemed a bit floppy. His speech also seemed to have deteriorated again. The same on-call doctor had stayed behind after his shift had finished waiting for James to wake up and perform the same set of fine motor skills tests. These involved asking James to perform various tasks such as reaching out and grabbing the doctor’s finger, putting his hands together at his chest (like in prayer), putting his hands together behind his back, and trying to balance on one leg. At this stage we noticed that there had been a change for the worse and James wasn’t able to perform the tasks as well as the previous night.

James got progressively worse throughout the day. The Doctors changed is obs from 2 hourly to hourly, and at around 12:30 a decision was made to give him a head CT scan. For this test, he had to be sedated so was given a drink to help him sleep. Once he had drifted off, we were taken down to the scanning suite and the test was carried out. We were told to expect James to waken after his sedation within an hour or two. After 3 hours, he was still in a very deep sleep and the nurses were getting concerned that we could not rouse him. Mark and I put it down to the fact that he had broken sleep the night before, and he’s a deep sleeper anyway. However, the staff kept insisting that we try to rouse him. Once he did eventually waken. It was apparent that James’s condition had deteriorated rapidly. He lost his neck control so could not support his own head, was unable to walk, couldn’t grasp, had difficulty swallowing, and lost the ability to talk. All of this James seemed to take in his stride, and he really did try to carry on as normal. There was something horrifically comical about him trying to move around his room to explore his surroundings when we literally had to prop him up as he went about it.

At around 7pm the Consultant came in with the results of the CT scan that showed James has swelling to the brain. This was possibly the worst news we have ever received and didn’t know how to process it. Especially as the consultant couldn’t tell us what had caused the swelling or what they could do to treat it. By now James was unable to swallow at all and had been put on IV fluids. We were all really frightened as James was starting to struggle with his breathing also, and we were advised by the staff that he might have to be intubated to help him breathe if he continued to deteriorate. It was definitely the darkest hours for us as we were so upset that we would literally have to wait for him to choke before the staff would help him, but understandably they didn’t want to unnecessarily intubate. We also asked the questions at this point, “How bad could he get? Could he lose his sight, his hearing?” All the answers came back as “we don’t know, but possibly, yes”.

James eventually drifted off to sleep at around 9pm and had an uneventful night, but was still under hourly obs, and was received IV fluids as well as IV antibiotics & antivirals, thankfully he slept through everything.

The next morning, James woke up seeming a little better. He was in a very good mood and was blowing raspberries and laughing, as it was the one thing he worked out he could still do. I recorded this moment and sent the clip to Mark who has spent the night at home with our other son, Harry who was 12 months at the time. Mark was really lifted by receiving the video clip and we resolved to take as many videos as we could to chart James’s progress. James was still unable to swallow and even giving him a yogurt resulted in him almost choking, but at least his was breathing okay so the need to intubate went away – such a relief!

By now it was Tuesday 10th August and the Consultant booked James in for an EEG to monitor his brain activity. This went well and James sat as still as a 2 year old can when the electrodes were being applied. He even allowed his favourite teddy, Tigger to have some electrodes put on his head as well. The results of James’s blood tests had come back and they found traces of a highly contagious virus in his system. Although the virus was almost gone, there was still a big risk to other people, especially pregnant women, so James was placed in quarantine. This wasn’t as drastic as it sounds, but he was confined to his room and any staff that entered had to wear infection control masks, gloves and aprons. He was gaining bladder control again, but due to the quarantine, was given a potty to use in his room instead of being able to go to the bathroom. It was then that we realised James hadn’t opened his bowels since he was admitted on the Sunday night. When we queried this with the staff they put it down to him not eating or moving around very much and told us not to worry. The obs were decreased to 2 hourly by the Tuesday which made James a lot happier, and by the evening he has managed to eat a yogurt and have some apple juice through a straw so he was taken off IV fluids. His IV antibiotics & antivirals were given during the night, so he was able to move around his room freely – all minor achievements but they each made us so happy!

The next few days in the hospital were much the same and we fell into a routine. Though the doctors still had no idea what was wrong with James, they adopted a wait and see approach. Although we were seeing slight improvements every day, such as his walking getting steadier, though he really struggled with his speech and when he was tired, he would give up altogether and just point to what he wanted. We would never let him give up though, and were determined to help him, so we always gently pushed him to say his words. Quarantine was lifted so we had a little more freedom; and James’s walking came back. He was still very unsteady, but he didn’t let it stop him and he would run up the hospital corridor to the playroom at every given opportunity. We felt it very important to introduce some kind of normality to James’s days so we set up a routine for him and us. James was always a child who worked best in a routine so it seemed natural for us to get him into on in the hospital, despite the circumstances.

7 – 8.30 am – James woke and we gave him his morning milk and fruit, followed by cereal. We would then get him washed and dressed before he had some toast from the hospital cart.
8:30 – 12pm – James would spend time in the playroom, and the hospital staff doing their rounds would come and assess him.
12pm – 6pm – James would have lunch, and then his afternoons would be spent playing with his visitors, watching films (we brought the laptop in so that he could watch DVD’s), reading stories etc, followed by dinner at around 5pm. The staff would do their evening rounds and assess James again.
6pm – We would start James’s wind-down for bed routine. He would have a bath, followed by a story and maybe a little bit of TV before settling him down for the night at around 7:30/8pm. Though, due to the fact he wasn’t as active as he would be at home, he wouldn’t drift off sometimes until after 9pm.

Our daily video clips we took to chart James’s progress also formed part of his routine, and the parent that had stayed in the hospital would always do a quick “Good morning” clip to send to the other parent at home. Then the “at home” parent would do a clip of themselves and James’s baby brother, Harry and send it in. It was our boys’ own little way of keeping in touch. It also helped with James’s speech therapy as we would coax James to talk on the videos, even if it was just a “Good morning Mummy/Daddy…”.

One of the downsides of those days was the antibiotics that James was on were rotting his cannulas and his little veins collapsed on an almost daily basis. This meant he had to have a new cannula put in almost every day, which was incredibly traumatic for him and so hard to watch. In total, he had 6 cannulas inserted whilst he was in the hospital. Some Doctors were really good and managed to get it first time, whilst other were not. We didn’t blame them, it must be an incredibly hard skill to master. But we did used to pray that one of the “good” ones would be on shift when it needed to be done again.

On the Friday, a decision was made to send James to Heartlands Hospital (about 10 miles away) for an MRI scan to monitor his brain and try find out what was happening. We went in the ambulance to the hospital, a real event for James who had not been outside for what seemed an eternity. On the journey, he fell asleep and was still fast asleep when we got to the hospital. This seemed to anger the Head Nurse on the Paediatric Ward at Heartlands as he was due to be sedated and she felt it would not work effectively if he had already slept. James is a very deep sleeper, but when the Nurse assessed him, his heart rate had dropped dramatically and she was concerned that he wouldn’t be able to cope with the sedation so she suggested performing the MRI whilst he was still asleep. Mark and I disputed this and felt if he woke up whilst in the machine he would be terrified, but the Nurse insisted. Our boy must’ve known what she was up to because as she lifted him to carry him into the machine he woke up and started crying. A decision was made to leave James to wake up for 20 minutes, assess his heart rate again and decide whether he would cope with the sedation. I did everything I could to rouse James so that they would be able to carry out the MRI. The thought of having to go through it all again the next day was too much, so I was determined he was having that test! I tried tickling him, singing and dancing with him, anything I could think of! When his heart rate was checked again, sure enough it was back to normal so he was given the sedation. It usually takes around 45 minutes to take effect, and in James it took longer but given what we’d been up to it was no surprise.

After the MRI was carried out, we were transferred back to the ward where were again encouraged to wake James from the sedation as quickly as possible. We were very worried about how he would be, as after we roused him from his sedation after the CT scan, he was very poorly, but that was the illness in its most acute phase. When James woke up, he was okay and had some toast and juice, before the ambulance arrived to take us back to Good Hope. The journey back was bittersweet. James was glad to be going back to “his hospital” as he called it, he had become used to it so quickly. I could see from the back of the ambulance that the route we took also took us past our house. I sat there silently crying that this was the closest James had been to home in what felt like an eternity, and wondered if he would ever be there again.

That night, the results of the MRI came back with a diagnosis – James had “Acute disseminated encephalomyelitis (ADEM)”. When he contracted the virus a couple of weeks previously, his body created anti-bodies to fight the virus, and these anti-bodies attacked his central nervous system. Specifically, it attacked his myelin sheath which is the coating that protects the “body’s internal wiring” that sends messages from his brain to the rest of his body. Due to this wiring being broken down, his brain was sending signals but they weren’t reaching their destination as efficiently, hence him losing his balance, speech, etc. The Doctor who explained it likened it to the plastic coating on an electrical wire, and if this is damaged, the electricity doesn’t travel down the wire.

Most importantly, with a diagnosis came a treatment in the form of IV corticosteroids which he was put on straight away, and the IV antibiotics & antivirals stopped. We were advised that James would have a good chance of recovery, though the Doctors were reluctant to say if he would ever be 100% again. When we “googled” ADEM, we were incredibly upset with what we initially found, and some comments on Wikepedia such as these really upset us, “…full recovery is seen in 50 to 75% of cases, while up to 70 to 90% recover with some minor residual disability. The average time to recover is one to six months….”. We were struggling to accept that James “might” never fully recover. We also read that there were some connections between ADEM and MS, and were worried that this was in fact, only the start of James’s problems. The doctors arranged for us to see an Paediatric Neurological Specialist at Birmingham Children’s Hospital as soon as possible, so that she could explain in more detail what ADEM was, as well as the recovery. This Specialist had been involved in James’s assessment from the beginning, so already had a full understanding of his case.

On Saturday 14th August, it was decided that one final test needed to be carried out and that was a Lumbar Puncture. We were terrified of this test, as it involved placing and restraining James in the foetal position whilst a needle was placed into his spine to extract fluid. The Doctors had talked about carrying this test out for a few days, but as they told us it would not provide a diagnosis, as parents we refused. Unfortunately they had to carry out the test at this stage to confirm what the MRI had shown so we reluctantly agreed. We took James down to the treatment room, the torture chamber Mark and I silently named it. I sat James down and explained what was going to happen, whereupon he got understandably very upset. It was important to me that I told him what was about to happen. As his mother, James trusts me and I felt by not saying anything, I would betray his trust. It took 5 staff and myself to restrain James and keep him still enough for the test, which took about 5 minutes. It was physically hard, but emotionally horrific. To assist in my child going through such a procedure was awful and I was racked with guilt, but had been assured by the Staff that it was absolutely necessary. Thankfully, once the procedure was over, we went back to James’s room, had lots of cuddles and some chocolate, and we found his smile again.

It was also on the Saturday that James opened his bowels for the first time in a week. We had been keeping the doctors informed on their twice daily rounds that he had not done anything, and we were concerned that his illness had affected him in this way as well. It made sense to us, as he lost control of so many other movements, we wondered if affected in this was as well. The doctors kept saying we were wrong put it down to the fact he was not moving around as much as normal, but as parents, we were less sure. Finally the day before, he was prescribed a powerful laxative to help him go. Understandably James was really scared of going, and when he felt the need, he tried to hold back. We had a little chat and decided to put a nappy on him to go, instead of him going to the toilet so that he could do it without too much bother. This worked and within about 20 minutes he had done it! I was so happy and relieved that I called a nurse in to show her. James went another couple of times that afternoon, the last being on the toilet so we were happy that things had got going again. I was still convinced that this was connected to his illness, and that his sphincter had been affected, but as he had started on the treatment for ADEM, it became less of a priority.

On the Sunday morning, the Doctor said that as we lived so close to the hospital, we could take James home for a couple of hours in the afternoon. We were so excited and couldn’t believe how far he had come in just a week! We had been to hell and back, and were still on a very long road to recovery, but to be able to take him home made us so happy! We took him home, where he and his baby brother had a very emotional reunion, followed by completely forgetting they had not seen each other for a week and they set about the very serious business of playing. It was the most amazing sight, and we all cried tears of joy. Unfortunately, James’s cannula had slipped out and his arm started bleeding, so we had to go back into the hospital to have it put back in. The nurses, knowing we were keen to get him home again, did a quick repair job and told us that they would put a new one in when we came back to the ward later. It was during this trip home that James got upset to be leaving “his hospital” as he had become institutionalised so quickly. We think it was partly to do with the fact he had the 100% attention of mummy and daddy, as well as the medical staff, and all the visitors. Not to mention the endless supply of presents that our friends and family kindly sent in for him. Whilst at home, he felt things were “normal” with no special treatment. He was really excited after we gave him and Harry a bath together, put Harry to bed, and brought James back into the hospital. Though it just gave us another thing to worry about… how would he cope when he eventually went home for good.

The next day was Monday 16th August, and we asked the doctor on his morning rounds if we could take James out for a walk and possibly pop into his nursery to see his friends. The doctor agreed, as James wasn’t contagious and said we could do so for a couple of hours. Mark took him out in the pushchair, first to the park across the road from the hospital, and then to his nursery which fortunately was very close by. His carers at nursery were delighted to see him, and made a huge fuss of him, though they were all visibly shaken at the change in James. He was walking steadily by this point, though he was quite weak, but his speech was still incredibly slow and pronounced. James was really pleased to see his friends, and Mark promised to take him back to the nursery the next day so he could have another play. All in all, it was a really successful visit, and it helped us to realise that James would adapt to whatever situation he was put in, as hard as it may seem to us.

By Wednesday 18th August, James had finished his course of IV steroids, and we were given the news we thought we would never hear – we could take him home!! It had been a long 11 days in hospital, and we’d all been to hell and back, but he was coming home and that was all we cared about!

We were packed off with his discharge notes, a batch of oral corticosteroids the he was to take far another 10 days, and the promise of a call to arrange an appointment with the Paediatric Neurologist at Birmingham Children’s Hospital who had been on annual leave for the previous few days. We had only been home an hour when we got the call, asking us if we could get to BCH for our an emergency appointment in the next two hours. The Consultant was back and was really keen to see James, so off we went. The Neurologist was brilliant, showing us pictures of James’s MRI scan and pointing out the legions clearly visible in his brain. She put our minds at rest regarding MS and told us that James had “textbook” ADEM, not MS as was often confused in years previously. She was also really pleased with his progress and told us to keep up the good work. Her estimated timescales for recovery were about 12 months, though she could not guarantee 100% recovery, she was very happy with his amazing progress so far. Though we were really keen to start James with some speech therapy, she felt at that stage there was no need as it was still really early days in his recovery and she encouraged us to adopt a wait and see approach.

From then, we tried as much as possible to get back to some level of normality. We took James and Harry out every day, sometimes to the park, others to play centres, or even just a trip to the local library. It really dawned on us when we got home just how hard his long term recovery was going to be. Our once confident, happy little man had become withdrawn and shy. He refused to leave my side for a moment, and was jealous of any attention I gave to his brother. This was very hard, and we mourned for the boy that James once was, whilst feeling guilty that we were lucky enough to have James home from hospital and recovering. To try and help, I joined some online support groups aimed at sufferers and carers of those affected by Encephalitis. I was always a silent member, not telling our story as it was still very raw. But it was an enormous help to read other people’s stories, and see how they were coping. I looked for similar situations to what we dealt with, sometimes there would be a child James’s age affected, or someone had suffered with ADEM. Here were people feeling the same pain we were, and in a way it helped. Though at times, it made me feel worse as their stories were more horrific and their long term prognosis wasn’t as positive.

On Monday 23rd August, just two weeks after waking up in hospital for the first time, James started back at nursery. It was really important to us that we tried to get James’s life back on track and this was another step towards his recovery. The nursery staff were fantastic and all had training in dealing with children with Special Education Needs. The Nursery also sought the advice if their SENCO representative to ensure they were doing the right things by James, but the only adapting they had to do was to ensure they always took the time to listen to James s his speech was still slow and he really struggled if he felt under pressure. The staff, as well as ourselves vowed to make a huge effort not to finish James’s words/sentences for him and to let him get his point across no matter how long it took.

We had tremendous help from our amazing friends and wonderful family, especially my mum who flew over from her home in Ireland the day James was admitted to hospital and stayed until after he was discharged, often becoming the third parent we needed to get us through those 11 days. We had constant messages of support and an endless stream of visitors to the hospital, as well as countless kind gestures, and many prayers said on our behalf.

The following weeks and months passed by sometimes quickly, sometimes painfully slowly. We often looked back to James’s lowest points and looked at the video clips we took of his progress to see how far we had come. Though at times, it felt like we were struggling. His moods were affected in the initial weeks and months, and he often had tantrums bore out of pure frustration at his own lack of ability. The steroids altered his moods as well as his physical appearance, his face was rounder and he gained a lot of weight initially. But slowly, James started to come back. He lost his puppy fat again, his face shape returned to normal and most importantly, his confidence returned.

We followed up his speech problems with an assessment with the local speech therapist, but by the time his appointment came through in February 2011 his speech was 95% back to normal. In fact, it was almost embarrassing when we walked into the assessment and the first thing James said was, “Mummy, I’ve been a good boy sitting still so now can I have my cereal bar”. I am sure she thought we were just over-protective parents, but his recovery was that fast! James still stammers now if he is worked up or feels he can’t get his point across, but this is a very small price to pay considering how things could have been.

James had a second MRI in December 2010, 4 months after his illness and the results were very promising. The legions in his brain had almost gone, and his recovery was amazing. James is now part of a Research Project studying the effect of Demyelinating illnesses, which involves a twice yearly visit to Birmingham Children’s Hospital for a catch up with the Paediatric Neurologist. At his last check up we highlighted that James often has blank spells where he goes into himself for about 15 seconds before snapping out of it. Due to this he has had a second EEG to monitor brain activity and see if there is any link with epilepsy. We are still waiting for the results, but are sure that whatever the outcome, James will battle through in his own way.

What a wonderful post, thanks so much for sharing mrs. M.

It's fantastic and I know many others will benefit!