James and Kara's Family Blog

2007-02-15T20:08:00.000+11:00

Here the text from a Speech that Kara is giving at a Starlight Foundation function about Amelia's illness and her wish:

Hello everyone. I am delighted to have the opportunity to share with you tonight how the Starlight Foundation has brightened our family's lives. I would like first to tell you a little about how our daughter became ill so that you can hopefully appreciate just how good the Starlight Foundation has been for us.

When our eldest daughter Amelia was 3 ½ and our second daughter Emma was 14 months, James and I found ourselves enjoying the quintessential happy family.

6 months later, when Amelia was 4, the lightness of being we'd thus far enjoyed seemed to have left us. A cloud had decended on Amelia and our whole family.

We stopped going out because the chances were Amelia would become so angry about some insignificant thing that she would explode and the outing would bring more stress than it was worth.

She was suffering but we didn't know why.

We despaired at not understanding why our happy little girl had become so dark and angry. In retrospect, it must have been terrifying for Amelia to have been witnessing on some level what was going on in her body, yet not being able to articulate it or understand it.

When Amelia was 4 ½ and our third daughter was just 4 days old we realised that Amelia was very sick. She got out of bed that morning and couldn't walk straight. She bounced off walls, her head wobbled, she was drooling, her hands shook and her speech was slurred.

She was hospitalised and the tests began straight away.

It was so sad to see her subjected to a barage of frightening, invasive tests : CT scans, general anesthetics, Mri's, an EEG, a lumbar puncture, ultrasounds and loads of blood tests.

Even sadder though was the look of hurt and shame on her face when the doctors spoke about her as if she wasn't in the room. The doctors had to ask their questions, they didn't know what was wrong. Amelia had neurological symptoms, but a cat scan had shown no tumor on her brain.

It turned out that Amelia had a neuroblastoma on her adrenal gland, which was surgically removed. She was also found to have a rare auto-immune condition called OMS, which affects about 1 in 10 million children each year. Her immune system had been attacking her tumor but got confused and started attacking her brain as well, hence the neurological symptoms.

Being such a rare condition, we poured our energy into researching the illness and its treatment. The doctors didn't seem to know what treatment to follow. Even though we found that most children with OMS in the US were treated a certain way, our first doctor wasn't prepared to give Amelia that treatement because it was too costly. So we looked for a doctor who would.

It was really draining having to ask for things all the time. I often wished that I could just trust the doctors to know what to do for Amelia, but I wouldn't. The medical system offers all kinds of wonderful support for patients, but my experience was that unless I asked for it, it wasn't forthcoming.

The treatment of Amelia's OMS was immune suppression with very high dose steroids. This mediacation caused her to double her weight in a few months. She also had to go to hospital every month for a 10 hour infusion, which made her very sick. And all the while we were trying to care for our other two children and keep some normalcy to our lives.

We heard about the Starlight Foundation's wish-granting work through a family in the US at our on-line OMS support group. James suggested we apply. I remember when we received the notice that Amelia would be given a wish, the letter said that we would also go onto the Starlight Escapes data base and would be invited to activities for the next couple of years. Oo, this is a bit of a bonus, I said to James.

What a breath of fresh air Starlight Escapes was. Neddless-to-say, I was too distracted to think about organising family outings at that time of our lives. But these letters would turn up inviting us to an escape. Just RSVP and turn up. I could manage that. I remember saying to James “isn't it nice to have something good like this just fall into our laps right now” when an Escapes inviatation turned up one day.

It wasn't long before Amelia came to recognise the Starlight logo on the envelope and would jump up and down excitedly about the prospect of another escape when a letter arrived.

The escapes programme has given us the opportunity to go to Boy from Oz, Walking with Dinasaurs, movies, a christmas concert by the Brandenburg Orchestra, a behind-the-scenes show with the Australian Ballet at the Opera House, a tour of one of the navy's submarines and more.

The escapes have given the whole family the chance to go out and do some fun things together.

Also, very importantly, they have given Amelia the opportunity to meet with a range of friendly, supportive people, which has been an important part of restoring her ability to go out and be in the world. You see she had, understandably, become such a fearful child.

When we told Amelia that she was being given a wish, initially she wished for a sleepover at her grandparent's house. Then she wished for a bike, but we told her to try to wish for something that we could never give her without the help of the Starlight Foundation.

She talked about it with her art teacher. Amelia had started private art classes as a kind of therapy to help her deal with the baggage of her illness and treatement. Her teacher suggested she wish to have her work hung in the NSW gallery. Amelia thought this was a great idea and that it would be fun to work with a 'proper' artist to create the work for her exhibition.

Working with Sydney artist Janet Laurence was a most beautiful experience. Amelia's visits to Janet's studio were always special, creative and uplifting. It was lovely for James and I to see Amelia slowly coming out of her shell a little more at a time. It was such a positive experience for Amelia to be able to create art, express herself and enjoy herself with Janet and her dog Wax in Janet's space.

It was wonderful for Amelia to have a wish that took many months to be fully realised. She had the opportunity to develop a special relationship with Max, her wish granter, and with Janet and her dog Wax. By the time the exhibition day came around, 2 ½ years after Amelia was diagnosed, Amelia had finished her monthly infusions and was weaned off nearly all her medication.

The day of Amelia's exhibition nobody could quite believe how Amelia was able to rise to the occasion and absolutely relish having such a large group of her friends and family there for her. It was an extremely affirming day for Amelia.

Max, her wish-granter thought of all kinds of details to make Amelia feel like a princess on her special day. She had her hair done in the morning, she had a beautiful new dress with layers and layers of pink tuelle, a limo took the family to and from the gallery, her work looked superb hanging on the wall of the NSW Gallery, the gallery provided refreshments for Amelia's guests, and she had posters of her work which she signed and gave out to her friends.

The day was a real celebration of what Amelia has achieved.

We are so grateful to the Starlight Foundatin for the immeasurable ways in which they have contributed to Amelia's healing, and for just giving the whole family a good time when we really needed it.

Weaning off IVIG

2006-04-17T21:35:00.000+10:00

Amelia had her last IVIG about 6 weeks ago now and we are hoping that she won't need anymore. She has been having IVIG every month for nearly two years now, so it is a great relief to think that it might end soon.

The last month had been the best since she got sick. Amelia was totally normal behaviourly for almost a whole month. Her body is slowly but surely recovering from the steriods, she is losing about a kilogram per month and she seems to be getting her muscle tone back. Our hairdresser even said that she could see a mark in Amelia's hair from where she stopped taking the prednisone. Her coordination is much better now, today she learned how to ride her bicycle without training wheels.

Unfortunately she has deteriated a bit over the last few days. She has been having more and more periods of mania where she is extremely happy, bubbly, chatty and gradually more crazy until she gets repremanded and then she suddenly becomes depressed and tearful. She was so bad tonight, with two or three manic cycles, and at the end of the last one she actually said that was so sad that she wanted to die. This was a terrible shock for Kara and I to hear. We had just been getting used to her being normal and now she seems to be going backwards.

We are just starting school holidays which is often an unsettled time for Amelia anyway. We also started giving her fish oil a few days ago which may be confusing things even more. The plan for now is to stop the fish oil and hope she settles down. If the behaviour continues we might have to get another IVIG infusion, but Amelia hates the procedure and since she has been told that she won't need another, it will really upset her to have to go back to the hospital.

Irritability, sleep and Clonidine

2006-01-18T22:55:00.000+11:00

Milly can be very irritable at the moment. She has good and bad days. Today she was quite normal, but yesterday she was really bad. We had our monthly apointment with our neurologist yesterday and we went to the hospital cafeteria for dinner after. Amelia had been very irritable most of the day and became increasingly uncooperative during the visit and in the cafeteria. She was constantly verbally attacking her sister Emma, complaining about everything and making unreasonable requests for junk food. She got so bad during the meal that I told her that I would take her straight home when she finished eating unless she stopped whining. She kept on whining so I committed to taking her straight home, as per my threat, and letting Kara and her sisters have a bit of peace while they finished their meals. I had driven to the hospital separately so when she finished eating I dragged her away from Kara, Emma and Sophie and headed for my car. She screamed at the top of her lungs all the way through the hospital, down the street, into the car and then for the next ten minutes as we drove along. I had to carry her half the way through the hospital (she ways 38 kg now), drag her down the street and force her into the car. I was expecting some do-gooder or police officer to stop me at any moment!

At least we have not seen any physical OMS signs since we weaned prednisone, so we are going to wait and see if the behaviour calms down. She has been irritable like this at various times over the last year and we can't tell how the IVIG or prednisone makes any difference.

We got some Clonidine yesterday and it seems to be helping a little bit, but she has only taken it twice so far (25micrograms at a time), so we can't really tell. At least today, the first day after we started taking it has been her best day in weeks and she went to sleep easily tonight.

Weaned off Prednisone

2006-01-14T10:57:00.000+11:00

Well, it has been a long time between posts.

Not a great deal has changed since last post. Amelia's condition has been relatively stable, but we are still struggling with sleep and behavioural issues.

The big news is that Amelia has just been weaned off the prednisone. She has not had any for a few days now. We have been weaning for a few months now and her behaviour has been a bit unstable throughout the process, but it did not change significantly as the dose got lower. We had worried that we would hit a dosage limit where the OMS symptoms would come back strongly, but it didn't happen. Huge relief.

Most of the time she is a normal girl, but there are still a few 'OMS' moments. In particular she usually has trouble going to sleep and every now and again she gets the 'black cloud' over her and she is terribly irritable for a few hours. She is a very anxious child, but we don't know if that is steroidal, OMS or just her nature.

After reading the latest Pranzatelli research, We are trying to get some trazadone to help the sleep problems, but it is not generally available in Australia, so we are fighting a bit of a battle to get it. It is tempting to just buy it over the internet, but for now we are relying on our neurologist to get a special order through the hospital pharmacy. I wish we had trazadone two years ago!

Our plan now is to wait for Amelia to stabilise and then work on reducing the IVIG.

2004-11-10T19:45:00.000+11:00

It has been a long time between posts. Fortunately this has been because there has not been much to report. There have been a couple of minor wobbles, but overall Amelia is doing very well. She is now on 26mg Prednisolone every other day and there are no OMS signs most of the time. In fact the only sign that there is anything wrong is her incredible weight gain. She how weighs 31kg and she has very chubby cheeks.

She had a little relapse for a couple of days last weekend and we got lots of tantrums and a few other more subtle symptoms. We think she had a virus which must have stimulated her immune system and made the OMS flare up. Because of the relapse we are pausing tapering the Prednisolone for a couple of weeks.

The IVIG has been continuing every month at Westmead and has been going very smoothly.

Weaning

2004-09-26T08:23:00.000+10:00

I am very happy to report that Amelia has progressed very well over the last couple of weeks, to the point where we hardly see OMS symptoms any more. In particular, she is experiencing a lot less irritability. We are now going to try to reduce her steroids as fast as we dare. We initially reduced by 5mg and then by 2.5mg about once a month, but that knocked her around too much so we are now reducing by 1mg once a week. Overall, we have gone from 40mg to 29mg per day over about 3 months. The 1mg reduction seems to be working as the last reduction, a few days ago, didn't seem to have any effect on Amelia's behaviour like the earlier, larger attempts which made her behaviour monstrous for a few days.

One of the reasons that we are so keen to reduce the steriods is that Amelia is getting more and more 'cushingoid'. She now weighs in at about 28kg and she is still putting on 500 grams per week.

Wikipedia update

2004-09-26T08:16:00.000+10:00

I have setup an area on the Opsoclonus Myoclonus Wikipedia page for doctors information. I wasn't sure if I should do this, but many people inthe OMSFamilies group encouraged me, so it is done. The page is attracting a lot of interest and if you look at its history you can see there have been quite a lot of people editing it now.

Note, for anyone who is a 'slashdot' reader, you might want to look at my slashdot post.
This post created a lot of interest in the Wikipedia page and about half of the edits happened after the slashdot post.

IVIG at Westmead

2004-09-19T07:22:00.000+10:00

Amelia had her third infustion of IVIG (Sandoglobulin) on Thursday 8th September. This was her first treatment at Westmead and it went very well. It seems that things are done quite differently at Westmead to what we have experienced at Randwich, and it was a welcome change. Everything went to plan, so we got in at a reasonable hour and got home before bedtime too.

The treatment did not seem to have much of an effect on Amelia. In the week before, she had been very well and had almost no tantrums or 'OMS'ish behaviour, but in the week after she had quite a bad run. We think that the week was bad because she had two very short nights sleep and we also reduced the prednisone from 32.5mg to 30mg on Sunday 12th. Because the IVIG did not seem to have an effect, we are hoping that the OMS has gone into remission and the behavioural issues we see now are due to the prednisone. Most of Amelia's tantrums and outbursts are triggered by lack of food (or just the thought of it!) and they are made worse by lack of sleep, but every now and again she gets irritable for no apparent reason, so we are still not sure what is going on.

The prednisone is certainly having a big impact on Amelia's body. She weighs about 26.5 kg now and she has a typically round, cherub like Cushingoid face. We looked at some photos of her from a year ago when she was underweight (at about 17kg) and we nearly didn't recognise her. Apart from the weight gain, there are various other steriod side effects that could be happening in Amelia's body, so we are very keen to ween her as soon as we can.

Amelia's social behaviour has been improving a lot over the last few weeks. She is generally much less withdrawn and she is continuing to attend preschool normally. Her teacher, Chandini, reported to us yesterday that Amelia is quite normal in class now. She is doing her 'work' well and moving from task to task normally. Amelia is telling us more about the other kids in class too, and we think this is because she is having more to do with them. We had some friends over a week ago and Amelia really enjoyed their company. One or two weeks earlier she would have hid away in her room.

Melatonin is bad

2004-09-07T21:34:00.000+10:00

The last few days have been the best for a long time. I don't think we have seen Amelia this happy and 'normal' for nearly a year. She hasn't even been sent to her room for a few days.

The contrast in behaviour between the last few days and the end of last week has made the current behaviour seem especially good. Last Thursday and Friday were two of the worst days we have had since Amelia was in hospital. As you can imagine, Kara and I were very depressed at the time because it seemed that the gradual improvements that we had seen since the treatment started were all lost. Amelia was irritable the whole time and constantly throwing tantrums that would last for up to an hour. At one stage she got so angry at Kara for some trivial reason that she was saying the most hurtful things she could imagine. She doesn't know any 'bad' language, but she still managed to upset Kara terribly.

The relapse co-incided with us giving Amelia 3mg of melatonin at night to help her sleep. For the previous week Amelia had been going to bed two hours later then usual and she was tired and getting more irritable, so our neurologist prescribed melatonin. The melatonin certainly made Amelia sleep better (in fact she was still sleepy the next day), but after the behavioural problems started, we were suspicious that the melatonin was making the OMS worse, so we posted a question to the OMSFamilies yahoo group and did a Google search. The results were startling. Melatonin is BAD for OMS. Lisa W. from the OMSFamlies group warned us about it and the warnings we found at various sites specifically stated that melatonin should not be given to children, people with an auto-immune disease or people who are taking cortico-steriods. I can't believe our doctor prescribed it! It just goes to show that OMS is so rare and obscure that we have to check everything ourselves.

The day after we stopped giving Amelia the melatonin she improved, and she has just got better and better since. She is interacting more with her peers at preschool, she is talking in more complex structures and a lot less withdrawn. We will have our third IVIG infusion on Thursday at Westmead, so it will be interesting to see if she improves more after that. Her speech is still a little mumbled sometimes and there are occational outbursts of irritable behaviour that have an OMS edge to them.

Amelia is sleeping better now, even though we have stopped the melatonin, so perhaps it did help set a good sleep pattern at least.

MRI

2004-08-31T19:14:00.000+10:00

Amelia had her first check up MRI at Randwick on Tuesday 31st August. She needed a general anaesthetic, but the whole procedure went very well and amazingly, it was quite a fun day for Amelia and me. We didn't have a pre-medication because of problems in the past, but the gas wasn't too bad without it.

We had originally been scheduled for a CT scan, but on advice from Amelia's grandfather, John, we had it changed to an MRI (we have since discovered that they don't use CT scans at all at Westmead).

The great news is that the scan was all clear. We were expecting good news, but we were greatly relieved to hear it anyway. We know that Amelia's prognosis is very good, but there is still a small chance that the cancer will reappear so this first follow up scan was a bit nerve racking.

IVIG and colds

2004-08-14T10:27:00.000+10:00

Amelia got her second infusion of IVIG on Thursday 12th. Medically it went very well, it was done over 6 hours (as opposed to 12 hours for the first one) and there were no complications. Organisationally it went quite badly, Amelia and I arrived at 9:30am and we didn't get the infusion started until 5:00pm which meant that we finally got home at 1:00am! Amelia didn't sleep at all, but fortunately she wasn't irritable and so it wasn't too bad. A few weeks earlier this sort of a day would have produced multiple screaming tantrums.

We were in the neurology ward again, and there was the usual array of depressing cases. For the first time I noticed that Amelia got upset by seeing one of the other patients. There was a boy with a tracheo(sp?) in his neck that made metalic hissing sounds when he tried to talk and this really freaked Amelia out. My Aunt, who we haven't seen for a while has a permanent tracheo, so we will have to prepare her before their next meeting. Being is a shared ward of six beds, we overheard a 14 year old girl next to us tell a social worker her life story, which was interesting and luckily she was only in for a broken bone. I hope I never meet her again, I would know too much about her to be comfortable!

Amelia, Emma and I all got terrible colds in the days after the IVIG. Initially, we didn't think Amelia got it, because she didn't show any typical cold symptoms, but her OMS symptoms (irritability and sleeping problems) flared up quite badly, and we think it was probably the virus doing it. A relapse due to a virus is quite typical of OMS. Hopefully when the virus goes, the OMS symptoms will too. This is one of the worse colds I have had for years, I have had two days in bed and after seven days I still feel fatigued. Such bad timing!

Westmead

2004-08-05T21:06:00.000+10:00

The last week was a rollercoaster ride for the OMS. The first three days after the IVIG were amazing. Amelia was totally back to normal. No irritability, tantrums or slow speech and she was a happy, normal four year old! We had forgotten what she was like because she hadn't been like that for about 8 months, she was singing and dancing around the house and interacting with people gregariously. Unfortunately, this only lasted 3 days. On Friday she reverted back to her irritable, withdrawn state. This may have been due to reducing her prednisolone from 40 to 35mg/day on Thursday. We had forgotten what a happy child Amelia used to be and having her back for only 3 days just reminded us how much we have all lost to OMS.

The last couple of days have been relatively good though, and we are hoping that things are settling down after the 5mg/day prednisolone dosage change. Today was probably the best day she has had other than the few days after the IVIG.

Today we went to Westmead hospital and saw our new neurologist, Dr. Jayne Antony. We were extremely impressed with her and are very happy to be seeing her (in a strange coincidence, we established that her father was born in the city where Dr. Pranzatelli works). She presented us with a treatment plan which involves IVIG every month for the next six months with very gradual reductions in the steroids. This is the treatment we have wanted for a while, so we feel a big sense of relief that we don't have to chase after it anymore.

We got the speech pathologist's report this week and the summary was:

Milly presents with ataxic dysarthria that is interfering with her speech intelligibility. Reports suggest that she has made significant gains since her treatment has commenced, but the situation is not yet stable. Her language skills were assessed as being average for her age and do not appear to have been affected by her condition.

So the good news is that her language skills have not been affected.

Meanwhile Amelia is slowly getting back to preschool. Kara left her there for over an hour today and it went very well. Unless she has another relapse, we think she will be back into a normal Montessori preschool routine in a week or two. Wow.

Check out the new Wikipedia page for OMS that we have written. Please change and update it as required!

Sandoglobulin

2004-07-28T21:20:00.000+10:00

Amelia had her first infusion of IVIG (Sandoglobulin) on Monday. We had been pushing our neurologist to get this for a few weeks now and so we were pleased to finally get it.

The infustion was uneventful. The worst moment was getting the canula put in and despite Amelia's tears of protest, thanks to the Emla patch she didn't feel a thing (we love Emla). After the last few needles when we used Emla patches, Amelia knows that she won't feel anything, but she still protests and has to be physically restrained.

The great news is that Amelia has been quite normal ever since the infusion (two days so far). Previously she was quite withdrawn and shy and as you would see from the previous posts, she had daily bouts of intense irritability. We think that the previous treatment, consisting of the tumour resection followed by 500mg/day IV Methyl Prednisolone for 5 days and then oral (40 mg/day) Prednisolone, fixed the ataxia and other movement problems, but it didn't seem to improve her behaviour much, so we are very excited to see the IVIG improving things further.

We are watching her like a hawk now, nervously expecting a tremor or tantrum, but so far so good.

We also saw a Speech Pathologist, Linda, on Monday morning. The consultation went well, especially since Amelia took a liking to Linda and was very cooperative. We haven't see the report yet, but the early signs are that she is doing the correct things for her age, but there are a few sounds that she is having problems with that don't normally trouble a girl of her age (4 years 9 months). We will be seeing Linda weekly now, so it will be very interesting to see if the articulation problems have gone away since the IVIG.

We now in the process of having our neurologist changed. Our current one says that he is too busy because he is trying to go part time from 5 days to 3 (and I think we have been difficult patients because we have been very demanding!), so he has referred us to someone else. Our new neurologist is the head of the pediatric neurology department in a different hospital (Westmead) and she has a 'special interest' in OMS, so we feel like we will still be in good hands, if not better, since even though our last neurologist made a fast and accurate diagnosis and had seen a few OMS patients, his 'special interest' is movement disorders rather than OMS.

Meanwhile we are slowly reintroducing Amelia to her preschool. Kara is going along with her and staying in class at the moment and we hope to phase that out over the next week or two (it depends on how irritable she is). She is still very shy, but we are hoping it is mainly because she hasn't been attending for a few months.

Back to school

2004-07-21T07:07:00.000+10:00

Yesterday was a highlight in Amelia's recovery. Amelia went back to preschool successfully for the first time since the diagnosis. We had planned this for a while and had been to the school with Amelia to meet the teachers the day before.

It was a short visit, but a very positive one. Amelia interacted with her peers quite normally and really seemed to enjoy herself. Her friends, including her great friend Ben, were very happy to see her and Amelia was keen to interact. This is a marked change from the withdrawn behaviour we have seen recently.

The preschool's systematic application of the Montessori system showed its value when the teachers used their reports of Amelia's progress to show that she has not gone backwards. Her letter and number work picked up from where she left off which was a huge relief. We will try to use the reports to help the speech pathologist and the neuropsychologist.

There are still some OMS symptoms present. Amelia's speach is now adequate and she communicates well, but it is still a bit slow and badly articulated. We will see the speech pathologist on Monday to get a proper assessment. The tantrums are still a problem, but they have got better over the last few days. There didn't seem to be any improvement for about a week, but it looks like she might be getting better again now.

Amelia is now up to 23.5 kg and looking more and more cushingiod. We have a good diet now, and even I am losing weight, but I think the steroids are winning the battle at the moment!

Steady improvements

2004-07-14T20:53:00.000+10:00

The last week has been marked by very positive consultations with our oncologist and neurologist.

We had a meeting with our neurologist on Monday in which we had anticipated that we would set a date for the IVIG treatment, but after considering Amelia's progress we agreed that we would hold off the additional treatment for another week. Amelia has regained all of her coordination now, with no sign of opsoclonus, myoclonus or ataxia at all. The tantrums and bad sleep are still a problem, but the tantrums have become shorter and fewer. We are hoping that at this rate she will not need the IVIG at all, but if the symptoms don't continue to abate, we will start it.

We got the tumour pathology results from Amelia's oncologist yesterday and they were, as expected, good. There was no N-myc amplification (only one copy) which means the neuroblastoma cells are less agressive and hence there will be no need for any more treatment for the neuroblastoma. Unfortunately, there is no guarantee that the the cancer is completely gone so we will be booked in for a CT scan and an mIBG scan every three months for a while.

We have been working on Amelia's diet in an attempt to control her weight. When Amelia started the steriods she developed a very strong appetite and her weight went from about 21kg to 23 kg in a couple of weeks. We were told to try to remove as much sugar from her diet as possible to control the typical Cushingoid (named after Dr Harvey Cushing) weight gain associated with steroid use and we decided to take a Low GI food diet approach. This seems to be working well. She hasn't put on any weight over the last week. Amelia asks for high GI foods, but if they're not available she often won't eat anything, which makes me think that she's not really hungry, but just craving sugar.

Visitors from abroad

2004-07-08T07:01:00.000+10:00

Yesterday we had the pleasure of hosting the Wright family from LA at our house. Those of you who frequent the OMSFamilies yahoo group would know the Wright family from their long time active membership of the community and the friendly caring advice they have given over the years. Milly, Emma and I picked up Ellie, Jim, Jessie and Katarina from Woloomooloo in the late morning and after a brief stop at the fish markets to stock up on king prawns, we brought them back to our home for a BBQ. We had a rather eclectic mix of food including sausages, a dish of antipasto and a mystery bottle of wine (which turned out to be a very enjoyable sparkling red - thanks Lynette!).

Amelia was a extremely withdrawn at first and quite shy and retiring for most of the visit, but the Wright children managed to draw her out enough so that Kara and I had lots of time to chat with Ellie and Jim. Both Jessie and Katarina were great with our younger two, Jessie was fantastic with Emma and Katarina loved Sophie.

We found a lot to talk about and the adult conversation ranged over various interesting topics, but we always came back to OMS. Katarina has had the condition for many years now, so we had a lot to learn from their experience. As well as confirming many of the things we have been reading about, some of the points to note are:

Surrounding Amelia with pillows and soft toys might help her sleep by making her more secure when she wakes up (we tried it last night and she did sleep well).

IVIG has very minimal side effects and we should try it as soon as we can

Dr Mitchell says that every OMS relapse costs 5 IQ points

After such an enjoyable visit, it was sad to say goodbye to the Wright family knowing we probably won't see them again for a long time. We felt that we could have easily spend more time chatting, but at least we will be connected by the internet.

Two steps forward, one step back

2004-07-03T07:16:00.000+10:00

We have been home for five days now and Amelia's OMS symptoms are up and down, and now we have the added complication of steriod side effects. The most notable steriod effect is Amelia's ravenous appetite. No food is safe in our house! In particular she is craving sugar, but we have been advised to try and remove it from her diet altogether to stop her from ballooning out and becoming 'Cushingoid'. We are trying, but it is extremely hard to avoid sugar in our society. Almost everything we buy has added sugar, so the only way seems to be to cook everyting from raw ingredients ourselves. The Atkin's diet would work, but we don't think we are ready to do something that radical yet.

Suzette from OMSFamilies sent us a very nice email encouraging us to be patient with the treatment. We have only been taking the steroids for about 10 days and it can take weeks for even very effective treatments to work, so it is early days.

The following list shows changes in Amelia recently. I have categorised the changes based on what I think the cause is:

OMS Symptoms

Tantrums - None for a few days and then a few recently
Bad sleeping - This has got worse, Amelia is now waking up many times in the night and she gets up very early. OMSFamilies members have overwelmingly recommended trazodone (Trazadone?).
Tremor - A fast, fine hand tremor has developed. It is different to the original slow, large tremor, so it may be from the steriods.
Steriod Symptoms
Increased appetite - Amelia is eating at least twice what she used to and she is putting on weight.
Craziness - Amelia seems tipsy a lot of the time and she has developed a manical laugh. We love to hear Amelia laugh and the laugh is infections, but it has a disturbing insane edge to it.

The hardest things to deal with are the tantrums and sleep problems. I think we are better at handling tantrums now that we understand the condition more, we don't try to fight it, we just wait for it to pass and try to isolate Amelia while it lasts. The sleep is a problem for Kara and I because we get woken up whenever Amelia wakes and it is bad for Amelia because we suspect her OMS symptoms are worse when she is tired.

Fortunately Sophie is setting good behaviour records for a baby and not disturbing us too much at night. The last five nights, Sophie has slept 7, 7, 7, 8 and 8 hours respectively. Amazing for a six week old! I think she is achieving this by feeding hourly from 5pm to 10pm, which is hard for Kara, but worth it.

Settling back home

2004-06-29T21:34:00.000+10:00

Today was the best day for a long time. Amelia's was so well behaved that she didn't have to go to her room once, which hasn't happened for months (excepting hospital visits!). Kara and I noted several incidents that would have previously caused tantrums, pass without any unusual behaviour.

Last night's strange behaviour has not reappeared, so we hope it was a one off occurence caused by over tiredness and a change in her medication.

The highlight of the day was Amelia wearing her new fairy dress to the zoo. The sight of a pretty little fairy wandering around caused a great deal of interest and comments from the other zoo visitors. She had been desperately waiting to get the dress for over a week, and she loves it so much that she took it to bed with her.

Hopefully, with some more settled days like this, Amelia's language and coordination will continue to improve.

Back home

2004-06-28T22:09:00.000+10:00

This evening saw some new and strange behaviour in Amelia. Her speech started to slow down again, and by about six o'clock it was as slow as I have ever heard it. At about this time Amelia started to insist that I write something down, but she couldn't remember what she wanted me to write. It seemed that she had lost her short term memory, and she was thinking faster than her mouth could keep up so she wanted me to write down her thoughts for her. Eventually, after about an hour, she dictated the following sentences to me and insisted that I read them every morning:

I don't want to talk because I don't remember the things I want to say to Daddy

I can't remember those things that I want to say

Despite saying that she didn't want to talk, she kept on talking, just very slowly. I think that this disturbing development is just a combination of over tiredness and the different steroid dosage. We are due to see the our doctor on Monday week, so we will just watch and wait until then. If Amelia is not almost back to normal, we will start the next treatment which is IVIG (gamma-globulin).

I think Amelia is becoming very aware of her condition now and she is starting to get very frustrated with the OMS symptoms, such as the slow speach. I think this awareness might be turning what used to be tantrums into frustration.

Back to hospital

2004-06-28T21:49:00.000+10:00

23rd June

After a couple of frustrating days waiting, we finally got back into hospital to start the steroid treatment.

The conditions here are not nearly as nice as we have had in the past. We are in the same hospital but a different ward, which is much busier, and we are in a shared ward. We are in the 'neuro' ward where there are six children per room as opposed to four in the oncology ward, and the parents have to sleep in a separate room. Kara would have liked to stay here with Amelia, but it wouldn't work with Sophie, so I will stay here while Kara looks after Emma and Sophie at home.

We started the intravenous Methyl prednisolone at 17:30. The dose is 20g/kg for six hours every day for five days. The first one has gone well. We were warned of a host of potential side effects, from high blood pressure problems to total physical collapses, but we haven't experienced anything bad yet.

The only change I noticed was that Amelia woke up without matted hair. Most mornings Amelia's hair is a tangled mess of knots and craziness which takes a lot of hairspray and careful brushing, but this morning it was almost knot free. I hope that this is sign that she is sleeping soundly, but it might just be the new conditioner!

25th June

We got to know a little girl called Bronte today. Bronte is three and a half and suffering from epilepsy of some sort. It seems to be quite severe at times and apparently she has grand mal seizures. She was a very sweet little thing and a bit manic from the medication she was getting. When she was very manic she couldn't stay still and ran around the ward chatting to the other kids and trying to get in bed with them.

24th June

It is quite a sobering experience being in the neurology ward. There are a wide range of problems here, and many of them make Amelia look lucky. There is a natural bond between the parents here and a lot of discussion about conditions and treatments. It is all very interesting, but like watching a horror movie, and sometimes I would rather not know and I am worried about Amelia listening too. Too much for such a young mind.

25th June

We have been here for a while now and Amelia seems to be much the same. The only change I have noticed is that her tantrums seem worse and longer lasting. This may just be the new environment, which would be challenging for any four year old. Amelia is constantly attached to a drip and she is continually prodded and poked (but only two needles so far) and observed. Visitors seem to unsettle her even though she says that she wants them. Her neigbourhood friend Georgia and her mum dropped in for a visit during a terrible tantrum the other day. The tantrum of screaming and kicking continued for the 30 or so minutes while they where here and didn't stop until well after they left. Poor Georgia was very distressed by the hospital scene and apparently cried when she got home. However, when Amelia described the visit to Kara over the phone, she said the visit went well!

26th June

Last night Amelia was very settled, much more than any night at a hospital yet. She was quite normal for most of the day too. She made friends with a girl called Cassandra early this morning and spent a lot of the day with her. Cassandra is seven (and about the same height as Amelia), she is here visiting her brother who is about four and seems to be very sick and disabled. Watching the two girls play really emphasised how much better Amelia is today. Her balance is improved to the point where she did not fall over when Cassandra walked into her and she could run around the fairy garden at a reasonable pace. Her language seems better too, the words are coming out much faster and more clearly. In the morning Amelia was having trouble finishing a sentence because she went so slowly that Cassandra would be bored and interrupt, but by the afternoon they were having normal conversations. The doctors and family have also noticed the improvement. Today is the fourth day of the steroids and a milestone in Amelia's recovery.
The activity of the day seems to have caught up with her now, it is six o'clock and despite the din of the other four families in our room, she has just fallen asleep.

27th June

After Amelia fell asleep at six last night, she only slept for a little while and then woke up in a foul mood. She finally got to sleep at about nine o'clock after screaming and kicking for hours.
Fortunately Amelia woke up this morning in a good mood, and today was a good day. The methyl pred. seems to make Amelia quite hyperactive and euphoric at times, as if she had drunk half a dozen beers.
Thomas, Amelia's cousin visited today and cheered her up quite a lot. Thomas' dad, Paul dropped in with him. Paul had not seem Amelia for over a week and he was quite struck by the improvement in her condition. He really noticed her improved balance and improved mouth. In particular he thought Amelia's bottom lip was much more relaxed, which is something neither Kara nor I had noticed. We suspect she had been tensing it to compensate for tremours.

28th June

Last night was another good one. Amelia went to sleep without any trouble despite an infusion of ranitidine (to settle her tummy) and the final removal of the canula during the night. This morning was a happy one too and Amelia was quite chirpy and cooperative, but the mood was spoilt by our first oral dose of the steroids. The dose arrived in four horrible tasting pills which were painfully swallowed, one by one. We tried different methods and the last pill was taken covered in strawberry jam and washed down by milk. A year of four pills a day sounds like a harsh sentence.

Amelia recovered from the yucky pills quickly and was good for the blood test that followed shortly after. We were discharged late in the morning and finally got home. We have numerous checkups and blood tests with various doctors scheduled, but we are hoping that we won't need to be hospitalised again.

OMSFamilies to the rescue

2004-06-21T17:18:00.000+10:00

The big news of the last few days has been that we have found the OMSFamilies Yahoo group. We are registered in the group as 'jameskara2002'. Thanks to Lisa who started the group! There is a very active community of OMS families using the group on a regular basis and it is a great comfort to us to feel that we are not alone and there are people out there who know what we are going through. We already have got lots of advice and information from kind OMS families. We are even planning to meet one of the families who are coming to Sydney from the USA next month.

Thanks OMSFamilies!

The other news is that our admission to SCHR has been delayed. It looks like we will probably go in on Wednesday now.

MIBG Scan

2004-06-19T07:57:00.000+10:00

Amelia had her MIGB scan yesterday. We don't know the results yet, but we are not expecting anything bad. The actual scan went well, we have learned a lot over the few weeks and applied it all to make Amelia more comfortable. She had to be sedated and we specifically avoided pethadine (which we think makes Amelia vomit) and Midazolan (which we think makes Amelia extremely irritable) and used medication that has worked in the past (morphine and ???). When in doubt have been trying to avoid drugs from a list at the omsusa.org site.

Amelia's symptoms seem to be much that same, perhaps a little worst after the sedation. The next step is to start a week of intravenus Prednisone treatment on Monday.

Meanwhile the rest of the family is well. Sophie is doing all the correct things for a three week old baby which is sleeping, eating and pooing regularly.

Back to Hospital

2004-06-17T19:24:00.000+10:00

We went back to hospital for a few hours today to prepare for an MIBG scan. I am not sure of the technical details, but the scan will detect if there are anymore tumours. Because of the favourable pathology so far, we don't expect any nasties to be detected.

The preparation involved an intravenous injection of an isotope. Kara was there for the injection which was a change because I have been there for most of the previous procedures while Kara has looked after Sophie. I am glad she had a chance because it is very special to be able to comfort your child at such a time. Kara tells me that it went well with just a few tears and Amelia was very brave.

Amelia's neurology specialist, Dr. Grattan Smith, is still away, so we didn't have chance to discuss Amelia's OMS symptoms today, but we did see Amelia in a new environment and we noticed that there does seem to be an improvement. The hand tremor is nearly gone and her overall coordination seems to be better. She is still stumbling around like she is a little bit drunk, but not as much as before. It will be very interesting to hear Grattan Smith's observations after he has not seen her for two weeks.

Amelia's tantrums are still a problem. She had an appauling outburst today when I took her and Emma to the supermarket to get the groceries and a milkshake. As usual, I did the groceries first, but about half way though Amelia wanted her milkshake and wouldn't be told to be patient and wouldn't stop whining and screaming. Eventually I had to go straight home and put her in her room, with no milkshake and only half the shopping done.

We are also concerned about Amelia's speach. Her sentences are complete and complex, but just slow and we can't tell if it has improved since the operation or not.

Opsoclonus Myoclonus Syndrome links

2004-06-15T13:12:00.000+10:00

A list of links I have found for OMS

Link	Description
Opsoclonus Myoclonus	An article about Opsoclonus Myoclonus on Wikipedia (written by me!)
gabriellemoss.com	Gabrielle's story
omsusa.org	The National Pediatric Myoclonus Center. An American site.
suburbanchicagonews.com	Cori Salomon's story. Cori's OMS started in a very similar way to Amelia's. Cori did not have a neuroblastoma and seems to have had worse symptoms compared to Amelia.
cancer-info.com	A discussion about OMS
www.necker.fr	Contains a reference to a French research paper.
OMS Support group	Web page is dated 2002.
OMS Support group stories	There are some interesting OMS stories here.
cgi.videosilva.com	An OMS story
dancingeyes.org.uk	Dancing eyes syndrome (UK name for OMS)
www.icongrouponline.com	A link to a book about OMS

Symptoms

2004-06-15T13:05:00.000+10:00

Amelia's Symptoms

This page documents Amelia's symptoms from OMS.

Symptom description table

Date	Opsoclonus Myoclonus Symptoms
Late 2003 - early 2004	A lot of baby talk. Unusual dislike of going preschool (but happy when she was there).
Early May 2004	Daily tantrums about trivial problems. Waking up a night very distresses every few nights. Intense resistance to going to preschool and refusal to work with other children while there. All speach was in 'Baby talk'
Mid May 2004	Onset of hand tremors. Clumsiness such that she was falling over once or twice a day. Could not walk in a straight line. At least one tantrum each day. The hand tremor made it almost impossible to use cutlery.
Hospital May 26th to July 8th	All symptoms were a bit worse except the tantrums stopped. Speach changed from baby talk to normal sentences delivered very slowly. Ennuciation was bad and there was some mumbling.
Bone biopsy July 8th	Probably the worst day for the OMS. Nil by mouth until the general anesthetic a 14:00, which made everything worse. Went to theatre screaming and came out screaming. The screaming and tantrums continued for two hours after she woke up. Tantrums continued almost non stop into that night and most of the the next day. My fear that this behaviour was going to be long term made this one of the worst times of all, but I suspect it was bad reaction to the sedation.
At home June 8th to 15th	The hand tremor has reduced greatly. Hands are almost normal with just a bit of gross tremor sometimes. Walking is still unsteady, but walking in a straight line is usually possible. Speech seems to have improved slightly, but it is hard to tell (see attached sound files). Still having one or more tantrums daily. Had to let her cry herself to sleep on the first three nights that we go home, but the following nights were normal.
At home June 16th to 21th	In general the symptoms seem to be fairly stable. Tantrums and irrirability are probably the biggest issues at the moment, with a least one or two daily outbreaks. Overall Amelia seems a bit withdrawn and uninspired. I took back to preschool for a couple of hours last week, but she didn't seem very happy. She held my hand most of the time and didn't want to interact with her friends.

Sound Files

Some recordings have been made of Amelia talking. These recordings have been made as a record which may help speach therapy and also to measure any changes.

Drawings

Amelia has made a series of drawings of cirles, squares and spirals since early June. These pictures have been made at the request of Dr. Grattan Smith in an attempt to measure changes in Amelia's condition. Digital scans to be completed.....

Video Files

A number of videos have been made of Amelia by Kara and I as well as Dr. Grattan Smith. None of these videos have been digitized yet. To be completed....

Amelia's Story

2004-06-15T13:00:00.000+10:00

Amelia's Story

Introduction

Amelia was born on 12th October 1999, the first of James and Kara's children. The birth was normal, albeit very fast and exciting. She was a few weeks early, but healthy and she didn't need any special care. The next four years passed happily and Amelia grew healthily and quite normally. One point of note is that she was has always been very tall, much taller than her contemporaries and generally taller than kids one year older. Since Kara and I are both quite tall (179 and 181 cms respectively) this has never been seen as particularly strange.

Everything Changes

In late May 2004, Amelia was diagnosed with a neuroblastoma in her adrenal gland and Opsoclonus Myoclonus syndrome (OMS). In one short week, our lives were turned upside down as we struggled to deal the knowledge of Amelia's condition and the treatment that was to follow.

The first symptom that made us seek help was a tremor in Amelia's hands, but in hindsight, there has been changes to Amelia's behaviour for some months previously. The first thing that was different was her attitute towards preschool. Amelia had attended a Montessori school since she was three and always enjoyed herself, her teachers once commented on how she used to sing to herself as she did her activities. Amelia was very happy socially as well and she made some very good friends. This began to change in about November 2003 when we noticed that she started to resist going to preschool, she complained about the work being boring and in the parent teacher interview at that time we noticed that she seemed to be lagging her peers in a couple of areas.

Amelia's dislike of preschool slowly increased over the months and she started to show other changes as well. She started to talk like a baby at times, she started to have temper tantrums on a daily basis, she was becoming very clumsy and though we didn't notice it at the time and she was putting on a lot of weight (15kg to 23kg in about six months). Most of these changes we attributed to stress from the immanent arrival of our third child. Kara stopped work because of her 'irritable uterus' and she started to rest a lot and probably gave Amelia and Amelia's younger sister, Emma less attention than they were used to.

The hand shakes started to appear just as Sophie was due. Kara and I were both a bit worried about it and decided to seek some advice after the baby arrived. Sophie finally arrived on May 20th and I brought Kara and the new baby home on the 24th. Kara had not seen very much of Amelia while she was in hospital and when she got home she was distressed to see that the shaking was much worse than she remembered. Amelia's behaviour has also gotten a lot worse, she was talking almost entirely in baby talk and she was usually having two or more screaming tantrums each day. It was now obvious that this was more than just a reaction to the new baby. Kara called a social worker and described Amelia's condition and she suggested we speak to our doctor, so we booked in to see Dr Dowd.

The Journey Begins

We saw Dr. Lynn Dowd at 16:15 on May 26th. It was our first outing as a family of five and we had our new people mover filled up with an assortment of nappies, child seats, changes of clothes, milk drinks, three small girls and two very busy and worried parents. It would have been more practical if just mum or I had taken Amelia to the doctor, but we both wanted to be there. Dr. Dowd proded and poked and observed and to her great credit (I later found out that this condition can be mistake for a virus), she decided that something was seriously wrong. She didn't say at the time, but I think she suspected a brain tumour and she sent us directly to the emergency department of Royal North Shore Hospital. She even rang ahead and asked that a doctor she knew, Dr. Helen Young, see Amelia.

I was distressed that Amelia's problems seemed to be so serious, but I was also relieved that we were getting prompt attention. I had been dreading a long and protracted diagnosis that might involve months of seeing specialists and I didn't realise how serious it was about to get.

Royal North Shore Hospital

We got to the hospital a bit after 18:00 and went straight to emergency. Kara had rung her parents and her father, Tony arrived not long after us. The staff were great and after various prods and pokes we were sent up to a ward to wait for CT scan. I first got really worried when I was in the CT room with my lead suit on. Seeing Amelia in that big machine got me thinking about what they were looking for and I think that I first started to consider that there might be a brain tumour. I remember thinking that a four year old girl should not have to go through this.

The CT scan didn't detect anything and I felt very relieved. I thought that a brain tumour was the worst case and Amelia seemed to be in the clear. We were also booked in for an MRI scan which didn't happen until Thursday.

Kara went home after the CT scan to get some well needed rest. Sophie was only 6 days old and nobody could believe that Kara had only just given birth.

We had great care while we waited for the MRI. I stayed all the time and Kara came in during the day, we had our own room and the staff were terrific. Amelia had a few needles over this time and was starting to realise what being in hospital meant, but it was still a big adventure for her and she didn't have any tantrums. I think Amelia had been trying to hide her symptoms for sometime and she was relieved that it was out in the open and getting attention. Apart from the better behaviour, her speach changed from baby talk to normal sentences expressed at a very slow and measured pace.

The MRI was what picked up the neuroblastoma. The scans of the head didn't show anything, but on incredibly wise advice from Dr. Paddy Grattan Smith, they scanned Amelia's abdomen and found the tumour in the right adrenal gland. I realised that this was very serious when Dr. Young asked us to sit down before she gave us the diagnosis. I didn't even know what a neuroblastoma was but the doctor's manner made me think it was nasty. What I didn't know then, and fortunately didn't find out until it was irrelevant, was that neuroblastoma is usually fatal in children over about 18 months. My naive assumption, which turned out to be correct in a way, was that the tumour was readily treatable and so I felt quite relieved that the cause of all Amelia's problems had been found.

So we said good by to all of our new friends at RNSH and Amelia and I were transfered to Sydney Children's Hospital in Randwick in the back of an ambulance. Kara and various relatives followed us.

Sydney Children's Hospital Randwick

Sydney Children's Hospital Randwick emergency was a very different experience to RNSH. RNSH was quiet and comfortable, but SCHR seemed was like an episode of ER, with constant action of patients and doctors all around us in cramped conditions. Fortunately, we didn't have to wait in emergency very long because Dr. Grattan Smith was expecting us and we were in our own room within a couple of hours. The next week went as follows:

Saturday - Went home in the afternoon.

Sunday - I took Amelia back to hospital on Sunday night.

Monday - CT Scan of the chest and abdomen which confirmed the neuroblastoma. Nil by mouth since last night, but Amelia didn't need sedation after all. Around this time we got the diagnosis of Opsoclonus Myoclonus. It seems that Amelia's immune system was attacking the tumour, which was fantastic news, but he immune response was also attacking her brain. We still hadn't seen the statistics for neuroblastoma, but we were given the impression that the tumour will 'shell out' and there was a high chance that Amelia would not need chemotherapy.

Tuesday - Nothing?

Wednesday - Bone scan. This was a bad day and Amelia was getting tired of hospital. Nil by mouth and sedated for the scan. The sedation wore off before the scan was finished and Amelia paniced about being restrained for the scan. She had to be very still for the scan to work and I avioded getting her sedated again by telling Amelia stories for 40 minutes where the heros had to be very still!

Thursday - Nil by mouth again today. Today the tumor was removed by Dr. Bruce Curry. The operation went well. A highlight of the day was going down to the operating suit being led by the music therapist who sang and played guitar as we walked along the corridors. A frozen section showed the tumour was relatively benign as expected. I got gastro during the night and Amelia and I were moved into isolation.

Friday - Today was a disaster because I got sick. The hospital closed the ward down and no doctors, patients or visitors were allowed. I was told to leave and not come back for 48 hours. They didn't want Kara to come in because they were worried that she might get it (or already have it...) and that would be bad for her milk supply, so we had to start looking for alternatives for being with Amelia. She was still taking a lot of morphine for the pain so it was a very bad time for her to be without mum and dad. Kara's father Tony got sick at the same time as me and Emma had been sick a few days earlier. My side of the family planned out for the two days that Kara and I were locked out.

Saturday - Kara went in to take over from my mother and look after Amelia during the day. My sister, Marion looked after Amelia that night. I had to tell Amelia stories over the phone to settle her down before she went to sleep.

Sunday - I returned to look after Amelia at about 07:00

Monday - The ward was reopened and Kara and I had a conference with the doctors. We finally saw some statistics showing the recovery rates from neuroblastoma which showed a very high (over 95%) survival rate for neuroblastoma associated with Opsoclonus Myoclonus and a very low rate for other cases.

Tuesday - Amelia had a bone marrow biopsy which was very traumatic because she had numerous severe tantrums. We suspect she had a bad reaction to the sedation and it took a couple of days for the tantrums to subside.

Respite

We had a week at home between Tuesday 8th June and Thursday 17th.

- 14 Jun 2004

James and Kara's Family Blog

Weaning off IVIG

Irritability, sleep and Clonidine

Weaned off Prednisone

Weaning

Wikipedia update

IVIG at Westmead

Melatonin is bad

MRI

IVIG and colds

Westmead

Sandoglobulin

Back to school

Steady improvements

Visitors from abroad

Two steps forward, one step back

OMS Symptoms

Steriod Symptoms

Settling back home

Back home

Back to hospital

23rd June

25th June

24th June

25th June

26th June

27th June

28th June

OMSFamilies to the rescue

MIBG Scan

Back to Hospital

Opsoclonus Myoclonus Syndrome links

Symptoms

Amelia's Story