Settling back home

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

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

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

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

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

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

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

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

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