Tuesday, June 15, 2004

Amelia's Story

Amelia's Story


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.


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

- 14 Jun 2004


Blogger Zoe B said...

Paul and I have so much admiration for you both. You have shown such strength and courage. Amelia is very lucky to have such wonderful, caring parents. Thanks for setting up this blog to keep us updated. If there is anything we can do, we are just a phone call away!

Thursday, June 17, 2004 6:20:00 pm  
Anonymous Anonymous said...

We are Amelia's maternal grandparents and we are so full of admiration of Kara and James for the way in which they have handled this most distressing chapter in their lives. We pray for a complete cure for Amelia and we will always be there for the whole family. We love them with all our hearts. Precie and Paaps.

Thursday, June 17, 2004 8:06:00 pm  
Anonymous Anonymous said...

Keep meaning to respond to your e-mail through the OMSFamilies e-mail group. I'm so glad that you at least found that forum. No other families are in Australia that I know of. I know a family in New Zealand and India... which would be closer to you than Arizona, USA.
If there's any advice I can offer and that's to have your docs contact both Dr. Mitchell at Childrens Hospital Los Angeles and Dr. Pranzatelli in Southern Illinois. The treatment of OMS has progressed TREMENDOUSLY since Nov. 1996 when my daughter, Justina, got sick.
I'm pleased to hear that you got a correct diagnosis so quickly. It's so rare that that happens. Like your daughter, most get the head CTs/MRI because most doctors are looking for brain tumors. Happened with us and my daughter was misdiagnosed for five months. I think this lapse of treatment did some damage.
Another observation I want to make is that Amelia is "much older" than the normal NB/OMS age. The average age is 19 months old. From the children I've learned about over the years is that it appears that the older the child is, the better off they are in the long run. They have developed many fine motor, gross motor, and a large vocabulary skills that it's a matter of "getting them back". My child falls into the average bucket of kids that have extreme symptoms, complete loss of standing, walking, crawling, sitting, or lying without great support. Justina appeared like a drunk child who had parkison's disease. Every inch of her shook and trembled and she screamed all day and all night. Am I understanding correctly that Amelia's behavior has been affected along with some tremors and speech issues? If this is "all" you have to deal with she will do very good in the long run.
I'm one to advocate treatment to stop and prevent further assault on the brain. I highly suggest that you yourself contact Dr. Pranzatelli too. He's doing quite a bit of research and applies various treatments based on the clinical findings of cell lines that are causing the OMS in your child.
My daughter has been through a gamat of thrapies due to chronic relapse of the OMS. Every time the OMS relapse it became harder and harder to control.
We started with the IV prednisone... It completely whigged Justina out and caused her to go into a psychotic like phase... So, stopped that quick...
Went on to ACTH--- amazing response
added IVIG--- up and completely walking
tried to switch ACTH to prednisone and relapsed.
back on ACTH... higher and higher doses but, not getting best results.
started plasmapheresis, cyotoxan (chemotherapy), IVIG and prednisone.

cytoxan, IVIG and prednisone gave her complete sympton free but, when we stopped suddenly she relapsed.
Any treatment chosen must be weaned extremely slow.... talking about months and months
Used cyclosporine (oral immune suppression drug she was too sensitive to)
back to chemo,ivig and prednisone until 11/99
she then had a stemcell transplant with her own cells in 12/99. This was done to wipe out her immune system, and return cells so her immune system could come back
"normal"... this worked fantastically!!! She's never returned to the obvious OMS signs of opso (eye movement) and myo (ataxic body).
Though last year we give her IVIG every few weeks because she see deterioation in other areas- particulary behavior and cognitive. The IVIG improves this greatly.
In fact, IVIG ALWAYS provided great results for the behavior and the sense of well being.
I wish I could say that my daughter didn't suffer much brain damage. Unfortunately her story is a little harsher. She's 9 and has extremely hard behavior problems, Cannot add or subract without great help, speech is extremely slurred though, this last year she's made HUGE progress. She cannot read or write on her own (can copy). After all these years we've moved our focus from education to "integrating" into society. It's more important for Justina to fit into society appropriately, have friends and interact with the world.
The last 8 years have held soooo much for us that I'm sure I'm missing very important details or omiting obvious things you'd be interested in.
I know a flight agency that will do an international flight for free if you are interested in seeing an "oms" specialist in the flesh.
Stay in contact with the OMSe-mailing group. The experience of everyone involved is tremendous. I sure wish I had it the first couple years.
Lisa Korenko
Justina's webpage

ps- Justina is extremely tall too. Was always extremely long and skinny. But, my husband and are are tall too. me 5feet, 11 inches and my husband 6 feet, 2 inches

Sunday, June 20, 2004 3:02:00 pm  
Anonymous Anonymous said...

I am a member of the oms support group just stopping by to read your story. I know how terrible this disorder is, we all do, all of our stories are very similar. My daughter was diagnosed with oms at 16 months and is now almost 5. I am so glad you found the network, it helps to know you are not alone. I wish the best for your little girl and your whole family, you will all be in my thoughts and prayers.

Thursday, July 29, 2004 12:00:00 am  
Blogger Sandra said...

My name is Sandra and my daughter Madeline has recently been diagnosed with OMS with onset of symptons starting on her 5th birthday approx. 5 weeks ago.

We are an Australian family currently living in Hong Kong.

We are in the process of ruling out common causes such as neuroblastoma which seems unlikely at this point, however still awaiting results of some urine/blood tests.

One week ago we admitted her into hospital where she received 25mg/kd/day of methylprednisole for 3 days. She is currently on oral steroids of 1mg/kg/day for 2 weeks then we'll go to every second day. So far she has shown a good response and I feel that an improvement of approx. 80% has been observed.

I'm not quite sure what to ask you at this point as I'm still coming
to terms with the diagnosis and have only started looking on the internet where I found your site. I guess I will be in touch at some point in the near future.
Many thanks

tel. +852.96092025

Sunday, October 12, 2008 1:06:00 am  
Blogger James said...

Hi Sandra,

I am very sorry to hear that you daughter has been diagnosed with OMS. I remember how hard it was for us when Amelia got sick, especially in the early months. I am very encouraged to hear that Madeline has had such an improvement from the steroids. As you may have read from our blog, Amelia had a similar treatment and it helped a lot.

It has now been nearly 5 years since Amelia was first diagnosed and she is still on Trazadone, but otherwise she is very well.

A lot of kids in the US are being treated with Rituxan, but it wasn't used when we were seeking treatment. I think the usage is still experimental and I am not sure if it is appropriate for your case, it it might be worth investigating. Not sure if you have found it yet, but I think the best place to find out more is the OMS Families Yahoo group (http://health.groups.yahoo.com/group/OMSFamilies/), we have met lots of families through the site and it is a great place to ask questions.

Best wishes,


Monday, October 20, 2008 12:01:00 am  

Post a Comment

<< Home