Wednesday, July 28, 2004


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 Previously she was quite withdrawn and shy and as you would see from the previous posts, she had daily bouts of intense irritability.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.

Wednesday, July 21, 2004

Back to school

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!

Wednesday, July 14, 2004

Steady improvements

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.

Thursday, July 08, 2004

Visitors from abroad

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.

Saturday, July 03, 2004

Two steps forward, one step back

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.