12 years old and doing well

Yesterday Freya picked up her box of watercolour pencils and drew a lovely sketch of a gum tree.  She used various shades of brown and grey to recreate the contours of the branches and bark.  Not a remarkable effort for most girls, but for us it's something we were delighted by.  For one, it is the first time since her surgery she has shown much interest in art - she has not had the concentration or interest to do any drawing, something she previously enjoyed and was very good at. Just as heartening is that the drawing is  further indication that Freya's left hemisphere is reorganising to take over the tasks of her disconnected right hemisphere.  The right hemisphere is responsible for visual and spatial skills and it is generally considered that creativity, visual arts and musical talent are all right hemisphere skills. Unfortunately, there has been no improvement in her singing voice which is just as ordinary as ever - I don't think we can blame the encephalitis or hemispherectomy on this though, given the appalling lack of vocal and muscial talent in her gene pool.

Last Wednesday (Australia Day) was Freya's 12th birthday.  She declared it her best birthday ever.  It was a great day, she was thrilled with her presents and was happy all day. It is wonderful to see the improvement in her mood and a corresponding willingness to try doing things with one hand that she said were "too hard" just a few weeks ago.

2011 - A new and brighter year

Four months has passed since Freya's surgery and a new, and hopefully better year has now begun.  This time 12 months ago we were in the process of requesting approval from the hospital ethics committee to commence Freya on a new drug being trialled in the US for Rasmussen's Encephalitis (RE).  Approval was granted and Freya was provided with the drug over a course of several weeks in January and February.  Because a response to the new treatment can take up to six months, we then had to endure a long wait while we all hoped that a dramatic improvement in Freya would occur - unfortunately this wasn't to be, with Freya continuing to deteroriate over this period. This is one of the many challenges of RE, not only is the course of the disease extremely variable, so too is the response of children to the exisiting arsenal of treatments available.  What is not so variable, is that ultimately most children with RE will need to have a hemispherectomy once the treatments no longer hold the progression of the disease, if they have at all.

Today, as she happily plays the Wii with her cousins, it is hard to believe how far she has progressed in the last four months.  From being barely able to engage in conversation or concentrate for more than a couple of minutes in the early weeks post-surgery, she is now back to doing most of the things she liked to do before her surgery.  She is back into enjoying music, scouring Itunes for her favourite songs and downloading them onto her Ipod.  She has been to the movies many times, she watches her favourite TV programs, music clips on the Ipad, argues with her sister....all the normal activities of the average 11 year old girl.

Of course, she is not quite a normal girl:  She is still learning to adjust to a life with the use of one arm and hand.  While her left arm has a quite a good range of movement it is difficult for her to translate this movement into any real functional use, but both Anthony and I and the OTs will keep encouraging her.  She is not back on her scooter yet (a great form of transport when you have one weak leg) but this is probably more from lack of confidence than lack of ability - we will keep encouraging her on that front too.  Her planning, problem-solving and decision-making skills are also still quite compromised and it may be some time before they are back to pre-surgery levels.

One disappointment has been that we have not been able to stop all of Freya's anti-epileptic medication.  A recent EEG showed that the temporal lobe of her left hemisphere is having some sub-clinical epileptic activity.  Her neurologist is confident that this is a result of the left side of her brain being irritated by the constant seizure activity on the right side in the weeks and months before her surgery, particularly all the seizures that were originating from her right temporal lobe.  He is also confident that this will settle by itself, but in the meantime she will need to stay on one medication to be safe.  Still, she was on five anti-epileptics and she is now only on one - quite a reduction.

Freya will continue returning to the hospital a few times a week during January.  When school resumes in February, her therapy schedule will be reviewed and possibly reduced to one day a week in which all her therapy sessions will be scheduled.  A lot will depend on Freya's level of fatigue, and how she is coping with a return to school.  This is still over a month away - quite a long time in the scheme of things.

settling in

After a lovely holiday in Noosa, we are all settling into our post-hospital routine.  This routine revolves around a weekly timetable of daily out-patient appointments at the RCH which can involve only one or two sessions or a full day.  Freya has also been very keen to return to school and participate in as many activities as possible before the end of the school year next Friday.  The rigorous RCH timetable doesn't allow for much school time, but she has managed to spend a couple of hours at school several times over the last week and a half.  In addition to the excitement of seeing all her school friends and teachers again, it has given her mood and confidence a huge kick-start to be doing 'normal' things again.  Thank you so much to Ms Sear and  5/6S for giving her such a heroes welcome.

Freya is also receiving Occupational Therapy at home twice as week as part of a new initiative of the RCH "Hospital in the Home" Program.  The home visits are about encouraging her to use her left arm and hand as "helpers" as much as possible in a practical, everyday way.  Because her left arm and hand ares so weak, and using either takes such effort, is is very easy for children who have had hemispherectomy surgery to simply ignore their weak arm and hand and rely entirely on the stronger ones.  Without constantly thinking about, and  trying to use her weak arm and hand Freya's brain will not make the neuro-connections needed to make them functional, so it can become a cycle of weakness and neglect, something that we would really like to avoid.

This week at the hospital is a busy one:  On Thursday, Freya will receive her first botox injections (young, I know).  The botox is intended to reduce the stiffness in her left calf muscles to give her a more natural gait and also allow her left hand to relax more readily.  I'm sure I won't be the first mother that has argued for a bit of botox to administered in my direction given the all the extra wrinkles she has caused me over the last months (years).  Freya will also be having her first EEG and MRI since the surgery.  Although she appears to be completely seizure-free, the EEG should confirm that there are no sub-clinical seizures occuring in her brain (those that have no obvious or outward presentation).  All fingers crossed.

Here's Freya enjoying the Noosa beach from the safety of her beach towel.  Despite the balmy 27 degree weather she declared both the resort pools (heated) and sea too cold for swimming.  3 months in the airconditioned comfort of the RCH has made her very soft!

home

Today Freya was discharged from the RCH, just short of 100 days since admission.  In this period and the weeks before she was admitted, she has experienced status epilepticus (a life-threatening state of near continuous seizure activity), the most radical of all brain surgery on 31 August, and then following this surgery total paralysis on the  left side of her body.  In these same 100 days she has also learnt to sit, stand and walk again. It has been a busy few months.

Physically, with the exception of her left arm and hand, she is stronger and fitter than she has been for a long time.  She can now walk without experiencing leg seizures and today she was assessed as having 80% of the walking speed and endurance of the average 11 year old girl.  Over the last few weeks, we have been regulars at the hospital orthodics department to have her leg orthosis re-adjusted as her left leg muscles have continued to bulk up.

Cognitively, things are improving at a slower pace. By the end of each day (and often for periods during the day) the demands of her physical and cognitive rehabilitation leave her exhausted to the point where even a  simple conversation is beyond her capacity.  During these periods of cognitive fatigue, she cannot maintain eye contact, follow the thread of a conversation or even respond to questions.  As her brain recovers from the surgery and the left hemisphere continues to pick-up the functions of her now defunct right hemisphere, this cognitive fatigue should reduce and Freya will become more like her happy, bright former self.

In light of what her brain has been through over the past five years, we need to be patient and accept that full recovery is going to be a slow process.  We are really hoping that now she is home and out of the hospital environment and engaging in more normal interactions with friends, family and neighbours, her cognitive recovery will be given a healthy boost.

a discharge date

Following our family meeting with Freya's doctors and therapists on Tuesday a discharge date has been agreed;  Freya will be leaving the Royal Children's Hospital next Friday, the 19th of November - 3 months and 1 week since admission and 2 months and 3 weeks since her surgery.  After such a long stay, the RCH has begun to feel like a second home, and while we're all keen to come home, I know Freya will miss the many nurses, doctors and therapists who have provided her with such support, kindness and encouragement over the last few months.  The hospital umbilical cord will not be severed abruptly though, the discharge plan that we will be given before we leave will include out-patient therapy appointments three times a week for the next couple of months.

In addition to the out-patient therapy we will also be given a rigorous schedule of physio and OT exercises to complete daily at home. As the doctors have explained to us, Freya's brain is currently going through a spurt of activity that is resulting in movement in her left arm, with almost daily improvements over the last week.  It is important that we capitalise on the new connections her left hemisphere is making before this spurt of activity slows down or stops.  The photo is Freya doing one of her physio exercises - putting weight on her left arm (and being kept company by Ella). 

To celebrate Freya's recovery from her surgery and triumph over the devastating epilepsy and encephalitis that has plagued most of her childhood, we are off to Noosa the day after hospital discharge.  A new 5 star resort has opened a couple of months ago, with a lagoon style pool - just the thing for continuing her hydrotherapy program.  The doctors agree it's a great idea too.  Ella doesn't seem to be too devastated by missing out on a week of school so close to the end of the year either.

two triumphs

This weekend has been a good one for Freya, with two terrific milestones being reached.  The first is that it is now 2 months since Freya's hemispherectomy surgery, and 2 months since she has had any form of seizure.  It is hard to imagine that such a short time ago, her brain was so on fire from constant seizure activity that she could hardly walk 50 metres without experiencing a seizure in her leg.  Even in her sleep, she was continually woken by many seizures a night.  Given her surgeon's complete confidence in the success of the surgery, her medication is also being further reduced. Another of Freya's anti-epileptic medications is now being weaned (3 down, and 2 to go) and the plan of her neurologist is that she be medication-free by Christmas.  After years of increasing medications at increasing doses, it's great to be on the reverse cycle.

The second of the weekend events, is that Freya has started to gain a bit of movement in her left arm.  When she concentrates, she can now flex her arm at the elbow. This is exciting as it indicates her brain's left hemisphere is making the necessary new wiring to "talk" to her left arm.  Having the ability to control her arm at the elbow will also have big implications for it's functional use.  It is hoped that eventually she will be able to put her left arm on a table or desk, without having to lift it with the other arm.  The left arm can also become much more of a 'helper' arm if is not just dangling uselessly by her side.  Freya has been worried for sometime about the lack of progress in her arm, so it is really encouraging for her to experience this small amount of movement.

home on the horizon

With the possibility that Freya will be discharged from the RCH in 3-4 weeks, all of her rehabilitation sessions will be directed to getting her ready to come home and function as independently as possible on her return.  We will be having some of her O.T. sessions here at home, so Freya can practice doing things such as making her own breakfast and making her bed by herself in own environment.  (I've been too embarrassed to tell Jane, the O.T. that these were things Freya actually didn't do by herself before the surgery!).

Very important will also be teaching Freya to safely navigate our winding staircase to the upstairs bedrooms.  She is beginning to re-learn the skill of walking up and down stairs in physiotherapy, but it will still be a couple of months before her left ankle is strong and she will be declared safe and steady enough to tackle the staircase at home.

Over the next few weeks, Freya will also be having a range of cognitive tests to establish if there are any particular areas that she will need additional assistance with next year at school.  We are expecting her to be challenged by maths concepts and visual spatial tasks, as these are functions that usually reside in the right brain, and often their transfer to the left brain can take some time.  Last Friday, Miss Sear, Margot Sheridan and the acting principal of Fairfield Primary School,  Anne Morton came to the hospital to meet with Anthony, myselfI and the Rehab. Unit's education co-ordinator to discuss Freya's transition back to school.  Thank you so much Rachael, Anne and Margot for your wonderful concern, support and enthusiasm for Freya's return to school - we are so grateful for all your efforts and ideas.

Freya has been missing school very much, and we really hope she will be well enough to join in some of the fun end of year activities, before returning for the serious stuff in 2011.

up and away

In the 6 weeks since the surgery that left her with a complete left-sided hemiparesis (paralysis), Freya has re-learnt the skill of walking independently.  Assisted by her ankle and foot orthosis (AFO) Freya's gait is now actually better than it was immediately before her surgery.  Proof of this came when we viewed video footage of Freya's walking made by the physiotherapists the day before her operation.  Anthony and I hadn't  realised just how badly Freya's limp had become in the last six months until we saw this video.

Everyday Freya wants to walk further and further, and today she achieved over a kilometre. The physios are actually telling Freya not to walk as much as she wants to, because physical fatigue is still a big issue and it important that she maintain a quality gait, which does tend to deteriorate as she becomes tired.  Being able to walk again has had a huge positive impact on Freya's mood and confidence, and has inspired a  determination in physio and OT that I had doubted she would have.  We are very proud of her.

Here is a photo of her taken today, in full stride.

new hope for Rasmussen's Encephalitis (RE)

Tomorrow my sister Deirdre arrives home the US after attending the first major conference on Rasmussen's Encephalitis.  Deirdre and I have both been involved (Deirdre much more than me) in researching and writing background papers for the conference.  There has been very little research into the causes and medical cures for RE due (1) the rarity of the disease, and (2) a surgical cure is available through hemispherectomy surgery.  The problem is of course, that this surgical cure comes at a significant cost to the child, and it is a very blunt and non-targeted way of dealing with an immunological disease.  Also, this surgical cure has not changed much in the last 50 years.  We think 21st century medicine should be capable of providing a more sophisticated and less brutal approach to dealing with chronic inflammation in the brain than simply removing or disconnecting the effected half.

At the conference, over 50 of the world's key players in RE and top immunologists, biochemists and geneticists came together to discuss the future course of research that may yield a cure for RE.  They all agreed that RE is 'ripe' for research and a key group of doctors will meet again in February to agree a research agenda.  Top of the list will be setting up a process for researchers to gain access to brain tissue from RE children.  We are very glad that we insisted the Royal Children's Hospital store some of Freya's brain tissue for future research purposes.  We will happily donate it to a research brain tissue bank.

Some of the specialists at the conference also felt that specific treatments available for MS sufferers may potentially benefit RE children.  Deirdre tells me there was a great deal of excitement and enthusiasm at the conference for the type of research that could be conducted, and some believe that current treatments for other neuro-inflammatory disorders might be applicable for RE children.

Fingers crossed that this conference represents a turning point that will jump start research into understanding and curing RE - hopefully saving future children from this debilitating disease.

Sunday 10th October

Today marks the end of Freya's 8th week at the Royal Children's Hospital. She was admitted two weeks prior to her scheduled surgery due to a severe exacerbation of her Rasmussen’s Encephalitis.

We are now well into the swing of the hospital routine and daily rehabilitation sessions. Weekends that have previously been slow and quiet at the hospital are much more pleasurable now that Freya has been granted weekend day leave. This weekend she came home for a few hours, went shopping for new shoes that will fit her AFO, went to the local park where she bumped into her best friend Edie and saw her cousins Hannah, Joel and Julia.
Conversation is still a little difficult for Freya, but she is slowly getting better at staying focused and ‘on topic’.

Here is a photo of Freya delighted by a cake made for her by her friend Adele. Thanks Adele it was delicious – you are a fantastic cook just like your Mum!

the odd couple

Freya is continuing to make great strides in her physical recovery from surgery.  She can now walk assisted for about twenty metres.  She tires easily and her left foot then begins to drag, but her AFO (ankle and foot orthosis) which arrives tomorrow should help significantly with this.  The physiotherapists have instructed Freya that she is not to attempt to walk unassisted until November, a frustratingly long time for her.

Here is a photo of Freya and her hospital friend Becky enjoying a bit of afternoon tea together - a couple of previously normal girls, one coping with the after effects of a shattered body (following a serious car accident) and the other from surgery to repair a "broken" brain, as Freya's surgeon Wirgina Maixner refers to it.  Wirginia uses this term to remind Freya that although the scars on her scalp are quickly healing, her brain has not recovered from the surgery and it will take several months to do so, just like a badly broken leg, but without the plaster cast.

Becky has many reasons to smile; on Friday she had her 'halo' removed and she is likely to be discharged in 2 or 3 weeks.

new challenges

This week the occupational therapists have begun working with Freya to equip her with the skills to manage with the use of only one functional hand.  While it is hoped that Freya may regain some limited movement in her left hand, this hand will never recover to the point where there is any of the fine finger movement required for functional use.  At best, she may develop the ability to create a loose grip, but even this is not guaranteed.  From now on she will need to learn how to shower, dress, feed herself and perform all school-related tasks with the use of only her right hand.

While watching her struggling attempts in therapy to put on a t-shirt, cardigan or shoes with one hand (and a limp left arm) is it very difficult not to jump in and help her out.  This of course would not be very helpful in the longer term, as Freya needs to be able to function in daily life independently without our constant assistance.  Still, it's hard not to be effected by how frustrated and fed-up she is by the amount of effort now needed to do the small things we all take for granted.

our first outing

Today we all left the hospital for the first time since Freya's surgery.  She is quickly getting over her flu and the doctors said that provided Freya covered her mouth and nose with a face mask whilst leaving the hospital we could take her out for a short time.  Her destination of choice was pizza in Carlton.  I've attached some photos of us making our escape and our 'parole'  lunch.  Freya will be re-tested on Wednesday for influenza and if the test is negative, she will be able to move around the hospital again.

I've also attached a photo of Freya in her helmet that she was fitted for on Friday.  The helmet became necessary following a couple of not-so clever incidents where she thought she could stand by herself.  As both incidents occured during the night, she must wear the helmet during the night and anytime she is not supervised.  The helmet will protect her fragile skull and brain from any potential fall during this critical recovery period.  As you can see by the expression on her face, she is rethinking the wisdom of her nocturnal hijinks.

 leaving the hospital

pizza tastes so much better in the sunshine

mmm...maybe I won't try out any of my tricks by myself again

pressing the pause button

After developing a high temperature yesterday morning, the doctors ordered a nasal swap to test Freya for potential viruses and unfortunately the swab tested positive to Influenza A.  We are not sure where she managed to catch it, as we have been fanatical about handwashing, she is in a private room and I can't think of anyone she has been in contact with who was ill.  We are told that September is the peak flu season in Melbourne and this is evidenced by every spare bed on the neurology and rehab. floor now being occupied by children from the Children's Cancer Centre on the 6th floor.  It is not safe for these patients to to be accommodated with other immunosuppressed children receiving cancer treatment.

I have never considered the 8th floor of the hospital to be a particularly cheery place, but now with the arrival of these little children from the Cancer Centre, all sadly alike with their bald heads, white skin and nasal gastric tubes, I realise there is definitely worse places we could be.

Freya's flu means we have to press the pause button on all her rehabilitation sessions until she is well again.  Like anyone with the flu she is feverish, aching, tired and miserable.  This is a pity as tomorrow in physio we were due to learn how to tranfer Freya into our car - our ticket to freedom (at least for short periods at weekends).

3 weeks on

Today is 3 weeks since Freya's radical brain surgery.  In these 21 days it has been incredible to see the physical process of her recovery.  Everyday she is amazing us with progress that we did not expect to see for several weeks.  Yesterday in physio she stood unassisted from a sitting position, not using her right hand for support.  Today she can do this with ease.  With the support of the therapist she also walked about 3 metres.  Tomorow she will be casted for an AFO which is an ankle and foot orthosis - a moulded plastic brace that will support her weak left leg during it's recovery.  The physio has asked that the AFO be made "urgently" (probably about 2 weeks) as it is now clear that she will be ready for walking weeks before the therapists had anticipated.

The photos I've attached are already a bit dated.  The first is of Freya and Ella about 8 days post surgery and the other is of Freya sitting unsupported for the first time (and feeling very pleased).  Freya's lop-sided smile is typical after hemispherectomy surgery and usually resolves within 6 months.

an overstimulated brain

On the recommendation of Freya's neurologists, we have had a very quiet weekend.  After a week of her sleeping very badly at night, the doctors have declared that her left hemisphere is "over-stimulated" and struggling to cope with all the extra connections it is needing to make to take over the tasks of her non-functioning right hemisphere.  Her left hemisphere is firing and not letting her calm and settle enough to get a good night's sleep, despite medication.  Even activities such as watching TV, listening to music or books were not recommended this weekend, as her brain needs the necessary down time to allow the rewiring to occur.  Unfortunately, this means we will be putting off visitors for a little while longer too. In itself, the  stimulation of the OT and physio is enormous at this early post surgery stage.

On another note, one of the right brain functions that we were told does not always transfer well from the right to the left hemisphere is facial recognition.  Some children who have had a right hemipherectomy will always struggle to recognise unfamiliar faces and have to develop compensating strategies to remember people they do not see regularly.  It is already clear that Freya's left hemisphere has no such problems - despite a rotating roster of about 20 or more nurses, Freya remembers the names of all of them.  When Anthony asked her whether she recognised them by their voices or their faces, she replied "their faces of course".

a goal scored!

Despite our scepticism that she was way off being able to sit independently, Freya can now sit by herself without support.  She cannot do it for too long, but yesterday she sat cross-legged on her bed playing with her ipad for at least 15 minutes.  In her wheelchair she no longer slumps to the side, but is able to stay upright without the support of pillows.  Her brain's left hemisphere is rewiring itself to make the necessary connections to her left side to give her neck, shoulder and trunk the necessary strength and balance to achieve this important milestone.  We have taken some photos that I will include in my next post.

a few goals

Today we had a meeting with the staff of the Rehab. Unit to discuss Freya's progress and map out some goals for her in-patient stay.  This meeting will occur every Tuesday morning and will be an opportunity for all those from the unit who are involved in Freya's care to come together to talk with Anthony and I about how she is tracking.  The short-term physical goals the OTs and PTs have set for Freya are to be:
1.  Sitting independently by the end of this week
2.  Standing with support by the end of next week
3.  Taking a few steps with support by the end of the following week.

From where we are now these seem like pretty ambitious goals, but we are assured by the team that they are achievable.  While Freya has agreed to sign up to the plan in principle, the difficult part will definitely be maintaining her motivation.  For all those who know Freya well, you will know that perseverance is not a quality she is over-familiar with, and unfortunately, there is no easy path on this trip.
Thank you to everyone for your encouraging and supportive blogs. (and I'm sorry that the mechanics has been such a pain for so many), now that Freya is much more alert and bright she loves being read the messages.

Getting around

We are getting around the hospital now that Freya can get in and out of her wheelchair with more ease.  She is now able to stand with support and swivel herself into the chair.  The support she needs from us is more for balance, because she takes all her weight herself indicating the return of strength to her left leg and hip.

The number of places to visit within the hospital is pretty limited, but she has made friends with another young girl in the rehab. unit., Beckie.  Beckie and Freya are only a couple of weeks apart in age and have already formed a bond.  Like most of the other children in the rehab. unit Beckie was involved in a serious car accident and sustained head and spinal injuries.  She is doing well, although it is likely to be another 6 weeks or so before the "halo" that is supporting her spine can be removed. There are only about 6 or 7 children in the rehab. unit and all except for Freya and a 9 year old boy (who had a stroke) are victims of rural car accidents.  Talking to these families, we realise how lucky we are to be local to the RCH with a trip home for us taking 15 minutes.  For these families, home is several hours away, and some have not even been home since the time of their accidents weeks or months ago.

Freya's other favourite place to visit is the RCH Children's Garden which is beautifully designed and maintained by Sarah Baldwin's sister Julie.  Julie is an amazing individual and is famous throughout the hospital for her devotion to the garden and getting the kids involved in gardening activities.  We can't wait for the weather to improve so we can spend more time out there with her, her volunteers and the other children.

some sad days

As we were told would occur, Freya has continued to ride the emotional rollercoaster over the last couple of days.  The reality is, that despite pre-surgery counselling, an 11 year old child has limited capacity to understand and rationalise the long term effects of hemispherectomy surgery.  An 11 year old brain operates almost exclusively in the here and now. 
For Freya, the loss of movement in her arm and hand and the loss of her peripheral vision in both eyes are all causing her distress.  She is anxious and easily startled by movement and noises in her periphery that she can no longer see.  Apparently children do adapt to this visual deficit quite quickly, but in the context of her total loss of ability to self-care and function since the surgery, it is understandably an additional reason for sadness.