More challenges in troubled times, another Spinal Surgery!

We found out a couple of months ago that Evie has almost grown out off her current set of spinal rods. It was not a surprise, and we were pleased to be able to pick the date for the surgery we wanted, mid-year, to fit in with school holidays, and a good amount of time to recover before end of year activities and high school.

As Australia followed the rest of the world into shut down due to Covid-19, like many others, I've been struggling with the new reality of having the kids learning from home, whilst I try to work from home as well. There was also the added worry of keeping Evie away from the virus due to her lung condition.

Then a phone call came. All elective surgeries had been cancelled. However Evie's spinal surgery did not fall in that category, as she would not last indefinitely without the new rods to allow her body to grow. As such, her surgery was made Category 1, and we had a new date, April 17. This added another added layer of anxiety.

Of course, as many of you know, the prospect of surgery in itself is extremely hard. Those who know us, or have read previous posts would know that the pain for Evie is extrordinary, and me watching her, it is awful. Add in the additional risk of Covid-19, a hospital in lockdown which means Jon and Sam can't visit, not being able to see grandparents, or stay with them after for more recovery time, all this is overwhelming to say the least.

The last couple of weeks have been hard, and will be until after this is all over. So many aspects about what is going on at the moment are completely out of my control. All I can do is trust God that Evie is in His hands. Whilst hard, it is a comfort to know I need not fear, but I can call out to Him in hard times.

This Psalm from the Bible is so comforting right now.

Psalm 46

1 God is our refuge and strength,
    an ever-present help in trouble.
2 Therefore we will not fear, though the earth give way
    and the mountains fall into the heart of the sea,
3 though its waters roar and foam
    and the mountains quake with their surging.

4 There is a river whose streams make glad the city of God,
    the holy place where the Most High dwells.
5 God is within her, she will not fall;
    God will help her at break of day.
6 Nations are in uproar, kingdoms fall;
    he lifts his voice, the earth melts.

7 The Lord Almighty is with us;
    the God of Jacob is our fortress.

8 Come and see what the Lord has done,
    the desolations he has brought on the earth.
9 He makes wars cease
    to the ends of the earth.
He breaks the bow and shatters the spear;
    he burns the shields with fire.
10 He says, “Be still, and know that I am God;
    I will be exalted among the nations,
    I will be exalted in the earth.”

11 The Lord Almighty is with us;
    the God of Jacob is our fortress

June - Scoliosis Awareness Month

Yet again I've left it over a year between posts! There are probably many reasons. Busyness, less changes in Evelyne, more stability as she ages, or maybe I don't 'need' to put my feelings and thoughts down like I used to. Not sure. But I have been again reminded recently how helpful my blog has been to others over the years. I started this blog as there was little to no info out there about Beals Syndrome. Now 8 years later not much has changed. There is still barely anywhere to find out about Beals Syndrome, and even less helpful information for a new parent faced with an unknown diagnosis.

I am frequently contacted my young mums and dads faced with a baby who doctors 'suspect' has Beals Syndrome. They turn to google to look for answers to no avail, just as I did. It brings me straight back to the uncertainty I felt. Holding my little baby and unsure of the future. I have been encouraged by them to continue writing our story down, so here I am!

Evelyne - enjoying mushroom picking in the forest this Autumn

I have spent the last month looking back through photos as I was thinking of writing a post about how far Evie has come. But where do I start! The early years of casting and bracing, the photos of her in appointments and procedures, or the photos of her showing her determination to be a kid despite her physical struggles. She has been through so much and continues to amaze me.

With June being scoliosis awareness month I am again motivated! Whilst there is so much more to Beals Syndrome, for Evie, her scoliosis has been by far the biggest impact to her life.
When she was just a tiny baby, 10 weeks old, I noticed a bump on her back and realised something was not right. It did not take long before we realised she had a rapidly progressing kyphoscoliosis, which meant her spine was curving outwards as well as sideways. 

Over the past 10 years Evie has gone through 4 casts, 9 braces, 2 major surgeries and 12 lengthenings. The years of casting and bracing were not easy with the effect to her lungs, mobility and energy levels. Whilst surgeries and lengthenings are also hard and risky, Evie is now growing taller, stronger, has more lung capacity and thrives in so many ways. You would not know what she has been through to see her now, and many new friends are astounded when I share her story. She loves singing, debating, public speaking, musical theatre, art and craft, and brings joy to so many people. We are so grateful for the amazing medical assistance we get here in Australia, our wonderful Orthopaedic Surgeon and the new technology that is available.

Broken Bones

At the end of the January school holidays Evelyne broke her arm. Not just any break of course! A complete displaced fracture of the upper humerus, almost compact, but thankfully didn’t quite make it through the skin! 

It was an ordinary day at the park with Evelyne doing an ordinary activity for a 9 year old – climbing on monkey bars. 
It ended with her on the ground, an obvious serious injury and a Triple Zero phone call for an ambulance. Leading to transport to another hospital, a 'closed reduction' in theatre under general anaesthetic, 3 days in hospital getting pain under control, 2 weeks in a traction cast and a further 4 weeks in a shoulder immobiliser sling. The positive news was that her spinal rods were not impacted!

It’s one of those difficult questions parents with Beals kids (and others) will face. How much do we protect and wrap in cotton wool, and how much do we let them go and be normal kids? It is a balance I’ve struggled with over the years. Sometimes I think I’ve restricted her too much due to worrying about injury. But the fact is, she is going to be at a higher risk of all sorts of injuries. Joint injury and dislocation, risk to damaging spinal rods, and it appears broken bones as well. At school she is not allowed on the play equipment, is restricted from many sports activities and has an aide to watch her in the playground. This has made it hard for her over the years, as she at times comes home from school upset that she was left out of the action. At home, we are slightly more relaxed, and really just ban trampolines, bouncing castles, and I may add monkey bars to the list.

Whilst I’ve never had definite confirmation, it does appear that Beals Syndrome is associated with lowered bone density. One study I read said 'generalised osteoporosis is well documented'. Many years ago, another Beals mum spoke of bone density testing, so I had Evelyne’s checked. At the time her results came back as normal. Apart from some minor reduction in the spinal column, but that was explained by lack of use as it was in the days of her being in casts and braces. From my reading, bone density can often be lower in individuals with reduced physical activity, reduced strength activity, low body weight and small frames and that physical activity is widely acknowledged as a factor in increasing bone density. So perhaps the lowered bone density in Beals Syndrome is by association with lowered activity and low body weight. Either way, it still brings me back to the same conundrum. 

Evelyne is now 12 weeks post break and her arm has healed beautifully. She is about to start physio to get full range of motion back in the shoulder and is looking forward to starting back at Jazz dancing. In regards to my thoughts on physical activity, I'm happy with most of our decisions with restriction vs allowance. In reality, she is often much happier doing drawing and craft than sports and she enjoys her dancing which she will continue. I do think there is more we could do for joint protection rather than just avoiding certain activities, as her knee has partially dislocated twice. Evelyne is fortunate to have been granted an NDIS package (National Disability Insurance Scheme in Australia). With some of her funds she will start seeing an Exercise Physiologist to help strengthen her joints, increase endurance, as well as some hydrotherapy and swimming. We are both looking forward to seeing the benefits. 

Second surgery - new rods

Two weeks ago Evelyne had another major surgery to remove her old Magec rods and insert new longer rods. Since her first surgery, Evie has been lengthened every 3 months, and reached the end length of her rods sooner than expected. A result of a Beals Syndome catch 22. Whilst the flexibility in the connective tissue is what made Evelyne's spine super bendy in the first place, the flexibility also meant her surgeon could straighten her relatively quickly, thus running out of length.

Leading up to the surgery Evie (and myself) were very anxious. Unfortunately having been through it before meant we were not looking forward to the surgery or aftermath. Anticipating the pain she would be in was the main worry. We distracted ourselves by planning a brilliant 9th birthday party the weekend before. As well as a bit of activity planning, relaxation oils, music downloads and lots of prayer.

The operation was longer than expected, so Jon and I were pleased when the surgeon emerged to tell us it was all over and she was in recovery. He informed us that much to his surprise, the existing screws had come loose in her spine. In his wording, two were extremely loose and one was "swinging in the breeze"! He was surprised that she had not been experiencing associated pain. We had hoped the surgeon would be able to just replace the actual rods, however, he had to remove all 4 screws, with 3 larger diameter screws, whilst having to drill a new hole for the 4th. He was happy that the anchors were now very secure and as a result she won't have the same restrictions as her first surgery. He was also confident that her recovery would be quicker than last time. All in all, very positive news.

The following days in hospital were still painful and long. Long days and nights with frequent repositioning required. Evie also had some issues with pressure injuries this time, but as an OT, I'm glad to be in the right industry to know how to deal with these! We were prepared with movies, music and audio books, and the DVD stand was again a godsend. Overall, everyone was pleased with her progress and she was able to get up and walk earlier than last time. The surgeon was happy with her x-rays, so we left hospital on her birthday with a bit of determination to get out of there! 

Heading home

For the last week Evie and I have stayed in Sydney, at our 'home away from home'. My parents have opened up their home, and in fact partially designed their house with Evelyne's medical appointments and recoveries in mind.  I am so grateful to have this provision, and my mum to look after us! We have enjoyed many hours of colouring, visits from cousins, craft activities, movies, and our now tradition of her first outing to Cold Rock Icecream. It has been a smoother process this time, knowing what the main issues would be. So were well set up with a lowered bed, lots of pillows, shower chair and toilet rails brought from home. 

Today we have finally made the trip home to Bathurst. Dosed up on pain meds and lots of pillows, it is always sad to leave my parents, but will be so good to be home!

Spinal Lengthenings ..... and an update

One of the major benefits of Magec rods is the fact that they can be lengthened externally. Each rod has a small motor that is activated magnetically via remote control and will expand the rod to a predetermined length. This means that the rods can lengthened to suit the amount she's growing, as well as the surgeon being able to lengthen one side more than the other to gradually correct the spine.

Evelyne has been having lengthenings every 3 months since the rods were placed in January 2016.
Whilst it is so much better than surgery, it is still painful. The first lengthening was especially painful. Possibly due to the length of time since surgery, possibly due to recent bone work and bone grafts, possibly her spine may have been starting to fuse in places. Yet again we weren't prepared for it to be painful. I had been told by other parents that lengthenings were pain free, but unfortunately this was not our experience. Each time the pain has lessened, and now we give her pain medication before we go.

Today was Evelyne's 6th lengthening. To our surprise, it turns out today was the last lengthening for this set of rods. As she was so small when her surgeon first inserted the rods, he was only able to fit the smaller length in. We had thought we had a bit more time before the next surgery, but it is now to be scheduled for September. The good news is, the doctor anticipates that he will be able to keep the current screws in place and just replace the rods to the longer version. This will result in a slightly shorter surgery, and hopefully shorter recovery.

A few tears from Evie today as she thought of another surgery. Last time we saw the surgeon and he mentioned another surgery soon, I said "I only just got over the last one!" To which Evie replied "I still haven't gotten over it!

I was recently reminded of a previous blog post of mine Dealing with disappointment. Whilst some appointments and parts of this journey are disappointing, it is good to remind myself how far she's come since 2011, as well as remind myself that God is indeed in control, and find comfort in that.


A painting Evie made for her doctor today. He affectionately calls her "E.B"

New Wheels Arrive!

Thanks to the amazing generosity of so many people, the 'Wheels for Evie' wheelchair arrived last week. There was a lot of excitement (from all the family).
We are so excited about this chair in so many ways. It is a manual (Zippy) chair base, but has a power attachment (ZX1). As a result we have two chairs in one. This means we can still use the lightweight manual chair and fold it up in the car, but that she can also independently attach it to a power base. 

This amazing chair gives her independence and control over her own mobility. Yet we don't have to have a modified car like with most power wheelchairs. The school has paths and ramps so she can access all areas. The main issue we have to deal with is stopping her from increasing the speed and basic driver training! She wouldn't let me put 'L' plates on, but there have been a few moments I felt they were needed. She's perfecting wheelies and donuts.

We are still astounded of the generosity from friends, family, church, school, community, anonymous donations, Lions, as well as complete strangers. We are very thankful to God for the amazing supports he has given us.

First trial                         -                         Off- road                         -                       Watch out Sam!

Welcome to Holland

c1987 by Emily Perl Kingsley. All rights reserved.

I am often asked to describe the experience of raising a child with a disability - to try to help people who have not shared that unique experience to understand it, to imagine how it would feel. It's like this…

When you're going to have a baby, it's like planning a fabulous vacation trip - to Italy. You buy a bunch of guide books and make your wonderful plans. The Coliseum. The Michelangelo David. The gondolas in Venice. You may learn some handy phrases in Italian. It's all very exciting.
After months of eager anticipation, the day finally arrives. You pack your bags and off you go.
Several hours later, the plane lands. The stewardess comes in and says, "Welcome to Holland." "Holland?!?" you say. "What do you mean Holland?? I signed up for Italy! I'm supposed to be in Italy. All my life I've dreamed of going to Italy."

But there's been a change in the flight plan. They've landed in Holland and there you must
stay. The important thing is that they haven't taken you to a horrible, disgusting, filthy place, full of pestilence, famine and disease. It's just a different place.
So you must go out and buy new guide books. And you must learn a whole new language.
And you will meet a whole new group of people you would never have met.
It's just a different place. It's slower-paced than Italy, less flashy than Italy. But after you've been there for a while and you catch your breath, you look around.... and you begin to notice that Holland has windmills....and Holland has tulips. Holland even has Rembrandts.
But everyone you know is busy coming and going from Italy... and they're all bragging about what a wonderful time they had there. And for the rest of your life, you will say "Yes, that's where I was supposed to go. That's what I had planned."
And the pain of that will never, ever, ever, ever go away... because the loss of that dream is a very very significant loss.
But... if you spend your life mourning the fact that you didn't get to Italy, you may never be free to enjoy the very special, the very lovely things ... about Holland.

The time is drawing near

Evelyne had a spinal appointment this week. We have been plodding along since last winter with the current brace, but can tell she is growing more uncomfortable. Evie has had a few issues with redness and pressure on the point of her kyphosis, and occasionally issues with pain, but it's been pretty non-eventful. Every time we see the specialist we get asked "...and how old is she now?"... with the underlying question being "is she old enough for growing rods yet?". This time was no different, however, I think I knew that now Evie is aged 6 we are getting closer. 

They first mentioned surgery when she was 3, and we have persisted in casts and braces for the past 3 years to try to hold off until she is bigger. Basically, the older/bigger they are, the less complications and the more successful the surgery.

The specialist is wanting to try another brace design first, to see if we can hold off slightly longer, however it looks like surgery will be in the near future rather than the distant future. In his words "I'd rather put growing rods in an 8 yr old than a 6 yr old, however we have done well to be putting them in a 6 yr old rather than a 4 yr old". We have a brace review at the end of January to discuss options for a new design which might help, however it is clear surgery is not too far away.

Evie, brace free, with her cousin Lydia

One positive is that it looks like she is eligible for Magec rods - magnetic growing rods. This means she will not have to have surgery every 6 months, but the rods can be lengthened via magnets. I wrote about it here over 3 years ago when I heard of it, hoping it may be an option for Evie. 

I am nervous for what the future holds, but I trust our specialist and his experience, and I am happy with how long we have managed to delay surgery so far.

Wheels for Evie

In past blog posts, I have mentioned that Evelyne uses a specialised pram for school and outings. She has joint pain issues, spinal pain, low muscle tone, fatigue and reduced lung capacity due to her scoliosis. In summer months, she becomes quite exhausted from the heat due to the brace. Her school use the pram towards the end of the day, or when they have to go further distances within the school, such as the oval or the library. We use it on holidays, for hospital visits and at the shops. It has been an great help to both reduce her pain and fatigue, and save our backs from carrying her as she gets older.

Recently at school she has been teased about being in a pram, "only babies go in a pram". This is understandable as a pram is inappropriate at her age. As a result we have come to the realisation she will need a wheelchair. This was particularly apparent at her recent sports carnival. Her teacher was kind enough to race with her around the oval, but it was very obvious she had difficulty participating in many of the activities, even though she tried so hard to be involved.

The first wheelchair trial was confronting. She at first refused to get in it, she said it was too 'scary'. Once she agreed to try it, I also got quite emotional seeing her in the chair. It felt like such a big transition. While she was young, we could hide her disability in the pram. Now  it is more obvious. After an hour of trialling the wheelchair, she realised using it gave her independence. This was by far the biggest impact. She loved that she could get around herself, without someone else having to push her.

The hospital lent us a chair to trial which gave us the opportunity to see if it would be appropriate for school and home. After a successful trial, we have now started fundraising to purchase her own chair. In Australia we can apply for funding through the government, however, as she can walk, she is seen as a low priority category. This means we could be waiting approximately 2 years for the chair. 

Some of my mum's gorgeous clothes

My mum made a huge amount of clothes to sell, and various people donated clothes for a facebook sale site. On top of this, we have been blown away with the generosity of people within our church, school, family and local community, as well as some people we have never met.



Wheels for Evie facebook site

Handmade girls clothes

Girls auction

Boys auction

Donation site

Another winter, another cast

Last Friday Evelyne had another spinal cast applied in Sydney.
It is always tough leading up to casting, emotionally preparing for pain, discomfort and sleepless nights. I am usually a mess the week before. And this time was no different. But as usual Evie was relatively calm and although she admitted she was not looking forward to the cast "because it hurts more, and you cant take it off!", she was her normal happy, positive self before (as you can see from the photo).

She recovered after the general anesthetic much better than her last cast, and was able to stand and walk quicker also. However she still needed pain killers and was in alot of pain if they wore off. She also slept very well the first few days and managed the 3 hr car trip home to Bathurst the next day with only one stop. 

We again decorated her cast in her choice of print, and her doll Milly (now renamed Annabelle) again shared the same design.

One week on she is still struggling to sit for periods of time and prefers to stand, kneel, use a bean bag or lie on her tummy. And at times she still needs pain medication. Evelyne attempted school for a few hours each day Tues-Fri, with the help of her aide, and although the first few days went well she only lasted half an hour Friday before her back was hurting too much and she needed to come home.

I'm not sure what the next few weeks will hold. It's hard not thinking that she may already have a pressure sore, but maybe she is still just adjusting to the change in her spine position. I recall it taking about a week to recover last year also. We are also awaiting the x-ray results to see what angle the cast has achieved. In the meantime we continue to pray for her to be more comfortable and that this cast will last longer than the others.

Starting school

Evelyne started school last month and she is loving it! She is attending our local public school (same school as Samuel) which is only a short walk from our house. Incidently it is also the same school I started Kindergarten in almost 20 yrs ago. She is in a standard Kindergarten class with a part-time aide and some extra equipment to help her.

This includes:
- Her pram/buggy, to get to school, and to leave at school for when the kids walk longer distances or she gets tired
- A cushion for her chair as she was experiencing pain on her kyphosis when sitting
- A peanut cushion for sitting on the floor, to avoid 
W-sitting
- A splint for her wrist and thumb and a pencil grip
- A beanbag for floor sitting when tired

We are also in the process of organising a step and rails for the toilet (she is in a classroom with it's own toilet). This will be mainly needed for when she is fitted with another cast in April.

Her main issue, as we expected has been fatigue. As expected she has friends, loves classwork, loves her teacher, and is very independent. However she is exhausted by the afternoons. Although all new school kids experience this, with the added difficulty of her lung restriction, she was struggling. The first few weeks were especially difficult with the heat. The teacher and aide have been using her pram for longer distances, but have found they were even needing it for the short distances also. As a result the school have applied for more funding so her aide will be available more hours. This again, will be especially helpful come April, when she will need more assistance in the cast.

I was a mix of emotions leading up to her beginning 

school. Some excitement, some nerves and worry. It was hard to hand her over to others for so many hours in the week. However I am very happy that the school have been so helpful and I am confident that they are looking after her.

Our local paper did an article in her first week of school. It was a great article you can read the online version here

The 'skinny' gene

All ready for a recent ballet concert

One of the known features of Beals Syndrome is long slender limbs and fingers. From what I've learnt from other Beals families, this is one of the most noticeable outward factors of Beals and many speak of comments made about long fingers, arms, toes etc. It appears to be linked to the genetic mutations in fibrillin-2 (Beals) and fibrillin-1 (Marfan) as both conditions display factors of increased height, armspan can be greater than their height, and slender fingers (arachnodactyly). Beals Syndrome was previously known as Congenital Contractural Arachnodactyly.

From birth we noticed Evelyne's long feet and long fingers. Even before we knew she had any sort of genetic condition we were aware of their unique shape. One nurse commented at birth "look out Thorpe" (the Aussie swimmer) while we had numerous comments that she "would be a piano player". As she grew we could see more and more, not just long slender fingers, but her overall long, slender shape.

Until recently it has not been a major concern to us as we knew it was a common aspect of Beals. However a trip to the nutritionist, made us realise we need to be striving to put more weight on her. Due to her scoliosis, and her future need for surgery, it is important to increase her body fat. At present she is so skinny, and due to her kyphoscoliosis, her spine is very prominent. This means she gets frequent pressure issues, but it may mean more complications in surgery. The surgery involves rods and bolts to attach, and basically, they just need more flesh to work with to cover over all the hardware.

She does not eat huge volumes of food, possibly due the restriction on her stomach from her brace and scoliosis. So our first attempt involves adding calories without adding much volume. Her new diet is everything I would normally associate with a poor diet. But I've had to change my mindset. As the nutritionist said, for the next few years, sugar and fat are our friends. For every meal I have to add a high fat or sugar content to Evelyne's serve. I either add oil, butter, margarine, cream, sour cream etc. I know of other kids with Beals Syndrome who have not put weight on by diet alone. They have ended up with PEG tube feeds in an attempt for weight gain. This is a good motivation to work hard on her new 'diet'.

Busyness and daily struggles

I'm sure most parents will agree that life with kids is busy. But those of us with kids with special needs will know that the busyness triples at times. I've realised it has been over 2 months since I last posted and I was trying to think about what had happened in the past 2 months. I feel like its been a whirlwind of work, housework, church, meetings, kids sport, ballet. But then added on top is OT appointments (for Sam as well as Evelyne), physio (for me as we'll as Evelyne), GP (for all 3 of us), paediatrician, rehab specialist, orthopaedic specialist, spinal surgeon, dietitian, orthotist, yearly visits to the cardiologist, ophthalmologist, and I may have missed one or two.

I'm not sure how to avoid this busyness.  Sometimes I don't know how long I can continue the relentless appointments and treatment. I have wondered at times if Evelyne really 'needs' to see all those people. Or if I should work on one thing at a time. But unfortunately the nature of Beals is it affects so many different parts of the body. Connective tissue is everywhere! And appointments with spinal specialists and orthotists are very important to keep on top of when dealing with a worsening scoliosis. 

As well as busyness in our daily scedule, there is also the tendency to feel overloaded emotionally. I tend to go through stages where i feel very overwhelmed and out of control. I hate watching the daily struggles that Evelyne goes through. When she is in pain while her body adjusts to a new brace or cast,  when she struggles with sickness and chest infections and we have to force her to use the nebuliser, or simple things like her struggle to run or do ballet steps like her friends.

I have come to realise the importance of friends and family who understand. The times I can cry on a friends shoulder, have a cuppa and some chocolate and a debrief. It is so important to have these people in my life for support when I do feel down and out. To have their emotional support as well as practical support when I need it. I am very thankful to God for the family and friends he has given me who are such an encouragement in the tougher times. 

Another short-lived cast

On Friday we had Evelyne's cast cut off. It had been on for 5 weeks this time, so I was happy that it was a longer stint than the last one. We had three good weeks after she got used to the new cast. She was happy, independent and enjoyed playing at home and preschool. However over the last week she started complaining of pain. In particular she found sitting in chairs and in her carseat difficult, and she started waking in pain through the night also.

As a result we had the cast cut off locally. It was a very hard decision, as we really wanted this one to last longer. The ordeal of travel, general anaesthetics and xrays mean that the longer a cast can last, the better. However the change in her mood was demonstrating that something was wrong. We found that again there was a pressure sore on the point of her kyphosis. In the same spot as the last one. This sore was slightly wider, but thankfully not as deep as the previous sore. 

It is disappointing, but I am glad that we made the right decision. Leaving it longer would have meant a deeper sore, and a longer time for it to heal without any sort of support. On the positive side, Evelyne is enjoying her 'freedom' and she can now put on her shoes and socks by herself. The sore is healing well and we may even be able to put her brace back on her this week. Plus the first bath in 5 weeks was exciting!

P.S. Milly had her 'cast' cut off too. Evie talked her through the procedure and she was very brave.

Sleep Studies

Late last year Evelyne had a sleep study done as a follow on from her Restrictive Lung Disease diagnosis. They wanted to check that she was getting enough oxygen through the night.

After we checked in at Westmead, we got our beds set up and PJs on. Then the Sleep Technician came and put a series of wires and electrodes over Evelynes Head, face, and chest. They attached them with a paste much like toothpaste which was interesting to get out of her hair the next day! We then had to put the brace back on over the top. This was probably the trickiest part, as it was difficut to make sure the electrodes were not digging in too much underneath.

All up it was a relatively positive event. Much better than what I expected, and Evie coped much better than I expected too. There were a few hours in the early morning when she woke and was upset, and the Sleep Tech's had to come in a few times to check on her, so we were a bit sleep deprived the next day. 

We recently saw the doctor for the follow up. He was very pleased with the results. He had no concerns in regards to her oxygen levels at night. A great outcome!

She can do anything!

I have previously been told that Evelyne may not be able to ride a 'normal' bike. This was thought due to her low muscle tone in her legs as well as her lack of core strength due to the scoliosis and brace. So when we went for walks we pushed Evelyne in a younger child's trike which had a parent controlled handle to push it. I wasn't too concerned as I knew of a company TAD, that helps modify bikes for older kids. I just assumed we would draw on their services one day.

Recently, a friend offered a bike for sale with training wheels and a parent handle. I thought, that's perfect! It's a standard sized bike, but we could still push her along.

We took her for her first ride, expecting to be pushing her along, and you can see the results from the video clip below. I'm not sure who was more excited, Evelyne or me.

Another lesson to never underestimate my girl!

Song : Jay Laga'aia - Bikeling - (used with permission)

An interview ...

My sister recently interviewed me for her blog on my experience living with a child with a disability.

A child with a disability - My sisters story

Siblings

Over the last couple of years I have been thinking about siblings of kids with disabilities. I have met many people over the years, through work as an OT and personally, who have talked of the effects of growing up with a sibling with a disability. I have also recently read the book 'Siblings' by Kate Strohm. 

The impact appears vary case to case. Many people report to have issues with feeling left out, forgotten, not as important. Some report that it strengthened their character, and they feel they are more tolerant, understanding people as a result. In Kate Strohm's book she says siblings are 'often overlooked in families struggling to cope'. She gives some practical strategies to help give siblings the attention, care and time they need.

A friend of mine once told me that as a child, her main memory of her sister's disability was that her mum would leave her so often. When she would travel away for medical appointments she felt she was always the one left behind. This is something I am very conscious of with Samuel. I am often away with Evelyne in Sydney, and now Samuel is at school, he stays at home with Jon. Jon's parents often come to stay to help with school drop off and pick up which has been wonderful! However I feel it's important to make sure Sam and I get one on one time also. 

As a result last school holidays Sam and I had a special trip together. We caught a train to the Blue Mountains, bush-walked down the giant staircase and around to Echo Point, stayed in a motel, went up and down the scenic railway and cable car, and bush-walked some more before catching a train back home. Samuel took my old camera and took pictures along the way, and made a scrapbook afterwards with photos and brochures and maps. We had a great time together and he was so excited that he got to come on a special trip with me. It was a wonderful 'adventure' together.

I'm a big girl now!

Evelyne recently celebrated her 4th birthday. She is very excited to be 'big now'. We had a lovely 'rainbow party' for her in a Sydney park with both sides of the family. She ran around, played in the sandpit, and enjoyed the fact she is cast and brace free at the moment.

Curve progression and casting

Evelyne's last x-ray in her quad brace showed that the curve had again progressed. It was not a huge surprise to us, as we could visually see the spine not staying in place in her brace.  Her scoliosis had progressed to 57 degrees in the brace. As a result we decided to go ahead with casting while we are still in the cooler months. I was quite optimistic about how the casting would go this time around. She is older, walking, not in nappies, feeding herself. All factors that made the cast when she was 12 months difficult. I continue to forget however that each new step brings it's own challenges!

Last week Evelyne was fitted for the new cast.  It was done by our new specialist on the 'risser frame' in the same style as the 'Mehta' casts. She was put under a general anaesthetic and they needed at least an hour to mould it to shape.

Waiting                                                                     Recovery

Leaving the hospital

The next few days were very hard.

Day 1 was especially as she struggled to learn to balance again under the weight. Simple things like walking and sitting up were difficult.

By day 2 she was up and walking and even trying to run a little, but still unable to get on and off the toilet herself, turn over in bed, and get on and off chairs.

Day 5 I took her back to our local physio to trip some of the plaster from around her arm holes to improve movement in her shoulders.

Day 6 She was at preschool playing in the playground with the other kids.


Today, 10 days on and she is her normal happy self. 
She is sleeping in her own bed, playing, running, dancing (although she still needs help for the toilet).  She copes better than I do when she goes through a new stage. 

It takes me a while to get used to seeing her struggling. It is also hard getting used to seeing how bulky it is on her. However she has the determination to work at it until she achieves what she wants. She's my little trooper!