The end of an era

The time has come!

At Evie's lengthening last year her rods were reluctant to lengthen. After a check of her bone age and growth plates the surgeon informed us that the time had come for Evie to have her final spinal fusion.

Today we headed to Sydney for the surgery Monday afternoon.

It will involve removing the current Magec rods, then placing 2 x new rods, fixing them to her spine at multiple points and fusing her spine in place. It will mean no more growth for her spine, however the rest of her body may still lengthen.

It is bitter sweet. It is a very daunting prospect to have such a large and intensive surgery, for both Evelyne and I. However it also means no more lengthenings and no more surgeries.

For 7 years Evie was casted and braced to hold off for her first surgery. We were told we would only get about 5 years out of growing rods before her spine would spontaneously fuse so we tried to wait as long as possible. She managed through 7 years of Magec rods, with 3 sets of rods and 24 lengthenings. We are incredibly grateful she had no complications in all that time. Her surgeon says she is one of his top success stories!

Whilst we are both a bit of an emotional mess, it is a comfort to know we have a God who cares and has a plan for us. We hold close to that knowledge at this time. Evie's favourite verse is the one below:

"Do not be anxious about anything, but in every situation, by prayer and petition, with thanksgiving, present your requests to God. And the peace of God, which transcends all understanding, will guard your hearts and your minds in Christ Jesus."    Phillippians 4:6-7

Life with Beals in 2021-2022

 A well overdue update!

The last surgery at the beginning of the first lockdown feels like a lifetime ago. 

Surgery during lockdown was very difficult. We were in a public ward, with lots of noise, people coming and going at all hours, and limited space. It was difficult not being able to leave the room or have any visitors also. Yet again there were issues with managing the pain, but once we made it home all was well.

Evie recovered well and has continued to love music, art and had a great end to year 6.

She was one of the leads in our local musical in year 7 and continued to thrive in all areas. Yes she still required lengethings every 3 months, but all in all, life now looks vastly different than it did in the early days. 

Whilst initially worried about how she would cope at our local high school with it's large grounds and many stairs, she has managed them well with some adjustments. She is able to use a key for the school lifts, and we have arranged with her teachers for her to complete her work on an ipad rather than carrying a heavy backpack of books around. She is not a fan of school sport or PE, but loves the beach, walking the dog and dancing when no-one is watching!

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

'Normal' Life. 6 months in the life of Evelyne - end of 2019

I wrote most of this post before the global pandemic we are now in, but found it still in my drafts. So bear in mind, this was all from the end of 2019.

......................................................................................

I've had a number of people ask what life is like for Evie now.

As I've previously written, life was hard in the early days. Casts, braces, the unknown.
Life is so different now. Not always easy, but so much better.

Evie loves dancing, singing, performing. We discovered she can do all these things through our local Junior Theatre Company. She danced up a storm in her year 5/6 dance group in the local eisteddford, coming 2nd, and performed in the combined school choir at the Sydney Opera House.

There are, of course, still some difficulties
- 60% lung capacity means it is difficult sometimes to clear the chest when she gets sick, and we still resort to the nebuliser most winters.
- she has other joints that can be problematic such as her knees. This last year she has had a number of subluxed patellas (where her knee cap slips out of place, then back in again)
- as well as the regular lengthenings and xrays.

She rounded off 2019 being badged School Captain for 2020. A great achievement!

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!

Dr Min Mehta

Dr Min Mehta passed away yesterday in the UK. She was a pioneer in the understanding of infantile scoliosis and her research into scoliosis and treatment has changed the outcomes for many children across the world.

The below is an excerpt from the Infantile Scoliosis Project website on her life and achievements.

Min H. Mehta, M.D., F.R.C.S

“From the age of six, I always knew I wanted to be a doctor. That is true; it has never changed” says Dr. Min Mehta about her life’s work. “Miss” Mehta, as she is respectfully called by her British colleagues, is the world’s foremost expert on infantile scoliosis.

Miss Mehta tells the story of discovering her own adolescent scoliotic curve. “At about age 11 ½,” she recounts, “my parents and other adults began to tell me to ‘sit up straight.’ At last a family friend–a nurse–said, ‘the child can’t sit up straight – she has a scoliosis.’” And, Dr. Mehta observes, the process of detecting scoliosis hasn’t changed much over the years.

Dr. Mehta attended medical school in her native Calcutta, India. During the course of her training, she discovered that she was interested in surgery, and had a natural aptitude for it. After completing a three-year surgical training program in Calcutta, she moved to Britain to continue her studies and gain further qualifications. There, she took her Fellow Royal College of Surgeons (FRCS) examination, which is necessary to become a surgical specialist, intending at that time to practice neurosurgery.

Miss Mehta was required to study orthopaedics as well, to pass her examinations. She had the good fortune to work with Mr. Wiles and Mr. Batchelor, two of the foremost orthopaedic surgeons of the time at London teaching hospitals. As a result, she became so intrigued by orthopaedics that she changed her specialty focus.

Quickly, Dr. Mehta began to appreciate the challenge she had taken on. “It will be tough because you’re a woman,” one of her teachers cautioned. “But you must go for it, because you are good.” It was the 1950s. Miss Mehta, because of her gender, faced enormous difficulties securing interviews for the positions she needed to advance her career. There were few female surgeons practicing in Britain at the time, and the establishment was eager to maintain status quo. She made dozens of applications and received no calls to interview. Eventually, Miss Mehta received an application form that did not ask the formidable “gender” question. She returned it and, due to her impressive credentials, was invited immediately to interview. Although they were surprised to see a woman, it happened that none of the other three applicants for the post showed up for their appointments. She landed the position.

In 1968, Miss Mehta was the second woman in history to be awarded the prestigious Robert Jones Prize and Gold Medal in Orthopaedics for her work on “A Study of Infantile Scoliosis.” By then, she had already discovered the Rib Vertebral Angle Degree (RVAD), which is the standard and routinely accepted measurement approach employed today to determine whether a scoliotic curve is progressive. She had also come to understand the benefits of early treatment of progressive infantile scoliosis. However, the journals would not publish this work because they felt there was not enough evidence.

A year later in 1969, still struggling to climb the London surgical ladder due to her gender, Dr. Mehta returned to India. She worked in major teaching hospitals in New Delhi and Calcutta for five years. Her paper on the RVAD was published in the Journal of Bone and Joint Surgery in 1972, while she was practicing in India. In 1974, Miss Mehta moved back to England, stepping into a hard-earned and well-deserved position:
Senior Lecturer in Clinical Research at the Institute of Orthopaedics, University of London. She also served as an Honorary Consultant in Scoliosis at the Royal National Orthopaedic Hospital during this time, until 1992 (this is the British equivalent to the American “Chief of Surgery” position).

Since then, Dr. Mehta has continued her research on non-surgical methods of treatment for infantile, congenital and adolescent idiopathic scoliosis at the Royal National Orthopaedic Hospital. Although Dr. Mehta has “officially” reached the British obligatory age of retirement, she continues to passionately pursue her life’s work. She continues to see private patients. The paper, “Growth as a corrective force in the early treatment of progressive infantile scoliosis,” published in September 2005 in the British Editorial Society of Bone and Joint Surgery, chronicles years of infantile scoliosis case studies.

Now, Dr. Mehta knows, her challenge is to spread the message about her findings and methods; to educate as many doctors and to help as many children as possible. “It is easy to learn how to apply a plaster jacket,” she says about the casting technique she has perfected. “The trick is to learn to look critically at each child and understand how it can be assisted. The earlier you treat children, the greater the chance of making them straight. But all children are different: their build, tissues, size, and shape vary. Some can be straightened completely and, for some, it may be too late. They are like little drooping flowers. Braces don’t work, and the flower will just flop back to the way it was. It needs to get is own sap, so it can hold itself straight.”

“I intend to keep on and on, to see that these messages are delivered,” Miss Mehta insists. And the Infantile Scoliosis Outreach Program is determined to help her achieve that goal.
Copyright 2017 Ability Connection Colorado. All rights Reserved

Growth as a corrective force in the early treatment of progressive infantile scoliosis 

by Min H. Mehta, M.D., F.R.C.S. Published in The Journal of Bone and Joint Surgery (Br) Sept 2005

The story of Mehta casting being brought to US
The Greatest Gift - Dec 19 2000

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"

A year on with MAGEC rods - A 'work in progress'

It's been a year since Evelyne's surgery and over a year since I last posted. So much has happened last year. Thank you to those who have sent messages asking about her progress. Some of this was written 12 months ago and has been sitting in my drafts.

Magec Rods (MAGnetic Expansion Control)
On January 7th 2016 Evelyne had surgery to implant magec growing rods. We had been awaiting this day for years and the time finally arrived.  Surgery was discussed as the next option in May 2011, but I sought out a new specialist with new options such as Mehta casting who encouraged us to wait as long as possible before operating. Waiting the extra 5 years not only gave her body valuable time to grow, but it allowed us to have access to the amazing technology of Magec Rods. They have a small motor in each rod which is activated via an external remote control. This enables the surgeon to lengthen the rods as Evelyne grows without re-operating each time. Dramatically reducing complications associated with frequent surgeries as required with traditional growing rods.

Surgery
Leading up to the surgery we were all nervous wrecks. I had little sleep before and so many doomsday scenarios ran through my head. But Evelyne was so brave going in, and then we had to wait.
4.5 hours later her surgeon came out and told us he was finished. He was happy with the final results but reported that it had been "tricky". The kyphosis hump had become quite rigid (a negative side effect of waiting longer) and he had to chip some parts of the bone to get it straighter.  Also, once in place, when beginning to stretch the rods, one of the screws started to come out of the vertebrae.  A bone graft and a few extra secures later and he was confident in the end result. He informed us that he saw her as a "work in progress", with this surgery being a stepping stone in the process of straightening her spine over the years to come.

Recovery
I wasn't prepared for the pain she would be in post-surgery. It was so hard to see her lying there and I was completely powerless to help. I probably hadn't prepared her for it either. It will be hard to forget the look in her eyes when she looked at me and said "Mummy, you said 'a bit' of pain". It was a long week. 
We made the most of movies on the ipad to pass the time. Another mum I have 'met' via facebook, has written some really helpful things down on preparation for hospital in her blog post.
A major milestone was walking again on day 4. With the main motivation being using a toilet rather than a bed pan. Then on day 8 we left the hospital to spend another week at my parents place before the longer trip home to Bathurst 2 weeks after the operation. 

Other things we had to put in place post surgery 
- A bed lower to the ground. 
- Sleeping in our room for the first few weeks as she needed help to move in the night
- Help on the toilet
- Pillows. Lots of pillows. In the car, on chairs, in bed.

Longer term recovery
Again, I wasn't prepared for how long it would take Evelyne to recover. I had thought about the short term, but not the long term implications. 
She returned to school for the start of the new school year, 3 weeks after surgery. However she was still on strong pain medication, and would fatigue very quickly. Due to the bone graft, she also had added restrictions in the classroom and playground. We put in an application for additional funding at school and were fortunate to be provided with a full time aide Deb (pictured right). Amazing grandparents also spent 2-3 days per week with us for the entire Term 1, so I could continue to work. 
By term 2 she was off pain medication, we no longer needed our parents help through the week, but she was still reliant on her aide and her wheelchair for school.

Wrapping up the year
Now this all seems like a distant memory. Although she still has restrictions, occasional pains, discomfort and fatigue. By the end of 2016 Evelyne was able to participate in some of her school athletics carnival, was a star on stage singing and dancing at her school talent quest, and won an end of year academic achievement award. She's pretty special.

Last brace!

Sometimes it can hard to put a positive spin on things. But often that's the best option.
Evelyne will have her first spinal surgery on the 7th January. It is a major surgery in which they will implant growing rods, attaching them to her spine. I'll write a longer post on this process soon.  
Although we are a bit apprehensive, we know that the time is right. Evie's last x-ray showed that her curves have reached 68 degrees and 49 degrees IN the brace. As a result of this progression she has been experiencing more discomfort and pain, and is finding more tasks and positions difficult. 
It is time.

We have so much to thank God for.
- It's been 4 years since I first thought surgery was imminent as I wrote about in this post
- She is now eligible for Magec rods which I first wrote about here in 2011 and hoped would be an option 
- This is the last brace! And what 7 year old Taylor Swift fan can resist heart and rose tattoo print

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

A catch up and a birthday

It has again been a long time since I've posted. Thank you to those who sent lovely messages asking asking how we were going. In my last post Evelyne was in a cast and we were praying she could last until the 6-week check without developing pressure sores. 
And she did! 
It was a hard 6 weeks, and we went through various times when we thought we might need to cut the cast off. I always find it difficult to know what to do. As you cannot see what's under the cast, it is up to us to decide if we think she has a pressure sore or not. Which is ultimately based on if we think her pain and discomfort is increasing. 
She got the cast cut off at the follow up appointment, revealing a few surface sores developing, but no need for dressing. It was wonderful news!

The next day she was cast for a new brace and then fit the day after that. The quick turn around this time was great, with her new brace x-ray measuring the same as her cast x-ray at 53 degrees for the lumbar curve in the brace.

Note that her new brace is plain purple this time, as she is apparently now too grown up for butterflies and bunny rabbits.









MY BIG 6 YEAR OLD

This week my 'grown up' girl celebrated her 6th birthday. I've included a couple of photos from her 'Frozen' themed party with school friends.