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.

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

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.

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'.

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.

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!

Respiratory problems

A couple of months ago Evelyne was diagnosed with Restrictive Lung Disease. It came as a bit of a shock, although we knew there were issues with her lungs. We were sent to a respiratory (lung) specialist for a check up after I identified she had undergone more than her fair share of chest infections last winter. She also often sounds a bit wheezy, finds it difficult to cough strongly and can get quite breathless. I had always assumed these symptoms were to do with the restriction the brace puts on her chest cavity.  The specialist informed me that he would categorise her as having Restrictive Lung Disease, and that he believes this is due to her scoliosis. He believed the curve was reducing the space available for her lungs to function normally. Although our spinal specialist believes the restriction may be more from the effect of the tight brace.

Since this diagnosis Evelyne has been using a nebuliser daily. She has hypertonic saline in the nebuliser in order to reduce the build up of mucus in her lungs. It encourages her to cough and clear the airways.  It is again not something she enjoys, but something we have had to build into her daily routine. She had a couple of chest infections at the beginning of winter, but they seem to have slowed and be less severe than last winter.
We also have been using a pram more to reduce her breathlessness, and have applied for a specialist pram through the hospital.

Deformity Downunder

Evelyne was presented at another international conference earlier in the month. The conference had the unconventional title "deformity downunder". There were specialists from a few other countries including the US and New Zealand. Evelyne's 2 x spinal surgeons were present and outlined her case for the participants. Like the last conference, they presented her case on powerpoint, then gave specialists the opportunity to come forward and examine her.


Yet again Evelyne was amazing, and dealt with the experience very well. She did everything they asked and charmed them all with comments like "there's a lot of doctors here" as she looked around the room of about 30.  About 10 doctors examined her this time, crowding round. It is a strange experience, to see her on display as they crowd to check her out, yet valuing their expert advice on her case. There was a discussion on her current brace and its effectiveness and a discussion from the US specialists of the style in comparison to some braces used in the US. We then left the room and left them to discuss further.


This week we received the followup phone call from her specialist informing us of their conclusions. Overall they are very happy with her current brace. The plan is to make some slight adjustments at our next clinic in June. Thankfully there was no talk of surgery for the immediate future.

Brace shirts

Evelyne has had frequent problems with rubbing from her brace. This has significantly worsened in the new higher design, in particular under her arms. Through a couple of my online contacts, I heard of shirts specially made for under casts and braces. There are different types, one brand called 'knit-rite' which make a cast t-shirt (to be worn under a plaster cast), and another 'the Boston T' or 'the Boston Silver T' (made for boston braces). They reduce perspiration, bacteria, are cooler in summer, and can be changed to reduce smelling and itching. 


I tried to find the shirts online and always hit brick walls. The main being that they would not ship to Australia. In a last attempt to find one I posted a request on an online forum for infantile scoliosis. Success! Within a week I had an offer to post some to me from the US and not long after they arrived. The shirts we got sent are the Boston Silver T and are specifically designed for a brace. They even have flaps under the arms to reduce rubbing in that area. They have made a phenomenal difference to Evelyne's comfort levels. She is kept cooler, less sweaty and smelly, and most importantly - no sores under the arms or on her spine!


Here is a picture of her showing off her new singlet.

Magnetic growing rods

There is a new technique being trialled in Melbourne which uses magnetic growing rods. My specialists here in Sydney are also trialling a similar type of system. They have done one surgery at present, with another to be completed soon. This technique may be an option for Evelyne. The benefit is the rods can be extended without 6 monthly surgeries. There is a recent article below on a 7 year old boy who recently had the surgery.

http://www.theage.com.au/victoria/magnetic-attraction-gives-matthew-chance-to-stand-tall-20111023-1memn.html

Reflux and pressure sores

Unfortunately, there are many 'side effects' of having a brace or cast on.

Two we are dealing with daily are reflux and pressure sores.

Both my kids had reflux as babies, Samuel took until he was 4 to grow out of his and wean him off his medication. Evelyne's reflux was not as severe as a baby, and it wasn't until we applied the cast that it became much worse and medication was needed. The tightness of the casts and braces squeeze and put pressure on the tummy and can cause the contents to be pushed back into the oesophagus. It is also uncomfortable to eat big meals and I've heard others with spinal braces as teenagers recommend smaller, more frequent meals. At the moment Evelynes reflux is managed with medication, leaving more time between meals and bed, and avoiding some foods close to bedtime.

Pressure sores are also reasonably common on the bony prominences of the kyphosis of the spine (the hump), and sometimes on the hips where the brace or cast sits. Evelyne has a constant red area on her back which occasionally breaks open and requires something like duoderm to dress it. Fortunately in a brace, you can check it and dress it if needed, in a cast often sores are open and remain untreated. In the picture you can see the gel dressing with a groove in it from the brace digging in! (The red area is a birth mark).

Both these issues can be painful for her, but unfortunately, they are the lesser of the two evils. The treatment of her spine and attempting to manage the progression of her scoliosis is more important at this stage. So for now, we attempt to alleviate the symptoms as best we can.

Mehta Casting

I have been doing more research into different casting techniques in the hope of finding another alternative to try before surgery.

I had heard many times of Mehta casting, which is perfomed by some doctors in the US. However I did not think it was possible to have this technique done in Australia. I have recently found out that there is a doctor who has hands on training in Mehta casting in Australia, and that his hospital has one of the specialised casting tables required. We have booked in to see him in the hope that this style of casting may be an option for Evelyne.

Although we know she will eventually require surgery, we are still hoping that we can hold out longer. You can see a clip about Mehta casting below.

http://youtu.be/zuQE77EGgNU

Mehta Casting

UAB Orthopedic Surgeon Dr. Joseph Khoury explains the process of Mehta casting and how it is used to treat infantile scoliosis.

by uabhealth | 3 years ago | 24,994 views