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!