Frame removed and end in sight!

This will be my last blogpost for this procedure as we are nearing the end. Ava had the initial surgery in July (which lasted 6.5 hours) which was to attach the frame from the outside to the inside of her leg. During the surgery Ava’s leg, ankle and foot were broken to allow us to use the frame to pull her bones into a different place. This part of the process lasted 6 weeks and involved daily manipulation by 1mm which pulled the breaks in bone apart and into a new direction.

This was incredibly painful for Ava and she was kept on diahydracodine for 3 months. She was very brave throughout this and very determined to just get on with it. As you’ll see from my various posts throughout this procedure Ava had to endure several extremely bad infections (images illustrate this far better than words) and was repeatedly taken back to hospital so the Drs could monitor her. She also ended up taking penicillin for 4 weeks which did eventually reduce the infection.

On 14 November Ava had another operation to remove the frame and she is now in a plaster cast. While she is still unable to walk she can be weight bearing. The plan is to put her in an air cast just before Christmas but we’ve been told her walking could take months. At present she is showing no signs of being able to walk unless she has crutches.

Ava has been at school throughout the procedure, only having time off for hospital appointments and when she had the infections. Her school have been amazing and have made lots of provisions to help her - from changing all her classrooms to downstairs, reserving a room for her and her friends at break time, having her canteen lunch delivered to the room and allowing her to leave lessons early to arrive at her next class. Ava’s friends have also been brilliant and have looked out for her throughout. 

The consultant is happy with the progress so far and we are looking forward to seeing her new foot in a few weeks time when the cast is removed. We do know, however, that the foot correction has made her leg length difference worse and it’s now estimated to be 6cm. This will have a huge impact on her walking (which obviously isn’t happening yet) so the next leg lengthening procedure is planned for June 2020. Once this is completed we should have completed the major surgeries. 

Tips for other parents who have a child who needs a frame on their foot (although there are quite a few from my previous posts during leg lengthening) 

1. Apply for a blue badge. We have this and it’s a godsend when your child can’t walk. 
2. Have the conversations with school as early as possible so they can make the necessary provisions for your child. They are definitely better off in school and having as normal a life as possible while the frame is on
3. Plan in advance for weekend activities - it’s incredibly restricting for a child with a frame on their foot. Most activities are off limits and the foot needs to be elevated a lot. 
4. Clothing has been a struggle as Ava is now 12 and wants to wear certain clothes. Shorts have been the easiest thing to wear with the frame. 
5. Showering has been challenging too. I would recommend buying a bath chair and wrapping the frame in bin bags covered with a towel. It can’t get wet so Ava used to balance her leg outside of the bath while having a shower.

Pictures below show the x ray before and after. I’ll add new images to this post once the cast is removed and we see her foot for the first time.

Thanks again for reading. Nicole x

Pin site infections continue!

The pinsite infections are still there. This has been the 3rd week of penicillin and some of the swelling has reduced but the pin sites are still bad. The pictures show how much of the skin is affected and gives you an idea of how sore they are.

We are going back to hospital each week to have them checked and for the time being the pin site cleaning is being done by a trained nurse and not me (something I’m thankful for). The cleaning is really painful for Ava and involves lots of screaming.

We are all quite weary at the moment.

We are due an x ray in 2 week’s time which will hopefully show that new bone is starting to grow in between the various breaks in bone in Ava’s foot, ankle and leg.

So far this has definitely been worse than the leg lengthening. We were told to expect this by the consultant but until you’re in it you can’t necessarily prepare. 

The 4th week of penicillin begins tomorrow and hopefully by the end of the week we will see some progress.

PIN SITE INFECTIONS!

We’ve had a bad week! It started last Saturday when I was doing Ava’s weekly pin site cleaning.  Of the 16 open wounds going into Ava’s leg, 4 of them were oozing liquid and the skin in between others had burst (almost resembling a burst sausage skin). The smell was also unpleasant- like rotten meat. Sorry to sound so graphic but things didn’t seem right.

I started giving Ava the penicillin we’d been discharged with from hospital as this showed all the signs of an infection. Ava went to school with painkillers but by Tuesday her foot and lower leg were badly swollen. On Wednesday I called the hospital who wanted to see her the following day. When they saw her foot and leg a Dr and wound specialist were called who wanted to admit her to hospital due to the severity of the infection. This would be to monitor her and hook her up to IV penicillin.

Ava was hysterical - the fact she would be returning to hospital but also because the nurses had to clean the infected wounds. Thankfully we have managed to avoid the hospital stay on the condition that she keeps her foot elevated for the next few days and we monitor the swelling. She has also been prescribed some super strength Penicillin for the next 14 days.

The good news is that today the swelling has already reduced considerably. The Drs have draw on Ava’s foot to monitor the swelling and how it reduces (see images).

This weekend will be about rest and continued foot elevation. This is a particularly low point for Ava as under normal circumstances with no surgery/frame she would be away camping this weekend with her friends. Not only is she not doing that but she is on strict instructions not to move anywhere.

Here’s to a better week next week. We’re all counting down until the frame is off!

More Correction/Current X-ray

Ava has now completed 2 weeks back at school with a frame. The school have been great and have moved all of Ava’s classes downstairs. They have also made other provisions such as allowing Ava to leave lessons early to navigate the corridors to her next class before the other kids, and setting up a room for her and her friends to use at break time. 

Generally Ava is more settled. She has the odd moments of frustration but on the whole is coping well. She is keen to understand when the frame can be removed but we have not been given a definite date. This all depends upon how quickly the various breaks in her bones heal. This could be anytime between the next 6-8 weeks. Ava will then go into plaster cast followed by a walking cast boot.

Her foot and leg are still incredibly swollen and the various wounds are oozing a bit. I’m cleaning them regularly with the hospital prescribed equipment but they are quite sore and painful. Hopefully as the correction has finished they will start to scab over as we don’t want Ava picking up any infections.

To give you another view of how well the treatment has worked look at the X-rays below.

Will update again after next hospital appointment. 

Correction complete/School begins

We’ve come to the end of the second programme of correction. As you’ll see from the photos below it’s working!

The first programme was to open the breaks in Ava’s bones - the leg break, the ankle break and the breaks along her foot. The second programme has been to move/rotate these breaks in bone (1mm per day) to eventual correction.

The school holidays have been long and very frustrating for Ava. She started them in a lot of pain but this has gradually reduced to more discomfort. Ava is also sleeping much better and on most days sleeping through. She has been very restricted on what she can do and we have relied on friends coming over and watching films. When we have ventured out the frame obviously draws a lot of attention. Lots of stares which Ava finds difficult but on the whole people look and then ask what’s happened. We’d all far rather that than people just gawping (which a few have no shame in doing).

Ava is due to go back to school on Wednesday which she is incredibly nervous about. The school have already made provisions and moved all of Ava’s lessons to ground floor classrooms. She also plans to leave each lesson 10 minutes early so she can navigate the corridors with no other kids around (she is still on crutches and non weight bearing). We’ll have to see how this goes. Ava is understandably nervous about other kids reactions and any potential teasing. Hopefully this is all positive as she has been through a lot so far both physically and mentally. Fingers crossed it all works out ok.

Before school on Wednesday we are due at hospital at 8am. The consultant is going to make a decision as to whether he creates a third programme to improve the foot positioning even more. He is doing this as Ava has been able to tolerate the pain with medication and feels he can improve things more. We’ll have to see what the outcome is on Wednesday.

More to follow on life back at secondary school with a frame on.

3 weeks in/Foot correction

We are now 14 days into the manipulation process of Ava’s  foot. As expected it has been quite up and down. I’m now back at work and Simon is on parental leave/working from home looking after Ava. She is in some discomfort as we are pulling broken bones apart via the frame so she is still on strong painkillers to help manage this. 

Ava has met up with some friends which has helped take her mind of things but she is quite frustrated and is not sleeping well. Having a frame is very restrictive for a child - there really isn’t an awful lot you can do. It also has a huge psychological impact, something we were informed about by the hospital and remember from when she was 6 years old. Thankfully the wonders of new technology have helped - Netflix, Amazon Prime, YouTube, Insta.... (anything goes at the moment!)

I have managed to do the pin site cleaning a number of times which involves cleaning the open wounds (16 in total) with special sterile medical equipment and then re applying new foam pads to minimise the risk of infection. This is still quite time consuming (takes me an hour) but Ava copes well with this.

Simon has also taken Ava for her first of many follow up appointments at the hospital. She had an X-ray (see below) to monitor the gap in her bones and the progress from the manipulation. During this appointment they also saw the physio who has given special exercises that we have to do with Ava on her toes each day. This is to prevent them from changing position due to the breaks in her bones (keep them straight and upright). If the bones drop Ava will have to have another surgery to put more wires into the end of each toe to keep the bones in the correct position which we obviously want to avoid (particularly as this is even more painful).

So all in all this has been a difficult few weeks. We’re all quite exhausted and counting down the days until the frame is removed (hopefully mid October all being well). The frame is removed by another surgery but it’s obviously a much simpler process than having it fitted. 

65 days and counting..........

Coping, Pin Site Cleaning and Correction Begins

Over the past few days Ava has had time with the physio and has learned to use her crutches. These can be used to get around the house but for longer journeys we have a wheelchair. She is coping remarkably well though. She does have moments when she feels down and frustrated but on the whole she is being positive. Hopefully this isn’t short lived as the correction of the foot hasn’t begun yet.

We have also had the first pin site cleaning. This is something that we have to do weekly to surgically clean all the open wounds into Ava’s leg and foot. There are 14 in total and doing this will help minimise risk of infection in the wounds while the frame is on. Having an infection can hamper the treatment so it’s important this is performed with sterile medical equipment which the hospital provide you with when you are discharged. I’ll have a prescription to get extra supplies from the GP as and when I need it. Ava didn’t even flinch when this was being performed - a slightly different reaction to when she was 6 when she used to scream non stop.

The re positioning of Ava’s foot begins tomorrow, which is the hard part!  The surgery itself was not to do any correction of the foot or bones, it’s to break the bones themselves and to put the frame on. The correction needs to be done by us at home by following a bespoke programme compiled by the consultant based upon X-rays of Ava’s foot (see picture below).  Unfortunately this is a very slow process and has to be done gradually so as not to damage any nerves, tendons or blood vessels in that region or most importantly to cause too much pain. To be honest this really is the painful part as each day by performing the various twists on the frame we are moving broken bones further apart and into different positions. We also need to keep an eye on Ava’s skin surrounding the area as it is stretched as the foot moves position too.

It’s during this correction stage (which will last between 3 - 6 weeks) where Ava needs to be really brave and where we visit the hospital weekly for X-rays and chats with the consultant to determine progress and whether we should slow down further.

As Ava has been able to move on her crutches and is relatively comfortable with her painkillers, we have thankfully been discharged early. I know Ava is looking forward to sleeping in her own bed tonight. 

Operation Complete - The Frame Is On

The operation has happened and the frame is now on. The surgery lasted 6 hours so was quite intense. This is due to the sheer number of bones in the foot so any surgery here is complicated.

The surgeon has broken the bottom of Ava’s leg and broken all the bones across her foot. The various wires from the frame are attached to these bones and we begin the process of moving their alignment on Saturday. 

Ava is very spaced out as didn’t return from surgery until 6.30pm. She is also on a self administered morphine drip so is topping herself up regularly (children’s dose!) which is obviously making her very sleepy.

A few people have asked why the operation/procedure is being performed. To put it simply if we had left Ava’s leg and foot and performed no surgery she would be in a wheelchair for life, with a very visible deformity too. It would be impossible to walk with a 12 cm leg length difference (which is the difference predicted by the consultant at skeletal maturity) let alone the fusing of bones in her foot.

Years ago before the illzarov frame people would have been given the option to amputate but that would be such an incredibly difficult decision for any parent to make for their child. In some cases today that is still the only option for parents.

Thankfully we can go down the surgery route, even if it is a long drawn out process that is painful for Ava. We know looking back  she will thank us for this.

So tonight is all about Ava resting and being comfortable. Hopefully we all get a few hours sleep as it’s been a long and emotional day. 

Hello. It’s been 6 years since Ava’s last illzarov frame which corrected her leg length deficiency by 6cm, and since my last blogpost. Obviously Ava has grown during this time and the difference is now at 4cm. We have decided to delay the next leg lengthening for a year and focus instead on her foot correction.

Ava hasn’t had much surgery over the past 6 years, the exception being a small operation last year to insert a metal plate in her knee to correct the alignment. This was performed as an outpatient and Ava was back on her feet within 2 weeks. 

Unfortunately we are now facing our next major procedure which is to correct the alignment of her ankle and heel (which are currently fused) and the positioning of her foot.

We are now 3 days pre operation and Ava is understandably very nervous and very aware as she’s now 12 years old. The operation will take around 4/5 hours and during that time her foot will be broken in several places and an illzarov frame will be attached with wires connected from the outside to inside of her foot (similar process to the leg lengthening).

An illzarov frame  looks like a form of medieval torture but it is the best way to correct complex deformities in bones as it’s a gradual process that puts less pressure on the various blood vessels, nerves and tendons surrounding the area. The aim of the frame is to move her ankle, heel and foot into a better position - 1mm per day. We have been told the frame will be on for 4 months, followed by 2 months in plaster casts. Ava will not be weight bearing during that time so unfortunately for her no walking, running, swimming or dance classes until January 2020. 

It feels a bit surreal at the moment for all of us but I’m sure on Monday once we arrive at the hospital things will hit home. Ava will be in hospital for around 10 days and we’ll be staying with her throughout (alternating sleeping there each night). I’ll update more post surgery but have included some before operation images.