Tiredness, school, pin sites and clothing

It's been a tough few days.  Ava continues to sleep badly (averaging about 5 hours a night) so it's having a huge effect on everyone in our house. This lack of sleep means that Ava is very grumpy and tearful.  It also means our days are very long.

Without doubt this is one of the hardest things we've ever had to go through - its physically and mentally draining on everyone and its only been 3 weeks.  It's going to be a long 9 months!  We have to keep reminding ourselves of the end result, although this offers little comfort to Ava who 'just wants the frame off'.

Despite the lack of sleep Ava has managed to attend school for a number of mornings. This has been instead of the home schooling that we were initially told about.  This has come about following several meetings between us, the school, the hospital and our county ESTMA team (educational support team for medical absences). Hopefully this continues as Ava is so much happier being in the school environment surrounded by her friends. It provides a sense of normality for her which is a good thing at the moment.

She is, however, very embarrassed and self conscious about her frame in public. While she is ok with her classmates, she covers her face with her hands when she sees any of the other children as doesn't want them to look at her. Little does she know that this draws even more attention to herself.  I'm hoping this will become easier for her over the next few weeks.

Some good news is that the pin site cleaning only took us an hour this week. The pictures below show the equipment needed for this. The hospital provided enough material for a few weeks, but the rest has to be ordered from the GP.  I've included a picture of what the frame looks like minus the foam pads covering the pin sites so its possible to see what needs to be cleaned.  Although it was quicker this time, the whole process is still fairly stressful for all involved and is definitely a 2 man job in our house (i do the cleaning and Simon tries to distract Ava as much as possible).

A few tips on clothing for parents who have a child who will require lengthening, although this is more appropriate for girls.  The best item of clothing is obviously dresses as they go over the head. However, if your child is wearing the frame in winter months (or like us is experiencing the coldest March for 10 years!) then a dress is not always suitable as its impossible to wear tights or leggings with a frame.

We have bought a variety of track bottoms which we have had altered so they can fit over the frame. I had been told by the hospital to cut down one side of the trousers and stitch in Velcro. We found a dressmaker who did this, but Ava found it quite itchy on her skin and when she sat down the Velcro often unfastened. The solution has been to put a zip down the side - from top to bottom. This means you can zip up the trousers from the waist down to the start of  the frame.  In terms of knickers, the dressmaker has cut down one side and stitched in poppers. So far this has been working really well.

1cm achieved and x rays

Today was our first follow up appointment with the consultant since Ava's operation to have the frame fitted. As per the previous posts we have been performing the lengthening every morning for the past week. The good news (as you will see from the x rays below) is that by turning the struts on the frame according to Ava's programme we have achieved 1cm.

Although the new bone has yet to grow, the break in her bones (tibia and fibula) has widened by 1cm. Over the coming weeks the new bone will start to form and we'll be able to track this with future x rays that will take place fortnightly when we see the consultant.

We were told today that we are aiming to achieve 5cm of growth, rather than the full 6cm. This is because of Ava's age and length of leg. As I've explained in earlier posts Ava will need a second lengthening at 12 years old as her 'good' leg will continue to grow at a quicker rate. The remainder of the difference will be made up (hopefully) during this second lengthening.

We were also told its highly likely that the consultant will inject into the growth plates of Ava's 'good' leg at some point between the first and second lengthening.  This will be to slow down the growth which would reduce the discrepancy in length further. If Ava takes after her 6'4" dad then this should still make her taller than my 5'5", even with her growth being slowed down.

In terms of how the days are going, they are still very much up and down. Ava generally is in much better spirits though, particularly when she sees her friends. Unfortunately she is still not sleeping well and wakes on average 3 times a night. This lack of sleep is likely to be the cause of some of her low points, as apart from the morning lengthening and pin site cleaning, I don't think Ava is in any real pain (which is good).

1cm down. 4cm to go!

Life at home

We have finally been discharged from hospital.  We asked for this to happen as Ava was not sleeping well on the ward. It's great to be home and Ava seems more comfortable in more familiar surroundings. She is managing to get around (albeit slowly) with her zimmerframe from room to room.

She still has a few tearful episodes a day which tend to be out of frustration and the general feeling that 'this is unfair', but they are becoming less frequent.  We do know from the consultant and nurses that this is all part of the lengthening process. There will be days when Ava is in better spirits and those when she is quite low - sometimes out of pain, but more commonly frustration and isolation (not being able to do what her friends are doing and staying at home more than she previously did).  At weekends we previously would have been out a lot with both of our kids, whether this was going to the playground in the park, to soft play or swimming (Ava's favourite). But this is not possible for the next 9 months, so we need to find other activities to keep Ava's boredom at bay.

We are slowly getting to grips with how our routine needs to be adapted and have found new ways of doing things. For those reading this blog who have a child who will be undergoing lengthening this is what is currently working for us:

Sleeping
Ava is sleeping in her own bed with her leg elevated with a pillow. She has to sleep on her back as it is impossible for her to turn on her side due to the weight and size of the frame. This is one of reasons why she isn't sleeping well as she is used to being on her side.

We were considering moving her room downstairs and buying a sofa bed as we were unsure how Ava would manage the stairs. However, we have discovered that a way to master this problem is for Ava to go up the stairs on her bottom with either Simon or I holding her frame up. The same applies for  coming down the stairs.

Some may prefer not to do this, but we felt it was important for Ava to be in her own room and for us to be on the same floor as her (this is particularly useful as there has been quite a bit of night waking so far).

Washing
As I've mentioned in my earlier posts, it is only possible for Ava to shower once a week during the cleaning of her pin sites. She is not allowed any baths for the duration of the treatment as this increases the risk of infection as you are in effect sitting in your own 'dirty' water.

Cleaning of the pin sites and showering is pretty traumatic and involves lots of crying. Thankfully we only have to do this once a week. The cleaning of the pin sites took the nurse 30 minutes in the hospital, but took Simon and I nearly 2 hours. I doubt I'd make a very good nurse, but hopefully as the weeks go on I can beat that time.

Going to the toilet
Ava can manage this on her own with the use of her zimmerframe - which is good news. But does need help re fastening her knickers as these now have buttons down the side (not an easy task while standing on one leg!).

Affect on other siblings
This is quite tough as invariably Ava has the majority of our attention. We have a 2 year old  so have to devote time to him too. The test will be when Simon returns to work when I have both kids on my own (you obviously can't push a buggy and a wheelchair simultaneously).  Luckily I have my mum nearby and some good friends who have said they'll help out.

Lengthening
This has to be performed daily and we find the best time to do this is in the morning. We give Ava some paracetamol and then turn the various struts about 30 minutes later. I do the lengthening and Simon distracts Ava as much as possible. It shouldn't yet be too painful, but I can tell Ava has some discomfort by her reaction and reflex. Hopefully I'll have some better tips on what to do as the weeks progress.

For more information on how to cope with a child undergoing lengthening you can visit www.steps-charity.org.uk.  STEPs is a charity for people born with lower limb conditions.  They have a booklet for parents that provides information on caring for a child with an external frame/fixator. I found this to be really useful in informing us on what life will be like for the whole family, plus advice on what you need and ways of coping.

4mm down.  5.6cm to go........

The lengthening begins......

Today the first lengthening began. We have our program (see photo below) which tells us what we need to do daily. There are 6 struts on the frame and we move these according to Ava's prescription. These open the bone by 1mm per day. We were initially told that the lengthening would achieve 1cm per month, but Ava's program will be quicker than this as she also needs the bone rotated. The procedure will still take 9 - 12 months, but the length will be achieved quicker. The leg will remain in the frame to help it heal and become stronger.

During the 9 months we need to see the consultant fortnightly so he can take x rays and monitor the bone growth and Ava's general progress. They need to ensure the bone is healing and that it doesn't bow (which would cause further complications).

This first lengthening was thankfully pain free. It starts to become more uncomfortable as the bone grows, as the muscles and tendons connected to the leg aren't developing at the same rate which can cause spasms which are quite painful. We'll need to take each day as it comes and where necessary give Ava pain relief beforehand or perform the lengthening twice a day (so the increase in strut changes is more gradual).

I know we only achieved 1mm of growth today, but it makes us feel more positive that this is going to be worth it. The only downside of today was that the consultant is slightly concerned about Ava's foot. Prior to the frame being fitted she wore a splint which kept her foot in place (following her previous operations). As she is now out of the splint the foot is staring to turn inwards again, so we were told that she may need the frame extended so another ring is attached to her foot. Unfortunately this would involve another operation and hospital stay during this process. It's not definite, so we feel we have to forget about this for the time being and cross that bridge if and when we come to it.

1mm down.  Another 5.9cm to go!

Day 5 and pin site cleaning

Over the past few days we have been shown how to clean the pin sites on Ava's leg. There are 18 in total (which all go from the outside to inside of her leg). Although this is a large number of open wounds, with correct care we're hoping to prevent any germs from entering her leg which would result in an infection.

The nurse in charge of lengthening took us through the process on Ava's leg. It takes about 30 minutes and has to be performed weekly. This is then the only time Ava can have a shower ( she's not allowed baths as you're sitting in 'dirty' water).

Ava did not react well to the cleaning and pretty much screamed the ward down. Although this process does sting, Ava's reaction was more shock than pain. This is because the cleaning involves removing the pads that cover the pin sites, which make the entry into the leg very visible. As the operation had only recently been performed the pin sites were quite bloody, and Ava was shocked (hence the reaction). The nurse said her response was common and that it will be easier when we start to do it at home (fingers crossed!).

Ava has also had an X-ray of her leg. This will be used to calculate measurements of the leg which will then be fed into a computer by the consultant to calculate Avas's prescription for lengthening. Each case is obviously different and we have a specific program for Ava's leg. This will be to correct the twisting (tibial torsion) and lengthen (fibula hemimelia) by 5-6 cm.

 I find it absolutely amazing (and am thankful) that this type of procedure can be done.  If Ava's leg was left as it is the difference in length would continue and there would come a time when she simply wouldn't be able to walk.  This is why the decision to undertake lengthening was a no brainer for us.  I know it has been and will continue to be hard for Ava, but it will be so worth it in the end and something we know she'll thank us for when she is old enough to understand.

2 days post op - the frame is on

The past 2 days have been incredibly hard. Although Ava has had several operations and hospital stays before, the impact of this operation has hit her (and subsequently all of us) hard. We were told that having a frame fitted has a huge psychological impact on a child, but I presumed this would happen later after discharge from hospital when Ava realised she wouldn't be able to do certain things she did before.

I know it's still early days and she just needs to get used to the frame on her leg - the weight of it, discomfort and what it restricts her from doing. At the moment she is very tearful and perhaps because of her age, she is very aware. She's made lots of comments such as 'Why are you doing this to me', 'I hate my stupid leg and foot', 'Why have I been born like this'. To date Ava has never said anything like this, even when she has been in hospital before. I suppose it was always inevitable, but it is very difficult to find the right words to respond that reassure her and make her feel positive.

Thankfully the wonders of new technology has been a godsend. The best distractions involve listening to One Direction or watching Jessie on the iPad.  Ava's been bought lots of iTunes vouchers from friends which I'm sure will all be spent by the time we leave hospital.

The physio came today to show Ava how to use her walking zimmerframe. This will be used to keep her more mobile in the home. She was surprisingly good at it which is great, as the more mobile she is the better. The wheelchair will be used when we go out.

I know as the days go on she'll hopefully get more used to everything, and we'll find our own ways of doing daily things like dressing, washing and going to the toilet.

On Monday we will be shown how to clean the pin sites on her leg, which is how the frame is attached from the outside to inside of her leg. These will have to be cleaned once a week for the 9 months to try and minimise the risk of infection as she has in effect 'open wounds' on her leg. She'll be discharged with a large supply of antibiotics so I can prescribe them to her at the onset of an infection. The hospital will teach us what to look out for, but this generally involves a high temperature and a highly inflamed pin site.

On Wednesday the lengthening will begin. We will be shown how to turn the struts on the frame to pull the bones apart so new bone can grow. More to follow on this next week.

2 days before the op/Before and after

It's 2 days before Ava's operation to have her bone broken and the frame fitted. We're feeling nervous and I'm sure Simon and I won't get much sleep over the next few nights. The good news is that Ava seems currently unphased by everything and I'm sure will sleep like a baby. It's amazing how resilient kids are, and its the parents who constantly worry.

We need to be at the hospital on Thursday at 6am, as due to Ava's age and the length of time the surgery takes, she will be one of the first procedures performed by the consultant that day. It will take about 6 hours so I'm sure I won't be updating you all with posts for a number of days, at least until Ava is recovered from the surgery and has acclimatised to the frame on her leg.

Ava should be in hospital for 2 weeks and during that time Simon and i will take turns to sleep on the ward.  The lengthening procedure won't begin for at least a week. We'll then be given our programme and shown how to turn the various screws and struts to open the broken bones, with the aim of new bone growing in between. The consultant hopes to achieve at least 1 cm a month.

I'm going to post images so Ava's progress can be tracked. The first on this post are 'before' shots. Let's hope we see huge improvement from these pictures as the weeks progress.