The Latest Kinsley Update

We had another big appointment at Shriner's this week which brought a lot of new changes. New medication, new orthotic devices, and plans for upcoming surgery evaluations that could possibly bring more long term solutions for this little girl. 

The main purpose of our visit was to get more x-rays and botox injections. We had x-rays done on her hips to make sure they were holding at 30% subluxated, and thankfully they are. If they move to 50% we have to do hip dysplasia surgery, and the number one goal the last two years has been to keep doing injections on her leg muscles, which will lessen the pressure of her hips being pulled out of the sockets, and prevent that surgery for as long as possible. The current treatment is working, and we're super grateful for that. 

Another bonus to the botox injections is that it turns off the spasticty in Kinsley's legs, helping her to strengthen weaker muscles, walk better, and hopefully walk with less interventions in the future. She's been making amazing progress and strides over the last several months, so we've been looking into options that will allow her spasticity to be permanently removed, and not just temporarily shut off with botox injections. 

Our two main options for that are Selective Dorsal Rhizotomy, which I talked in depth about in this post here, or a Baclofen pump. Baclofen is a drug that relaxes the muscles and turns off spasticity. They can place a pump under the skin in her spinal cord that would give a continuous drip of this medicine to her spastic muscles and essentially keep the spasticity away and allow her to function better without getting botox injections all the time. The downside is that this pump would need to get refilled every six months or so, via injection of a needle into her spine that accesses the pump and fills it. So we're essentially getting rid of botox injections, for a spinal surgery and more injections, which isn't really my favorite option. I'm really hoping they just let us do SDR since it's more of a one and done surgery and no one has to go poking around her spinal cord ever again afterwards. 

Baclofen does come in an oral medication form as well. We tried it two years ago and Kinsley was the 1% of kids who had insomnia from it, so we had to take her off of it. Now that she's five, her doctor thinks she'll handle it better, and so we're currently trying oral baclofen again. As of right now when I'm writing this, she took a full dose at bedtime and went to sleep, so I think we're in the clear. 

The downside to oral baclofen is that there are side effects which wouldn't necessarily be as prominent with the baclofen pump. The major side effect we're most nervous about are seizures, so we don't want her to be on this long term, but it will give us a good idea of how her body will work when she has SDR or a baclofen pump placed, so it's a good short term solution to see how she'll react to more long term solutions. 

We made an appointment to go to the Gait Lab in January where they will fully analyze how Kinsley walks and uses her muscles, and from there they will be able to have a better idea as to whether or not Kinsley is a good candidate for either of these operations, and then we can start making more plans from there. 

There was a bit of bad news at this last appointment. If you watch this video of Kinsley walking at the grocery store, you'll notice that her right foot is turned in quite a bit when she walks. She walks really well, but would walk even better if this wasn't happening. We thought this was a spasticity issue, and that they could inject some botox in her foot today to solve the problem, but they were able to determine that the issue was related to bones and not muscles, meaning botox cannot correct the issue. 

Her foot is turning in as a reaction to the position of her hips, from the hip dysplasia. Basically her hips being out of alignment are causing the leg to be out of alignment (see pictures at top where knees are pointing in at each other) and therefore causing her foot to be off track as well. It's hard because she wears leg braces to hold her feet in the correct position, but they unfortunately don't correct the foot turn the way we'd like or hope. The only way it could be really corrected is to do the hip dysplasia surgery, but it's just so invasive and everything else on Kinsley looks fine, so they really don't want to do it unless her hips subluxate at that 50% mark. Meanwhile, Derek and I are nervous that she's going to cause permanent damage to her leg/foot, so what do you do? 

We basically have one option for right now, and that is this strap thing that essentially attaches to her leg brace, wraps up around her leg, and then around her waist. This strap will hopefully prevent her foot from turning in, and we're hopeful that her walking will look even better once the foot is in the correct position. The downside is that as you can imagine, Kinsley will likely hate this and not want to wear it, and the doctor told us the benefit won't be worth the fight. So it might work, or it might not. To get a better idea of what this would look like on Kinsley, see this video of our friend willow here. 

I know a lot of people are probably thinking, is walking worth it if you have to go through all these hoops, interventions, and adaptive equipment? I used to think it wasn't. I honestly thought that we would have given up on this goal a long time ago. But Kinsley has determination and fire. She wants to be on her feet and walking. She chooses to use her walker over her wheelchair everyday at school. As long as this is something that she keeps wanting, we'll keep fighting for her until she says otherwise. Bearing weight on her legs and using them is also really good for her hips, and helps them to not get worse. Hip dysplasia surgery is incredibly invasive, and would mean Kinsley would be in a body cast for three months straight, and we don't know that she would regain any or all of her skills again if we had to do that. 

There are also plenty of studies that state people who are full time wheelchair users have more health related concerns than able bodied individuals. The one that pulls at our heart strings the most is that full time wheelchair users are more likely to have flu related complications, such as pneumonia, and their bodies aren't strong enough to fight off infections that they sadly die from. We've known too many sweet kiddos with holoprosencephaly over the last several years that have died from pneumonia and flu related complications. It seriously breaks my heart. It's my hope that should Kinsley ever get one of these infections, that she would be strong enough to fight it and get better. 

Nothing's guaranteed, and time has never been on our side in any of this, but in a lot of ways, the fight to walk is about so much more than just walking. 

1 comment

  1. I think the bracing option you are referring to is twister cables. A lot of kids in my spina bifida group use them. I would give it a try. It's non invasive so there is no harm to trying.