When Crutches Are a Good Thing

One of the biggest challenges for children with Cerebellar Hypoplasia is learning to walk. There are several reasons for this.

1. Balance- Because of the undersized cerebellum their balance is compromised. It's harder to walk if you don't have good balance. This is why drunk people and the elderly often fall. This is pretty obvious, but I think for people with CH it is a permanent condition that they learn to compensate for. 
2. Coordination/Control- Walking takes coordination. Children with CH have a harder time controlling their body, much like people with Cerebral Palsy, the degree to which they are affected varies.
3. Motor planning- Motor planning is an important piece of walking, and walking safely. A person needs to be able to plan the required movements to go from A to B and react quickly to unexpected changes in the environment. This can be harder for children with CH and is a particular challenge for Ainsley. 
4. Tone/Strength- Children with CH typically have some degree of low muscle tone* which delays the acquisition of skills such as walking, running, jumping etc. which in turn reduces strength because they tend to get less exercise naturally in their day-to-day activities as neuro-typical children. 

*The low muscle tone associated with hypotonia must not be confused with low muscle strength or the definition commonly used in body building. Neurologic muscle tone is a manifestation of periodic action potentials from motor neurons. As it is an intrinsic property of the nervous system, it cannot be changed through voluntary control, exercise, or diet. From Wikipedia

Unfortunately for Ainsley in addition to CH she had Bilateral Hip Dysplasia at birth(probably due to excess amniotic fluid, due to difficulty swallowing due to CH). She spent a lot of time in a Spica Cast when she was one but the treatment wasn't successful and it had to be done again when she was 3 but the second time with bilateral femoral osteotomies (they sawed her leg bones in half and reattached them with bolts and screws to get the head of the femur into her hip sockets better) and then put her in a spica cast, followed by a brace, again. It seems rather extreme but the orthopedic surgeon felt it was necessary (to avoid painful arthritis as she got older and give her stability in her hips for walking, what can I say we were optimistic then, she seemed so close). Thankfully the surgery and treatment was successful, she now has good hips that have remained stable with growth (she gets x-rays annually to check for relapse but after several years she looks good). Not only did she lose muscle during those periods of treatment, she also lost progress and had to start over, not once but twice. It's not the entire problem, just one of many factors as to why she isn't walking independently, yet.

I say yet because I think I have good cause to believe that she will in her own time. Many other children with CH have learned to walk at a younger age. Always with more effort than for neuro-typical children, but sometimes as young as age 2.  Like individuals with Cerebral Palsy, it cannot always be known whether the person will be able to walk independently until they have done it. Some adults with CP will, some will need crutches, some walkers, some wheelchairs. In my on-line CH support group I have heard stories of kids taking their first independent steps as teens.  Emotionally I am prepared for the possibility that it may not happen for her, but it won't stop me from continuing to try. She will be okay either way, but I want it for her.

Ainsley is very quick in her walker and has been using one since age 3, transitioning to full time use at school after her decannulation in 2014. However, after years it seemed clear to me that success in a walker alone doesn't help a child progress to independent walking.  They have to learn to let go of the support they've learned to rely on. As Ainsley grew taller and bigger falls got riskier and for several years now I've felt that fear was a major obstacle we would have to work on. She is afraid to even try to take steps unassisted. I've been asking the physical therapists (the school PT and the one we consult with at Children's) for ways to help her get over her fear and even teach her how to fall safely. That hasn't happened yet, so if you know a therapist in the Seattle area who works to teach kids how to fall safely, please e-mail me.

We built a stander last summer using PVC and particle board (I designed, Steve built) so Ainsley has a safe place to practice a lot of independent standing while having a table surface to play and work at.  That has been helpful but I see she still loses her balance. It isn't the entire solution, just one tool to help. Since Ainsley is highly motivated to watch videos it is a good way to get her to spend a longer period of time on her feet. She knows that she has to stand if she wants to watch, not all the time, but as much as I think she can handle.

The school therapist starting working on using forearm crutches in her PT sessions last year.  They require more balance and control than a walker and are the logical next step in the progression toward independent walking for Ainsley. This November she was still pretty fearful and unstable, but the school started using them at other times during the school day and her confidence and stability improved with the increased practice.  In January I made an appointment to go observe at the school and was surprised to see the amount of progress she made in a couple months.  Check out this video from November, if you want to see just how much.  I was relieved to see that she feels more confident and to confirm that the school will continue to have the aide or therapist right behind her to catch her with the gait belt if she starts to fall. It is a real concern until her form improves. You can see in the photo below that the manufacturer shows the proper technique on top.

Right now she isn't using them properly, she looks just like the picture on the bottom of what not to do and in addition she is placing the crutches too far forward not just out to the sides), but with some work on it at school and at home I am hoping she'll get there soon enough. She will continue to use a walker for long periods, when speed is a factor or on uneven terrain. I expect it will be a long process of learning, much like we've experienced in other areas of her development.

Last Monday I ordered a pair of forearm crutches from WalkEasy, in yellow, her favorite color so we can start the process of working with them at home. I was delighted when they rush shipped them to me at no extra charge, showing up Thursday. What awesome customer service! Friday morning we tried having her walk out to the bus and she was able to show them off. Afterward she said she prefers using the walker to go out to the bus so I think we need to work on it a bit more before we make this a permanent change. It's easy to get hasty in our excitement but hopefully she'll be ready soon. Enjoy the video.