An Unexpected Turn

I would like to take a moment to thank you all for your love, support, thoughts, and prayers. We felt them all last Thursday when we made our trip to Nemours duPont Children's Research Hospital where Liam was evaluated for his candidacy to receive assistance from their Wilmington Robotic EXoskeleton (WREX) device which would provide him with freedom of mobility in his arms so that he could easily feed himself, hold crayons and draw, and wrap his arms around our necks.

Liam was not considered a candidate for the robotic arms at this time. This turned out to be unexpectedly great news. The doctors and therapists (five in all) that evaluated him agreed that he shows so much potential in the progress he has made so far, that they are concerned that the WREX device would only hold him back. It was recommended that we continue the aggressive therapy he has been receiving since birth. They offered tips and advice which would accelerate these efforts. They asked us to keep them updated with photos and videos of his progress and perhaps sometime in the future, he will be considered again for his candidacy for the WREX device once he has reached his expected potential.

In the meantime, the doctor, who is a specialist in Arthrogryposis, and the occupational therapist on her team recommend that Liam gain as much passive motion in his elbows as possible. This means that they want him to be able to bend his elbows a little more on his own. The occupational therapist fashioned an elbow splint to be applied to Liam's left elbow while he sleeps so that it will start bending as well as his right elbow currently bends.

While we are on the subject of great news, the physical therapist examined his use of his new ankle foot orthotics (AFO) and determined that he is ready to be walking any day now. She predicts that he will be walking in them, unassisted, in two months! Before the physical therapist saw him, the doctor had just suggested that he may need a cumbersome leg brace which would reach from above his hips to his feet and help him develop walking muscles over time. Thank goodness that won't be necessary! 

The physical therapist also recommended we no longer use the supine stander which is pictured in an earlier blog post, as at this point, he needs to work on balancing on his own two feet. Furthermore, she suggested we cut holes in a pillow case, put Liam's legs through the holes and let him walk, catching his falls with the pillow case so he can learn to balance himself. Alternatively, we could use a pair of denim jeans for the stronger, sturdier fabric. My husband's stepdad suggested we use Liam's overalls! 

 You can see how ready he is to take off running! 

We certainly feel our trip was worthwhile. Liam was evaluated by doctors and therapists specializing in Arthrogryposis. They took one look at him and knew everything about him and his needs before they examined his joints and body. This is what they do and they are exceptional at it. They would like to continue seeing Liam for treatment and future surgeries to loosen the tendons in his elbows and wrists. They also asked us to consider returning for therapy services on top of his existing therapy services. They see what we see in Liam. Hope for his Arthrogryposis.

However, we do not currently have out of network coverage with our health insurance and continuing treatment with these specialists is beyond what we can currently afford. We are discussing strategies and options at this point and are open to suggestions. We ask that you continue to keep us in your thoughts and prayers. Thank you all again.

Using Technology

It sure has been quiet around here, hasn't it? Well, we're still here and Liam is growing up and proving to us each day what he can do. So where do I begin? To start, here is how he is eating his food these days.

Snack Time

We are still working on his arms, loosening the tendons in his elbows using therapy, so that someday he might be able to bring a spoon or his fingers to his mouth. He's almost there, but he has a little way to go. In the meantime, he's figured out that he can pick up finger foods with his mouth and this is the only way he wants to eat now. We can help him if he has trouble picking something up, but only if he's tried to pick it up himself first.

Interestingly, a couple of my friends sent me this fascinating article. It's a beautiful story about a little girl with arthrogryposis who is fitted with "magic arms" to help her bend her arms so that she can bring food to her mouth and draw with crayons for the first time.

Here is another amazing article about another little boy close to Liam's age with arthrogryposis. He was also fitted with an exoskeleton to help him move with greater ease.

So how does this work? A huge obstacle for people with arthrogryposis is gravity. As described in this medical journal entry about arthrogryposis in the elbow joint, strong  flexors in our forearms allow us to effectively bend our elbow joints against gravity so that we can easily touch our noses, feed ourselves, pick up objects above our heads, and so forth. However, in the case of  patients like Liam, who display arthrogryposis in his arms, these flexors are weak and his tendons in the joint are tight, so his elbow does not bend with ease, thereby creating a struggle to resist gravity's pull to the center of his body.

To remove the obstacle of gravity from the equation, a device called the Wilmington Robotic EXoskeleton (WREX) was designed using 3D printing technology and has been undergoing clinical trials. I am currently corresponding with the responsible research hospital in hopes to have Liam fitted with a WREX device.

In this correspondence, it was brought to my attention that this blog lacks videos displaying Liam's range of motion capabilities in his arms with or without assistance. This week, I kicked off a Youtube channel where I will post videos from time to time, such as the one shown below, of Liam's abilities through his therapy and playtime sessions. Everyone is welcome and encouraged to subscribe to the channel.

Making Steps Toward the First Step

Our big boy turned one this month. Though I wrote a letter to him for his first birthday expressing my pride in him, I still find it difficult to sufficiently put it into words. He is a hard worker. He doesn't like doctors and therapists messing with him and making him do things with his body that feel uncomfortable to him. But I think on some level he really does understand that he is deriving benefit from everything he has gone through because he tends to maintain a tolerance that I find myself envying.

His newest challenge is in the form of a "supine stander," which will strengthen Liam's legs and teach him to become more comfortable with standing on his own. As you may recall, his legs had been in and out of casts (Ponseti method) for the first 8 months of his life. The primary objective of the casts was to straighten out his clubbed feet. They also served to initiate mobility in his ankle and knee joints which gives long-term benefit to his arthrogryposis.

As of now, we are still struggling with Liam being able to sit up completely on his own, unsupported. Also, we don't expect him to learn to crawl because his knee joints are still too stiff to bend fully. Now we are teaching him to stand, using the stander, hoping he will someday take the initiative to stand on his own; and eventually take his first step. Seeing how well he tolerates the stander, I believe we are well on our way in the right direction.

Playtime and Other Things

Just as the last several months, this past month has flown and now we are few days away from Liam's 10th month birthday! I have been witness to a steady increase in his alertness and perception of his surroundings. In therapy, Liam has been introduced to toys that create a "cause and effect" scenario. For example, when he presses a button or a switch, the toy will make a loud cheerful sound or release another toy such as a ball. His success in activating such toys is lately met with a delighted screech. It's so unexpected, I haven't been able to catch it on video yet.

Further to Liam's success with his toys is that he is even more motivated to learn to sit upright unsupported. This is also somewhat unexpected. After many months of castings on both of his legs, we expected Liam to be further behind schedule in this milestone. In this, he is proving to be an overachiever and in a good way!

And speaking of milestones, we have accepted that Liam will likely learn to walk without ever crawling due to the restrictions in his elbow and shoulder joints. However, he is rolling around all over the place. So we now have a large play mat for him to roll all over to his heart's content. He loves it!

As a last note regarding toys, he particularly favors any that he can easily get a grip with his hands as pictured
below. His favorite is a rubber ball with holes all around it so he can hold on to it and move with it. It provides an incredible stretch to his fingers, hands, wrists, elbows and shoulders that he does all by himself! So, to any others out there who know children with arthrogryposis in the upper extremities: this toy should be high on your list!

Road to Progress: Part One

12 days old (yes, that is a stain on his cast)

The very day he was born, Liam was outfitted with casts on both of his legs. The only joints not affected by arthrogryposis were his hips. He had no trouble (and still doesn't) kicking those legs all over the place. You'd better stay out of their way during diaper changes! The best thing about his mobile hips combined with casts on his legs, is that he now has a very strong mid-section.

10 weeks old - occupational therapy

Liam had his casts changed each week for the first 3 months of his life before he had his first procedure to his Achilles tendons. During this time, he had therapy once a week in the hospital to work on the joints in his arms and hands. He was even given tiny customized hand splints to continue giving his wrists a constant mild stretch.

11 weeks old - recovering from surgery

Liam continued to wear casts constantly for about another 4 weeks after his first surgery. He was able to enjoy a couple of months of freedom and we are back in casts again. But that story will be for another post.

Let's Get Technical

Arthrogryposis

So what is arthrogryposis, anyway? Well technically, it is called arthrogryposis multiplex congenita (AMC for short). It is defined as a non-progressive, congenital disease referring to multiple joint contractures and muscle weakness. The most common form of which, as in Liam's case, is amyoplasia, a lack of muscular development and growth, showing deformity and contractures at most joints. As I posted in Liam's story, in layman's terms, it means his joints are abnormally stiff and lots of therapy and possibly numerous surgeries will be required to make them not so stiff.

The exact cause for arthrogryposis is largely unknown. There are a number of theories, but the doctors involved in Liam's treatment have not been able to match his case with any of them. My pregnancy was perfect as far as my doctor could tell and there have been no genetic instances of contractures in the past except for the occasional instance of the more common clubbed foot.

There is a theory that suggests that at about the 8th or 9th day of gestation, there is a crucial development phase in which the nerves fuse with the muscles in the fetus. And during this phase, the fetus should be mobile in the womb. If the fetus, for some reason, remains immobile, then the result could be arthrogryposis. This is the theory that we have heard in Liam's case, though no one is entirely certain. Whatever the cause may be, we have been and will continue to do everything we can for Liam to be fully ambulatory without the need or support of devices.