Pool Therapy

It's hard to think of anything more relaxing than going swimming on a hot summery day. That is what Liam did over the past weekend for the first time and he loved it! We have a little float for him to recline in and go with the flow. He was kicking his legs to paddle himself along and even started smacking the water with his hands to cause little splashes in the water, all while shrieking in excitement and laughter. It's safe to say I know what where Liam will be spending a lot of time this summer!

 I can't get enough of these pictures of Liam in the pool, so I have been sharing them for all to see this amazing boy enjoying the pool. Over the summer, we plan for his therapists to do their work with him in a wading pool. The water does wonders on his joints. He doesn't get upset or fussy when we work his muscles in the water. It also certainly helps him sleep good!

Work and Play

It seems we are always looking for new ways to help Liam increase mobility in his muscles and joints. Well, it turns out, that over the course of the last few weeks, Liam has shown us a thing or two in what works best for him. When his physical therapist introduced him to a toy that he can activate by sitting and pressing a button, he was thrilled and shrieked in excitement.

Overnight, he seemed to be sitting up so much better on his own. Keep in mind, that though he is 10 months old, he is still struggling even with prop sitting. This is due to the awkward positioning of many months worth of castings on both of his legs. He has not been able to work to this milestone without a major obstacle. In the meantime, we have worked on other milestones, such as rolling front to back and back to front which he has mastered.

Most recently, we have been trying to find a way for Liam to effectively play with his toy laptop computer. It has these wonderful buttons that he loves to press. However, the restrictions in his elbows, wrists and shoulders prevent him from controlling the toy. Then I remembered we have a little stool that converts to a little chair. We use it as a makeshift desk for his little computer. Now, for the first time, he is motivated to press the buttons with his individual fingers rather than the flat side of his hand.

Can't you see I'm working here, Mom?

This little guy is going to be a computer whiz like his Dad!

We are also seeing a lot of increased range and mobility in his shoulders. We can lift his arms pretty high and open them up pretty wide. But the distance he can stretch himself is a different story as he is challenged by the shortened tendons in his joints. But as he continues to explore his environment and play with his toys, the tendons stretch and loosen and create less of a challenge each day. Also, of course, aggressive therapy plays a major role in his continued success.

Adaptations

It's hard to believe that in just a few days, Liam will be 9 months old already! Time has flown by so fast and this little boy is growing even faster. He is as fun and as motivated as ever. What a joy to have around! Also, Liam's orthopedist and his Occupational Therapist have said that every one of the arthrogrypotic patients they have worked with have been exceptionally bright and quite distinctly so. We can already see that Liam is very smart too. He had started to say "mama" and "dada" at barely 8 months old! What an incredible guy!

One of my goals is to give Liam the gift of independence. It became easy for me to see now how much we take our independence for granted once I realized that Liam may not even be able to feed himself for several years to come unless we take the necessary steps to facilitate self-care. I have asked his therapists to help me brainstorm ways that we can adapt his feedings so that I can start teaching him how to hold his own bottles and eventually to bring his own spoon to his mouth. He may seem a little young for doing all of that now, but now is the time for him to start learning. 

The challenge that Liam is facing is that his elbow joints are still too tight that they do not fully bend yet. They are getting looser, thanks to the daily massages and regular aggressive therapy he has been receiving. And while we haven't thought up any major adaptations yet, we have figured out how to prop his bottle in such a way that he can hold onto it while giving his fingers, thumbs and wrists the mild stretch that they regularly need. He really enjoys it!

Play Time

One of the many challenges for an infant with any kind of restrictive muscular disease is finding motivation to explore the world around them. Fortunately, Liam has been very motivated since birth to seek and explore his environment. In the beginning, my husband and I were instructed to give Liam therapy in his hands, arms and shoulders during feedings to loosen up the tightness in his muscles and his joints. Regular therapy sessions have instructed us on different approaches to take to maximize his range of motion. 

It has been one of our goals to teach Liam how to move and play independently, therefore providing himself with therapy while having fun with it. As he gets older (he is now 8 1/2 months old), he becomes more alert and aware of everything around him and what he wants to do with it. No matter the obstacle, if he wants to do something, he will find a way to do it. An obstacle he faces right now is his special boots with the Ponseti bar. But even with this device on, he plays and moves. Currently, his favorite mode of transportation is rolling over onto his belly and continuing onto his back, repeatedly. In a blink of an eye, he is across the living room moving this way, Ponseti bar and all!

A Modified Approach

Modified belly time

In addition to the serial castings that Liam receives in effort to increase mobility in the joints of his legs, he must also receive aggressive therapy not only to work the joints in his upper extremities, but also to help him reach major milestones that are expected of all infants his age. His joint restrictions make meeting certain milestones more difficult, such as the ever important belly time.

We have had to make several modifications to help him achieve such milestones and while he may not entirely achieve them in the average time window of most infants, his milestones are certainly emerging through the efforts in his therapy sessions. Because of the joint restrictions in his arms, Liam cannot raise his arms over his head or even very far out to the sides of his body (such as in a wingspan motion). But one thing I have learned on our journey so far is that if a baby wants to do something (and Liam is no exception), then that baby will do it. Even though I don't need reminding that he is a normal baby, he does seem determined to remind us that he is just as motivated to learn to function as though there are no restrictions to his movement. In short, Liam is driven to succeed, no matter the obstacle.

Below is a video demonstrating the restrictions in the joints of Liam's arms. He cannot bend his elbows as the tendons are so short, they do not bend at all.

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.