James' Journey with TSC

Although the title might suggest some deep metaphor, we are too tired to mean anything other than the literal sense. March officially marks six months from our initial appointments after James’ first seizures, so we are on the path of follow-ups now. But what would life be without a little newness to it? Enter James’ newest fashion accessory: his cranial orthosis helmet.

With the challenges that James has with mobility and the delays with sitting up, he developed a flat spot on his head called plagiocephaly. It’s not a huge issue, and even babies who aren’t medically complex can have the same thing to varying degrees. Luckily, we still have time where his new helmet can help adjust the growth of his head to be somewhat more symmetrical. Our hope is that be he’s around a year old you wouldn’t even know that he had a helmet in the first place and there won’t be a flat spot!

It does pose a couple new challenges though. He has to wear the helmet for 23 hours a day, which doesn’t seem to bother him at all, but does make it a little harder when he is wanting to be rocked to sleep at 1:00 a.m. It also had given him a new challenge with both sitting up and rolling over. The helmet itself is fairly lightweight, but at this age even a little weight can throw them off. In a week or two he should be back to working hard and progressing.

Onto the eyes. James’ medication called “Vigafyde” is a form of Vigabatrin. Vigabatrin carries a rare but known side effect that can cause permeant peripheral vision loss. Before starting the medication, children are given a baseline evaluation, then have to follow up with the pediatric ophthalmologist every three months. Originally, we weren’t going to see the ophthalmologist until October, but now Billings is going to be seeing a little bit more of the Maxey family.

Thankfully this time James did not need to get his eyes dilated. He got to watch a few different toys and one striped spinner so that we could determine that his eyes were working correctly thus far. Although he was very much ready for a nap and didn’t really take interest in the first couple of toys, he passed with flying colors as soon as the spinner made it’s appearance. We will be checking again on his vision in June.

Lastly, James had a follow up echocardiogram (heart ultrasound) with the pediatric cardiologist to see if the rhabdomyomas in his heart have changed at all. We are happy to report that the three existing rhabdomyomas have gotten a bit smaller! However, he does have a few more visible rhabdomyomas that we hadn’t seen on the first scan. They are smaller than the first three, but it does mean we need to do another echocardiogram in six months to see that things are on the right track. At some point, he may have to do a cardiac MRI to check on everything, but nothing is set in stone yet. For now, we wait.

On the day to day front in the Maxey home, James is continuing to progress well in occupational therapy, feeding therapy, and physical therapy. We have an amazing team that we are so grateful for and they all want James to shoot for the stars just like we do. James has been off of his corticosteroids for a little over two weeks at this point. He is much happier, and his laughter and smiles brighten everyone’s day. We ask that you pray that he is able to get back to a “normal” sleep pattern. After being on that medication for so long, it has caused issues with his sleep (and therefore our sleep), from fussiness to abnormal wake ups.

We are tired, but again so grateful to God that the steroids did their job and managed his spasms. Seeing James smile and grow is the biggest blessing that we could ever have as parents. Thank you all for continuing to pray and love on our little family!