It's been a rough week for our family.
While the girls and I had a blast on our trip to Chicago I started noticing some unusual behavior from Keira over spring break. We were hoping that the behavior was due to being on vacation and out of our routine but after returning home we started noticing other signs of OMS. Keira's fine motor movements were less fluid and she seemed to be more wiggly than normal.
Her behavior didn't improve so Dr. Effinger asked us to visit OT and PT before our regularly scheduled Clinic Visit. Usually I feel reassured after these appointments and although neither therapists saw anything they thought was alarming, my mom instinct still thought that something was off and this might be the start of something bigger.
I would be lying to say that I wasn't scared.
We've come so far since Keira's diagnosis and things have been going so well in the last 12 months. However, our appointment with Dr. E was the tipping point. There was definitely an unsteadiness in her hands. The shakiness in Keira's left hand was quite apparent but Dr. Effinger was able to see unsteadiness in her right hand as well. Keira was also off balance compared to what we know she can usually do during these check-in appointments. Furthermore, Dr. Effinger noticed that Keira had trouble jumping up onto the exam table and that she was leaning a lot more than usual, which indicates muscle fatigue/weakness.
What really scared me that day was the look on Dr. Effinger's face. Usually the doctors reassure our worries and say that we should be vigilant and watch for another month, but clearly she was worried and already making a plan for who to call and what our options for treatment would be if things progressed.
That was last Wednesday.
The rest of the week felt like watching a pot waiting for water to boil. Keira started waking up agitated at night without explanation. We also started noticing little things on a daily basis, even though she kept right on living life, and doing all the things she loved. Keira amazes us at how resilient she is and how easily she compensates when something isn't working the way it should be. I wish she could just tell us when things are different for her. She is beginning to try - "My body feels funny." That's all she can say and it's so hard for her to articulate what that means. I have to remember that she's only five.
Dinner on Saturday was telling. She kept trying to eat with her hands instead of her fork. She was making a huge mess. She couldn't sit still in her chair. Naveen asked her if it was hard to use her fork. She said, "a little." Then he asked her why and she replied, "I don't know." Of course she doesn't understand why this is happening to her body, as adults we don't understand it... how can we expect her to be able to comprehend what is happening?
As her symptoms continued to get worse our anxiety increased. At this stage, there is no protocol for what to do next. We know she responded well to dexamethasone when we couldn't get her off the prednisone. She finished her last dex pulse in October 2013 which was about six months ago with just a 1 pill dose. We could try another full pulse. There is also rituximab. This drug is being used with more and more OMS patients but there are pretty high risks/side effects associated with it.
Our appointment Monday afternoon couldn't come fast enough. By Sunday evening we were certain that we could see signs of OMS. Keira lost her balance twice and just fell over. Once trying to get into the bathtub and once when leaning down to pet Nip. She was clearly taken aback by her inability to stay standing. Heartbreaking.
Dr. Effinger spoke to several of the other OMS doctors and the consensus was to try one 3-day dex pulse to see if this is just a minor blip in Keira's recovery. The big elephant in the room was "Are we just delaying the inevitable? Is rituximab going to be what we have to resort to in a few more months?"
Maybe. We don't know. We hope not. But it wouldn't be fair to Keira to not try the more conservative dex that we know has worked for her in the past before resorting to the more aggressive and risky Rituximab.
The funny thing with the last week is that once we explained to Keira that she was going to have to take her "little blue pills" again for a few days it seems to lift a weight off of her shoulders. Her mood was light and happy tonight. She took her meds without complaint and we had a great discussion about how sometimes our bodies need medicine. I could be reading more into it than I should but I think she was relieved that we were doing something to help her feel better. I hope that someday she will be able to tell us and her doctors when she thinks it's time to for medication... actually I'm lying, I hope she never needs another pill or treatment again but I know that is not being very realistic so I will settle for Keira having insights into how her own body feels and I will fight to get her what she needs.
I know that the next few days will be tough for Keira. Please continue to send her happy, positive thoughts that she can get through the worst of the steroids and begin to feel better again.