On Monday November 15, Keira had a her monthly clinical visit, where her primary doctor evaluates her and checks in to see how she is doing with her OMA symptoms. Keira also had her weekly Physical Therapy session, which helps us know how her motor skills are progressing. This day served as a good checkpoint for us to gauge how Keira has progressed over the course of her first full month of treatment.
Now if you've been following Keira's story all along, you've probably received updates on Keira's improvements and milestones over the course of this past month. But it helps to take stock in all that has happened:
- When we left her last clinic visit on Monday October 18, Keira could barely stand with support, couldn't walk, and was still having some behavioral episodes and sleep disturbances.
- A week or so after that, Keira stood up and started walking
- A week or so after that, Keira started sleeping 10-12 hours through the night
- A few days after that, Keira started sleeping solo in her own crib through the night
- And since then, we've seen continued improvements in her sleep, mood, behavior, and motor skills
From her doctors' perspective however, they only remembered the Keira that was immobile from the last clinic visit, so you can imagine how elated they were when they saw little Keira bouncing all over the clinic. We had called one of her docs shortly after she started walking, so she was aware of her improvement, but it's one thing to hear about it and another to see it. She was so excited, that she called her attending physician who came over from her office across the street to see Keira first hand. Needless to say, she was equally as excited and amazed by Keira's improvements. Actually every doctor that came in to see her (from both the oncology and neurology teams) were pretty surprised to see how well Keira was doing - so much so that Crystal and I were a little taken aback by the enthusiasm, probably because we had no other basis for comparison or any expectation around her improvements. While we were very happy with how Keira had been progressing this past month, we hadn't realized that this was on the very positive end of the spectrum.
The clinical trial that Keira is part of has a specific treatment protocol that adjusts drug dosages/frequencies based on how patients are doing. The evaluation scale consists of five dimensions for OMA symptoms that are graded each time she goes in for her monthly clinic visit:
- Stance
- Gait
- Arm/Hand Function
- Opsoclonus
- Mood/Behavior
Based how patients are graded on this scale dictates drug dosages/frequencies. Keira's grades have progressed so well to the point where they are cutting her steroid dosage in half now. While this is undoubtedly very positive news, we're also a bit nervous and anxious about it because we're not sure exactly how Keira will respond to being tapered off these drugs that seemed to contribute so much to her improvements. We've heard from other parents that behavioral and mood flare-ups are common when steroids are reduced/removed, but the longer term side-effects of such high steroid dosages are what we're concerned about now, so on balance, the tapering is the right thing to do (and is exactly what is called for in the protocol).
All of her improvements are very encouraging and as parents we're pretty happy to see Keira able to do the things she loves so much. However, we still need to be aware of the long road Keira has in front of her as we manage her treatment over the course of this coming year and beyond. Her immune system is still only mildly suppressed, but with each subsequent IVIG/Cyclophosphamide treatment, it will be further suppressed so we'll have to continue to be vigilant about preventing infections/illnesses. The steroid tapering is something that we're both nervous about so we'll be monitoring her mood/behavior over this month to see whether or not this is affected. Finally, there is also still the general uncertainty around the waxing and waning nature of this disease, where certain symptoms could flare-up for no apparent reason. Even this past month with so many improvements, we did see tremors return then subside, we noticed that Keira was tripping more on certain days, and we still see some randomness in her behavior, and each time we're not sure whether we're seeing the beginning of a trend, a minor flare-up, or if this is part of normal toddler behavior. :)
Taken as a whole though, this past clinic visit validated much of what we had been witnessing with our own eyes over the course of the month, and then some. I don't think that we could have asked for anything greater than what we have already received.
We hope that you all have a Happy Thanksgiving. We have so much to be thankful for this year and appreciate all the love and support we continue to receive from all of you!