Keira's behavior has been a bit off and there were several small things noticed the last two weeks. A big part of me thinks (hopes) that it's just part of her being older and more independent, but then there is that nagging suspicion that it might be early signs of OMS still being active. We've been here before and we know we just have to wait to be certain, but I don't think it has gotten any easier. :(
I love that our doctors are so involved and spend the time to think through scenarios with us when stuff like this happens. Unfortunately, we're not thrilled with what our next step might be. If Keira does start showing more signs of OMS, we would have to add Rituxan to her drug therapies. This is a "big gun" in terms of medication and has a lot of side effects.
She's been doing SO WELL that I hate to think we have to do something this big, but we wouldn't be good parents if we weren't mentally prepared for what a relapse would mean for her and the rest of the family.
We are continuing with the Prednisone wean this month. There is really no reason to keep her on it since it's such a low dose, however, we will now give her hydrocortisone daily until we're sure her remaining adrenal gland is making steroids again.
Several people have asked why we wouldn't just up her steroids again which is an incremental adjustment to her current course of treatment, and has known to bring her OMS symptoms into check.
The reason is that we are at a stage now where we are fighting TWO battles: OMS Symptoms AND the long-term affects of steroids. Keira has been on Prednisone for nearly 2.5 years of her 3.5 year-old life. While Prednisone has been seemingly successful in addressing OMS symptoms, it simply cannot be a long-term solution, which is why we initially started weaning it about 2 years ago, and very slowly got down to a dosage that we simply couldn't wean beyond without causing a flare-up. This was about 10 months ago when we added Dexamethesone as a pulse-dose (every 28 days) to her every-other-day Prednisone regimen. The reason we did this was to see if the Dex pulses would counteract OMS activity while we continued to wean off of Prednisone. This may seem counter-intuitive (why add one steroid to get off of another?), but the pulse doses have fewer long-term issues than an every-other-day dose, and hopefully the pulses of Dex would allow us to wean off of the every-other-day Prednisone. And for the past 10 months it mostly has... We're down to the lowest dosage of Prednisone we've been and in fact, we think that the Prednisone is so low that it likely isn't even having an affect.
However, we've been at this stage for 2 months now and are seeing several small signs of flare-up (behavioral issues, minor instability, etc.) starting to surface. So as we wean off of Prednisone, the big question is what we do if Keira can't be stable with the Dex pulses alone? Going back up on Prednisone isn't really an option because while we know that it will likely work, we're trying to find a long-term solution here. Increasing the Dex pulses (bi-monthly perhaps) is one option and we could try to push for that, but again, that would introduce a dependency on Dex (albeit less of an issue given the dosage frequency relative to Prednisone, it's still an ongoing steroid dependency).
Rituxan is another drug that has been used in the US and Europe to treat OMS. It is a "big gun" when it comes to drugs. It is typically used to fight lymphoma, cancer of the immune system, by attacking and obliterating B-Cells, which is where lymphomas are generated. While we don't know the exact auto-immune mechanism of OMS, we do know that B-Cells are likely involved because uses of Rituxan for OMS patients have seen generally favorable affects (implying that by wiping out B-Cells, we're wiping out the generation of the auto-immune antibodies causing OMS).
(Thanks Naveen for the well written medical info!)
Keira is an amazing little girl. We feel blessed that she has been doing so well and that she has so many people who love her and look out for her well being.
She makes me want to do more for all the children that have to deal with the uncertainty of OMS. We're trying to do our part to raise awareness and money for this rare and underfunded disease, but it's hard to find the time and energy after working all day and being a mom. :(
As many of you know we are hosting the 2nd Annual One More Step hike in Golden Gate Park on April 28th. Please consider hiking with us or donating to this worthy cause!