We went back to LPCH today to check in with Dr. Effinger/Dr. Twist about some of the more concerning things we have been seeing with Keira. I have to say that I may be biased since I'm her mom, but I think that Keira is just amazing. Through all of these ups and downs she just keeps living life and finds ways to do the things she wants to do.
Unfortunately, there have just been too many coincidences for her symptoms not to be an OMS flare up.
On Wednesday morning I noticed that Keira was using her left hand to help stabilize getting the spoon to her mouth. After talking at length with Dr. Effinger we decided to try to get her in to see OT before Friday but at this point we knew that a medication adjustment was needed. Keira and I were able to get in to see Eiri and although Keira is able to do all things age appropriate (sting beads, cut on a line, lace cards, color with a crayon, etc) Eiri did notice a shakiness in her hand and definite tremors when she was trying to stack blocks. In the past Keira has been able to build a tower with 10 blocks and was only able to stack 6 before they would tumble.
In addition, this week Naveen and I have both observed Keira pressing on her eyes. She has told us separately during our bedtime routine that her eyes feel "tired" and that she is ready for bed now and will forgo rocking or songs. There have been no noticeable signs of opsoclonus but she never did present with traditional opsoclonus symptoms even when she was at her worst. We do know that her eyes have bothered her in the past and she tries to express it to us in her own way. We really try not to put ideas or labels for things on her but rather listen to what she says and observe her behavior.
As we left the house for our appointment on Friday morning I asked Keira to let me go down the stairs first. She has been quite stable on stairs for a while but after all these little things popping up this week I just felt like I should be in front of her. Thank goodness I did that because as we made our way down the stairs she tripped and fell forward sliding down a few stairs into me. Poor Keira didn't understand what happened to her body since this has never happened before. It's heartbreaking to see your little girl start struggling with things she knows she can do. :(
Dr. Effinger and Dr. Twist had already talked at length before our appointment and we've decided to take a few steps back with her steroid dose. We know that she was stable this summer when she was taking 2ml/twice daily, every other day, so this seems like the most appropriate place to go back to. We are hopeful that this will be what Keira's body needs to get back to her baseline, but keep in mind she has not had IVIg for almost 12 weeks. For the past year the longest we have gone between IVIg treatments has been 8 weeks. While we know the medication only stays in the body for 3-4 weeks the combination of IVIg and steroids might have been what K's body was doing so well on. Putting IVIg aside for now, we are going to see how K does with an increased dose of steroids and re-evaluate in another 2 weeks. Obviously, if she gets any worse we will make another medication adjustment sooner.
While we know that OMS is a lifelong condition that will undoubtedly have its ups and downs it is hard to comprehend how this is happening when Keira has been doing so well for the last few months. She is healthy and has been handling the steroid weaning relatively well. It just appears that her body wasn't quite ready for the next step.
Although this is a time of uncertainty for our family, we have faith in the knowledge of Keira's doctors and in our daughter's determination to overcome OMS. We are blessed to be surrounded by the love of our family and friends which help us to remain positive and strong. Only happy thoughts for the rest of 2011.
