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We're thrilled to be at home and Keira is most definitely happy to be in her familiar space.
I know that many friends and family still have questions about OMA, Keira's condition, and the treatment path. While blog posts and emails help, it is hard to gain a full grasp over the subject matter even for us, who are living and breathing it every day, given that it is such a rare condition. So please continue to send in your emails to me or Crystal and we will do our best to continue to educate and update everyone.
To recap, about 3 weeks ago, Keira was a fully walking, running, climbing, grasping, chatting, exploring, rambunctious 17-month old. On Saturday September 24, we noticed a minor hand tremor and mild irritability after waking from a nap. 5 days later, this had quickly progressed to losing balance and coordination of her limbs, and 2 days after that, the loss of her ability to walk, along with long episodes of inconsolable irritability and body tremors. These were the peak of here OMA symptoms as of Monday October 4 (the day of Keira's surgery).
Since that day, Keira has started some initial steroid treatment for her OMA and everyone who has been at the hospital has observed some improvements in her symptoms. Keira's body and hand tremors seem to have subsided and are relatively minor now. Furthermore, she seems to be a bit more stable at sitting on her own. However, we're still not confident that she can sit without someone spotting her, she cannot walk, and her fine motor coordination has not returned to normal. Furthermore, here episodes of irritability seem to still come and go, and we're figuring out behavioral strategies to manage these.
Moving forward into more intensive OMA treatment, the hope is that we can get these physical and behavioral symptoms to subside significantly, although as I've mentioned, there is not enough data to make statistically relevant projections as to how Keira will respond.
As we've learned, there are a handful of drugs that are used in the treatment of OMA, and while there appear to be different "philosophies" depending on whether the OMA is being treated from an Oncologist as a result of Neuroblastoma (which Keira is) or whether the OMA is being treated by a Neurologist (which some hospitals do because this is technically an auto-immune neurological disorder), in either case, the drug treatments aim to suppress the immune system to allow for OMA symptoms to abate.
The first line of treatments that Oncologists tend to follow the use of some combination of steroids, immunoglobulin, and chemotherapeutics. If these don't work, there are other drugs such as ACTH that Neurologists tend to use.
For the OMA treatment itself, we're actually lucky that Keira qualifies for Stage 3 Clinical Trial that will use Prednazone (steroid), IVIG (intravenous immunoglobulin), and Cyclophosphamide (chemotherapeutic) over a period of ~18 months to treat the OMA. It should be noted that the Cyclophosphamide is being used to treat OMA and not cancer, even though it is a chemo drug. It was discovered some years ago that when treating more advanced Neuroblastomas with Cyclophosphamide, those patients that were also diagnosed with OMA saw some improvement in symptoms, and hence it is now part of the treatment protocol.
We are scheduled to go back to the hospital on Monday to get an understanding of the treatment schedule and trial roadmap. Her first round of treatments will likely happen next week though so we expect it to be another tough week as it will entail 3 hours of IVIG treatment on Tuesday, 3 hours of IVIG treatment on Wednesday, and 2 hours of Cyclophosphamide treatment on Thursday. These treatments typically have side-effects for a few days following treatment so we anticipate that Keira will be feeling pretty crummy most all of next week. However, we are thrilled that we are beginning treatment so soon after being diagnosed as in many OMA cases, this doesn't happen for months so we're very encouraged that this is happening so quickly.
We'll provide more details when we learn more on Monday.