Now for a Jacob update...Many of you may have seen requests for prayer and small updates last month on facebook about him. Here is a synopsis of what happened. Jacob began complaining of a headache in mid-October. His headache persisted until he began having nausea and vomiting. The next day his headache got worse until he was literally screaming and crying in pain. He is a tough little guy so needless to say, I got really concerned. I took him to the doctor who was further concerned as he didn't seem to have a virus or anything (also unlikely as he had shared his sippy cup with Luke the morning he got sick, and Luke was still fine). Since his illness presented with a headache, his doctor felt we needed to go to the hospital for a CT to make sure there wasn't something we were missing, especially in light of his condition and its potential. On the way to the ER, I called our team at Boston Children's Hospital, hoping that they would tell me we really did not need to worry about that. They did not, though. They said that we couldn't discount his condition as a possible source of the problem, but really needed a MRI rather than a CT to ensure we would see any potential vascular issues. They also said that since we were putting him under, we needed to MRI his chest and spine as well, to see that lesion, as growth into the spine could cause a blockage of Cerebrospinal Fluid flow, which would give him a severe headache as well as nausea and vomiting. So we moved forward. I will say, that it was our first ER visit since his diagnosis, and it was my first experience having to explain his condition to every physician and nurse (as none had heard of it understandably). They tried to prepare us for this in Boston, but it is still such an uncomfortable feeling being the authority on his condition in the hospital, and telling them what tests to order, and reviewing the results as well. I have never been more thankful for my medical background which enables me to communicate on their level, but it would still be nice to simply be the parent and let the doctors do their thing. They were great in asking questions and learning about this condition they had never heard of, and allowing, no encouraging me to make the treatment decisions. It was scary though, and never was the burden of responsibility for his care heavier.
I stayed in constant contact with Boston in the weeks to follow, as they received and reviewed the MRI's. The process took longer than normal as they brought in a Neuro-Interventional Radiologist (who we have never had to see before), to consult on the findings in his brain. The news was good as I expected on the chest/back scans. NO regrowth or rerouting of lesion in the chest at all! Residual scarring from our treatments in 2014 as to be expected, but absolutely no visible lesion. This was awesome news, as the lesion is completely involuted after our aggressive treatments. They said it will likely regrow in the future as such is the nature of his condition, but we potentially could have bought ourselves years of respite from this lesion. They said they could not have hoped for so successful of results from the treatments, especially considering the large size and difficult location of this lesion. They did see new growth of the lesion on his back and even new lesion on the other side of his back, but it is still clear of the spine. They asked if it hurt him or bothered him, or was disfiguring, all of which I answered, no. I elected not to have this treated right now since it is not hurting him, and they said to let them know if it did and we could arrange treatment. We are thrilled at the prospect of him not needing treatment for the foreseeable near future, though we know that that could change at any time. We'll take what we can get though, so this was great news.
As far as the lesion in his brain goes, it is a bad news-good news-bad news situation. The bad news (in addition to there being a brain lesion at all), is that the lesion is large, 3.5cm X 1cm X 1cm. That is pretty big, especially when you consider it is in the brain of a 4 year old. The good news is that they have never had to treat a brain lesion in a BRBNS patient before. The other bad news is that that really doesn't mean anything considering there are only a handful of the 150 or so patients with BRBNS, that have brain lesions. So the sample size is too small to say with any statistical probability that Jacob's will not be a problem or need to be treated. When you are talking about a handful of people in the world, anything is possible. They are hopeful that his will follow the trend and never cause problems or need treatment, but cannot tell us that for sure. It is too large to assume it will be fine, so they want to look at it again in a year to determine a baseline. Is it growing, changing, etc. So basically, it is there; we have no idea if it will be a problem, and like everything else, there will likely be no warning or predicting if or when it will be. It is one more thing (and a huge thing at that) looming over us, that we have to worry about. One more potential for harm to our Jake. We desperately ask that you keep Jacob in your prayers that this will never be an issue.
Happy Holidays to all of you from the Casteel's!