Well as usual we know these appointments take 90% of your day. We spent about 6 hours at JH waiting and talking and waiting and talking some more with Oncology. Her doctors went through all of her test results and went over a few other items with us. Leaving us happy, confused, and then concerned.
First off - her tumor was about 90% benign...great news. She was not found to have any cancer of the bones and everything was looking fine on the MRI's she had taken. With the entire tumor being removed she has been placed in a Stage 1 and everything is favorable. The only unfavorable element is her age. We received somewhat of a schedule for the next year and what should be expected from the cancer side of things.
Haven will have an MRI of brain and entire body every 3 months. First one being March 3rd.
Then every month she will have blood drawn for testing. This is all for the first year. After that it will either go to quarterly or every 6 months depending on condition. We will not need to do chemo for the cancer at this time.
YEAH...great news. But with great news comes bad news.
As her doctors (hours now) kept explaining and we kept feeling more and more confused. If Haven only had the tumor and the cancer we'd be looking great and pretty much in the clear. But Haven does not only have this. She has Hypothalamic Dysfunction and Paraneoplastic Syndrome. What does this mean? After 6 hours I can give a brief definition to the best of my understanding.
When Haven's tumor developed around age 2 her immune system kicked in to try to kill the tumor. However, not being a normal tumor, her tumor kinda morphed itself into a Hypothalamus. Meaning - it took on the same characteristics and hormones of her Hypothalamus. In turn this confused the immune system and it was unsure of what to attack. In the confusion, the immune system began attacking her actual Hypothalamus of the brain causing functions that most of us do not think about to stop working or change and did not go after the actual tumor. Notable changes - personality, bodily functions, vision, appetite control. Her tumor was actually giving her the hormones and not the brain. So as it stands right now her immune system is still attacking the Hypo.
Our doctors at JH have not had a case of this ever, and have made it clear to us that there are less than 1% of children in the world - meaning approximately 5 - 1o kids in the entire world that have had this. Not thousands but only 5 to 10. Haven now being one of them. Over the past few months our doctors have been working hard to find anyone that has had a child come in with similar symptoms as Haven....and recently they found a doctor with a similar case. He's in Chicago - had a child with the same disease - and found a way to treat it. Only this case was further along than Haven due to not knowing what it was. Our doctors are working on a way to make sure Haven does not get worse.
Another item they are working on is part of PNS - called - Opsoclonus-Myoclonus Syndrome. I have added a link to this on the side. I have not had time yet to fully research it - but her doctors are working on this as a possible item.
Treatment discussion: We're going to give Haven approximately one month to see if her brain can counteract the immune system and fix itself sortof. We want to see what changes are made to her by just removing the tumor. Already in 2 weeks we have seen major changes in her moods, emotions, and other areas. We're taking notes of anything we may notice - as we have noticed things that most people wouldn't. If her brain is unable to start fixing itself we have a few options that the doctors are looking in to with the doctor from Chicago.
1. Chemo - they would do chemo on this part of the brain to try to correct it.
2. Immune suppresant - pretty much shut down her immune system until everything in the brain starts to fight back.
Well right now #1 doesn't sound half as bad as #2 to us. But we are currently in a standstill. We could tell her doctors don't know what to do and are pretty much in the waiting game with us. We're very excited we are not starting chemo right now - yet I'm sad I went ahead and cut her hair off. I really wanted to wait until age 5.
We have another full day meeting with the doctors on March 4th - after her MRI - and will be able to give more updates as to Miss Haven.
Love to all