Warning: This is a long and sad one.
I’ve been putting off writing this update all evening. I just wasted thirty minutes looking at forearm tattoos, but I know how important it is to keep all of our family and friends updated, so here it is.
Today felt all too familiar. The emotions of defeat hurt deeply. This NF2 roller coaster is not what I wanted for my child or for my family. Today I cannot stand it. Today I hate NF2. Hate it. I am very aware that this life isn't meant to be smooth sailing. It isn't intended to be easy or fair. I am familiar with scripture that suffering is necessary to enter the gates of heaven, but I'd like to come up for air and get out of the deep waters for a little bit. Tomorrow, I know I will find the positive or see how much worse things could be for Jacob and our family. Tonight, however, I am crushed.
The hardest news today was that Jacob needs a spinal fusion, immediately. Dr. A is working on finding the doctor he thinks will be the best in Florida to perform said procedure. Dr. A started by contacting a few surgeons he knows at Shands UF in Gainesville, FL before we even left clinic. For those wondering, yes we had to make the decision during the cervical spine surgery whether to have a fusion. We contemplated it for over a year. I’ve cried all my tears out today and beaten myself up for choosing to not have the fusion during his surgery in August. I assume this would be the initial reaction of any mother, but when I get passed the painful thoughts of my precious child having to have another invasive surgery, and when I swallow the displacement surgeries cause our family and put aside the worry of Jacob missing more school, I know I made the right choice back then, that Jacob and I together made the right choice. Dr. A, without a doubt, was the right surgeon for the cervical tumors removal. It was also the right choice to help the NF2 study and for the NIH to be able to use Jacob's schawannomas for research. We knew Dr. A would do his best to preserve Jacob's left hand. I know he cares and has Jacob's quality of life at the top of his priorities. I couldn't have the fusion for Jacob and the surgeon I wanted, so we took the chance, hoping Jacob wouldn't be in the 15% of people who need the fusion after a lapendectomy and went with the right surgeon for tumor removal. Even though I know all of this, even though I truly believe we made the best decision at the time, I am still extremely saddened that Jacob has to have another extremely invasive and painful surgery and recovery. Regardless of my emotions, I do not regret having Dr. A do his cervical surgery.
In random order, or a “stream of consciousness” as I hear so frequently:
Many worries come with a fusion. After the fusion, Jacob's spine, from roughly C-4 to T-4, will be very hard to see on any imaging. With CT scans, the metal makes everything look white and the MRI is unclear as well. Dr. A said clinic exams will be crucial and if there are worries, they can use a myelogram, which is not pleasant at all. Being able to clearly see the entirety of Jacob's spine with MRI in order to monitor his other spinal tumors was the primary reason we wanted to wait and not do the fusion. Because he doesn’t have a fusion, we were able to see that the area from August where the tumors were resected looks pretty good. The spinal cord is not compressed and the tumors have not grown back. That’s about the extent of the good news.
Jacob is having left hand function issues with increasing frequency as of late. He struggles doing simple tasks like opening drink containers, opening the front door or tying his shoes. We have high hopes that straightening up the deformity in his spine will take pressure off the nerve that has the tiny bit of tumor which was left at that C-7 nerve, but Dr. A said it very well may be from scar tissue forming or that specific tumor changing since surgery. He said it is very far out from the spinal cord and they don't see it on MRI. We all agree that having some loss of function is better than them removing the entire nerve, and tons better than Jacob not having any function left in that hand at all.
He’s also having leg weakness and knee and hip pain, all of which have progressively worsened in the past few weeks. We didn't do any sightseeing in DC this trip because Jacob can hardly walk straight or without pain. Dr. A believes this is also from the curvature in his spine. When the spinal cord is misplaced in the “S” shape, being stretched can cause problems with sensations coming up from the legs and the ability to feel properly. With these issues, a person doesn’t know where their legs are and causes difficulty walking, the reason Jacob is stumbling so bad. The fact that he feels better when he is laying flat (and his legs are “warm”) suggests the pain and discomfort is from the deformity. It also explains why it’s getting worse. Dr. A said it is obvious on Jacob's x rays from this week that there are severe changes.
As for the numbness in his belly, Jacob most likely has a belly tumor, but since the option would be to remove the tumor, which would cause numbness after removal, they feel it is unnecessary to locate it. The area in the spine that effects the belly area (at T10) was clear of tumors, so they do not think Jacob is in any danger because of the numbness.
There is an NF2 study at Johns Hopkins showing that lapatinib can be taken up to the day before surgery, so we’re in the clear for that as well.
I could write more about the fusion conversation, but you get the idea.
The other big, but not AS pressing, concern is the right VS. It's grown about 3-4 mm since July, which is considered fast. I’m sending Dr. A Jacob's MRI from Orlando to see if he can tell when it grew. We don't think he’s been on the Tykerb long enough to say whether or not it is working. Jacob's hearing is still pretty good (thank goodness). His ABR testing shows changes for the worse, but that’s expected with the growing VS. We’ll think about it more after the fusion business.
Lastly, I want to say thank you to Casey for driving us to and from the airport. Not having to catch the bus or ride the metro was so awesome, especially with how Jacob has been feeling. Jacob and I both really enjoyed spending time at Casey's house Wednesday afternoon and really, really enjoyed dinner! Becky and Savannah joined us and we had a great time. Jacob said it was fun to hang out with Casey's girls like he did growing up. Oh, and I held a rat for the first time too. Lacy has two!
I will keep everyone updated. I know I am always begging for prayers, but Jacob needs them. Oh, yeah, and we made it home safely, with a much-too-eventful ride home, complete with every redneck adage in the book! I couldn't believe it was 80 degrees in Tally when we landed, but I didn't care I was so happy to be home and reunited with the rest of me.