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Powered by LifeTypeSome pretty good appointments yesterday, and the snow (9 inches?!) wasn't too bad at all driving up to Dartmouth (one car sped by our line of traffic and a couple minutes later was spun off the road in the median, but she was already out of the car and on the phone, so presumably was fine, but hopefully learned that the passing lane during snowstorms is more difficult to drive in because there are fewer cars and so more slippery). Sutton was snowier than I would have liked, so I took the highway the whole way home.
I got to see the actual MRI results, and some of the pictures are terrible - with huge movements making the images basically useless. I thought the tech would have been able to see that and re-do particular images as they came in, but maybe not, or maybe it is too expensive time-wise.
I could sort of see/understand the "widening" of her right optical nerve vs her left, but I guess we don't have a good picture from May to compare to, so we are going to have another MRI in February (or hopefully January which is potentially better for insurance reasons). The opthamologist reported that functionally, her eyes are fine, and they were (of course!) surprised by how much she can follow directions and they were able to do eye tests that they can't usually do on kids that are so young.
She said that statistically, 14% of plexiform neurofibromas turn into malignant cancerous tumors, but out of the 500-800 kids she has seen over the last 20 years (they transition out of her practice in their 20s) she has seen maybe 3 PNs turn cancerous. I think the 14% number comes from adult studies, so that might be in her future.
We asked some questions about supplements for the side-effects she has been having from her meds. We had gotten some recommendations about taking fish oil for her skin and protein powder for her hair. The doctor uses protein powder herself, but as it is unregulated, she is concerned about getting the right kind, and there was also a recent study showing a "particular mineral", (she couldn't remember what) that was in the powder, and when Jonathan googled (since he is taking protein powder himself) he saw that some of the powders have been found with elevated lead levels (I think most of them violated the Proposition 65 stricter standards, but a few were violating or close to violating the FDA standard). She also pointed out that with Grace's borderline kidney results, adding in protein powders (that are filtered out by the kidneys) would likely be a bad idea. She did support Grace getting more protein via dietary methods, and I expect we'll have an appointment with a dietician in the near future, as that came up a couple times throughout the conversation.
She said she would talk to the Koselugo vendor and see if there were any issues with probiotics or fish oil.
We talked about my expectation that being on Koselugo would inhibit/reduce any future fibromas, and so was surprised when the new glioma appeared on her optical nerve. She said there is an active trial to use Koselugo for optical gliomas, but it isn't as targeted for them, and so if the optical glioma ever causes eyesight issues or maybe even if it continues to grow (which she was careful to say it might not actually be growing, but just wasn't seen on the first MRI due to where they were looking) she might switch to a different med (which isn't as targeted for PNs, so not good on that side).
We also talked about the increase in her skin side-effects (though once we took off her shirt, I saw that it was better than a week or two ago and after I got home, Heather verified that it has been getting a lot better since she has been more careful on always using the skin lotions) and how she didn't have a "peak side-effecfs at 3 months" as expected, but got worse around 7 months or so, but basically the answer is just that everyone is different, so nothing really to make of these symptoms now.
The doctor also explained some more about the difference between plexiform neurofibromas and "regular" neurofibromas. The PNs are congenital (existing since birth), so it is likely that she doesn't have any other ones. They grow along the nerve sheaths and these are the more serious ones, both in terms of potentially becoming cancerous at some point, and also potentially disfiguring at some point, though she thought that Grace's current PN wouldn't be disfiguring, which isn't intuitive to me, since it seems like it is exactly in the place that could cause facial growth/changes, etc, but basically we will deal with that when we get there, and not worry about it now anyway.
On the other hand, I thought the reason she gets regularly scheduled ECGs and MRIs is to look for new PNs and I thought those were going to continue for a while, or maybe her whole life, but maybe the statistics of them happening goes down over time. I think this doctor might like to take the optimistic stance, and our oncologist likes to be pessimistic and then be happy if the worst things don't happen. There are pros and cons to each method I suppose and probably good if we know which "box" a particular doctor is in, so the statements can be filtered one direction or the other, as the pessimistic statements, while good for preparing for bad things, also leads to overthinking what might go wrong.
The regular fibromas can be on top of the skin or under the skin, and those can appear at any time, typically spikes during childhood growth, and then at other times throughout adulthood, triggered by a variety of sources. And then are sometimes just bumps that don't need to be treated, or sometimes are painful and/or restrict motion, etc.
Story time: I've often had a bad experience at Dartmouth's blood lab (as opposed to getting blood drawn/IV inserted in a specialist's office within the hospital). Last month, I had the MRI team leave her IV in since she was getting blood drawn, but the blood lab isn't allowed to use the inserted IV for blood draws, but need a specialist team to come, who they forgot to call, so we waited an hour with repeated assurances of they will be here any minute. I finally asked if they could remove the IV and just do another IV, but they aren't allowed to do that either, so I asked for a bandaid from the receptionist (who agreed the whole thing seemed very strange), and the technician's response was to call security on me because apparently they thought that was crazy risky to remove an IV on my own (which probably Grace knows how to remove it in a sterile manner and definitely she knows about keeping pressure on it, so I don't understand why the tech can't do it, and thinks it would be dangerous for me to do it), and when security got there, they couldn't figure out why they were called, and the receptionist agreed that there was no reason. But, at that point, I said I would go over to the oncology department where they could handle it. (It wasn't until later that I realized I should have asked the blood lab to put an IV in her other arm, and avoid the first IV altogether until we got out to the car). The IV team go to the oncology department just as I got there, and said they've had problems with the blood lab with other patients, and so weren't too surprised by the whole incident, and agreed that removing an IV was trivial. I submitted a formal complaint to the hospital, but never heard anything back. Oncology agreed to do the blood draws in the future, but I met with the neurologist in the opthamologist's office rather than oncology, like I had expected, and opthamology wasn't able to do a blood draw, so I figured I would try out the blood lab, and I think this was the first time they did the blood draw easily and without incident, though they still always get confused by the standing orders that tell them which vials to use, and ask me if I want to override the doctor's instructions or not; which also seems strange to me, but that happens every time I go to the blood lab.
Anyway... her blood was drawn successfully, and her thyroid tests came back today and she is no longer "off the charts" and while not within the normal range, is looking better, so the increased dosage is working well. Her kidney numbers also look good to me, and the November tests appear to be an anomaly for some reason. Her red blood cell counts and some other "regular" bloodwork is still lower than she had been, so we don't know what is going on there, but the doctors aren't too concerned that it is "too low" yet (and it isn't trending downwards, in my opinion).
Grace has an appointment with a new doctor on Friday, which we'll look at her kidney results, and I imagine that appointment will be less interesting now that yesterday's results show that her kidneys are doing better.
She has an detailed (2 hour), sedated MRI scheduled for her eyes on February 4th. The neurologist asked if there are any issues with another contrast-dye MRI since her kidneys were questionable (and the kidneys filter out the dye), so I'll check with the nephrologist on Friday, but I haven't heard anyone ask that question before.
Insurance is a little weird about prescriptions, and we've never gotten her Koselugo prior to the maximum deductible being hit, so I'm a little concerned that if her prescription comes in as the first expense in January, it might end up costing $7k or so (the monthly cost is $9k, but insurance has always paid 100% of it for the last 8 months). So, we are trying to get the MRI scheduled for before that, so hopefully we'll be able to fit in where there is a cancellation. I've talked to the pharmacy and the insurance company and they think it might work fine, but no one can know the actual answer until the dose is starting to be filled on January 10th. And they can't send a double-dose in December, which would also solve the potential problem.
Oh Daley family, God has certainly trusted you with difficult assignments.....that you have accepted and excelled at....I know He takes joy seeing how well you handle each situation, I am praying for all of you, as I know this touches each of you