Daley Ponderings

Lest you think nothing has been going on, here was Grace's medical schedule since we last posted.

• 8 visits to primary care for vaccinations - she had to restart over as a baby, and get all of them over again - just one left, plus flu and COVID, which oncology told us yesterday they highly recommend for Grace since one of the chemos she received can cause long-term lung problems and she is more likely to have a harder time with respiratory illnesess.
• 3 visits to dermatology - this has gone really well, and it would have been good to meet with a dermatologist earlier, as the doctor changed out the skin meds she was on, and (perhaps in combination with figuring out thyroid dosages and lowering the Koselugo), her skin is 100% better, though as we enter into the drier winter season she'll need to use her lotions more often than she needed to over the summer.
• 1 visit to our dentist - nothing to report. Though oncology told us yesterday that one of the chemos she had causes long term teeth problems, and before pulling any teeth or major dental work, they need to check to make sure that the adult tooth exists and is in the right place, and is shaped properly, etc.
• 3 visits to endocrinology - mostly just to lay eyes on her; the doctor has been working on getting the dosage to get her thyroid levels within normal limits, and that has been a success.  Oncology just said to keep an eye on it as she gains weight, as the dosages will need to change.
• 1 visit to opthamology - everything looking pretty good, though they have some concerns about "thickening" around one of her retinas, but is is good for now.
• 1 visit to neurology - we like to do combination visits with neurology and oncology to make sure we are covering the bases with NF1 and JMML.
• 2 visits to oncology - this is Dr. Santacruz at Dartmouth, who we love and who we basically consider her primary care provider, though after the conversation yesterday with Boston, we need to have some conversations with all of her providers, as it is depending a little too much on each individual to be thinking about the whole picture, and I'm not sure specialists are that good at thinking that way, and while I'm comfortable managing much of her care, it is easy for us to not connect the dots across everything.
• 1 visit to audiology - hearing is still good (even with the lower dose of the anti-tumor med, so that is good news!).  There is always a question about one of the tests (air pressure/response), and from my view point, the test is greatly skewed by how the sensor is physically held into her ear, so I'm not entirely sure we are getting consistent tests to compare to each time, but the regular actual hearing tests are good, and the frequency responses are mostly normal.
• 1 visit to cardiology - this was just yesterday, so I haven't talked to anyone yet, but Heather said the report looked good.  And the valves look good to my inexperienced eyes.  I am going to ask someone to double check they aren't anything, as I don't remember those being there before.  Gemini tells me bright spots can appear due to reflections of the ultrasound waves, so perhaps it isn't anything.
• 1 visit to pulmonology - also yesterday, and because she is considered too young for a spiromometer test, they just do a walking test while monitoring SpO2 levels, and she did fine on that.  Prior to the appointment, I was wondering if they were going to have her do the spiromometer, so we practiced some in the car, and the oncologist was interested to try, partly just to get her to understand the test (everyone fails the first time they take it - I failed and the tech had to show me how to do it), but said typically they don't use that test until they are 6 or 7 years old.
• 2 visits to radiology: 1 "regular" MRI on her head/ears/eyes.  Another one scheduled for November.  She also got a bone density scan yesterday for the first time - the whole skeletal picture is pretty cool, so hopefully we'll get to see it in more detail than just me watching over the tech's shoulder.  People with NF1 are susceptible to weak bones, etc. When Grace fractured her leg last year, they were concerned about whether it was due to NF1, but due to how it broke, decided it was due to being on steroids too long, so were less concerned about anything going forward, but she'll get a scan every year or two.
• Countless blood draws, which fortunately she doesn't mind at all.

Whew! 

Most doctors are starting to talk about longer time spans between the visits, so that will be nice to have those slow down a little.  We did meet the "long term survivorship" medical team yesterday; when Grace heard about this, she said, "Oh good, more doctors!"

Nathaniel and Grace made brownies which we delivered to Boston Children's Hospital 6th West, where she stayed during the bone marrow treatment, and they were all happy to see her, both the nurses that we knew, who were especially happy, but even some other nurses thanked us for coming, that everyone likes to see success stories.

Boston oncology does want her to get a bone marrow aspirate, not entirely for chimerism, which they did via a blood test yesterday, but there are also some other things that are important to check that I didn't really understand.

And as I referenced above, there were a number of times yesterday that the oncology team said things like, "well, her <insert other specialist> will be keeping an eye on that, so we will defer to them..."  Because of Grace's NF1, she gets seen more than the average JMML patient, and so the onocology team isn't as used to having so many other reports and so many other specialists watching over her.  So, I just need to check to make sure that the other specialists are, in fact, actually thinking about the total picture and keeping an eye for something that might be pointing to a larger issue.  Case in point, I hadn't realized that GVHD was still a concern at this point, and I think acute GVHD isn't likely, but chronic GVHD is more of a possibility, thought we've not seen anything so far that is particularly concerning.  Her Boston oncology team was surprised to hear about her lack of hair growth and hair color changes, but now that she is through it, it appears it wasn't GVHD related, but I'm not sure anyone was actually thinking about that previously.

We also got some conflicting information yesterday compared to what we were told previously about the purpose of removing and freezing one of her ovaries, so we need to check into that.  The nurse practitioner yesterday said that her ovary would definitely not be used for hormone therapy during puberty, but only for potential fertility later.  I now know more about NF1 than I did, mostly due to being on Facebook groups with other patients and parents and watching them go through NF1 as an adult, or learning that they have NF1 after passing it onto their children, though I've attended some medical provider trainings on NF1 as well.  My counsel to Grace would currently be to not have children, where previously I was thinking that would be okay, and we wanted her to be able to make that decision.  The annual costs of freezing are going up, and we're now wondering if it is worth paying all that money each year if she won't eventually even use it.  Apparently, hormone therapy/replacement for teenagers is reasonably well understood, so they have synthetic meds or whatever that can control and compensate for imbalances, so they are confident they wouldn't need/want her ovary for that.  We need to ask some other providers about that, since if you recall, this journey started with bad information from a nurse practitioner who insisted she knew what was going on...