2025-04-11T12:24:58Ztag:blog.limedaley.com,2018:/isso/thread/post/plexiformtag:blog.limedaley.com,2018:/isso/826/36172025-04-11T12:24:58Zjoyful<p>Trametinib</p>tag:blog.limedaley.com,2018:/isso/826/36162025-04-11T09:43:20ZLinda Wightman<p>In less than 24 hours I've gone from praying she wouldn't need the nasty drug to praying she'll be <em>able</em> to take it.</p> <p>Great news about the insurance; I didn't see any numbers for this particular drug, but when you inquire about cost and the company assures you that there are resources to help, you know it's out of sight. I was reading, also, that some insurance companies aren't nearly so helpful.</p> <p>I know about selumetinib (Koselugo) and mirdametinib (Gomekli); what's the third drug?</p>tag:blog.limedaley.com,2018:/isso/826/36152025-04-11T01:55:03ZJon Daley<p>Yeah, we asked about &quot;what happens if we do nothing&quot;, and the answer was that it would continue to grow, and eventually would be disfiguring (if you want to see some gross pictures, google for &quot;nf1 disfiguring&quot; and click on images. The typical neurofibromas are the small skin flap things, and then the large things, often on the head and face are untreated plexiforms, so you really don't want to let them grow.</p> <p>Grace's current tumor already extends from behind her ear inside to behind the ear drum, and then down in her cheek, and near her tonsils, etc. When she was having her biopsy last week, the doctor took the opportunity to feel around while she was sedated, since she noticed that her face wasn't quite symmetric, and so was more suspicious of a large plexiform, even before the ear biopsies confirmed it.</p> <p>And of course, we don't know what direction it might want to grow, so could do more damage to her Eustachian tube, or towards her brain, more into her ear canal, etc. so there really isn't much of a choice, other than which of the three meds to choose from, and the doctor prefers Koselugo, and so that should be starting next week.</p> <p>Though, due to her creatine numbers from yesterday, they are too high to add in a medication that increases the creatine, so they have prescribed 1500 ml liters of liquid a day, though that is way too high to be realistic - even when she had the NG tube, we never did more than 1200ml total, but we're giving her as much water as she can drink on her own for now, and we'll see what today's more extensive lab draw says, as well as a urine sample.</p> <p>The preferred med only comes in pill form, so we have be practicing with tic tacs, and the first attempts didn't look like it would work, but Faith worked some more with Grace, and she swallowed one, and then tonight she swallowed one on the first try (the actual pills are slightly larger than tic tacs - 14mm long). She gets to chew a tic tac after she swallows one, and she is pretty happy about that plan, so she'll probably be fine once the med actually starts.</p> <p>Insurance is going to pay all of it, and the pharmacist who called (and talked for 30 minutes, so you know it must be expensive...) said there weren't going to be any payment issues, but verified that it was expensive and that is why Shaws won't carry it - they apparently have to pay for the drug upfront and then get reimbursed later, and it is too expensive for their budget...!</p> <p>But the Dartmouth specialist pharmacy is willing to mail it to us, and will call each month to check on symptoms as any changes might require dosing reductions/stopping, etc.</p>tag:blog.limedaley.com,2018:/isso/826/36142025-04-10T23:56:36Zjoyful<p>She loves taking pictures!</p>tag:blog.limedaley.com,2018:/isso/826/36132025-04-10T22:57:31ZKathy Lewis<p>Praying!</p> <p>So has Grace decided that she likes being on the other side of the camera?</p>tag:blog.limedaley.com,2018:/isso/826/36122025-04-10T18:25:13Zjoyful<p>This just in: Grace's kidney numbers from her lab draw yesterday were concerning to the doctor, so she's getting another draw today and then an ultrasound on Tuesday.</p>tag:blog.limedaley.com,2018:/isso/826/36112025-04-10T17:12:54ZLinda Wightman<p>How hard it must have been to make this decision for someone who is to all appearances as happy and healthy as anyone could want, free at last of medications and living a normal life except for the more frequent medical examinations and tests. When I think of the terrible potential side effects of this very new drug—it was granted FDA approval only in 2020—I remind myself that until five years ago there was <em>nothing</em> they could do for inoperable neurofibromas but let nature take its course, likely with effects worse than the drug.</p>tag:blog.limedaley.com,2018:/isso/826/36102025-04-10T16:44:42ZJen Newhouse<p>We continue to pray.</p>