Lime Daley Blogs

MRI/visit results

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The MRI went smoothly. The anesthesiologist listened to Jon and adjusted some things, and it seemed to be good.

There is not a glioma on her eye. Her plexiform neurofibroma is about the same as it was last time. (They were concerned about some abnormal tissue in her right cheek, but that's where she fell down the stairs the other day. It was a particularly bad bruise.)

We are taking a break from the Koselugo for two weeks to work on getting her rash under control. We have a new steroid cream to use.

Her TSH went up, so they have upped her levothyroxine dose again. I don't know what it means for the T4 number to be good but TSH to be high, but we have another Endo appointment next month.

Day +725 update, MRI this week

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Well, it's been a couple of months since we posted an update. We're thankful for this break from medical appointments.

With the dry weather, Grace's skin has really broken out. It gets dry and itchy, and then she scratches so hard that she makes herself bleed, and gives herself welts. So our nighttime routine involves slathering her with cortisone and Vaseline before she puts her pajamas on. I've also tried cutting her nails more often and filing them more often, but the thing that works best is just having her wear mittens to bed. Her pain tolerance is still so high that she doesn't really realize that she's scratching herself to excess.

Wednesday, she has her long MRI, for which she will be sedated. This will be to get a good detailed baseline for checking out the possible tumor on her eye, and for checking on her ear tumor. If her ear tumor is looking good and small, we may take a break on the NF medicine to give her skin a break.

Yesterday, we went out to dinner to celebrate two years since Grace's transplant! (The official day is next Sunday, but this night worked out better.)

Some Grace quotes:

12/23 - "The big toe is called 'Market.'"

1/9/26 - I exclaimed, "How can it be Friday again!?" And then Grace said, "Mom! It's 'cause the calendar goes 'round and around and around. That's what it does."

And pictures of a happy shoveler during the big storm:

Appointment Yesterday

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Some 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.

Opthamology

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.

Neurology

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.

Side-effects

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.

Plexiform Neurofibromas vs Sub-cutaneous and Cutaneous Neurofibromas

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.

Blood Lab

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.

Success and Results!

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).

Next Steps

Nephrology

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.

MRI / Insurance

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.

Isaac's 23rd birthday

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23 years ago, we started this blog to have a good way to keep everyone informed of how things were going. In some ways, it really does feel like a lifetime ago. However, Isaac is still close in our hearts. His life and death are a part of our family and influence our relationships to each other and to God.

The thing that is blowing my mind today is that Isaac would now be the age that I was when he was born.

Testing preliminary results

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We have only read the test results ourselves and have not heard from any doctors yet.

Her TSH is uncomparable, since the tests they do at Dartmouth don't go any higher than ">100" so is it 400 again, or 101 or 1000? But her T4 was up, almost to the normal range. So hopefully that means her thyroid medicine is starting to work. Jon read something about hypothyroid diseases being passed to people through bone marrow donations. But Faith's TSH was normal just before transplant. Faith hasn't had that tested since November 2023. Jon has in inquiry in to the doctors at Boston Children's about that.

Grace's heart is perfect.

I think her MRI showed that her ear fibroma is about the same. But they reported that some results were not clear because she moved. Jon had been interested to note that she didn't fall asleep this time. Despite getting up very early and not even falling asleep in the car on the way up. (She told me, "It was dark, and then it was morning!") But the MRI report also says to further investigate a possible thing near or on her eye nerve. Please pray for us to not worry and for that to be nothing.

Her kidney numbers were good. Her iron and related numbers were on the low side.

Testing tomorrow and catch up

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Grace is getting an MRI, and ECG, and bloodwork (including thyroid) tomorrow morning at Dartmouth. I'm not sure how much the thyroid medicine is working, so we'll see what the numbers say. Her skin is very dry and she has rashes in places, and also the dryness is itchy so she scratches it, sometimes to bleeding.

Today is day +641! What a big number, and we are thankful. We're coming up on two years since diagnosis and oh, how far she's come!

Grace turned four last month!

Making her own birthday brownies:

She can make fun shapes in her fruit: smiley oranges and bunny apples:

She went ice skating for the first time:

Hope's words

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mom/mommy/"mahmeemahmeemum"

dad

ball "bahw"

dog "dah-dah" (she used that word for a cat yesterday also)

bellybutton "bah-bee"

bottle "baba"

wow

bop (as in noses)

baby

hat/head "at"

hot "ah"

whee!

happy

up, (please) "uppy"

out/in/open/close/on/off "ow"

ow "ow"

thank you "dah-doo"

Signs: shoes, socks, please, more, thank you, change, toilet, hat

Two Boston Visits

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On Sunday, we went to the Franklin Park Zoo. This event was hosted by the Jimmy Fund clinic for its patients. It was sobering how many families were there, and it reminded me how many different types of childhood cancer there are. We had a fun time, though . There was a bubble lady, face painters, free food and swag, and of course animals. There was a special event for stem cell transplant patients. It was surreal seeing some of her nurses again, one of the doctors, and other patients. There was one boy whom we recognized from the transplant floor. There was a girl there who was still on feedings, and I saw my other kids look at each other with knowing when her pump beeped an error. Grace said, "Look, Mom! Like me, a long time ago." Hope was overwhelmed with excitement about the giraffe that was nearby!

On Wednesday, both Grace and Faith had appointments in Boston. Faith to check out possible influences of the JAK3 mutation. Grace for her annual Boston checkup.

They both got a lot of testing on their blood. Dr. Prockop was happy with Grace's appearance.

But on Thursday she emailed because Grace has hypothyroidism. She made me laugh because of the way she phrased that we didn't need to go to the ER emergently. Apparently, with numbers like Grace's, that would be the protocol, but since she had just seen her, she didn't think it necessary.

So Grace will go on thyroid medicine today and is supposed to be seeing a Dartmouth endocrinologist next week. We will get more answers then, but it appears that this is related to her NF1 medicine.

And there are other tests that don't have results until next week.

So please keep us all in your prayers as we navigate this new complication.

And here's a photo of Grace looking cool at her brother's soccer game.

Day +571

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Well, I didn't manage to post this yesterday when the day was a nice round, even number, so I get to post it today when it's a prime number!

This has been a full summer for us.

Noah was home.

Grace got to ride at Summerfest just like all the other healthy children! She had such a blast!

Grace was so excited to be able to go to the Maggie P. She has been looking forward to swimming at the Maggie P ever since last year when she couldn't swim because of her central line. She talked about it every month at least for the whole year, "When I go to the Maggie P, I'm going to go swimming!"

At playgrounds while Nathaniel was at Ninja Camp:

She loves hugging bananas and asks me to get a picture every time we buy some!

Smoothie mustache:

Being a Viking at History Alive:

Grace has been doing very well. One side effect we have now noticed from her koselugo is that her hair is thinning out. I'm very thankful that she does not have the more serious side effects, but I am also sad to see her beautiful wavy brown hair starting to come out again. (No, I do not know the future prospects for that and I keep forgetting to write to the NF1 doctor about it.)

Grace and Faith have appointments in Boston this month, and Grace has another hearing check in October.

Sailing Trip!

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Jeremiah and I will be with Heather's first cousin once removed and sailing from Florida to Connecticut starting tomorrow.

We've thought about going in prior years, but the schedule never quite worked out, but this year we decided we would jump in; we don't really know what to expect, but should be fun.

Great results

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Wednesday's many tests all went well. The MRI showed shrinkage of the tumor, especially around the ear canal (less so in the cheek/salivary gland.) The hearing test confirmed Grace's attestation that her right ear is significantly improved. Her eyes are still perfect. Her heart is also good, no changes.

So we praise and thank God for these results. And continue to pray for the tumor to shrink to nothing and for no side effects from the selumetinib. She occasionally has diarrhea, but one half dose of Imodium every couple of weeks is enough to keep it at bay. The nutritionist also gave us a list of binding foods that had more in it than the classic banana that Grace is kind of tired of. (:

She also got another stuffed unicorn, which she insisted on naming Candy Corn just like the first one!

They stopped by David's House after all the appointments and played at the playground a while.

Healing and Testing

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Jon just put this comment on a recent post, but I don't think most people get notified of comments, so I'm posting it here:

She's getting an echocardiogram right now [July 9, 2025, 8:42am] , and then an MRI, regular oncology checkup, opthamology and audiology.
We don't expect too much interesting, though we'll see what audiology says.
I don't remember if it has been posted here yet [nope] but Grace told us that God has healed her ear and she no longer needs her hearing aid, so she hasn't worn it for over a week [two weeks] and I've only heard her ask for someone to repeat themselves a few times so I'm hopeful that her tumor is shrinking (which is faster than expected). I'm curious to see what the hearing test says and whether they'll want her to wear it even if it is getting better - there is some brain training that is good to make sure it is used to using both ears.

Day +500

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Today is Day +500 and we have much to rejoice over!

We are so thankful for Grace's good marrow results, so thankful to reach this milestone.

I feel so much lighter now, and the smaller things that bothered me last week don't seem so bad anymore.

Here are some recent pictures and quotes from our Amazing Grapey Grace Victoria:

Someone was talking about tax, and Grace thought they said tictacs, so I said it was spelled T-A-X, and she replied, "E-A-O-B: tictacs!"

Me: "Why do you have to ask a question about everything I do?"

Grace: "'Cause my voice has lotsa questions!"

When we all had sore throats: "Why Daddy not reading, mom? Daddy's voice is itchy, too?"

Grace's first time painting pottery.

Little pigtails; we think she looks like Boo from Monsters, Inc.

Hiking

She called the Rubik's cube "cuber-solve."

Jeremiah wasn't careful when he sneezed, and Grace said indignantly, "Don't bless you on me!"

She cut her own bangs; "Now they're not in my eyes anymore!"

June 2025 Biopsy Results!

jondaley

Short version of the post - all good results! You can read the longer version below.

We are very happy and it has been a weight off of us. Until a couple days ago, it was seeming like it was quite likely going to be a bad answer, as we've been praying and thinking over this last week, I had eliminated a couple possible theories. The clerical copy and paste error wasn't correct, because there were some small changes from the previous test. The false positive result is quite unlikely.

Two new theories emerged this week, that Faith's marrow is successfully working in Grace, but had become "infected" (probably not the right word for gene mutations) by Grace's JAK3 mutation, and the second is that Faith actually has the JAK3 mutation herself. It turns out that the latter is most likely the right answer. We've been talking and emailing the doctors over the last couple days and it turns out that the JAK3 was originally reported as pathogenic since it was in the presence of leukemia, but they are now thinking that it is a "regular" mutation and not going to be a problem. Faith's bone marrow wasn't tested, but JAK3 (and others) can be detected in the blood, but, basically, since she is old enough that if it was going to cause a problem, then weren't concerned about it, and so the pathologist who saw Faith's results considered it a normal variant and it was "filtered out" of the prior reports. They are going to go back and modify some of the reports to make it more clear for future readers.

It is also likely that since both Faith and Grace have it, that either Heather or I have it as well. There is an immunologist at Boston who agrees with our oncologist that it is "overkill" but is willing to do some blood tests to do further checking. There are very few documented cases of this particular variant in gnomAD (good luck trying to figure out that site; even when I type in the specific protein alteration/variants, I still can't figure out how to find any useful data), but there is a similar variant that causes some immune system problems, so in the interest of reducing anxiety, the doctors thought it could be worth a meeting and a blood draw, but they don't expect anything interesting to come out of that. We aren't currently due for a visit to Boston, but the doctor suggested we schedule one for Grace and Faith and meet with immunology on that day, so we'll probably do that once summer vacations and trips are over.

We can't quite tell if the chimerism test result came back, as Heather and I interpreted an email differently, but other related tests don't show any presence of JMML, so everyone thinks everything is still going well. And I forget if I wrote this before - Grace's blood tests are the closest to within normal limits than any test I can remember. She just has a few tests (out of ~30) reporting just barely outside what would be normal for the general population, and her "normal" isn't normal, at least not yet, so nothing interesting to report there either.

Her loaner hearing aid is doing well, and we are going to purchase one at her next appointment. (probably the main reason to do that is if we lose or break the rental one, we have to buy a new one, and the owned one has insurance). She went swimming yesterday and it wasn't until after I dunked her under that I thought to remove the hearing aid, but fortunately, Heather and Joy are more on top of it than I, so it was already taken out...

She continues to impress everyone taking her daily medicines, she now takes them without water, but just swallows the pills, though does still insist/enjoy the post-med tic tac.

She'll be going to Dartmouth on July 9th for a pretty extensive workup: audiology, opthamology, MRI, CAT scan, and the regular blood draws.

Bone Marrow Biopsy

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The test is done and we are back home. Nothing too eventful to report. Our regular physican's assistant did the biopsy, so got to talk to him a little bit, and he is going to research one question that we aren't sure about regarding what 97% chimerism test means.

He did say false positives on a JAK3 test are possible, though very low probability. He agreed that a clerical error would be ideal, but he didn't think that was too likely either.

I said that I've been assuming a positive JAK3 test is a pre-cursor to the chimerism test dropping to a "mixed chimerism", and he is always very careful with his words, and said that it isn't a harbinger or a pre-cursor, but it is a pre-disposition.

We will find out all of the results in a week or so.

Prayer and fasting Wednesday

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The day of prayer and fasting for Grace has been scheduled for this Wednesday, June 11, 2025.

Please observe this in the way that seems best to you. Jon and Grace will be at the Hillsboro Methodist Church (16 Henniker Street, Hillsboro, NH 03244) from 11AM - 6PM.

Pray for complete healing, continued remission, 100% tumor shrinkage, peace, and whatever God puts on your heart. And Thursday is Grace's repeat bone marrow aspirate.

The scriptures that we are clinging to and praying through:

"And he said unto me, My grace is sufficient for thee: for my strength is made perfect in weakness. Most gladly therefore will I rather glory in my infirmities, that the power of Christ may rest upon me." - 2 Corinthians 12:9

"It is of the LORD's mercies that we are not consumed, because his compassions fail not. They are new every morning: great is thy faithfulness." - Lamentations 3:22-23

Cast Your Cares On Him

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Jon was reading through Grace's test results and saw that the most recent bone marrow report mentioned the JAK3 mutation. He contacted her doctors because we thought that one was gone following the transplant.

Dartmouth doctors can't see her Boston results to compare so they contacted the head transplant doctor there. She said that seemed odd given the good chimerism results, but she wants another aspirate as soon as possible to make sure. So that is scheduled for June 12. We aren't clear as to why no one noticed that before. We think the Dartmouth doctors thought it was expected, and I guess the Boston doctors didn't see the results? It does make us wonder if we need to be paying more attention - we try to read all of the medical records, but don't always read through everything, and sometimes it gets pretty technical.

Please pray that Grace has no trace of her old marrow and for peace. We know a boy who had a relapse and had to go through the whole transplant process a second time and we really don't want to follow in his footsteps.

I'm letting the Cares Chorus be an earworm in my head, and when I looked up the reference, I found the rest of the chapter to be encouraging, too.

Likewise, ye younger, submit yourselves unto the elder. Yea, all of you be subject one to another, and be clothed with humility: for God resisteth the proud, and giveth grace to the humble. Humble yourselves therefore under the mighty hand of God, that he may exalt you in due time: casting all your care upon him; for he careth for you. Be sober, be vigilant; because your adversary the devil, as ar oaring lion, walketh about, seeking whom he may devour: whom resist stedfast in the faith, knowing that the same afflictions are accomplished in your brethren that are in the world. But the God of all grace, who hath called us unto his eternal glory by Christ Jesus, after that ye have suffered a while, make you perfect, stablish, strengthen, settle you. To him be glory and dominion for ever and ever. Amen. - 1 Peter 5:5-11

Please join with us in resisting the devil. It takes energy that I don't always feel I have. We haven't had a chance to talk to our pastor about the previously mentioned day of fasting and prayer because we've been away from our church for several weeks. Most of those were because we went to Smith Church but one was camping. And he was on vacation at some point, too.

Grace is doing really well with her hearing aid. She even wanted to show it to a lady at Shaw's who talked to her as we were shopping. It makes a helpful difference.

Hearing aid

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On Thursday, Grace got her hearing aid! They did a whole hearing test so they could program the device to her exact needs. The inner ear test shows that she hears fine on that end. So the aid amplifies the sound so it can reach the inner ear.

She noticed new sounds right away, and she played around with her voice in the car ride home. She still asks us to repeat ourselves, but a lot less than when she doesn't have the aid in.

Day +456

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Here's a quick update. Grace has some virus that she's fighting off and she gave it to me, too.

In medical news, she had a checkup and echocardiogram on Tuesday and all that looks good. So far we haven't observed anything noticeable, either good or bad. She got fitted for a hearing aid, and after much debate picked blue, pink, and red. That will be ready at the end of the month. I'm hoping that will significantly reduce the number of occurrences of, "Wha' you say?" I'm also hoping it will be temporary; that she won't need it once the fibroma is shrunk to nothing.

Segue:

Our pastor is planning on organizing a church-wide day of fasting and prayer for Grace's healing. I figure many of you blog readers would like the chance to participate, so I will post with more details when we know them.

Holy Week

jondaley

Our week has been somewhat full, with a few extra church services, including Maundy Thursday with handbells, which I didn't find out until the end of the service that it wasn't recorded, and I meant to record it during rehearsal, but I forgot. But, it went well, despite Jeremiah being sick, but fortunately, Faith's part was pretty easy, so she was able to take over his bells as well as her own (so, I think that means she had more than an octave of the highest bells).

We also had a combined service with the Methodist Church and Smith Church today. They had a "tables of the cross" type setup to explore after the service, and Nathaniel in particular stayed for quite a while.

Grace started her new medicine, Koselugo on Tuesday(?) and she has been doing really well. Sometimes the first pill in the morning takes a little bit more work, and the second pill goes down quite well. And then the one at night has been going well too. She has been putting the pill in a little yogurt along with some water. No side-effects so far!

Unrelated to the meds, she has fallen a couple times this week, and we figured out at least one of the falls was due to really tight jeans, which limited how much she could lift her legs, and that resulted in a spectacular-sounding fall, which was her going face-first down 5 stairs or so to the first landing. And she has some pretty significant bruising from that. She now insists she'll never wear jeans again, but maybe she'll be able to figure out that bigger jeans will fit better.

This evening, Nathaniel and Jeremiah and I were able to get back to The Curse of Monkey Island, which we hadn't played in months, and made some significant progress, and as I write, I'm listening to Faith and Joy singing hymn/duets as they make some gingerbread!

Joy was a reader at the Good Friday service.

We (Heather, Jon, Hope and Grace) went on a time-share presentation vacation last weekend, and (short version - all time-shares, even those that pretend to not be time-shares, are all bad) we went for a walk on the train tracks behind the hotel. Aside from the presentation, the rest of the weekend was nice - basically nothing on the schedule, went out to dinner every night and swam in the pool a few times. Grace is quite bold and willing to jump in from the side and always held her breath without any problems!

Plexiform

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We had our appointment with the NF1/oncology doctor yesterday. It turns out the neurofibroma is even bigger than we thought. In addition to what we already knew, which is that it goes from her external ear where we can see into her middle ear, it also extends down under her ear into her parotid gland which is a salivary gland in the cheek. This is a plexiform neurofibroma, which is something that has actually been there since birth. And it has grown over time. (There are other neurofibromas that can come later possibly, but they are not the plexiforms.) This plexiform right now is benign, but it could potentially become malignant at some point. An initial screening has indicated that this one is one step towards becoming malignant, but they are still unsure of how many steps the body takes to get there.

Grace will be going on a medicine which is designed to shrink the tumor, and it may prevent some other kind of tumors. This medicine can have some unpleasant and even dangerous side effects, including diarrhea and rashes, vision impairment and heart issues. So Grace will be going in monthly and then every 3 months to check on all those functions. Right now, the plan is for her to take this medicine for 2 years, and then reevaluate, based on new research and her body's response to the drug.

One fascinating thing that I learned yesterday is that the longer hair on her right side that we have noticed since she was very young is related to this neurofibroma. The doctor said that because of extra vascular activity on that side it is not surprising that it would cause faster hair growth. When I heard that, I went back to my list of Grace's symptoms that we made before her NF1 JMML diagnosis while we were trying to figure out what was going on with her. I noted that she also would get redness on her right cheek when she ate sometimes. We thought at the time it was an allergic reaction, but it is likely due to the extra vascular activity. (It still happens sometimes.)

Please pray:

That Grace will be able to swallow the capsule (twice a day!)

That she will not have any serious side effects. We would also appreciate if she didn't have any other side effects. It's been really nice the last few months to not have to deal with diarrhea.

That the medicine will do its job, which is to shrink the tumor. I forgot to ask if her hearing loss is permanent or if it could come back with tumor shrinkage.

Some of you may be wondering ( I hear you Grandma!) why could they not see this in the imaging. The reason stated is that the plexiform neurofibromas are a soft and spongy tissue, and is easy to not notice them, and/or think that they are something else. The ENT even said that it's made of the same kind of tissue that your outer ear is made of anyway, so then it's really hard to distinguish.

I guess that's it for now. Here's a collage of pictures that Grace took of Hope in the grocery store:

One year home; biopsy results

joyful

One year ago today, our older kids got to see a total solar eclipse, and Grace came home! These annual milestones are flying by this year, and we are thankful.

Grace's NF1/oncology doctor called Jon with biopsy results on Friday. It is the opposite of what we were hoping. Both biopsies indicate plexiform neurofibroma and it's really one big tumor that connects from the outside where we can see to the inside behind the eardrum. Surgery in this delicate area is not advised. We will have a meeting with the doctors Wednesday to discuss treatment. Some good news is that Jon has talked with other NF1 parents whose kids have done well on the medicine.

That news was a blow, but I am doing better after attending a prayer meeting on Friday and church on Sunday. I am holding onto the hope that God is still working and will heal her one way or another and in his timing.

Both Jon and I are going to the meeting tomorrow, so we can be clear on the options, side effects, pros, cons, etc.

Here is Grace with some presents from ladies at church:

And helping Daddy with his project:

Day +413, ear surgery

joyful

As far as we know, the surgery went well. He took two biopsies, one in front of the eardrum and one behind. There was not much fluid, so he did not put in a tube. We will get results in several days. The bone marrow aspirate also apparently went well, and that also takes about a week to get all the results back.

The cotton ball they put in her ear got soaked through, though it was not supposed to come out until tomorrow. But Jon put in a new one and there seems to be no more bleeding.

Surgery Tomorrow (Thursday, March 27)

joyful

As Jon mentioned, Grace is having ear surgery tomorrow to explore what's going on, get a biopsy, and maybe get a tube in. The oncology/NF1 doctor also managed to schedule her bone marrow aspirate for the same day so Grace will not have to be sedated twice in a few weeks.

Prayers for all that accompanies sedation (including fasting, Jon's conversation with the anesthesiologist, and emergence) are appreciated. And that they will find exactly what they need to, that everything will be clear. And that her marrow will still be all Faith's!

When she bites into a clementine, it gets a face!

She is in a phase where she loves making dough. She has requested it nearly every day the last week.

Plexiform Results

jondaley

An ENT, a radiologist and an oncologist walk into a bar...

Oh, maybe not, but they at least had a discussion about Grace while looking over the MRI and CT scan together and the conclusion is that it isn't a plexiform fibroma at all! Which is what Heather has had the faith to pray for all along! She has had a number of positive signs from God this week and so she is greatly encouraged. The lunar eclipse was pretty neat to watch, and she was also blessed with a meteor just after as she was going to bed.

I haven't talked to the oncologist/NF1 specialist who was the one to diagnose it in the first place to hear why she changed her mind, but collectively, they don't think what is going is consistent with an NF1 fibroma, so they are looking for another cause.

Grace now has surgery scheduled for next Thursday, the 27th, to check out the potential fluid behind her ear (last time I talked to the ENT, he was thinking it might not be fluid, but some spongy tissue), possibly install a tube in her ear drum to let the fluid out, if it is there but getting blocked from going down her eustachian tube, and do a biopsy to grab near the potential NF1 fibroma to see what it is.

She will be sedated for the procedure. They haven't scheduled the time yet, but it will likely be at 6:30AM or so.