Lift Up Your Hearts!

I know many have been waiting on tenterhooks for the results of Grace's recent testing, as have we. Sometimes good things come to he who waits.

The bottom line: The news is a bit confusing, but it's wonderful! Grace remains fully engrafted and has no evidence of JMML!

[Insert Doxology here.]

Here's the story, as Jon wrote it (except for a section in brackets that I added for what I hope is clarity, as there seems to have been a typo or cut-and-paste error). 

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, [it would have shown itself already, they] 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.

Our deepest gratitude to all of you who participated in the day of fasting and prayer, are praying regularly for Grace, and/or are supporting her family in so many other ways.

Grace's church has organized a day of fasting and prayer for Grace.  For anyone interested in being a part of it, here is the information from the Daleys' website:

It's wonderful to spend time with Grace in person and see what a bright, capable, and happy three-year-old she is! And apparently healthy, though the knowledge that appearances can be deceiving is always in the background. Her new medication appears to be proceeding with only minor side effects, though it's still too early to see any noticeable good effects. She takes her pills like a champ: not only has she learned to swallow them, but she does so without any help, not even a drink. (The single Tic Tac given afterwards is still an important part of the routine, however!) She is adjusting well to her cute multi-colored hearing aid.

However, the background concerns are poking into the foreground, after Jon (Jon, not the doctors!) noticed something strange in her last bone marrow aspirate results. The communication between Boston and Dartmouth is STILL horrendous. (Boston is where the bone marrow transplant was done, and where all those records are, despite them officially having handed her care off to Dartmouth.) As a friend who has had plenty of her own experience with childhood medical issues, wrote, "You want to trust that [the doctors are] doing everything, but the reality is that no one will pay attention to the details and care for her health as much as you."

The upshot is that the results showed evidence of something called the JAK3 mutation, which apparently is related to but not the same as the mutation that causes her NF1. Maybe; I'm not clear about that. But it's in the bone marrow and was supposed to have disappeared in the transplant along with everything else about her original marrow. So the urgent question is: Is this an error (they happen) or a sign that her old bone marrow is still lurking in her system? This coming Thursday the doctors will take another aspirate (with all that comes with it, including general anesthesia) to check the results. As Heather said, Please pray that Grace has no trace of her old marrow and for peace.

Below are more details taken from the Daleys' post.

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 [insert eye-roll emoji here] 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.

Thank you, as always, for your love, your concern, and your prayers.

Grace's next appointment is May 6. In the meantime, she has started on the new medication, Koselugo, and has been doing really well. This is the first time she has had to swallow pills, so she spent quite a bit of time practicing with Tic Tacs while waiting for the medicine to arrive. (Swallow one, chew one.) The candies are slightly smaller than the real deal, but they at least taught the idea and technique of swallowing a pill without chewing it. They tried to get the pharmacist to make up practice pills of exactly the right size but without the drug, but he thought that was a crazy idea and refused their request. Considering that the suggestion was made by a friend of ours who is a speech therapist, and speech therapists know all about teaching people to swallow, I'm inclined to think that it was the pharmacist who was the crazy one here. No matter now—Grace has been swallowing the real pills for over a week, and only occasionally needs to try more than once to get it down.

She was able to begin the treatment because although on retest one kidney number was still a little concerning, the other was fine, and her kidney ultrasound was normal. So they're still pushing fluids, but there was no reason not to go ahead with the new drug.

So far Grace has experienced no side effects of the medication. it's very early yet, of course, but we'll take all the good news we can get for as long as we can get it!

We walk by faith, not by sight. — II Corinthians 5:7

The "faith" part is still strong, but the "sight" part is hard to take.

For several months, to all appearances Grace has been as happy and healthy a three-year-old as anyone could want, free at last of medications and living a normal life except for the more frequent medical examinations and tests. Her language has been exploding; she is bright and funny and happy. She delights in her family, and frequently initiates video calls with Grandma, Dad-o, and Noah.

But NF1 (neurofibromatosis type 1)—a genetic disorder that Grace was born with, though it was not discovered until after her second birthday—has long been the elephant in the room. For a year NF1 considerations had to take a back seat because of the immediate need to deal with the dinosaur in the room, her JMML (juvenile myelomonocytic leukemia), which was caused by the NF1 mutation. Thankfully, for all that time the leukemia was the only manifestation of the disorder.

Actually, another problem had been there all along, but was hidden. As you may remember, her audiologist noticed a mild hearing loss in her right ear at her first exam, and again subsequently—but in every case there was something else to blame it on (a cold, COVID). Now, however, the doctors have turned their attention to it, and to a small lump Heather had noticed in that ear. After months of bouncing around—from "no worries, it's just fluid in her ear," to "maybe it's a neurofibroma, we'd better do some testing," to "it might be a fibroma, but that's not causing her hearing loss, so we'd better do some more testing," to "great news, it's not an NF1 fibroma at all, but we're going to take biopsies to be sure," to "oops, the biopsies say it's definitely a plexiform neurofibroma, and the options for treatment are not pleasant." And then on Wednesday, "the neurofibroma is much larger than we thought, and Grace needs to start on a novel medication that has all sorts of potential nasty side effects, for which she'll need to learn to swallow a pill whole twice a day—and take it for at least two years, maybe for life." Knowing Grace, she'll take it all in her stride, but it's rather hard for this Grandma's heart to take.

In Heather's words:

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.)

This will be TMI for many of you, but there are several medical people who will read this and be curious about the drug. It is called Koselugo (selumetinib) and you can read all about it here. 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 nothing they could do for inoperable neurofibromas but let nature take its course, likely with effects worse than the drug.

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.

On top of all that, Heather just added:

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.

Prayer requests:

• 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.
• That the results of her kidney tests will be good news.

Thanks for hanging in there with us!

Tomorrow (April 9) is a big meeting with Grace's doctors to discuss how to move forward with her ear situation. The results of her biopsies were not great news. It gives one the ping-pong ball feeling: No worries, it's just fluid in her ear—Maybe it's a neurofibroma, we'd better do some testing—It might be a fibroma, but it's not causing her hearing loss, so we'd better do some more testing—Great news, it's not an NF1 fibroma at all, but we're going to take biopsies to be sure—Oops, the biopsies say it's definitely a plexiform neurofibroma, and the options for treatment are not pleasant.

As usual, Heather says it best. You can see some other news and pictures as well on their site.

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.

Enthusiasm, fortitude, patience, and joy are good qualities in a president.

I wouldn't wish that job on one of my grandchildren any more than I'd wish them leukemia. Nonetheless, I would vote for her.

President Grace Daley speaks at the Smithsonian.

From Daley Ponderings:

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!

I'm more than pleased that they thought to spare her the extra sedation.  I'm less happy with the idea of ear surgery, but sometimes you have to trust that the doctors know what they're doing....  And pray.

Jon began his latest update on Grace this way:

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! 

So, great news!

He continued, however with something scary: Because they are still looking for the cause of her mild hearing loss, Grace is scheduled for surgery, and more anesthesia. :( Thursday, March 27, time TBD but probably early morning.

[The plan is] 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.

There's so much to be thankful for, and still so much to pray for. Thank you all for your loving support.

Grace has a bone marrow test at Dartmouth today, where they check to make sure it's all good cells with no lingering defective ones. This is big, even if routine.

I haven't published results from last Friday's appointments, because the doctors are still pondering them.  Here's all I know, from Heather:

[They don't] think the hearing loss is related to the fibroma. There may be bone erosion, not sure what cause. So a lot of discussion needs to happen.The fibroma really is pretty small. I do not know if they can show a shrinking between tests, but I wouldn't be surprised.

Here's an update on Grace's situation after the meeting with all the doctors, to clarify everything about the neurofibroma in her ear, and the next steps to take.

Ha! Just kidding.

The great news is that Grace is still a bright, happy, joyful, competent, and apparently healthy three-year-old.

But the appointment was, shall we say, less than stellar. Here's Heather's report, taken from their website:

This is my memory's summary of Jon's report to me: A lot of time for not a lot of progress. Education/information, but no decisions. The ENT was not experienced, so they want her to see the adult ENT because that person knows more. They also want to do a CT scan before deciding on treatment plans. They think from the MRI that it is a very small fibroma, but they want to check. Turns out the medicine for reducing the fibromas has terrible side effects and you have to take it for the rest of your life. So we want to delay that as much as we can, hopefully avoid it forever. They want to know if it is affecting how her bones are growing. If not, they will probably do nothing. If so, Jon is wondering if surgery is better, because it's so near the surface and so small. But he didn't understand why she didn't get the CT scan today [as scheduled], because it's just delaying treatment decisions. I say it's to give our prayers more time to work to just reduce it without medical intervention. Her hearing loss was less than in November. The audiologist wants to give her a hearing aid, which might be fine. Her eardrum is funny-shaped. 

When I asked about the hearing aid, Heather explained, "The thing about the hearing aid was for balance I think, and the audio "depth perception," and for keeping those brain pathways from dying off. But it did not appear to be very bad. It would be like getting weak prescription glasses.

So, prayers for wisdom, please, for all concerned, and especially that the neurofibroma will continue to shrink, and disappear altogether. Thank you all for hanging in there with Grace and her family.

Whatever you think about Facebook, there's no doubt it can be unintentionally amusing

I mostly find its "Reels" feature to be annoying, and have more than once looked without success for a way to turn it off completely. The short videos it shows are mostly reposted from Tik Tok, which I don't otherwise see. Sometimes they are interesting, sometimes they are genuinely informative and helpful, but all too often I find them infused with a negative view of life, even when they are undeniably—even addictively—entertaining.

Sometimes, however, something unexpected shows up and catches my eye.

If you don't have access to Facebook, you may not be able to watch the video, unfortunately. I spent too much time trying to find a version I could embed here, without success. I hope that link will take you to something you can see, but if not, it doesn't matter.

My readers know that one of our granddaughters plays on her high school girls' soccer team, and that the team has been wonderfully encouraging and supportive of her family during her sister's leukemia journey.

Here's another way they showed their character.

What caught my eye (more accurately, ear) in this video, and made me listen all the way through, was that it's not often when I hear mention of their tiny New Hampshire high school in nationwide media. I think this is the only time I have, actually. So it made me jump.

The short version of the story is that some of the team members did not want to play against a certain other team on their schedule, which included a boy in their lineup. First, in principle, because theirs is a girls' league, not a mixed one, and also because they found the boy physically threatening. The team's coach handled the situation extremely well: those girls who objected to playing that game were excused without any penalty, and the team played the game without any fuss. Somehow it made the news anyway, but I'm proud of the way they handled the situation calmly and fairly.

Our granddaughter? She played the game, with the support of her parents, even though they all thought it unfair for a boy to be on the opposing team. Why? I can't speak for them, but here are a few reasons that came up in our discussion:

• After all she's been through, Faith wanted to support her team, and to play soccer.
• It wasn't the other team's fault that they had a boy on the team—it was a state ruling that forced them to do so.
• Boys and girls often play successfully on the same soccer team—although that's usually at the younger levels, before males gain a significant physical advantage over females.
• They've played against other teams with girls she found more physically threatening than this boy.

The game was played successfully and without incident. I honestly don't remember which team won. In a way, they both did. Don't misunderstand me: The teams should never have been placed in this position, and the state rule that made it happen needs to be fixed.

But bad things happen in this life, and when they are met with quiet grace, that deserves to be celebrated.

These are the preliminary results of Grace's MRI. The NF1 team isn't available on Wednesdays, but the oncology team was merciful and gave them what they knew. You can get more details on the Daley Ponderings post.

As previously mentioned, Heather noticed a spot in Grace's ear last summer, and when the NF1 doctors heard about it, they were concerned that it might be a plexiform neurofibroma, and so scheduled an MRI for today to take a closer look. While it has shrunk on the surface, it is still there inside her ear canal, and they officially diagnosed it today. It is a benign tumor, like most NF1 tumors that she is at risk for. Typically, they will grow and cause problems as they bump into other things, though sometimes they stop growing and might not need anything done, and really rarely, especially in children, can shrink and/or disappear.

The great news is that it doesn't appear to be impacting her brain at all. In all likelihood, however, it is the cause of the mild hearing loss in Grace's right ear that has been observed consistently since before her transplant, but was always attributed to something else, such as her having a cold or recovering from covid. And once again, it was Heather who noticed the spot in Grace's ear, though it took months for the doctors to become concerned. How on earth do doctors decide when something needs to be looked into, and when "wait and see" is the better option? Sometimes intervention works best when it's done soonest—and sometimes it really is best not to intervene at all. I think teachers must have the same dilemma.

I'm very glad that one of Grace's doctors specializes in both oncology and NF1; I suspect she will be getting notified directly of everything medical that happens to Grace from now on, because communication amongst doctors doesn't appear to be all that it should be. The NF1 specialists, by the way, are at Dartmouth, not Boston, which is a much more manageable drive for the Daleys. It's shorter, for one thing—and there's no Boston traffic to deal with.

Hopefully today we'll see what the NF1 team has to say. They already have an audiology/otolaryngology appointment scheduled for March 4, and hope to get both oncology and NF1 doctors involved as well for a group discussion of what the next step should be.

Now for two fun stories from yesterday's adventures.

The oncologist confirmed that Grace is "one in a million," saying that she had never heard of a three-year-old undergoing an MRI without sedation (which was my [Jon's] request), and she did a terrific job. She winced once at a loud noise, and then fell asleep to the soothing sounds of the MRI. (And that is sarcasm, if you couldn't tell.) She twitched a little as she fell asleep, but her head had some padding around it to hold it steady, and I was told the pictures came out great.

Grace had been nervous about "going into the machine," but I know no one better than Jon at calming a child who is angry, fearful, or upset. And Grace has had more than a year of practice at lying still for medical procedures. I suspect that a good part of the reason the doctor had never seen nor even heard of a child that age not requiring sedation is that most parents don't know it's an option. Just as I finally discovered that dental procedures and colonoscopies go much better without anesthesia, but few dentists or doctors give patients this option. Kudos to Grace, and kudos to her parents for their strength in following through with what they knew was best.

They gave her a stuffed unicorn to keep her company in the MRI, and she named it Candy Corn. And she insists that it isn't a boy or girl, but just "Candy Corn," which has caused much debate among the kids about whether she understands the question, or whether unicorns have a different set of genders...

Now you know why unicorns are extinct.

I know I've already made one post today, but Heather just posted this on Daley Ponderings, celebrating Grace's one-year post-transplant anniversary! I don't want to wait to share the news, so I'll replicate most of it here (and trust I'll be copyright-forgiven).

Today is Day +365. One year ago, this is where we were:

Today, Faith is recovered enough to participate in On Belay's Teen Winter Outdoor Adventure (pictures may come later, since she is there as I type.)

Grace had her first post-chemo haircut (just bangs) the other day.

Even more expert at using chopsticks now:

Little girl hugs:

We plan to celebrate as a family by going out to a restaurant tonight and watching Spy Next Door.

We are so thankful for God's grace and all your prayers and support to get us this far.

The spot on Grace's ear has lightened and shrunk! Keep up those prayers!  It looks like God is answering my prayers to heal it up before the MRI. Praise Him from whom all blessings flow.

It has been too long since I've posted an update on Grace for her friends and pray-ers; the fact that she's doing very well is no excuse. She is active and lively and very smart and such a joy! She does miss her brother Noah, who is now living with us while he continues his flight training here in Orlando, and she always lights up when she sees him on Skype.

One present concern is for a brain MRI coming up on February 12. The reason for this procedure is that Heather noticed a spot of some sort in Grace's right ear, which has consistently shown a slight hearing loss. Each time she was tested, the doctors blamed the diminished hearing on something that could cause congestion (e.g. a recent COVID infection), but it has been consistent and the doctors hope to rule out the possibility of this being another consequence of her NF1 (neurofibromatosis type 1). They had been expecting to do a brain scan sometime in the future as a routine part of the NF1 care; the ear situation just hurried it along a bit.