Lift Up Your Hearts!

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.

What's missing from this picture?

That's right—no NG tube! No tape marks on her face. No heavy backpack of formula to lug around everywhere she goes.  Grace is eating well enough now to get all her nutrition that way, and the one medication she's still on (hopefully not much longer) she takes orally.

Congratulations, Grace!

Also, not only is her hair growing, but I see a little curl.

Having achieved his private pilot's license, and now being qualified not only to fly other people, but to take them up at night, Noah has been getting in his flying hours by treating his family members and friends.

It didn't take long for Grace to realize what was going on and start demanding her own turn.

It's not every three-year-old whose brother takes her flying in a Cessna 172P Skyhawk!

Grace had her appointment with the NF1 (neurofibromatosis type 1) specialist this week, and the news is good.

The possible complications of having that rare genetic mutation (1 in 2500) are many and varied. Her very rare JMML (juvenile myelomonocytic leukemia, 1 in a million) is just one of them. NF1 is the elephant in the room that has had to take a back seat to the dinosaur in the room, so I was glad to get a specialist's view of the situation. (For Heather's full report, click here.)

The appointment with the NF1 doctor went well. One of her oncology doctors was also there, the one who works closely in the NF1 world. Jon didn't remember meeting the NF1 doctor, but it was right at the very beginning, during Grace's first hospital stay, when she was diagnosed. So, much to be thankful for from that visit. They are sure that her fracture is from steriod weakening and not NF1. The kind of bone issues that NF1 causes is not evident in her, and it would be by now. Several other NF1 symptoms are also ruled out for the same reason.

But there is still the possibility of developing brain tumors. This is why she is scheduled for eye exams every six months for the rest of her life. They are working on a trial of oral chemo that looks promising. Because there is no sign of brain/retinal tumor in Grace now, that study will be finished before she would even need it. And we pray that she never will! I think they said she'll get a brain scan every year, but they did not do one this time. She had an eye exam in June and will have one next month. And they asked about her cognitive development. The tumors really affect that, so we are reassured by her development there. Jon asked me to look up milestones for three-year-olds and the only things she is behind in are some gross motor skills like jumping/hopping and riding a tricycle. (She tried riding a tricycle the other day, and her legs did not reach the pedals!) I had already planned on taking her to the playground more often, but that now has to wait while her leg finishes healing. That is on the mend, she does not limp much at all anymore. She still has some flareups occasionally, but the curve is dampening.

So Grace is doing well! She had another bone marrow aspiration in Boston yesterday—routine, except that I don't call anything that involves general anesthesia "routine." Results will be back in about a week; we're praying that her cells are still 100% Faith's.

Fun quotes from the last weeks:

9/7/24 - Nothing like older brothers to help you develop cognition. When she was in an "answer everything with 'no'" mood: "Grace, are you dumb?" "No" "Are you not dumb?" "Not dumb."

9/11/24 - "Mommy, all done eat my eggs. Please wash it, my fork."

9/11/24 - "Hope 'wake now. Not sleeping."

10/26/24 - Nathaniel and I were talking about when Grace can shower and bathe now that her central line is out. Grace then exclaimed, "Swim! Me swim! Maggie P! You come. Hope come."

Monday, 11/4/24: The kids were doing a survey for fun. "What's your first name?" "Grace." "What's your middle initial?" [They helped her say 'Victoria' and that it starts with V] "What's your full name?" "Amazing Grapey Grace!!"

Here's Amazing Grapey Grace in her Little Red Riding Hood Hallowe'en costume; one of her brothers was the wolf.