Lift Up Your Hearts!

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.

Dear Grace,

Please stop trying to distract me from politics by getting hurt.

Love,
Grandma

From Heather this morning:

Sometime recently, Grace fell off a wooden block that she was using as a stool. I thought she had twisted her ankle. She would complain on and off, but still often insist on walking. Then Sunday, Noah came to me and asked, "Did you know that Grace's foot is crooked?"

So we decided it was time to see an orthopedist. Monday, she got X-rays and lo, and behold, a bending fracture in her left fibula. (Imagine a green twig that just started to crack, but released before it broke.) They do not cast this kind of break and said to follow her lead about how much she walks. However, she has such a high pain tolerance that I think we need to restrain her a bit more. She was limping more yesterday.

We have messages in to her other doctors, to discuss the effects of steroids and also NF1 on her bones.

Please pray for healing and wisdom and that I will not worry about future fractures.

She's not kidding about Grace's high pain tolerance. When the doctor was gently pressing on places on her leg, beginning with her toes, she did not react at all until he reached the exact place where the x-ray indicated the break, and then she only flinched.

Young children's bones are amazing when it comes to healing; if I broke my fibula no doctor would casually say to me, "Do what you feel like, but no bouncing on a trampoline, and stay away from the playground for a few days." Maybe Grace takes after her great-grandfather, who followed his doctor's advice as to restrictions when he went home after surgery: "Just do what you feel like doing, but I'd avoid chopping wood for a while." Apparently the doctor didn't anticipate that he would feel well enough to go bowling, and he ended up back in the hospital.

Young children's bones are remarkable, but what makes this a special matter for prayer is that one of the many possible complications that can occur with NF1 is bone problems. Grace has shown no other symptoms than the big one (JMML), and there's every possibility that this event is just the result of (temporary) long-term steroid use and being an active toddler. But something more serious is not off the table.

Grace's third birthday started normally enough. We enjoyed a video chat, watching her come to grips with the fact that the answer to "How old are you?" had changed dramatically!

However, her very busy day ended with a trip to the Dartmouth emergency room because a key part of her central line broke during the routine evening flush.

After a night and a morning filled with what would have been a comedy of errors if there had been anything funny about it, she had surgery to remove the entire central line. This major milestone was intended to be reached two weeks later, rather than on an emergency basis, but she did well, and the job is done! I've copied the following directly from Heather's post, so you can see the pictures.

Line removal surgery was successful. She did great for the IV.

She came out of anesthesia quickly and they were home by 4:30. It is strange to her to have her line out. But next week she'll be able to have her first bath in a year! She is excited about that. 

After she got home, Grace opened her birthday joy jar.

Heather's note on the Joy Jar:

NEGU Never Ever Give Up. This organization was founded by a girl who had a terminal brain tumor. She wanted to spread joy to her fellows and give a Joy Jar full of goodies to every child diagnosed with cancer. The organization has now grown so that they give Joy Jar subscriptions to the children and their families. Every month, Grace gets a jar with a book, crayons, coloring book, stuffed animal, and other toys. She loves her Joy Jars and is so excited when a new one comes. They also have given us all t-shirts, a beach towel, a blanket, a popcorn bowl with popcorn, and numerous other goodies. One month, they included the book Jessie's dad wrote about her life, which was powerful. I certainly cried hard, but it has such a hopeful theme even while dealing with all the hard questions of life, death, and terminal illness in a child. negu.org

Good news: Grace is getting her central line removed! The procedure was scheduled for two weeks from now, but it's coming out as an unexpected birthday present.

Bad news: The present came wrapped in less-pleasant circumstances: one of her lines broke during the routine flush. They were able to clamp it off, but Grace and Jon are now headed to Dartmouth to get her started on antibiotics, in case the line was contaminated. They will probably do the removal procedure tomorrow.

Prayer request (from Heather's blog post): Please pray for safe travel and no infections and a successful procedure.

Microsoft caught me.

I have been avoiding ChatGPT and other AI temptations for a long time, particularly when I receive invitations to use AI for my writing. I am confident enough to prefer what I write myself, thank you!

Drawing, however, is another matter. When Microsoft's Copilot recently—and unexpectedly—appeared in my Windows Taskbar, I was a bit disconcerted, but intrigued enough to give it a try.

I wanted a picture for Grace, to go with the caption, "Happy 3rd birthday, bonnie warrior!" After about 15 minutes of work, this is what I chose.

These are some of the iterations along the way. My second choice was the manga-looking image on the right.

That was fun!

I have no further news on Grace, other than that her rash is responding well to the cortisone cream. Her mom just turned 45, which means my daughter just turned 45, which means.... You do the math. Or don't.

Grace herself will be turning THREE YEARS OLD this week. A year ago we feared she would not reach this milestone. She still has a long battle ahead, but we are so grateful she has come this far. Grace is such a cheerful fighter, a patient patient, and a delightful person! Thank you for all your efforts on her behalf.

Because so many people take pleasure in blessing Grace, and she has no physical needs except health, Heather has requested that continued prayers would be the number one gift they could ask for. That link also includes a list of some of the organizations that have provided them immeasurable help throughout the past year, in case anyone wants to support the good that they do for families of children with cancer.

We just returned from a great visit to New England. (Yes, all through Hurricane Milton, which was a bit of an adventure, but I'll get to that later.) In addition to attending my nephew's wedding, we had a lot of fun with grandchildren.

Grace was amazing! Except for the NG tube, and the bag she carries around all day, wherever she goes, and her central line, and the fact that she asks "Milk in it?" of any new food, you'd never guess she was sick. Just shy of her third birthday, she's as active and energetic and competent as you could wish. And very much in the "Me do it!" stage. Cute as all get out, too.

You knew there was going to be a "But...."

Suddenly, she is sick. Two of her siblings recently developed regular colds, but this doesn't look like a cold: so far, at least, the only symptom has been throwing up, which she did several times last night. At last word, she has no fever—and this is a big part of the prayer request, because in her case a fever sends her immediately to the hospital.

Many thanks for your continuing long-distance love for our little girl.

I've been remiss in updating Grace's Journey; my only excuse is that no news is good news! She has been doing well. For a more complete update from Jon, see their new Daley Pondering blog post, from which I will quote extensively. Jon is writing.

It has been great to have less medical stuff going on these days, and we get more comments about her being "so cute" in the grocery stores and the soccer games, etc.

Her hair is growing in, and I don't think I've heard her referred to as a boy in a while (for a while she just looked like she had a really short haircut, but it is growing out more). She discovered the 18 month clothes bin in the attic the other day and has been wearing some onesies, since she mostly still fits into them. She'll turn three next month.

Life, however, is still far from normal.

We continue to do weekly dressing changes - we had a full day yesterday and so she fell asleep during the change - it is hard to imagine kids screaming through it, but we are certainly blessed to not have that problem. She happily puts on her mask and then holds her hands above her head until it is done.

She has been gaining weight, and we've started up the steroid tapering off again and doctor visits are every other week, once a month to Dartmouth and once a month to Boston, so that schedule is nicer too. It is possible that the steroid taper will be increased and be done soon, which then triggers the start of the cyclosporine (immunosuppressant) taper, which will be the end of the meds entirely!

Her nasogastric tube fell out while she was sleeping last week.... She woke up in the morning and brought the tube down already pushed out of her throat. So, we proceeded to the semi-unapproved installation of a new tube, and it went successfully.... We'll see what Boston says about that on Wednesday. Last time, Dartmouth recorded the procedure as "without contacting medical providers", and did an x-ray to confirm proper placement, but didn't explicitly forbid it in the future.... I think that because we know the exact length of the insertion, there is fairly little risk involved—a nurse at Children's told me that she will really start coughing if it is in her lungs rather than her stomach, and we verify the pH after insertion as well. (The supply house apparently thinks it is reasonable for us to do the procedure, because they send all the equipment to do so, though maybe they are thinking a visiting nurse—they are sometimes surprised to hear that we do dressing changes ourselves.)

Faith, big sister and bone marrow donor, has been doing well also. This donation recovery period, which was supposed to last no more than a couple of weeks, has been a long, slow process involving physical therapy and chiropractic care. (The transplant doctors pretty much washed their hands of the problem.) But she estimates that she is now "98% back," and has been able to enjoy playing on the high school girls' soccer team, which means a lot to her. Her coach and her teammates have been great, encouraging her to play when she can and rest when she needs to. They even celebrated Leukemia Awareness Month—the "color" of which is orange—by dedicating a game and a fundraiser to Grace, Faith, and their whole family.

(click to enlarge)

The high school principal said there was a "sea of orange" in the school that day, and there were a lot of people wearing orange at the game.

The team raised about $1600! The opposing team gave the family flowers, though they did not go so far as to refrain from winning the game.

Grace's "numbers" have been doing very well, as has she herself.  But at today's clinic visit, her creatinine level was elevated, and that was confirmed by a repeat test.  This indicates potential trouble with her kidneys, so she is scheduled for a kidney ultrasound on Friday.

Thank you, as always, treasured pray-ers.

For those of you who enjoyed Charles Cornell's analysis of the writing of the Pirates of the Caribbean music, and/or Grace's family's production of the same, here's another Cornell video, and not coincidentally another Daley production, this time for The Lord of the Rings.

I have mixed feelings about those movies, which to my mind do a grave injustice to J.R.R. Tolkien's creation, but they have their good moments, and the score is incredible. I'm a devoted "classical" music fan with little patience for so-called popular genres, but modern art music has veered off into such strange directions that I'm more than half certain that all the good composers have deserted to movie music. And I say, more power to them!

The Daley version was created two years ago this month, a year earlier than their Pirates production. Grace's contribution comes at the end of the credits.  (I was disappointed that there was no 2024 family musical production, but there was this small matter of Grace's cancer consuming every spare moment of their lives.  Maybe in 2025!)

(Family still trumps politics.)

I recently spent several days with Grace and her family. It was SO GOOD to see her in person and in action.

Grace is my hero.

There's a reason Christ said of children, "of such is the kingdom of heaven," and "whoever does not receive the kingdom of God like a child shall not enter it."

Grace has faith and trust as strong as any I have ever seen.

Her central line is covered by a dressing that needs to be changed periodically. I watched the process take place on our sun-drenched deck, complete with sterile gloves, masks, disinfectants, the whole nine yards. And what did Grace do? She lay flat on her back with her arms over her head, absolutely stock still, until the procedure was finished. This is the same procedure the hospital assumed would take five assistants to hold her down!

It's not as if she's a passive patient. She knows, for example, exactly how the daily procedure that flushes her lines must go, and doesn't trust the strange nurses who do it when she is in the hospital. When they do not follow the procedure, she does not hesitate to let them know.

But Grace trusts her parents, and relaxes patiently when she knows they're in charge.

What's more, she quietly accepts the necessary restrictions on her life, all the while living as normal a life as possible. (Well, mostly quietly. She IS a two-year-old.)

That small backpack contains the nutrition and whatever else goes into her stomach via her NG tube, plus the pump and associated controls. She's small, and it's heavy, so she'll more often be found dragging it along the ground instead of wearing it on her back, but it goes everywhere with her, except at night. She takes it as a matter of course, and is adept at manoeuvring all the associated lines as she goes about her normal play. (Not to mention that she is aware when the machine's beeping alerts her to a situation that needs attention, and sometimes even knows what must be done to fix the problem.)

Grace trusts her family (siblings as well as parents), and does the best she can to live a good life within the parameters that have been given to her. That's faith. (Her sister Faith is another one of my heroes.)

This faith and heroism is true of the rest of her family as well. When cancer comes to visit a family, it brings with it a host of opportunities for heroic behavior. Here's a great picture of the family at camp recently.

Those of you who enjoy puzzles might appreciate zooming in on the shirt that Jonathan (at the right, holding Grace) is wearing. Just to set the bar for you, our 14-year-old grandson, visiting from the other side of the Atlantic, figured it out in a matter of seconds. Don't let that deter you; most people take a lot longer than that, if they get it at all. (Though I will brag that I wasn't much behind him.)

In case you didn't catch it, a very important part of our vacation was getting the New Hampshire and Swiss cousins together for the first time in seven years. You won't see as many photos of that, as the latter folks are understandably more photo- and social media-shy, but it was such a great time, and they played together so joyfully as to do my heart a world of good!

For a complete Grace update, including a couple more adorable pictures and a cool story about Faith's soccer team, see the Heather's Day +170 post here.

Bragging about her grandchildren is written right into the Grandmother Contract, and I've certainly done my part in the past. But never like this.

The pathology report on Grace's colon biopsy indicates graft versus host disease. (That's not what I'm bragging about.) It is a mild form of GVHD, and they believe a couple of weeks of steroids should take care of it.

My own grandmother-gut suspicion, based on no medical expertise at all, is that the GVHD showed up in her colon because that's been the weakest and most stressed part of her body for most of her life. It was intestinal problems that eventually led to her NF1/JMML diagnosis. (Once the doctors got out of the rut of thinking, "it's just food allergies.")

We thank God that the doctors believe this will be a short-lived problem.

And here's the punch line. Two-year-old Grace has no doubt been set back somewhat in her development by all her medical problems and procedures. But there's no doubt that she listens, pays attention, and thinks about what she hears. When she overheard that the diagnosis was GVHD, she got very upset!

They anticipate discharge on Wednesday.

Here's the latest Daley update, complete with pictures.

The Reader's Digest version:

• Grace and Jon are still at Boston Children's, so she can be observed while awaiting the test results.
• The bone marrow aspiration went better than last time and they got all the marrow they need for their tests (but results will come later).
• Grace's esophagus looks fine.
• Her colon is quite inflamed, which the doctors are sure is causing her GI symptoms. They have no idea why, but are hoping for enlightenment when the pathologist looks at her colon biopsy. Hopefully that will be early next week, as in the meantime the doctors involved don't agree on what to do with her diet.
• She had some cardiac irregularities when waking up from the anesthesia, but they were short-lived, and otherwise her recovery went well.
• During a video call last night, she was eating a bagel, laughing at her brothers, talking, and playing.
• The rest of the family had a great activity day, arranged by on-belay.org, an organization supporting kids impacted by a loved one's cancer. They all had fun, but Faith is still frustrated by the pain that continues to restrict her activities, so please remember to pray for her as well.

As always, we are so grateful for your support.

From Heather (emphasis mine):

Grace has been on a clear liquid diet until today where she is getting nothing into her stomach until anesthesia at 5:30. She will be getting a complete GI scope and a bone marrow biopsy. Please pray for the anesthesiologist and the surgeons as well as for Grace.

Jon called this morning to say that they will be at the hospital at least until Monday, because the doctors want to observe her as she goes back on feeds. Also, for the results of the biopsies. (I'm assuming intestinal, because the bone marrow takes a week.)

Jon is discouraged and sleep-deprived, and it's not really practical for me to go there because of Hope.

Grace and Jon did have a good day yesterday, playing games, doing crafts, reading books, painting, watching Mary Poppins.

We're holding on here at home, but now will need to prepare for and go to Family Camp without two important members of the family. Hopefully, they will be able to join us soon.

In other news, Noah is 18 today, and to celebrate true adulthood, has a written FAA test and a dentist appointment. 

In case you were wondering, those of you who know of Noah as a baker, he is making his own cake today. Maybe I'll have pictures later.

And here's a joyful update, which I'm sure is helping Jon a lot:

We have a friend who just volunteered to go down and give him a break. Praise God for his people!

This was Heather's morning post. (I'm copying the text, but if you click on the link, you'll see a cute picture.)

When Jon and Grace got back from Dartmouth, Jon asked me to take her temperature. He could feel that she was hot, but the thermometer they used at Dartmouth did not register a fever (it was the kind that you hold a centimeter away from the forehead.) My initial armpit reading was 101.8. That means Boston admission. So I had Joy and Nathaniel pack a few more days of clothes for Jon and Grace (our emergency bag in the car only has two days' worth) and Jon got her medicines and his work stuff ready. By the time they left, her armpit temperature was 103.3.

Jon's midnight update included that her fever had gone down to 38.1 (100.8) by the time they arrived at the ER and that she had eaten half a popsicle and smiled for the first time all day. She got all sorts of tests done and abdomen x-rays. So far, according to the portal, she is negative for flu and cold viruses and x-rays show nothing unusual. Her hemoglobin fell significantly, so I expect she got/is getting some blood this morning. At midnight, she had not been admitted yet, but she is in 6 West now.

First, I have to make a correction. That may have been the first smile Jon saw yesterday, but we Skyped with her mid-morning, and she was smiling then, even though she had just thrown up.

(Kudos to Heather, who had to clean up what must have been an all-time record for spreading vomitus in the most inconvenient places.)

Since then, she has had some very welcome visitors, having had the good sense (?) to get herself admitted just in time to catch our favorite Boston Childrens' pediatrician before she becomes our favorite pediatrician at Children's Hospital of Philadelphia. (Whether it was a good move to show up just in time for the arrival of the annual batch of newly-minted residents is another question.)

The plan is to talk with the gastroenterology people today. In preparation, Grace is not allowed to eat. Apparently that is making her sad, which is actually good news, because since getting sick she has had little to no interest in eating. Is she still getting nutrition through her NG tube? I don't know. Is there any medical person who can tell me if "NPO" means "no input to the system" rather than literally "nothing by mouth"? If the point is to have her system empty for her procedure(s), putting the food directly into her stomach would seem just as bad.

I don't know if she received blood or not, but maybe not, since they're blaming her hemoglobin drop on having pumped a lot of fluids into her (because of the fever, I assume).

That's all, folks ... for now.