Lift Up Your Hearts!

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.

The doctors seemed to be taking Grace's recent intestinal problems very casually, but since she threw up her NG tube this morning, they're sitting up and taking notice. The new plan is for Grace to be admitted (in Boston) on Friday for observation and testing. (Not the way they wanted to celebrate her brother's 18th birthday!) Here's Heather's latest post.

Today, Boston called to say they'd like to observe Grace over the weekend and to prepare for admission on Friday. They will likely do a colonoscopy and maybe even an endoscopy. (And Dartmouth suggested they get some bone marrow while she's sedated.)

She threw up her NG tube this morning and so Jon took her to Dartmouth. They were able to do her regular appointment stuff so we don't have to go in tomorrow. It was a more traumatic NG tube placement than the other two times, and their tape is not as good.

We have been giving her zofran the last few days. Her appetite has been practically nil. There's definitely something going on. It's not the typical GVHD symptoms, but they haven't ruled it out yet. There might be an NF1 related cause; I've done minimal research on that and it's a possibility.

We depend on God to hold us up, so please intercede on our behalf.

How quickly things can change.

With good reason, we have been focusing here on Grace. But there's another half to this bone marror transplant, and that's her sister-donor, Faith. They were a perfect bone-marrow match, and Faith was delighted to be able to provide this life-saving gift to her little sister.

But it has not come without significant sacrifice, and I daresay insufficient preparation. The bone marrow transplant literature makes it out to be a much lighter procedure than it is. The pre-donation testing is rigorous and extensive—Faith fainted at one point—and the donation itself involved forty-seven separate insertions of the needle into her bones! (Possibly twice that many; I don't remember if that number was total, or for each side.) They had made so little of the strenuousness of the procedure that Faith had hoped to be able to join her friends on a ski trip a few days afterwards—not to ski, of course, but to sit in the lodge and enjoy the camaraderie and atmosphere. That was so far from the reality that I question whether "informed consent" was taken seriously.

Here's what happened, and is happening, according to Jon's most recent post:

When Faith signed up for the donation, she was told to expect one or maybe two days of significant pain, and then a week of pain that should be managed with Tylenol and then no running and jumping for six weeks, even if she felt like it.

She had 3-4 days of significant pain, and has certainly gotten better since the first week, but at more than 4 months, continues to have 2 out of 10 pain 24 hours a day. And more pain if she exerts herself too hard.

Grace's doctors referred Faith to her primary care, who took some x-rays and referred her to physical therapy. The x-rays didn't show any fractures. The physical therapist didn't really know what to do, in that she didn't know anything about bone marrow donation, but asked a bunch of good questions and gave her some exercises to do daily. She is supposed to go back to her primary care if she is still in pain in a month or two. We did find on Google that 4% of donors are still in pain at 9 months and 1% are in pain at 12 months, but the cancer doctors said that Faith shouldn't be in that class of donors, since she is so young. We do wonder if the "hard time" the doctors had when collecting the bone marrow would result in this extra pain, but we don't know.

Faith wants to play soccer in the fall, but if the pain continues, will not be able to. She would appreciate your prayers as she tries to rest, but also work on getting her body ready for soccer.

I know Faith, and I know that she would have made the donation anyway, even if she had known the consequences. But facilities that do bone marrow transplants should be paying more attention to the donors, who are an absolutely essential part of their cancer treatment process. Shouldn't there be someone on staff who knows about the consequences and complications, and can do more than send her back to a primary care doctor who undoubtedly knows little to none of that? I have great respect for physical therapists, and this one sounds good, but they don't have this specialized knowledge.

When you pray for Grace (thank you!) would you please also pray for Faith?  For wisdom for her, her parents and the doctors; for complete healing; and that she will be able to get fully back to normal life (and soccer!).

It has been blessedly quiet on the Grace front for a while, giving the family a chance to focus on Hope, her new little sister. No news has been great news, with Grace being weaned off the cyclosporin, laughing, smiling, playing, growing hair, and generally enjoying life despite the remaining presence of her nasogastric tube and central lines.

But now we're back with another prayer request. In Heather's words,

Grace has been having belly pain and a lot of mucous in her stool, often with blood as well. We have taken her off cow's milk again (she had been asking for it instead of almond milk.) We haven't seen much improvement in the last week, and she does complain that her belly hurts and she also has a diaper rash. There is the possibility that this is GVHD (graft vs. host disease) and for the moment we have stopped weaning her cyclosporine. We are waiting for her Dartmouth team to communicate with her Boston team to see what to do.

Please pray for complete gut healing, normal stools, and normal appetite. And wisdom and peace.

Thank you all.

The chimerism results from Grace's most recent bone marrow aspiration are in:  >97%!

I've seen chimerism results of 100%, but with this particular laboratory the best you can do is >97%.  Sort of the way the highest grade on a number of educational standardized tests is 99%.  So it's great news!

Hope is doing great, nursing and sleeping well.  Heather, too, feels well and actually (to her mother's eyes, anyway) looks more rested than she did in the last month of pregnancy.

On Wednesday, Jon and Grace will go to Boston, so she can have her post-day-100 bone marrow aspirate. Prayers will be much appreciated!

Grace appears to be doing very, very well. We do video chats, and it's wonderful to see her smiling, hear her laughing, and observe as her language blossoms.

The BMA is an important test, to give a picture of what's going on under the surface. Plus, it involves anesthesia, which is always a special concern. Last time they had to poke her twice to get enough sample, so we're praying that they can do it with just one this time.

Thank you all!

UPDATE 6/6/24 Grace made it through the procedure well, though that's all I know at this point.  Boston isn't the best at sharing results, seeming to prefer the "no news is good news" approach.  Chimerism results come much later, anyway.

Hope Serena Daley
Born Friday, May 31, 2024, 6:50 p.m.
Weight: 9 pounds, 11 ounces
Length: 22 inches

Hope was born at home, 17 minutes after the family's midwife arrived. Her timing was amazing; she made her appearance at the exact time her oldest brothers had planned to leave for their orchestra concert. Needless to say, they hung around a little longer. But they made it in good time, and it was an excellent concert—we watched the livestream. Too bad the audience was slightly reduced, but Hope can be forgiven for stealing the show at home.

Grace, the proud big sister.

Twenty-three hours later: Hope and Joy.

I am brazenly stealing Heather's post; I can't say it better.

This is the day the doctors consider the transplant to be successful. 

This doesn't mean that she's not still at risk for a bunch of things. But it does mean that Faith's cells are firmly established in her system and doing their job!

The long-anticipated Day +100 is rapidly approaching, but Grace managed to make it more exciting by picking up her first post-transplant cold. Turns out it is just a simple cold, but in her situation a runny nose and mild fever sent her on her first unscheduled return to the hospital post-transplant. There are so many things more serious than a cold that they have to check for, and they need to observe her closely to make sure her body is handling the challenge well.

I worried that she might fear the prison doors were closing around her again, but she took it well, and was very happy to beat her dad in Chutes and Ladders twice in a row. (Does anyone besides me remember that the game was once called Snakes and Ladders?)

Here's the short story version from Heather:

Overall, things have been going well, Grace's numbers coming back satisfactorily.

But then she got a cold. Just runny nose and sneezing. But after her nap, she had a mild fever. Boston wanted us to take her to Dartmouth, but Dartmouth said that if her fever went up, they'd be transferring her to Boston. So Jon decided to take her straight to Boston. (That was Tuesday night.)

They did a plethora of tests and the only positive was rhinovirus. But they wanted to keep an eye out on her for at least 24 hours, so they admitted her. She did get blood overnight, but everything else is looking good. Jon is hoping to come home tomorrow afternoon. She did some painting and lots of games with Daddy. He went over to the Dana Farber resource room to get her more books, since I had only packed her a few.

This is a really short version to get the word out and still get myself to bed relatively on time. Please keep up the prayers. Day +100 is just around the corner, and Jon discussed with the doctors the weaning process for the immunosuppressant drug.

I'm so very thankful that our family has not had any sickness from the time we had COVID at Christmas until now. Please pray that general trend continues and we keep sickness (and hospital visits) away.

Here's a really cool thing about the timing: Our nephew's wife (niece-in-law?), the one who's a pediatrician at Boston Children's, happened to be on duty in the Emergency Room Tuesday night!

You like Pirates of the Caribbean? You especially like the music? I think you'll enjoy this 13-minute story of the soundtrack.

Having shared that, I can't resist reprising my personal favorite version of the Pirates themes. For all of our Grace fans, this is her primary support group; Grace is in the backpack, contributing to the percussion by dropping a French horn mouthpiece, twice. (Summer 2023)

It's been a while since a Grace update, but the news is good, as the family is settling back into life together. Here's what the Daleys wrote—for pictures you can check out their blog.

As they say, no news is good news. Not much exciting has been going on lately. Grace is eating a fairly regular diet at meals, which has always been pretty small. Yesterday, she drank a lot, which hasn't been common for her, so we give her 600ml of water via her NG tube, 50ml every hour throughout the day. She gets 2 bottles of baby formula 50ml/hour throughout the day, which means she doesn't have to be on the pump at night, which is nice for everyone involved.

We thought she had c-diff last week, as the symptoms returned shortly after getting off the antibiotic, but the test came back negative, and her symptoms have mostly resolved, so perhaps it was just related to a switch from magnesium sulfate to magnesium oxide for a few days due to it being impossible to get from a pharmacy within an hour of us. Heather had to crush up the pills and make a solution herself, which you would have thought a pharmacy could do... But, she is back on her regular magnesium sulfate now, and we just have to remember to schedule and pickup a few medications at the Dana Farber pharmacy when we go for weekly clinic visits.

Jon has returned to work, with mostly regular hours. The family is happy to be back together and enjoying conversation and games and just regular life together.

Grace has a bone marrow aspirate scheduled for tomorrow, to verify that everything is working as it should, and we expect those results to be good. She'll get another one around day 100 and that is the next milestone we are looking forward to, as she should be able to get off cyclosporine at that point, which causes lots of side-effects, so the other medications can stop soon after that.

One such side effect that we had heard about but she had not experienced until this week is that she is growing hair on her face and ears, so think of a combination of teenager peach fuzz and old man hairy ears and eyebrows, and you'll have a good idea of what it looks like.

Grace often asks if the baby is awake and likes to feel its kicks. Heather has been feeling pretty big, and measurements confirm it and there is a question if there are any tests to do (glucose or an ultrasound). We've been joking about whether there is a hidden twin, but we're pretty sure there isn't. 

The BMA mentioned for "tomorrow" was yesterday. She's never minded them before, but this time they had to poke her twice, so she's a little sore. The hair-in-unwanted-places story reminds me of the well-known fact that a dieting woman will lose weight from her bust sooner and faster than from her hips....

All continuing to go well, Grace will return to her New Hampshire home on Monday! This is huge for her and for the whole family.

She will need to return to Boston once or twice each week for clinic visits. At some point, the Dartmouth hospital should be able to handle some of the appointments, but for now, they've decided it's worth the long drives to be able to have the family all together. After all, they've been driving almost that much going the other direction while living in Boston.

Grace has had less nausea, has gotten her formula intake down to 16 ounces per day, is eating more on her own, and is steadily gaining weight. Thanks be to God and all your prayers!

Please pray for:

• Continued progress for Grace
• A smooth transition back to being a family all together.
• Wisdom for Heather, Jon, and the doctors as they figure out the best way to keep Grace healthy while reintegrating her into normal family life.
• Strength for Heather as she handles the normally-exhausting late stages of pregnancy.
• The birth and the baby. I'm rooting for a girl, and in my mind I've already named her "Hope"—but I have a less-than-stellar batting average when it comes to baby guesses.

Grace's next milestone will be the three-month mark (May 8), where if all things continue to go well, her transplant will be considered to be successful. It's only the next of many more steps, but it's an important one.