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Powered by LifeTypeJon was reading through Grace's test results and saw that the most recent bone marrow report mentioned the JAK3 mutation. He contacted her doctors because we thought that one was gone following the transplant.
Dartmouth doctors can't see her Boston results to compare 
so they contacted the head transplant doctor there. She said that seemed odd given the good chimerism results, but she wants another aspirate as soon as possible to make sure. So that is scheduled for June 12. We aren't clear as to why no one noticed that before. We think the Dartmouth doctors thought it was expected, and I guess the Boston doctors didn't see the results? It does make us wonder if we need to be paying more attention - we try to read all of the medical records, but don't always read through everything, and sometimes it gets pretty technical.
On Thursday, Grace got her hearing aid! They did a whole hearing test so they could program the device to her exact needs. The inner ear test shows that she hears fine on that end. So the aid amplifies the sound so it can reach the inner ear.
She noticed new sounds right away, and she played around with her voice in the car ride home. She still asks us to repeat ourselves, but a lot less than when she doesn't have the aid in.
Our week has been somewhat full, with a few extra church services, including Maundy Thursday with handbells, which I didn't find out until the end of the service that it wasn't recorded, and I meant to record it during rehearsal, but I forgot. But, it went well, despite Jeremiah being sick, but fortunately, Faith's part was pretty easy, so she was able to take over his bells as well as her own (so, I think that means she had more than an octave of the highest bells).
We also had a combined service with the Methodist Church and Smith Church today. They had a "tables of the cross" type setup to explore after the service, and Nathaniel in particular stayed for quite a while.
We had our appointment with the NF1/oncology doctor yesterday. It turns out the neurofibroma is even bigger than we thought. In addition to what we already knew, which is that it goes from her external ear where we can see into her middle ear, it also extends down under her ear into her parotid gland which is a salivary gland in the cheek. This is a plexiform neurofibroma, which is something that has actually been there since birth. And it has grown over time. (There are other neurofibromas that can come later possibly, but they are not the plexiforms.) This plexiform right now is benign, but it could potentially become malignant at some point. An initial screening has indicated that this one is one step towards becoming malignant, but they are still unsure of how many steps the body takes to get there.
Grace will be going on a medicine which is designed to shrink the tumor, and it may prevent some other kind of tumors. This medicine can have some unpleasant and even dangerous side effects, including diarrhea and rashes, vision impairment and heart issues. So Grace will be going in monthly and then every 3 months to check on all those functions. Right now, the plan is for her to take this medicine for 2 years, and then reevaluate, based on new research and her body's response to the drug.
One fascinating thing that I learned yesterday is that the longer hair on her right side that we have noticed since she was very young is related to this neurofibroma. The doctor said that because of extra vascular activity on that side it is not surprising that it would cause faster hair growth. When I heard that, I went back to my list of Grace's symptoms that we made before her NF1 JMML diagnosis while we were trying to figure out what was going on with her. I noted that she also would get redness on her right cheek when she ate sometimes. We thought at the time it was an allergic reaction, but it is likely due to the extra vascular activity. (It still happens sometimes.)
Please pray:
That Grace will be able to swallow the capsule (twice a day!)
That she will not have any serious side effects. We would also appreciate if she didn't have any other side effects. It's been really nice the last few months to not have to deal with diarrhea.
That the medicine will do its job, which is to shrink the tumor. I forgot to ask if her hearing loss is permanent or if it could come back with tumor shrinkage.
Some of you may be wondering ( I hear you Grandma!) why could they not see this in the imaging. The reason stated is that the plexiform neurofibromas are a soft and spongy tissue, and is easy to not notice them, and/or think that they are something else. The ENT even said that it's made of the same kind of tissue that your outer ear is made of anyway, so then it's really hard to distinguish.
I guess that's it for now. Here's a collage of pictures that Grace took of Hope in the grocery store:
One year ago today, our older kids got to see a total solar eclipse, and Grace came home! These annual milestones are flying by this year, and we are thankful.
Grace's NF1/oncology doctor called Jon with biopsy results on Friday. It is the opposite of what we were hoping. Both biopsies indicate plexiform neurofibroma and it's really one big tumor that connects from the outside where we can see to the inside behind the eardrum. Surgery in this delicate area is not advised. We will have a meeting with the doctors Wednesday to discuss treatment. Some good news is that Jon has talked with other NF1 parents whose kids have done well on the medicine.
That news was a blow, but I am doing better after attending a prayer meeting on Friday and church on Sunday. I am holding onto the hope that God is still working and will heal her one way or another and in his timing.
Both Jon and I are going to the meeting tomorrow, so we can be clear on the options, side effects, pros, cons, etc.
Here is Grace with some presents from ladies at church:
And helping Daddy with his project:
As far as we know, the surgery went well. He took two biopsies, one in front of the eardrum and one behind. There was not much fluid, so he did not put in a tube. We will get results in several days. The bone marrow aspirate also apparently went well, and that also takes about a week to get all the results back.
The cotton ball they put in her ear got soaked through, though it was not supposed to come out until tomorrow. But Jon put in a new one and there seems to be no more bleeding.
As Jon mentioned, Grace is having ear surgery tomorrow to explore what's going on, get a biopsy, and maybe get a tube in. The oncology/NF1 doctor also managed to schedule her bone marrow aspirate for the same day so Grace will not have to be sedated twice in a few weeks.
Prayers for all that accompanies sedation (including fasting, Jon's conversation with the anesthesiologist, and emergence) are appreciated. And that they will find exactly what they need to, that everything will be clear. And that her marrow will still be all Faith's!
When she bites into a clementine, it gets a face!
She is in a phase where she loves making dough. She has requested it nearly every day the last week.
An ENT, a radiologist and an oncologist walk into a bar...
Oh, maybe not, but they at least had a discussion about Grace while looking over the MRI and CT scan together and the conclusion is that it isn't a plexiform fibroma at all! Which is what Heather has had the faith to pray for all along! She has had a number of positive signs from God this week and so she is greatly encouraged. The lunar eclipse was pretty neat to watch, and she was also blessed with a meteor just after as she was going to bed.
I haven't talked to the oncologist/NF1 specialist who was the one to diagnose it in the first place to hear why she changed her mind, but collectively, they don't think what is going is consistent with an NF1 fibroma, so they are looking for another cause.
Grace now has surgery scheduled for next Thursday, the 27th, to check out the potential fluid behind her ear (last time I talked to the ENT, he was thinking it might not be fluid, but some spongy tissue), possibly install a tube in her ear drum to let the fluid out, if it is there but getting blocked from going down her eustachian tube, and do a biopsy to grab near the potential NF1 fibroma to see what it is.
She will be sedated for the procedure. They haven't scheduled the time yet, but it will likely be at 6:30AM or so.
Today is the day, one year ago, that Grace was allowed to exit the hallway doors of 6 West and live in a place other than a hospital room! They played Raining Tacos for her exit parade, so my thought is to celebrate each year with tacos for dinner. (And maybe a shamrock shake thrown in.)
Although Alexa will play the correct song if you only ask, "play tacos," Grace can now ask for "raining tacos."
I used to tear up every time I heard it, but we do hear it a lot now. (:
It is interesting to me how fast these anniversary markers are coming. The days between admission and discharge last year were so very long and hard. This year, January 30 to now has sped by.
The results from Friday's CT scan and ENT appointments need to be discussed among her doctors. The ENT doesn't think the hearing loss is coming from the fibroma, because it is so small. But there may be bone erosion, from a cause yet to be determined (chemo?)
And since now more than one person misread my last post, I will clarify that Grace's bone marrow aspirate is scheduled for next 17th, April, not today.
Selfie
She made this into a double stroller herself.
And I'll end with a quote:
"Dis little toe wenta market. Dis little toe 'tay home. Dis toe, what? [ate roast beef] Dis toe? [had none] And dis little toe went aaaaaaallllllll a way to Papa's house!"
Grace has her ear CT scan, and audiology and ENT appointments this Friday, March 14.
April 17 is the scheduled date for her next bone marrow aspirate; that will be at Dartmouth this time.
Fun quote from yesterday, "You aren't Joy any more. Your middle name is 'Mommy' now."
I get daily emails from GodTube.com, and they are often worth reading. Today's devotional was good.
While I don't ever feel (well, not since just after the bone marrow transplant) that "life is over", it is sometimes hard to think about what Grace's life will be like, and the hard decisions we, and eventually she, will have to make can be overwhelming.
As one friend said the other day, (paraphrasing) "I thought things were settling down, but I hadn't realized the full extent of what was going on. I will re-add Grace to my prayer list."
Grace had several appointments at Dartmouth today: audiology, ENT, oncology/nf1, lab draw
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, 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.
Here are a couple of pictures from our extended family vacation to Washington, D.C.
At the National Children's Museum - flying like Noah!
Smithsonian American History Museum - future president of the United States??? She sure thought this speech-on-camera simulator was fun!
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 NF1 team at Dartmouth Hospital doesn't work on Wednesdays, but the onocology team didn't want to leave us hanging, and so called with the preliminary results this afternoon. The good news is that it doesn't appear to be impacting her brain at all.
The radiologist recommends a CT scan to see how much the tumor is impacting a bone in her middle ear. We already have an appointment with audiology and otolaryngology on March 4th, and the oncologists are going to try to get the NF1 doctors there as well, so they can have a group discussion about what to do next. The oncologist's opinion was that surgery wouldn't be a good option, based on where it is. So, I think the other option is "medicine", which I don't think she specified whether it is an oral chemo (which I know is the solution if she gets a tumor on her retina), or some other sort of medicine. In any case, there isn't anything particular urgent about it, but we'll hear more next week and then I assume some really good answers in March.
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 request), and she did a terrific job, she winced once at a loud noise, and then fell asleep to soothing sounds of the MRI. (and that is sarcasm, if you couldn't tell. And MRIs have gotten signficantly louder than when I was involved in the Cognitive Center for Brain Research at Carnegie Mellon. I think of the "chunk chunk" sound as a normal MRI, but it is apparently now common to have really loud motor/whining/horn sounds. 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.
I asked to see the pictures at the end, and Grace and I enjoyed looking at her eyeballs. She also showed us her ears, though she scrolled by quickly (though I had a suspicion that her right side looked different from her left (which is possibly why she scrolled so fast), but Grace thought her eyes looked pretty interesting.
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...
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.
I'll try to get a picture of Grace and Faith together on this anniversary. Hopefully our whole family!