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Powered by LifeTypeBased on the number of supportive messages we got yesterday, I know you are all eagerly awaiting an update.
We arrived at the Dana Farber Jimmy Fund Clinic for lab draws and "here's what's happening" meetings. We sucessfully ordered Grace some food, and then we had to pop across the street for an audiology appointment. The audiologist had wanted to retest her right ear because of fluid, but there wasn't enough time for the fluid to drain, so the results were exactly the same (left ear fine, even though there is fluid, right ear slight hearing loss because of fluid.) She did not keep us long and will see Grace again in about 6 months.
Then back over to the Jimmy Fund clinic, and waited a long time. Jon got a message from some Hillsboro friends who saw us at the elvators in the morning, and so he spent a good time there with them, helping them interpret and understand the nurses' explanations of the chemo the husband was getting.
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Today is our last day all together as a family for a while. God blessed it by sending a beautiful six inches of snow that cancelled our outside activities for the day. We plan on spending it cuddling and playing games (and packing for Boston.)
Prayers for this week:
Praises:
We got a visiting nurse for Grace's dressing change instead of having to drive to Boston this weekend.
Snow Day!
Grace's bone marrow aspirate gave results that made the doctors happy (though I don't really understand the details.) The bottom line is that it means she is in a good position for transplant.
Requests:
That the chemo will do its job to wipe out Grace's bone marrow entirely while giving her minimal side effects.
That we will have good communication with the doctors and they will have good communication with each other.
That the other kids will be sustained and at peace while they have to be separated from their parents and sister.
Mental health for all of us as we deal with all the hard things surrounding these circumstances.
Thank you all so much for your support and prayers. We can feel it!
Monday, Grace had pre-transplant bone marrow aspiration and bone biopsy. That's kind of boring. The interesting surgery came right after that procedure.
Fertility preservation is something I had vaguely heard of. I know it's rather personal, but so is all this health information. We won't be sharing her future decisions (she can decide that for herself) but I think
the idea is so interesting that I wanted to share.
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Jon and I both went with Grace up to Dartmouth today. The dentist met with us pre-op and said after looking at the x-rays, she thought her front teeth looked really bad and she expected to have to take out the four upper front teeth. This just seemed horrible to us, but I signed the consent because it fits in the "best for transplant" box. Jon left after that, but he did figure out how to get on the list to play the piano so that was good.
I went with Grace into the PainFree clinic and she got the anesthetic directly into her line, without needing a mask first. She just laid there, eyes closing and yawning and I said, "Goodnight, I love you, I'll see you when you wake up." Then they gave me a pager and I went down to the chapel. I pled with God with tears that she might keep her teeth. I texted/emailed a few people also to ask that they plead for her teeth as well. Then Jon came down to the chapel and we spent some time there together, encouraging each other with scriptures.
Her oncologist called and asked when a good time would be to meet, so we were fine with going up then. So we talked to her and she is so compassionate. We like her a lot. She understands that there are things we don't like about what we have to do, and she did her best to help us understand and comfort us. After that meeting, we went down to the mall/food court and got some lunch, which was good.
Back up in the family waiting room, the dentist came post-op and told us that she had only had to take out the two front upper teeth, and then also several fillings and three caps on other teeth. So that was somewhat of a relief, and part of me wonders at God's sense of humor "well, I'll give you two, but I'll give the doctors two also." I hope that doesn't sound sacriligious to some people, but it helps me.
Grace also had an eye exam and a dressing change while under anesthesia, but that did not take very long. What did take a long time was for her to completely wake up. I got to hold her in my arms and rock in a chair, but she did a lot of napping and sort of waking up and napping again. Eventually, she was ready to read books and eat a popsicle and play with some toys. After that, she had her CT scan which was very quick.
Home for pizza and hymn sing, which is a blessing. She is now back to her cheery self. Her smile is so wide that you can see all her caps, even though they're in the back. And I do have to admit that the "all I want for Christmas is my two front teeth" smile is pretty cute. I'd like to post a picture sometime soon, but now it's time to go to bed. Thank you all for your prayers and support.
Please pray for Grace's appointments today. Extensive dental work at 11AM under sedation (fillings and crowns), and sedation means fasting from now until after she wakes up; and then CT scan in the afternoon. We are scheduled to be there all day, so coming home to pizza and hymn singing will be a relief and a blessing.
Warning: I'm feeling better now, but the following post pretty accurately how I felt for most of today.
After a good appointment in Hillsboro, they still wanted us to go to a pediatric dentist who was familiar with chemo/transplant issues, so Heather headed up to Dartmouth this morning in the snow.
She slipped off the road, thankfully pretty slowly, and hopefully just damaged the plastic front bumper, but there might be some more significant damage. The police had the car towed to a nearby garage and we picked it up on the way home (and did you know that they make 3 and 5 foot long zip ties? I stopped by a hardware store on the way to be able to temporarily put the car back together. We'll have it checked out at our mechanic tomorrow or Thursday to see what the plan is.
We went to the Methodist Church today. It was so refreshing. It was communion Sunday, which was such a blessing. It's been a long time since I took communion. They anointed both Grace and Faith with oil and prayed for them and us. Such outpouring of love and support. And I don't know exactly how to describe it, but being there in person helped me really feel all the support from all the churches and people everywhere who are praying for us.
For the most part, these last two days went pretty well. Grace was a trooper as we navigated the multiple confusing buildings and talked with many providers and was poked and prodded by various specialists.
Her audiology appointment went pretty well. The doctor was impressed with her development, and there were some humorous things, like when they were playing a "put it in" game, and she was supposed to feed the plastic dolphin a plastic fish whenever she heard "put it in" in one of her ears at varying volume levels. At one point, the doctor handed her an apple and then a green pepper and though she heard the sound, she didn't want to put the the fruits and vegetabes in the dolphin's mouth - apparently didn't think those were appropriate food for a dolphin, because she made a face, and was happy enough to put them in a box, when they switched games.
She had more trouble with the varying frequency tones, and the doctor was really good at reading body language to know when she had heard the sound even if she didn't follow the direction. The hardest game for her was to look to one side or the other to see a character on the TV screen when she heard a tone. She was mostly concerned about where the character went whnen the TV turned off, so I'm not sure how much was hearing versus not understanding she was supposed to look at the TV before it turned on. Sometimes, when the frequency would change or the volume was lowered, she would stop responding so it seemed like she wasn't hearing it, but then the doctor would go back to the frequency and volume that she knew Grace could hear and she still wouldn't respond.
But, it does seem like there might be some problem, possibly in her right ear, but the doctor saw some fluid, probably leftover from COVID or when her tonsils were clogging up her sinuses, which might have been causing the problem, but since since hasn't had chronic ear infections, she wasn't too concerned, though hoped to see her again post-transplant to see if there were any changes.
Had sushi after a day of appointments at the Dana Farber Cancer Institute.
We're staying overnight tonight. Had a lot of blood tests today and then a bunch of meetings with various providers to discuss next steps.
Audiology, dentist evaluations and an infusion with a bunch of timed blood draws to see how Grace's kidneys are doing over the course of the day.
And Faith is coming tomorrow for her blood test and psych evaluation and pre-op planning.
And then dinner with Heathers cousin tomorrow night and then we'll be home.
We are heading to Boston tomorrow and Thursday for a myriad of appointments. Faith will also have her donor preparation appointments.
I also discovered yesterday that a segment of our friends still had old dates. Because of our Christmas COVID, everything got postponed.
So if all goes as planned:
1/10-1/11 testing in Boston
1/19 CT scan at Dartmouth
1/22 pre-transplant surgery requiring anesthesia
1/30 admission to Boston, hard chemo begins
2/8 transplant (Faith requires anesthesia, Grace gets Faith's bone marrow through her central line.)
Then, step-by-step, day-by-day recovery.
Due to getting COVID, Grace's transplant date has been re-scheduled and we are currently scheduled to start the prepatory chemo (much stronger than the previous chemo she has received) on January 30th and then the bone marrow transplant a week later.
We expect to be in the hospital for at least a month and then staying near the hospital in an apartment for a month or two.
Jon's work and insurance has been really great. He has been told "work is optional" and to not stress about working, but just get done what he can. Insurance is expected to cover just about everything, including the hotel and apartments in Boston. There are also some other state-based insurance programs for people with severe disabilities and we are in the process of applying for those. There are also a lot of non-profit organizations who exist to support the families of those children with leukemia. There are fun blankets, special snap clothes that allow good access for medical procedures and stuffed animals to make for more cheery hospital visits.
The only known curative therapy for JMML is a bone marrow transplant, so we will be going down to the Boston Children's Hospital/Dana-Farber Cancer Institute. Their team is one of the leading cancer centers for treating JMML. We had our first visit on December 12, and are still processing what we learned. We have begun the process of getting everyone in the family tested as possible donors. When all is set for Grace’s transplant, Grace, Jon, and I will temporarily relocate to Boston.
The whole family got HLA typing tests and it turns out Faith is a perfect match (and Jon turns out to be an almost perfect match, which is quite interesting genetically.) We all got COVID over Christmas, so that canceled Christmas plans with the extended family and delayed Grace’s transplant process. (After a horrible day where communication lines got crossed and Jon drove Grace down to Boston only to have to turn right back again because her appointments were all canceled.)
Because of the delay, Grace got another round of azacytidine. Also, remdesivir for the COVID symptoms. Jon had to push to delay the aza a few days to avoid reducing her immune system while she was sick, but they did eventually listen to him. This time, he and Grace stayed up in a hotel near the hospital so he wouldn’t have to drive back and forth every day. Grace did great the first three days. They came home Sunday night and it was so nice to be together as a family again. Monday afternoon, they went back up and then they stayed at David’s House.
Grace and I (Jon) had a great time together for the most part - she did throw some unconsolable fits a few times so that was hard, both trying to keep her quieter to not disturb the other guests and also to figure out what she wanted - in many cases, I think she didn't really know, and the best solution seemed to be to fall asleep.
One sort of humorous note is that for a regular kid, when they are crying uncontrollably, they can't ask for anything at the same time, but Grace really loves sign language and has learned more than any of our other children. And while she is screaming she can still sign at the same time, and I think it has been harder for her to learn than our other children that she can't get what she wants while yelling, because I think that is somewhat obvious to a child who isn't able to communicate while screaming.
Tuesday morning, Grace was quite sad – I (Heather) think she didn’t like the idea of being away again. So I went up Wednesday to take her to her appointment while Jon stayed at David’s House to get some work done.
I had lunch with her at David’s House and then played and talked in the room for a while. Then she was happy to have Daddy take her to the play room and say goodbye to me. Though, unfortunately, when they got down to the playroom, there were others already there, so they didn't join them since we were getting over COVID. We had some miscommunication with the David's House staff, as they don't have a COVID policy published, and had given us some different information and forgot to ask us the screening questions when we arrived, so we were allowed to stay, but were told to isolate and they probably didn't really want us there, but didn't want to kick us out either.
Before Heather left, she noticed Grace was developing mucositis. She was so uncomfortable and it was so sad. They gave us an ointment and sent a prescription to the pharmacy. It did help by the next day which was quite a relief, to us and to her. We came home on Thursday and she has been much better since.
The pathology report indicated no parasites, and no bacterial infection, but confirmed the swollen lymph nodes. The doctor said, “I do not like the look of her paleness, and that spleen is really big. We need to see inside; go downstairs and get to imaging right away.” Grace had an x-ray and an ultrasound, confirming a much-enlarged spleen. At some point she had another blood test, which revealed severe anemia and a WBC of 55,000/mm3 (normal for a child of her age is 5,000-19,000).
We happened to also have her tonsillectomy pre-op appointment that afternoon, and as we were in the waiting room for that, Jon got the call from the GI: go to the Dartmouth-Hitchcock Children’s Hospital ER immediately. We continued with the pre-op appointment, but let him know that we might have to cancel the surgery depending on what happened at the hospital.
We drove all the way up to Lebanon from Manchester, meeting Jonathan partway to pick up an overnight bag, and Grace was admitted to the hospital. After more tests, they decided to give her a blood transfusion overnight. That was an adventure in itself, since they had difficulty putting in the IV (two failed attempts, even with a fancy video ultrasound to locate the veins, and after the successful infusion of two bags of blood, the third came detached and sprayed all over the room, an event that required not only extensive cleanup but reporting to the CDC! Eventually she received what she needed, and it made a great difference in her color.
The hematologist-oncologist interviewed us about Grace’s history. When I got to the part about the doctor not listening to me, she actually cried. “Always listen to the parents,” she said. “I’m so sorry that happened to you. Café au lait spots are the #1 indicator for NF1 (neurofibromatosis type 1).”
It would not be confirmed for a few days, but the doctors immediately suspected JMML (juvenile myelomonocytic leukemia). These are very rare conditions, caused by very rare genetic mutations; it is not an encouraging diagnosis.
On Friday, they put in a Broviac central line, which went super, super great. The first time they put her under anesthesia on Thursday (for a bone marrow biopsy) they had to try three times to get the breathing tube down her throat because of her swollen tonsils, so this time they went straight for a laryngoscope and that worked nicely. I was expecting her to be gone two to three hours, but she came back in an hour, everything having gone great. She woke up from the anesthesia well. She had one more blood transfusion after that. Her IV’s had been a problem; for some reason, they could put stuff into her, but they couldn't take blood out, so they had poked her all sorts of different places all over her arms. It was good to have the central line because then the rest of the labs were easy to draw.
Friday night she was officially diagnosed with JMML, so on Saturday she started chemo: one dose per day for seven days. This chemo is not curative but is intended for maintenance and to relieve symptoms. And it worked really well: after just a few doses her breathing and snoring became noticeably quieter, and she started sleeping a lot, including through the night. Her appetite also became ravenous; she had no obvious nausea from the chemo. The doctors said her body was attempting to make up for lost sleep and nutrition.
The hospital experience at Dartmouth was good. The nurses were great, the doctors were great, everybody was kind, nice, sympathetic, and careful. They even changed Grace’s diapers while she was having the chemo, since our baby must be protected in utero from those toxic chemicals. David's House, the facility where Jon and I alternated staying (and got some much-needed sleep) is a beautiful place. So peaceful, and they have thought of everything you might need before you think of it yourself.
The hospital food was not restaurant quality, but it was definitely closer to home quality and far from cafeteria food. It was so nice to be able to call up and order food for Grace and ourselves and have it delivered. They took good care of us there.
By Monday, Grace was doing really great and so we were sent home to do the rest of the chemo on an outpatient basis. That meant driving an hour each way, every day, and spending hours at the hospital, but we were so happy to have the family together again.
(this was written January 2024, referring to Grace's first two years of life)
When Grace was about six weeks old, right at the beginning of December 2021, I noticed that she had “café au lait spots.” I looked it up on the internet, and discovered that these spots are considered perfectly normal if there are fewer than six, but if there are more than six you should contact your doctor because it could be an indicator of something called neurofibromatosis.
I tried not to think too much about that at first. At about the same time, however, she started showing some blood in her stools. So I contacted our midwife, and she said, “Oh, that really looks like allergic colitis; you should come off dairy.” We were exclusively breastfeeding, so I eliminated milk from my diet, but it did not seem to help. At the time, I didn’t understand the difference between lactose intolerance and a milk protein allergy, so I didn't go off of all dairy; for example, I was still eating butter.
At some point before four months old, I had noticed that Grace’s lymph nodes were swollen, though at the time I didn’t know they were lymph nodes; I only knew that she had swellings around the back of her neck.
At Grace’s four-month doctor’s appointment, we were referred to a gastroenterologist for the bloody stool issue. When I mentioned the café au lait spots, the nurse practitioner said that she couldn’t have neurofibromatosis because if she did she would have a lot of obvious bruising. Neither was she concerned about the swollen lymph nodes. She mocked me for looking up stuff on the internet, and really didn't like the fact that we were not giving Grace any vaccinations. We are not generally against vaccines, but our reasoning was if she has unknown, multiple allergies—or something worse, like an autoimmune disease—we shouldn’t be stressing her system before knowing the true situation. I left that appointment as soon as I could and hoped never to see that lady again. It took me several months to get over the trauma and move forward, switching all the children to a different practitioner.
But back to April. We made an appointment with the gastroenterologist, and he was impressed with how healthy Grace was. He said, “That looks like classic allergic colitis; go off dairy; she should outgrow the problem at the age of a year or so.” He then explained what you need to do in the case of a milk protein allergy: eliminate all milk protein everywhere, which includes many things you’d never think of. Did you know that “natural flavors” on an ingredient label can hide milk proteins?
We also saw an allergist, who tested for a few things, but they all came back negative. Both the allergist and the gastroenterologist said that gastrointestinal allergies are not the same as the kind that provoke an anaphylactic reaction, so the testing doesn't really tell us anything useful. I had some online conversations with the gastroenterologist over the course of the summer as eliminating milk protein didn't solve the bloody stools problem. It did help with her eczema, and it did reduce the bloody stools some, but there was still something else going on.
So we tried going off beef and all other cow products; we tried going off soy protein; we tried eliminating many other things, but there was still sometimes blood in the stool.
At her first birthday, we tried giving dairy again, because the gastroenterologist thought she should have outgrown a milk protein allergy—most kids do. But that was a fail. Also, it seemed like peanuts triggered a more severe bleed, so we took peanuts out of my diet—and hers, as she was now eating regular food as well as nursing.
I had also noticed that it took Grace a long time to get over a cold. Whenever a normal cold would go through the family, it would take her three weeks to get over it. I mentioned this to the gastroenterologist on the online portal, and was told, “It's common for kids to get lots of colds; she'll be fine.” So at that point we continued to try to narrow down her problem foods, and waited for her to grow out of the condition.
At her next regular check-up, I brought up all Grace’s random symptoms that by themselves did not cause the doctors’ concern. It just seemed to me that, when put all together, it might indicate that something more serious was wrong, like an autoimmune problem.
We were then referred to a rheumatologist, but stalled at the point of getting an appointment, as the only pediatric rheumatologist in the state was not taking new patients. And the next two closest general rheumatologists were also not taking new patients.
So the next stop was an otolaryngologist (ENT), because Grace’s breathing was loud and her tonsils were very swollen. She was a great snorer and would have apnea-like episodes. The ENT said, “Those tonsils are huge; you know it's affecting her breathing. We should get those out.” An x-ray confirmed that her adenoids were also really swollen. I expressed my concern that this might be an autoimmune problem, and said that I hesitated to treat the symptom and miss the underlying cause. But he said those were “very rare.” I thought, “Well, what if she’s one of those rare kids?”
I was fed up with regular doctors at that point, and decided to see someone who is both an MD and a naturopath, and had been very helpful to a friend of ours. She ordered some blood tests, and when the results came in, she said, “Oh yes, her white blood cells are high (WBC); this is concerning. Her iron and some other measures are a bit low as well. She’s clearly fighting something. Let's put her on some anti-microbials and some immune-boosting nutrients and see if that helps.” She did some other allergy testing, which we knew was controversial—the medical community basically says it's no good, and the natural community is divided on its efficacy. That testing suggested still more dietary restrictions, so we decided to try both those and the supplements for a month. The doctor also offered to do stool testing to check for parasites, but we thought to try the diet first.
When her WBC came back high, leukemia came to mind, and we tried to assuage our fears. When Jon mentioned that growth would be hindered, I pointed out that she hadn’t been growing very much in the last year or so. I finally looked up her weight and height and discovered that she was in the 1st percentile for height and 7th for weight. This is so much smaller than our other babies, and though we didn’t have much data for her because of missing well child visits, it did seem like there had been a growth slow down at some point.
Soon after starting the restricted diet, I found out that I was pregnant. Grace seemed to react to my prenatal and iron supplements, so I decided to go ahead and wean her so that I could eat anything and thus take care of the baby's nutrients. I made a two-week goal so that it would be quick but not too quick. That was successful, though quite the emotional energy drain.
Repeat blood tests a month after the first showed Grace’s WBC improved (down by about half) but still high; other results were about the same or slightly improved. The naturopathic doctor said she wanted a hematological-oncologist to check out the blood-count numbers. In the middle of dealing with insurance to find one, Jon spoke to a pediatrician online who suggested we go back to the gastroenterologist. So we did. Because she was still having blood in her stools, he scheduled an endoscopy and a sigmoidoscopy and took a stool sample. When her stool sample came back with very high proteins, he changed the sigmoidoscopy to a full colonoscopy.
Colonoscopy prep is no fun even if you understand what’s going on, so that was a hard time, but she did better than I expected. The doctor said that the upper GI tract looked normal, but her colon was full of swollen lymph nodes. Did you know there are lymph nodes in the colon? We saw the images—it looked like a ball pit with tiny pink balls. He also took some biopsies and said the pathology results should be back in a week.
In the meantime, he and the anesthesiologist strongly urged us to get Grace’s tonsils out as soon as possible. She gave the anesthesiologist some scares when her swollen tonsils stopped her breathing a few times during the colonoscopy, we assume similar to what happened when she is sleeping. They scheduled the tonsillectomy for the Monday after the colonoscopy follow-up. (Then it got rescheduled for Wednesday.)
Grace had some bleeding and some black stools a little less than a week after the colonoscopy. Jon noticed a hard place in her belly, and that she was looking pale, so the doctor changed the planned video follow-up to an in-person visit.
Wednesday, November 29th, 2023, the day of that follow-up appointment, was the day our lives were upended. (click next below to continue reading)