Daley Ponderings

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 1^st percentile for height and 7^th 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)

We haven't been blogging much, just living life and having fun together.

But, it is sort of sad that Grace didn't even have a category for her until today.

We are working on more content and to make a central place for people to get information so we don't have to re-type it all the time to each person who asks.

But, I'll at least post some cute pictures for now.