Mom Needs a Nap

Keira and I have been in the hospital for 20 days now.

Originally, the plan was for Dave and I to switch each week or two. However, because of COVID-19 concerns and him flying to Amsterdam each week with Livvy, they wanted me to stay until Keira was past her mucositis phase, when her immune system would be at its lowest. Fair enough.

The sleep (or lack there of) schedule has been rough. And as mucositis kicked in Friday night (Oct 9th) even more so.

As all the side effects began she needed more medicines to keep her out of pain and more snuggles with Mom. While some nights she was able to sleep well (thanks Morphine), I, however, had to get up with every beep of the machines, which meant one medicine was done and it was time for the next. Time to get up, pause the noise on the machine and hit the nurses button. This happens hourly throughout the night.

Usually by the time they take her daily blood draw at 6am she can go back down until 9am so that gives me a good 3-hour block of time to sleep more and sometimes hurry up and shower while she sleeps.

Needless to say, Mom needs a nap. Or a spa day. Or both.

Once the mucositis started on Friday, so did the hair loss. But only a couple hairs at a time. Now it’s becoming a little more but nothing extremely noticeable when you look at her.

Two days later, on the 11th, she needed a blood transfusion because her hemoglobin levels were low, on the evening of the 12th she needed a platelet transfusion, on the 13th she got a immunoglobin infusion, on the 14th, she needed another blood transfusion and today, the 15th, she needs another platelet transfusion.

This is all normal and par for the course at this stage in the game. They also took an xray for pneumonia (which came back negative) and did an ultrasound of her liver (which looked great).

And I was beyond happy to see her acting more like her normal self for the first time yesterday. Smiling away and even playing! 🤗❤ It made our day and so far she is feeling good today as well.

Can’t wait to see our strong girl push through this phase and be on the road to recovery and a normal life without all the struggles and pain that comes with MLD. Truly a blessing and a miracle!

Chemo Side Effects for Keira

I’m sure I’ll update this post a few times (scroll to the bottom), as I know more side effects will be coming. But over the past few days the chemotherapy side effects began for Keira.

First, was the loss of appetite. She started veering away from solid foods and only wanted milk.

Then it was diarrhea. Ugh.

And while I learned that chemotherapy does not effect children with as much nausea and vomiting as adults because their skulls aren’t yet fully formed so there is less pressure building up in the brain, the vomiting began nonetheless. It only happened once on Monday, twice on Tuesday but on Wednesday…woah. I went through 8 outfits for her, 2 for me and lost count of how many times she threw up (at its worst it was 6x in one hour). She couldn’t keep milk or her meds down. Finally they gave her anti-nausea medication and it helped SO much. She was fine after that and they have given her that each day now.

I know mucositis can worsen (which is what is partially causing loss of appetite) but sores can begin to form in her mouth that cause pain. This generally happens when neutrophils (the most common type of white blood cell) hit zero. She was at 600 yesterday but down to 200 today so they think in the next day or two we can expect to hit zero and will probably need to feed her through the IV.

And, of course, the most well-known side effect of hair loss is yet to come. I hope it doesn’t but she has so little hair now that I’m sure it’ll grow back in no time.

Weight loss can also sometimes be an issue but despite all the puking she remains stable. When we first arrived to Italy in August she weighed 9.99kg and got up to 10.76 kg before this extended hospital stay. In the past week she has gone down to 10.1kg (then yoyoed throughout the week) and is up to 10.91kg as of yesterday.

And all of that aside, she continues to be in great spirits! The puking doesn’t even bother her and she goes right back to smiling and playing. Such a trooper! We are so grateful she is such a strong baby girl!

I’ll continue to update this post as things change but so far not bad!

UPDATE 11/10/2020: Keira has had a rough few days. The mucositis worsened causing some bad pain in her throat and she is still unable to eat or drink. Even swallowing saliva hurts her. So she is on pain meds for that and is getting calories added to her electrolytes to ensure she gets the proper nutrients. She also continued puking and gagging so she is on anti-nausea meds as well.

Her hemoglobin levels dropped within the past day so she needed a blood transfusion. And this was also the first day her hair began to fall out. 🥺 She doesn’t have too much to begin with though so hopefully it will all come back quickly.

The pain in her throat seems to be the worst part. And she is not even interested in playing and just wants to be held by Mom every waking moment (which is fine by me)! While her constant smiles are now few and far between the nurses and I still manage to get a few out of her each day. 😊

Chemotherapy & Blood Transfusion

We are on day three of four days of chemotherapy and Keira is doing great! Still eating well, sleeping as normally as she can between blood draws, has great color and is being her usual happy self!

Yesterday was a MUCH better day in thanks to a nurse named Marino who came in and got an IV placed in Keira’s foot in 5 minutes by herself! She’s my new hero after the IV debacle the first day of chemo.

They say Keira could start losing her appetite this week (so far so good though) and that the side effects of chemo (mucositis, hair loss, vomiting, etc.) won’t not occur until day 10 or after.

They’ve been leaving us to ourselves most days aside from nurses bringing in medicine, taking vitals and the physiotherapists coming in to “play” with her once a day. It’s not until the evening, after chemotherapy, that they need blood draws every two hours (in order to determine the next dose of Busulfan needed). Since tomorrow is the last day of chemo, tonight is our last night of blood draws (then hopefully we can get some sleep)!

One new thing on the schedule today was a blood transfusion, which they say is normal. Because of the continued blood draws during chemo they need to raise hemoglobin levels and this is how they do that.

Reading books while getting her blood transfusion.

Many have been surprised that chemotherapy is part of gene therapy at all but the reason is they need to weaken the immune system and make room in the bone marrow for the renewed stem cells with the corrected genes and ARSA enzymes which will be administered via IV through a virus (with the bad parts of said virus removed) in the gene therapy treatment on Friday. Once the new cells go into her bloodstream they will travel to the bone marrow and begin making new red and white blood cells. From there they monitor her closely as levels begin to increase and then she will hopefully live a long and happy life thereafter!

It’s honestly amazing this is even possible, and we are clearly so glad it is. The entire process is fascinating. I’ve always thought if I could choose another profession in another life that being an archeologist would be fun but now it may be a doctor doing this; not only because it’s a truly mind-blowing process but more so to be able to change the lives of so many children and their families like they are doing for us. Our world is changed for the better and we are so grateful.

Up next: Keira’s “rebirthday” on Friday, October 2nd! ❤

Chemotherapy for Keira

Well. This day did not go as I expected.

I was nervous for chemotherapy to start mainly because of the upcoming side effects but really didn’t know what chemotherapy itself entailed. And I ended up finding it, thankfully, very anticlimactic. They put the syrgine of Busulfan in the machine and in it went to her IV over the course of 3 hours. She didn’t notice a thing and that was that. Major relief!

The blood draws to follow, however, were a completely different story.

We have now been through SEVEN failed attempts to place an IV in one of her arms.

First, let me share the point of this. While they could easily draw blood from her central line, since that is where they had to run the Busulfan through they needed another vein or entry point to draw blood for the following regular checks to ensure the Busulfan was doing what it was supposed to in her system. Pulling it from the same line the Busulfan went in wouldn’t provide accurate numbers.

So, a team of 3 nurses came in to place the IV in her arm. On the third attempt by the same nurse she got it but then looked away for a second and let Keira’s arm go; allowing her to bend her arm WITH the IV needle in it. My jaw dropped. I wanted to faint. After getting the bleeding to stop she immediately tried again to no avail. I was furious. They could barely speak English and all left the room without saying a word.

One of them came back with a new nurse a few minutes later, letting me know they would need to try again. To which I was able to explain it would NOT be done by the same nurse who did the first 4 attempts. They agreed that was fine.

Attempt number five: three people from that team (minus the initial nurse) all made an attempt (with the same needle). No dice. I’d had enough.

Attempt number six: I, of course, had just gotten her to sleep after her traumatizing evening when the night crew came in to do the 9pm draw. I will preface this by saying I really like the night crew because Keira was so taken by them on our first night here. While they were able to get the blood they needed the line wouldn’t stay.

Because additional draws were needed at 11pm and 1am they would be back again for the next attempts.

So here Keira and I await them while she plays, laughing in her crib. THAT sound, and the end result of all this, makes the rest fade away. On to the next day…