Stuck in an Elevator

I cannot make this stuff up. Yesterday, I got stuck in our apartment elevator with Eva and Olivia. 😳

We were headed out to meet Eva’s friend Chiara when right around the 1st floor (we live on the 6th) the elevator made a big th-thunk noise as it shook and came to a stop. None of the elevator buttons would work. We were stuck.

And all I could do was laugh. 🤷‍♀️😆 What are the chances?? Apparently 1 in 100,000. So of course because we don’t have enough rare experiences in our life it was time for a new one.

With Dave out on a trip to the store my first call was to my Mom, who was still at the apartment with my Dad and Keira. She heard the “th-thunk” and the first thing she said was “Tell me you’re not stuck in the elevator.” After confirming we were indeed stuck in the elevator and asking her to call someone, my Dad flew down the stairs, made sure we were ok and got the front desk concierge. And Dave arrived from the store.

Meanwhile, I tried speaking via google translate to the Italian man that answered the elevator alarm call. I wasn’t sure if he understood me or not but he hung up.

Only a few minutes later Eva said she was bored. 😆 I was just thankful we made her use the restroom before we left and that Livvy wasn’t going to need meds anytime soon. In case we were stuck for a while we also had water with us and a few candy snacks we brought along for her and her friend.

Livvy didn’t understand what was going on so she was fine until she got tired of sitting in her stroller. So Eva and I started singing songs, like Itsy Bitsy Spider and Little Green Frog, to keep her smiling.

After some complaints from Eva, we heard a noise and saw light shine through a crack in the door. Someone was here to free us from the elevator!

After a few minutes they opened the door. We were indeed between floors. Because the opening on the bottom was bigger than the opening on the top we were going to have to get out that way.

I had told Eva she would get out first but she said “No! Livvy needs to go first because she is special and we need to take care of her.” 🥰 I reassured her they were both special and equally as important but because she was closest to the door it was ok for her to get out first.

At dinner last night Eva said “When will we get stuck in an elevator again? That was fun!” 😆🤦‍♀️

Yet again, another unexpected event happening in our lives. It doesn’t even surprise me at this point. Just another day of rolling with the punches with a smile on our faces!

A Little Bit About Eva

Most of the blog has been about our youngest girls, Olivia and Keira, as they are undoubtedly going through the most these days. But I would be remiss to not share more about our oldest daughter, Eva.

With all that our family is going through she is handling it incredibly well. While she may not understand everything yet, what she does know she processes, asks her questions, and moves right along; always rolling with the punches.

She is truly coming into her own as a big sister; talking with Livvy to cheer her up when she cries, including her in her activities, and being silly to make Keira laugh every chance she gets. She makes them both pictures with their names on them + “I ❤ U” and is already planning what she wants to get them for Christmas.

While school is different for everyone due to Coronavirus, it is a little more so for Eva. Her school has been absolutely amazing and so supportive as we navigate through these uncharted waters together. But because Milan is 8 hours ahead of Phoenix, Eva only gets to partake in her online Zoom session with the other kids at 4:15pm then it’s time for dinner. Because of that, we work on activities with her throughout the day and she has started taking Italian lessons twice a week (also on Zoom).

When she is not doing school projects she is always inventing and creating things of her own; zip lines for her stuffed animals, sleds out of Amazon boxes, jewelry for her sisters and decorations for the holidays. But her new favorite activity is riding her bike – on two wheels only! (Thanks to lessons from the self-proclaimed best teacher ever, Dad, and to Laura & Silvio who lent us this bike to use for her while we are here) “I’m so glad I got to learn to ride my bike in Italy before going back home to Arizona!” she said the other day.

She is growing in so many ways and we couldn’t be more proud of her! ❤

Seeing the Girls Together

It didn’t hit me until I saw it in front of me. All three of our girls sitting next to each other at the table (and Livvy doing so semi-independently). This was something I never knew I would get a chance to see. And it was amazing!

I never thought I would see this day because I never thought of Livvy being able to sit independently without one of us helping.

While her weekly treatments in Amsterdam may be helping her stabilize somewhat her disease is still progressing and she is still losing abilities. She can’t sit in a chair on her own because she can’t hold herself up. She can’t sit in her stroller to eat because she can’t bend her body at a 90-degree angle to sit in it comfortably. So every day, every meal, one of us has to hold her and feed her while we eat as well (or we take shifts eating).

But now that she has her customized stroller/chair (a Zippie Kids Xpress from Sunrise Medical), she is learning to get used to that and it has been a game changer for all of us.

To see the girls together and to know that there will be many more days of them having this time together (where Livvy can feel like a big girl sitting on her own), is priceless.

I was so in awe during the moment that I took only one picture. 😍🤦‍♀️❤

41 Days to Freedom

Yesterday, as my Mom and I were getting Livvy ready to head to the hospital and get fitted for her new customized stroller/chair, Dave video-called right as the doctors came in Keira’s room and said her neutrophils were up to 2,000 (from 200) and they could head home!

41 days in isolation, 4 days of chemotherapy, gene therapy treatment, daily blood draws, and a constant rotation of medicines had finally come to an end. We were beyond ecstatic!

While it was a bittersweet day with Livvy getting her new stroller (her body can no longer bend properly to be comfortable in a normal stroller), having a healthy Keira come home was truly a wonderful thing; a miracle really.

Eva ran up to us at full speed as we walked in the door and yelled “Hi Baby Girl!!!” to Keira.

From personal experience weeks earlier, I knew the joy and sense of freedom that comes with leaving the box and being at home. But now being on the opposite end and having Dave and Keira come home was…just an overwhelming sense of happiness and like everything was as right in the world as it could possibly be for us at that moment.

After bringing them home, my Mom and I walked to the store to get a few things for dinner. I had chills the entire walk. Not because it was cold but because of that feeling; our family was finally together again after 41 days apart.

Our lives are an emotional rollercoaster to say the least but we could not be happier to have everyone under the same roof as we head into the holidays (and a potential lockdown in Milan).

The girls were all so happy to be with each other again and so were we! ❤❤❤❤❤

Day 40 in Isolation

It is the 40th day Keira has been in isolation and in just a few short days she and Dave may be coming back home! 🤗

I am SO happy to share that she has only had one blood transfusion in the past week, her platelets seem to have engrafted as those levels are spiking, and her neutrophils are on their way up! The results from her first bone marrow aspirate following her gene therapy also appear to be in line with the results of other successful patients so we are beyond happy with that!

The doctors said we could expect to be discharged by the end of this week or beginning of next. This is how excited Dave and Keira are (and us too)! ❤

Halloween in Milan

Halloween for everyone was a little different than normal this year, but it was especially so for our family.

While kids do dress up here in Italy, there isn’t trick-or-treating or big parties like how people celebrate in America. And finding a pumpkin? Forget it. We did manage to find two at completely different stores though, one of which cost 27 Euros. 😳 Definitely the most we have ever paid for a pumpkin but worth it to make this seem as close to the normal holiday as possible for our girls.

Last Halloween, Eva had already decided on our group costume for this year: pirates! ☠ So we got everything ordered once we got to Italy. The 5 of us were pirates, my Mom was a pirate’s parrot and my Dad was Captain Hook’s croc.

To replace the absence of trick-or-treating we had to get creative. So I made a treasure map that would direct Eva to various X marks the spots around the apartment where she would find buried pirate treasure.

Livvy can’t participate too much but we carried her around for as long as she could go. She also hates getting changed (she has lost most of her arm control the past few months) so getting her in and out of a costume is a challenge and she did not want to be in it for very long since it was a hotter fabric. But we made sure she still got some smiles in, and enjoyed a little chocolate candy too.

Dave and Keira also got dressed up at the hospital, and the nurses and doctors loved it!

After the treasure hunt was over, Eva declared it the best Halloween ever! 🤗 And now, she is already working on our costume ideas for next year.

Happy Halloween from all of us to all of you! Hope it was a safe and happy one for everyone! 🎃💜☠

Day 32 in Isolation

Dave has officially settled in at the hospital and Keira is as happy as can be! She hasn’t needed a transfusion (for hemoglobin or platelets) in five days, which is amazing, and she is well on her way to a quick recovery – albeit with 50% less hair. We are so relieved she is doing well and back to eating and drinking on her own. Her weight has also stayed steady, at around 10.6 kg, this whole time, which was something I was concerned about when we started this process.

Today, the doctors said if her neutrophils rise as they should she may even get to come home next week! Yay!

Meanwhile, on the home front, we have had a busy few days with Eva and Livvy and I have loved it!

We ate lunch outside at one of our favorite restaurants, made origami hearts and Halloween decorations with Eva, played dress up with Livvy, went for a walk to see all the fall leaves and horses that live next door, completed a puzzle with Eva, walked down the street to wave at Dad and Keira in the hospital room, played at the playground, and danced in the rain!

Switching Out

I may have mentioned that when we first planned on Keira’s stay in the isolation room for 40+ days that Dave and I planned to switch out each week. Well, thanks to COVID-19, this was one of many things that had to change.

Thus, my staying for the first 4 weeks until Keira was past the mucositis phase, when her immune system would be at its weakest. Now that she is past that, recovering well and Dave passed his COVID test, we were able to switch.

Being outside, walking around without a time limit, felt so weird and so wonderful at the same time. That smile under mask didn’t fade the whole walk home, thinking about seeing my girls.

While I am having trouble even wrapping my brain around not seeing Keira in person for the next few weeks, I am beyond excited to be with Eva and Livvy, who I have missed SO much! Giving them hugs in person literally brought tears to my eyes today.

And I know Dave was so happy to see Keira. And vice versa, once she realized the masked man with the longer locks was her Dad. 😆

Never when we first heard the words Metachromatic Leukodystrophy would we have imagined our lives would lead us here. But we are so grateful it has. This journey has not been easy and we know the road ahead won’t be either. But these gorgeous faces make it all ok, and we will always do everything we can to keep them smiling.

Here’s to hoping these next few weeks fly by! We miss Dave and Keira already!

Day 26 in Isolation

It has been a whirlwind to say the least. Not just this entire journey but these past 26 days in isolation with Keira as well.

I was hoping to write an update sooner but hadn’t gotten to it solely because I’ve been exhausted. As my Aunt Deb says, you don’t go to the hospital to get good sleep. That’s for sure (at least for the caregiver anyway). With Keira needing to be on so many meds through her central line – specifically these past 10 days – there was always a machine beeping or a nurse coming in and out. Now that she’s starting to feel better the meds are more minimal and my nighttime naps span a little bit longer in length.

Keira is definitely back to her happy self. She’s playing, babbling non-stop and even starting to side step while she is standing on her play mats or in her bed. She’ll be cruising furniture – and walking – in no time!

As far as the science behind her recovery goes, here is the latest:

Thus far, she has had 3 blood transfusions, 2 platelet transfusions, her mucositis has subsided (and as such her neutrophils are rising), she is currently off of morphine for pain and is beginning to drink and eat again on her own.

Her hemoglobin levels have stayed on the higher end which is a great sign of engraftment and her Monocytes (a type of white blood cell) are also on the rise, which is another sign her body is processing things as it should. (I think I said all that correctly 😆). So far so good!

Now, we just continue to monitor as levels rise and around 30 days following her gene therapy (the first week of November) she will have a bone marrow aspirate to really look and see how the body has accepted the renewed and healthy stem cells.

It’s amazing we are at this point and we are so grateful to everyone who has helped us get here and get her this treatment. ❤🙏

But one bittersweet thing: this weekend Dave and I will switch out. Meaning my time in “the box” will be over and his will begin for the remainder of her stay. While I will not miss the hospital food or the broken sleep I will be so sad to be away from Keira these next few weeks. Dave needs some good one-on-one time with her though and I am in major need of time with Eva and Livvy (and my parents too)! I miss those girls and can’t wait to have cuddle time with them both!

We will continue to keep everyone updated as we venture forward. Halloween is coming up and we are determined to still make it a fun one (for Eva especially) since they don’t exactly celebrate it here like we do at home. 🎃

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!