italy

3 Months Post Gene Therapy

Today Keira began her 3-day 3-month post gene therapy check up. She had an EEG, bloodwork and saw the orthopedic surgeon. Tomorrow, December 29th, she gets an MRI, lumbar puncture and bone marrow aspirate. Then on December 31st, she has a neurological evaluation and physiotherapy evaluation.

We get some results as they arrive this week (we already know her hemoglobin levels are rising, which is great) but the most notable results won’t come until the beginning of February. That is when we will be able to get our first big picture of how her body is accepting the renewed genes.

In the meantime, the team at San Raffaele has asked us to send videos of her development (walking, talking, etc) so they can monitor her progress from afar prior to our return in April for her 6-month check up.

She already took her first step on Christmas Day and is beginning to talk more (saying hi, bye bye, waving ciao, etc), so we are not concerned. However, Olivia was the same way at this stage in life as well. It wasn’t until around 18 months that she began to have issues. So we will of course be nervous once that day rolls around but the results in February could put all those worries aside.

We have already said many goodbyes to the team at San Raffaele (who are true angels and miracle workers – I will share more about them soon) because days after Keira’s check up we leave for Amsterdam for Olivia’s final treatment there before heading home to Arizona on January 8th.

What a whirlwind this experience has been. This year has been. We will miss our new friends, and the many doctors and nurses, who have been by our side during our time here. But we are excited to be home and beyond hopeful for Keira’s future.

Heading Home Soon

It’s taken me a while to write this post – some VERY exciting news – not just because of lack of time while juggling the girls, work, the holidays, etc. But also because, for me, it’s bittersweet.

On January 8th we are heading home to Arizona!

It’s earlier than we expected based upon Keira’s rebirthday/gene therapy date (Oct 2nd, 2020) and how quickly her body has recovered from the chemotherapy. We could not be happier with how well she has done throughout this whole process!

At the end of December she will have her final hospital stay, which will be her 3-month post-gene therapy check up. During this stay, they will do a lumbar puncture, bone marrow aspirate, MRI and remove her central line. Results won’t come in for 4-6 weeks following but we remain so hopeful and positive given how she is continuing to develop. Fingers crossed!!!

The bittersweet part? For me, it’s a few things. Italy now has a special place in all of our hearts for the potentially life-saving treatment Keira just received, the team in Amsterdam that has been treating Olivia is absolutely amazing (she loves them and we will all miss them), but I also dread going back home and facing the reality of Livvy’s very limited future. I know that’s inevitable and we would face it no matter where in the world we are but going back home makes it feel one step closer. It will be time to enroll her in long term care, figure out how we may need to handicap the house for her future needs, schedule in-home PT, OT, etc. It’s a lot.

But as Dave continues to remind me, there are so many positives that will also come along with being home…back in our own house and our own beds, not having to worry about a time difference to talk to family or schedule work calls, Eva getting back to school and life as “normal” despite COVID, etc. It WILL be a good thing for all of us.

And from this point forward Italy and the Netherlands will both hold a special place in our hearts. ❤❤❤❤❤❤❤

Italian Cooking Lessons

With COVID-19 being an ongoing issue, we haven’t been able to get out and do much of anything during our time here in Italy. But we have been blessed to meet some truly wonderful people who have taught us a thing or two in the kitchen.

Laura came over one day to teach us how to make a proper Italian pizza. She and Silvio always make their own dough but because it takes 2-3 hours she did that ahead of time and brought it over. She said the bakeries here also premake it for you to purchase to save time.

Eva took everyone’s orders – she and Laura were making cheese pizza and olive pizza with capers – then started rolling out the dough. We always try to keep Livvy as involved as we can so I helped her roll some dough too.

Next steps were spreading the sauce, putting on the mozzarella and drizzling some olive oil.

After adding any toppings, the pizzas went in the oven and ta da! The perfect Italian pizzas were made! And they were delizioso!

Our next lesson came from Luciano, who taught us to make a great pesto sauce for any pasta.

Take some tomatoes (supplemented with some tomato sauce if they are not as ripe or in season), basil leaves, olive oil, cheese (we used parmigiano I believe along with a goat cheese), pine nuts and blend it all up in a food processor or with a hand mixer. And you’re done!

Luciano also gave Dave a great recipe for pasta with broccoli and another homemade sauce. It was so easy to make that Dave no longer wants to buy pasta sauce at the store. Super easy and even our toughest critic (aka pickiest eater) Eva liked it! 👌🏻

Last but certainly not least, tiramisu! When Roberto heard we really enjoyed that he surprised us by bringing over the ingredients one day so we could make it while Eva and his daughter Chiara played. Here’s the recipe from Dave’s notes:

Tiramisu

Espresso 200ml-ish
Put into bowl to cool

1/2 kg marscapone
Sugar
6 eggs
Savoiardi biscuits

Separate yolk and whites
Whip/froth whites
Put marscapone into mixing bowl
Fold in whites
Fold in yolks
Fold in sugar
Then whip again

Dust pan with Cacao zuccherato
Dunk biscuits (quickly) into coffee and layer pan.

Layer chocolate chips

Then layer marscapone whip

Then dust more cacao zuccherato

Switch biscuit direction for next layer and repeat.

Then let it chill in the fridge overnight. We can guarantee it is so good! And much easier to make than we would have thought. Hope you enjoy it as much as we did!

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!