Traveling with Special Needs

We’ve all heard the stories of parents traveling with children on airplanes and the issues that can arise while doing so. The crying, the yelling, the bathroom breaks, the snacks, the kicking of chairs, you name it.

I would take all of that any day over what we have had to deal with when flying with Livvy each week.

It’s not just her special needs but also the COVID rules which can cause issues with the airline.

Because she is 2 years old, she is required to have her own seat. However, she cannot support her upper body so we have to hold her upright in that seat (which she doesn’t like). So, instead, we hold her on our laps during take off, in-flight and landing. Some airlines are understanding but others not so much.

Also because of her age, she is required to wear a mask on some flights. This she not only doesn’t like but also can’t understand. So we usually let her eat/drink on the plane to avoid this issue.

Another problem is that while she is 2 years old and 3 feet tall, she cannot use the bathroom like a normal child her age. She can’t walk and has to wear diapers. We have actually been told by a flight attendant to take her to the airplane’s baby changing table in the lavatory (which she does not at all fit on). So we have to lay her down on the seat between us and speed-change her diaper.

Traveling with her (or taking care of her in general) also requires two adults. One that can carry her on/off the plane and another to carry our back packs, and open/close the stroller.

It’s not an easy trip. Especially if she is screaming in pain or crying out of frustration.

I have my elevator statement to flight attendants down to a science due to the amount of times they have chastised us for her in-flight care, or needs.

“She has a terminal illness that affects her brain and she can no longer talk, walk or support her upper body.”

I genuinely feel for every parent of a special needs child that has to fly with them and explain over and over again the issues they/we face. It sucks having to repeat that out loud so many times in front of your child who can still hear and understand most of what you are saying.

The travel agency who books our weekly flights for her clinical trial does notify the airline of our situation but we still have issues.

And to top it off, seeing all the children her age walking, talking and running around the airport is like a punch to the gut. That should be her.

One Year Ago

Thanks to Facebook, I was reminded that this was a snapshot of our “new normal” as a family of 5 exactly one year ago today. Three healthy girls, one of which may need some PT for walking. And no pandemic. Easy.

Then life happened.

1 move to Italy. 5 surgeries. 4 days of chemotherapy. 1 gene therapy treatment. 40 days in isolation. 30+ weekly flights. And countless hours of learning everything about MLD from fellow parents since the disease is so rare the internet grossly lacks anything remotely helpful to anyone affected by it.

Days in our life are often still unreal and I wonder if this all really just happened/is happening. And what could possibly be next?? Do I want to know?

For now, here’s what we do know regarding the girls’ progress:

Keira:

What a sassy, funny, wiggly girl she has turned into. She copies every word she hears, tries to run even though she hasn’t even mastered walking and says hi to literally every person.

Unfortunately, we just found out her body is still fighting the ARSA enzyme it is now creating because it never had it before (like it should have). This is not normal but it does happen and we have been reassured that the antibodies do go away and her body will adjust. But because of this result we will most likely be going back to Italy in April for her 6 month post-gene therapy check up and any accompanying procedure that may help her body adjust. We will share more as we know more there.

Olivia:

In each recent week of Olivia’s clinical trial it has been harder to pull spinal fluid from her internal port, yet still possible to insert the drug. Because this is not normal and it got to the point of not getting any spinal fluid out, she had to go under anesthesia (something that is known to progress the disease) to get x-rays and find out if there is a problem. There was. The catheter had a leak and must now be replaced.

Unfortunately, that meant she probably hadn’t received her full dose in the weeks prior and will now have to undergo another surgery to replace it. We can only hope that the weeks and infusions to follow help stall the disease as it should.

She continues to have hard days and we can only hope for a miracle. In the meantime, we are working on getting her a machine that will help her communicate using only her eyes! We are so excited about this and the opportunity to know exactly what she wants or needs rather than playing the guessing game.

Eva:

Since returning to school in-person her reading and writing have greatly improved and she is so happy to be around her friends in real life! We do miss having that extra time with her but so happy to see her developing in more ways than one. She brings so much light (and loudness) to this house of ours and her sisters absolutely adore her. As do we!

While it’s insane to think how the last year has changed all of our lives, it’s even harder to imagine how it could change in the year to come. But, we are remaining positive and hopeful and are doing everything we can for our girls. Keep the positive thoughts, prayers and virtual hugs coming! We appreciate them so much! ❤

Worrying & What Ifs

When we left Italy I assumed I would no longer need to worry constantly about Keira because she will (hopefully) be “fixed”; that my focus would shift solely on Livvy and making her as comfortable as possible as this disease continues to take her life. That was wrong. I feel as if I am even more concerned about all the girls than ever before.

For Keira, while it’s an absolute miracle she received gene therapy treatment, in a few months she will be approaching the age when symptoms began to show in Olivia. So I find myself surveying her every move to ensure I don’t see anything “off”, even though she is developing normally thus far.

For Livvy, it is as I expected. We know what her future holds yet each day can be a different symptom causing her pain. While we manage that, we are working on getting her long term care, discussing if “schooling” is even an option for her and continuing her weekly treatments.

For Eva, we answer any questions she has about her sisters as they arise. Things like “Why can’t Olivia get the treatment Keira got?” and “Why didn’t I get MLD?” My heart is already breaking on her behalf in having to lose her sister much too soon. I can only hope and pray that Keira’s treatment does its job and she has a long life ahead of her to make memories with Eva. Otherwise, I am already thinking about the therapy and/or survivors guilt she may deal with as a result.

As any parent of a child with a rare disease, it is hard to keep the “what ifs” at bay and not consider what the future holds but we do our best to stay strong for the girls and keep those faces smiling as much as possible.

Utah Bound

During the last part of our stay in Milan, we found out that the Utah site of Livvy’s clinical trial may be opening soon. Thankfully, it was in time for our return to the States and this week we made our first of many weekly trips to Salt Lake City, Utah for her infusion.

The team was very nice and understanding and the treatment went really well (no tears from Livvy!).

But one of the best parts was the travel was much easier on her (and the rest of the family). At one point during our weekly trips from Italy to Amsterdam Eva said to me “I don’t want you to go! It doesn’t feel like home when you, Dad and Livvy are away.” 😥

Because it is such a short flight (1 hour) from Phoenix we are working on making it a day trip in future weeks so that we have minimal time away from Eva and Keira. And both the PHX and SLC airports are much closer than they were in Milan so the drive time is even quicker (another plus for Livvy who does not like being in her carseat).

While the clinical trial is not a cure for MLD and is only meant to help stall the progression of the disease, we are very hopeful and grateful for the extra time it is allowing us to have with our Livvy.

Snuggles with Livvy as she naps before her treatment.

Home Sweet Home

On Friday, January 8th, we arrived home after 5 months in Italy. While we were essentially quarantining most of that time due to the pandemic in various stages and out of safety for the girls, each day was non-stop and it went by in a blur. As a client and friend of mine, Chris Rose, told me about being a work-from-home parent, “the days are long but the weeks are short.” 5 months seemed like an eternity at the beginning and now seems so minor.

Once we hit the halfway mark, I was actually dreading coming home. And I think I was the only one to feel that way. I felt like coming home meant while Keira would be saved it would be time to face Olivia’s very limited future and the decline to get there. Granted, this would happen no matter where we were in the world but, to me, our time in Italy felt like we were in a bubble, away from reality. And coming home meant popping that bubble.

The week before our flight my stomach was in knots. But I knew it would be good for every one of us to be home. Livvy was actually clearly excited, which can be a rare occurence. While she can’t talk to us she still understands everything so we told her we would be going home the following day and that she got a new big girl bed in her room. Her eyes lit up, she got a big smile and gave her little laugh. That night, she couldn’t even get to sleep like normal because she was so excited. And on the plane rides home she didn’t sleep a wink; eyes wide open and ready to be back.

Now, having been home a few days and readjusting after jet lag, I am beyond glad we are here. Eva has her room and her “poochie poos” (our dogs, Watson and Sherlock), Livvy has her new bed and her normal surroundings with better temperatures to be outside (she loves being outdoors), and Keira has her actual home, her own room and so many toys to re-explore since she may not remember much after spending half of her life in Italy.

What an unreal journey to say the least. But we are beyond grateful for this entire opportunity and the many people who have touched our lives to make it possible. We will definitely be leaving behind a piece of our hearts in Milan but are looking forward to figuring out whatever our new “normal” may be here at home.

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.

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!

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. 😍🤦‍♀️❤