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.

One Last Hospital Stay for 2020

Just so we could really round out 2020, Olivia and I are currently going on our 2nd night in the hospital at San Raffaele.

The other day around 2am she started to get a fever and it kept coming back. The following morning (yesterday, the 29th) it was accompanied by some strange eye movements. So after a call to the pediatrician on call in we went.

While awaiting a urine sample (because we suspected it to be a UTI), they took a blood test which confirmed her CRPs were up significantly (a c-reactive protein test measures the level of c-reactive protein (CRP) in your blood. It’s sent into your bloodstream in response to inflammation).

We were then admitted and taken to get her an EEG because of her eye movements that morning (she kept blinking and looking around and it was as if she had trouble keeping her eyes open). Keira had an EEG before as part of her gene therapy treatment so I was prepared – they put a little cap over your head with nodes in it that have gel, and the wires are connected to the machine they use. Results, thankfully, came back normal. Her eye movements were determined to be due to her very high fever (nearly 105) and we were instructed to call immediately and video it if it happens again.

Since then, Olivia has been on antibiotics via IV to take care of the infection and Ibuprofen/Tylenol to handle the fever.

Today, they also did an ultrasound of her kidneys to make sure everything was functioning properly.

After more antibiotics today and a blood test tomorrow we will hopefully be discharged so we can ring in 2021 with the rest of the family and not here at the hospital.

What a year it has been! Never a dull moment. Full of surprises. But also full of hope.

Here’s to a much better year in 2021! 🎆

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.

What is Normal?

It is 2 a.m. on Monday, December 28, 2020 and I am currently watching the snow fall through the glass doors of our balcony here in Milan while Keira falls back asleep on top of me in our bed. Meanwhile, Livvy also just woke up and Dave just went to readjust her (because she can no longer move her own body she sometimes gets stuck in an uncomfortable position and will need help to move and go back to sleep). Meanwhile, Eva sleeps through everything (thankfully) and my parents usually do too. This is our “normal”.

As someone who writes for part of my living I develop relationships with certain words and normal is one I now struggle with. There really is no normalcy in our lives anymore. And what’s considered our new normal may be understandable to the only 30+ other families in the world who have gone through this themselves but is probably unbelieveable to many others. It’s honestly still unbelieveable to me.

If a psychic were to have told me at the beginning of the year what 2020 would hold for me and our family I would have thought they were the worst psychic ever because there is no way that could happen. But here we are. Living in Milan, Italy. Amidst a global pandemic. Two of our three daughters have terminal illnesses. One may only have a few years left while the other just received a potentially life saving treatment. And snow is falling from the sky (a rare site to see for this Arizona family).

Life is unreal and so unpredictable. There is no way of telling what our next “normal” way of life will be. But for now, I’m enjoying the snow and the baby snuggles.

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!

The Riley's Road to Italy

Author: rileysroad