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.

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. ❤❤❤❤❤❤❤

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!

Ready for a Rebirthday

Yesterday it hit me. The enormity of what Keira is about to go through tomorrow. And how it wouldn’t have been possible without Livvy going through this first. She truly is the hero of Keira’s story. If only hers could have a different ending…we can only hope.

I usually power through each day, not focusing on the surgeries or the tests or the bloodwork and the medicines but the end result; Keira one day running, talking, going to school, being sassy to her parents, having pool parties in the summer and stealing her sister’s clothes. It is always eye on the prize.

However, I am usually too busy in the moment to really think about it. But last night it snuck up on me.

Keira is one of only 31 children in the world to receive this treatment. The process by which they make this happen is incredible. The fact that it’s even possible, and that it really works? Even more so.

While there are no guarantees, we have SO much hope based on everything we have seen.

At first I thought calling her gene therapy day her “rebirthday” was a little silly but it’s just accurate. Friday, October 2, 2020 will be the start of her new life. The life she should have had originally. The life that will promise all the things we normally take for granted every second of every day. The life she will live for not only herself but for Livvy as well.

I’ve said it many times before but every day of our lives since hearing Livvy’s diagnosis initially has been unreal (and it got even more unreal from that point forward) but this day may take the cake. I guess we’ll make it a “rebirthday” cake. 😊❤🎂

Keira done with chemo and still happy as a clam! 😁

Gene Therapy Day 1

Since we arrived in Italy it was like a weight had been lifted off my shoulders. We made it. Keira would have a chance at a normal life and all will be well.

A few days ago, however, I was a nervous wreck and my stomach was in knots. Livvy hadn’t been doing well and Keira’s admittance into the hospital was just around the corner.

There was A LOT to do to prepare for this extended hospital stay, where we would be in an isolated, sterile room for 40-60 days.

A panorama of the room. The little couch pulls out into a bed for whichever one of us is here and there is also a big closet (not shown) to keep our things.

All clothes brought into the room needed to be washed with special detergent and put into ziploc bags to prevent germs and infections getting in. All clothes also needed to be changed every 24 hours and not used again until rewashed which means for one week you needed 7 pants, 7 shirts, 7 pairs of underwear, socks, etc. We needed to buy padlocks for the lockers in the common area, shoes that were able to be washed that we could ONLY wear in the isolation area, face masks, unopened items like toothpaste, toothbrushes, handsoap, etc. And the only food/drink that could be brought in had to be pre-sealed. Anything opened must be thrown out within 24 hours even if it keeps just fine in the fridge provided. And that’s not even all of the guidelines.

After getting everything ready to go this morning, my parents, Dave and the girls walked us to the hospital. A surreal moment, knowing what was ahead.

We said our goodbyes, gave hugs and kisses and got checked in.

While I forgot one or two things that Dave will have to bring for us, I’d say we did pretty well.

Now, we are settled in and officially starting Keira’s gene therapy treatment!

The first day was easy. They did bloodwork, cleaned and redid the bandages surrounding her central line, and gave her a few medications, along with liquid through the IV that goes to the central line.

Every time she drinks milk we also have to use a swab dipped in a specific formula, followed by a droplet of another liquid, to clean her mouth and prevent any mucous build up or infection.

Tomorrow, we start 4 days of chemotherapy. This is in order to breakdown her immune system and make room for the new genes that will be given to her on Friday (other MLD families call the gene therapy day their “rebirthday” since this is essentially the start of their new life without the terrible symptoms of MLD).

While we are beyond excited for that day, chemotherapy is scary. Especially for the Mom of an 8 month old. The usual side effects that accompany chemotherapy can also effect her (hair loss, mucositis, etc). And from what we hear from other families, the mucositis is the worst of them, as the pain in their mouths may prevent them from eating normally for up to two weeks. After that, it sounds like they bounce back rather quickly.

While we know the “plan” for these 40-60 days, I’m sure we will have our ups and downs. But as I told my friends, “eyes on the prize”. In this case we have to focus on the destination more than the journey. We have seen the results that this treatment provides for other children and are beyond grateful to be able to give this chance to Keira. ❤

Thank You is an Understatement

On June 19, 2020 our world was shaken a second time when we found out our youngest daughter Keira also had the same gene mutations that caused MLD. Thanks to being involved in the MLD Foundation‘s Family Support Group, we met Maria Kefalas of CureMLD.com who immediately informed us there was another option for Keira – in the form of gene therapy in Milan, Italy.

The potential cost? Hundreds of thousands. On top of moving our family to Italy for 5 months for this potentially life saving treatment. Another hurdle? Having our middle daughter Olivia’s weekly treatments in her clinical trial moved to a European site. The road head seemed daunting to say the least.

My sister-in-law Nina immediately created a GoFundMe page and another in-law, Aunt Malinda, introduced us to Jennifer Armer of the Armer Foundation, who could help us raise funds via tax deductible donations. Then off we went. Spreading the word far and wide of our family’s story.

Suddenly, not one or ten or one hundred but THOUSANDS of people – family, friends and strangers alike – came together amidst one of the hardest times in today’s world, to support our family. My colleagues also reached out to their networks, having media outlets from the Independent Newspapers, Mom.com and FOX 10 to 3TV and People Magazine sharing our family’s tragic story.

“Thank You” does not do justice to the appreciation we have for each and every one of you.

“Thank You” is an understatement.

I wish I could list every single person who helped us here. But that would make this post quite lengthy and many wished to remain anonymous. So to sum it:

To our family: Many of you we haven’t seen in so long because of the pandemic. A few of you have been right by our side, day-in and day-out, as we navigate the world of MLD with Olivia and Keira. No matter the circumstances, we would not be able to care for our girls or make this journey to Italy without your support. You have provided life to our children, love for our children, happiness for our children and now…a second chance for our Keira. A second chance at life, love and a normal future. We love you so much and can’t wait for the many memories to come with our girls!

To our Friends: You have rallied like we never expected. From bass fishing tournaments in Yuma and t-shirt fundraisers online to proceeds of wine sales, a “shoulder” to lean on or someone to share feelings with…we are speechless at the kindness, generosity and loyalty that comes from being part of your circle. Thank you!!

To Those We Have Never Met: You have left us in awe and restored our faith in humankind. KIND being the key word. The smallest donations bring us to tears as much as the large donations. We are all struggling during this pandemic and the fact that many still choose to help and give back means the world. It may very well save the life of our daughter and we are forever grateful.

Donations aside, the LOVE, support, positivity, prayers and virtual hugs sent our way mean the world. Thank you is truly an understatement and we want you to know how grateful we are for each and every one of you.

With love and endless thanks,

The Riley Family