Today, thanks to the wonderful souls over at Make A Wish Arizona, Livvy’s wish came true – after only requesting it a few short weeks ago – and she was so happy!
If you saw my previous post, you know it was a swing set for her to enjoy with her sisters. And while parts of it (like the sandbox) still need to be added in, the whole thing is up and was revealed to the girls today – they were ecstatic!
Every time we talked to Livvy about it prior she would get a big grin on her face so I think when she saw it today she was partially in awe; it’s actually here!
And for a girl who normally hates laying down (she will usually just start screaming) she is on Cloud 9 in her magic carpet swing!
To know that she and her sisters will have so many more memories made together with this swing set brings me to tears.
On May 11th, Dave, his Mom, Eva and Keira returned from a two week trip to Milan, Italy for Keira’s six month check up after the gene therapy she received in October 2020. I stayed home with Livvy since the travel, time changes, etc. would have been too hard on her. And let me tell you those two weeks felt like two months.
While Livvy did seem to regress a bit while they were away, I’m very happy to report that all of Keira’s tests went so well! It’s a bittersweet balance with these two. As one regresses the other progresses. It is truly a miracle that Livvy was able to be Keira’s guardian angel and we got her diagnosis in time to get Keira treated.
And what a treatment it was! We will forever be grateful to the team at Ospedale San Raffaele in Milan for their hard work, dedication, compassion and kindness.
Keira went through quite a few tests in the two weeks they were there (see my previous post for a list) and everything is, so far, coming back within normal range!
The doctors said that based on her current development they would put her in the same realm as the children who have had the best results thus far! These children are now 5, 6, 8, and 10 (at the oldest) and are living completely normal lives – walking, talking, playing sports, going to school! Things Livvy will never get a chance to do but because of gene therapy Keira will! I can’t even put into words the relief we felt.
While we are still waiting on one major test – which will show whether her body is still creating antibodies to the ARSA enzyme it now creates (and never used to) – we are so pleased, to say the least, and so hopeful for her continued development.
She is saying two and three-word phrases now, copying everything we say, picking up on things so quickly, walking so much better, and at 16 months old is already showing interest in potty training (which our speech language pathologist was blown away by). It truly warms my heart to know we have years and years ahead of her where she will be doing more and more each day. Yet at the same time breaks my heart to know it was all taken away from Livvy. As always, bittersweet. But every day and every smile with our girls means the world.
Since getting Olivia’s diagnosis, I have heard other MLD families talk about Make a Wish. I was familiar with the organization from being on the committee for their annual Wish Ball one year, which helps raise vital funds to grant wishes for all of the kiddos. Never once did I think my kiddo would be one of them.
But here we are. So, naturally, I started thinking about what Olivia’s wish would be since she can’t tell me herself. Most of the families seem to do trips somewhere but with how much we have traveled in the past year alone I didn’t think that would be at the top of her wish list. Not to mention I thought it would just make the destination this sad place where we once went for this sad purpose. And would we ever go back there?
So as I brainstormed other options over the last few months, we were also in the midst of looking for a swing set and playhouse for the girls. But how could we make it more accessible for Livvy?
Then it hit me. That could be her wish!Her two most favorite things in the world (aside from Mom and Dad of course) are “playing” with her sisters and being outside to watch the birds and planes go by. So I asked her, do you want a special swing set just for you and your sissies? She got the biggest smile and laughed. That was it!
Dave wasn’t at home when I got this epiphany so I immediately texted him and he loved the idea.
Thankfully, one of my friends and mentors was on the board for Make a Wish of Arizona so I asked if she would be so kind as to make an introduction. She did, and we got a referral from Livvy’s neurologist, and within a week we were meeting with the wish granters to discuss Livvy’s wish.
They asked all about Livvy’s favorite things (sisters and outdoors aside). To which I answered birds (the theme of her upcoming 3rd birthday), dogs, Minnie Mouse, and her fave bands, Maroon 5 and The Beatles.
Within two days Make a Wish had sent her a little bird house with stuffed animal birds that tweet (she and Keira both love it), and a Minnie Mouse with a dog on a leash. She was so excited! While she can’t make them move herself, the Minnie actually walks the dog and talks (which actually scares Keira 😆) and we help her out with the birds.
We are so grateful to have Make a Wish be a part of her life and bring even more smiles to her face. Within the next few weeks her wish of a play set will be granted and we’ll be sure to share pics!
I have never met author Maria Kefalas in person. Yet she helped to save our daughter Keira’s life. Within 24 hours of receiving Keira’s diagnosis on June 19, 2020, Maria informed us we had a chance to save her life with gene therapy, connected me with the team of doctors in Italy and sent us a $2,000 check to kick off our fundraising to get there. I will forever be grateful to her and the work she does through CureMLD.com and the Calliope Joy Foundation (named in her daughter’s honor).
Of course, I could identify with much of what her story entails as one of her daughters also has MLD. But I had no idea how instrumental she and her family truly were in developing so much research, attention and care for those within the MLD community. I cried, I laughed and was even surprised at some of the likenesses between our lives.
It’s nearly impossible to make sense of your life once your child is diagnosed with a terminal illness for which there is no cure. What is the meaning of this? Of life? And why would such a darling, innocent soul be faced with such a death sentence?
“Sometimes I wondered if I was supposed to have Cal in my life to learn a different way of looking at the world, to see the perfection of her divine love, gifts that do not require words and which must be condensed into such a short life…The fact that she [Cal] is so joyful despite this terrible disease was in so small part because Pat and I were her parents, he believed, and we shouldn’t underestimate how much our love saved her.”
I agree completely with Maria and Pat’s assessment to make sense of all this, and feel the same way about our Livvy. She is smiling more in the past week alone than she ever has before. How could so much joy be radiating from a child with her fate? Because despite the pain and suffering she is truly happy; she has us.
Another hard thing for parents to do when it comes to this disease is consider what terrible fate the future holds for their child and looking back at all they could accomplish just a year, a month, or even a week ago. This disease strips them of their abilities that quickly.
“In the week’s after Cal’s diagnosis I realized how dangerous these visits to the past can be. You can lose yourself in the grief over what’s been lost…The same thing can be said about contemplating the future; you can become immobilized with fear if you dwell too much on the ending of your child’s story. Living with Cal called us to live with no sense of the past and no thought to the future. Even as the disease stole more and more from her, we had to train ourselves to be grateful for what was possible each day.”
For us, it’s Livvy’s smile and her laugh. If we see those even just once a day it’s a win. Looking back at the memories thrown at us via Facebook feels like a punch in the gut. It’s devastatingly sad to think about the future that was stolen from her by this horrible disease.
So how do you take this fear of your child’s future, your future, and turn it into something positive? Cal (short for Calliope) taught Maria just that:
“Cal has taught me that when the worst possible thing happens, you have nothing left to fear…She has taught me that when you have tamed your fear, you harness its power to do extraordinary things. “
And the work Maria and her family have done to help others inflicted with their same pain, and grief, is nothing short of extraordinary.
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.
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.
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. ❤❤❤❤❤❤❤
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!
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. 😊❤🎂
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.
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. ❤