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.
With ten perfect fingers and ten perfect toes. A tiny smile and button noes.
A brain that works as mine and yours. Tests coming back with perfect scores.
A heart that beats strong—the rhythm of drums. And the in and out breath of healthy lungs.
But then you find out it won’t be so. An unhealthy child— so much unknown.
A journey full of winding roads. Ups and downs. Such highs and lows.
A little one fighting for their life— And you, strong beside them in perfect stride.
Just pushing them forward with all that you are— a mother who hasn’t backed down thus far.
This motherhood it will indeed be hard. The heartbreak will leave inevitable scars.
Not a motherhood you had ever planned for— But it won’t be less. It will be more.
More love needed and more to give. An understanding compassion that is so so big.
More strength than you had ever known. A faith in God and Him alone.
You’ll learn to hope beyond all reason. And lay down burdens in every season.
You’ll fight and give up and fight some more. You won’t be stopped by seemingly closed doors.
You’ll give more than you knew you could. And though you’ll grow weary you’ll still see the good.
Yes this child— unhealthy as they may be. This child has allowed you so much to see.
Joy and beauty. Pain and sorrow. A gratitude for every single tomorrow.
This child is adored— a gift from above. A newfound passion full of motherhood love.
So this is to the mother of an unhealthy child. Who holds up her head, moves forward and smiles.
Your motherhood was not the way that you planned. But today you love more— and stronger you stand.
I’m not sure who wrote this but it was shared with me by another MLD Mom. And it is so true. To all my other extra special Moms, a very Happy Mother’s Day to you! ❤
I don’t like posting about the hard days. They are hard enough as it is. Why rehash it for everyone else?
What I like to see on social media is the GOOD. There is enough bad in the world and on the news every day. And while we have been dealt plenty of bad in life, there are ALWAYS good moments and that is what I like to share. The positivity. The smiles. The laughs. It’s what keeps us all going each day.
In the nearly 48 hours since Dave and the girls have been gone I can’t even tell you the amount of times Livvy has cried. Sometimes it’s pain related or because she has food stuck on the roof of her mouth or because she needs to burp but mainly it was because I needed to get something done so she had to be held or strolled around by someone else. The moment she’s back in my arms? It’s this 👇🏼
Will it be a long two weeks if she only wants to be held by me and cry with everyone else? Yes!
But I have to always remember these moments. The smiles, and the laughs and the fact that we have no idea how much time we have left with this sweet girl. Every day counts. Every moment counts. Every smile counts.
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!
Schooling was not something we considered would be possible for Livvy given all of her limitations. However, our coordinator with the Arizona Early Intervention Program urged us to look into it and apply so that she is in the system whether we decide to move forward or not. I figured how hard could it be to apply her to school?
Well, it has been a process.
After a zoom call with someone from her school district, an in-person evaluation was planned for a month later. Of course this fell the week after her surgery in Utah (that caused the CSF leak she had) so she was not in the best of moods but I had canceled once before and just wanted to get it over with.
Assuming we would be meeting with that same person, we walked into the room and were surprised to see 6 additional people sitting in chairs bordering the room, each spaced 6 feet apart and wearing their face masks. Woah. This truly caught me off guard and left a bad taste in my mouth upon leaving. They grilled us with questions about her abilities (or lack there of), one question after another.
Can she sit upright on her own? No.
Can she crawl or roll? No.
Can she feed herself? No.
Can she point to what she wants? No.
The list goes on and on. It was essentially an ugly reminder for us of everything she has lost from this terrible disease – all just within the past year.
While they understandably needed to get to know her if she would be in a classroom, it was a rough hour.
Weeks later we had a zoom call with everyone that was at the in-person meeting to determine eligibility together (insert eye roll here).
At this zoom meeting they proceeded to tell us that she has severe delays in each area. Please tell us something we don’t already know, I thought. But that she does in fact meet eligibility for schooling in their special needs program.
“Would you like to know what this looks like?”, they asked? Why not, Dave and I thought. They then proceeded to tell us that if she starts in the fall, it would be a three-hour day beginning at 9am where she would have both time in her stroller and being held by a teacher for more interactive “play” with other students. Snack time would include food brought from home. There would be one teacher and two assistants in the classroom who would all learn her medical needs to care for her properly and keep her comfortable.
Prior to all this, Livvy was in so much pain each day, and so irritable, that we thought there was no way she would be able to attend “school.” But since her last surgery which removed the internal port she has been so much more comfortable, and happy! While it pains me to think that thing caused her unnecessary pain this whole time, now that it’s out and she’s more stable I could potentially see this as being an option for her. Being around her sisters makes her so happy that I think being around other kids would make her just as happy.
So, I asked Livvy if she wanted to go to school with other kids and she smiled the biggest smile and laughed. So that takes care of that! We are now beginning the process to enroll her at the school down the street from our house and come August will see how she is doing and if she is still up for a change of scenery. 😊
On June 5, 2020 Olivia had surgery to implant the intrathecal port that would be used for the weekly infusions in her clinical trial. The trial itself was aimed at stalling the progression of her disease, Metachromatic Leukodystrophy (MLD). It required weekly infusions for 2 years, with an optional 3rd year.
After her initial surgery in Iowa
We enrolled for a few reasons. Mainly because it was the only option in the world for symptomatic MLD kiddos but also because after doing our research other parents in the study seemed very pleased.
The only site in the US that was taking patients amidst the pandemic was in Iowa. So off we went every week from Arizona to Iowa. Not an easy trek for Livvy but hopefully one that would be worth it.
Once Keira was diagnosed on June 19th though, we were soon on a different path and that lead to Italy. So part of the coordination there was getting Olivia moved to a European site for her weekly infusions. And that site was Amsterdam, which will now forever have a special place in my heart (but I will share more on that in another post one day). It was a much quicker trip than AZ to IA so we were pleased with that.
Talking a walk through Amsterdam with Livvy
After moving back to the States in January 2021, the Utah site for the clinical trial had opened. It was much closer to home so we were excited about that change.
Unfortunately, Olivia’s internal port had stopped working at that point and x-rays showed the catheter had a leak so she would need surgery to replace it.
The x-ray of her catheter leaking internally
Surgery is never an option you want for your MLD child. It requires anesthesia, which we now know can progress the disease. The MLD Foundation has done plenty of research and recommends using Propofol via IV for the best possible route.
So on March 5, 2021 she had a 2-hour surgery in Utah which replaced the port and over the next two weeks it worked better than it ever had before (the initial port had issues pulling CSF in a timely manner). We were relieved that maybe now she would better benefit from the study.
But on Sunday, March 21st we noticed the incision on her back was swollen. The doctors said to keep an eye on it and let them know if it gets bigger because it looked like a CSF leak. Within the next two days it had doubled in size. So on Wednesday, March 24th we were off to the ER at Phoenix Children’s Hospital.
We were told she would need surgery again to fix this. Now we were at a crossroads.
At the last surgery, Dave and I had discussed pulling her from the study if another surgery was ever needed. But how can we not get her the only potential treatment available to her? We had to weigh the pros and cons. Was the travel too much on her? Was it too much on her sisters who had to stay home with grandparents? She has only regressed since starting the study and we have seen no improvement but how do we know if maybe it was helping? If we keep her in the study, we can’t possibly ask a grandparent to take her to Utah in this fragile state while we are in Italy for Keira’s next check up. This and more went into our decision-making process.
At our meeting with the neurosurgeon we had our answer. But before we could share it, they told us the port needed to be removed completely in order to fix the leak.
I immediately felt relief. This confirmed our decision. The port was coming out and our intensive travel schedule was coming to an end. This would give us all more time to together with Livvy for however long we have left with her.
Since the surgery, which went very well, the doctors told us they found not one but two leaks (one from this port and another presumably from her first port). They also shared that it wasn’t just a little leak but an abnormally shaped hole. They stitched it up well, topped it with many closing methods and do not expect it to reopen.
As she lay flat, as per doctors orders, on the bed next to me, I am relieved to know this chapter is closing.
My Aunt said it best: it was a tough day but another one behind us. We are looking forward to more smiles with our Livvy in the days ahead.
Recovering from surgery with some sweet sleep
Side note: I have spoken to many parents about the trial. Some who had kids enrolled, some whose kids got denied entry and some whose kids couldn’t get in because they were no longer accepting patients. To the ones who couldn’t get in I would like to remind you of our situation. There is no guarantee it will work and our family is unfortunately proof of that. Know that you are doing the very best for your child given the resources available to you. Let’s all hope a better option is on the horizon. ❤
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.
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.
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.
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!
The Riley's Road to Italy 