Last year after we returned from Italy for Keira’s treatment we were connected with a non-profit organization called Sparrow Clubs USA which is based in Oregon but getting into the Arizona market and looking for a family – in particular a child with medical needs like Livvy – to partner with as they launched their first Sparrow Club at Hamilton High School in Chandler, AZ.
After hearing the story of how Sparrow Clubs began and what it does for high school students, as well as families who have children with extreme medical needs, it was a no brainer that we wanted Livvy to be part of the impact it would make on our local community.
To put it simply: “Sparrow Clubs exist to set the stage for simple, yet heroic, acts of kindness in schools and youth culture by empowering kids to help kids in medical need.” And as Matt Sampson (the Executive Director of Sparrow Clubs) and their community partner Amy Anderson of Black Rock Coffee explained, Hamilton High would adopt Livvy as their first “sparrow”, the entire student body would do up to 300 hours of any kind of community service work and as they did so it would unlock the funds that Black Rock Coffee donated for Livvy.
Sparrow Clubs made flyers for the school, created this lovely video about Livvy (which brought us to tears), and before we knew it we were touring classrooms at Hamilton High this week with the Student President of Hamilton’s Sparrow Club Richa Churravuri.
Students watched the video, learned about Sparrow Clubs from representative Cory Burket, I briefly told our family’s story and the kids all lined up to meet Livvy and give her a fist bump. She loved it!
The following day (today) we were invited to their school wide spring assemblies which were divided into two assemblies on the football field due to the school size (4,000+ students) where Richa got to speak about Sparrow Clubs, how students could get involved and to introduce Livvy.
It was a really cool experience for her (and us) as she got to meet the Hamilton Husky mascot, take a picture with the cheerleaders and get cheered on by all of the students. I overheard one student who met her yesterday yell out “LIVVY! That’s my girl!”
And as I shared with someone today, having her be a “sparrow” and part of this experience is not about the funds for Livvy’s new stroller but more so about the impact that meeting her and being a part of her story would make on others. How it would instill kindness, compassion and empathy in these students, and ultimately make a ripple effect of kindness in our community.
We are honored to be a part of this experience and hope to see more Sparrow Clubs throughout the state.
We all know that our little girls are warriors. All three of them. But this month, they actually got to feel like superheroes, thanks to the amazingly kind-hearted folks at the Colten Cowell Foundation and Campbell’s Crew Cares.
A friend of the family who had been following our story – and whose son also happens to be a patient of the girls’ neurologist, Dr. Narayanan – nominated Livvy and Keira to have a one-of-a-kind superhero experience. To touch, feel, see and act like an actual Superhero. But not just any superhero. Like Batman.
We were told nothing aside from to be ready for a night we would never forget with our girls. Grandparents were invited. And we all showed up not knowing quite what to expect, aside from a letter we received via email from Bruce Wayne himself inviting us to tour of the world renowned Wayne Research and Development Labs.
The letter read… “It has been brought to my attention that you have shown extraordinary strength and courage in the face of great obstacles. You are an example to us all, and I wanted to personally congratulate you for the fine precedent you have set. You should be proud of yourself, as I know many around you certainly must be.” Isn’t that the truth?!
Upon arriving at this top secret location in central Phoenix, we entered the office of Bruce Wayne himself where radio host Bruce St. James began the tour. The girls were shown “documentaries” of Batman and Robin (aka the Batman series from the 1960’s, which we had shown Eva before and she made sure to point out she had seen that episode before). This office looked almost identical…the same bookcase that was a secret door (which Eva spotted immediately), the red phone, and the bust of Shakespeare with the secret button. After getting to actually press the button, the girls superhero experience really began.
There was Batman and Robin’s firemen poles down to the Bat Cave…which we all went down – even Livvy! Gadgets galore met us at the bottom and the girls got to touch it all! They were in awe. But the best part was yet to come. Right next to laboratory was THE REAL BATMOBILE! In the room next to it…Batgirl’s Motorcycle, the Bat-copter and other classic cars that were seen in Batman’s “documentaries” of his life.
Suddenly, alarms sounded…there was an emergency and we had to get in the Batmobile immediately! The garage door opened, Livvy and I got in and off we went! As we paused in the parking lot, Dad was invited to DRIVE IT! Everyone took a turn going for a ride around the buildings nearby AND the girls all got to ride in Bat Girl’s motorcycle and sidecar. THIS was what they all loved most. Eva and Keira rode in the sidecar together while I held Livvy for her turn. And remembering that one little ride brings tears to my eyes every time I think of it, just as it did that night. She LOVED it. Livvy may not have words to tell us how she feels but man can she squeal with glee and laugh so hard! She was not quiet that entire ride. Truly unforgettable.
After our joyrides were through, we went back inside and were surprised again to see that they had made checks out in the girls names to both the TGen Foundation (a non-profit dedicated to the research of rare diseases, which is how we were introduced to Dr. Narayanan) and Campbell’s Crew Cares (a non-profit founded by a fellow Wish Kid named Campbell, his mother Carrie and their amazing family), two causes and groups of people that have become very dear to our hearts.
As if that wasn’t enough both Livvy and Keira were given the actual keys to the Batmobile. For Eva? As the oldest, when she turned 16 she would get to come back and drive it herself!
Calling it an amazing night would be an understatement. The first thing Keira said to me the next morning? “I want to ride motorcycle!”
NOW, let me tell you about who made this all possible: the Colten Cowell Foundation, co-founded by Erika Cowell and Charles Keller.
By watching how the replica Batmobile he purchased inspired wonder and joy in his own children, Charles knew it was more than just a great set of wheels. Charles could see that sharing the Batmobile was a great experience not just for themselves but something that others should enjoy as well. Colten, Erika’s son who passed away from Leukemia two weeks after his ride in the Batmobile, is the child who inspired it all. And now, the Batmobile is at the heart of this one-of-a-kind experience that transforms children into Superheroes, creating unforgettable memories for families.
We were the 680th family they gave this experience to, they have served 250 charities and raised over $1.5M for other non-profits.
They say it best on their website:
Most children will have a full life to dream — and the luxury of time to make those dreams come true — sadly that’s a much different story for many of the children we serve. For some, their abilities are limited, and for many others their time is running out.
We designed the Superhero Experience to benefit the entire family. For the children, they get to forget that they are sick and simply enjoy being a kid. For the parents, this is an evening to momentarily let go of their worries and share a moment of pure joy with their children. In the end, memories are all we have, and the best are the ones that we get to share with the people that we love.
THANK YOU to the entire team at the Colten Cowell Foundation for letting us have this evening of pure joy with our girls. It is most definitely a memory we will cherish forever.
While we are still waiting for one last result (regarding any ARSA antibodies), I couldn’t think of better news to share to end the year: all of Keira’s other testing from her 1-year post gene therapy appointment have come back great!!
We are beyond overjoyed and so releived to know her MRI looks perfect, EEG was normal, her audio/visual nerve testing was “glorious” said the doctor, and all other bloodwork is in normal range.
The last test she had was for her neuro psych evaluation, which we were not worried about at all given her speech language pathologist’s recent assessment. But there is always that what if…
Dave had taken her to that appointment that morning (because only one parent is allowed still due to covid) and we were both so busy with work and the girls’ schedules that day that I didn’t see him until near dinnertime. Finally, he gave me the results: “She was above average in all categories…except communication.” I looked at him shocked because she is speaking so well and then he finished…”in which she was extraordinarily above average!” 😁👏🏻🎉
PHEW!! So much relief just washed over me.
We are so incredibly grateful that her body has accepted this treatment and she is doing so well.
At this stage Livvy was already losing her abilities to walk and talk so it truly is a miracle and a joy to see Keira developing and doing new things every day.
We are in awe of Keira every day and so grateful Livvy has been her guardian angel on earth.
Words cannot express our gratitude to each and every one of you who has been a part of our family’s journey to help Keira have the chance at the normal life that was taken from Livvy due to this terrible disease.
We wish each and every one of you a Happy New Year!
While I wouldn’t normally write a blog about something like this, I felt it was almost my duty as an MLD parent to share our story. Even the upsetting or sad parts. Because MLD is so rare some doctors have never even heard of it. And oftentimes it’s the other MLD parents who I get the best information from. Because they have been there done that and know what works (and what doesn’t). The amount of information on the internet regarding Metachromatic Leukodystrophy is alarmingly small.
So in case there is another parent in my shoes looking for answers, I wanted to share another part of our journey. Today, Livvy had her first seizure. I say first because seizures can be common in MLD children and once they happen they are more likely to reoccur.
This morning when we went to get her out of bed, we found she had thrown up and was actively having a seizure. I rushed to get the Diazepam (Valium) and as our doctors instructed gave her 10mL to stop the seizure. It took anywhere from 15-30 minutes for it to stop. We got her all cleaned up, and stayed by her side talking to her until she was able to fall asleep.
In the meantime, I had also called Hospice of the Valley who had a nurse on their way (aptly named Angel) and called both of our mothers, who rushed over.
Hospice took her vitals, which were normal. Said we did all the right things and the nurse called the doctor to get his thoughts. He initially recommended Attivan but we had tried that with Livvy before simply for anxiety and she did not like it at all. Was very uncomfortable. So we asked to just remain on Diazepam, which they thought was fine.
While they were here we also called the girls’ neurologist Dr. Vinodh Narayanan. He got back to me after Hospice had left and recommended we first get an EEG to check activity before deciding on a plan. He also recommended a nasal spray form of Diazepam (which I can’t remember the name of now but will update the post once I get it). This comes in two bottles, each 5mg. Use one bottle in one nostril and if it doesn’t stop the seizure you use the other bottle. He recommended this since it can be more quick acting than administering Diazepam through her G-Tube.
After we got off the phone I also sent him a video of Livvy mid-seizure. It’s terrible to have to take a video of your child while they’re undergoing something so traumatic but it came in handy and was important for our doctor to fully understand what was happening and decide the best course of action.
From the video, he thought it looked like she was having an epileptic seizure; a complex partial type, rather than a grand mal convulsion. Based on that he recommended we start anti-seizure medication right away but still get the EEG done (which can be done here at home) and use Diazepam when they do occur.
The Hospice doctor suspected that her immune system may have been weakened by a viral infection which lowered her threshold for seizures. Given that we were all sick last week (not from Covid) and she threw up again later in the day we think he was right. We had hoped since it had been a week we had done enough to keep her clear of it but unfortunately not. And as is the case with MLD kiddos, anytime they get sick their disease progresses (another reason we have still been semi-quarantining and taking extra measures to keep the girls safe and healthy).
Since the seizure Livvy can no longer move her left arm or control facial movements. Things that would normally make her smile so big (like talking about her baby sister Keira) don’t get a reaction. And it almost seemed as if she realized she couldn’t do it and got sad and almost started crying. I told her it was OK and that sometimes after seizures we can’t move like we used to but it can come back. As I have now learned post-seizure paralysis can be temporary. So we will see how it goes in the days ahead and keep everyone updated.
Last week, we enrolled Livvy in Hospice of the Valley. Not because she is drastically and rapidly declining, but more so to have that support system in place for when she does and in order to have physicians come to her rather than putting her in her stroller and driving anywhere (she has always hated carseats and found them very uncomfortable since her diagnosis), or having her anywhere near COVID patients at a local hospital.
Surprisingly, to me, this process was not as difficult or emotionally trying as I thought it would be. Honestly, the hardest part was talking to our palliative care team about end of life scenarios prior to enrolling in Hospice. That is how we came to the decision that it was indeed a good time to enroll Livvy.
And what might come as a surprise to many is that enrolling a child in Hospice is not the same as enrolling an adult. For an adult, Hospice means they have very little time left. For a child, like one with MLD, they could be on Hospice for years (or days…there really is no way of knowing because the disease is so rare). But the point of enrolling a child is not only to ensure end of life scenarios are completed as the patient’s family prefers (at home versus in a hospital with a million tubes connected to them), but also to ensure their comfort until that point arrives.
Livvy’s meds will now be delivered instead of us running back and forth to Walgreens, a Pediatric nurse and social worker will come to the house to check on her every two weeks (or as needed) and a pediatrician will visit every two months (or as needed), and we now have a number to call them 24 hours a day 7 days a week for any medical questions or concerns rather than guessing what’s wrong and having to rush to the ER. Any time, day or night, they will come to her.
For the parents, siblings and patient, this is a game changer. It keeps disruptions and any further trauma to a minimum. We are incredibly grateful to have their support as we contunue to navigate this heartbreaking and unbelievable situation.
For more information on Hospice of the Valley, visit www.hov.org.
When you become a parent it’s almost automatic to write down the dates of your children’s milestones. Rolling, sitting, walking, talking, running, etc. But when they have a terminal illness like MLD, you begin to avoid those dates because it generally means loss of milestones.
Looking back at my notes, I did not put a date, or even a month, next to when Olivia stopped talking. Stopped walking. Stopped sitting up on her own. Or lost control of her legs entirely.
But I did recently write down a month for when she stopped being able to eat and drink on her own. It was this month, July 2021.
We had been working for weeks to keep her eating orally as much as possible and drinking from the one sippy cup that she could actually sip from. But there was no denying this was something she could no longer do.
Sometimes she could barely open her mouth to put food in it. Things that she could normally get down no longer worked and would cause her to choke. Even water in her sippy cup didn’t work. We thought of thickening it as some MLD families do but she simply could not use the cup to make a sipping/sucking motion at all.
She is now 100% fed through her GTube. She gets Kate Farms formula for nutrition, water and her meds all through the tube. However, we do give her small amounts of water and juice in her mouth through a syringe so she has something to enjoy and keep her mouth from getting dry.
This recent loss of development was one of the many reasons we also decided she would not be starting school this week.
On top of the eating/drinking issues she has become more lethargic and is experiencing more pain. This has required higher doses of Gabapentin and THC 3x per day. Sometimes we even have to use Valium when those don’t do the trick.
She also usually only wants me for comfort. Dad, Grandma’s and her nanny Sheena will work here and there but more often than not, it’s Mom she wants.
So as we continue to keep her comfortable amidst her decline we chose to not send her into a new environment potentially filled with germs and people she doesn’t know but to keep her home where she is most comfortable and we can spend the most time with her for as long as we have her.
Rod Paige (the 7th United States Secretary of Education) once said “There is no more powerful advocate than a parent armed with information and options.”
Now, as a special needs parent, I fully understand the truth in this quote.
Yes, every parent must know that part of their job is advocating for their child. It’s common sense, right? We all want what is best for them.
But, prior to January 2020, when I was just your average Mom with normally developing kids, there really wasn’t a need for me to advocate for them. We of course would research the best schools, pediatricians and other options out there but that was every day stuff that parents do.
In the past month alone I have done more advocating for Livvy than ever before.
Getting anything you need or want for your special needs child is a MAJOR task. And don’t expect for companies or vendors you work with to follow up with you. None of them will. It has been our job to do all of the follow up to ensure she is getting everything she needs when she needs it.
I have had to text our pediatrician requests on 8 separate occasions.
I have had to call a new pump company we are transferring to for Livvy’s GTube supplies 10+ times.
We have had 3 meetings to trial eye gaze devices, with a follow up meeting to come, before we decide on one and then wait weeks to months to receive it.
I have had to speak with our neurologist about medications twice.
I have had to schedule PT, OT and feeding therapy appointments.
I have had to personally email prescriptions and referrals from doctors to vendors because their fax number isn’t working or they gave me the wrong one. Side note: can we please stop using fax machines?!?!
The list goes on. Keeping Livvy as stable as possible is a full-time job. Especially as her disease progresses and her needs (medically and for physical comfort and mobility) change.
To any parents who are new to the special needs run around, know that you are not alone. The frustrating back and forth and hoops to jump through is (unfortunately) normal. It is up to YOU to make sure your child is getting what they need.
Support systems through DDD and the like offer some wonderful assistance but it still requires work on the part of the parent to get the ball rolling when it comes to getting any kind of device or specialized care.
Be your child’s voice. Be their strong arm. Go with your gut. You do know best.
It has been a while since I posted. Mainly because it has been a busy month for all of us and one of many ups and downs for Livvy.
While Dave and the girls were in Italy, Livvy started having these random bouts of pain that were inexplicable. Nothing looked like it was physically or outwardly hurting her so we assumed it must be internal. After seeing her neurologist and explaining the issue he suggested trying a low dose of Gabapentin. We had tried Gabapentin when she was first diagnosed last year and she had a weird reaction like she didn’t like the way it made her feel. But this time, it worked! Those random bouts of pain went away and we were so grateful!
We also met with a palliative care doctor at Phoenix Children’s Hospital. He agreed with the medication routine she was currently on and we also inquired about THC drops, as we heard they worked well for other MLD kiddos. He said other families he has spoken have said they have changed their childrens’ lives and that it is worth a try so I placed an order at a local shop (Marijuana is now legal recreationally in AZ) and picked up some drops. Those also seemed to do a great job at lowering her anxiety/frustration and feeling better overall.
Next, (while everyone was still in Italy) we ran out of her Amitriptyline (drops that were prescribed to us in Italy because they couldn’t get her usual Nortriptyline). Thinking nothing of it, because doctors assured us these meds are really one in the same we moved her right on to the Nortriptyline. And it did not go well! She started having a similar reaction to it as she first did on Gabapentin. She clearly did not like the way it made her feel. So after consulting the doctor I cut her dose in half. Still didn’t like it. So I cut it in half again. And at that point the dose was so small I wondered if she even needed it. So we weaned her off and she has been doing great without it! MLD kids always have so many meds they take so when we can do away with one it’s amazing!
Next up on the list was getting an eye gaze device. These things are such a game changer! For kids and adults alike who can’t speak, it allows them to look at words and/or pictures on a screen and by a glance of their eye alone they can speak, make choices and express feelings. Getting one in a timely fashion, however, is no easy task but that’s a whole other blog post.
We began this process working with Livvy’s team through the Arizona Early Intervention Program (who children are enrolled with prior to three years of age and then they go into the Long Term Care program through DDD). After she turned 3 in May we had to send in the necessary paperwork to get it referred by our pediatrician and she just had her first two trials of eye gaze devices!
The first one we trialed was EyeTech, which seemed much more extensive in its options but maybe a little more cumbersome to learn and navigate. The rep mentioned one gentleman actually used CAD via this device!
The second we trialed was called PRC which Livvy seemed to be able to use a bit better but the options didn’t seem as extensive.
Tomorrow, we have one more trial with Tobii Dynavox, which is the option many other MLD kids use so we are looking forward to that and then meet with our therapy team next week to make a decision.
I hope this will be a device that Livvy is able to learn and use so that we can again hear what she is thinking or wants (or doesn’t want). Such a miracle! We are so excited!
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.