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.
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.
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.
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.
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.
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. ❤