Why Holidays are Exhausting (Now)

I’m finally coming up for air from Easter weekend. And I’m still exhausted…mentally.

As with most holidays since receiving Livvy’s terminal diagnosis that stripped her of her abilities to walk, talk, and enjoy so many things in life, I dread them. Holidays are no longer what they used to be. They’re all now a glaring reminder of everything our daughter can no longer do.

While you would probably never know that by seeing me during the holidays because we keep things as absolutely normal as possible for the girls, I have to mentally prepare before each one.

How do we keep Livvy as involved with her sisters as possible? What “toys” do we get her now that she can’t even hold on to anything? What can we do to still give Keira and Eva the same opportunities as we normally would but not exclude Livvy? How can we have her help us decorate? Will the clothes we order be stretchy enough to go over her arms when she’s having trouble straightening them? What equipment and supplies should we bring if we have to go to a grandparents house? The questions go on and on.

It’s exhausting. And stressful. And sad.

But these are the things parents of children with MLD have to think about.

Not to mention the fact that with each passing holiday, we are one more holiday closer to her not being here. Which, as I write this, I suppose is a true statement for all of us but not a thought that is as omnipresent as it is for a parent of a terminally ill child.

Will this be her last Easter? Her last birthday? Her last Christmas? I think that thought on every holiday.

This Easter was particularly hard because Livvy was battling her 5th UTI in a row, spiked a fever and then had a seizure the morning we were going to take them all to meet their cousins at a nearby farm for an egg hunt with fair rides.

With an Easter excursion out of the plans for her, it was all about rest, relaxation, keeping her food and meds down and doing whatever we could to get a smile back on her face.

Thankfully, we got a new, additional medication prescribed (Augmentin), and it’s doing the trick to get her well. We are hoping it keeps these infections at bay (since Keflex did not) as we prepare for her 5th birthday next month. A very happy occasion, absolutely, but another event that will take its toll both mentally and emotionally.

To my fellow parents of terminally ill children: stay strong and don’t feel bad about taking a break after the holidays. I know I always need one, and Lord knows we ALL deserve it! ❤️

Right to Try 2.0 Passes in Arizona

After testifying at the Arizona Senate and House of Representatives, sharing our family’s story and how the expansion of the original Right to Try Bill could help families like ours, I’m thrilled to share that Right to Try 2.0 has passed and was signed by Governor Doug Ducey!

Right to Try 2.0 is a first-in-the-nation reform that will empower seriously ill Arizonans to access personalized treatments that have yet to receive full approval from the U.S. Food and Drug Administration.
Goldwater Institute

We had the pleasure of meeting Governor Ducey last week during the Ceremonial Bill Signing at the Arizona State Capitol. The girls were thrilled to go (after a quick lesson in US government so they knew what a Governor, Senator and a Bill is).

Governor Ducey, Senator Nancy Barto and myself all spoke before he signed the Bill. You can view a portion of that here:

“Now, critically ill patients can get access to these groundbreaking treatments here in Arizona, so no family has to go through what the Riley‘s did.”

SB 1163, sponsored by @NancyBarto, was just signed into law. Hear how this bill will create new opportunities for Arizonans. ⬇️ pic.twitter.com/b0kkGMSOtn
— AZSenateRepublicans (@AZSenateGOP) August 15, 2022

The Goldwater Institute’s Right to Try for Individualized Treatments reform builds on the original Right to Try law and protects patients’ right to try to save their own lives by seeking treatments tailor-made for them that are not yet FDA-approved. This common-sense measure accounts for new innovations in medicine and helps get those innovations to the patients who need them the most.

As I mentioned in my speech, the United States is the greatest country on earth and access to life-saving medical treatments should be among its many benefits. We are thrilled Arizonans now have that right and are hopeful other states – and eventually the nation – will follow suit.

You can read more about Right to Try 2.0 and our efforts here on Reason.com.

And here some photos from the signing at the Arizona State Capitol:

Photos by Neal Mei Photography, courtesy of the Goldwater Institute.

Breaking Records at Wish Ball

A few months ago, we were approached by Make-A-Wish Arizona to see if we would interested in sharing Livvy’s wish (her swingset) in a video that would be played at their biggest fundraiser of the year, the Wish Ball.

If I’ve learned anything from this journey so far it’s that sharing our story can only help others. We said yes, filmed it in our backyard with the girls and below is the final product which was played during live auction and Fund-A-Wish portion of the evening at Wish Ball this Saturday.

After this moment, records were broken. The Wish Ball raised more money that evening than they ever have before. And we are so honored to say that Livvy was a part of that. That her story is now helping other children get their wishes granted.

Take a moment to watch it below and if you feel so inclined help Livvy give more kids their wishes by donating to Make a Wish Foundation of Arizona here.

Thank you Make-A-Wish Arizona for not only giving Livvy her wish, but also giving her sisters (and us) memories with that will last our lifetime.

More photos from the evening below:

Testifying for Updated Right To Try in the Arizona Senate

Back when we got Keira’s diagnosis in June of 2020 and were figuring out a plan for her treatment I was connected to Arizona Senator Nancy Barto and the Goldwater Institute. With the Right To Try bill having already passed (in AZ in 2014 and federally in 2018) they thought maybe there was a chance for us to use this to our advantage but unfortunately it only encompassed groups of individuals (that could lead to clinical trials); not for individuals on a case-by-case basis.

Fast forward to last week when I was contacted by the Goldwater Institute who asked me to help them expand upon this right-to-try bill by sharing our family’s story and testifying at the Arizona Senate next week. What for?? To expand the bill to cover individuals that need life-saving treatment. My jaw dropped. This is AMAZING.

Former Arizona Senator Barry Goldwater who founded the Goldwater Institute in 1988

Had this been in place when Keira needed it (and the treatment was in place within the US and being offered by the drug company) we could have had her treatment done in the States without having to fundraise hundreds of thousands of dollars and moving our family halfway around the world for 5 months.

So, naturally, I jumped at this chance and on February 2nd will be off to the Arizona Capitol to meet with the Goldwater Institute and Senator Nancy Barto’s Health and Human Services Committee to testify in support of patients getting access to individualized treatments without having to beg the federal government for permission to save their own or their loved one’s life and without having to cross an ocean to do so.

The proposed legislation adheres to the strictest patient protections and physician involvement, ensuring that these treatments work in tandem with the highest standards of care.

Should this pass in Arizona, as the original Right to Try bill did (view more on that here), other states will likely follow suit and then the federal government to follow.

A copy of the bill – the Right to Try for Individualized Treatments – sponsored by Senator Nancy Barto within the Arizona legislature be found here:

https://www.azleg.gov/legtext/55leg/2R/bills/SB1163P.pdf

I can’t even imagine how many lives will be prolonged or saved entirely by doing this and it brings to tears to my eyes thinking of all the other special needs families out there who have always held on to hope for a chance like this. A chance at healing. A chance at a normal life. Something every single one of us deserves.

I am beyond honored to have this opportunity to share our family’s story in hopes that it can truly make a difference. I will be sure to keep everyone updated as this moves forward within the Arizona legislature.

Livvy’s 1st Seizure

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.

Love from the Rileys. ❤

Enrolling in Hospice

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.

One of many lovely photos of Livvy captured by our friend and photographer Kay Eskridge of Images By Kay. ❤

Loss of Timeline

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.

Advocating for Your Special Needs Child

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.

For example…

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.

Med Changes and Eye Gazes for Livvy

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!

Livvy’s Wish Came True

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!