A Very COVID New Year

Well, wouldn’t you know it that we started the New Year by welcoming COVID into our home?! On January 1st, Keira – one of the most sheltered of all of us – tested positive after an overnighter with grandparents, one of which also (unknowingly) had it.

Thankfully, only the first day was the worst with 100+ degree on-and-off fevers. And it’s been easy sailing from there. No cough or any other symptoms aside from fever. It’s now been almost 3 days with no fever and we are just awaiting that negative test for her to be in the clear.

To say it’s been quite the first week of 2022 is an understatement. With one of her grandparents also positive, they couldn’t help us juggle work and the girls. Our nanny couldn’t come here. Nor could other grandparents who have auto immune sensitivities. So Dave and I divided and conquered. I have been quarantining with Keira in her room while Dave stays with Livvy and Eva on the other side of the house.

As she gets better and better the harder it is to keep her quarantined. And let me tell you, racing behind her with wipes cleaning every surface or toy she touches is no easy feat. She’s fast!

On top of all this, we just found out another grandparent also has COVID! How this is hitting our family now, after all of our travels abroad for Keira’s gene therapy and Livvy’s clinical trial is beyond me. But here we are!

The first night, I’ll admit I was terrified. While I’ve kept COVID related news on the peripheral (because we have our own life-threatening problems) I couldn’t help but think about the horror stories. And I just had an older friend even pass from COVID. Would Keira’s immune system be up for this after all it has been through? My mind raced.

I couldnt help but think of all the people lost or suffering from this virus. Not to mention the many other diseases, natural disasters and million other dangers we face as human beings.

The amount of suffering placed upon any one person can be insurmountable. Unbelievable. Unfair. Downright cruel. But here we all stand, fighting. Fighting for the light. For the good. For health. For hope. For love. For family.

And fighting is not easy! After the past two years of what our family has been through, do I sometimes get tired of fighting? Absolutely. I want Livvy to be free from pain. I want Keira to live the life Livvy deserved. I want Eva to have her childhood back. I want no child to suffer from MLD ever again!

So to lose Keira from COVID after all we have done to save her, it’s just unacceptable in mind.

Thankfully, the universe and her immune system agree. She’s back to her normal self and soaking up all the alone time with Mom.

While we had to cancel her family-only birthday party on Sunday, we are so relieved to know that we won’t be in a hospital and we will still be able to shower her with the gifts and attention she so deserves. Our baby girl is turning 2!! And she has lost ZERO abilities to MLD thanks to the gene therapy she received in Milan. That is what I plan to focus on remembering from this time in our lives. NOT COVID!

Keira’s 1-Year Post GT Results

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!

Goodbye Sherlock

Saying goodbye to someone we love is never easy. Whether it’s expected, in the case of a terminal illness like MLD, or unexpected, as was the case with our dear 7-year old French bulldog Sherlock this past week.

After a week and a half of on/off appetite, throwing up and being generally lethargic some days, we headed to the vet. Surely, it had to be something weird he ate that was stuck in stomach because he would eat everything in sight! After his xray came back all clear we took him home and changed his diet to chicken and rice. Should his symptoms continue then we were to head back for bloodwork.

That evening Dave’s Mom took Eva (our oldest) for the night and thankfully she did because in the morning we found Sherlock had passed away in his sleep (and Eva was normally the first awake and would always get the dogs). We’re not sure whether it was cancer or maybe heart failure but either way it was crushing nonetheless.

While it was the hardest on us and Eva, I hope that having this close, and unexpected, experience with loss will help her when it comes time for Livvy to leave us as well.

I broke out sobbing in front of Livvy that morning and she actually laughed at me, thinking I was just making a funny noise to be silly. But I made sure to talk to her about Sherlock being in heaven and how we will get to play with him again when we go there. That prompted a really big smile (this isn’t the first time we have had a private conversation with her about heaven).

I assumed Keira would be mainly oblivious and possibly ask where he was. When she didn’t, I explained to her that Sherlock went bye bye to heaven and showed her the adorable paw print and picture frame that was given to us by All About Restful Pets (more on them in a second). Hours later she remembered the paw print and picture, looked at it and said “Bye bye Sherlock”. It took everything I had not to tear up right then and there.

But back to All About Restful Pets. They are who my mother called for me that morning when I told her the news sobbing. It is a company owned by her friend Bonnie which goes to people’s homes to put pets down peacefully and/or take them away for the family to be cremated (or whatever their wishes may be). She showed up at our house within the HOUR! And this was during morning rush hour in the fifth largest city in the nation. I was beyond grateful to have her there and help us through this process. For anyone in the greater Phoenix area, I highly recommend them.

Sherlock is greatly missed, and we can tell Watson misses him too (even though he is generally just a grumpy old man) so we have been giving him extra love and cuddles.

Rest peacefully little Sherlock! We love you!

Happy 1st Re-Birthday Keira!

Yesterday, October 2, 2021 was Keira’s 1st “re-birthday”, one year from the date that she received her renewed stem cells that would allow her to live a normal life.

It is beyond surreal to say that our almost-2-year-old is a 1-year survivor of MLD, of all things! And it is even more surreal that she is doing SO many things that Livvy never could because this very disease had already begun stripping away her abilities at this age (21 months old).

Just some of the things she can do now that she wouldn’t have been able to without treatment:

  • Walking
  • Talking in sentences
  • Running
  • Climbing
  • Walking up steps
  • Counting to ten
  • Learning her alphabet
  • Spinning around

These simple things, that we as “normal” parents can take for granted, are things that amaze us on a daily basis.

We are beyond grateful for so many people who helped us get her this life-saving treatment – not just the doctors and scientists who made it possible but the nurses, the hospital staff, the volunteer families in Italy who helped us during our stay, our own family, friends and even strangers who became so invested in our girls and their journey.

The connections we have made are priceless and Italy will forever have a special place in our hearts. We are looking forward to returning next year for her next check up. ❤🤍💚

Here is a flashback to one year ago today at Ospedale San Raffaele:

Keira’s 1-Year Post Gene Therapy Check Up

I absolutely cannot believe it has been (almost) one year since Keira received her life-saving gene therapy treatment in Italy (her official re-birthday date is October 2, 2020). Our time in Milan seems like a lifetime ago.

Yet here we sit at Phoenix Children’s Hospital for her 1-year post gene therapy check up!

And I am so happy to say I am not worried about her results one bit! She has been running, climbing and talking more and more each day. She is even advanced for her age in some ways and we are in awe of her every day.

While we normally would need to return to Milan for her follow ups, because of COVID the doctors there managed to figure out a way to do the testing here locally and send back her most important blood work on dry ice overnight to Ospedale San Raffaelle. While I love Italy and have no problem with returning, it is especially nice to stay home for this check up so we do not have to leave Livvy who continues to worsen.

As for the check up itself, we have a busy two weeks ahead which will include the following appointments:

  • Bloodwork
  • Ultrasounds (abdomen and thyroid)
  • MRI
  • Echocardiogram
  • EEG
  • Appointments with Pulmonary, Pathology and Neurology

There might be one more I’m forgetting but needless to say, there are a lot of things they will be looking at to ensure she is developing normally.

The one thing in particular which can only be checked in Italy is one of the results from her bloodwork which looks for any ARSA antibodies. The ARSA enzyme is something her body could not create prior to gene therapy. And now that is does her body began trying to fight it because it wasn’t used to it. However, the antibodies have only gone down in numbers since and hopefully at this point will be completely gone. Keep your fingers crossed for us there!

We will be sure to keep everyone updated once we receive her results. Thank you for your continued prayers, love and support! It means the world! ❤

Being Apart

This weekend, we have the honor of attending one our best friend’s wedding in Montana. While Dave (the Best Man), and Eva (the flower girl) have been there since Thursday for the rehearsal, I am heading out today (Saturday) so that we only leave the other girls (Livvy specifically as she is the most medically fragile) for one night.

Ever since I left the house this morning I felt sick to my stomach. I even slept terrible. But I know she has been doing well this week and my parents are taking excellent care of her at home. Yet there is still that nagging feeling, or feelings, of dread, sadness, separation anxiety and nausea, but also hope and reassurance. It’s a weird mix.

I never used to be this way prior to the girls’ diagnoses. I’d enjoy our child-less nights while the girls were with grandparents and have complete peace of mind. Would think nothing of it. But now even when Eva or Keira stays overnight with grandparents I miss them…a lot more than I used to. It’s weird without them around and I feel like it’s only because we know how fleeting life can be. We know how important every moment is with them. We know our world can change in a matter of seconds.

It is so weird to now think of life with such finality in every choice or action but I suppose when you have a child with a terminal illness it is inevitable. Life is fleeting.

So it is especially hard leaving this time because it is the first time since the girls’ diagnoses that both Dave and I have been away. And with Livvy only continuing to decline we have no idea how much time we have left with her. It could be days, weeks or even years. The disease is so rare that doctors won’t give a timeline.

So as I sit in the airport terminal forcing myself to eat something I am also trying to push away the butterflies; reminding myself that she will be ok for one night. She knows where I am and who I’m bringing back home with me tomorrow. She smiled when I told her.

But I will think only of her and Keira’s sweet faces until the moment we get back home and see them safe and sound. 💜💜

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. ❤

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!

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.

Thank you so so much, Make A Wish Arizona!! ❤🙏🏼

For Those Extra Special Moms

To the mom who didn’t get “just a healthy one”:

A healthy baby .
That’s what you want.

Boy or girl? Doesn’t matter.
Just a healthy one.

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!

Focusing on the Smiles

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.