78 Days Old: Gone to Heaven

This afternoon we said goodbye to our sweet, sweet Olivia, as she began her next big adventure: a journey to heaven.

Her passing was peaceful,  comfortable, and cozy, as she spent her last minutes cuddled in our arms. Yesterday she was held all day by family, and her final night and morning were spent playing, holding, and visiting with her favorite nurses.

We’ll have more on funeral services, photos, stories from mom and dad etc. later.

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73 & 74 Days Old: Muscle Biopsy

Yesterday morning Luke and I arrived at the hospital at 6AM. Olivia’s muscle biopsy was scheduled for “first case”, which is set to start at 6:30AM. Prior to her being called to the operating room , we had brief visits from a surgical resident, and an anesthesiologist resident. Both provided us with an overview of what their teams would be doing, and were prepared to answer any questions we had about the procedure.

She was eventually “called” down to the OR just before 7:00AM.

Transporting a baby from the NICU to anywhere else in the hospital is a bit complicated, especially when the baby is ventilated. We knew this from Olivia’s prior trips downstairs. Once for an MRI, another time for the HIDA scan. The transport consisted of her nurse, a respiratory technician, a NICU fellow, and the anesthesiologist resident. Luke and I were able to travel downstairs with Olivia and the team, but had to say our goodbyes just inside the surgical waiting room.


Thankfully, I found saying bye far less traumatic and emotionally draining than I had anticipated. A quick kiss and a squeeze and we were off.

The procedure was pretty quick, and went well. Taking the muscle sample was met with no complications, and Olivia had no negative reactions to the anesthesia. The fact that she would be going under anesthesia was more risky than the procedure itself, because people with myopathies can experience a range of adverse reactions to certain anesthetic drugs used during surgery. Certain ones can cause potentially fatal reactions, one being malignant hyperthermia, which refers to a dangerously high increase in body temperature.

The pediatric anesthesiologists were aware of this potential complication, so they took a series of specific precautions designed to (1) mitigate the likelihood of it occurring and (2) prepare the room should it occur.

But that didn’t happen, and the procedure went fine, and the tissue sample was “checked in” to the hospital’s pathology department yesterday… so now all we do is wait for results. We’ve been told to expect a turnaround time of anywhere from 2-3 days, to 2 weeks, so we are once again planning to practice being patient.

After the procedure, Olivia returned to her room wide awake and wiggly. Not the sedated and sore picture that we had expected!

The recovery from the procedure is minimal. She has an incision on her left upper thigh, but the list of things to avoid is short. No baths for 3-5 days. No physical therapy for 2-4 weeks. She isn’t on any pain medication, and the team doesn’t expect her leg to bother her much. She’s still kicking and stretching it out, and we’ve been touching and lifting it to change diapers, positions, etc. without much fuss.

She slept quite a bit yesterday, but besides that, she’s pretty much back to her usual self.


Today we did start her 2 month vaccines, which will be given in a series of 3 shots, spaced out over 6 days. She barely flinched when she received the first shot, and hasn’t cried or fussed since, but we’re now on the watch out for a cranky baby!

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72 Days Old: A Baptism, A Long Walk, and the 4th of July

Some days in the NICU are quiet days, and others are so full of activity and events and fun that it makes it hard to summarize in a single update to friends and family. Today was one of those days.

First, and most importantly, this morning we were joined by Father Mike from our church and Olivia was baptized. It was a short, but special ceremony, with just Luke, myself and our nurse Inna as a witness. We didn’t pick out a traditional baptism gown, but we did get Olivia ready in her best dress!

She looked like a angel. A perfect, little, sleepy angel.

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After the morning’s event, Olivia snuck in a snooze and a hold with Dad, before venturing outside for… get this… the 3rd day in a row! Late last week word began to get out that we were really itching for Olivia to see a bit of the world beyond her NICU room. The staff knew how much we wanted to take Olivia outside, and have graciously allowed us to do so every day this weekend. It takes extra equipment and extra staff, but with everyone on board it’s been such a gift.

We have some of the funniest pictures (and videos) of Olivia’s outdoor adventures, so we’ll share another post with our favorites soon. For now, here are some from today’s trip.


Olivia had even more visitors this afternoon. Mike and Jen met us outside and joined us for a stroll around the garden before a mini-meltdown made us decide to head back inside.

Jen came bearing gifts, which I’m going to keep as a surprise until the time is right to share them with you all. I’ll give you a hint: both items will help Olivia check off items from her bucket list! And by her bucket list, I mean, all of the things we want to make sure she experiences, despite being in the NICU.

Last but not least, since today is a holiday (and Aunt Bey’s birthday!), Olivia was subjected to another one of Mom’s holiday photo shoots. Per usual, she slept through the activity.

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68 Days Old: Daily Update

We’ve had a few nice, uneventful (in the good sense), and fun days around here. We survived Dad’s first business trip since Olivia was born (although we did miss him terribly), Nana and Grampa Prior visited, and Olivia and I had lots of girl time. She’s now spending multiple hours at a time out of her crib, either in her bouncy seat or playing on her boppy lounger in our arms, she’s tolerating PT and OT sessions really well, and her alertness and overall cognitive capabilities seem to be changing daily. All of which make for good, good days. The kind that make us momentarily forget about the bad days, forget about where we are and why, and forget about what lies ahead.

Speaking of which, her muscle biopsy has been scheduled for next Tuesday, July 5th. Yesterday we had been told it was going to be mid July, but this morning we got word she’s been scheduled in for next week! This is awesome news, as we already feel like we’ve been waiting for a long time, and otherwise we would have been waiting another long and likely intolerable two weeks.

So between now and Tuesday we don’t expect many changes to her usual regiment or daily plans. Just days of hanging out, holding, playing, and hopefully long naps!

Probably pretty sick and tired of these afternoon photo shoots!
Probably pretty sick and tired of these afternoon photo shoots!
Not too sure about this mohawk business...
Not too sure about this mohawk business…
Thursday morning quiet time with Mom... getting ready for nap time.
Thursday morning quiet time with Mom… getting ready for nap time.
Who better to swing by a children's hospital window, but superman himself!
Who better to swing by a children’s hospital window, but superman himself!
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66 Days Old: Daily Update

Olivia seems a bit more like her usual self today. Full of ‘tude and facial expressions that tell us exactly what she’s thinking. Yesterday’s lab results came back normal (meaning she likely does not have an infection), but an x-ray did show quite a bit of atelectasis in the right lung. We increased her ventilator settings overnight, and as of this morning’s x-ray the lung looks to be fully recruited again. So, the couple of tough days we experienced Sunday and Monday were likely due to her lungs struggling… just because of their usual things, rather than being an indication of an infection. Good news she doesn’t have an infection, bad news that we can’t blame her lung challenges on something else other than her myopothy…

Other things: her Tuesday labs looked good. The liver enzymes that have been elevated for the past month or so finally began trending down as of this week, and her bilirubin level is basically normal, for the first time ever! Her total bilirubin was 1.2, with a direct bilirubin level of 0.9.

We were visited by Dr. Pegoli today. He’s the pediatric surgeon that will do Olivia’s muscle biopsy. It was nice to see him again and have an opportunity to ask him a few questions about the procedure. His answers and demeanor once again reinforced that this is a very minor procedure (relatively speaking), and that it’s a very common procedure in his world. We still don’t have it scheduled, but hopefully we’ll get a date confirmed shortly.


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65 Days Old: Daily Update

We had a great weekend with Olivia. Friday was full of new activities, and Saturday was a nice long day with Mom and Dad. We had lots of girl time, which Mom soaked up. Sunday started off on a good note as well. We visited for a couple of hours in the early morning, before stepping out to celebrate our goddaughter’s baptism. Hi Emma!

Sunday evening Olivia started having more respiratory events, which continued throughout the day today. Last time her events started acting up like this, we later realized she had an infection… so the team was concerned that she may be getting sick again.

We ordered an x-ray, loads of blood work, and a urine sample this morning, but all initial reads look normal. Since she’s continued to struggle a bit today, Dr. P is recommending we increase her ventilator support overnight to let her rest and ensure she has fully recruited both lungs.

Not a ton to share otherwise. We’re still waiting for her biopsy to be scheduled, and in the meantime are hoping she starts to feel better soon. Watching your little one fuss and cry and struggle all day is no fun!


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Two Months Old

Guess who’s due for a celebration today? Olivia Grace is, that’s who.

Now 21″ long and just over 7lbs, today Olivia celebrates her 2 month birthday. Technically, in the medical world, she’s “on day 61 of life” but by our standards she’s 62 days old and today’s her monthly birth “day”. To celebrate, she spent the morning with Mom and Dad, dressed in her birthday best: an outfit with green animal print and glittery gold hearts.

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In the afternoon – after a snooze in Mom’s arms – we got even more crazy, first with a little play time in a “crib gym”, and later was her first time in a bouncy seat.

First up: crib gym time. The voice in the video is our occupational therapist Meg, who is helping Olivia interact with the toy by propping up her arm. This helps “remove gravity” for Olivia, which makes it easier for to move her extremities on her own.


Afterwards, when it came time for a field trip to the bouncy seat, we positioned the seat on the floor along the windows, so little miss Olivia could catch a glimpse of the great outdoors!  As I sat with her, I couldn’t help but think about how she’s 2 months old now, and never even been outside. She’s never really seen the sun or laid on the lawn or felt a rain drop. It made me think of a JJ Heller song that I’ve grown to love (lyric below) and I sure hope Olivia loved staring out the window as much as we loved giving her the opportunity to do so. Little does she know just how big the world outside room 15 really is!

“It’s a big world, baby, big enough to dream, you’re not too small to do great things”.

It’s worth noting that taking Olivia in and out of her crib is quite a production and always runs at the risk of sending her into a bit of a tizzy, so we’re very thankful for the nurses and therapists who are willing to do it for the sake of these great “normal” child development opportunities.



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61 Days Old: Genetic Test Results

First a little update, then a big one.

To start, the little lady packed on the pounds over night, and is now officially a 7 pounder. Her overnight weight was 3,230g (7.12lbs) which is just about 2 lbs up from her birth weight. She’s still way, way low on the growth chart, but any growth is good growth for us.

For the big update: earlier this week MNG Labs completed the Next-Generation sequencing that we ordered in May. We met with Olivia’s core team, along with the pediatric neuromuscular specialists on Tuesday to discuss the results.

Unfortunately, despite sequencing nearly 200 genes that are most commonly associated with known neuromuscular disorders, the panel came back “indeterminate”. In other words: the test did not provide us with an official diagnosis, and we are still unable to associate a name… a specific disorder… something well known and understood… to Olivia’s condition.

The panel did however identify mutations on two of the analyzed genes.

  1. One mutation is being disregarded as benign, as it’s on a gene that isn’t responsible for anything that fits within Olivia’s clinical picture.
  2. The other mutation is of much more interest to us and her team, because this specific gene is understood to be closely related to an important part of muscle function (Olivia’s primary issue).

This mutation is incredibly rare, in fact never seen before, so it is considered a “variant of unknown significance”. But… based on: the gene’s role in muscle function, the type of mutation (“likely pathogenic”), and Olivia’s clinical picture… it’s seemingly significant.

In order to more confidently confirm that Olivia’s myopothy is in fact a result of this gene mutation, she’ll undergo a muscle biopsy. A muscle biopsy is a relatively small procedure in the realm of pediatric surgeries, but it is still a surgery. Under a microscope, muscles affected by various myopathies have fairly distinct appearances, and depending on whether something atypical is observed in her muscle sample, Olivia’s team may be able to officially connect the dots between this mutation and how it’s impacting her muscles.

If they are able to connect the dots, Dr. C (the pediatric neuromuscular specialist we’ve been working with here at University of Rochester) will have the data points she needs to begin consulting with other specialists in the field with more of an expertise on this particular gene. One doctor in particular works out of NIH, and has dedicated his career to studying this one, specific gene!

Over the next couple of weeks as Olivia’s biopsy takes place and we learn more about what these test results mean for her, we’ll keep you all up to date. In the meantime, here’s today’s installment of: a snoozy, sprawled out baby.





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60 Days Old: Daily Update

Uneventful and peaceful day in room 15. Olivia was awake all morning, first spending a couple of hours in Dad’s lap, followed a couple more hours out of bed in Mom’s lap. When we put her back in her crib for her 12PM care routine, she tolerated it nicely (with no respiratory events) and then stayed awake for most of the afternoon.

Since she was so alert – and not agitated or angry – we took the opportunity to try out a new mobile in her crib. Olivia has responded well to all of the toys that the child life and OT teams have provided for her crib, and she especially loves the mobile we introduced a month ago. But after listening to the same tunes, and looking at the same pictures for a month straight, we figured it was about time for a change.

Within seconds of installing this mobile, the girl was hooked. Absolutely captivated. She remained fixated on the toy for quite some time, before taking a snooze. Toy time is hard work!



Playing with new toys is exhausting!

In medical news: the additional labs that GI ordered yesterday came back within normal range, indicating that her overall liver function is okay, despite the elevated ALT and AST. We’ll continue watching these numbers each Tuesday, but for the time being, no action.


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59 Days Old: Daily Update

Another short and sweet update.

Olivia’s weight today is 3165g, up 65g from her last weight. While she’s back on track in terms of gaining, Olivia’s been pretty pukey the past couple of days. This may or may not be a lingering symptom of last week’s infection and subsequent antibiotic regiment. We’ll see if her stomach and motility issues continue in coming days.

In other news: her direct bilirubin is down to 1.3, but ALT and AST are elevated, and continue to climb week over week. The GI team came by this afternoon and although they are quite pleased with the direction of her bilirubin, they ordered 2 additional tests to evaluate overall liver function.



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