Today was the annual Christmas Concert at the kids’ school. Amber, Ryan, Brianna, and Marie have been practicing their songs for weeks, and were ready to get up on stage. But, Amber wasn’t destined to perform today.

Yesterday, we had a follow-up with the opthalmologist. As the exam was wrapping up, and she told us that Amber’s eyes looked great, she took one quick last look and just sort of made an off-hand comment to the effect of “huh, she seems to have a very slight difference in pupil size”, but didn’t think it was anything of concern.

This morning after breakfast, because of that comment yesterday, Caron was looking at Amber’s eyes and noticed that the pupil of Amber’s right eye was a little larger than her left. On one hand, this immediately set off alarms for us both – when Amber was first brought to the ER at Backus Hospital, 262 days ago, the first thing Dr. Adams noticed was her enlarged right pupil; and then, during our next 50 days at CCMC the pupils were checked all the time for signs of pressure in the brain. On the other hand – we have witnessed this poor little girl go through so much, physically and mentally, that it can be tough to determine the right level of concern. But, even with the news this past week, this difference in the pupils was unexpected, so we called CCMC. After a quick exchange and deciding that while it /might/ not be anything of concern, it’s better to err on the side of caution, I brought Amber up to the ER at CCMC for a quick check, while Caron brought the other three children to their concert.

Once at the ER, they immediately start checking out Amber’s eyes, getting her vitals, reviewing the latest notes from MSK, and I end up repeating the story of the past 262 days several times as each new person enters the room, as it is probably faster for me to tell the story than for them to read the encyclopedic volumes of notes to get up to speed.

After some challenges getting the most recent MRI from MSK, they decide to put her through a quick CT Scan. Unfortunately, they do see some unexpected changes in the tumor/mass, which is causing some irritation and swelling, putting some pressure on the brain.

So we start discussing options while waiting for radiology to be ready for us for an MRI. Dependant on the results of the MRI, we might be considering something as simple as a steroid to reduce the inflammation, or might be considering immediate surgery. Or, possibly the worst case, doing nothing at all.

So then off into the MRI for the one-hour scan. As always, Amber is a trooper. She doesn’t want to be sedated, no movie or music, doesn’t even want ear protection (but she does eventually have to give in on that one).

Following the MRI, Amber has been admitted to the PICU, where we are right now (about 11PM). Amber is sleeping, receiving a steroid drip. We will determine in the morning if this works and gives us the time to develop and pursue a plan (as we have been anticipating), or if we go right into surgery tomorrow.

It’s rather surreal to be back here in the PICU, where we spent the first two weeks of this journey, starting 262 days ago. Nurse Alex is here, as well as Nurse Steve and Respitory Tech Craig. There are new faces too, of course, but these familiar faces are weirdly comforting. We wish we weren’t here at all, yet there is nowhere we’d feel more “comfortable” right now.

It’s going to be a long night. We hope that this is just a speed bump on the way to a better plan, but we won’t know till morning. Until then, we pray. There is nothing else Caron and I can do; this is in God’s hands. So as we have before, we pray that He gives strength and clarity to the skilled medical professionals who are determining the best course of action for Amber. And we pray that Amber rests comfortably and that God strengthens her for whatever comes next. And Caron and I will figure out what’s next after we hear from the team tomorrow morning.

It’s been almost 3 weeks since our last post.  It’s been an interesting few weeks, with ups and downs.  But most importantly, this week was Thanksgiving.  Even with the roller-coaster that life has been lately, we made sure to stop and enjoy the long Thanksgiving weekend as a family, and to really focus on what we are thankful for.

Since our last post, Amber had her third infusion 2 weeks ago.  The infusion went smoothly, but while we were at MSK we discussed some pain Amber has been complaining about in her right eye.  We were able to see the oncological ophthalmologist, who took a look and saw nothing immediately concerning, but gave us some ointment for a stye that had developed on Amber’s right eye.  As the next couple of days continued, the pain increased, and Amber came home early from school twice.  MSK advised us to see our pediatrician, who also didn’t see anything that should be causing pain, but he immediately got us into the ophthalmologist at Yale-New Haven.  After almost two hours of different doctors and assistants looking in Amber’s eyes, they came back with their findings — she has dry eyes!  So, we started giving her some simple eye-drops, and this has helped significantly.  It hasn’t made the pain totally disappear, but it has made a huge difference.  We are very thankful to be able to reduce her pain, and not have this be something more serious.

This past weekend, we got to visit the residence of Little Audrey Santo. Audrey died in 2007, but experienced miracles and may some day be Canonized.  We met Audrey’s mom, and talked about Audrey’s life.  Reverend Peter Joyce joined us, and gave a blessing over Amber, asking for Audrey’s intercession.  Having Father Joyce is quite coincidental – he delivered the Wedding Mass when my brother got married in 2012, and the Funeral Mass when my brother died in 2016.  There have been many similar “coincidences” over the past 249 days, so maybe his appearance at this most recent event in our lives wasn’t just accidental, but rather, is evidence of some larger plan in the works.  Seeing Father Joyce, having Amber receive a blessing, and praying for Audrey’s intercession was all together an incredible experience, and we are very thankful to have some really good friends (who have helped us quite a bit through this entire ordeal), who set this up and joined us.

And then we had this beautiful Thanksgiving weekend.  We hosted (and by “we”, I really mean Caron — I just try to stay out of the way) my parents, my aunt, Caron’s parents, her grandmother, and her brother and sister-in-law — along with our own family of 6.  It was a beautiful time, some time to relax and enjoy a meal with the family, and ponder what we are most thankful for (I’ll say more on that a little later).  On Friday, while many people were enjoying the chaos of the shopping malls, the six of us went and found our Christmas tree, and then decorated it on Saturday.

It was while decorating the tree that it hit me, what I’m really most thankful for right now.

As I’m watching the kids hang their special ornaments, I forgot for just a moment that Amber has a serious illness, most likely deadly.  For a few minutes, she is “just” one of my children – laughing, enjoying herself, talking about Christmas.  But then I remember, and struggle to maintain my composure as I realize that statistically, the odds are against Amber being with us to do this again next year.  I remember that right now, the tumor is most likely still growing, and that we may be considering alternative plans over the next couple weeks.

But then I also remember that we have this time together.  March 22 could have been it, could have the last time we had Amber with us.  But she is still here.  She is still happy, and we still have the possibility for a positive outcome.  We have our whole family, right here, right now.

And so, even though the past 249 days have been difficult for all of us, and Amber most of all, I am thankful for this time we have together.  However short or long it may be, we are going to appreciate every moment we have.

We now look to the next MRI, scheduled for next Monday (8 days away).  This one should provide some direction — either the treatment is not working, the tumor is progressing, and so we need to consider alternative plans; or (somewhat less likely) the treatment is working and the “growth” that was apparent in the previous MRI was just “pseudo-progression” and we will start seeing the tumor shrink.  So for now we pray simply to be pointed to the right path, whichever gives Amber the best chances.

Just when we think that we are in for a “yes” or “no” type of result, life reminds us that their is always room for “maybe”.

Amber had her MRI today. The results show some increased growth since last time, still in the same location as the original tumor. We were hoping to see some reduction, so of course this is disappointing. But, the Doctor gave us some interesting detail – it is not uncommon for this medicine to cause some inflammation of the tumor at first, and this would show up as we saw in today’s scan.

So unfortunately … we are squarely in “maybe” territory. It’s possible the medicine is having no effect and the tumor has grown a bit, and it’s possible the medicine is having a positive effect and has inflamed the tumor.

We had a good conversation with the Doctor, basically coming down to two options: we could abandon the current treatment and move to something else, or we can hold steady for now and continue to monitor the growth. More conversation will happen at next week’s appointment, but we are already fairly settled that we will continue with the current treatment for now.

So … “maybe” we are making progress? We pray that we are.

Today was another trip to MSK in NYC, for Amber’s second dose of pembrolizumab.  As always, this started with checking Amber’s vitals, doing some blood work, then waiting around for a while until a room is available for the infusion.  Amber had a little trouble with it this time, getting some flushed cheeks, a minor fever, and a little stomach ache.  Nothing unexpected, nothing major (the list of potential side effects is very long and scary), but still meant needing to hang around a bit longer for observation – plus Benadryl, Tylenol, Famotidine, and Ceftriaxone (and antibiotic, just in case).  Amber’s a trooper though, taking it all in stride.

I’m reminded of our time in the hospital room at Connecticut Children’s Medical Center.  The beeping of the machines, Amber being connected up to the machine supplying her medicine, her lying in the bed napping.  But beyond all of that, just as I was at CCMC, I am again amazed by the level of care the nurses provide.  Not just the knowledge they have in their field, but the human, caring, supportive approach they take with Amber, and with us.  At CCMC, our emotional state survived only through the help of the nurses; and while we certainly hope to not have to spend 50 days in a room here at MSK (or any other hospital), I see MSK’s nurses share that same critical quality.

We also got the results of the genetic testing from MSK’s genetics team.  All the tests came back negative.  This is a mixed blessing – on the positive side, this means that there is little chance of our other children being at risk due to genetics; however, this also means we still do not have any clear cause for all of this, and so cannot target her treatment to that cause.

So, we keep doing what we’re doing – weekly trips into NYC, weekly OT and PT sessions, watching Amber all the time for anything that causes concern, keeping her happy and moving forward, and praying – lots and lots of prayer.

Just back from a couple of days in NYC for Amber’s first dose of pembrolizumab. We went in on Sunday so we could be well rested for a 7:45 AM start on Monday. After an x-ray to confirm her port is looking good, we continued on to a loooong MRI, looking at both the brain and the spine. Amber is a trooper, as always, and did great. Then, we walked through Central Park on the way back to the hotel, enjoying a little fresh air and exercise.

Today (Tuesday) we got to start a little later, going in at 11:30. While waiting for confirmation of lab results and getting the pembrolizumab prepared by the pharmacy, the doctors and nurses came into Amber’s room with a birthday cupcake, gifts, and sang Happy Birthday to her! It was a beautiful gesture by the group, as they get to know us and we get to know them. They are certainly doing everything they can to make Amber (and us) feel comfortable.

While we were waiting, Dr. Kramer also fills us in on the results of the MRIs – both show as expected. A little growth since the last, but nothing to cause any additional concern.

Then came the infusion. With all the build up to this, and with all the importance we place on it, it was remarkably (and thankfully) unimpressive. Amber is hooked up to an IV (via her port) for 30 minutes, and the medicine slowly pumps in. And then they disconnect her, and send us on our way. It was almost a “really, that’s it?” kind of moment – but I’m not complaining. These were incredibly long days for Amber (and for us). But if this works as we hope, and it can be this non-invasive, we will consider ourselves blessed.

So now, we have to come back to MSK weekly for the next 6 weeks for routine labs and vitals checks, and then we go to three week cycles. We pray this works, that the tumor’s growth halts and then begins shrinking. It’s going to take some time to see results, though, so for now we wait and pray.

Amber, Caron, and I jumped on a train into NYC this morning, to go visit Memorial Sloan Kettering and make a decision about the trial we are going to pursue.  It was a gray, rainy day, kind of fitting our mood.  It’s funny how Caron and I flip back and forth in our moods – sometimes sad or angry that Amber is in this situation, sometimes inspired by Amber’s determination and the support everyone is giving us, sometimes hopeful that there is a path forward and God is leading us in the right direction.  And that whole emotional roller coaster repeats roughly every 5 minutes of every hour of every day.

Much work has gone into this visit with MSK already, with CCMC working with multiple hospitals to determine what trials Amber is eligible for, ultimately identifying a few at MSK that have the greatest potential.  So today, we met with Dr. Kramer again, and then met with the geneticist.  For now, we are pursuing two paths: genetic testing to determine if there is an underlying “predisposition” (a root cause) to attack, and a trial of a drug named “pembrolizumab“.  While the doctors did a great job of explaining terminology while still going into depth, for sake of this blog post it boils down to this drug helping the immune system get past the defenses of the tumor and attack it.

So, next week (hopefully – schedule TBD) we return to MSK for an MRI, X-ray, and to begin the infusions.  The infusion takes about 30 minutes, and then we return every 3 weeks for another infusion and an MRI on alternating visits, possibly for the next 2 years.

If this drug does not appear to be helping and the tumor continues to grow, our current “fallback” plan is to leave the trial and go in and surgically remove the tumor.  But given the risk of brain surgery, even in Dr. Martin’s skilled hands, we are going to pursue this trial first.

And we will keep investigating other trials, treatments, options, trying to make the best decisions we can as we go.  Please pray that we find the best options and make the best decisions along the way.

The MRI of Amber’s spine came back clean!  This is very good news!

We still have to go after that one tumor that was found last week, but for now, we wait.  The MRIs and other results have already been sent out to a few places, and hopefully we hear back next week about which next steps may be appropriate based on these results.  Then, we choose which we are going to pursue.

Some big decisions ahead.

Thank you to all that have been praying and thinking about Amber, and our whole family.  Now, I ask if you could please pray that Caron and I be granted the clarity to make the right decisions for Amber.

Amber is getting her spinal MRI right now. She went in about 6:00 PM, and it will take roughly two hours. It looks like we will have to wait until tomorrow to learn the results, so please don’t worry if you don’t see us post about it tonight – but we will post as soon as we learn them.

Update – Amber is out of the MRI! But, no results tonight – we will post as soon as we hear, hopefully tomorrow.

Tomorrow evening Amber goes into the “Truth Machine” (MRI) again. Following the discovery of some tumor regrowth this past Friday, we now are going to check if there has been any spread – with a focus on her spine. Spreading to her spine would be a particularly bad development, so we are praying that this comes back clean.

Caron and I also want to thank everyone that has been part of this with us. While we rarely have the oppportunity to respond to them all, we do read every comment left for us on this blog, sent via Facebook, email, voicemail, text message, postal mail, and other messages, and we so appreciate the Masses and Holy Hours for Amber – the support means so much to us.

Thank you all, so much – while this has been a difficult time for Amber and the whole family, we do feel blessed to have such a community around us.

The MRI results today did show “recurrence” – some regrowth of the tumor.

Of course, this isn’t the news we’ve been hoping and praying for. At the same time, though, this doesn’t mean we’ve lost the war. The regrowth is fairly small, and we know where it is and what we are dealing with. Over the next week, we’ll review options (some of which are already in motion), make decisions, and move forward with treatment. When I think back almost 6 months to the beginning of this journey, we are in a much better position now than then.

We have been preparing ourselves for this possibility. But it still hurts, a lot. This is not what any of us wanted. As her father, I just want to take this from her – my six year old daughter should not have to go through this. But, we also haven’t been fooling ourselves, we knew that recurrence was likely, this type of cancer (GBM) doesn’t go down without a fight. We are prepared for ongoing battle.

The next step is another MRI, looking at the spine to make sure this thing hasn’t spread.  This hasn’t been scheduled yet, but should be early next week.  This will help determine which of the potential next steps we are going to pursue.

I am so appreciative for Dr. Gillan and Dr. Martin (and all the innumerable staff that we’ve met at CCMC). They are very direct, yet sympathetic. They understand what kind of detail we need to hear, and how to convey it so we can understand it. They care about Amber, and about us. We know that we were brought to them for a reason, and that they are fighting this battle with us.

And we continue to be amazed at The Good that has come from this journey, in the community that has formed around Amber and the impact we have been told she has had on others.

And so we continue to pray, and ask for prayers for Amber and the medical professionals helping her. We continue to work with Amber on her physical recovery from the stroke (her progress continues to be incredible), and continue to attack this cancer with everything we have. And we’ll just keep moving forward, one step at a time.