Thursday, February 28, 2013

Info from Consultations

It's been a whirlwind month.  Most our time has been spent collecting information to help us decide which way to take Emmett's treatment.  Surprisingly, every doctor we've consulted has shed new light on Emmett's situation...every visit has revealed a new piece to this difficult puzzle we hadn't considered.  We're so grateful for the prayers that have sustained us through it all.  And now it's time to share.

Let me preface this by saying that we absolutely love our team in Boston, especially Dr. Chi, and we definitely know we came to the right place for Emmett to receive treatment.  It may seem like we've been at odds while collecting information from other institutions (which Chi has whole heartedly supported -- she loves to hear what other experts recommend in what she admits is one of her most difficult, ethically-challenging cases), but I feel that we've worked together very well in learning all we can to secure the very best for Emmett.

Ever since Emmett's 9/5/12 MRI which showed remaining bulk in his left brachial plexus the consensus in Boston has been that he needs more aggressive therapy including surgery to remove the rest of the tumor followed by high dose chemotherapy with stem cell rescue.  Clearly the surgery will be debilitating, and stem cell brings more side effects and higher risks, especially given Emmett's previous therapy.  The only complication was that the folks in Boston could not find a surgeon willing to do the surgery...except perhaps Allan Belzberg at Johns Hopkins.

We consulted with Massachusetts General Hospital in January and they recommended foregoing surgery and stem cell and instead essentially winding up therapy and watching Emmett closely.

We drove down to Johns Hopkins in Baltimore to consult with Dr. Allan Belzberg on 2/5.  Hopkins conducted an MRI the day before.  After reviewing the MRI with his radiologist Belzberg says there's nothing to get -- no cancer to target, even for a biopsy.  So there's no reason for surgery, which is great news, he pointed out to us.

In some previous communications with St. Jude they had mentioned that if you find a surgeon who's been into the brachial plexus five times then you've found the world expert.  Dr. Belzberg says he does five brachial plexus surgeries in two hopefully he and his radiologist know what they're looking at and if they say there's nothing to get, there's nothing to get.

We did get a chance to chat with Dr. Kenneth Cohen at Hopkins pediatric neuro-oncoloy because of Emmett's platelet troubles and he said that even with the scar tissue argument he would still recommend stem cell transplant -- "it's an awful tumor so you might as well throw the kitchen sink at it so you can walk away knowing you did everything."

When we sent word to Boston that there was no target for surgery they said they would reserve judgement until after they reviewed Hopkins' MRI.  To be fair, the Hopkins folks also needed time to compare Emmett's 2/4/13 MRI with his previous scans in Boston.  After reviewing scans Hopkins said the area was unchanged since 9/5/12 and any remaining bulk is scar tissue.  Meanwhile, Boston holds out that they believe they still see some bulk in the 2/4 MRI that could be tumor.  And if surgery is off the table because no surgeon will consider the case, then we should move on to stem cell.

We're a little confused that they could be recommending stem cell at this point.  In December they told us Emmett was incurable because without surgery to remove the bulk the stem cell team wouldn't put Emmett through transplant.  When we pressed them on whether they would accept Emmett if a biopsy revealed the tumor was dead, they said no because it would only provide info on the sample removed and would not prove that the rest of the bulk was dead.

And now two months later they're saying they'll put Emmett through transplant when the bulk hasn't been removed?  Yes, perhaps Hopkins' opinion on the MRI strengthens the argument that it's dead, but it still doesn't prove me it feels like the stem cell team is talking out of both sides of their mouth.

Either way, we're VERY glad we went to Hopkins and got their opinion on Emmett's response to treatment.  We hope and pray that the tumor is indeed DEAD AND GONE!

Now on to Children's Hospital of Philadelphia.

We met with Dr. Michael Fisher on 2/14 -- what better way to spend Valentine's Day.  He clearly had given Emmett's case a lot thought.  He shared with us that he had spoken with Dr. Chi about Emmett's case for about an hour on the phone the day before, and at the end of that conversation he still didn't know what recommendation he was going to make to us the next day.  But after more thought and consideration, a few insights helped him come to a conclusion.

One thing he did was go over the MRIs with a radiologist that specializes in reading this particular area in the body.  And what he learned from that radiologist is that the tissue that is showing up on the MRI is consistent with the type of scaring radiation often causes in the tissues in the brachial plexus.  Dr. Fisher pointed out that a brain tumor radiologist reading this MRI may come to a different conclusion because the rules that apply in the brain don't necessarily apply in other parts of the body.  And this is why he sought out this particular radiologist.  I can only assume that Dr. Belzberg's radiologist also specializes in the brachial plexus.

On the topic of surgery, Dr. Fisher admitted that he was the biggest advocate of an aggressive surgery when Dr. Chi first presented on Emmett at a meeting a few months back.  But after further consideration in preparing for our visit, his thoughts on the matter changed.  It likely isn't possible to get everything out that shows up as suspect on the MRI.  The suspect area brushes up against the spine, so if you were to go after everything the surgeon would have to remove the arm, shoulder and a few ribs.  And even then it would likely be physiologically impossible to get everything, assuming you could find a surgeon that would consider it.  And if you can't get everything, why would you ever do it?  And the fact that the radiologists believe that what they're seeing is scar tissue weakens the argument for surgery even more.

As far as stem cell goes, Dr. Fisher stated that all the data they have on stem cell suggests that it is not effective against bulk tumor.  So if Emmett has tumor bulk, there's no reason to do it because it won't cure him.  An awful lot of collateral damage for no gain.  And then if what they're seeing is indeed scar tissue, again there's no logical argument for stem cell.  The IRS-III protocol is what's been shown to be the most effective against AT/RT, especially when disease free at the end of induction chemotherapy.  If what they're seeing on Emmett's 2/4 MRI is scar tissue, and if the MRIs haven't changed since September, then Emmett has had no residual disease since he finished induction and continuing the IRS-III protocol is the way to go.

The one other thing I should mention about CHOP is that they insisted on conducting their own pathological review.  So I had slides sent from Boston and from New Mexico.  The slides came in at different times and unknowingly two different pathologists conducted a review of the tumor.  The first pathologist to review his tumor, who also happens to be the very pathologist that defined AT/RT years ago, decided that Emmett's tumor is undifferentiated sarcoma, not AT/RT!  She also pointed us to a paper showing that of five kids with undifferentiated sarcoma treated on the IRS-III protocol, all five were still alive at the publication of the paper, some over 20 years out from diagnosis!  The news knocked our socks off and rocked our world!  Then when we met with Fisher he shared that the other pathologist, who also consulted with Dr. Biegal who conducted the genetic testing on Emmett's tumor, decided that the tumor is indeed AT/RT.  And about a week after our visit with Fisher, we got an email saying that the first pathologist amended her report saying that she agrees that it's AT/RT.

But regardless of the final pathological decision, hearing about the other diagnosis put the idea into our minds that maybe the best thing for Emmett is finishing the IRS-III protocol.  And the other data we've gathered since that time has strengthened that resolve.

Dr. Fisher made it very clear that his professional recommendation of finishing the IRS-III protocol is not dependent on the pathology.  Undifferentiated sarcoma and AT/RT are both malignant, highly aggressive tumors.  And either way, he would still suggest we finish the IRS-III.

So as far as CHOP is concerned, goodbye surgery, and goodbye stem cell!  GREAT news to us!

So then we returned to Boston and got together with Dr. Chi and company.  While they appreciate the input from CHOP, they're standing firm in their position that stem cell is the best option for Emmett at this point.  They acknowledge the side effects and risks, but they feel Emmett will likely make it through the procedure okay and that long term this gives him the best chance of cure.  When I pressed them on whether they've had a brain tumor radiologist or a solid tumor  radiologist reading his MRIs up until this point they acknowledged it has been a brain tumor radiologist all along, though a solid tumor team has reviewed Emmett's case in conference (a solid tumor is defined as a tumor outside the brain and blood (blood tumors are considered leukemia) essentially a solid tumor is a tumor that's not in the brain, and this is the category that Emmett's remaining tumor falls into).  When we elaborated on the insight Dr. Fisher gained when consulting with a radiologist that specialized in the shoulder area, they agreed to have their solid tumor radiologists read Emmett's scans and provide feedback.

As we've learned more about the role of a solid tumor oncologist over the last few months we insisted on meeting with one.  Our appointment with Dr. Carlos Rodriguez, the solid tumor doc from the Jimmy Fund Clinic, was this afternoon.  We gained a few key insights.

Oncologists are always looking for ways to give higher doses of chemotherapy.  The limiting factor is the organs -- they can only take so much before they fail, and once they do it's over.  The organ that takes the most damage is the bone marrow.  But now the oncologists have found a way to protect and preserve the bone marrow so they can give higher doses of chemo -- high dose chemotherapy with stem cell rescue.

Brain tumor oncologists are especially prone to use high dose chemotherapy because of the blood-brain barrier.  The brain is like a sanctuary for tumor cells because it's protected from the rest of the body, and figuring out how to get at cancer in the brain is one of the challenges in treating brain tumors.  Higher doses of chemotherapy allow more chemo to cross the blood-brain barrier to where the cancer is.  This is why some success is shown in treating brain tumors with high dose chemo/stem cell, and so it comes as no surprise that brain tumor docs are keen on this therapy.  However, Emmett's case is exactly the opposite at this point -- the bulk tumor we're trying to treat is not in his brain...(and whatever may remain in the brain is receiving treatment via intrathecal chemotherapy)...and so the natural conclusion would be that the rules of treating solid tumors might be more applicable than the rules of treating brain tumors.

From the perspective of a solid tumor doc, Rodriguez would not recommend surgery as a therapy against Emmett's tumor.  They never go back in unless something is shown to grow, they always consider it to be scar tissue unless proven otherwise.  He would recommend another MRI in 6-8 weeks and see where things stand.

As far as using stem cell transplant against solid tumors, research in this area started about 15 years ago.  It initially was studied in treating a lot of solid tumors.  The data has shown, however, that it isn't effective.  Rodriguez said there are a couple of tumors that they do consider stem cell for, metastatic neuroblastoma and one other.  But that's it.  The many other tumors it was studied against, including rhabdoid tumors, showed no benefit from stem cell.  Especially against bulky disease.  There may be some effectiveness when it comes to systemic control, but none against tumor bulk.

Also, kids normally go into stem cell about six months into treatment.  Emmett is a year into treatment and has received much more therapy.  Rodriguez was very concerned about the amount of chemotherapy his organs will have already seen if he's sent to stem cell at this point.  Any percentage point or two gained in effectiveness against the cancer is almost assuredly outweighed by the increased risk of organ failure.

And so in summary. Dr. Rodriguez would not recommend surgery and he would not recommend stem cell transplant for Emmett.  Instead he would recommend finishing the IRS-III protocol with close monitoring.  If Emmett's tumor does come back there will likely not be much we can do -- no one has ever been cured of recurrent AT/RT.  But he doesn't believe stem cell transplant would increase Emmett's overall chances of survival at this point, especially considering the therapy he's already received.

We haven't discussed Rodriguez's recommendations with our team yet so we're not sure what they'll say.  Surely they'll still press us for stem cell.  But with all of the information we've gathered over the past month we feel we're much better equipped to make that decision than we were a month or two ago.

Not that we put much stock in rankings, but in the last year we've consulted with the four "number one" hospitals in the nation.  Boston Children's was the No 1 pediatric hospital in 2012 and CHOP was No 1 in 2013; Johns Hopkins was the No 1 overall hospital in 2012, and Mass General was No 1 in 2013.  If nothing else, hopefully this means the doctors we've consulted with are abreast of all the latest research applicable to Emmett's disease and are best qualified to help us decide which route to go.

Ironically, we somehow find comfort in the fact that there's no professional consensus on the best treatment path for Emmett.  If all the doctors were in agreement on what was right, and yet we felt impressed to go another way, there would always be opportunity to look back and doubt.  In this case, however, all options seem equally valid.  Clearly this is a very difficult case with no clear best answer, and no matter which way we go, there's little reason to look back and question.

Meanwhile, our doctors started another round of IRS-III maintenance chemotherapy on Monday (week 39) as they've continued waiting for us to gather information to make our decision.

Happy Pig Day!

We were unaware when we entered the clinic yesterday that it was a special day.  But it was and the place was decked out like a royal birthday party.  It's Pig Day!  (Wed-Fri this week, so we got to celebrate yesterday and today!) I guess 5 years ago, there was a patient who liked pigs and so for fun, she and the Resource Room Director started a pig party....and it apparently got bigger every year.  This was the 5th year anniversary and I'm pretty sure it was bigger than Christmas was at the clinic!  Plus, I got to meet the patient who helped start it all-she's still around-or at least she was yesterday.

Here's his clinic nurse, Erin, who's dressed in pink for pig day!  Emmett wouldn't look at the camera at all, but he's looking up at the tv, watching Mickey Mouse club, which Erin introduced him to during a port access one time, so him watching the show is the next best thing to smiling for us.  Erin's a lot of fun and we really like her!

 A big cake to fit the theme

 They had 'build-a-bear' pigs;  Emmett thought he was super cool for holding them both in one hand.
It took a few tries but he finally got a grip on both, thanks to lion's mane!
Pink bubbles entertained us all for a little while yesterday while he got chemo

There was a pig at each bed when we got back to the infusion room
 Here's the mastermind behind the party-Martha!  She is awesome and probably has the best job ever!
Emmett lately was into tattoos so every time we came into clinic last month, we would find Martha who would happily find us more tattoos.  She keeps the place well stocked and fun.  We love her!  Of course, he wouldn't look at the camera either, but had fun showing her how he can (kind of) blow ones, of course.  Do you love that she dressed up, too?!  Complete with pig slippers...I wish I'd taken a picture ;)  She's great!
 Pin the tail on the pig, goody bags with pencils inscribed with Happy Pig Day!!!, football pigs, stuffed pigs, pig pins, pink beads, walls of tubs of pink cotton candy, pigs on the walls everywhere....
Part of this is the lunch buffet, which was gone by the time I took this picture, but there were BLTs, pigs in blankets, pink lemonade....then pizza and salad and fruit and cheese.  
 A craft station with piggy banks to paint and decorate, as well as picture frame blocks. 
 They watched Babe on the big screen.  I asked her today if she was getting sick of watching Babe and she said No-because apparently there are tons of other movies they've had running.  Babe is the only one I could think of but she had a big list...Olivia, the Muppets/Miss Piggy, Babe -the 2nd one and a Christmas special...can't even remember but there were quite a few!  Emmett really liked watching Babe today.  We were both surprised how much he got into it-meaning he watched it for maybe 15-20 minutes.
 Pink candy buffet with cupcakes to decorate like pigs.  She even 
'reupholstered' the bench there so it was more festive.
 Here's Emmett with his piggy bank he painted pink!
  Micah playing cat's cradle with the pink beads
I'm telling you, she thought of EVERYTHING!
Emmett of course was a little too young to really appreciate some of it, but I thought it was so cute and fun...obviously! Emmett kept trying to walk off with the big pig balloons and loved painting his pig pink!  It was just fun to have something special and totally unexpected to cheer us up while we're at the clinic again. Maybe we'll put Pig Day on our calendar for next year ;)

 And I'll leave you with this last message (Emmett learned pig noises from Angry birds, so it's more of a grunt than an oink oink and it makes us laugh whenever he spontaneously makes pig noises.  Last year, he was more into angry birds and would try playing it a bit and would grunt alongside with the pigs and it was pretty funny...and we couldn't ever catch it 'on film' of course)

This is what you get....

when you ask a 2 year old to pick out his New February.
He still loves them as much as he did last summer!  Except his feet have grown...quite a bit.  His heel is hanging off at least a half inch in the back, but that didn't slow him down at all!  Luckily, it was almost spring weather today and we didn't have to walk outside from the parking garage to our appointment today, so why not?  Here's to hoping for more spring weather!

Sunday, February 24, 2013

Here's to a year!

One year ago today our lives changed forever.  The feelings of that day are forever seared in our memory -- the fear of Emmett undergoing emergency spinal surgery, the possibility of cancer, and the subsequent diagnosis that was worse than we could possibly imagine.  In many ways the life we had planned for ourselves fell apart that day...and the fear of losing Emmett crushed us to pieces.

And yet as we look back, the good we've experienced over the past year has far outweighed the bad.  Yes, we've had our bad days in the hospital, but we've had many more good days outside the hospital.  Treatment has been difficult, but Emmett has tolerated it extremely well.  Consulting with doctors and deciding between treatment options has been heart wrenching, but the faith and prayers of so many have strengthened and sustained us, and the hope and peace of the Gospel of Jesus Christ has continued to bring comfort and peace through it all.  And we've seen so many miracles, small and large, that have comforted, strengthened, and reassured us along the way...that we are now walking a new path, a new life, that God has chosen especially for us.

Our new appreciation for life has been life changing.  Every day has never been so sweet, every second with Emmett so precious, every smile, every laugh, every memory treasured so much.  We've never drunk of the cup of life so freely and so deeply as we have in the days since diagnosis, and that new appreciation for life has made us new people, a new family if you will, that will never be the same.

I never thought I'd relate to a country song, but Tim McGraw's "Live Like You Were Dying" really hits home.  And I wish everyone had that opportunity...because it changes you...forever.

So here's celebrating Emmett's birthday...and the birth of our new life as a family.  Perhaps we should call it "Miracle Day" as it truly is a miracle that Emmett is alive and well one year after being diagnosed with AT/RT.  May we celebrate many more.

Thank you all so much for your prayers and faith which have helped bring about this wonderful year for our family!

(the video's a little long but parts are entertaining)

Sunday, February 3, 2013

Another one...

Also from the fall - I was surprised to see this on my phone one day!  A dear friend (mission companion) from a decade ago (I can admit I'm that old because someone asked me today if I was Emmett's sister.  Then he said he thought I was probably 18 and definitely didn't look like his mom. I could have kissed him! Just kidding....I'm not that worried about looking old, but how does a comment like that not make you smile?!) chopped her hair in support of Emmett!  I told you- we are never short on feeling the love!  Thanks!!

Uncle Jason's Visit

We weren't kidding when we said we were behind on posting! I guess fall was a very busy time for us, so here we are reliving it by blogging it months later! Micah's brother Jason came to visit in September. I am realizing that he was apparently the official photographer of his trip because he's not really in any of the pictures, even though we did a few fun things with him while he was here.

What day this summer would be complete without a visit to the truck?! 
Here we are on the back patio of the house and the truck was usually parked in the backyard, too.
We went apple picking with cousin Tyson

Which included a little hayride, too - the little boys loved it
They had a petting zoo (with probably the fattest pig in existence) and a duck pond, where Lion took a swim
The ocean was nearby the orchard, so Micah & Jason ran out to the beach while
I stayed in the car with Emmett, who was almost asleep.  He loves the beach and
we knew if he saw it, we'd be stuck there for the day.
September 15th was my brother Cris's birthday and Jason recommended 
going down to Providence, RI for WaterFire so we celebrated in style with a night out.
I'm not sure we knew what to expect, but it was pretty awesome.  There are a bunch of cauldrons on this river that runs through the middle of the town and on certain nights during the summer/early fall they light them all. There are of course vendors up and down the side of it (we stood in line no less than 15 minutes to for one bag of popcorn) and music and these cool boats in the water with lit-up fish on them (hard to describe, but you can kind of see the bright blue fish on the back of a kayak in the bottom of this picture).  It was fun to be outside and definitely a totally unique activity we enjoyed!

Emmett let us know it was time to go.  He's such a trooper to put up with all our partying!
Sunday afternoon we took a walk around Horn Pond
Emmett always loves throwing things in the water.  
There is no doubt that this hobby came straight from Micah!
 Hey, I'm out of ammo...can you restock me please?
He likes throwing sticks, too, but those are a little harder to get all the
way down to the water & see a good splash

We did a lot of playing outside, as it was still really warm in September. 
The week Jason was there was the same weekend they delivered trucks 
to the yard, so he got to be there for that, too.

And just one day in clinic.  Not bad!

We're so glad Jason got to come visit. Emmett enjoyed having him around to play with!

MRI Escape

Back on Sept 5, Emmett had his 'big' MRI after his first phase of chemo. He hadn't had a scan since May, so it was a big deal to see how well treatment had worked. We had decided beforehand that after the scan, good news or bad, we'd just go away for a few days and enjoy some fun time to ourselves. By the time we got out of town that night, it was pretty late. We went down to the Cape (Cape Cod) and stayed in East Sandwich for a few days in a little cabin. It's a small beach town. We went out one night to this family owned pizza shop.  There were a lot of take out orders, but we were the only ones in there, as it was past normal dinner time.  We had stayed at the beach throwing rocks until every drop of sunlight was gone.  The owners started chatting with us and turns out his oldest daughter had been a patient at Children's when she was a baby also.  They were a very kind family.  When we were finished, they wouldn't let us pay our bill at all and let Emmett help himself to another orange soda, for the road, out of the cooler.  I didn't realize this because I was picking up our stuff when Micah went to pay.  I didn't get a chance to say thank you because we were in the car when Micah told me.  We are continually amazed at the goodness of strangers.  Of course our natural reaction is to insist that they don't need to do this for us, but strangely, we have started to let go of that reaction.  Partly because truthfully, you hardly ever win that argument in our situation and partly because it is somewhat insulting and rude to refuse a gift like we're too good for it, but mostly because we feel that is one of the few benefits to Emmett being sick.  Not the benefit of free pizza, but the benefit to see the goodness of so many people, their small and large acts of kindness, not wanting any praise for  it, just doing it out of the sincere goodness of their hearts.  You will never read about it on the news, which makes you feel like all there is left in this world is greed and selfishness and fraud and danger and crime.  The benefit of being in our shoes is that I know there is plenty of good left in this world and we couldn't even post about all of it that we have personally seen, let alone all the kindness that is shown to plenty of others that we don't know about.  There are LOTS of good people 'out there'!

Apparently we didn't take a lot of pictures, or I just haven't searched long enough to find them, but here's The Man. Not tired at all, ready to go, despite it being 10:30 at night. He loves going new places and exploring. That is one part of his personality that does very well with his life-hospital, hotel, cabin, Ronald McDonald House, our home away from home in Boston, our visit to Michigan....wherever it is, Emmett sleeps well, loves being in new places and has no problems with traveling. I hear that some kids don't sleep well if they're not in their own crib/bed, or it takes a while to adjust to a new place. Yay for Emmett being such a flexible kid! (or maybe he's so flexible because he has moved around so much? Either way I'm not complaining)  And yes, those are purple pants!  From the hospital.  He's grown out of the 'white size' and the next ones up are purple.  Looks pretty good on him, I must say.  PS Besides orange, I'd say his favorite color is pink. 
After a few days on the Cape, we spent our last night up in Maine at York Beach. 
This beach (or at least the time of day we went) was very kid friendly-the water was really shallow for a while the evening we went and Emmett loved it.  He could splash around without being taken down by the numbing cold of the water (truthfully, I think Plum Island is the coldest; these other beaches aren't too bad) or waves crashing in on him. 

The next morning we went to Nubble Light House.  You can't actually go over to it, but it's pretty neat to see from across the water.  There are sometimes scuba divers that dive around in this rocky water.  The waves crash pretty hard on this rugged area.  Emmett was pretty brave climbing these big, slippery rocks.
We actually bumped into one of our 9N nurses while we were here!  (Heidi-we'd actually had her for the very first time the week before;  otherwise, I'm not sure I would have recognized her so quickly)
There's a little Family Park near York Beach so we got Emmett a pass....
and I think this is the only time he smiled!
He hated the carousel, even with Micah holding him the whole time!
So we didn't even try to get put him on the train alone.  
He was ok just watching all the other kids ride it, though.

Luckily, there's also a zoo!  This was a closed in area with a bunch of deer and they would wander in and out of the fences and come up to you (especially if you had a handful of food).  We thought it was kind of cool.  Can't say I've ever seen deer in a zoo before.

There were also some lions.  Two of them.  I think Emmett had been totally spoiled by the Franklin Park Zoo Lion, because every time we've gone there, the lion won't sit still!  So these 2 lions were just sitting a ways back from the fence, mostly sleeping, and barely moving, until one woke up and opened his eyes and lifted his head a little bit.  Emmett is very creative. He knew exactly how to solve this problem.  All on his own, he walks away from the lions and goes over to a tree, picks up a handful of mulch, walks back to the lion cage and throws it at them.  Of course, it only goes about 8 inches and is no where near them (which is probably a good thing.  I doubt they encourage throwing things at the lions) and Emmett tries this technique multiple times, with no luck.  He was trying to feed them!  We thought that was pretty smart of time we'll just have to go see our other lion instead :)