Monday, May 23, 2016

MRI Results

Everything looked fine on Emmett's 4/28 MRI!  This MRI only included the spine and brachial plexus as those weren't included on Emmett's 3/3 scan.  They did note one spot at the C6 level that might be questionable, but looking at previous scans they can see the same thing all the way back to 2014, and it's unchanged today, so they're not overly concerned about it.  (We think this might be the same spot near Emmett's primary tumor that the Boston calls a "tethered spine" and has been watching closely?)  Emmett's next scan is 6/2, and that scan will include the brain, brachial plexus, and spine.

Because there weren't any tumors identified in the scan (yay!), Emmett is still not a candidate for the EZH2 study.  This is the study we've been hoping for because it has no significant side effects.  This means that any treatment we consider at this time will likely have a negative impact on Emmett's quality of life, and that's never fun.  If Emmett were experiencing symptoms from a tumor currently, it would make the decision to put him through harsh treatment easier to make.  But as it is, it seems the doctors are really reluctant to make him sick when he's so healthy otherwise.

The one thing that's become clear to us is that there's no clear, best path forward in the doctors' minds.  In some ways that's a good thing; it means Emmett is doing well and things look good enough that it's hard to justify making him sick.  At the same time, we do wish that the best treatment plan to help ensure Emmett's long term survival was more obvious.

Thursday, April 28, 2016

MRI's Over

Emmett completed his MRI this morning...and the boys are watching iPad together again.  On our way home.  More later.

Sunday, April 24, 2016

No news is good news!

Sorry it has been a while, but despite being a few months, nothing has really been happening.  yay!!

We came home the day after radiation ended back in January, thinking we would have a few weeks at home, an MRI then right back to Boston.  But, as scheduling usually goes, it took 2 months to schedule an MRI (which in the end, he did UNSEDATED!!!!  His first ever!  It was only a half hour and only the brain, but still, that was huge!  Had we known he wouldn't need anesthesia, it probably could've been scheduled much sooner).  Then to get results back...another few weeks.  In fact, it took a full month to hear back from our team.  Here are the results of 'the spot:' UNMH compared it to a different scan, so they concluded no change.  MGH, where Emmett did proton radiation, concluded about a 20% shrinkage and clean auditory nerves now.  Dr. MacDonald, our radiation oncologist, was pleased with those results she said and strongly recommends a systemic therapy now.  DFCI, our main team, reported they see 50% shrinkage and concluded that the spot is very radiosensitive and hope for continued shrinking.  Initially, when we saw UNMH's results, we were excited because if it hadn't responded at all to radiation, that strengthens the case that it is NOT cancer.  Because of that news, we felt that we would be done with treatment for now and just continue to watch and scan like normal.  However, just a few days later, that peace was brought to a quick end with MGH's news.  I mean, shrinking for cancer is good news, but 'probably not cancer' is obviously better.  But we were very happy that the doctors were seeing good results from the radiation. We are back to considering our next treatment.  Dr. Chi is still not as strongly convinced we need further treatment as Dr. MacDonald is. 

Here are the options we're considering for those who like details or are reading this blog in search of direction for their own child's treatment.  The first is a study from the company Epizyme, who has produced a new drug called EZH2.  This is Dr. Chi's research and is not traditional chemo, but a new type of drug that is hopefully the way treatment is going in the future.  Instead of defining cancer by where is occurs in the body, they are defining tumors by what they genetically have in common.  For ATRT, it is an INI-1 deficient tumor.  This gene normally is what codes for tumor suppression, or what recognizes tumors and then tells the body to squash it.  So you can imagine if you have a mutation or genetic error in this gene, then your body does not produce the right proteins to send the signals to squash the tumor and becomes very problematic.  What this new drug does is hopefully treat this deficiency.  (At least this is my basic understanding)  They have given the drug to 5 adults and 4 of them had shrinking; 1 of which had the tumor totally gone.  We are told this is HUGE success for such an early study.  Fast forward maybe 2 years and they are just giving this to children now:  6-12 children worldwide have taken it starting in February.  The side effects so far seem to be mostly tiredness.  This was funded NOT by any donations to the Jimmy Fund but entirely by a certain team for bike ride fundraiser, headed by an incredibly enthusiastic mom who already lost a child to this disease.  Janet Larkin O'Shea and her team, Team Lick Cancer, have raised around $250,000 (yes a QUARTER MILLION DOLLARS!) each year and give it to Dr. Chi.  THAT is how needed research for children gets funded.  Thanks to them, we have options for treatment as this point.  She said when her daughter was sick, they would do radiation on the little spots as they popped up and do some chemo here and there, but there really weren't options.  Emmett was a pedal partner for this team 3 years ago and will be again this year!  I thought we had a post on the PMC, but apparently we don't because that was a pretty busy time of life.  I will put that on my to do list.... 

While we are excited about the new EZH2 drug, we are a little hesitant because really, who wants their kid to be a guinea pig and work out all the kinks of a new drug?  And also, the whole purpose of this particular study is to find out the dosing, which means, how much can they give a kid before the liver and kidney or other organs start having problems.  Just in this last week, we have heard that 3 of the kids on the study just dropped out because their tumors grew.  Which makes us hesitant.  We also found out the adults that did well had solid (body) tumors so the drug could easily get to it.  With a brain tumor, a drug has to be able to cross the blood brain barrier, which is made by divine design to be hard to cross, thus protecting your brain.  Maybe at a higher dose it will be able to and if we join the study, it will hopefully be a little bit higher of a dose.  This is what I mean by working out the kinks.  Emmett's quality of life right now is AMAZING and completely asymptomatic so we are drawn to this study because it seems that immediately it will have less impact on his quality of life.  The children are closely monitored and the drug is given daily orally at home.  But we would still have to be in Boston for 1-2 years (you have to be in clinic to get the drug very regularly and get your check ups and lab draws....  They do offer this study in Denver, so if we choose to go this route, our doctors have agreed that we could work with Denver, too, which is a 6-7 hour  drive away.  That would be a lot of road travel, but it could be done and would allow us to be home much more without the expense of having to fly to Boston).  Right now he does not qualify for this study because his pea size tumor is now half that size thanks to radiation and if it continues to shrink they are not sure it would be from radiation or the new drug, so it wouldn't give accurate study results. More on this later, but he has an MRI this week and if new spots are found on the spine, then he would qualify.

Second option right now is called the Vienna study, and -you guess it-comes from Vienna Austria.  It is traditional chemo, and while it is called a study, it is not really, so it could be given at any hospital that is familiar with interthecal chemo, which I don't know if UNMH is, but we would not choose to do chemo at home anyways, so that doesn't matter.  Dr. Chi said that although it is chemo, it's not nearly as harsh on the body as the chemo he has been through.  However,  it is 'labor intensive'/time consuming, in her words.  Part of it would be interthecal chemo, which is the one they put through his Rickham Reservoir right into his brain.  This is done 5 days a week, every third week and obviously  has to be done in clinic.  It involves systemic (IV) chemo, interthecal (brain) chemo and radiation, which he has already had.  This is about a 1 year protocol also.

The third option, which we haven't considered as much so I don't know as much about is Alisertib, which is St. Jude's research baby right now.  We have heard of other families on it with mixed results.  Also pretty new and not readily available, but possibly we could get it in Boston, but Dr. Chi would have to apply for compassionate use.  I believe it is not 'traditional chemo' (which I define to mean drugs that kill any quickly dividing cells-cancer, hair, throat lining, etc...) but I think it is being used in conjunction with chemo.  I guess another option with this is possibly going right to St. Jude in Tennessee for this.

Last option would be to wait and see...?  Dr. Chi says they do 'spot-weld' small tumors with radiation sometimes and don't do further treatment.  But the haunting question from Dr. MacDonald always remains "But if it has traveled to his brain, where else is it traveling?"

To be honest, Emmett is doing absolutely great still and we are still very slow to call him in recurrence and have clearly not been overly anxious to get treatment started.  He will have another MRI this week because the initial MRI on March 3 only checked the radiation spot and not to see if it had spread down his spine or in the old tumor bed.  This MRI will be 4 slots instead of 1 like the spine is, so it will definitely be sedated.  They said splitting it up into shorter ones without anesthesia makes for choppy pictures and also means he has to have contrast injected into him 4 times instead of 1, which isn't ideal either.  We are very hesitant to disrupt his quality of life and are very hopeful he's still fine and will be just fine.  He's very bright and excited to be starting kindergarten in the fall and besides some weak muscle tone, really has no side effects from previous treatment.  We are hoping not to be pushed urgently into treatment and kind of hope radiation will just do it's thing and maybe do some more treatment just in case, but expecting him to be spot free and clean and life back to normal sometime. 

We're grateful for all the prayers and have loved being home some extra time.  We still really don't know where we will be in a few weeks, but are starting to expect that by early summer we will be headed back to Boston to discuss and start treatments.

Phew, enough of all that serious chit chat.  On to the fun stuff!!

Team Lick Cancer's Jersey

Family Photoshoot right before we left for boston in the fall-we had literally walked out of a doctor appointment and had tons of packing left to do to leave town the next day....but somehow between all the chaos we fit this in and we're glad we did

oldie but goodie
the whole team in 2013 at the end of the 2 day ride in Provincetown, MA

my numbers were way off-looks like they raised almost a half million dollars in one year!  That is the complete funding for this EZH2 study.  Absolutely amazing!  Janet is 2nd from the right and Dr. Chi is holding the check next to her

 Our Annual Winter Family Campout     

Matching Easter-they both fit in last year's clothes! Score!

Micah graduated from his neat internship

Showing off eating skills to his awesome and tough uncle
'Honey I shrunk the kids' garden

First suit!
on a bug hunt
brother love
Flashes of Hope (before we left Boston-photographers volunteer their time and do a free photo session and prints!  IN clinic, which is AWESOME because it's one less appointment and they totally accommodate you)
visitors at the temple
Breakfast in bed...i mean bath
Watching General Conference and taking notes
friends from az!!
top of Sandia Peak

'Big brother excavation co' and 'l'il bro wrecking co' how cute is that?  My sis is pretty sweet

Monday, January 18, 2016

Radiation ends, and a trip home!

Emmett's last day of radiation was 1/8/16.  He ended up doing very well throughout the whole seven weeks of treatment.  After getting his port-a-cath placed the week after Thanksgiving, he wasn't afraid of the sedation every day and actually looked forward to going in and getting his 'doctor nap' each morning.  Sometimes he would get hungry because he couldn't eat until after he woke up from sedation each day, but besides that he did very well.

The doctors want to wait six weeks before doing Emmett's next MRI to give the acute radiation side effects a chance to subside (radiation can cause inflammation of the surrounding tissues, and they want that swelling to subside before the MRI).  In the interim, they let us go home for a break.  We flew out on the 9th and have thoroughly enjoyed being back in New Mexico this past week!!  I cant't express how great it feels to be home for a while.  What a blessing and a miracle!

Emmett continues to do well clinically.  There's no indication that anything might be amiss.  We pray that's truly the case.

Emmett was excited for the plane ride back home and couldn't wait to see all his old toys and ride his jeep.  He was such a big boy walking through the airport with his own backpack that the proton center had given him as his graduation gift (he's been asking for one since before awesome it is for a child to want a practical gift for once).  We threw all his toys and goodies in his backpack and suitcase, and it was awesome.  I guess there are small benefits as your kids grow up.

However, there are downsides too.  The worst part of the flight home was when Emmett realized that the button on the armrest doesn't make the plane go up and down.  He's believed it controls the plane for years, and it's been the cutest thing ever to watch him wear out his fingers during takeoff and landing...but you have to take the bad with the good as your child grows up, and sometimes it melts (or breaks) your heart in the process...

I initially blew off his questioning, but he insisted: "No, come on Dad, tell me what the button really does".  So I eventually gave my big boy... *sigh* ...and the guy sitting behind Emmett cursed me for the rest of the flight.

Emmett had a hard time sharing the window with Clayton.

We're so excited to be home and look forward to enjoying what home life has to offer for the next month.  Afterwards we'll likely head back to Boston for some continued treatment, though exactly what that will entail is unclear.  The doctors want to be proactive in case this has spread elsewhere.  Dr. Chi listed five options we could consider before we left Boston, and we'll be weighing those carefully over the next month.

But for now, we're enjoying life and living it up in the Land of Enchantment!

Sunday, December 6, 2015

Camp Sunshine

Last weekend we had the opportunity to go to Camp Sunshine, a retreat for children with life threatening illnesses and their families.  It's located on a Sebago Lake in Maine.  It was a wonderful experience.  Emmett and Clayton both made lots of friends.  70 volunteers from across the northeast came to host the camp.  The weekend was full of lots of fun activities including swimming, ping pong, air hockey, pool, arts and crafts, Frisbee golf, miniature golf, and much, much more.  The ice skating rink wasn't frozen yet, and the lake activities were closed for the winter, but there was still plenty to do.  

Here's Emmett enjoying some time by the lake in what turned out to be a weekend of amazing weather.


Emmett and Clayton quickly made lots of friends with the counselors.  One of the most rewarding experiences as a parent is to watch other people get to know your kids and fall in love with them.  And that's exactly what all these counselors did.  They were so excited to get to know and love these kids with all these challenging illnesses.  They'd come up to us at the end of the day and tell story after story of all the things Emmett had done or said throughout the day that were so cute or hilarious...and the same thing with Clayton.  And we couldn't help but fall in love with all of them too.  Here are a few of them.

And here are some of the activities we got to enjoy over the weekend.

Clayton chowing down on popcorn.

Clayton enjoying kicking back on the mini golf course.

Emmett on the Frisbee golf course.

We had to get a picture with Mike and Sully!
And Emmett had to bring his new helicopter to camp with him (yes, Emmett found a new helicopter on his second "toy day" at radiation.  So mom, me, and Emmett are all feeling much better after letting his first one fly into the street and get run over).

The weekend at Camp Sunshine culminated in a talent show put on by the cancer kids.  It was one of the most awe inspiring things I've seen in my entire life.  Imagine these precious children, fraught with the physical and mental challenges that accompany malignant brain cancer, climbing out of their wheel chairs and walkers to break dance and lip sync to pop songs such as "what doesn't kill you makes you stronger" and "this is my fight song, take back my life song" in an effort to inspire one another to keep fighting the monster of cancer that haunts them all.  This was nothing less than the 'YouTube cancer kids' music videos come to life....for real.  And some of the kids we saw are not expected to survive much longer.  And watching them up on the stage absolutely melts your heart and breaks it at the same time.  Heroes.  Warriors.  Angels.  Precious Angels...that are facing the greatest challenges in life...greater than I can comprehend.

And to think that our precious little Emmett is one of these cancer kids.  I can't dwell on the thought.

After the talent show there was a masquerade ball.  Emmett has never been a dancer, but he threw on his green hat and ran out on the dance floor before we knew it.  And it wasn't long before he was in the middle of it all.  He ended up being the star of the show.  He told me afterwards, "at first I was nervous because I don't know how to dance, but then I realized, I'm a really good dancer."  All the counselors thought so too.  It reminded us of the days on 9 north when Emmett would hang out at the nurses' station.

What a fun, inspiring weekend.  What a wonderful opportunity for cancer kids and their families.  God bless all those who donate their time, energy, and resources to lighten the burden of pediatric cancer!  Go Camp Sunshine!

Thursday, December 3, 2015

Proton Radiation Therapy

Emmett is back in treatment.  And I can't begin to describe how sobering those words are.

Emmett started his radiation treatment last Monday, 11/23.  The treatment is every morning, Monday through Friday, except holidays.  So that means he had a short week last week with Thursday and Friday off.  He got anesthesia with a gas mask last week because he didn't have any central access (i.e. permanent IV).

It was nice to have a holiday break so soon after starting the next phase in Emmett's battle against ATRT.  It's so emotionally overwhelming to be back in treatment.  We were glad to spend a few days with family and enjoy the distraction of making a Thanksgiving dinner.  My (Micah's) parents and two of my sisters drove here with their families for the holiday.  All told, we had six kids under the age of five in the house, and it was so much fun.  No shortage of excitement and people to play with.

This week we're back to the grind stone.  He got a port-a-cath surgically installed Monday so he no longer needs a gas mask for his anesthesia.  This allows the doctors to administer the "sleepy medicine" directly into his blood stream.  This is much less traumatic for him and he isn't afraid of his treatments anymore.

Emmett has his treatment every morning.  Because he gets anesthesia he can't eat before treatment.  We spend about an hour in traffic driving to the Francis H. Burr Proton Therapy Center.  Then Emmett gets to play in the toy room for 10 minutes or so until they call us back to the pediatric staging area.  Katy and I alternate who takes him back each day and the other stays with Clayton.  When we get to the staging area we spend another 10 minutes with the nurses while they connect his tubes, check his vitals, and get him ready for the beam room.  While he's waiting he gets to play with one of nurse Rachel's iPads which he loves because they're full of games.

Then we get called back to the beam room and they wheel Emmett's bed down the hall while he's still playing on the iPad.  Then we carry him over to the table, and he enjoys changing LED lights on the "spaceship machine" or continuing to play on the iPad while they prepare him for anesthesia.  The last two days the only thing that's been able to tear him away from Minecraft is IV propofol.  Then it's a kiss goodnight, then I head out and meet Katy and Clayton and we go upstairs for a prayer and something to eat while Emmett gets his treatment.

About an hour later we get called back to the pediatric staging area where Emmett's waking up from sedation.  He usually wakes up pretty fast.  Then it's back to the car and we're on the road home.  (Sometimes Emmett talks us into stopping for a treat somewhere along the way.)  All told it's about a four hour round trip.

Friday is toy day.  Emmett is so excited to get a toy tomorrow!  Last week he chose a toy helicopter.  He was so excited to fly it when we got home and he kept asking if he could fly it outside.  I told him no, absolutely not.  Uncle Mike also told him no.  But then he asked Katy, and she said yes.  Well, the next thing you know the helicopter's in the middle of the street where it gets run over by an SUV.  We're just so glad Emmett had the sense not to chase it.

Emmett was really sad by the loss of his toy, and Katy felt so awful.  Yet I felt this was a great learning experience for a young boy...though I felt bad too.  Hopefully he'll find another good toy tomorrow. 

And this is our life until the beginning of the new year.

All said, it's not a bad life.  Emmett's condition is stable.  He's receiving treatment each day to control his disease.  He's tolerating it well.  And so far it's not making him miserable like the chemotherapy he's had in the past.

I fear the day at the end of radiation when we have to decide whether to subject him to more chemotherapy.  That will be a very hard day.  But we're not there yet, and Emmett is doing well.  And we'll continue to take one day at a time.

Sunday, November 22, 2015

Back in the saddle...

Quick recap of the last two weeks:

11/10/15:  We met with the radiation oncology team at MGH about Emmett's radiation plan.  They want to treat this spot as AT/RT recurrence.  While it's not growing quickly like AT/RT, they say given Emmett's history, it's more likely AT/RT than anything else.  They also say the spot has qualities that make it look like a secondary tumor and not a primary tumor.

They also mentioned that just the day before, Dana Farber sent word that their final review of Emmett's 8/27/15 MRI revealed two spots that are enhancing slightly.  There's no bulk to be seen here, but the nerves in these areas are picking up contrast.  Looking back in time, these spots have been picking up contrast since 2014, so whatever is happening here is nothing new (good news -- we think).

These spots are very close to the nodule of concern (within millimeters) and could be included in the radiation field and treated.  They may be part of the nodule (best case) or they may represent metastasis.  Dana Farber asked MGH to closely examine these spots with a KISS (?) image sequence on their MRI the next day.  They also wanted MGH to carefully screen the rest of Emmett's brain and spine to make sure there were no other areas of enhancement, suggesting possible metastasis (this scenario would beg for systemic treatment first instead of focal radiation treatment).

They want to treat Emmett with 30 days of low dose radiation instead of 5 days of SRS radiation.  The main reason is because Emmett got 30 days of treatment last time and he tolerated it very well and it appears to have controlled his disease.  They can't predict if SRS would have the same effect on his tumor, although they believe the SRS and 30-day dose are "biologically equivalent". 

11/11/15:  Emmett has his radiation planning MRI at MGH.  The preliminary word from the fellow was that they do not see any other spread of disease (great news), that the two additional areas of concern are still showing enhancement, and the nodule is showing a small increase in size (approximately 1 mm in each direction).  This is on par with the last few MRIs (i.e. the nodule is slowly growing).  We were hoping for a reduction that would lead the team to reconsider the need for treatment...but at the same time, were very glad to see that things hadn't changed much from his scan in August. 

11/12/15:  Emmett had his radiation planning CT.

11/16/15:  We heard from the radiation oncologist on the radiation plan.  They plan to include the two additional spots in the radiation field.  Because they're so close to the nodule they can easily be included in the radiation field.  And the radiation field will still be quite small.

When asked about the size of the nodule compared to Emmett's last MRI, she said that because they did a more detailed image sequence this time with 1 mm slices, there's no fair baseline of comparison to say exactly how it changed from last time.  They call it "no appreciable change".  She said that it might have grown slightly, and that she's sit down with us to go over the images in the coming weeks, but her travel schedule has prevented that from happening yet.

11/17/15:  We met with Dr. Chi at Dana Farber.  This is only the second time we've met with her since coming to Boston in September.  It was insightful to get her perspective.  She said that MGH wants to treat this really aggressively, Dana Farber wants to be more conservative, and at the other end of the spectrum you have Children's Hospital of Philadelphia which suggests we watch and wait (though we haven't talked to them since Emmett's last MRI).  She was a little concerned when she heard that Dr. MacDonald at MGH was going to reduce the inner ear radiation field from 50 to 45 Grey, and she wanted to talk to Shannon about that.  Dr. Chi apparently thinks we should give the full dose, even to the outlying areas.  So they need to get on the same page....especially since radiation is supposed to start tomorrow.  Likely this will result in a delay in the radiation start date.

Chi also talked about options for follow-up, systemic treatment at the conclusion of radiation.  At this point she's not in the camp that we MUST do systemic treatment after radiation, though it sounds like she would recommend it.  But the question is WHAT -- there's no protocol for recurrent AT/RT.  Essentially you're on clinical trials exploring new drugs and options.  Thankfully there is a new drug that's shown some promise against AT/RT that will be available in the next month or so.

Our perspective:
  • While we were hoping for a miraculous reduction, we are encouraged and thankful that whatever this spot represents is progressing very slowly.  In fact, we DO consider this a miracle.  AT/RT doesn't creep back; it explodes back.  So why is this growing so slowly?  Maybe because Emmett's body is recognizing it and fighting it.  Maybe it's because of all the prayers.  We like to think it's a little of both.  And throw in radiation, hopefully it runs away with its tail between its legs.  No disease has returned where Emmett was radiated.  Maybe radiation's the silver bullet.
  • We're still very anxious about commencing radiation treatment...and for several reasons.  It's scary stuff.  It's lots of sedation.  It comes with side effects, and only some of them are known.  But probably the most overwhelming aspect is the fact that by commencing radiation we're admitting that Emmett has least in some fashion.  We're stepping into the ring watch our child fight cancer.  And that's my biggest nightmare come to life.
As we mentioned in our last post, we have so so SO much to be thankful for.  We're in a great place in so many ways, and continue to feel very blessed.  The last two years since Emmett finished treatment have been absolutely wonderful.  And Emmett's still doing wonderfully.  He's loving and enjoying every day so much.  And we have reason to hope he'll continue to do well for a very long time, and ultimately will beat this disease.

And those are the things we will focus on this week as we commence radiation and celebrate Thanksgiving.