May MRI Results

Emmett had his next MRI today and the results confirmed that his spot has reduced at least 25% since starting the trial...and now that he's had two scans in a row that show a reduction of at least 25%, Emmett was officially declared a partial response today!  This exciting development is a follow up to our 5/7 post and we were very thrilled that "official partial response" is the word we got today.  So YAY for good news!  We'll take whatever we can get.

The team also had us sign new consent forms today which were updated with the information about this girl on the trial who came down with lymphoma.  The team didn't really have any new information to share about this case, the FDA hasn't released any conclusions yet from their investigation...so for now we're in a holding pattern.  We again decided to continue the trial at Emmett's current dose, and we will probably continue at this dose until more information becomes available.  It's no surprise, but oncologists generally want to hit with everything they've got...and so that's what we're going to do for now.  We did reach out to Emmett's primary oncology team in Boston to get their input on what we should do, and we were really hoping for some thoughtful guidance, but we never heard back. Ugh.

So that's where things stand today.  Thanks for keeping Emmett in your prayers!  We hope to keep finding more good news to share!

A few inspiring verses from my Old Testament study today when Joshua was preparing to lead the Israelites into the promised land:

"Have not I commanded thee? Be strong and of a good courage; be not afraid, neither be thou dismayed: for the Lord thy God is with thee whithersoever thou goest" (Joshua 1:9).

"Sanctify yourselves: for to morrow the Lord will do wonders among you" (Joshua 3:5).

May we ever be courageous and worthy of the Lord's blessings!

The best part of the weekend for the boys was the Fathers and Sons camp out.  In order to get back in time we moved Emmett's MRI to Thursday and rented a minivan for the drive north, then we dumped it at the airport and flew back Friday afternoon after our morning clinic appointments.  They loved camping out -- and playing with laser guns and exploring the great outdoors with the Cook boys.  Clayton did fight sleep, however ;)

A Glorious Decade

Celebrating 10 years with the love of my life today!

Info from Dr. Hoffman

We met with Dr. Hoffman on Monday and gathered as much information as she had to offer about the girl who came down with lymphoma and the options for Emmett moving forward.  I know the specific details she shared won't mean much to the casual reader, but because our blog provides us with a concise, searchable medical history for future reference, I'll add most of what she shared below.

• The girl who came down with lymphoma had a different primary tumor type than Emmett (epithelioid sarcoma).  Her treatment history (and associated risk factors from her prior treatments) is still under investigation.  The drug company and the FDA want to gather and study all the available details of her medical history before determining what any impacts may be on the study moving forward.  Dr. Hoffman doesn't think this will cause the FDA to close the study, but perhaps they'll cap the total drug dose a patient can receive or limit the number of months a patient can be on the study.  But it's really too early to tell at this point.
• A secondary lymphoma is generally not a risk factor for most treatment protocols -- leukemia, on the other hand, is rather common.
• Radiation may increase the risk for a secondary lymphoma, and Dr. Hoffman believes this patient had received some radiation; however, lymphoma is still a rare side effect for irradiated patients.
• In laboratory studies, Emmett's EZH2 medicine did cause a secondary lymphoma is rats in very high dose levels (orders of magnitude higher than what humans receive).  This leads the doctors to think that this girl's lymphoma is likely caused by Emmett's medicine.
• The girl was on a lower dose (900 mg/m^2) than Emmett (1200 mg/m^2)
• She had been on the study much longer than Emmett when she came down with lymphoma -- 19 cycles is what Dr. Hoffman recalled, and each cycle is 4 weeks.  So that means she had been on the drug 76 weeks and Emmett has been on the drug ~40 weeks.
• In terms of total dose, Emmett has received ~50% of what the girl had received when she came down with lymphoma.  Perhaps this means that we have a little time before things get critical for Emmett.
• A particular patient may be genetically pre-disposed (i.e. more likely) to develop lymphoma upon exposure to a new drug than another (i.e. this girl's unique DNA may make her more susceptible to developing lymphoma than Emmett would be)
• 700 total patient have received the drug, and only 1 lymphoma case has been reported.  The 700 patients includes mostly adults -- only about 25 pediatric patients have taken the drug.  About 12-15 patients are further along on the clinical trial than Emmett is.
• Pediatrics receive a higher dose of the EZH2 medicine than adults because kids metabolize drugs differently and generally can tolerate much higher levels than adults.  Especially for kids with central nervous system tumors, you want the dose to be as high as can be tolerated to help it cross the blood brain barrier.  The adult dose is 800 mg/m^2.
• Non-Hodgkins lymphomas -- the type this drug can cause -- are generally treatable with success, though they do require a traditional chemotherapy protocol that is relatively tough on patients (and ~9 months long).  In Emmett's case, however, he's already had one of the main drugs that would be used to treat a non-Hodkins lymphoma (doxorubicin).  This drug has a total-dose cap due to potential organ damage (heart).  Dr. Hoffman was going to look into how much more doxorubicin Emmett can handle and also the treatment options for NHL without using doxorubicin (and their expected effectiveness).  We hope to hear more soon.
• The FDA and the drug company are currently agreeable to having patients (1) continue therapy at their current dose, (2) continue therapy at a reduced dose, (3) pausing therapy with the option to restart later after more information is available, (4) ceasing therapy completely (an option at any point).  These options may change at any time pending their investigations.
• If the FDA was extremely concerned, they would put an across-the-board hold on the clinical trial.  The fact that they haven't done this is good sign...for now.

After a thoughtful weekend of fasting and prayer, and after considering Dr. Hoffman's information, we decided to continue the trial at Emmett's current dose for at least two more weeks until next next MRI.  This will give the FDA/drug company (and other) doctors a chance to review all the available information and put together their recommendation.  We're also hoping to get some input from Dr. Chi in the meantime.

We know that the drug in its current form has helped Emmett significantly, and we think it's prudent to get him at least two more weeks on the medicine when weighing the risks against the benefits it's provided him.  Hopefully by the time we go back to Denver we'll have a more complete picture from the doctors that will allow us to clearly discern what our next move should be.

Some good news....and then a twist

The message we've received from the doctors on the size of Emmett's tumor since starting this clinical trial last August has always been consistent: after initially shrinking ~25% in the first two months, the spot has remained stable.  When Emmett had his March MRI we heard the same thing -- the spot was still approximately the same size as it's been since October.  However, when we met with the doctors two weeks later the message had changed.  At our early April clinic appointment they said that upon closer review the team had concluded that Emmett's spot has still been slowly shrinking, and now it was officially small enough to officially re-classify Emmett as a "partial response" to this medicine.  (In order to officially re-classify a patient they require two MRIs that both indicate at least a 25% reduction that are at least 8 weeks apart, so Emmett won't officially be reclassified until after his May MRI, assuming his spot hasn't gotten any larger.)

We were very excited to hear that Emmett's spot has continued to shrink!  Another miracle to add to our list!

We hadn't realized the detailed requirements for a "partial response" classification -- we thought he was already considered a partial response.  But we're not too concerned about the semantics -- news that the spot is continuing to shrink, albeit slowly, is welcome news indeed!

And then just this week we got an unexpected phone call from Dr. Hoffman.  She wanted to let us know that one of the kids on Emmett's trial was just diagnosed with a secondary cancer: lymphoma.  It's possible that the lymphoma is a side effect of their initial cancer treatment protocol.  It's also possible that the medicine that Emmett's on caused the lymphoma.  Unfortunately it's impossible to know conclusively given the very little available data, and that's the whole purpose of a clinical trial -- to measure treatment effectiveness and to monitor side effects. 

And so here we are today -- with a medicine that is continuing to benefit Emmett, but that may give him another cancer (or worse).

Our next meeting with the medical team is on Monday.  They're going to gather as much available data to share with us at that time, and then we'll decide on our path forward.  Possible options include:

1.  Continue the trial at Emmett's current dose.
2.  Continue the trial at a reduced dose.
3.  Discontinue the trial (and perhaps consider other therapies).

Some of the questions I have for the medical team are:

• What dose of Emmett's medicine was this patient on?
• How long had the patient been on the trial?
• What primary cancer did the patient have?
• What treatments did the patient have for the primary cancer?
• What were the risk factors for the above treatments?  Was there an increased risk factor for a secondary lymphoma given the initial treatments?
• How many total kids have been on Emmett's clinical trial?  How many of them have been on the trial longer than Emmett?  And at what dose?
• Based upon the data available today, and assuming that this patient's lymphoma was caused by Emmett's medicine, what's the percentage chance of getting lymphoma?  And at what length of time on the trial?
• What are the treatment options for a secondary lymphoma given the therapies Emmett has already had?  And how effective might they be?
• Would a PET/CT or other imaging study help reveal whether there's still active tumor that needs further treatment?
• If we discontinue the trial, would Emmett be able to get more of this medicine if his tumor starts coming back?
• If we discontinue the trial, would the recommendation be "watch and wait", or would the doctors want us to consider another trial?  If so, what?

As Dr. Chi has discussed with us many times, when a child has a malignancy that is threatening their well being, that malignancy presents the most immediate danger to the child, and doctors generally want to focus primarily on treating that condition rather than holding off therapy because of the other conditions that the treatment might cause.  And in Emmett's case, we know that this therapy is effective against his greatest risk.  So there's a part of you that doesn't want to stop the full frontal assault.  At the same time, the thought that the medicine you're giving your child each day may give him another cancer really shakes you to the core.

And so here we are again, with big decisions, and very limited data, and Emmett's health and well being in the balance.

As always, we will make this a matter of fasting and prayer.  We've been led and inspired by divine power in the past, and I'm confident that the Lord will walk with us again this time.  I'm thankful for an opportunity to fast and pray this weekend for divine guidance, and we would welcome any and all to join us. 

Thank you all for your prayers and faith!

January 2018 MRI

Today Emmett had his third MRI since starting the clinical trial at Children's Hospital Colorado.  The scan showed that everything has remained stable since his last scan -- yay!  Emmett has now had three scans since starting the clinical trial.  The first scan in October showed a significant reduction in Emmett's tumor, and the second and third both showed stability, and that is amazing news.

While we've hoped to hear of continued reductions of this spot, it's important to remember that Emmett has always had scar tissue in this area since his last spinal surgery in 2012.  A few times over the years the doctors have wondered whether this area might contain active tissue, but it's never showed activity until June 2017, and so they've never been concerned about it until then.  Given that he's had scar tissue here for the better part of six years, it's understandable that it may not respond completely (i.e. reduce down to zero) because it wasn't zero to begin with.

In looking at the imaging myself, what remains in this spot looks about the same size as what it was in January 2017 when the doctors had no concerns.  Perhaps it's slightly larger now, or changed in shape slightly -- but overall it's roughly the same size as when the doctors were not concerned.  So maybe he's close to his "non-zero" baseline.

Upon reviewing the MRI results with our doctor she mentioned that sometimes tumors reduce significantly upon treatment but never completely go away, even though they are shown to be clinically "dead" upon subsequent biopsy.  Without a biopsy it's impossible to prove whether residual tissue is benign, and in Emmett's case there's too much risk to do a biopsy, so we'll never know for sure.  But we can hope and pray that this is the case.

Our doctor did mention that spots that pick up contrast in MRIs are more likely to be vascular (which scar tissue generally is not), so that fact that Emmett's spot lights up on an MRI may be a bad omen.  But then again, with each additional surgery / treatment / intervention, the less the traditional rules may apply...so we'll take that for what it's worth.

What we will take (and are extremely excited about) are two more months of life the way we're currently living it -- trips to Denver every other weekend to get more medicine, but otherwise home in Albuquerque living as close to a "normal family life" as possible.  The thought of another two months of this routine is very exciting!!! 

We feel so blessed and thankful to be where we are today and we're praying that this period of our lives may continue.  How thankful we are for everyone who continues to remember Emmett and our family in your prayers.  The miracles keep coming, and each of you play a part in that!

Welcome 2018

We have thoroughly enjoyed the last few months since Emmett's good news MRI in October.  The blessings of the past few months have been remarkable indeed.

After Emmett's October scan, we were thrilled by the realization that we would get to spend Halloween in Albuquerque together as a family.  It's been a long time since we've had the opportunity to do that.  We were likewise excited that we would get to celebrate Thanksgiving before Emmett's next MRI.  That was also another great blessing.  Over these past months we've also been blessed by several visits from family who made the trek out to New Mexico, and those have made for sweet memories.  And we've also been able to drive a "few" extra miles on our trips to Denver to enjoy family time in Fort Collins and Salt Lake City.  And those were wonderful times.

And to say that we enjoyed spending Christmas at home in ABQ is the world's greatest understatement.  The week off between Christmas and New Year's made for very treasured family time.

Overall, we just continue to feel so blessed and Thankful.

Emmett had another MRI on 11/30/17, and that scan showed stability from the October scan.  The spot still hasn't reduced completely, and the doctors are certainly still concerned about it, but we are very, very happy that it hadn't grow at all from early October to late November.  And our oncologist thought that it may have reduced 1mm in one dimension, and we will take whatever encouragement we can get.

We keep praying for further reduction and hope this medicine will continue working its magic!  Or at least keep this spot from growing!  As long as it doesn't grow more than 20% we can continue on this study.

Emmett's next MRI is a few weeks from now and we're praying for good news.  In the meantime, we'll continue watching two boys smother their new little sister with an overabundance of love and affection.

 

A Very Touching Gesture

To underscore just how remarkable it is that Emmett responded to his experimental medicine, I wanted to share a touching experience from Emmett's clinic appointment last Friday.

After a brief visit with our primary oncologist when we first arrived in clinic, she left the room briefly to go get something.  When she returned, the first words out of her mouth were "Without violating any HIPAA regulations...." -- I can tell you that Katy and I were both confused at this point and didn't know where this was going -- but then she continued "....without violating any HIPAA, I sometimes talk about the goings-on at work with the people on my street.  Naturally, a lot of people are anxious to hear how work is going for a pediatric oncologist...(which is a nice way of asking how the kids are doing).  Often there's not a lot of good news to share.   But this past week I couldn't help sharing how well things were going for 'unnamed patient X'.  The group of women I shared Emmett's news with were very excited to hear it....and they wanted you to have this!"  At this point we saw what she was holding in her hand:  Visa gift cards totaling $150 dollars!  Holy cow?!  Our hearts were touched deeply by the kindness of these women, an act of service bestowed anonymously upon a family they had never even met or seen!  Five years after Emmett's initial diagnosis, we continue to witness the goodness and generosity that abounds in the people everywhere.  It always catches you by surprise and moves you deeply.

A heart felt thank-you to everyone who reaches out in love to serve and bless those around you, in whatever forms your service takes.  Those who receive your kindness are blessed and strengthened beyond measure, and they feel the love of God through you.

The other take away from this experience is how remarkable Emmett's response to this medicine is to our medical team.  Clearly this is not everyday news, clearly this is something rare, something so novel that even the the doctors are anxious to talk about it and share.

And to me this reconfirms, that yes, this is a miracle!

Let me also add here how much we love our primary oncologist in Denver.  She is so real, so personable, so amazing.  This story illustrates so perfectly how human she is and how much she cares.  She also has three kids and they're about the same ages as ours, and I think helps her understand even more perfectly what we're going through.  And you don't always find that in an oncologist.  She's absolutely amazing and we consider her to be another one of the great blessings in our life!