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 weeks...so 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 anything...to 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)...so 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.