The Adventures of Little Muncher and his Lion: August 2017

Sunday, August 13, 2017

Clinical Trial Started

Emmett started the clinical trial on Friday. It's an oral drug called tazemetostat that Emmett will take twice a day. They treat the drug like it's toxic chemotherapy -- we have to return all the syringes, gloves and wipes that come in contact with the drug. It comes in a chemotherapy bag. It's especially important that Katy not come into contact with the drug being pregnant. Even though adults and other kids have been treated with this drug, the fact that it's still not FDA approved is a pretty big deal. It's a little unsettling to take something that's treated like poison and give it to your son.

Emmett has to take 28.8 milliliters of the drug morning and night! That's like having to take two tablespoons of nasty cough syrup twice a day. He did pretty good on Friday, but since then it's gotten harder and harder to get him to take it.

The good news is that the doctors have already identified the effective dose on this drug, and that's the dose Emmett will receive. Hopefully this means it will be more effective in treating his disease. The initial kids treated on this drug generally didn't do well, but they started off at 280 mg/m^2. The dose Emmett is on is 1200 mg/m^2. They've given kids up to 1500 mg/m^2, but at that dose the kids started showing side effects (increased fatigue, nausea, blood count suppression, etc.). So they backed the dose back down to 1200. The higher the dose, they more they expect will cross the blood brain barrier, and that's where Emmett's disease is. Hopefully 1200 is Emmett's golden ticket. (Somewhat ironically, the effective dose for adults is 800 mg given twice a day....notice that it's not "/m^2". So the kids can get higher doses of the drug than adults, depending on their surface area. I'm not sure what they've calculated for Emmett's m^2.)

They kept us in the clinic all day Friday so they could do blood draws and run EKGs on Emmett throughout the day to make sure the drug wasn't having any negative acute effects. Everything looked good so they released us Friday evening. We're not scheduled to be back in the clinic until next Friday, so Saturday we drove back to Albuquerque and we'll spend a few days here before making the return trip to Denver. We expected we would need to stay in Denver the entire time that Emmett is on this clinical trial, but it sounds like we will have some opportunities to commute back and forth.

We pray that this drug is effective against Emmett's tumor and that he'll be brave and strong enough to take it willingly. Go Little Lion!

Thursday, August 10, 2017

Children's in Denver (Aurora)

We arrived in Denver on Sunday night and have spent the week at the Aurora Ronald McDonald House. The Children's Hospital extension that Emmett is being seen by is in Aurora, CO. So far this week we've been working on all Emmett's intake activities. Before they'll let him start this EZH2 clinical trial they have to run a bunch of baseline tests to see where he's at. We did consents on Monday, an MRI, echo, and EKG on Tuesday, and then a Lumbar Puncture (LP) on Wednesday.

The MRI showed that this spot has grown slightly in the six weeks since Emmett's last scan. It was measuring ~11mm in late June and it measured ~14mm in length on Tuesday. We were hoping it would be stable or reduced, but we're glad it hasn't grown more. They also didn't see any spreading to other areas since his last scan. The oncologist wanted to at least mention the option of surgery because if it gets to the point that it's compressing his spinal cord, we would need to do something quickly. The team in Boston made it sound like surgery was off the table, but it sounds like Denver isn't in that same boat (though there certainly would be risks going into Emmett's spine for a third time). Unfortunately there isn't much room for this thing to grow before it would start causing problems.

The fact that it's grown confirms that it's cancer and needs to be treated. If it's not in the cards for Emmett's immune system to activate and remove this spot on it's own, then we shift our focus to pray that one of these treatments is effective. There's still the question of whether this drug will cross the blood brain barrier.

Emmett's next MRI isn't for another two months. I asked whether we should consider getting a scan sooner, but given the growth rate of the tumor the oncologist was comfortable waiting 2 months unless Emmett starts exhibiting systems (weakness in his legs, etc.).

We start the EZH2 trial drug Friday morning (8/11/17). We pray that it's effective against this spot!

Emmett and Clayton are loving their life here in Aurora and think we're on vacation at a cool resort with free arcade games you can play all day long. They made some friends with other kids staying at the house tonight and enjoyed an evening out riding bikes tonight.

Katy's doing well and the baby's staying put for now. Incidentally, we just got the results of Emmett's LP yesterday and it was clear!

That's all for tonight. Thanks again for all your prayers and faith!

Look who we ran into doing service at the Aurora RMH?!

Look who thinks it would be cool to grow up to be a geeky engineer?

Look who's in heaven? (i.e. we have no video games at home.)

The trash shoot on the third floor is Emmett's favorite thing!

The hangout.

Whenever Emmett's in scrubs, Clayton has to join him!

Fearsome threesome!

Little Muncher's Story

Background: On February 23, 2012, our 17-month-old son Emmett stopped using his left leg and was rushed into emergency surgery to remove a tumor compressing his spinal column. After a series of tests, he was diagnosed with Atypical Teratoid / Rhabdoid Tumor (AT/RT), a very aggressive and rare form of cancer that attacks the central nervous system. While the survival rate is very low for a toddler Emmett's age, we believe in miracles and have witnessed many already.

One of the best places for treatment is Dana Farber Cancer Institute in Boston and we relocated to Massachusetts where Emmett underwent a year and a half-long treatment program involving surgery, chemotherapy and radiation.

After successfully completing his treatment protocol in the summer of 2013, we returned home to Albuquerque and enjoyed two years of fun family time until Emmett had a small recurrence in the fall of 2015. That spot was treated successfully with proton radiation in Boston and Emmett was again stable until July 2017 when the doctors noted a small spot on his spinal cord in his neck. We enrolled Emmett on a phase 1 clinical trial in Denver testing a EZH2 inhibitor (Tazemetostat) which successfully reduced the spot in his neck within two months and kept him stable for over a year.

In October 2018 a MRI revealed a new spot on the outside of his brain near his left ear. We traveled to Boston to consider treatment options and are currently awaiting direction from doctors.

8/3/17 Status: After learning last month that Emmett's tumor is coming back, we're working with our medical team to determine treatment options and the best place for treatment. Emmett successfully made it through his first recurrence in January 2016, and we're hopeful that we'll find a solution again this time. We're praying that things don't get too crazy until after the baby comes in early September. It's likely that we will be headed to Denver or Boston.

11/30/15 Status: Emmett is getting a port this morning so he won't have to get gas anesthesia every day for his radiation treatment. His Radiation started last Monday. More on that in a full post.

7/19/13 Status: Emmett spiked a fever and was admitted to the hospital. His ANC was 22 yesterday so we'll likely be here for a few days until his blood counts recover and his fever goes away. This may be our last inpatient stay in Boston Children's Hospital. Mixed emotions...this has become a second home for our family.

7/10/13 Status: Emmett started week 51 chemotherapy today, his final round. Three days of infusions and Emmett is done receiving chemotherapy. Praying the protocol has done it's job and the cancer is gone for good!

6/21/13 Status: Emmett was admitted to the hospital with a fever today. Hopefully we're only here a few days before his counts come up and they send us home. His ANC was 56 yesterday and 50 today.

6/10/13 Status: Emmett started week 48 chemotherapy today. Five days of infusions in the clinic. Only one more round after this! We can't believe we're almost done. We continue praying the chemo has done its job.

5/2/13 Status: After a few weeks of delays Emmett started week 45 chemotherapy today. Last Monday he was too low (620 ANC), Monday of this week he was 750, and he needs to be over 1000 before we can start. (We could have started 4/16 when he was 3000 but we delayed as we had family in town.) Only two more rounds after this!

4/11/13 Status: We were discharged from the hospital on Saturday. Had perhaps the worst night in the hospital Friday night, at least physically, for Katy and me...we both came down with a stomach bug Friday afternoon/evening and it was horrid. Only upside was they moved us to a private room! Sure makes us appreciate and pity Emmett more knowing that he feels that way so often. The good news is that Emmett's counts were up on Saturday and we were able to go home as soon as his parents were well enough, ironically.

4/1/13 Status: Emmett got a fever this evening and we ended up in the hospital. We anticipate being here for a few days until his counts come up.

3/25/13 Status: Started week 42 chemotherapy. Five days of outpatient infusions. Micah traveled to California for work this week. Last week he was in New Mexico.

3/7/13 Status: Brought Emmett into the hospital with a fever. Will probably be here a few days before his counts come up.

2/25/13 Status: We're headed to the Jimmy Fund Clinc M-W this week for outpatient chemotherapy including intrathecal (week 39 of the protocol). The Doxorubicin is a quick push this time instead of a 48 hour infusion so they can use a heart-protection drug in conjunction. We'll post about our medical consultations later this week.

2/15/13 Status: Quick summary Hopkins and CHOP consultations: Hopkins would not recommend surgery, believe remaining bulk is scar tissue, suggest sending Emmett to stem cell. Boston doesn't know if they buy the scar tissue argument. CHOP doesn't recommend surgery or stem cell, finish current protocol instead. When MGH's recommendation is taken into account it's an even tie: two institutions recommend stem cell, two recommend wrapping up therapy now, all acknowledge this is a very complex and ethically challenging case. We're very thankful that surgery may be off the table!

2/6/13 Status: Met with Hopkins yesterday...at the time of our meeting they hadn't compared his Monday MRI to his previous scans from Children's so what they could tell us was limited. Preliminarily it looks like they wouldn't recommend surgery, but the jury is still out. We expect more info and a recommended path forward later this week.

2/4/13 Status: Made it safely to Baltimore late Saturday night, stayed in a hotel, then checked into the Children's House at Johns Hopkins Sunday afternoon. Had an MRI today, haven't heard the results yet. We meet with the surgeon tomorrow.

1/30/13 Status: Emmett was discharged from the hospital on Monday and now we're at the Ronald McDonald house here in Boston. The doctors want to limit Emmett's exposure so that he's healthy for his consultation at Johns Hopkins next week to prevent any delays. He's doing well! We got to visit Destiny and Brandon at the hospital last night and they're also doing well, best day Destiny has had in a week. Hopefully another good day today.

1/24/13 Status: Emmett had another round of maintenance chemotherapy last week in buy some time to visit Johns Hopkins in early February. He's getting to his low point in the chemo cycle, may head into the hospital with a fever in the next day or so. He's feeling especially crummy this time, he's been down all week. We think it's because he was already pretty sick prior to this latest chemo. Hope he feels better soon.

1/1/13 Status: We're doing Christmas today! Katy wanted to wait until New Year's Day so that there would be some holiday excitement. We really wanted to make this the best Christmas ever for Emmett. Had to break from opening gifts because Emmett needed a nap--rough life. We're very touched by the wonderful, thoughtful gifts so many of you have sent.

12/27/12 Status: Emmett's ANC was 580 today. He was still feeling a little crummy and needed morphine in the morning so we didn't want to head out right away. He got a platelet transfusion in the afternoon and then we were discharged around dinner time.

12/26/12 Status: Still inpatient waiting for Emmett's blood counts to recover. He's feeling pretty well today but his ANC needs to be above 200 and today it was 60 (yesterday and Sunday it was 10).

12/24/12 Status: Emmett's still feeling pretty crummy, probably the worst he's been since our first two admissions when he needed a morphine pump. Hopefully he perks up soon.

12/23/12 Status: Emmett didn't sleep well last night, was uncomfortable most of the night and border line feverish. He felt pretty crummy all day today and this evening his fever spiked so we headed down to the hospital for an admission. Most likely will be here for Christmas. Luckily the cafeteria food is amazing.

12/18/12 Status: Got to go home today! Looking forward to some time at home before Christmas.

12/14/12 Status: Admitted to the hospital today for a new port then three days of continued maintenance chemotherapy.

9/4/12 Status: PICC line inserted successfully today with no complications. He slept off the sedation over the course of a few hours and now is up making his way around the hospital. Praying for good news tomorrow after his MRI evaluation -- probably won't share the news for a few days, good or bad.

8/20/12 Status: Emmett completed his fifth round of chemo (weeks 13-15) without ever having to return to the hospital with a fever! He also never needed any blood transfusions! We went in today to start chemo week 16 expecting to be admitted for the week and were pleasantly surprised to find that it's all outpatient -- we'll go to the clinic for a few hours each day but otherwise will be at home. We're praying this round goes as well as the last.

8/13/12 Status: We took Emmett to the Jimmy Fund Clinic today expecting him to need a blood transfusion. Amazingly, his counts were so high that he needed no blood products at all! And instead of doing stem cell collection late this week or early next week like the doctors had planned, they sent us right over to the apheresis lab to collect stem cells today. We'll explain more about how stem cells may be used in Emmett's treatment on the blog soon, but for now just know that Emmett continues to do well.

8/6/12 Status: Emmett's chemo infusion completed late Thursday night and we were discharged from the hospital mid-day Friday. We expect to return with a fever any day now. Emmett has really enjoyed himself the last few days, he's still feeling well. We hope it continues a while longer.

7/30/12 Status: Last Friday his ANC was only 280; it has to be over 1000 before they'll start the next round of chemo. We didn't expect it to be high enough today, but Emmett surprised us again--his ANC is 1100 so we're admitted to Boston Children's Hospital for chemo week 13.

7/24/12 Status: Last Friday we were at Jimmy Fund, checking his counts and ready for an admission. All his other numbers looked good, but his ANC which is what really determines whether he's ready for chemo or not was still too low, not completely surprisingly. Took counts again yesterday, to prepare for admission today and he's even lower now! Dr. Chi called this morning to say she doesn't think he'll be ready this week at all and will check again Friday and plan for admission next Monday.

7/15/12 Status: We've been done with radiation for over a week now! Last Sunday Emmett got a fever and was admitted to the hospital and we were there all week. Micah flew to NM on Monday for business meetings. He got back yesterday afternoon, came to the hospital, and an hour later we were discharged and sent home. To make it a date night we picked up Shanghai take-out and had dinner at a park on Horn Pond where Emmett enjoyed throwing rice to the ducks.

7/1/12 Status: We're back at Katy's parent's home now. Micah's parents headed home this morning. Radiation continues tomorrow...four more days until Emmett's finished!

6/28/12 Status: Inpatient at Mass General for week 10 chemotherapy infusions, we hope to be discharged tomorrow. Almost done with radiation, the last day is Thursday, July 5th. Emmett continues to be active and is doing well. He got to see four helicopters land yesterday! And his grandparents are visiting from Michigan this week so he has extra entertainment.

6/21/12 Status: 5 weeks of radiation complete, 1.5 weeks to go. Chemo was scheduled to start tomorrow but Emmett's counts dropped to 410 today. So the admission is cancelled for tomorrow. Chemo will probably start Monday.

6/16/12 Status: 4 weeks of radiation complete, 2.5 more to go. Chemo week 9 complete. At the appointment yesterday when Emmett got his week 9 vincristine his ANC had gone up to 1000! Dr. Ebbs says this means he's on his way back up and that we won't be coming in with a fever this admission! So we're out of the hospital until next Friday when week 10 chemo starts!