Andy & Jessica

Tonight was a perfect night.  There was a breeze to keep the pesky mosquitoes away.  The boys and I spent the night outside.  Asher can stand unassisted for about 30 seconds at a time and then he slowly falls to his butt.  I was really impressed tonight with Asher’s ball skills.  Jude and I were playing catch with a plush ball and it was clear to me that Asher wanted in on the action.  I would roll the ball to him and he would grab it and throw it back to me.  I’ve never had a 10 month old do that trick before.  He was so proud of himself.  He is also big on waving at people.  He sure does look cute with his 3 teeth.

Levi and Jude took turns on the swing.  When Jude was on the swing Levi would yell out, “I too!”  Levi also was driving the dune racer fairly good tonight.  Before we would joke that he was the most dangerous driver ever since he’d frequently crash into the fench and people since he can barely touch the accelerator.  But, he’s much improved.

Although I would prefer that God just spoke audibly to us about how to treat Jude, in His providence, it appears that everything is coming together to give us all the information we need to make the best decision possible for Jude.  First, there was the timing of the HopeKids brain tumor family picnic where I met the mom who encouraged me to get into Mayo.  Then, there was the cool fact that Mayo got us in to see the pediatric neurooncologist we wanted to see this Friday, even when she had nothing available until the following Monday (while we are supposed to be driving to Indiana for the radiation mask fitting and simulation) when I first spoke with them.  Then, when I couldn’t get past the Mayo schedulers to get an appointment with the pediatric radiation doctor I wanted to see, I was able to talk to one of the HopeKids moms and email the radiation doctor directly and get her to agree to an appointment on Friday right before the other appointment I already had set up.  On top of all that, this week is an international pediatric brain tumor conference that occurs once every 2 years.  Our Children’s oncologist told me that today is ependymoma day and that she’ll be on the lookout for cases like Jude’s and what other doctors are doing internationally with respect to treatment of Jude’s rare case.  I’m so thankful that things are falling into place and I’m just hoping for clarify when we have all the information at the end of Friday since we’ll have to start packing for a 7 week trip if we are going to Indiana Monday morning.

Today Jude had a physical therapy evaluation (to get a baseline on gross motor skills in case we do radiation). It turns out that his gross motor skills are well above average for his age so radiation shouldn’t be a concern to those skills. He can’t really skip, but admittedly, I’ve never taught him how.

Afterwards, we went to the lab to get Jude’s blood drawn for a anti-seizure medicine level check. Poor Jude gets more pokes than any kid should ever get and he sure hates them. He told the nice lab technician that she was the “meanest doctor ever.”

On another front, our oncologist at Children’s responded to my courtesy email telling her we were going to get a fresh set of eyes at Mayo look at Jude’s case. She was very nice and said that was a good idea and that no one knows whether we should do radiation now or wait and see, but she wants us to feel comfortable with whatever decision we make. It turns out tomorrow is ependymoma day at the international pediatric brain tumor conference that she is at so she’s going to be thinking of Jude and seeing what others are doing internationally in similar cases (if there are any).

Yesterday we spent the day at a HopeKids event — a brain tumor family picnic.  It was awesome.  They had a human-sized hampster ball for the kids to run in, a video game van, booger wars, etc.  Jude even got to play football with Adam Weber, one of the quarterbacks for the Denver Broncos.  I think Adam was pretty surprised that Jude had brain surgery 2-1/2 weeks ago.  Jude told me something like, “It’s kind of good that I got my head owie cuz’ we got HopeKids.”  I especially enjoyed talking to some other moms of brain cancer kids at the picnic.  One of them encouraged me to get a formal 2nd opinion on Jude’s treatment.  I have done a lot of scrambling and now have an appointment set up at Mayo Clinic on Friday.  That will be just in time for us to decide whether we should pack for Indiana.  I know this is totally dumb, but I was hesitant to seek a formal 2nd opinion since I thought our oncologist is very competent and I didn’t want to offend her.  But, given the stakes, I feel good about this approach.  I just will have to spend my day harassing Children’s to ensure they get all the records/tissue samples sent by the end of today by overnight mail to Mayo.  I don’t want anything to go wrong here since Mayo went out of their way to squeeze us in on Friday.

I’m currently 70/30 in favor of waiting and seeing (no radiation) and Andy is now 55/45 in favor of radiation. We’ve got an appointment set for July 3rd in Indiana for Jude’s radiation mask fitting and simulation. Andy and I have set a deadline to decide by the end of this coming week since we’d need to spend next weekend packing if we are going to Indiana. Overall, my feelings are driven by the fact that radiation is not a guaranteed cure in ependymoma (if fact, I’m not sure it’s working much at all) and we’d have even more to worry about after radiation. And, if the cancer still came back after radiation (20-30% chance), I’d be so annoyed that we exposed him to radiation for nothing.

I’ve been agonizing over whether do pursue radiation.  Here are my own arguments for waiting and seeing (not radiating) and for radiating now.  I’m now 70/30 against radiation.  I think Andy is 60/40 in favor of radiation.  If we plug our nose and jump, which way should we go?

Wait and See (Do not Radiate Unless it Comes Back a 3^rd Time)

1. Supratentorial cortical ependymomas are curable by a gross total resection (it is a “surgical disease”).  We had a “name brand” neurosurgeon – the best in the world.  If anyone can get a gross total resection, it’s him.
2. The first time around, we were told that there was an 86% chance of cure with the first gross total resection (of course this was based on a very small data set).  It’s difficult to understand why, given that last time wasn’t (in retrospect) a gross total resection, the 86% figure wouldn’t apply this time.  Another article Dr. Bendel provided us said that 25-33% of patients can be cured by complete resection alone.  These children are probably the ones who got the perfect surgery.  Maybe the 2^nd time was the charm.
3. This 2^nd surgery should have been an easier surgery to get a gross total resection because (1) the tumor was only about 1 cm, instead of 3-4 times that in the first surgery; and (2) the tumor the 2^nd time was characteristically sticky like ependymoma cells and came out in more of a big chunk, unlike in the first surgery where it came out in little pieces, which made the 1^st one harder to remove.
4. Jude’s tumor bed (which is hopefully empty) is in a great location for surgical access and not too close to vital brain functions.  There is a 95% chance that if the tumor recurs, it will recur locally, making a 3^rd surgery possible and likely to result in gross total resection.
5. Neuro-type doctors do not like radiation in young children.
6. Andy and I both don’t want to radiate unless we have to.
7. Although we were initially told radiation is a 1 shot deal (Dr. Sullivan).  It appears it may be a 2 shot deal (Dr. Buchsbaum).  According to an article Dr. Bendel provided us, “in striking contrast with other pedicatric tumors” ependymoma tumors “can relapse even after several years.”  Do we want to waste one of our radiation attempts when we have a great surgery that could be the cure we are hoping for?
8. Radiation is not a guaranteed cure.   I found an article that said that ependymomas are “insensitive” to radiation.  Dr. Bendel said that we don’t know for sure that radiation is helping that it “could just look like it’s helping because so many get radiation.”  There is a 20-30% chance the tumor will come back after radiation, according to both Dr. Bendel and the radiation oncologist in Indiana.   The doctor in Indiana even only guaranteed the 70-80% cure rate for 5 years.  After that, all bets are off.  My gut reaction is that these ependymoma tumors come back if they want to come back – they do not care what you throw at them.
9. There is a 95% change of local recurrence (Dr. Bendel’s gut reaction).  I saw a paper that said 90% chance of local recurrence.  So, if it came back, it would most likely be local and we’d do another surgery and then radiate.  Jude’s tumor bed is not associated with the ventricle, which makes spreading less likely.
10. Dr. Bendel said that waiting and seeing instead of radiating at this point “would not be a bad decision.”
11. Dr. Nagib initially favored waiting and seeing on the tumor that was just resected.  In other words, we could have let it run amuck until mid-September.  If we decide against radiation, we will follow Jude closely with MRIs every 3 months and will catch anything early and then have an easy decision to pursue radiation after any 3^rd surgery.
12. Dr. Bendel hesitated when asked whether she would radiate her own child at this point.  Clearly, she would struggle with this decision for her own child, just as we are.  This is not a clear-cut decision.
13. The cancer cells have been behaving as good as we can expect from them for this past year and a half.  They did not spread.  They did not mutate further.  Both the pathologist at Children’s and the pathologist at Johns Hopkins said that the pathology is still Type II (not Type III or Type 2.5).  In addition, the cells are looking the same as they did under a microscope with respect to histology.
14. Waiting and seeing has the same risk that we’ve been taking on for the past year and a half.  In other words, nothing changes, except that we are more cognizant of the risks.
15. Radiation has some bad side effects – secondary brain tumors (Dr. Sullivan said 1%; Dr. Buchsbaum said 3-5% and when I said that sounded higher than I had heard, he said maybe it was 1/3 of that given that we would be doing proton not photon radiation); stroke from vascular changes (1%); fine and gross motor skill impact likely (Dr. Sullivan: “He won’t be the best athlete or the most coordinated person.  But, maybe you won’t notice a difference.”); hearing loss in the left ear to some unknown degree; maybe more seizures per Peter Clarine (Dr. Buchsbaum wasn’t sure if radiation increases seizures, but Peter Clarine said he’s seen it in practice); growth issues that can be cured with medicine; attention deficit type issues possible.  Dr. Buchsbaum had not reviewed the scans so he was relying on me to tell him the location of the tumor so his view on side effects are subject to change.
16. Only 2 things to worry about if we wait and see – coming back and spreading.  With radiation, I have 4 things to worry about – coming back, spreading, secondary tumor and stroke and the minor side effects.
17. There is not a ton of data on the long term effects of proton radiation since it has not been done for a long time.
18. Radiation will hit billions and billions of Jude’s healthy brain cells since he’d be radiated at a 5mm-7mm margin around the tumor bed, at maybe 3 different angles.
19. There are 2 other documented cases of patients trying wait and see that have worked out well, but for 1, the follow-up is only 15 months.
20. An article Dr. Bendel provided us says that deferral of radiation is a “safe option” since recurrence is usually local.  But, they were not specifically addressing wait and see at recurrence.
21. An article I found said with respect to supratentorial, low grade tumors, “After gross total resection these patients can do well without adjuvant radiotherapy.”  Admittedly, they were not addressing recurrence.  But, clearly we did not get the gross total resection the first time.  How comfortable are we that we got it this time?
22. I found a 2009 Case Report from India that says, “Even in the presence of recurrence some authors suggest total excision by a second operation.  Thus, the option for postoperative radiation is only for high-grade tumors and tumors in locations where total excision in not possible.”  (Citing an article Dr. Bendel provided us called “The importance of surgery in supratentorial ependymomas, Long-term survival in a series of 23 cases).
23. An article I found from about 2009 said that, “Ependymomas are known to be insensitive to radiation therapy.”

Radiate Now

1. It would increase the chance of a cure by some unknown amount.  We were told recently if we had done radiation after the 1^st surgery (which wasn’t even mentioned as an option), it would have increased the cure rate from from 86% to 95% (that is, a 9% increase in the cure rate).  The radiation oncologist in Indiana thought it would increase the cure rate by 50% this time around.  Our oncologist’s gut reaction is that it would increase the cure rate by 10-40%.
2. Our best in the world neurosurgeon thought he got a gross total resection last time and clearly some microscopic cells were left behind.
3. It’s very difficult for a human to get a gross total resection.  There are 4 million cells in every cubic millimeter.  Although our surgeon did the surgery under a microscope, he could have easily left behind 50,000 cancer cells.  It takes between 5-10 million cells to be seen on an MRI so even the intraoperative MRI that scanned Jude before closing him up would not show cancer cells unless there were 5-10 million of them clumped together.  An article I found said, “It is generally agreed that a complete resection – i.e., one that results in a very low probability of leaving even microscopic residual tumor – is rarely achieved in ependymoma (but this is from 2002 and maybe advances in surgery have been made in the 10 years since then).
4. We were fortunate that the tumor played nice and recurred locally.  We could zap it now before it tries to spread.
5. Radiation now will increase the likelihood of a cure and decrease the likelihood of another surgery and each time the tumor comes back and a surgery is required, studies have shown the patient drops 1-2 IQ points.
6. I know this shouldn’t enter into the equation, but we’ve met our outrageously high family out-of-pocket maximum for 2012 so the radiation would cost us nothing.  But, I don’t want to radiate my kid’s brain just because it’s free this year.
7. I know this shouldn’t enter into the equation either, but the radiation treatment is available to us now – it is covered in-network under an unusual provision in Dorsey’s health plan.  Not every plan would cover this treatment and not every plan would cover it in-network.
8. Again, this probably shouldn’t enter into the equation, but if health care reform is declared unconstitutional (we’ll know on Monday or so), then plans would be able to have lifetime maximums again.  Dorsey would likely add back its $1 million lifetime maximum.  Jude is probably at least ½ way towards meeting that maximum after this 2^nd surgery.  The radiation, if done in a different year because the cancer popped back up, could put us over the $1 million lifetime maximum that Dorsey’s plan would have and I’d have to scramble to get a new job with health care coverage to avoid going bankrupt with medical expenses.  And, it’s quite possible that not many plans would cover the proton radiation treatment.
9. In writing, our oncologist said radiation was his “best chance of cure.”
10. A 2^nd option from our oncologist’s peer in Denver (although maybe not with all the facts) is that, “To be honest it would make me nervous to not do radiation.  However if a family insisted I would agree with the proviso that one could not guarantee that would not upgrade if there was another recurrence.”
11. It is the standard of care upon recurrence in this situation.  But, this situation is not very prevalent – maybe 25 kids world-wide and even less in this location of the brain.
12. The same cells that were hiding from Dr. Nagib the 1^st time could still be hiding from him.
13. We would feel like we did all that we could do to get a cure for our precious Jude – 15% of kids with supratentorial cortical ependymomas die of their tumor somehow and we don’t want Jude to be one of them.
14. We would be listening to the doctors who are the most informed about Jude’s condition.
15. This would be the most conservative treatment option, at least with respect to the ependymoma and disregarding the side effects of the radiation.
16. There is maybe a 30% chance that any tumor cells left behind will morph into a more aggressive Type III tumor if they are still hanging out (Dr. Bendel’s gut reaction).
17. There is a 5% chance that the tumor will spread if there is any left.  This would likely be fatal.  However, this same risk of spreading remains if the tumor comes back after radiation.
18. The first surgery created a permanent tunnel from the surface of Jude’s brain into the tumor bed.  It is possible (5% chance) that any tumor cells left behind would seep out to the surface of the brain and then that would be deadly.
19. Dr. Sullivan (Abbott radiologist) and Dr. Buchsbaum (Indiana radiation oncologist) both said they would radiate their own children at this point.
20. Dr. Buchsbaum (Indiana radiation oncologist) said that if we don’t radiate now, we would be “playing Russian roulette” with Jude’s life.
21. The worst side effects (stroke and a 2^nd malignancy) are unlikely to occur.
22. If we feel like we’re going to have to radiate someday anyway, maybe now is the time since it seems that waiting won’t decrease the risks of radiation unless we made it to age 16 or so.

In the midst of our anguish about how best to treat Jude, we have to enjoy the fun and chaos the boys bring us.  I got this email from Andy who is describing Bobo (Asher’s nickname):

“I gave Bobo a bottle and felt he was a little wet, so I took him up to change him. It turns out the job was half done for me. I took his pajamas off and there was NO DIAPER!  I found it in the play room, I have no idea how he got it off. It must not have been off that long though, he wasn’t that wet.”

This decision is just torturing me. I was feeling great earlier today after meeting with the oncologist and was feeling like I had made up my mind that we should not pursue radiation at this time, but that we would pursue it if it came back a 3rd time.  However, after our 1 hour and 45 minute call with the radiation oncologist in Indiana, I’m now 50/50 on whether to pursue radiation now.  The most startling thing he said was that there are 4 million cells in a cubic millimeter.  So, even Jude’s 2nd brain tumor that was about 1 cm was 4 billion cancer cells.  I know we had the best neurosurgeon in the world, but he could have easily left behind 50,000 cancer cells without being the least bit incompetent.  It takes 5-10 million cells to be visible on an MRI so we’ll never know what was left behind until it grows big enough to show up.  And, while we’re waiting for it to show up, it could spread and become deadly.  Given how it’s easy to leave cells behind, no wonder these stupid ependymoma tumors come back up to 23 years later.  How could any human do a gross total resection?  Yet, there is still a high cure rate with a gross total resection.  This shows how important a good surgery is for curing this thing.  Maybe this 2nd surgery was the seemingly impossible perfect surgery Jude needed.

The radiation ocologist said that he thought the radiation cure rate was 70-80% over a 5 year period, and that he expected that there is a 20-30% chance of the cure without surgery.  In other words, he thinks the cure rate would go up by 50% with radiation.

He thought the side effects of radiation would be minimal, but hadn’t reviewed Jude’s scan so he was relying on me to tell him the location of Jude’s tumor.  He said St. Jude’s Children’s Hospital, where he used to work, had noticed that each time there is a brain tumor or a brain surgery, the patient’s IQ drops 1-2 points.  So, Jude could have been a super genius instead of just a genius if he’s right about that.

Jude is a smart kid so I figured I could give him a little information and figure out what he thought about whether we should pursue radiation treatment. It’s his brain, after all, that would be radiated. I didn’t tell him about the side effects of radiation or the risk of spreading if this tumor is not gone. I just said that the neurosurgeon thinks he got out the whole head owie, but that if he left any behind we could go to the “zap doctor” (yes, I made up this more fun name for the radiation oncologist) in Indiana and the zap doctor could zap the owie to make it go away for good. Jude said something like, “Silly Mommy, no one knows but God whether my head owie is all gone. There could be some hiding on the top of my brain or on the bottom of my brain. We should go to the zap doctor.” He then kept touching his finger to his head and saying “zap” while laughing hysterically. He thinks a zap to the head would tickle. Glad he is so smart, cute and innocent. But, I do hope he’s wrong that some is hiding and I hope the neurosurgeon really got it all this time since I don’t want to go to the zap doctor.

In preparation for the meeting with Jude’s oncologist today, I read all the literature (5 articles) describing what happened to various patients world-wide with this tumor who received different treatments.  So, I feel like I know more than ever what we are up against.  After the meeting, I’m feeling more comfortable taking our chances on the surgery alone being the cure for Jude.  The big take-aways for me from the meeting and articles were:

1.  Surgery alone is often curative for ependymomas if gross total resection is achieved and given that the tumor was smaller the 2nd time, and the surgeon thinks he got it all (as he did last time), the 2nd surgery should have been easier than the 1st one.  Nevertheless, the standard of care at recurrence is radiation.  We found 3 patients world-wide attempting surgery only at recurrance with the same type of tumor and it worked well for 2, but the other died from his tumor (it kept recurring and they hit it with radiation and chemo and it didn’t work).

2.  Given the small #s of similar patients involved (20 or so worldwide), we don’t have good data, but her gut reaction is that there is a 20% chance the tumor would come back after radiation.  There is a 30-60% chance the tumor would come back without radiation.  I would be sick if that tumor came back after radiation.

3.  If there is any residual tumor cells in Jude (which we hope is not the case), there is a 30% chance they will morph into a more aggressive Grade III cancer.

4.  Jude’s tumor isn’t associated with the ventricle, which lowers the chance of spread into the spinal fluid.  However, given that the surgeon had to cut a path from the surface of Jude’s brain to the tumor bed, any residual tumor cells could seep out to the surface of the brain and that would be most likely fatal.  The chance of metastasis at another recurrance (which would be fatal) is 5% or so.

5.  Even with radiation, the tumor can still come back and still spread before it is detected.

6.  15% of children with supratentorial cortical ependymomas die somehow from their tumor (from the tumor getting too deep in the brain to be removed or from spread.  But, this accounts for all Grades of the ependymoma and Jude has a Grade II rather than Grade II and Jude’s location is more favorable than other locations.  So, we are still looking at a big chance of a great out

7.  I told her that $ is no object here and we can self-pay if we have to and wanted to know if there is anyone else that she feels we should consult with.  She’s going to follow up with an oncologist with emphasis on ependymoma in Denver who doesn’t always do what everyone else does to see what he thinks about Jude’s case.  She thinks he is more likely to think that not doing radiation would be a good decision.  She did say that if we decided not to pursue radiation, it would not be a “bad decision.” 

8.  When I asked her if she would do the radiation at this point if this was her own child, she hesitated and said slowly (while thinking about it), “I think so.”  She said that that is a very difficult question to answer and she’d like to think she practiced medicine that way.  Overall, I got the impression that she would really wrestle with this decision, just like I am, if this were her own child. 

9.  She said that the radiation “could just look like it’s helping because so many patients get it.”  In other words, it may not actually be helping.