Just when you think you have things figured out......

6:24:00 PM Atticus Army 4 Comments

Hey Army, 

Well, it has been quite the crazy week.  Emmie and I were anxious as it was for the MRI scheduled on Tuesday as part of our preadmit visit for chemo cycle 4 on Wednesday.  As you have probably gathered from previous posts, CPC (choroid plexus carcinoma) is a pretty aggressive cancer.  So needless to say Emmie and I had "scanxiety" in full effect.

He likes coloring while we wait to go back

My wife's sister was soooo kind and drove out to be with us during the scan, and to take our kiddos for the week so that Emmie and I could both be with Atticus during his chemo treatment.  Sure enough Tuesday morning came and we found ourselves sitting in the radiology waiting room asking each other how we ended up here.  I find myself more ad more lately reflecting on the choices, decisions, paths, and turns that lead my little family down this path.  It's oddly amusing to look back just a few short months ago and recall, all too vividly, how I felt that everything was decided, and working out just how we had planned.  I guess I would say to my past self, "  The best-laid plans of mice and men often go awry.  No matter how carefully a project is planned, something may still go wrong with it."

Boy isn't this true.  I am not saying this to complain, or self-defeat, it is just a fact.  It seems often in life that once you feel that you have it all figured out that something comes along to test the foundation as it were.  It seems that our foundation was in need of some repair.  Emmie and I have spent many days and nights full of tears, frustration, anger, resentment, and fear.  But, above all weakness, we have found that neither myself or Emmie could have done this on our own.  The path we find our little family on is a tricky one and traversing it all the way to the end doesn't guarantee all of your troops will be present.  We have seen first hand how easy it is to fall off, and the results are devastating to a family.  It is so easy to let that anger, and frustration to turn onto those that are close to you.    We have out of necessity learned that our strength needs to be firmly planted both in each other and buoyed by the Lord.

We handed our little warrior off to the anesthesiologists once again and sat and waited.  After what felt like a lifetime our little guy was returned to us, and to him at least he was only gone for a moment.  Our wonderful nurse practitioner poked her head in and said, "Hey I just walked by radiology, and first there is no evidence of any tumor growth at all!!"  We, of course, were elated and relieved.  It felt like the biggest weight was lifted off our shoulders.  But then we realized that she said, first off.......  It was like waiting for the other shoe to drop,  she said that there appeared to be a "trapped ventricle" and our neurosurgery team was looking at it and we would meet with them later.

What did this mean??  Well, as it turns out as Atticus's brain healed after the initial resection there was a portion more towards the front that got walled off by scar tissue.  One way to look at this would be to think about a lake with multiple inlets but only one outlet.  If you were to dam up one of the streams that fed into the lake over time it would continue to swell and swell with nowhere for the incoming water to go.  The lake would receive minimal impact as this was only a small feeder stream, but to the areas surrounding the stream it could potentially have a big effect.  This is very similar to damming off a section of the ventricle.  The cells that reside in the isolated part will continue to produce fluid, and with nowhere to drain, it swells, and the body produces a cyst.  

Elsewhere in the body, we would probably just pop it, let it drain and forget about it.  In the brain, it isn't quite that simple.  In growing and swelling there are a lot of things that can be damaged, much like the vegetation, and life surrounding that little stream.  But in this case vegetation is represented, by motor function, speech, balance, higher learning and all the life sustaining things that your brain does each day without you even knowing about it.  This, of course, needed to be addressed.  At this point, the cyst wasn't big enough to cause any serious, or permanent side effects, so the question wasn't if the intervention needed to take place, but that of when.  

Chemotherapy is a very thin line to walk on, we want to give enough medications to disrupt and destroy cancer-causing cells, but not enough to cause irrebuttable damage to the normal function of the body.  The gap between these two effects is typically termed the "therapeutic index."  Think about it as the distance between two points, the further the two points, let's say..... Seattle to Pullman, the safer the drug, and less of a chance of undesired side effects.  But if those points were to be from 145th street to 150th street.  You can see how that window isn't very big.  Atticus currently was in this little window where he had recovered from the last round of Chemo enough to start the next round, but in starting the next round that gap would close and then doing other things like surgery would be much riskier.  

So the decision was made to postpone our next chemo cycle in lieu of this window.  So the next question was how to fix it.  They decided to have us wait and they would present it at the tumor board meeting on Wednesday.  So we meet with the chief surgeon on Wednesday afternoon and we concluded that we would open a portion of his original incision up and then endoscopically fenestrate (cut holes in) the cyst to try and help it communicate with the rest of the ventricle so it can drain.  Then they put in a catheter in that would come out to his skull just under the skin so that if it formed again they could tap into this reservoir and drain it without us having to go to surgery.  So we were set for surgery on Friday.

It is important to keep your mind occupied during surgery!  We resorted to cards.

While it is true that this surgery is very different then the last big surgery we went into, our nerves couldn't tell the difference.  It was hard to send Atticus back into surgery when he was doing so well. We felt that we had finally got on top of all the side effects of chemo, and he was acting like a normal terrible two years old should.  Telling us NO, getting into everything, throwing fits, and Emmie and I love every single one of them! In fact, he has got quite a sassy attitude on him, he cracks us up.  

Inevitably Friday came, and we came to check in for our procedure.  We were able to meet with our surgeon prior to him heading for anesthesia.  She reassured us as the chief surgeon did that this surgery is nowhere near the risk, or technical nature as the one we have already gone through, but, it's still brain surgery.  We handed our little boy off yet again around 12:30 for his MRI after which he would head straight to surgery, followed by a post surgery MRI to confirm placement.  The next 5 hours seem to just slither along and a snail's pace.  Emmie and I did the best we could to pass the time without worrying and found ourselves in the gift shop buying a deck of cards and commenced to play Jin, BS, and other such games to pass the time.

It was so sad to see this again.... we felt so bad for our little guy!

If you look close, you can see a little line that goes from the middle of his brain to that base of that little bump on his head.  That is the catheter they put in, so they could access the trapped ventricle if they had to.

Finally, we were able to hold Atticus again, it's always an emotional reunion to have him back safe in our arms! We snuggled him in recovery and then we were brought up to our room around 6:30 PM. Caleb and I were also exhausted from the day and the worrying about surgery! We ordered in pizza and were able to get our little boy snuggled in for the night! Atticus had a good nights rest, which we were so grateful for!

So we are off to recovery to get rested and ready for the next chemotherapy session to start next week!!!

we had to celebrate his birthday in the hospital.

He didn't seem to mind his birthday in the hospital.

We even had a fellow cancer friend come to visit.

Thanks for all of you support.

This was Emmie's present she got me for my birthday! I love this drawing!

Time to catch up!!

9:06:00 AM Atticus Army 1 Comments

Hey Army,

Well, first off I would like to apologize that we haven't posted anything in quite a while.  We have just been so caught up with living life here in Seattle with our little family writing a blog post as half way been forgotten.  I promise we have not forgotten about our army and all the thoughts and prayers on our behalf.  Nevertheless, there is quite a bit to catch you all up on.  So, without further ado, these are the highlights.

Some pics back when Atticus had his hair! :-(

As you may all recall, Atticus needs to have an audiology (hearing) test before each round of chemotherapy.  Besides causing nausea and the blood numbers to drop, the Cisplatin that he had to take for the first 3 cycles of chemotherapy is ototoxic (causing hearing loss).  The first test that we did to start everything off was somewhat inconclusive.  It did show a little bit of hearing loss, but Emmie and I weren't too convinced that it was correct as Atticus was a bit uncooperative for the test.  But, it gave us a baseline nonetheless.  After the first round of chemotherapy, we had our second hearing test and it went much better than the initial one.  Emmie and I were thrilled.  The second round of chemo was pretty rough on a few different levels which also encompassed his hearing we found out later.

Atticus at his most recent hearing test.

His third hearing test was quite a different story.  Emmie and I were devastated when we heard the news.  After all that has already happened, the surgery, the partial vision loss, and all the side effects we have already seen, here we go just adding on another.  Luckily, the current hearing loss has only affected the higher tones very few of which dip down into the areas of speech.  So at this point, we don't have to look into anything like hearing aides.  The only problem is that the full extent of the hearing loss won't be found for about a year.  What it does mean right now is that we had to change our third cycle of chemo a bit.  After consulting with our oncologists they decided to cut the cisplatin that caused the hearing loss had to be cut in half.  This should stop any further damage and then we will just have to play the waiting game to see the extent of the damage.  For now, he is unable to hear anything above 8,000 Hz, and our hope is that it will not get any worse over the coming months.  Even so, it is very likely in the coming months that we will have to start looking into some hearing aids.

Thanks giving was great! We even got served by a Seattle Seahawk!

Was able to keep up the Thanksgiving tradition of going to a movie.

Always so sad when Family has to leave.

The next big thing is that we finally got our genetic testing results back on the tumor tissue.  After the surgery, we sat down with our oncology team and had a pretty lengthy conversation on what to expect in the coming months, and about some of the options in the treatment protocol.  One such option was whether we wanted to have genetic testing done on the tumor.  When they test the tumor they will sequence somewhere around 150 or so different genes known that cancers typically overcome in formation of various cancers.  Having an idea of which genes a given cancer has mutated or silenced can, at times, give insight into how a tumor might have formed, and give valuable knowledge for possible treatment options, or future preventable medicine a patient may need to take.

Unfortunately, ours was a bit of both.  Our tumor came back with a mutation in a gene called TP53.  Without "geeking out" I will do the best I can to portray what this might surmount to.  TP53 is, among many things, a tumor suppressor.  Its primary job is to ensure that the cell is behaving and replicating as it should, and when the cell acts out it's much like a conductor of the orchestra.  The conductor is seated in just the right spot and has the power to stop the song at any point the orchestra starts to drift.  He may call the strings to attention to fix a certain part.  Once fixed the whole orchestra resumes and the song can continue.

Much like the conductor, TP53 does not like the cell act out, or just copy away without regard to fidelity.  If you think about how many times our ENTIRE genome is copied each day it is phenomenal that every person walking around us each day doesn't have cancer!!! But that is a topic for a different discussion.  Needless to say, when there are problems within the cell TP53 knows it and can do one of many things.
   1. If there is a problem in the copying of DNA, it may call to attention DNA repair proteins to fix the problem
   2.  It can stop the growth cycle, much like the conductor stopping the orchestra, to allow other things to find and fix the problem
   3.  If all else fails it can initiate something termed "apoptosis" or cell death.

At times a cell is damaged beyond repair, and to save the host, or us in this case, it is better to let a cell die than to risk the alternative.  Often times the alternative is tumor growth.  The exact type of tumor is dependent on where this cell lives, and it's function.  It may be a liver cell, Hepatocellular carcinoma, it may be a white blood cell, Leukemia, and so on and so on.  Now, I understand that I am simplifying the formation of cancer a bit, but suffice it to say that TP53 plays a major role in cancer suppression.  An inactive, or mutated TP53 gene can allow cells to replicate and proliferate unmonitored, resulting in cancer.

Atticus Army drawing! We LOVE IT

It should be no surprise then to find that close to 50% of all cancers are found to have a mutation in this gene!  At this point you may, and should, be asking your self. "well gee, if this is so common why are you telling me all of this stuff?"  Very astute question!

The real question is where did the mutation come from.  It starts to beg the question did the chicken come first or the egg???  Or in other words did Atticus's tumor mutate the gene......... or was the gene mutated and allowed the tumor to grow.   The former (and subsequently the desired result) would be a very unfortunate sequence of events that led to this horrible childhood illness.  The latter could be a foreshadowing of what might be, and subsequently called Li-Fraumeni syndrome, or an inhibited ability to keep tumors from forming.

While Li-Fraumeni syndrome is rare, it is the very thing we are currently testing for.   Because Atticus's tumor did show this mutation it is not clear which came first (the chicken or the egg).  So we will do some further testing to see if this mutation is found systemically (in is the whole body), or just in the tumor.  We, of course, are hoping and praying for it to just be found in the tumor. We will keep you updated!

I love this little backpack!

Playing Dr. during clinic.  He loves playing with these animals, but he makes sure they are always back on his backpack.

On a brighter note.  We have been so very blessed with such amazing friends from our community back home in Pullman Wa.  They have been working tirelessly and have put together a very special Christmas breakfast to support our little Atticus with his fight against brain cancer.  They have planned it to take place on his 2nd Birthday December 10th.  It is a quite odd thing for Emmie and I.  While we will be forever appreciative of all the love, prayers, and service we have received we quite often don't feel worthy of it.  That being said, this birthday for Atticus is quite special as without the all of the wonderful medical providers we have had the joy of being served by, this is a birthday that we may have never seen.

If you happen to be in Pullman, please share and join in with them in celebrating and supporting.  This is an activity of hope, thankfulness, and joy in this wonderful season.  We can't express how thoughtful, and meaningful this for our little family, especially in this holiday season.  Sometimes in life, you need to accept help when offered, and our family and friends there just seem to know what we need during this hard time.  So.... to all of those that have offered to serve, and have donated their time and talents, and to those that attend.  Thank you from our family.  I wish I could be there to give each and every one of you a big hug.



Click North Pole Breakfast for Atticus's Army for more information.