Liam’s Passing

This entry is part 10 of 10 in the series "Liam's Battle" --

2Harris-8vin1Sept2015 – Liam took his last breath at 4:36 am. His departure from this life was peaceful. Fred and I were by his side.

2 Timothy 4:7-8 “I have fought the good fight, I have finished the race, I have kept the faith. Now there is in store for me the crown of righteousness, which the Lord, the righteous Judge, will award to me on that day-and not only me, but also to all who have longed for his appearing.”

mIBG – We are back on!

This entry is part 3 of 3 in the series "Radiation" --

WARNING: Science Content


As with all my posts, I try to include some thought provoking science, as well as interesting water cooler talk.  Good luck.
 
MetaIodoBenzylGuanidine

MetaIodoBenzylGuanidine


[Prologue]

For those avid followers of all of the science that I have introduced during all of this, you will recognize that this post is a rehash, but an exciting rehash.

Early on, Liam was initially slated to participate in a mIBG (MetaIodoBenzylGuanidine) trial.  The trial wasn’t supposed to happen until after the induction period of chemotherapy.  The catch is that only 80-85% of neuroblastomas absorb mIBG.  This is why fairly soon after diagnoses, while there is still a lot of cancer in concentrated pockets throughout the body, an mIBG scan is given to see if the particular variety of neuroblastoma picks it up. This is done with [123]- mIBG (see below.)

When this scan was performed on Liam back in September, there had been problems and the scan was not performed right away.  The doctors and hospital staff were having difficulty stabilizing Liam.  In fact, that first session in the hospital lasted over 21 days.  He had been started on  a fantastic chemotherapy drug called Topotecan (which is a TopoIsomerase I inhibitor that is so darn cool, it deserves its own post and I will not discuss it here.)  The problem was that Liam was spiraling out of control while he was on it. He started having trouble breathing, and he ended up with a plural effusion (yep… that was a bad couple of days. Click here for the post from that day).  After all was said and done, the mIBG scan was pushed off until he was admitted for chemo round 2.

When the [123] mIBG scan was finally performed, it came back negative. We were bummed, but the chemo seemed to be going so well that we really didn’t give it much thought. Liam was feeling better.

After 6 rounds of chemotherapy, a follow up PET scan was performed. No cancer showed up on the scan. He had a remarkable response, and we thought we were doing pretty well.  In reviewing all that had happened over the course of 6 rounds of chemo, I wondered if most of the cancer had vanished just after the first round of chemotherapy.  If it did, it would have skewed the mIBG test to a negative result.

Looking back at the sudden improvement after round 1- the plural effusion (now believed to be caused by cancer dying his lungs) and all of the immediate weight loss (now believed to be the cancer dying in his abdomen)…. he looked normal for the first time in months; I contend that the Topotecan chemotherapy made most of the cancer disappear quickly.  His response even astonished his doctors.

Now that the cancer has come back,  it was suggested by the doctors at CookChildren’s that we look one more time at the mIBG.  So, we did, and it gave a positive response to mIBG.  It can clearly be seen in the left tibia and the pelvis.

mIBG Liam 24July2015

123-mIBG Scan of Liam on 24July2015. His neuroblastoma has soaked up the mIBG compound, and due to its radioactivity is exposing the film. His trouble spots in his left leg and pelvis can clearly be seen.

So what does this mean? It means that we now have a really awesome tool in our tool chest to fight this. It won’t cure the neuroblastoma, but hopefully we can knock it down and coupled with other therapies, we can get this disease under control for Liam.  This is an option that a week ago we did not have.

[End of Prologue]

So, how does it work? It turns out that Neuroblastoma has a strong affinity for mIBG in about 85% of cases.[1] In a very high percentage, the Neuroblastoma cells will take this compound up while the normal cells will not. This is called ‘selectivity’. (i.e. the Neuroblastoma soaks this compound up selectively over normal cells).  mIBG in itself, however, doesn’t do anything. It is taken into the cell, and then is excreted from the cell at a later time.  This means that the Neuroblastoma cells are not sensitive to the compound. 

A clever and ingenious pupil of chemistry can already see what to do next.  Swapping out the Iodine atom on this compound with the radioactive version  makes this molecule very useful.


[123]-Iodine

123 Iodine will decay by electron capture to form 123 Tellurium which will then emit a Gamma ray with an energy of 159 keV. This is useful for imaging.  This is like having an x-ray performed, but rather than having an x-ray source shining high energy light through Liam, the light will be generated inside him!  Since this radioactive atom is attached to a compound which is only selective to Neuroblastoma, Gamma rays (like x-rays) will be generated only at the Neuroblastoma sites.  With the correct detector, the Neuroblastoma will light up like a Christmas tree.


[131]-Iodine

If 131 Iodine is used, different results will be observed.  131 Iodine decays in the follow two manners (statistically a 90% Beta(-) Decay and a 10% Gamma decay):

(Beta(-) Decay ~90%) {^{131}_{53}\mathrm{I}} \rightarrow \beta + \bar{\nu_e} + {^{131}_{54}\mathrm{Xe}^*}  + 606 keV 

(Gamma Decay ~10%) {^{131}_{54}\mathrm{Xe}^*}  \rightarrow {^{131}_{54}\mathrm{Xe}} + \gamma  + 364 keV

The Beta(-) decay produces a very energetic electron and an Antineutrino which have a tissue penetration of about 0.6 to 2 mm. This is enough energy to destroy cells. (i.e. a cell sized atomic bomb) So in essence, this gives a pathway for the mIBG, which is very selective to the Neuroblastoma, to blow up the cells (and leave the good cells alone).


This mIBG scan that was performed today only involved 123 Iodine for gamma ray imaging (see above images). This indicates all of the places that the Neuroblastoma is, with a few exceptions. There are false readings in some of the places like the thyroid (which regulates Iodine containing compounds).    In the coming weeks, it’ll be time to bring out the [131] Iodine and give this cancer the radioactive punch it deserves.

  1. According to NationWideChildrens.org “…Roughly 80-85% of neuroblastomas will absorb MIBG. There are really 2 ways in which MIBG treatment is used. In both methods, the MIBG chemical is attached to an iodine molecule that has been made radioactive. The radioactivity can be either a low-dose or a high-dose…. ”

Gene Blues and MYCN Amplification

This entry is part 9 of 10 in the series "Liam's Battle" --

WARNING: Science Content


As with all my posts, I try to include some thought provoking science, as well as interesting water cooler talk.  Good luck.
 

Neuroblastoma Genetic Testing

There is a lot of stuff that is not known about Neuroblastoma, but there are a few key markers on the genetic sequence that have had some statistics gathered and can provide an inkling of whether or not the particular breed (or genetic makeup) of the Neuroblastoma is going to be more (or less) difficult to fight. The list I have here is not comprehensive. I am not a medical doctor. So, I offer my own interpretation, which I may find later to be inaccurate. If I do find inaccuracies, I will correct them. I am a chemist, but biochemistry is not my specialty. I am a material scientist, so there is a good chance I will get some of this wrong. I have approached this with the background that I have and with the attitude that I am a dad who needs to understand what the heck is going on, and I am learning as fast as I can!

I have included a *brief* reminder on the genetics of the human body for those audience members who have (ahem) forgotten some of their high school biology.  Also, there is a lot of blockquotes in this post. Feel free to follow the references and see just how deep the rabbit hole goes. 🙂

What are genes?

BY-SA 3.0 Zephyris

Genes are like the lines on a blueprint for living things.  Your genes are in-fact what make you distinctly a human and not a cat, dog, or dinosaur. All living things have genes. Genes are the sequences of DNA that instruct the cell on how to build protein or peptide sequences. Genes (DNA sequences) are all lined-up into libraries called chromosomes. The human body has 23 chromosome pairs (46 chromosomes, half from mom and half from dad).  No matter which cell you look at within one individual, whether it be from the big toe or the liver, each cell in a body has a copy of all of the chromosomes.  There are approximately 3 billion base pairs (Yes…~3,000,000,000) required to make the 46 chromosomes in each cell. That is a lot of copying of base pairs that is going on all the time. It is quite phenomenal that it happens correctly once, much less regularly in the ~37 trillion cells in an adult human.

Neuroblastoma Genes – As far as we know, what can go wrong?

Well, I think that it is pretty clear that there is a lot that is not known about what goes wrong in cells that have turned into Neuroblastoma. There is currently no known link to any cause, and to make matters even more confusing, some kids can actually get Neuroblastoma cells, and at a later date be completely free of them without any treatment. (This only happens with Stage 1 low risk Neuroblastoma. There are no known cases of Stage 4 high risk Neuroblastoma suddenly disappearing.)

Given all of this, however, there are some things that can be determined from a genetic test of the Neuroblastoma cells. The primary reason for testing the cells is to estimate how challenging the fight will be for a patient’s particular Neuroblastoma.

 MYCN Amplification

The  MYCN protein regulates fundamental cellular processes from proliferation to apoptosis (cell death). If the cell has more than 10 copies of this protein, it is bad. So bad, in fact, if Neuroblastoma cells have this, then it is automatically classified as high-risk (even if it were discovered early and would otherwise be classified as low-risk). The MYCN protein genetic information is located on chromosome 2p24.3 between base pairs 15,940,560 and 15,947,006. [Ref]

MYCN is a protein and a member of the MYC family of proto-oncogenes.  A proto-oncogene is a normal gene that has the potential to become cancer because of mutations or some type of increased expression (expression is how information from a gene is utilized to create another genetic product such as a protein).  “Like many other MYC proteins, MYCN is a transcription factor that controls expression of many target genes, which in turn regulate fundamental cellular processes including proliferation, cell growth, protein synthesis, metabolism, apoptosis and differentiation” [Ref]

Having too many copies of the MYCN protein makes this type of cancer hard to fight, but some interesting findings actually show that the MYCN protein may in fact be involved in the creation of the Neuroblastoma [Ref], although this is disputed other places [Ref]. It is possible that there is a system of checks and balances with some of the genetic information on chromosomes 1 and 11, which may help regulate over duplication of the MYCN protein, and the amplification of MYCN is closely related to  “missing” information on these Chromosomes. (Also called a ‘deletion’)

Deletion From Chromosome 1 and Chromosome 11

The deletion of a chromosome implies that there are areas of the genetic code, which we know are typically present in the DNA, that are missing from the sequence of the person whose DNA is being examined. Deletion from chromosomes 1 and 11 seem to be linked to to Neuroblastoma:

… Researchers believe the deleted regions in these chromosomes could contain a gene that keeps cells from growing and dividing too quickly or in an uncontrolled way, called a tumor suppressor gene. When a tumor suppressor gene is deleted, cancer can occur. The KIF1B gene is a tumor suppressor gene located in the deleted region of chromosome 1, and mutations in this gene have been identified in some people with familial neuroblastoma, indicating it is involved in neuroblastoma development or progression. There are several other possible tumor suppressor genes in the deleted region of chromosome 1. No tumor suppressor genes have been identified in the deleted region of chromosome 11. [Ref]

About 25 percent of people with neuroblastoma have a deletion of 1p36.1-1p36.3, which is associated with a more severe form of neuroblastoma. Researchers believe the deleted region could contain a gene that keeps cells from growing and dividing too quickly or in an uncontrolled way, called a tumor suppressor gene. When tumor suppressor genes are deleted, cancer can occur. Researchers have identified several possible tumor suppressor genes in the deleted region of chromosome 1, and more research is needed to understand what role these genes play in neuroblastoma development. [Ref]

The Gene List: (Changes in These Genes are Associated with Neuroblastoma)

 

Liam’s Results

Fluorescence in-situ hybridization (FISH)

When Liam was in the hospital the first time, a sample of the neuroblastoma was collected from his bone marrow before he began chemotherapy. This sample was sent off and analyzed by the FISH method. (Specifically the report says “FISH for MYCN (2p23-24) gene amplification,” which we know from above is one of the important mutations that predicts poor prognosis.)

Out of the 200 cells probed for MYCN Amplification by the FISH technique, 16 showed to be abnormal, and 184 showed to be normal. Indeed, his Neuroblastoma cells show MCYN amplification.

Chromosome Analysis by Karyotyping

Karyotyping is a way of analyzing the chromosomes for number and completeness. The results from Liam’s test are as follows:

  • 21 cells were counted and analyzed, and 3 of the cells were karyotyped.
  • 19/21 cells were of normal male chromosome compliment
  • 2/21 cells showed a gain on Chromosome 7 and multiple “double minutes

The gain on Chromosome 7 and extra fragments of DNA material are likely a direct cause of the amplification of MYCN gene.

Further Discussion

Now, this is discouraging news, and the words “poor prognosis” have bounced around my cortex for a while. What do we make of this? How can we put this all in perspective?

I was encouraged by a Japanese article which investigated the use of blood stem-cell transplantation (SCT) to treat Neuroblastoma (SCT is a procedure that Liam is scheduled for early next year). It would appear that SCT increases the odds of survival of Neuroblastoma patients with MYCN Amplification significantly (from the low 20% to about 50% survival after 66 months). The article concludes with the following statement:

Not all patients with advanced neuroblastoma who have more than 10 copies of MYCN will die. The requisites for survival in such patients seem to be intensive induction chemotherapy, effective surgery, irradiation, and the use of SCT.  [Ref]

Positive things to consider:

  • This test did not show Chromosome 1 or 11 deletion.
  • The DNA gain was in Chromosome 7 and in double minutes. Chromosome 7 is not tied to anything normally seen with Neuroblastoma. So it is possible that the MYCN has amplified a part of the DNA that might be easier to fight than a Chromosome 1 or 11 deletion (which takes away some of the regulation of MYCN). We will have to wait and see.

MetaIodoBenzylGuanidine (mIBG) [update]

This entry is part 1 of 3 in the series "Radiation" --
MetaIodoBenzylGuanidine

MetaIodoBenzylGuanidine

[Update to the Update: On 24July2015 after his cancer had relapsed, another mIBG scan was performed. This time, the cancer was shown to be mIBG avid]

[Update: On 4Sept2014, during the imaging 123-mIBG, it was determined that Liam’s Neuroblastoma is one of the types that does not take up mIBG. This means that he will be excluded from participation of the 131-mIBG study and we will be performing more chemo treatments]

Let’s not leave out the cool science.  During the course of his treatment Liam will encounter mIBG (MetaIodoBenzylGuanidine).  The first time that he encounters it, should all go well, is this week (~4Sept2014). He will be injected with the chemical so that his Neuroblastoma can be imaged. So, how does it work? It turns out that Neuroblastoma has a strong affinity for this compound in about 85% of cases.[1] In a very high percentage, the Neuroblastoma cells will take this compound up while the normal cells will not. This is called ‘selectivity’. (i.e. the Neuroblastoma soaks this compound up selectively over normal cells).  mIBG in itself, however, doesn’t do anything. It is taken into the cell, and then is excreted from the cell at a later time.  This means that the Neuroblastoma cells are not sensitive to the compound. 

A clever and ingenious pupil of chemistry can already see what to do next.  Swapping out the Iodine atom on this compound with the radioactive version  makes this molecule very useful.


[123]-Iodine

123 Iodine will decay by electron capture to form 123 Tellurium which will then emit a Gamma ray with an energy of 159 keV. This is useful for imaging.  This is like having an x-ray performed, but rather than having an x-ray source shining high energy light through Liam, the light will be generated inside him!  Since this radioactive atom is attached to a compound which is only selective to Neuroblastoma, Gamma rays (like x-rays) will be generated only at the Neuroblastoma sites.  With the correct detector, the Neuroblastoma will light up like a Christmas tree.


[131]-Iodine

If 131 Iodine is used, different results will be observed.  131 Iodine decays in the follow two manners (statistically a 90% Beta(-) Decay and a 10% Gamma decay):

(Beta(-) Decay ~90%) {^{131}_{53}\mathrm{I}} \rightarrow \beta + \bar{\nu_e} + {^{131}_{54}\mathrm{Xe}^*}  + 606 keV 

(Gamma Decay ~10%) {^{131}_{54}\mathrm{Xe}^*}  \rightarrow {^{131}_{54}\mathrm{Xe}} + \gamma  + 364 keV

The Beta(-) decay produces a very energetic electron and an Antineutrino which have a tissue penetration of about 0.6 to 2 mm. This is enough energy to destroy cells. (i.e. a cell sized atomic bomb) So in essence, this gives a pathway for the mIBG, which is very selective to the Neuroblastoma, to blow up the cells (and leave the good cells alone)

This mIBG therapy is typically only given in cases of Nueroblastoma that have relapsed, however, we may be one of the lucky families that gets this treatment as a clinical trial directed at making this part of the standard treatment.  If everything works out well, we will be part of this trial sometime after 5 or 6 chemotherapy treatments.


This mIBG scan being performed this week will only involve 123 Iodine for gamma ray imaging. This will indicate all of the places that the Nueroblastoma has metastasized to. It will be used as a baseline for how he is progressing in his treatment later on.

  1. According to NationWideChildrens.org “…Roughly 80-85% of neuroblastomas will absorb MIBG. There are really 2 ways in which MIBG treatment is used. In both methods, the MIBG chemical is attached to an iodine molecule that has been made radioactive. The radioactivity can be either a low-dose or a high-dose…. ”

Pleural Effusion. Headed in the Right Direction?

This entry is part 4 of 10 in the series "Liam's Battle" --
Pleural Effusion

Pleural Effusion

So, those that have been following along this week know we have seemed to go downhill rather than up. Round One of Chemo was finished 24Aug2014. While the chemo seemed to be a non-event, these other complications have kept occurring one right after the other.

On Tuesday, he developed a rash for no real good reason. Initially, we thought that this might be due to one of the blood transfusions; however when the rash came back even stronger the following day after one of his medications, it strongly pointed to a possible allergic reaction to the drug. My understanding is that this is a preventative medication that is given during the chemo process to avoid a certain type of pneumonia developing while the immune system is weakened. It is a type of sulfa drug. I personally have had strong reactions to sulfa-drugs in the past, and while I am assured that the allergy to sulfa drugs is not genetically linked, sulfa drugs do often have strong allergic responses in people. So, while the rash is still dissipating, this is the strongest lead we have to go on. The medication has been switched to a different one, so we will see as time progresses forward.

Of biggest concern this week was when they were trying to wean him off of his requirement for [concentrated] oxygen, and they couldn’t. An X-ray showed us that he had developed a pleural effusion in his left lung. Initially, the fluid on ultrasound appeared to not be bloody (They would be able to see clotting). They decided that they would drain the fluid with a needle to see what it was.  Right before the procedure (or maybe it was during), an ultrasound this time showed that there was clotting. And sure enough, when they sucked the fluid out, it was bloody. Where was this fluid coming from? A CT was performed so that they could see the chemo port that was installed to determine whether the port site was bleeding or leaking into the space.  The CT showed that the port seemed OK.  This left us with a wait-and-see approach to the problem.  The only good explanation was that since he had been low on platelets, maybe he had spontaneously ruptured in the pleural space. They would wait until the next morning to see if the cavity had filled back up. If it had, they would do emergency exploratory surgery to:

  1. Find the source of the fluid
  2. Install a drain tube into the pleural space
  3. Possibly remove the port, just as a precuation

The next morning, 28Aug2014, an x-ray was performed.  The plerual effusion was back just as large as it had been the day before. Liam was headed for surgery as soon as it could be scheduled.

A little after 12PM, Liam was wheeled into surgery.  The surgery took about 90 minutes. They removed his port, just in case they missed something in the CT. They installed a drain tube into the pleural space, and they looked around for the leak with a camera.

They did not find one.

They sent the fluid for analysis to see if it contained Neuroblastoma, which we do not have the results for; however, whether or not it is there may not tell us much.

So what is going on?  Nobody knows!  But here is the best hypothesis that I heard in the debrief:

If the Neuroblastoma had made it into the pleural area, and the chemo had killed it, it is possible that the fluid and blood could be a response to the now dead Neurblastoma cells.  That would mean that the chemo is having an affect on the cancer and this whole plerual effusion business is a consequence of healing. Only time will tell.

This morning, 29Aug2014, an x-ray showed us that the pleural area is being drained effectively with the chest tube.  Now we wait for the body to heal the pleural area like it would heal a bruise anywhere else on the body.

 

Damaged Veins and the Worst IV Ever

This entry is part 3 of 10 in the series "Liam's Battle" --

[This post mentions blood and needles. Do not read if you are squeamish.]

On 17Aug2014 after it was determined that Liam had cancer, I was trying to put all of the pieces together. Jenn was working to get herself up to the hospital. I was in the ER room with Liam, staring at a word on a napkin. The word ‘Neuroblastoma’ written in a quick but legible way was a word I had never heard of, but at this point it had at least two reasons to despise it:

  1. I knew it was cancer.
  2. I was told not to freak out when I looked it up.

Some people do not like to know what they are dealing with, but I am not one of these people.  I reached for my iPhone, and I started to Google it.  Right then, the nurse walked in and announced that they needed to start an IV.  Another nurse followed behind her.

“OK.” I said.

The nurse grabbed several pieces of equipment from an OmniCell which looked like a vending machine, but had all of the different types of equipment needed for the various procedures that can be performed in that little room.  She grabbed several items out of each bin making sure to push a button located on the front of each bin. Each time a button was pressed, a little green light came on for the individual bin.  “Clever,” I thought, “That is a neat way of keeping and managing the inventory.”  Then I thought about how much each item would cost as it eventually showed itself on a bill from the hospital labeled ‘Services Rendered’.  After some quick thinking, I came to the conclusion that it really didn’t matter.  These were the things that were needed at that moment, and I was glad they were available.  The nurse closed the door to the OmniCell, pushed a master button in the middle that told it she was done, and laid out the various pieces needed to start the IV on the bed.  All of the pieces seemed necessary for the insertion of an IV.

The nurse announced that she would be giving Liam a topical anesthetic before the IV was started that should help with the placing the IV. It sort of reminded me of an epi-pen, but it was much smaller in size. The topical made a mark on his skin on the top of his hand. He cried, but I knew it would feel better than the alternative.  The nurse grabbed the needle and started going after the vein.  As with most things to do with a needle, I looked away. In this case, I looked at Liam’s face. As I watched his contorted face from the painful needle being manipulated under his skin in such a way to find the vein, it was apparent that the topical did not seem to be working very well.

The IV went in, and then it was time to flush it.  Which involves pushing fluid back up the IV line.

“It blew.”  She said.

“Blew?”

“The vein fell apart.” She said “We’ll have to try again on the other side, but this time I don’t think we’ll be able to use the topical.  His veins are weakened from his low platelet count, and they are just falling apart. An additional puncture could cause the vein to blow again.”

I remember when I was a kid I had to have an IV put in without anesthetic.  It was a pain that was like no other. Unlike giving blood, which is usually done in the crease of your arm where there are much fewer nerves, IVs usually go in your hand so that if they blow out, they can march up the arm and grab another part of the vein. If they started at the top of your arm and it blew out, there would not be any more good tries on that arm.

The nurse returned from the OmniCell with more equipment, and proceeded to try the other hand, this time with no topical anesthetic.

I remember thinking as I watched his face, “Crap, I guess the topical was doing something last time.”

“I think we got it… Almost there…  Nope, it blew again.” She said with disdain.

I looked down to see the two large bruises forming on each of his hands.

“We have a special team that I am going to call to put in his IV line that deals with special cases like this. I am going to call them down so that he doesn’t have to endure being poked all day.”

And within 15 minutes or so, a team showed up with an ultrasound machine with a probe head much smaller than I had been used to seeing during all of the pregnancies that we have been through. It was clear that the smaller head was for looking at veins and not babies.  I smiled for just a moment as I remembered the last time that an ultrasound had been used on Liam. He was just a baby bump on his mama.

I watched as this special team put in another IV. This time it was further up the arm. It blew.

The fourth attempt made it in, and it seemed to be holding for now. But by the time he had made it upstairs, and before a transfusion could be made along with platelets, that IV had blown too. It was clear that his veins were in pretty bad shape, but the paradox was that he couldn’t get very things that he needed for them to start healing without this line in place.

A fifth attempt was made. It held for a while, but it also blew. By this time, my wife was already there, and we were upstairs in our new room.  And I have to admit that even though I am not typically squeamish around needles, the drama and screaming from Liam had pushed me to the point of nausea. I couldn’t stay for this one.  I let Jenn take it on. It held for a few hours and into the evening, but failed as the ordered blood made it to the room. The hanging bag of blood sat there unused. It sat there too long. The first bag had to be thrown out.

The order was then placed for a central line to be installed.  Which, as it turns out, enters through the upper arm and enters one of the vein in the central [core] body– apparently close to the heart. The nurse said that the order had been placed for the morning because of the lateness of the hour, and yet a moment later, the PIC team showed up to put in the central line.  The nurse said, “The order said it was to be placed in the morning?” Which was a statement that had been inflected to act as a question.

The man puffed up his chest with the look of a hero.  He said,”Well, I’m here now. Let’s get this thing installed.”

A Mass. A Tumor. Cancer. Metastasis.

This entry is part 2 of 10 in the series "Liam's Battle" --

In the early hours of 17 Aug 2014, moments after I had the news in hand that there was an unwelcome mass in my son that had been the cause for all of his distress, the social worker came in. My immediate thought was, “Oh great! I get to endure the questioning that comes with all of the bruising and the black eye that my son has.”  I braced myself for the line of questioning that should accompany any child that shows up at a hospital with a black eye and bruising on his body, the apparent knowledge of the mass inside my son still rattling around the inside of my brain not really certain on which brain cell it should land.

I started to formulate a preemptive strike against the social worker.  I knew that I had no idea how the bruising got there; but how could I encase this in language that would allow her to believe that I had really not done it?

I started to speak, but then I decided not to.  Instead I stood there frozen while she came in and introduced herself.

“Do you have any questions for me?” She asked.

“Like what?” I asked, now on about my fifth iteration of my almost believable yet true story.

“Well, sometimes parents need to talk after a diagnosis of cancer is revealed, and we just want to make sure that you are OK.”  She said.

‘Cancer.’ This word started rattling around with the word ‘mass’. They really didn’t register quite yet. Cancer. The only childhood cancer that I could think of at this point was Leukemia. I didn’t know much about it, but it seemed that the odds of survival were pretty good for it. Of course, I had no data to support that claim, but I believed myself anyway.

“No, I think I’m OK.” I told her. I felt fairly relieved that I had not been asked to explain the bruising.

“OK, well, if you need to talk, just have the nurse page me.”

“Sure.”  I said having felt the victory of dodging the bruising question.

It wasn’t long after the social worker came in that I was meeting with the oncologist Dr. Tanya Watt. As I would come to find out later that day, she is the doctor at Children’s Medical Center that specializes in Nuerobalstoma.  I was assured that I was in the best hands possible.

“Right now, it is important to stabilize Liam. It’s likely that he has Neuroblastoma, based on where the cancer is in his body. It has likely spread to his bone marrow and started to compromise his blood production…” She said.

Wait, did she say ‘Spread’? I thought we were talking about a mass on the adrenal gland?  Wait! Spreading is bad!

The words: Tumor.  Mass.  Cancer.  Bone Marrow.  Spread. They all congealed in a place in my brain that controls the pit in the bottom of my stomach.  The fight or flight response now activated, I thought (and possibly said out loud), “Ohhhhh Crap.  Crap. Crap Crap Crap Crap Crap!” –Why had I been so foolish and not brought him in sooner? Had I really missed all of the signs leading us to this point?

The oncologist said,   “I want you to realize that you did the right thing by bringing him in. I realize that in the first twenty-four hours of a diagnoses like this, all the parent hears is ‘Cancer’, but I want you to realize that this is not your fault. You did not do anything to cause this.” She said with certainty. “I’ll be managing your case, so I’ll come talk to you once you are upstairs on the oncology floor.  Do you have any questions for me right now?”

“Yeah.” I remember stammering. “What type of cancer did you say this was likely to be?”

“Neuroblastoma. N-e-u-r….  Here let me write it down for you.”  She pulled a napkin from the counter and wrote it down. “I know that you are going to look this up, but try not to freak yourself out before we have a chance to talk,” she said. And with that, she left the ER patient room.

“We need to start an IV.” A nurse said entering the room.

“Sure.” I said. Picking up the phone to call Jenn. Synapses were starting to fire. I knew I needed my wife here with me.

Diagnosis

This entry is part 1 of 10 in the series "Liam's Battle" --

On Sunday 17 Aug 2014, Liam was diagnosed with Neuroblastoma.  This was after a month of fussiness that could not be explained.

Six weeks prior to this diagnoses, we had a normal, almost 2 year old.  When he started being fussy, we figured that he was sick, but nothing that a kid his age might be expected to get. He was fussy in the middle of the night, and this really caught us off-guard. He has always been such an easy going kid — He was sleeping through the night at 12 weeks. So what was going on?

We started noticing that their were bulges in his testicular sack. Hernia!  He must be  fussy because of an inguinal hernia. Knowing how painful that can be from my own personal experience, we got him to his PCP as soon as possible. She agreed that we should see a surgeon.  I immediately scheduled a consultation at Children’s Medical Center in Dallas, and we were seen in short order. Once seen, the doctor agreed, and we had him in for surgery the very next day.

Once inside to repair the hernia, the surgeon found no real evidence of an inguinal hernia. We were told that what we must have been seeing were large veins in the testicular sac. We accepted this, but really did not question from that point that the fussiness could be something else.

The three days following the operation, Liam was doing pretty well. He had had a local anesthetic which helped numb any pain from the surgery.  We thought we had whatever this was beat… Then the fussiness started again. After about a day of this, we called a nurse. A likely culprit: constipation.  So, we loaded the kid up with miralax for several days until the Poo ran clear. Still fussy.  A couple more days later, I called a nurse again. The thought  at that time was that that maybe he wasn’t eating, and that was causing his bowels to not be working correctly. Give more Tylenol and Motrin — ease his comfort from the surgery so that he can eat.

He seemed better while well stocked on the pain meds, but I kept thinking to myself, “This seems like a lot of pain medicine for just a hernia operation”

Around his birthday, he developed a black eye. Those bigger siblings of his must have been playing rough with him. So of course, a scolding was in order on the etiquette of being gentle with their baby brother.

As the next week rolled by. More bruising developed on his legs, and his color was turning pale. He looked normal to me, but he was turning paler by the day so slightly I wasn’t noticing.

The fussiness continued throughout the day and throughout the night.

It wasn’t until Liam had a chance to spend the night with relatives while Jenn and I went away for an evening to celebrate our anniversary that the grandparents had the chance to notice that something was off and the bruising didn’t seem right to them.  When they returned him to us, they voiced their concerns. I watched Liam as we fed the kids pizza that night. He ate a couple of bites and his belly became super distended. It was already looking pretty large. “Maybe there is something going on that is unrelated to the surgery,”  I thought.  Right then and there I decided that I would take him to the ER. Jenn had been working with him at this point and had dosed him again with pain meds. He fell asleep pretty soon after.

“Well, if he wakes up in the night I will take him then,” I thought. I want him to get his rest.

Sure enough at about 3:55AM, he stirred us awake. I loaded him up in the car and drove to Children’s Medical Center in downtown Dallas.  I had been thinking all night that he must have a blockage in his bowel. It was time for an X-ray. Let’s shine some light through this problem.

There was no one in line at the ER at Children’s at about 4:45 when I got there.  I was seen pretty fast. Imaging was occurring faster than at any other time I have ever been to the ER.

About an hour and a half later a doctor came in my room and announced that they had found a mass above the kidney.

In that moment, I felt relieved. There had been a reason that my calm and peaceful boy had been so fussy.

In the next moment, the name of the cancer that it was most likely to be (because of how it was presenting) was announced to me: neuroblastoma. I had never heard of it. Which is probably why I did not freak out like they were expecting me to.

So after they left the room, I googled it. This is what I found: Less than 50% survival rate when in the high risk category.

I called Jenn, “They found a mass on his right adrenal gland atop his kidney.  You need to get up here.”