A few pictures from the day. Liam had a pretty good day.
The day my son was released from the hospital after his first round of chemo (a 5 day round that with complications led to 22 days in hospital), I came home to find my wife frantically working on Excel with the hustle and bustle of a full house exploding around her. There was a lot going on, but it was clear that what she was doing was important. The kids, who had not seen her in a long time, were siphoned off into another room every time they would show up to hang out with her.
What was she doing that was so pressing? She was making a matrix of all of the medications that needed to be administered to Liam.
I found this odd. How many medications had been sent home with him? Quite a few. When it was broken down on how many doses he is getting a day there are thirty-one. Thirty-one different things to keep track of. Twenty-nine syringe cleanings. Thirty-one administrations by mouth with differing volume amounts.
As I watched Jenn frantically come to terms with the reality of this situation, i.e. that there were no nurses to administer and keep track of all of the medications that Liam needs, I had the stark revelation that many parents out there must not be able to keep all of this straight. All that the discharge paperwork said was which medications he was on, the doses per day, and when the last dose was given.
This seems like a poor system. We have figured it out, but perhaps there is a better way than, “Here you go. Good luck!”
Our experience at Children’s Medical Center has been very positive. The medical professionals (doctors, nurses, technicians, social worker, child life specialist, music therapists, physical therapists, kitchen staff, janitorial staff, volunteers, etc., etc.) have all been wonderful. They all created a very safe bubble for Jenn and Liam; however, I think that the transition from the hospital to home has been a little more like the bubble popping than staying in the bubble and floating home on the breeze.
It is good for the two of them to be back home. It has been a wonderful time for me to regain some of the sanity that I had lost, and I enjoy interacting with Liam all of the chances I can get. When he is home, I get more time with him.
The bubble with all of its iridescent protection returns next Wednesday 21Sept2014 when chemo round 2 starts.
Liam was allowed to come home for a week before he has to go back in to the hospital for round 2 chemo. Exciting, yes; however, I am forced to look at my home in a different manner. I have never had to worry about a weakened immune system before.
A typical motto in our house has been, “A few germs will build your immune system.” — You people out there with small kids know what I mean.
A few germs, however, can be deadly to a little guy that has an impaired immune system. It is true that after this first round of chemo, his white blood cells are coming back. But after every successive round of chemo, his immune system will get hammered to the point that it will be close to death (if not dead). So much so, that after the next round of chemo (round 2), they will harvest stem cells from his bone marrow so that they can use them later to reboot his immune system.
The process is pretty neat. They will actually administer a drug (Granulocyte colony-stimulating factor G-CSF) that stimulates the bone marrow to push stem cells out into the peripheral blood.
The collection is performed through a process known as “apheresis.” Blood is drawn and passed into a machine that spins the blood to remove certain stem cells (those identified as “CD34+”), and then the blood is returned to the child. 
After the cells have been collected, they will be used during the consolidation phase to give a boost or jump start to the bone marrow. The autologous (meaning he is his own donor) stem cell transplant will occur under a very strict, positive pressure, wing on the oncology floor.
Having the ability for forward vision, however, only makes me more concerned about what I can do today. While Liam’s immune system is not completely compromised yet, the house as well as the lifestyle of three siblings and a dog have me a bit on edge. What needs to change so that Liam is protected? How do we make it through this time in our life when it seems impossible to keep out external germs while he is at home?
So, the immediate family and myself have spent the day attempting to prepare for Liam’s return to home, albeit for a short while. We have vacuumed, mopped the floor, washed all of the sheets, removed all that can potentially have germs. Still, my vision leads me to despair. I know how tiny these bacteria are. I know how my kids will interact with one another.
This problem is beyond me. So, I must keep the cub scout motto in my mind, “Do your best!” It is all I can do, nothing more.
[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. 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 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.
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%) + 606 keV
(Gamma Decay ~10%) + 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.
- 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…. ” ↩
Liam is finally starting to act like himself. I can use a couple more days like this! He was playing and carrying on conversations with us for most of the day. While he was still in a lot of pain when he was moved (understandably), when he was being still, this is what he looked like. He hasn’t acted this coherent in weeks!
The pleural effusion appears to have been causing a considerable amount of pain. I was told by the nursing staff that the teenagers that require a chest tube find it fairly uncomfortable. Since Liam is only two, it is difficult for him to describe his discomfort to us; however, if this is how he is acting with a chest tube in, I am guessing that the pleural effusion must have been very discomforting.
A few more good days like this will be real progress. We need his strength to be up for Round 2 Chemo starting on Sept 10, 2014.
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:
- Find the source of the fluid
- Install a drain tube into the pleural space
- 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.
(I have moved this item to the *top menu* so that it will be easy to find. I will continue to update it as we build our network of helpers/ need categories.)
I know that many of you have asked how you can help out our family in this very overloaded and confusing time for us. (In many things we are not totally sure how we can be helped yet!)
I did want to pass along contact information fop those wanting to help with food.
- Food / Meal Coordination for Harris Household (To avoid duplication, or multiple meals arriving at the same time)
- Contact: Eyde Buxbaum – firstname.lastname@example.org
- Anyone can participate- Eyde has the details (and she is extremely nice to work with)
[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:
- I knew it was cancer.
- 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.
“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.”
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.
Today has been a much better day than yesterday. Even though we have been moved into the ICU, he has calmed down significantly. Thanks to some wonderful medication, he was finally able to stool and help relieve severe belly distension. Afterwards, he was able to relax and sleep. He hasn’t slept restfully in days. So, this is wonderful news.
Tonight he will start his 3rd day of chemo (out of 5) for this round. So far, so good; however, there is a long row to hoe.
Immediate prayer requests:
1. Continue to stabilize
2. That the need for supplemental Oxygen will decrease.
3. That the need for pain medication will decrease.
4. Healing for his belly will increase, esp. after such severe distension
5. That the chemo will continue to kill the NB