What is "normal"?

LONG UPDATE AHEAD

"Normal" for a cancer family becomes a very relative term. "Normal" can mean vomiting every day or not pooping for many of them.  "Normal" can mean rushing out for random food requests at 1:00 in the morning, and wiping sweat from a sleeping brow. And in"normal" even bad news can seem like good news.

This week in the hospital has been filled with many things that all the doctors and nurses are calling "normal", but it has been no where close to the normal that we want.  In fact, some of this week has been utterly traumatizing, if I'm being completely honest.

If you have not seen on our Instagram or Facebook pages, Hazel began to experience some pain in her back, near her tumor site, after only a day discovering her relapse.  By last Saturday, her pain was unbearable and unmanageable.  We were prescribed an oral pain medication, in hopes of handling it at home, but it came nowhere near touching it.  Sunday morning, after a completely sleepless night, Hazel vomited and was barely able to move.  The doctor requested that we bring her in for assessment in the ER.

While in the ER, Hazel has two doses of IV morphine, and still was in so much pain.  It was terrible to watch.  All I could do that would bring her any comfort, was hold her in my arms, trying to take the pressure off her back, and rock her side to side. Since her pain was clearly not able to be managed, we were admitted by that afternoon.

Hazel was put on a continuous drip of morphine, and it took us several days to find a dose high enough to make her comfortable, but even then, she was having breakthrough pain, and itchiness as a side-effect from the medication which just added to her discomfort.

One of the biggest concerns was that the pain was caused by the tumor growing, and possibly compressing her spinal column.  Monday, she was scheduled for an MRI, with sedation, to get a closer look at her spine, however, just as we were, most uncomfortably, getting in the wheel chair, I felt Hazel was burning up.  We took her temperature, and her fever was above 103℉.  For fear that this was caused by an infection in her port, the doctors did not feel safe pushing a bunch of sedation medication through her line, possibly spreading that infection to the rest of her body.  She had already not been eating all day in preparation for this scan, and being told that at 3:00pm it was all for nothing, was pretty devastating for a six year old girl.

But since her pain was still so great, they did not want to push the scan back, and asked if she would be willing to try it awake.  She has done many of her scans awake, so after some anxiety reducing conversation, she finally agreed to do it.  However, it wasn't until after 9:00pm that they finally could take us down, and the waiting wasn't good for her already high anxiety.

We got down to the machine, and the sounds and enclosed space made Hazel absolutely terrified.  They had noise cancelling headphones, and VR goggles to watch a movie, but nothing helped.  They put her in for only a minute, and she screamed in panic.  I asked if there was any way that I could sit on the bed with her, holding her hand, and the technician said the only way I could do it, was if I laid at her feet.  So, up I went, stomach down on the bed, arms above my head, holding her hands, and we went in that MRI machine together.  90 minutes together was better than 90 minutes alone.  I told her that I have always been by her side, and that we have been through so much together, that this would be no different.  We would get through it together like we've always done.  She cried a few more times, but held still enough that they only had to repeat one image capture!

Tuesday, we seemed to have a better handle on her pain, but she was already scheduled for her tumor and bone marrow biopsies, so it was going to be another day with a procedure.  Another day of anxiety. Another day of Hazel's "normal".  Since she was now on antibiotics for the possible infection, they felt safe to sedate her for this procedure.  It went smoothly, but she woke up in even more pain than before, I suspect because we aggravated the tumor and the needle pokes cause a lot of soreness, and became angry with what seemed to be the whole world.

She would say, "This is the worst thing to ever happen to me!  I hate this, I hate this!  I wish this never happened!  I just want to be a normal girl, and never have cancer.  I can't keep doing this!  Nothing is helping, and nothing is ever going to help!"

I can't even begin to describe to you the helplessness I felt in these moments.  All of the fears and frustrations I have had for her these last four years, and thankfully have been able to keep away from her, were all coming down on her at once.  The possibility of her cancer growing was becoming very real to her this last week, and there was nothing I could do or say to take that fear away, because I was fearing the same things.  All I could do was tell her that it was okay to get angry, to kick and scream, to cry and to be afraid, but that she should know we have the BEST Neuroblastoma doctor by our side, fighting with us, and we have a lot of different medicines that we could keep trying.  I promised her that I would never leave her side and that we would at least figure out how to manage this pain, it just was taking longer than we wanted it to. 

Most nights this week, Hazel has not even wanted me to sleep outside her own bed, and has been nervous when I step out, even just for a moment.  So even though I couldn't take her pain or fears away, I was glad that she felt comforted in my love and in my arms.

Wednesday, she had to have one more scan, to determine if her tumor is MIBG avid.  To Hazel, it didn't matter that it was important for us to do this so we could find the right treatment for her, it just mattered that she had to go through something else.  She was afraid of laying flat on her back, due to her pain, and didn't think she could hold still.  And if you know Hazel, once she gets stuck in a thought, it is not easy to get her out of it.  She spent almost the entirety of the two hour scan, crying, but to my amazement, still holding still enough to get all the images we needed.  We were both exhausted, frustrated and broken, but her resiliency and bravery kept me going.  I stroked her new hair growing in, and told her stories, sang her songs, and help distract and comfort her the best way that I could.

I was so nervous waiting for these results, but thankfully we got them sooner than later. The MRI showed no growth in the spine (thank GOD!), but the tumor seemed to be grabbing on the bone. However, this was not enough to be causing the kind of pain she was experiencing.  The MIBG/CT scan showed that, like her last relapse, her tumor is not MIBG avid, thus closing off one potential treatment, but that's okay, because it's not the only option.  The CT portion of the scan showed two things.  First, it showed that her tumor had not really grown that much in size since her scan on the 30th.  

This whole past week, I thought I was literally watching cancer eat my daughter alive as it was causing her so much agony.  So the fact that it hadn't grown, and hadn't spread felt like a ridiculously giant, cartoon, ACME anvil was lifted off my chest.  The pain, while awful, was not caused because the cancer was too quick for us, and it allowed me to feel safer taking some time to make decisions about her treatment moving forward.

Second, the CT showed something new. Hazel's lungs seemed to have a build up of fluid that was not present on any of the previous scans.  So, yet another CT, this time with contrast, was scheduled so we could get a better look.  It was supposed to be scheduled for Thursday, but in the middle of the night Wednesday, Hazel's oxygen levels began to drop, and we were sent down to an emergency CT at 2:00 in the morning.  The CT room that we were in was the  same room that she had her very first scan at Children's Hospital back in 2013.  And just like that first diagnosis time, Hazel has been in pain, having fevers, has been exhausted, been sweating like crazy (as you can see in the picture below) and was falling asleep during the scan.  Maybe it was the fact that I was sleep deprived, coupled with the fact that so many of her symptoms and our experiences were mirroring what we have already been through, but I felt like I was transported back in time and was living in the twilight zone.  My brain just did not know how to cope. My dreams, at this point, were feeling more real than the nightmare of a week we were living.

The CT showed that Hazel had a pleural effusion (fluid in the space around her lungs) in both lungs, but it wasn't clear what was causing it.  It could have been an infection, but the scan showed no signs of pneumonia.  It also could have been the tumor pushing into that space, making fluid leak easily.  OR it could have been that the tumor itself was growing into the lung space.  Something I don't think I shared, was that this tumor is located up between her diaphragm and lungs, wrapping itself around the aorta and growing roots that are attaching itself to everything it can.  It's grabbed onto one of her ribs and her spine, although not infiltrating them or any organs yet.  But it is in a precarious position and can cause a lot of issues if we aren't careful.  At this point, I'm calling her tumor a greedy little bugger.

Because the CT scan did not give us clear enough answers, and she spent much of Thursday needing an oxygen mask on her pillow, next to her face, she had to be sedated, once again, on Friday for another needle poke, in order to pull out the fluid to test it for infection and/or tumor cells.  While in there, the doctor decided to just remove the fluid from that lung he was testing, and ended up pulling about 200cc of fluid!  Thankfully, this seemed to help Hazel a lot because she no longer needed oxygen for the rest of Friday and all of the day Saturday.

During all of this time, Hazel's pain was still not managed, so the palliative care team was called in.  I know that "palliative care" is a scary term, but they are not only called in for patients needing end of life care but are called in for any and all patients in need of extra pain support.  My friend Melissa, another cancer mom on the floor, and who has been such an unbelievable support this last week, suggested that maybe they need a new name for when they are dealing with patients like Hazel, so as not to scare off the parents.  When I met with them, I felt for the first time that there was someone who was understanding Hazel's pain and knew exactly what needed to be done to help her.

The plan was set that Hazel was to go onto oral Methadone, instead of the continuous drip of pain medication she was receiving. Methadone is longer lasting, and does not usually have major breakthrough pain episodes, like she had been having this week.  Plus, since it was oral, if we could find the right dosing, we could eventually go home and continue treating her pain, instead of being stuck inpatient.  We slowly transitioned her over, and weaned her off the continuous IV med, leaving the pump on, if she needed to push the button for pain control. 

The first two days on Methadone were great!  I felt like we finally were ahead of her pain, and she was sleeping soundly at night, and feeling more like herself during the day, even getting up to play and be silly.  Plus, the soreness from the biopsy sites were finally fading, so I am sure that helped as well.  This is when Hazel's oncologist and I began to discuss treatment options for her tumor.  

When Hazel first relapsed, our goal was to get her on a new clinical trial, with a newly developed ALK inhibitor called Lorlatinib.  As you may remember, Hazel's cancer has a mutation of the ALK gene, which is thought to drive cancer growth.  These inhibitors target that mutation, and treat the tumor with much less toxicity than chemo and radiation.  It was the ALK inhibitor, Crizotininb, paired with two different types of chemo that finally got Hazel into remission during her last relapse.  We discontinued the chemo in July, and kept her on the inhibitor (which is a daily pill), in hopes that we could keep the cancer at bay, and give Hazel's body a chance to strengthen and be off of chemo.  However, it was on this medication that he tumor grew. 

This new ALK inhibitor, Lorlatinib, is much more promising thatn the Crizotinib, and was performing better on its own than the Crizotinib did when paired with chemo.  One article I read said that the lab results were showing "unparalleled responses" and causing mice to go into "rapid and sustained regression."  So we have a lot of hope for this trial.  However, since her rapid deterioration in just five days post her initial scan on the 30th, we were not sure that we were going to be able to make it to the start date for the trail, which is Tuesday Novemeber 14th, because it was just giving her tumor more time to wreak havoc. If we found that we did not feel it was safe to wait, we were going to push the trial back, and start high dose chemo immediately.

However, by Saturday, Hazel seemed much improved, and we both felt comfortable holding the chemo off, unless anything changed.  Dr. Marachelian also discovered what she thought was finally the answer to what was causing Hazel's pain.  She read about a phenomenon that happens with patients who's tumors only partially respond to the ALK inhibitor, Crizotinib.  It was like her tumor was a car that was driving down hill, and the inhibitor was the brakes, keeping it from growing/driving out of control.  Once the Crizotinib was discontinued on Halloween, something called a "tumor flare" occured, and it was like the brakes were lifted and the car shot down the hill at break-neck speed.

These "tumor flares" cause a lot of problems, but problems that are usually temporary. So the hope is that the fluid in her lungs will begin to disappear as the flare settles down, and that her pain will subside and she can be taken off the pain meds.  So please pray that this is the case!

(I hope you are still hanging in there, I know this update is long!  It speaks to the eternity that this week has felt like for me!)

Saturday evening, I was feeling pretty good.  Hazel's pain was controlled, I was coming off a good night's sleep from Friday night, and it looked like we could start the trial on Tuesday, and begin talking about going home!  At 9:00pm, Hazel's nurse came into the room to give her the next dose of Methadone, and I came over to her bed to wake her up, only to find that it was extremely difficult to do so.  She could open her eyes for a moment, maybe answer a question or two, and then her eyes would cross or roll back into her head, and she would be out again.  At first, I found it funny, but after a few minutes, my heart began pounding, as she was getting harder and harder to wake up.  I was shaking her, patting her face, tickling her, putting ice packs on her, and nothing was working.  I felt like I was facing the moment that we have spent the last four years fighting to avoid.

Then, the worst thing happened.  Her heart rate plummeted, and her respirations slowed so much so, that many times she even stopped breathing.  We had to rush everyone in the room, put her on oxygen and administer an emergency dose of Narcan (what they give to patients when they overdose on drugs). Narcan blocks the pain receptors in your brain, thus reversing the affect of Methadone in her system.  The team believed that the Methadone, since it is long lasting and can build up, was over sedating Hazel to the point of respiratory distress.  The Narcan did its job and woke Hazel, but she woke in a very traumatizing way.  She was screaming in pain, mostly in her head, and her heart rate skyrocketed.  She immediately needed to use the bathroom, and then began to uncontrollably shiver.  Eventually she drifted back to sleep in my arms, and continued to breathe safely the rest of the night.  Needless to say, I did not sleep very much.

My body was a mess that night, and I felt so broken.  I hadn't really had a break down all week, being in fight mode, but there was no avoiding this one.  I just fell apart.  I begged God to never let Hazel, or myself experience that again.  

They had decided to hold the Methadone dose overnight, and then restart it at a lower dose Sunday morning.  She had a wonderful Sunday.  The best I had seen her all week.  She was finally eating, albeit still very little, was laughing, dancing and playing, and said that she had zero pain.  I thought we were finally coming to the other side of the very dark tunnel we had been in.  

But then, around 5:00pm, I couldn't wake her again, and she spiraled down into the same symptoms she had the night before, not breathing, and needing a dose of the terror inducing Narcan.  And it didn't only happen once, but it happened twice.  The thing I had prayed to never experience again had now happened three times.  We both felt so overwhelmed and afraid.  Hazel didn't want me to leave her side, and asked me to sleep in her bed and hold her hand. I stayed up, constantly watching the monitor, hoping I would not see the trend begin again.  In the middle of the night, the decision was made that all Methadone would be discontinued, and she would use the pain medicine pump connected to her line until we figured out what to do for her pain.

Today however, her pain has not returned, and she has not needed to push the button once!  So our hope is that the "tumor flare" is no longer flaring, and has made the pain subside.  Either that, or there is still Methadone in her system.  We are going to watch her closely over the next few days, and if her pain returns, we will try something else.  Never again will I give her Methadone, that's for sure.

Despite having a very good, cheerful and pain free day, she spent most of it asking all of us if she would ever need "that medicine that made her head hurt" again, watched her own monitor closely and worry if she saw any sign of a dip (she is sometimes too smart for her own good), and afraid to go to sleep because "she felt like she would die if she stopped breathing again."  How do you comfort a six year old child, afraid she is going to die in her sleep, when you are fearing the same thing?  How do you help them find the rest they so desperately need?  I feel like her: It's not fair!  I wish this never happened!  She doesn't deserve this!

This should not be her "normal".  Celebrating little tumor growth should not be "normal". Being glad that she is breathing after respiratory distress from a medication meant to help her should not be "normal".  Being happy to see our hospital family because we are inpatient should not be "normal".  The fact that we survived enough days to make it to a clinical trial tomorrow, that we are hopeful about but do not know if it will work, is good news, is not "normal". Having childhood cancer should not be "normal".

As I am sure you can tell, I have really struggled through this week.  I have felt angry, terrified, bitter, exhausted, worn down, defeated and so so sad.  But I have tried to, everyday, hang onto any glimmer of hope I have for Hazel.  Hope in this new clinical trial.  Hope that her biopsy results will come back with information that will be helpful to us in her treatment.  Hopeful that God has her in the palm of His hands, and loves me despite my bitterness.  When my children are angry at me, do I turn away and abandon them? No!  I do everything I can to reconcile and to show them how much I love them.  My FATHER is no different.  Please pray that I can remember that, that I can feel that, and that I can hold onto that.