In the Afternoon

A Little About Her Treatment Plan

{This was written by my dad, James}

2014-02-21

It has been a long nine days since Nicole was re-admitted to PCMC on February 12. Sometimes she is content or even happy, and other times she is angry or hurting. There is nothing surprising about this though - not much different than the first time we went through this.

Also, today the nurses have started giving her oxycodone on a regular schedule to help manage pain; the doctors believe she is starting to suffer from mucositis; hopefully it will not get as bad as the first time she was here. She is still eating and drinking a little, but she's teetering on the brink of having to get a feeding tube to maintain her body's nutritional needs. They have already been giving her IV fluid support for days. Again, nothing surprising here.

Nicole's Absolute Neutrophil Count (ANC - a measure of the body's immune system strength) is now plummeting as expected, and will undoubtedly hit zero within the next few days. She is still strong, but her heart rate has been elevated for a couple days. They took a blood sample today to run cultures to see if she's coming down with an infection. It'd sure be nice to at least get through the first block of chemotherapy without at trip down to the Pediatric Intensive Care Unit (PICU), but I guess we'll just have to wait and see.

As I mentioned last time, the bump on Nicole's right temple is an ALL mass; this is known as an "extramedullary" relapse because it is isolated to a specific area in the tissues of the body, rather than in the bone marrow. Additional testing indicates that it has the MML gene re-arrangement as well, which provides additional confirmation that this Leukemia is a leftover from her original bi-lineal ALL/AML diagnosis. What appears to have happened is that the Leukemic cells infiltrated the muscle tissue in Nicole's temple. The combination of her previous chemotherapy, radiation therapy (including 3 rounds of cranial-focused radiation treatments), and her new bone marrow only managed to suppress the Leukemic cells in this area, causing them to go dormant for a long time, but was not potent enough to eradicate them.

If the Leukemic mass had somehow gone undetected/untreated, the ALL would have spread to Nicole's bone marrow within the next several weeks/months, and would, of course, eventually be fatal. Because Leukemia is a cancer of the blood, rather than cancer of a specific organ, or a cancerous tumor, it cannot simply be surgically removed, even in this peculiar "extramedullary" instance.

The plan is still to do three blocks of chemotherapy, with the goal of putting the ALL into a deep remission. Medically speaking, Leukemia is considered to be in remission if fewer than 5% of a sample of white blood cells are Leukemic, but in order to have a good chance of a successful bone marrow transplant, the doctors really want to see 0.1% or less Leukemic cells, and in the most ideal case, there would be no detectable cancer whatsoever in the blood and marrow. Even in this best case, where the Leukemia is below detectable levels, it will eventually return... Chemotherapy alone cannot save Nicole's life; she will require another transplant in order to be "cured".

The doctors will not use the previous donor again, because it is unlikely to a produce a different end result; Nicole's body needs to accept the donor's bone marrow, but she also needs to experience more graft-versus-host disease than she did with the original BMT. There are several known donor matches for Nicole in the registry.

There is also a possibility (due to the relative success of the first BMT) that the doctors could do a different type of BMT, using one of Nicole's parents as the donor. Some testing would need to be done on both Cathy and I, and on Nicole, in order to see if we meet the right criteria for this method. This option is not a 'normal' BMT, and at this point I don't understand much of the technicalities of this procedure, but there must be some sort of specific mismatch between the parent donor and the child recipient in order for this to have a chance of success. The doctors are leaving both options open at this point.

Also, just prior to BMT, Nicole will receive radiation treatment that is localized to the site of the relapse on her temple, but she will not receive the full-body and full-cranial radiation treatments she received the first time. She will still receive the high-dose chemotherapy, however.

Overall ALL relapse survival rates range anywhere from 20% to 50%, depending on how early the relapse occurs from the time of the original transplant, and whether the relapse is an extramedullary relapse or a marrow relapse. The fact that Nicole's relapse is about 20 months her original BMT, and the fact that the site of the relapse is isolated, rather than in the bone marrow, are both positive factors for Nicole's prognosis and the doctors remain optimistc that they can get the ALL into remission.

If the chemotherapy does fail to achieve a good remission of the ALL, then there is nothing more that can be done medically for her. If the chemotherapy is effective, then they will proceed with a second transplant, which is successful for about 60% of patients who get to that point. If the second BMT fails, then there is also nothing more that can be done medically for Nicole. Ultimately, however, I know that Heavenly Father loves Nicole even more than I do, and I know He is watching over her. I know that if it be so, our God whom we serve is able to deliver Nicole from the burning fiery furnace, and He will deliver her. But if not, be it known that we will still serve God and accept His will in all things (see Daniel 3:17-18).

Getting started

{Written by my Mom, Cathy}

I don't know even know where to begin.  James did a great job at explaining our journey; but, I don't know how to explain how things are going.  We kept Nicole private during her first journey with her cancer.  I am not sure how well I will be at sharing her relapse.  So much of our experience isn't secret it's sacred to us. 

Nicole first 8 days of treatment have been uneventful.  She mostly just watches the Little Mermaid and on occasion another Disney movie.  She loves to play with puzzles.

She loves to drive the little cars they have for the children to play with.  We walk up down the halls driving this little cars. She loves to wave and say hi as we drive by the nurses.  She also got to drive the car down to the RTU (Rapid Treatment Unit).  That is where she gets an LP (it's where they go into her spine to give her Chemo in the spine).   With an LP they need to sedate her so she will lay still for them to do this. 

Nicole cousin Erin gave her a little Kermit frog that is always with her.  Kermit also needs to wear a mask if he leaves the to go for a ride in the car or play in the play room.

Eating as become a struggle for Nicole again.  The chemo isn't helping.  The Drs and the nurses keep telling me that with steroids it should increase her appetite.  For Nicole she has had such struggle with eating since her last go around with cancer.  Food has become a power struggle with her or something she can control.  However Kermit tries almost everything before she does.  I guess you call Kermit Nicole food taster:)

As hard as it is to back in ISC (or the cancer unit) at primaries.  We have loved visiting with the nurses and staff there.  They are like family to us.  I know that they love Nicole and their hearts are as broken as ours that she has relapsed.  Last night while in the playroom her life flight nurse was in the unit and stopped at played and visited with her.  They see so many patience but Nicole in her short life as touched so many people. As we went to the RTU today for her LP the nurses there all had tears in their eyes as they came to say hi to their little Nicole who isn't so little anymore.  I know that the Lord watches over us.  I know that the nurses at Primary Children have a special gift.  One of my favorite scriptures Matthew 10:42 "And whosoever shall give to drink unto one of these little ones a cup of cold water only in the name of a disciple, verily I say unto you, he shall in no wise lose his reward." The nurses do more then give a drink of cold water to us.  They are our listening ears, they laugh and cry with us.  The encourage and love us. 

Here in Hyrum our ward family has also been a strength to us.  Here we go again with cancer and they are right there building and strengthening our family.  When David went to fight his Goliath, he also had an army behind him ready to fight.  Our ward family is part of that army.

We also have dear friends who we know are supporting us and sending many prayers for our Nicole and for us.  They also want to help us in anyway that they can.  Both James and I have been blessed with good friends throughout our lives.

Then there is family.  There are no words to express the love and support that family is.  My sisters always say that it takes all 5 of us to do what our mom did.  I often think how proud mom would be of us at they way we watch out and support one another.  I know that I just have to say a word and someone from our family would be there and do what we need help for.  James' family is also doing what they can.  We feel the strength from their prayers.  We know that our parents are on the other side doing all they can for Nicole.  It's nice to know that Nicole has her grandparents in the room when we can't be there for her procedures. 

 My children are standing tall and going to work.  They know what is needed at they are willing to give and sacrifice for their little sister.  It's amazing to watch family in action.

I'm trying to following sister Hinckley's counsel  “The only way to get through life is to laugh your way through it. You either have to laugh or cry. I prefer to laugh. Crying gives me a headache.” (Ensign October 2003)  This is heartbreaking news to our family and so many others around us.  But I know that the Lord is in the details of our lives.    He loves little Nicole.  John 14:18 "I will not leave you comfortless: I will come to you". Just like Mormon wrote to his son Moroni is true for each one of us. Moroni 8:2

"My beloved son, Moroni, I rejoice exceedingly that your Lord Jesus Christ hath been mindful of you..." I know that the Lord is mindful of our family and also for the army who is supporting us. 

Thank you!!

Relapse

{This was written by my dad, James}

Nicole was originally life-flighted from Logan Regional Hospital to Primary Children's Medical Center (PCMC) in Salt Lake and diagnosed with two forms of Leukemia in December 2011 when she was 4 months old. She had both ALL (Acute Lymphoblastic Leukemia) and AML (Acute Myeloid Leukemia), which is very rare. To further complicate matters, both the ALL and the AML had MLL (Myeloid/Lymphoid Leukemia) gene re-arrangements associated with them - in other words, even the Leukemia cells were not 'normal'.

ALL and AML are treated with different kinds of chemotherapy which cannot be easily mixed, and due to its rarity, a standard treatment protocol for her diagnosis does not exist. The MLL gene re-arrangements added an additional layer of complexity to treatment as well.

Due to her diagnosis and her very young age, Nicole's original prognosis was extremely poor, with perhaps a 5% survival rate (though hard statistics are unavailable for her diagnosis) - she was not expected to live. In fact, when she first arrived at PCMC, the doctors weren't even sure they could stabilize her long enough to begin administering chemotherapy. Even after she survived the initial trauma, privately, she was known as 'the scary baby' by the PCMC medical staff, because she was in such bad shape and her diagnosis was so bleak. By the end of her long journey, the staff referred to her as 'the girl with nine lives'.

I cannot adequately describe what she suffered and endured in detail - it was awful, even by 'normal' Leukemia treatment standards; I will simply say that she was hospitalized almost continuously for 10 months, with roughly one third of that time in ICU (the time in ICU was spread over numerous different incidents). I am aware of at least five occasions when her suffering was to the point of death. She is alive today due to the mercy and power of God, and the most incredible thing besides her survival is how well she endured it all.

Captured Moments Photography

Nicole came home in October 2012 at 14 months of age, following a successful Bone Marrow Transplant (BMT) in May of 2012. She had been confined to a crib for 10 months and was very weak - she couldn't even roll over at that time. For several months following, we had to administer 34 doses of 18 different types of medication around-the-clock, and she was also on a feeding tube (and had been during her entire hospital stay). Due to the side-effects of chemotherapy and radiation treatment, she had suffered severe cases of Mucositis (which is like terrible, painful mouth sores, but it affects the entire digestive track, not just the mouth), which resulted in a severe oral aversion - the only thing she wanted in her mouth was her pacifier, and everyone was amazed that

she would suck on it, even during the worst stages of Mucositis. We also had regular check-ups at PCMC, weekly at first, then less frequently as more and more time passed.

Bitty Bokeh Photography

By early summer of 2013, her medications had all been tapered-off and eliminated one-by-one, and she had finally started eating and we were able to get rid of the feeding tube. She was a year post-transplant, and her bone marrow and immune system were getting stronger and stronger. She was doing beautifully, and we started going to check-ups only once every three months. Though still a little behind other children her age, she was rapidly gaining on her developmental skills. The PCMC staff was amazed with how well she was doing. By the end of 2013, it was beginning to look like we were home-free. (Relatively speaking, of course. The potential long-term side effects of Nicole's chemotherapy and radiation treatments are numerous, and will always be a specter hovering in the back of our minds.)

However, about a month ago we noticed a ‘goose egg’ on Nicole’s right temple. At first we assumed she must’ve fallen or bumped into something, even though no one had witnessed it.

You can see the bump in this photo.

A couple weeks passed, and it didn’t seem to be improving/changing, maybe even getting a little bigger, so we took her to the local InstaCare in Logan (hoping they might verify that is was just a simple trauma bruise – though we were starting to have our doubts). Due to her past history, the doctor contacted Nicole's oncology doctor at PCMC, and then we went in to see that doctor, and to have some lab testing done. Her blood work looked good (and she hadn’t been showing any other symptoms of a relapse), but after some consultation, the oncology team at PCMC decided to have an MRI done on the mass in order to see if it would help them identify what it is / what is causing it. due to her age, Nicole had to be sedated for the MRI which occurred on February

5, 2014 at PCMC.

The conclusion from the MRI scan was that it definitely wasn't a trauma bruise (which we were already assuming at this point). The tissue was clearly inflamed, but they still were not certain of the cause; it is very, very unusual for Leukemia to manifest itself in this way (as a localized Leukemic mass, with no signs in the blood), but it is not completely unheard of. They scheduled a surgery for a neurosurgeon to do a biopsy of the 'mass' on Monday, February 10, and on February 11, the result came back that is was an ALL mass.

On Wednesday, February 12, Nicole went to PCMC to be admitted. As with the biopsy on the 10th, she had to go to the hospital fasting. They did a heart echo, then an EKG, then a bone marrow aspiration, then a lumbar puncture (spinal tap) which included a chemotherapy injection, and then surgery to place a central line in her chest (used to administer chemotherapy into the bloodstream. There was a minor complication with the surgery, and she had to have some follow-up x-rays taken to make sure no damage had been done to her right lung. She wasn't able to eat or drink anything until about 11 PM. Through all of this, she was very calm and uncomplaining - a miracle in and of itself, but she was like this throughout her first bout with Leukemia - things had to get really miserable before she would express it.

They started chemotherapy late the following night, February 13. Nicole remained in excellent spirits throughout the day on Friday, February 14. She seems very much at home, and has not been shy with the hospital staff, but by Friday evening, the effects of the chemotherapy were beginning to show, and it gets much worse before it gets better.

This particular chemotherapy protocol that is being administered originates from the U.K., and has been very successful at treating patients with an ALL relapse. One of the doctors that the PCMC staff consulted with during Nicole's original battle will ALL and AML due to her unusual case and treatment has recently transferred from New York to PCMC, and she has been put in charge of Nicole's case; she is a recognized expert in the field of childhood cancer.

Based on the results from the bone marrow aspiration, the lumbar puncture, and blood tests, Nicole's Leukemia relapse is totally confined to the mass on her temple, and it is only ALL (not AML and ALL like last time). Her spinal fluid, blood, and bone marrow are currently all still perfect, and her body is otherwise very healthy. This ^{is still a heart-wrenching diagnosis, but given what she faced previously, the doctors are very optimistic (not like} last time) about her treatment and recovery. Obviously, it is still cancer, and still potentially fatal, but we have had a peaceful feeling that she will survive, and this diagnosis only reinforces our hope. There are many things working in Nicole's favor this time compared to last time, and we are hopeful that the outcome will be a permanent remission of her Leukemia.

The current plan (and everything is always subject to change when it comes to cancer treatment in general, and Nicole in particular) is to do three blocks of chemotherapy, and then go to another Bone Marrow Transplant (BMT). Each block will be roughly one month in duration, with roughly a week at home between blocks, depending on her recovery each time. This is similar to the last time, but Nicole’s body was weaker and smaller, and her Absolute Neutrophil Count (ANC - a measure of the body's immune system) didn’t recover well; she didn’t get to go home at all between the third round of chemo and the BMT, and there were many other complications as well, which we will hopefully avoid this time. Also, the chemotherapy protocol is not quite as intense as last time, and she shouldn't suffer Mucositis as a side effect of these drugs. The BMT will also be similar to last time, with a battery of radiation treatments and some truly awful, high-dose chemotherapy at the beginning, immediately prior to the transplant, and then a few months of hospitalization following the transplant. Of course, everything is subject to change as time passes.

Nicole's current diagnosis is actually good only in comparison to her original diagnosis - in all reality, this is devastating news, and for a ‘normal’ case Leukemia relapse, the chances of survival become significantly lower. In Nicole’s particular case, however, they are significantly higher (since when you start at roughly 0%, it only gets better from there). That said, although the specifics of her treatment will be different from the last time, overall, it will likely be much the same as her first go-around, but hopefully without many of the particular life- threatening difficulties she previously experienced. I know what Nicole is going to go through again, and it makes me weep. I hoped this would not happen, but it has, and now she must endure again, and we must be witnesses to her suffering.

I know that God is a God of Truth and cannot lie; I know that He always fulfills His word. By the voice of His Holy Spirit, and by the voice of His servants, I know that God will strengthen Nicole and she will triumph over Leukemia again. When she was in the hospital the first time, we discovered that 'Nicole' means 'victory of the people' or 'victorious people'; by the will, the power, and the mercy of God, may it be so, in the name of our Savior, Jesus Christ, amen.