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).