Dude, You're a Bummer Man

Seriously.  Can't I ever come to the table with any good news.  Always doom and gloom.  Well, get ready for it.

So, remember how I was saying that things have not progressing as well as they should?  Still on that trend.  I am currently still on Tasigna 800mg, but it doesn't seem to be having the desired effect on my cancer.  The skin on my face is turning white...or maybe brown...I can't tell if the discoloration is too much pigment or not enough, it looks like my sunscreen application went seriously amiss...but the cancer reading (my BCR-ABL pcr) is still at over 8% as of the end of May.  So I know that 8% sounds like I am doing better, like things are moving in the right direction, since I was at 14% at the beginning of the year, but the truth of the matter is that I need it to go down below .1%.  This is the all important milestone that I am trying to reach - if/when I get there, it will mean that the cancer has very little chance of progressing.  (Progressing?  Huh?  What does that mean?  Well, if the cancer is not well controlled by the medication that I am on, there is a risk that it will progress into a more serious stage called Blast Crisis - which is very hard to treat, almost always requires full chemotherapy, and will almost always require a bone marrow or stem cell transplant - if the patient makes it that long.) So 8% - while it is less than 14% - is still miles away from where I need to be, especially since I've been on Tasigna for more than 7 months.

The one bright shining light throughout this has been that I haven't had to have a red blood cell transfusion since April 8th, 2016.  My reds have been falling at a much slower pace, while the other counts (platelets, WBC) have stayed MY regular standard sucky, but somewhat stabilized.  A transfusion MAY be in my future, however, as even though they have not been falling quite as fast, my counts have still been falling and I can feel that I'm getting low.

The PA that I have been shuffled off to in my new cancer clinic (oh yeah, that's right, they have handed me off to a PA to treat my refractory CML, that sounds like a brilliant decision, right?) has actually made a bit of a brilliant move, and sent in a referral for me to see a Dr. at UCLA.  I've walked this road before.  My original oncologist tried to get me in to see someone at UCLA, and the insurance company denied the request.  Seems like second time was the charm, as I had UCLA calling me to schedule an appointment, before I'd even received the approval of the request in the mail.  And, another shocker! they've already fit me into their schedule and I had my first office consultation.

Let me tell you how that went.  UCLA thinks that I might need a bone marrow transplant.  To which I replied, "Hmm.  No thanks."  Actually, what the Dr. I saw said was that I have had a suboptimal response to all of the medications that I have tried, thus far, and if one is not able to control the cancer level, and/or if my bone marrow does not start producing healthy cells, I MAY need a bone marrow transplant.  She has suggested that my Dr.(PA) change my medication to Ponatinib (if you don't know about it, don't look it up, the warnings are scary AF), and that she would present my case to the transplant board for approval IN CASE the Ponatinib does not work, and I do, ultimately, need a bone marrow transplant.  They are going to start the approval process with my insurance company, and get testing kits out to my sister and I for HLA typing.  The smarties at UCLA are loading me up in the chute.  But I'm digging in my heels.

IF Ponatinib doesn't do it for me, there is still one more currently approved medication to treat CML called Bosutinib.  The Dr. at UCLA said that she doesn't like to prescribe it, as it has a tendency to cause "acute intestinal distress", but personally, if it becomes my last option before a BMT, I would much rather take my chances with some diarrhea than chemotherapy, radiation, possible transplant complications (like death), severe Graft vs. Host Disease, and on top of that, probably some acute intestinal distress caused by trying to - oh I don't know - KILL OFF all of my blood cells and replace them with new ones, that HOPEFULLY my body will not reject.  That sounds like something that could cause some intestinal distress.  Let's try to avoid that if at all possible.

So here's my plan: Ponatinib first, and hopefully that's the end of the story.  Luckily, statistics that I've looked up regarding Ponatinib suggest that it is very good at treating refractory CML.  (Hey, that's what I have!)  Some studies have even suggested that as many as 65% of people with treatment resistant CML have benefited from Ponatinib.  So, I'm going to go ahead be encouraged by those statistics, while cautiously preparing for a less than stellar outcome, and in the meantime try not to be such a bummer, man.

Happenings

"God grant me the serenity to accept the things I cannot change,
The courage to change the things I can,
And wisdom to know the difference."

I'm not really a very religious person, but this has been my mantra for the past few months.  (I'm not a recovering alcoholic, either.)  Things have been going...not exactly poorly, but not nearly as well as one could have hoped.  When I was first diagnosed with CML, I was told that I was lucky because I got the "good cancer".  There is no such thing.  Cancer sucks.  It's a life sucking, soul sucking kind of suck, especially when you have the "good cancer". Having the "good cancer" essentially means that you are supposed to pretend that you don't have cancer, and go about your daily life.  It's harder than one might think.

Ignoring my cancer has been made more difficult by the fact that I've had Dr appointments every two weeks for nearly the past year.  My blood counts continue to be very low - and I've consistently had to have red blood cell transfusions approximately every six weeks.  Sprycel stopped working for me in November of 2015, as my PCR (BCR-ABL) went from 8% to 18%, and I was switched to a new drug called Tasigna.  I'm on the highest dosage recommended (800mg daily), and my Dr. had taken to calling my cancer "refractory".  (I looked it up.  It means: leukemia that does not respond to treatment.  Lovely, thanks for that.)  Speaking of Dr's, my medical group decided to "outsource" their oncology department, and I lost my Dr at the end of February.  Luckily, his replacement turned out to be the Dr. that I saw for my second opinion, so he at least had a cursory understanding of my case.  At our first appointment, he was a little shocked at how low my (blood count) numbers were, but with my platelets coming in at 47, I assured him that they were actually on the rise, as I had been hovering in the 30s for months now.

Besides my new oncologist, I also saw a "specialist" at Loma Linda in January of this year.  It took an unbelievably long time to get an appointment, and for the first 15 minutes, he thought that I had AML due to a typo on his summary sheet.  When he found out that I actually have CML - something that he would have known if he had, in fact, looked at any of my medical records that I had painstakingly collected and had sent over - he briefly reviewed my history, suggested that I move to a lower elevation, try not to have so many blood transfusions, get better health insurance, and have a bone marrow biopsy, then sent me on my way.  Three months of waiting, for that?  Really?  He must not have got the memo about the "refractory" nature of my cancer.

However, my oncologist did take his advice, and scheduled me to have a bone marrow biopsy.  I felt like I needed one, but by the time the day came, I was pretty much petrified.  Some hospitals/Dr's will put you under anesthesia for the procedure, but the practice that I am affiliated with just uses a local - lidocane.  If anyone is wondering, they lay you on your stomach, and numb you up with a couple of shots of lidocane.  The needle is pushed into your illiac crest (which is low on your back, almost down to your behind, either to the right or left.)  It takes quite a bit of pressure to get it through the bone.  They then take a bone marrow aspiration, which means that they suck some of the fluid from inside the bone.  This is the part that was the most uncomfortable.  It felt like a white hot zap was flowing through me, and out.  Like the jolt of a nerve.  Unnerving.  Next, they twist the needle around in order to take a core - a piece of the bone.  They're looking for about a cm piece, and unfortunately, my first core was insufficient, so they had to go back in and take another.  Then they bandage you up, lay you on your back to put pressure on the wound, and send you home.  Quite honestly, the anticipation was the worst part of my first bone marrow biopsy, for me.  I've had dental procedures that have been more painful.

Unfortunately, more bad news when the report finally came in.  It stated that I have a "variant" of the Philadelphia Chromosome that is resistant to therapy.  In CML, in most cases, a portion of chromosomes 9 and 22 break off and switch places.  This creates what they refer to as the "Philadelphia Chromosome".  Upon review, they found that in 85% of my cells, a small portion of chromosome 9 was lost during the switch - the deletion apparently means that the drugs that have been developed to treat CML thus far, don't work quite as well as they should for me.  This was further supported by a test that they also do which looks at 20 cells to see how many still carry the Philadelphia translocation.  Nineteen out of twenty were positive for leukemia.  So that's frustrating.  To have to endure low blood counts, and almost monthly transfusions, and STILL not be making much progress toward eradicating the leukemic cells is absolutely disheartening.  Crushing.  Devastating.

My Dr's have started slipping questions like "do you have any siblings"?  "does your sister look like you"?  "have you been HLA typed"? "has she"?  into the conversation.  (Yeah, they're none too sly.)  I haven't.  And I'm not going there yet.  Maybe Tasigna is going to kick in, and start kicking leukemia butt.  (I'm giving it another month.)  If not, then maybe Bosutinib is the one for me.  Or Pontinib.  While there is a chance that the medications are not going to work well for me, there is also a chance that one of them WILL.

And so...for now: serenity, courage, and wisdom, please be with me.