Oncology TeleVisit

I had my post bone marrow biopsy follow-up appointment with my oncologist today.  We discussed the biopsy procedure, my lingering pain at the site of the biopsy, my lab results and my current condition in a Zoom TeleVisit (seen in photo, at right) which meant we didn't need to drive to the hospital.  The appointment was in our living room.  

First we discussed how I have been feeling over the past few months.  

My oncologist seemed to be a little surprised that I am still feeling quite lousy since my ambulance ride to the hospital in January and that I am still having great difficulty in doing just small things for a couple of hours.  In order for me to do something for just a couple of hours (ie, grocery shopping, dinner with the grandkids, Kenzie's figure skating, Lukey's hockey, etc) requires planning and closely managing energy.  In order to do any small thing, I need to get extra sleep beforehand and taking extra medications for a day leading up to when we want to do something out of the house for an hour or two.  I'm still experiencing significant breathing difficulties every few days especially when I try to cut back to a "normal dosage" of my medications (although there really is no "normal" dosage) rather than continually pumping high doses of extra medications.  The bone pain and joint pain is also brutal.  Then, throw in all the other usual debilitating symptoms...  and I'm having difficulty doing anything for more than an hour or two.  My oncologist is finding this concerning.  She expected me to be fully recovered to my old "new normal" by now.  At this point I'm feeling like how I am struggling today is actually my new normal.

My oncologist feels these continued breathing issues sound like they might be due to a lingering "Long-COVID" problem which is, in turn, triggering a mast cell reaction due to inflammation in my lungs.  At least she feels like it sounds like this is what I am experiencing and this long-COVID thing is triggering additional mast cell issues what are also affecting my lungs in a very negative way.  As a result, I am being referred to a pulmonologist to check my lungs.  We'll see how long it takes to get in to see a new specialist...  my guess is it will be about six months.  

Now, the results of the bone marrow biopsy...

- The good news is my cells are looking normal in size, shape and structure (as opposed to clumping together or being a spindly shape) and the counts are in the normal range.  There are very few blasts which is good.  This is all very good news.  

- There is nothing in my bone marrow biopsy results to indicate that I have progressed to a worse stage/category of my mast cell disease.  This does not include problems seen in my blood labwork but I'll address that below with the other bad news.  Having no obvious signs in my bone marrow showing a progression of my mast cell disease is very good news considering how poor my overall health as become in recent months because, clinically, I'm exhibiting all the signs of a more aggressive form of my illness.

- At the moment, there is no sign of leukemias or lymphomas as a secondary illness.  This is also very good news, obviously. 

There are a few things that are not so good news...

- Apparently, they did find a gene mutation in my bone marrow biopsy which indicates a higher risk of leukemias, lymphomas, and a potentially worse prognosis of systemic mastocytosis.  I guess this one is not much of a surprise since I have been struggling with systemic mastocytosis for a couple of decades now.  I guess the real downside isn't that this mutation was found but that this finding likely points toward a higher risk of things getting worse.  Then again, like everything else, this finding could change in time in a positive way. 

- A problem with my chromosomes was found also.  This could be related to older age or it could be related to different leukemias.  A type of systemic mastocytosis falls into a category of leukemias.  This is something to follow in future bone marrow biopsies but, for now, we're leaning toward blaming this finding on old age.

- The other alarming thing is my tryptase levels continue to rise indicating a worsening of my primary illness.  These tryptase levels are found in my blood labwork.  These numbers are rising much more quickly now.  An elevated tryptase is a sign of unstable mast cells and/or having too many mast cells.  The higher this number goes, the worse my health will be each day which is really what we are seeing in my daily health lately.  So this isn't all that great but it does explain how poorly I have been feeling lately.

We have been trying to bring this tryptase level down with mast cell stabilizers for the past couple of decades.  Until the past few years, we have been relatively successful in managing this number.  Even when controlled relatively effectively, this tryptase level was still always higher than it should be.  Now my mast cells are appearing to be uncontrollable at my current intake level of medications.

As a result of my tryptase levels rising higher and higher over the past few years, we agreed it would be a good idea to attempt to increase my dosage of my mast cell stabilizer.  This seems like a no-brainer but there are some problems in doing this.  We are in uncharted territory with a dosage this high.  I am already on the maximum allowed dosage as far as the insurance company is concerned.  I know of no other patients at even my current dosage.  Sheila and I got the impression that my oncologist hasn't seen doses this high either.  Hopefully, this medication at this higher dose will help to stabilize my mast cells a bit so I can have some enjoyable moments every now and then again.

This mast cell stabilizer drug is currently my most expensive drug too.  When I started taking it about 20 years ago, the annual cost was around $72,000 (and that was at half the dosage I am at now).  It has slowly dropped to below $20,000 annually and my cost, fortunately, is negligible.  Considering the cost, we'll see what the insurance company has to say about increasing this dosage to beyond their maximum allowed dosage.  

Talking about the cost of this drug got me thinking about another spot of good news which is related to there being no concrete findings in my bone marrow of a more aggressive form of systemic mastocytosis.  If we saw signs of a more aggressive form of my disease, then I would be started on one of two new chemo drugs that range from $28,000-37,000 each month.  Just the thought of needing to fight with my insurance company over getting this covered is enough to cause stress and stress causes my mast cells to degranulate which then causes a significant and life threatening deterioration of my health.  Fortunately, my life is pretty much stress-free when I look beyond my daily health difficulties.  Regardless, I'm happy that I don't need to find the energy to fight for coverage for either of these new chemo drugs right now.  That is a fight I'd much rather put off another decade or, better yet, put off altogether.  

To sum up...  I need to see a pulmonologist for potential COVID damage to my lungs...  we are attempting to increase the dosage of my mast cell stabilizer drug to counter the poor health...  they found some problems with my chromosomes...  and, unfortunately, the bone marrow biopsy is showing a genetic mutation indicating I am at a higher risk for worsening systemic mastocytosis/leukemias/lymphomas in my future.  

For now, I'm hoping that the higher dosage of my mast cell stabilizer will get me back to the health I had before my January emergency hospital visit which was relatively stable compared to my health today.