Day 1 of Ibrutinib

 I took my first pill of ibrutinib today at 7am.  The pill "wallet" (individual pills in individual "blisters" on a 4-week folding card) had extra stickers warning that I should also be aware of potential dizziness and not being able to operate a car or heavy machinery.  

No dizziness so far and I had a good 9.6mile bike ride on an easy, safe, low-traffic path.  That's a good start.

Good Treatment News

Yesterday afternoon I got some good treatment news -- I am expected to be able to suppress my cancer to near zero via a well-tolerated daily pill. 

I am very happy as this should mean no disability and as good a chance at a near normal daily life, and as few doctor visits as any of us "experienced staff" can expect.

Alongside the official confirmation that the same cell line of Mantle Cell Lymphoma had re-emerged, I was introduced to BTK inhibitors, which are neither chemo nor immuno therapy, but rather block an enzyme.

I will know if that daily pill (which starts next week) is working because the lump on my neck should "melt away" by Christmas.  

There are potential side effects (atrial fibrillation and blood or infection problems) but there already exist alternative pills to pivot to when the first pill stops working (which it will in 6 to 36 months) or if I can't tolerate the side effects (20% of patients)

When the pill stops working we expect to see my neck lump return, like one of those Butterball turkey timers, and then we'll switch pills, having caught it early. 

I hope to either be gently buying time for many years, or that the price of the CAR-T "cure" will come down. (For now insurers prefer $8k of pills per month to $500k for CAR-T in a single go). Or that some other cure arises, which is the miracle of our age of progress.

Compared to debilitating chemo I had imagined this seems a great option.

PET Scan (Picture) sets up Core Biopsy (Needle)

 The pet scan showed two bright lumps in my neck.  We'd already seen the one on the front.  The second is deeper--you can't see it but I have placed my fingers on it.  It hurts only when I press on it.  It is located about halfway between my ear and my spine (going around my neck) and positioned about halfway between ear-level and collar-level vertically.

It has been caught very early, whatever it is.  There's a small chance it is a lymph infection or malfunction (it *feels* like a 10% to 20% chance), with most of the rest being different kinds of cancers.  And if it is cancer, it is not the crazy-fast-moving kind. (I'd say it has been growing at about the paced it did in April 2017, and I've already reached the diagnostic level that it took until June 2017.  So we're moving faster and the lump is progressing the same.

Just as I was happy to skip sonogram and CT scan and go right to PET, I'm happy that we'll skip the Fine Needle Aspiration (or FNA--a skinny needle) and go right to a Core needle biopsy (a big diameter--call it a matchstick size sample)

Last time we "tried to get away with" only doing the FNA and, when that supplied too little tissue to give a cancer diagnosis, we jumped up to a surgical biopsy (under general anesthesia).

I hope this time we get it all done with one procedure and in half the time.  The Core biopsy (scheduled for this coming Thursday) should get enough material that they can both look at the cell types and maybe do some genetic testing (to see how close or far, mutation-wise) it is to being the "same" or a "new" cancer.

The core biopsy is done with just local numbing, and there's no prep diet and I can drive myself home afterwords (was tempted to bike, but I think I'll have some hole-closure stitches to worry about)

Pictures before Needles before Knives

The German railways are famous for a mantra that describes how they solve problems:

Organisation vor Elektronik vor Beton

Organization before Electronics before Concrete

The idea is to try to address the problem with simplest and cheapest expensive solution.  Imagine a congested rail junction. Can we reorganize the timetable?  If so, great, we're done.  Still congested?  Can we redo the signals to get things flowing? If so, great, if not (and only then) try adding a new track.

Climbing the Diagnostic Ladder works the same way.

Pictures before Needles before Knives

Or, really, fingers, pictures, needles, knives.  I found my lump with my fingers.  I sent pictures (and dimensions) to my Oncologist and hoped we could go right to a needle biopsy (there's something so re-assuring about the thought of stabbing it).

But protocol (and insurers) demand Pictures before Needles before Knives.

In 2017 I believe we did 3 rounds of pictures:

1. Sonogram
2. CT Scan (x-ray)
3. Fancy Scan (either a PET or an MRI)

When you don't know what you're dealing with, they're hoping that the problem is solved at an early level. The sonogram hoped it was an abscess (or aneurysm).  The CT scan hoped for something similar.  By the time you get to the PET, you're asking "is it a hard lump that likes sugar a little too much?"  Only after this do they do a needle biopsy (sucking a little bit out).  Only after that was inconclusive did they authorize a surgical biopsy.

Pictures before Needles before Knives

My doctor's first offer was a CT (partly because that's what insurers usually want first because it costs about 1/4 as much as a PET scan).  I asked for a PET or a needle biopsy:  "C'mon, all a CT is going to say is 'you have a dense mass on your neck,' which is something that we all already know from my iPhone, or can tell from our fingers."

Doc says they'd never approve a needle biopsy--its "pictures first," but at least Blue Cross Blue Shield approved skipping right to the PET, which is scheduled for next Tuesday, with the official "what'd we find" appointment (these "diagnostic reveal" appointments are always in person, in case there's bad news, which we're expecting ...we expect it to say "your lump liked the sugar"

Knock, Knock

A variant on my 2017 invention:

Knock, Knock
Who's there?
Cancer
Cancer, Who?
Can't surprise me, I've seen this lump

On the center-left in this photo is the dimple and scar from the 2017 biopsy.
On the center-right is a lump which I noticed two days ago, 2022.

So this isn't a diagnosis, but the standard questions at every vigilance visit have always begun with "any lumps or bumps?"  The answer just 10 days ago was "no," though, upon finding this one while fidgeting on a video call, I had a sense that I'd felt this before, just below the threshold of consciousness.

,

If this were a Winnie the Pooh book, it would have a chapter heading like: In which Pooh finds a lump and sends a note to his friend, Piglet

Not worried about Liver, On to Vaccinations

My Liver biopsy was "unremarkable" nothing suggested a cause for my trouble. I stopped worrying and will let "tincture of time" address it (it is quite likely that it was just one of the post-transplant meds were irritating my liver). I stopped the antibiotic in November, which was also my last appointment, and at which we said I'd start "childhood" vaccinations in February 2021. We did a seasonal flu shot too, since it was (1) worth a try before the worst of the flu season (2) cheap and low risk and (3) if it didn't work it was still worth a try (and a try again next year) Feb 4th is my next appointment, and we'll probably do one or two of the kind of vaccinations that a 1 year old would have (but on a slower 3-month interval over the next 18 months of maybe 2 shots per visit, and 3 months between boosters). MEANWHILE: I got a slot for the Covid vaccine. I'm on that list of immune-compromised "at risk" people who, if it works as a vaccine, that'd be a much better way of encountering COVID rather than as a "first impression" contagion. I will go this Thursday to Temple Israel (the synagogue down the street from Beth Israel)

Officially Done with Cancer Therapy; Now on to the Liver

I'm a little embarrassed to say that finishing post-transplant immunotherapy happened in July and I failed to blog about it. It was July 23rd. It was all so gentle: a final blood panel and the injection of the honey-like Rituxan Hycela (rituximab/hyaluronidase) into my belly fat. Proud to say: daily cycling (and now swimming) has given me less belly fat (still plenty adequate for injecting Rituxan Hycela) Anyway, when you're done with therapy, they have you ring a little ships bell as you leave. It happend kind of suddenly. I probably should have taken a selfie. But I rang it and was happy and was then fixed at getting downstairs, on my bike, and biking home. My fitbit reports that it was 43 minutes to get there, and 51 minutes to get home (probably because it was *hot* and I didn't have the opportunity to stuff ice cubes into my clothes like I did for the home-to-hospital stretch). It is about an 8-mile bike ride. So what's this about the Liver? Well, back in Sept 2017, when I first started the harsher Ara-C chemo (which requires hospitalization, where previous Bendamustine was outpatient), my liver freaked out, adn they had to reduce, slow, and spread out my treatment (took Fri-Mon instead of the intended Fri-Sun) The ruling was: MCL was going to kill me in the short term, so let's fix that while minimizing the unavoidable liver reaction, and worry about my liver later. So now it is later. For my liver, I'm in the care of the Tufts-affiliated system (my insurance, and local to me) rather than the Harvard. It's a real joy to live in a City where Tufts and Harvard can vie for my business. A couple of weeks ago they drew a "personal record" 11 vials of blood (1 just to throw away, and 10 to do about 40 different liver tests). Result: Liver enzymes still high. Mitochondrial Antibodies high, suggesting that my inflammation may have an auto-immune source. But the diagnosis, so far, is Primary Biliary Colangitis. Which is "doctor" for "his bile ducts look itchy" (Primary = no other evident cause", Biliary = Liver Bile, Colangities = Bile Duct Inflamed). Clear enough? In fact, it is a recently-coined name for a condition in which the patient has no other symptoms but has liver tests suggesting inflammation. (if it had symptoms, they might call it Cirrosis) That autoimmune source could still be related to Rituxan, which is immunotherapy. Or it could be my white-cells-and-lymph systems are still wonky. Or it could be a coincidence and the real cause may be the Bactrim (antibiotic) or Acyclovir. Or I could just be That Guy With High Liver Enzymes. The ultrasound-guided biopsy on Thursday will take a first look at the tissue itself. I expect it to serve as a baseline, and probably the first conclusion will be: 1) Let's wait until October, when the Rituxan will be "long over" 2) Let's wait until January (ish) when I'll likely be off the anit-viral and anti-biotics I expect we'll be testing liver enzymes periodically along the way. Cheers and good health to all!

Experimenting with Google Trends for "busiest day"

Experimenting with Google Trends for "busiest day" https://trends.google.com/trends/explore?date=today%205-y&geo=US&q=jobs,movies,calculator,games

Medical: Healthy Enough to Fight the Flu

So, the good news is that they sent me home from the ER midday on Christmas Eve.

I went because I felt lousy on the 23rd and had called at 3am on the 24th when I was running a 101.1 fever. Standing orders from Hospital to me are call with fever and/or related symptoms (in this case fatigue).  Standing critique of Hospital by me is that they undervalue sleep as a curative.

They wanted me to come straight to the ER by 4am but I said, "how about I see how I feel when I wake up and check with the Clinic at 8am"  Fair deal?

The real issue (which I admit is not worth gambling on) is whether I had a functioning immune system at all.  Happily (1) I did, with a White Blood Count of 4.5--my first "Normal" range count since March of 2018, actually and (2) I was fighting plain old Influenza A, so there wasn't much for science to do anyway beyond (2a) Tamiflu, on the hunch that my first flu symptoms were monday and (2b) lots of liquids

And avoid people and wear a mask--which is a way, WAAY, better deal that spending Xmas Eve (& morning) in the hospital.

So I'm multi-grateful:
1) To have a functioning immune system
2) To have a "known quantity" virus with a known treament
3) To have no secondary (bacterial) infection (that'd require an IV, usually)
4) To be able to wear a mask and do xmas with the family

Merry Christmas!

Medical: July 2019, a great visit

Its been 2 years since my diagnosis, and about 18 months since my stem-cell transplant.

Had I not missed a couple of courses of Rituxan, I'd be nearing the end of my post-transplant infusions.

Yesterday I had a great visit:

1) Neutrophils at 2.5 are normal and total count in the high 3.x is near-normal.  So I have plenty of the right kind of white cells

2) Thanks to drinking about 1Liter of water before my blood draws (and taking my antibiotic late the night before). I had no trouble with the kidney function tests-creatinine and Potassium both normal. It did mean I had to go pee about every 15 to 25 minutes, however (before the commuter train, at North Station, as soon as I burst into the Shapiro Clinical Bldg lobby, after checking in on Shapiro 7, after having my vitals taken, and about 4 more times during my visit)

3) They still don't  like  my Liver numbers--which have always caused them to worry and scan (and cut short  my first Ara-C way back when). The numbers have been consistently elevated (and bounce around) for all two years that we've been regularly testing them as part of the "green top" blood draw (the Metabolic Panel).  Dr W wants me to see a Liver specialist to see if it has a cause or is just "Normal for Me".  Over time, we've done ultrasounds and x-rays looking for liver/abdominal abnormalities and never found anything in the liver.

4) But, Yay! I was approved for a 30 minute injection of Rituximab Hyaluronidase Pronounced: ri-TUX-i-mab hahy-uh-loo-ron-i-deys (brand name: Rituxan Hycela™).

The traditional IV bag of Rituxan, even administered at high speed was nearly 2.5 hours: 1:30 minute drip, which always came with about 20 minutes of IV setup before and 20 minutes of final-pump and cleanup after, and usually came with an IV of steroids before and Benedryl nap in the middle.

Rituxan Hycela, by contrast, is a thick injectable.  Only pills as prep (steroid pill, acetominiphan, Pepcid, and Allegra), and then after a wait, a 45 minute cycle:
- Vital signs taken
- 20 minute belly shot (it is thick, so the nurse maintains constant pressure and it makes a little hard blob under the skin.  I briefly had 7-pack abs.
- Vital signs taken
- Vital signs taken at :15min later

Since my pulse had slowed to the 50s at the :15 mark, we had me stay and do vitals again at the :25 mark where the pulse had returned to the 60s

I have good heart health, by the way, with a low resting pulse.

5) And I (fairly) easily climbed the very long escalators at Porter Square on the transit trip home. (Didn't attempt the stairs-only climb, but may next time)

FROM HERE (if all goes as well as today)

August: A Liver Visit
September: Labs+Rituxan Hycela (maybe a CAT scan?)
November: Labs+Rituxan Hycela
January: Labs+Rituxan Hycela
March: Labs+Rituxan Hycela

so we'll run a little bit past "2 years of Rituxan" because I fell behind on doses.

June 2020: Shingles Vaccine, TDAP vaccination, Pneumonia vaccination
Fall 2020: Flu vaccination

Finally got that Rituxan

Two weeks ago, I went in for counts which, had they been bad, we'd've tried IVIG (another "reboot white cell production" treatment that they don't know why it works, just that it does when others have failed).  Fortunately, the earlier Neulasta had resulted in hopeful counts (near normal)

On Tuesday (Feb 26) my WBC and ANC counts had recoverd further--some actually in normal--and enough to get Rituxan (and we'll test again in 2 weeks to see if they've been tanked or have achieved robustness)

Medical: No Cancer (but not enough Neutrophils either)

First the good news: tests show me about as "cancer free" as tests can.

Also, my Liver Enzymes are back to totally normal

Most people don't have a marrow biopsy (left hip this time) and a PET scan to tell them that the cancer is "very gone" (or very undetected, anyway).  But I did.

The Marrow Tests:

• Looking at the marrow core, it looked like normal, healthy marrow. It looked like a very red matchstick, with tiny little gnocchi (the marrow cells) clinging to it.
• FISH Test looks for mutations inside cells.  It found none (when done on my hip-marrow sample)
• Laser Cytometry looks for mutations on the outside of cells. It found none (when done on my hip marrow sample)

The PET Scan

• Looks for the "glow" of cancer that's sucked up the radioactive sugar they inject. 
• I was a 1 (the lowest possible) on the Deauville Scale (where a 1, 2, or 3 are all good)

1. no uptake or no residual uptake (when used interim) 
2. slight uptake, but below blood pool (mediastinum) 
3. uptake above mediastinal, but below or equal to uptake in the liver
4. uptake slightly to moderately higher than liver
5. markedly increased uptake or any new lesion (on response evaluation)

The bad news is that the tests were necessary because we're trying to understand why my white blood count (ANC Absolute Neutrophil Count) are low:

• 1500 to 8000 is normal
• 1200 would be "normal enough"
• 800 would be "low but probably enough in most circumstances"
• 600 to 780 is where I've been bouncing around (In weekly re-tests Jan 10th to Jan 25)
• 500 is "low enough to worry" and be "officially" neutropenic
• 200 is where I was last Thursday

There were two guesses: 

1. the cancer had returned and was crowding out healthy production.  This seemed unlikely because I was not anemic: my red counts and platelets were fully normal
2. the follow up Rituxan (immuno therapy) was to blame.  Recall that that as part of a 2-year post-treatment to mop up stray mutants, I am scheduled for Rituxan every 2 months and had had it 3 such times.

With cancer essentially ruled out, attention turns to it being a Rituxan side effect and me needing some sort of jump start on my neutrophil production.

They'd tried giving me neupogen (a once-a-day shot...but only once per week) just to see if it would jump start things.  It didn't.

On Thursday, when I was 200, they gave me Neulasta (a long-lasting shot) and scheduled me for a visit on Feb 12th, when they may try something else.

That something else will be an IVIG (Intervenous Immuno Globulin).  They don't know its method of action, but somehow it helps people in my situation.  

Medical: October Was Normal (or not)

In October I did my Rituxan with the proviso that my white count (neutrophils) were on the margin of being low.

Medical: "Id Reaction" to Poison Ivy

I have itchy red dots on my arms, legs, and along my spine. Here are your clues:

1. They appeared around August 23, several days after I got a 3-inch "stripe" of poison ivy just above my left ankle (around August 20), and shortly after a round of crazy antibiotics for my Pnuemonia
2. I never had a fever from this, so unlikely to be any of the usual "spots" viral diseases
3. They are symmetrical, generally appearing the same on the left and right sides of my body, even though they migrate up/down  to outer  and inner parts of my limbs. This suggests "an inside job" as the dermatologist puts it.
4. They don't look like lymph disease lesions, and aren't in the groin (where lymph stuff likes to go)
5. They don't look like Pityriasis Rosea (not red enough)
6. They don't seem to be a reaction to Bactrim or any of my other medications (they should have emerged closer to the time I started taking them
7. I've never had eczema or a rash like this (that I can recall)
8. The rash migrates: back of knee to front of knee to front of shin to back of shin at about the rate of moving to "the new place" every 2 days.  Same for elbow crease to elbow point to forearm inside to forearm outside.
9. The rash on my spine just sits there (as Pityriasis Rosea might, but not as colorfu)

I saw a Dermatoligist today.  His conclusion: My immune system is out of balance from the Poison Ivy.  He reasons:

1. Contact Dermatitis (Poison Whatever contact) can "go generalized" 
2. It is particularly likely to go generalized if it starts on the lower extremities (hard to get more extreme than the top of my ankle)
3. For an immune-compromized guy like me, it is very possible that even though I might be a little bit short of fighter cells, they can get into an auto-immune feedback loop and just  "keep fighting" if I happen to have a shortage of the immune "control" cells that might ordinarily calm things down
4. This generalized reaction is called an Id Reaction.

Were I not immune compromised, the solution would be oral steroids to force a calm-down and break the cycle.  But oral steroids can suppress the immune system, generally.

So the solution is a topical steroid, twice a day: covering every red dot with a white dot of cream (or rub on a concentrated area of dots).  Topical steroids do not suppress the general immune system, but have been shown to stop the runaway fighter cells.

Medical: Pneumonia Last week, Good This Week

For the record (I should have been blogging this as I went)

In early August, I developed a post-nasal drip, with a little tickle at bedtime and a roaring sore throat upon awakening (that quickly dissipated).

But on August 10th, after swimming 500m at the local 50m pool, I came home, felt faint, and had a fever of 102.1

So I called the BI, and they had me come to the emergency room.  X-rays, bacterial culture, viral culture. The Works.  Yes, my lungs were cloudy and I was admitted from Aug 10 to 14.

I had a follow up blood-draw on the 16th (which showed low blood counts and high liver stress).

I had a follow up visit to the BI clinic yesterday, mostly good, but my white counts were too low to do my "2 month" Rituxan (which both relies on the immune system and a can lower blood counts, so we'll try again next week)

First Swim & Dental Visit Next Week

I've never been so excited to visit the dentist in all my life.

I've been cleared to have my teeth cleaned, something I usually forget to do, but in this case it was a highly speculative appointment set back in January.  I'd always thought I'd have to cancel it--dental work has a lot of opportunity for infection if you're immuno-compromised, and they generally say "not in the first year"

Also, I was cleared to swim.  This happened before I went to the beach, actually, and forgot to blog about it. There were two precautions: 1) a belly-shot of neupogen (boosting stem cell production, and ultimately the whole white-cell family) and 2) no bare feet on deck

Basically, they worry about cuts in the mouth and cuts on the feet as being in places where there are a lot of bacteria, and slightly different ways the body heals things.

Now its "Maintenance", bi-monthly, til 2020

Once again, my CT shows clear of cancer.  This was actually not a regularly-scheduled one, though, it was because as I begin my "maintenance" phase, I had elevated liver numbers--my liver was clear, too (recall that you can't "PET" a liver because it metabolizes sugar right alongside cancer).

We don't have any good theories as to why the liver numbers were elevated, but it is true that they went down when I stopped taking my Chlorella and Spirulina (two superfoods that are good for your immune system).  The nice people at Beth Israel knocked me off them because they were *too good* for my immune system (which they were trying to wipe out at the time).  Now they've knocked me off them because they are suspected as being bad for my liver.

But here we go on "maintenance" immunotherapy--targeted to trick my body into thinking that any mutant lymph cells are a disease I've had before.  Rituxan every 2 months for the next 24 months.

Next up, August back at Harvard Vanguard in the Fenway.  Its an easier place to go now that the super-specialized care at Beth Israel is no longer needed. HV is perfectly capable of dosing me with a big bag of stuff.  And they have better WiFi

October, I will, however, go back to BI for a 4 month check in.

On a personal note, I'm crazy-busy with Data Science projects.  If you know anyone with a big pile of data that it's nobody's job to look at, let me know.

I'm also completely comfortable on my bike again, climbing hills and doing "Cambridge commute" type rides in the 6-mile, 35-minute range.

That Masked Man

I did some forbidden yard work--Mother's Day Mulching--but, in my defense, I was (1) careful to wear a mask to limit any dust (fungal spores) that I might have inhaled and (2) we kept the mulch pile cool and dry to minimize any fungus. I hope that worked!

Back on Antivirals and Antibiotic

We tried to stop my antivirals after my late-April appointment, but  by 5 days after stopping (last Friday ish?) I began to feel first an itching and then point-tingling, particularly around my mouth, but also at various points almost anywhere on my body.

The net is that it was probably the early stages of Shingles, which got as far as red, raised spots, maybe 4mm in diameter in some places.  So now I'm on Valcyclovir to stop it and then will go back to Acyclovir to keep it away--probably until Jan of 2019.  All that is a bit disappointing since my gut seemed to have been doing better during the break.  Oh well.

I'm told a full blown Shingles outbreak is something to be avoided.

They also didn't like my CD-4 count (a white blood cell).  They wanted 200 count and I was an 80, so I'm back on my ongoing Antibiotic too.

Results; no cancer was discerned in the CT Scan images.

I won't be released to "maintenance" treatment, however, until I have no lingering side effects, which have been identified as:

Impared executive function (impared learning and focus), which we will address with 30 minutes of uninterrupted book-reading per day followed by writing a summary of exactly 3 sentences. I managed 27 minutes of solid reading before peeking at the timer. (Thanks Tim M. for Ron Chernow's Grant biography!)

Gastro distress. I will stop my antiviral to see if it has been an irritant. I will cut my milk consumption and focus on lactaid and Kefir when I do. Also will do pre-biotic soluble fiber (oats, citrucel) followed by probiotic fermented foods (sauerkraut, kimchi, kefir).

Chemo is generally hard on the GI tract, and the effects can especially linger in the fine structures of the intestines (and the particular digestive processes that happen there).

IGR and IRR are things (enzymes?) That are produced by gut lymphocytes that keep down bad bacteria in the gut. The problem can be that the bad bacteria get entrenched in the interval where the BEAM chemo has wiped out all lymph cells. Sometimes the good bacteria naturally succeed in recolonizing, but sometimes a special (non absorbable) antibiotic is used to knock out the bad gut colonies to clear a spot for good recolonizing.

Ear canal pain. This may be scar tissue in my right neck (where the lymphoma began and was most present) messing up my right eustachian tube. My right ear hurt like crazy on my recent airline flight--recalling the pain that all kids risk when flying--and it still hurts occasionally if touched wrong. We will begin with heat treatment---funny because that's exactly the treatment I tried for a month a year ago hoping my cancer was just a matter of drainage!