Blog Archives

Final CT Results: "disease progression" turned out to be an 'Alternate Fact'

3/30/2017

Hello Madison fans and followers, good news! The target tumors being monitored did NOT grow: the larger 'watch items' actually decreased in size by 25%, which medically suggests the disease is currently "stable". A 30% reduction is considered in retreating or remission and the doctor reported that he's very happy with the results. Remember, we're only eight weeks in. The pleura is fairly messed up considering what's been going on in there, but "not especially alarming".

So wait, what!? How can it go from "worsening of metastatic disease" early in the week, to favorable results, the physician being very happy"?!

We suspect based upon the 'messed up pleura', told that reflections from the hardware in her neck, scar tissue, it can be extremely difficult to determine exactly what's going on without extensively studying and painstaking comparisons, while also factoring in all that's happened in that region of her body since November... not since just late January, all while understanding the aggressive nature of this rare disease. There's a high probability the person who read the initial imaging and wrote the preliminary reports has never seen or even heard of chordoma... not their fault.

We now have a much healthier level of patience and appreciation for the time it takes from the day of the CT scan or MRI, to get FINAL report by an expert physician. The roller coaster ride this week was avoidable but again, the coordinator who offered the preliminary written report did it with the best of intentions.

Chordoma and Madison Rose... both one-in-a-million!

A Nail-Biter At First

3/28/2017

Starting with the end of today here and now, we'll open this post with it's not all good news, but unclear until a deeper dive/read of the imaging taken on Monday... how bad... if at any bad. Wait, what's that mean? Exactly! How good or how bad depends upon our perceptions, entirely on each of our expectations blended with our hopes and interpretations of what we want/hope for. So were the results good?! There's no black and white, yes or no, so now to the beginning of this trip.

Madi and mama-bear left Sunday for SF. Madi had a CT scan on Monday and today Tuesday was the normal blood-work, EKG, and doctor appointment. This clinical trial coordinator provided a preliminary written report/assessment of the CT. The person who wrote that report compared Monday's CT, with the January 19th CT, and wrote the preliminary report as as he/she saw it, from the hip. Madi and mom were in the office for an hour... reports in-hand waiting to meet with the physician/NP. They'd texted a few paragraphs of a few pages to dad and for an hour we were each Googling to grasp an understanding of what the heck a 'patchy centrilobular groundglass nodule in the lower left lobe" means. Wait... "rogue lung" is her right lung. Is there a new nodule (tumor) in the left lung... or is this the left lobe of the right rogue lung? Another written observation was "Interval worsening of metastatic disease". Holy sh!t, that's hard to miss, and misunderstand, which is why we lay patients and parents need to exercise restraint not jumping to conclusions, and leave translating the data from data, to information by expert doctors. "Worsening of metastatic disease" implies the meds are ineffective. So wait, what... are they?!

In many perfect worlds, there would be no evidence of disease, the teaspoon of fluid drained every four days would be labeled "normal", and normally absorbed, so then ideally the drain tube can be removed from her chest. In this same perfect world the scans would be crystal clear and yes, and we would all sing wonders that there is a pill that eliminates all symptoms and evidence of this advanced, extremely aggressive metastatic disease in just two short months. Oh yea, and politicians would answer questions completely and honestly, and the parties would stop throwing rocks at one another.

To say the PA was upset that the preliminary report was offered to Colleen and Madi would be an understatement. Taboo I believe, for any non-physician to do what he did. We feel he's fond of Madi and believe he thought he was helping provide information but that's not necessarily the case.... in our opinions. He provided data that was interpreted by someone who only compared one CT to another, but without understanding her medical history. Our understanding was one 1CM nodule in Dec 2016, that grew to 4CM in January, and spawned other nodules. Had the person who interpreted the preliminary report ever seen images from this one in a million disease, or Madison's variation, about one in 20 million. Not likely, so perhaps he called it as he saw it, labeling it "Interval worsening of metastatic disease of disease" which rocked us until clarified.

So today was a nail-biter, all involved ready to start drinking by 10:30 AM. Heavily. What we saw in December and January... a dramatic increasing amount of fluid being drained every four days. Having started the trial meds Jan 31, we've seen decreases from ~3 cups of fluid being drained every 4 days, gradually reduced to a teaspoon. To the layman, what's that mean again? Exactly! It's subject to each persons hopes mixed with how they interpret what they read ... and what they (we) pray for. As a lay interpreter, the tumors appear to be infinitely less pissed-off given dramatic reductions of fluid pooling. That's good, yes? But wait, the notable tumors grew a tad. That's bad, correct? Depends on your own observations and expectations. So was today a good day or not? Depends upon you and your interpretations. What we perceived as explosive growth and spawning new nodules, was some slight growth. Heck, it's still early in the trial... we'll take that! We'll know more this Friday.

Notes taking by Madi and Colleen:

Decrease in pleural thickening
Nodularity is stable. There is a little bit of enlargement in a specific area. Will be remeasured and validated.
The lead physician doesn't always agree with the radiology preliminary findings. Some slight increase in size in some tumors... He doesn't count it as "disease progression". (the fact there isn't explosive growth to the lay dad again, feels like good news. Many people are so full of hope expecting miracles by the 8th week may feel disappointed. Reported today, "the medicine takes a while to work" and "already seeing improvements in pleural effusions (chest fluid)".
Doing clinically well: a good sign the drug is doing something positive

Next steps:

We should have the final CT report by Friday (which will be explained by phone by the physician :-)
Madison and mom left San Francisco with the imaging on CD. As per usual, we'll mail a copy to Madison's expert chordoma team at MGH in Boston, and another set to her local medical oncologist in Woodland Hills.
She has 28 days worth of meds (versus only 14 requiring travel twice monthly), so going to San Francisco should only be once a month looking ahead. (woooo hooo)

A fine box of wine preferably via IV, and aerosol cheese with crackers (a redneck weddin fixin/delight).

Wait... main-lining... Vella wine in a vein via IV, is that really a thing?!

Depends upon your interpretation of what "therapy" is... what do all y'all think?!

Thanks for your support and prayers!

With Love,
The Jones Family

Back to San Francisco on Sunday

3/25/2017

Week 8 on the clinical trial medicine Tazemetostat: Next steps will be taken on Monday, fresh CT and/or MRI, on top of the blood-work and EKGs. The new scans will be used for comparison purposes... how do the lesions in her rogue lung compare today, to the baseline pre-clinical trial images taken in January?

Scanxiety... it's a thing. That said we as lay parents and patient see what we see: less fluid drained each interval, so we're cautiously optimist full of faith and hope. As soon as we have the results, we'll post here.

Love,
The Joneses

Zero Dark Thirty

3/14/2017

Back up in San Francisco this week, the first appointment of the day was at 7:15 AM, this on a fresh change of the clocks.... so zero dark thirty. A walk to the bus/tram stop for a ride to the Mt Zion medical center where all of the poking, prodding, and exams happen.

Reporting to the physician/principle investigator today that the fluid accumulating in the rogue lung appears to continually be less and less: he like us, seemed cautiously optimistic. Had the tube dislodged, did it clog, etc. Showing him a picture of the last fluid drained, it was a much lighter pink than beet red... so what's that mean?! Exactly! We're not doctors...

...but wait, he is! He said he's "really really pleased, these are good signals". He did add that the CT in a couple of weeks will paint a much better picture. (typical doctor I think... always have to leave an 'out'... I'd likely do the same thing so understandable, but still appears promising!)

After a much deserved nap, off to Fisherman's Wharf for the evening. Wandering in to a card shop, she found this.

"Falling down is part of life... getting back up is living".

The doctor isn't concerned about her her post-pneumonia cough. He said a nasty bout of pneumonia is like Hurricane Katrina. A really really bad storm for a few days, then a long time for repairs.

All things considered, it was a good trip!!!

March 11th, 2017

3/11/2017

So it's always something, aye? An unfortunate case of pneumonia is slowly getting better with meds, but Madison's staying busy given it's not infectious... as far as you know. She is confident it's not an effect of the rogue lung, just a timing challenge when she verped (a wee bit of vomit and a burp)... a bit of that gurk (glock plus verp : ) traveled down the wrong pipe in to a lung.

So less fluid being drained every four days... so what's that mean? Exactly! We're not doctors but remain cautiously optimistic. Fluid that was accumulating ~125ml (1/2 cup) daily (drained every four days), is now less than a tablespoon accumulating daily... over a tenfold decrease in fluid build-up since starting the meds. Immensely less troublesome to lay patients and parents alike. Thank you to the research and scientist communities for making meds like these available to rare cancer patients, and a special shout out to Him... thank you God.

Monday brings another 2-3 day trip to UCSF in San Francisco, number six this year. This one should be rather routine ... a flurry of appointments that can be done in a couple of days. The 8-week mark is in two short weeks. That will likely be 3-4 days with new MRI, CT, x-rays to compare against those take in January.

Last summer Madison visited her uncle Jeff who helped bring her inner bad-ass and some old-school Nebraska redneck to the surface. Love it! We like to think of it as "hey chordoma... take THAT"! Video at this link.

Thanks for following along, and for the positive energy and prayers. We'll post updates mid next week after we meet with the UCSF team.

Love,
The Joneses

XMO 2017 Group Pic

3/4/2017

. Madi's in the black shirt with yellow print

... front row, 5th from the left.

Retraction... the tube was clogged, but still!

3/3/2017

The half-gallon milk example pictured here was after 10 days of build-up in Dec 2016
The cup was after 8 days of build-up on Thursday... the tube actually was clogged (2/27 thread below), hence the small amount of fluid pictured. That said....

We'll take it! While it would have been truly amazing for the rogue lung to have dried up almost completely... just one month of the new meds this picture is still worth a thousand words, and we shout again hallelujah. Hallelujah!