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Recently diagnosed. Many questions.

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Hi All,

My name is Jon and from Virginia.  I am a Naval Aviator and a father of 2 with a badass wife!

Recently diagnosed with CML at the age of 32.  No symptoms what so ever.  Went in for routine blood work early November, WBC was at 23. Doctor had me do more blood work 10 days later.  It was at 27.  At that point he ordered a battery of blood work to start ruling things out.  Thats when I found out I had CML.  ONC said it was caught it early and in the "chronic phase".  He had me on 100mg Sprycel 2 days later and now on day 4 of medication.  No side effects so far, and still carrying on with normal life.  Initially scared to death but the more I learn, I thank God every day I was diagnosed with the "best" worst blood cancer to get.  My next appt. is late Dec, and ONC expects to see positive results.  I'm very grateful for a community like this.  I have been trolling every day since I found out.  I have so many questions and look forward to learning more about best practices to manage CML and live a long healthy lifestyle.  Please see below for my initial questions.  Thanks in advance!

- What questions should I be asking the ONC?

- Is WBC at 27 high?

- Best Hospital of East Coast for CML?  Looking to get a second opinion.

- I read conflicting info on what extra vitamins I should be taking. Any info would be great.

- What not to eat?

- This was on my blood work.  Can anyone interpret the percentages?

e13a2 (b2a2) transcript Comment % 01 352.6411 ----- e14a2 (b3a2) transcript COMMENT % 01 <0.0032 % (sensitivity limit of
assay) ----- e1a2 transcript 0.1537 % 01 Interpretation: COMMENT 01
POSITIVE for the BCR-ABL1 e13a2 (b2a2, p210) and e1a2 (p190) fusion transcripts.

- How long can I wait to expect side effects from Sprycel?

Thats all for now! Thanks!

 

It does seem it was caught early before overt symptoms. That is good.

You should be having weekly blood tests to verify that your blood counts are returning to normal and not dropping too low. Neutrophils and platelets are often suppressed during initial sprycel treatment. Also, 100 mg sprycel is emerging as too high a starting dose. Newer data suggests 50 mg should be started initially and either increased or decreased depending on response. High dose sprycel can suppress your normal blood system and slow your overall response as well as side effects (pleural effusion). Something to ask your doctor.

Make sure you get a vitamin D blood test and if below 40 ng/ml, supplement to get your levels above 50 to around 70 ng/ml.Vitamin D helps activate T-cells which fight leukemia. Chances are if you had high normal vitamin D you would never have developed CML in the first place (my personal theory). I would bet your vitamin D level is very low (< 20 ng/ml).

Track your FISH and PCR levels. FISH is most important at this stage. Once your FISH levels go to zero, CML will be history for you. Not a cure, just 95% progression free survival. Perhaps a cure over time.

I take 20 mg sprycel - almost from the start. I am now 'undetected' and have been for 2.5 years. Soon, I will stop therapy and "test" remission durability. We'll see.

Hopefully David will see your post and post excellent links he has for new patients.

... About that badass wife ... pictures, please!

 

Hi Jon,

Welcome. I wish we didn’t have to welcome you to this group, but here we are!

It does sound like your CML was caught very early, which is a good thing of course. 

Here’s a few resources on this site that are useful for new patients:

Videos: I’m afraid these are not sorted that neatly. It’s something I’ve wanted to do for a while, but our budget is - be be kind - constrained. But the names of the videos give you a good idea of the content. 

https://cmlsupport.org.uk/videos

I’d start here, if I were you: https://cmlsupport.org.uk/videos/chronic-myeloid-leukaemia-–-what-it

If you are more of a reader, check out our primer with a scary name (I really want to rename this some time, as it is off putting) Quantitative Reverse Transcription Polymerase Chain Reaction: A primer for patients. But you ought to be able to read the first 8 to 10 pages and make sense of it. Return back to this later when you understand more. It’s a really good booklet which was published with leading clinicians here in the UK (hence the overly medical name of it) so all of the info in it has been thoroughly reviewed by professionals and isn’t just random info.

Under the “About CML” menu on this site, you’ll find a “just diagnosed” section, which has a link to a templates page which has pages of things to ask your doctor: https://cmlsupport.org.uk/section/templates

In terms of what not to eat, basically stick clear of grapefruit. You’ll see some people on the internet worry about pomegranate and starfruits and all sorts. But unless you’re drinking a boatload of that stuff, don’t worry. Just keep away from the grapefruit (in short, it amplifies the effects of your medication).

Side effects are a hard one. Many people have little to none! But they don’t post on the internet bragging about that, they just get on with their lives. So the “online” patient population perhaps isn’t fully representative. 

Your result ... P210 is the most common type of CML, P190 is rarer. You will probably find that fairly quickly the P190 (and b3a2) component disappears. Difficult to explain, but it relates to the exact fusion point of the genetic makeup of CML. 

Lastly, there’s a link to ELN (European) and US (NCCN) guidelines for treatment of CML. The ELN one is quite hard to read - but is really good - and the NCCN one has some good introductory information for patients: https://cmlsupport.org.uk/section/cml-treatment-guidelines-tki

 

David.

The NCCN Guidelines may be blocked by an Ad Blocker, in my case by U Block Origin (across multiple browsers). Temporarily switch off the ad blocker to connect.

 

Hi Jon.

Welcome to the club no one wanted to join. Diagnosis is a very scary time for you and your family. You will go through lots of emotions over the coming weeks/months. This is perfectly normal so don’t beat yourself up at all. Just reach out on here and any one of amazing people will support you.

You are in very good hands and it’s hard to believe that a previously guaranteed fatal disease is now just a chronic blood disorder.

Yes you’ve been caught very very early. A white cell count of 27 is nothing. I was diagnosed at 37 with a white cell count of 330, massive spleen and lost of weight loss (I was late chronic phase) one of the highest I’ve seen on this forum to date.

Luckily for us as hard as it is to believe we just pop a pill a day and get on with our lives. Side effects are a difficult one as some people get none or hardly any and some poor people really struggle. What I will say is you will prob get some and some will go and some will develop later on in time. Mostly all of mine have gone or settled to near nothing. Give your body a chance to adjust this will take a while and all will be ok.

There are several milestones that you will meet over the coming months/years and once you reach MMR you can mostly forget about it all. I am 19months into treatment and only just approaching MMR now with my BCR @0.118%. Because I had heavy disease load it can take a while. I wouldn’t be surprised if you reach MMR in 6-12 months. MMR is the goal of the treatment and once you are there it’s a managed condition for however long. If Sprycel isn’t for you you will be switched to 1 of 6 drugs or lifelines I call them.

I know it’s really hard to believe at this stage but my bloods all returned to normal in about 4weeks on these amazing pills. If your prefer this disease is only a Cancer by definition it’s more a chronic illness.

Most of my life touch wood has returned to near normal. I am back at the gym lifting heavy weights and doing lots of cardio.

I will say give your body it’s best fighting chance and eat and rest well. Digest it all I am sure you’re going to be fine.

All the best to you and your family!

Alex

 

Jon,

I have an amazing doctor, Harry Erba, at Duke Medical in Durham, NC. I'm not sure how far away you are though. When I first found that he was my doctor, I started researching him and found numerous videos on YouTube where he was conducting a panel or addressing a conference. Whomever you find, check them out. I am convinced that having faith in one's doctor is critical to recovery.

Hi

I’m pleased they found your CML early. I ignored my symptoms for too long and my WBC was 450. I asked my haematologist and he said I couldn’t get a prize for the highest as he had seen a 600! 
Anyway , 5 months on it is back to normal and I’m doing fine on Imatinib 400. I get joint and muscle pains but someone gave me an exercise bike and I find if I just spend 20 minutes on that each day it really helps with symptoms. 
Judith

Hey Jon,

I was just like you where it happened out of no where. I was at 450k WBC. I was admitted to MUSC in Charleston, SC. They took very good care to me and put me right away on Sprycel 100mg. I didn't notice any side effects until +3 weeks. I am currently on 70mg do to not able to keep up with some of the side effect in the work place. Personally fatigued was and still a struggle for me. I am going on 7 months this month and all my levels are down to a normal state with A BCR able Test at the end of this month. Stay way from anything with grape fruit if on Sprycel. 

My advice:

Have a good support group to talk to.

I have tried taking Vitamins gummys but it is in your blood so no real affect.

Try to get as much exercise as you can.

Did you put any sperm in the bank if you are planing on having more kids? I was not told this and me and my fiance are getting married this year and wanted to start a family soon and since with Cemo we have to wait at lease 5 years of start talking about having kids with my doctor.

 

Hey Jon,

I was just like you where it happened out of no where. I was at 450k WBC. I was admitted to MUSC in Charleston, SC. They took very good care to me and put me right away on Sprycel 100mg. I didn't notice any side effects until +3 weeks. I am currently on 70mg do to not able to keep up with some of the side effect in the work place. Personally fatigued was and still a struggle for me. I am going on 7 months this month and all my levels are down to a normal state with A BCR able Test at the end of this month. Stay way from anything with grape fruit if on Sprycel. 

My advice:

Have a good support group to talk to.

I have tried taking Vitamins gummys but it is in your blood so no real affect.

Try to get as much exercise as you can.

Did you put any sperm in the bank if you are planing on having more kids? I was not told this and me and my fiance are getting married this year and wanted to start a family soon and since with Cemo we have to wait at lease 5 years of start talking about having kids with my doctor.

 

All, 

Thanks for all the great info.  I'll def be asking a lot of questions at my next appt 23DEC.  

Another few questions for the group:

1) Immunity system - Is it compromised at all? Either with CML or Sprycel?

2) Alcohol - Still ok to drink a beer or two at night?

3) Is the PCR test always 100% the first time since its a baseline?

4) I'm going to ask for a Vitamin D test at my next appointment.  Do I need to ask for any other tests also?

5) CBD & THC - ok to use?

Best, 

Jon

Hi Jon,

In regard to your questions:

1) Immunity system - Is it compromised at all? Either with CML or Sprycel?

Sprycel can cause immunosuppression, especially at higher doses.  If you read the article below, don't be alarmed.  The patients in the study weren't typical CML patients and were taking a dose of 140mg.  They had failed imatinib and had more advanced CML than most of us when we were first diagnosed.

Immunosuppression and atypical infections in CML patients treated with dasatinib at 140 mg daily:   https://sci-hub.se/10.1111/j.1365-2362.2009.02206.x

2) Alcohol - Still ok to drink a beer or two at night?

Should be fine.

3) Is the PCR test always 100% the first time since its a baseline?

Quite often, sometimes it's even higher than 100% if the PCR score has been converted to International Standard (IS).  If I remember correctly, mine was 118% IS at diagnosis.

4) I'm going to ask for a Vitamin D test at my next appointment.  Do I need to ask for any other tests also?

They should be checking your magnesium and potassium levels.

5) CBD & THC - ok to use?

I've read of CML patients who do and they seem to be doing okay.

 

Good luck with your appointment on the 23rd!

Kirk

A bit of expansion on (3) ... the PCR test can be a range of values at diagnosis. Some people are as low as around 10% and others well above 100% (mine was over 300%).

So your PCR result at diagnosis is whatever number it is. However, for all treatment milestones we assume / pretend the patient was at 100% at diagnosis. This is because responses are measured as log drops (divided by 10) from the baseline of 100%. 1 log drop from 100% is 10%, 2 is 1% etc. So to ensure consistency between patients, we need to work off a common number. Otherwise what is MR3 (MMR) for one person would be a different number for another and that just wouldn’t work at all.

100% is not an absolute number either. It’s a ratio, and one that was come up with through the original clinical trial for imatinib called IRIS. They basically took the average PCR result from the patients in the trial and called that 100%. It needed to be something, so that was as good as anything  so your “average” CML patient will be diagnosed at circa 100%, on average.

David. 

Update:

FISH results came back. PCR @ 82% baseline.

Also noted:

Variant Philadelphia chromosome translocations, such as the 3-way, 9;22;11 translocation, are observed in up to 10% of cases of chronic myelogenous leukemina (CML). It is known from molecular studies that variant Philadelphia translocations nevertheless result in BCR/ABL fusion, as in the classic form of the translocation. There is no accepted clinical or prognostic difference associated with variant translocations.

Spoke to ONC, and he made feel better that although this is rare, it doesn’t affect the CML diagnosis and expects the TKI to continue to do its thing. Does anyone else have this? Or can speak to it? Thanks!

Hey Jon,

Looks like we're starting out on this cml journey about the same time.
Research and asking a lot of questions has really helped me. Also I started a notebook so I didn't lose papers.

In what I've read about leukemia, I've decided that I am extremely blessed that it is CML.
So much progress has been made in the last 15 years. What was fatal is now treatable!!

I am so greatful to God that I have an incredible husband to walk this journey with me.

It sounds like you have a sound supporter in your kick ass wife. If you can, take her to your doctor appts. It helps with processing all the new information.

Take care
Nancy

Hi Jon

Prior to the TKI era, 3-way translocations were associated were poorer outcomes.  However, studies have demonstrated that these complex variant Philly translocations in general respond similarly to TKIs compared with the normal 9,22 translocation.  You will just have to see how your next FISH results look and consider switching TKIs if your response is not adequate.   Fortunately we have an arsenal of choices and each can have varying effect on the bcr-abl kinase. 

Good news. Baseline PCR .1537% (16Nov). Wasn’t 82%. That was a misunderstanding with a phone call w my Doc. Went in for my 1 mo. Follow up and WBC dropped from 25 (26 Nov) to 3.6 (19 Dec). RBC 5.54 to 4.9. Platelets 195 to 106. Seem to be trending in the right direction. Bloodwork again in 2 weeks, follow up in 1 mo. Anything else I should be concerned about? Questions for next appt? Doc said he’d consider lowering the dosage at 3 mo. mark.

Hi Jon,

I could be wrong, but I think it's more likely that your BCR-ABL1 (p210 transcript) as measured by PCR was 82%.  Could it be that the .1537% was the p190 transcript?

Edit:  It's not bad news if your PCR score was 82% - p210.  It just means the majority of your BCR-ABL1 transcripts are the "normal" type.  I think they test for it at diagnosis just to make sure that your CML is typical versus atypical.  For example:  my PCR scores at diagnosis were p210 = 88.57% (raw score, not International Standard) and p190 = 0.01%.

It looks like good news on your blood work too.  Your platelets are a little below normal.  Mine also went down in the early days of treatment to a low of 97 before they came back to the normal range.

Merry Christmas!

Kirk

Kirk,

See below.  I may have misunderstood.  

INTERPRETATION:
Fluorescence in situ hybridization (FISH) analysis was performed on this patient's specimen using DNA probes for BCR/ABL1. Two hundred interphase
nuclei were examined and revealed a BCR/ABL1 translocation in a total of 82.0% of nuclei.

-----

Doc said below that my PCR is .1537%.  Does anyone know what the %01 352.6411 is?

e13a2 (b2a2) transcript Comment: % 01 352.6411 ----- e14a2 (b3a2) transcript COMMENT % 01 <0.0032 % (sensitivity limit of assay) ----- e1a2 transcript 0.1537 % 01 

Interpretation: COMMENT 01 POSITIVE for the BCR-ABL1 e13a2 (b2a2, p210) and e1a2 (p190) fusion transcripts.

On another note, should be getting a second opinion to Hopkins to back up what Navy Oncologists is doing to give me the warm and fuzzy.

Good morning Jon,

You may want to ask your doc for hard copies of your reports as it may make it easier for you to figure out what's what.  I think my doctor's pathologist provides good reports.  I'm not saying I understand much of it.  As I've learned more about CML, the reports have become more informative.

I'll try to show what my initial PCR report showed:

Assay TypeDetection ParametersResult without conversion to ISInternational Scale (IS) ResultMajor Breakpoint (M-bcr)p210 transcript b3a2 and b2a2Positive (88.570%)Positive (118.683%)Minor Breakpoint (m-bcr)p190 transcript e1a2Positive (0.01%)N/A

The full title of the report with the table above was:  BCR-ABL1 Fusion Transcript Analysis by PCR

My initial FISH BCR-ABL1 result was 93% of 200 nuclei examined.  So, pretty similar to yours.

The actual PCR numbers at diagnosis don't matter too much.  The doctor is mainly trying to confirm a CML diagnosis with the various tests.  The numbers become more important as your treatment progresses.  For the BCR-ABL1 tests, a downward trajectory is what you're looking for.

Edit:  For some reason the table I made shows up in the editor but not when I go back after saving the post.  I'll try it here without the fancy table thing.

Assay Type                                   Detection Parameters        Result without conversion to IS       International Scale (IS) Result

Major Breakpoint (M-bcr)    p210 transcript b3a2 and b2a2    Positive (88.570%)                          Positive (118.683%)

Minor Breakpoint (m-bcr)    p190 transcript e1a2                        Positive (0.01%)                          N/A

 

All,

I spoke to my ONC to get clarification on my PCR results.  See below. 

Is it odd/common or not a big deal that the e14a2 was undetectable on 16Nov but detectable 19Dec, and e1a2 was detectable on 16Nov and not on 19 Dec?  Seemed a little odd to me. 

I guess the good news is e13a2 has dropped dramatically within 34 days.  Have my 3 mo. PCR test mid-Feb.

16NOV19
e13a2 (b2a2) transcript: 352.6411%
e14a2 (b3a2) transcript: <0.0032% (sensitivity limit of assay)
e1a2 transcript: 0.1537%

19DEC19
e13a2 (b2a2) transcript: 39.8034%
e14a2 (b3a2) transcript: 0.7128%
e1a2 transcript: <0.0032 % (sensitivity limit of assay)

 

Thanks!

Is it odd/common or not a big deal that the e14a2 was undetectable on 16Nov but detectable 19Dec, and e1a2 was detectable on 16Nov and not on 19 Dec?

I don't think it's a big deal.  After thinking about it for a while, maybe what's happening is that there was so much e13a2 in your first sample that it masked what little e14a2 that was there.  Now that you've had a large reduction in e13a2 it's possible that the e14a2 is detectable even though it really hasn't increased.  They are both the p210 transcript and are the most common breakpoints for CML.

The e1a2 breakpoint is characteristic of Ph-positive acute lymphoblastic leukemia but is sometimes detected with a CML diagnosis.

Hi, Jon,

I have a caveat to the THC/CBD use. I have an issue with my heel that has caused painful achilles tendonitis and bursitis. I'd heard about the anti-inflammatory properties of CBD so I thought I would give it a try. After a week I decided I probably should have checked out any interactions with Sprycel.

What I found was, if you shouldn't eat grapefruit with the drug you are taking you might not want to use CBD or THC either. Sprycel, THC and CBD are all metabolized in the liver with cyp3a4. Sprycel is a time dependent inhibitor of cyp3a4 as is CBD and may decrease clearance of the drug thereby increasing drug levels in the body. THC is an inducer.

Is this a big deal with Sprycel?  Maybe not but since so much is not known about CBD I decided not to take chances.

https://profofpot.com/cyp3a4-genetics-cannabinoid-metabolism/

I understood about every 5th word of the following study, maybe you'll do better. smiley I mostly just checked out the charts and references to metabolism and cyp enzymes.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6473892/#!po=16.6667

I only take 20mg of Sprycel every other day due to chronic pleural effusion. If 100mg of Sprycel knocks your BCR/ABL level down quickly, as I expect it will, I would talk to your doc about slowly reducing your dose of Sprycel to try to avoid pleural effusion.

Good luck, Jon!

Pat

Can someone explain to me how you can have a PCR greater than 100%?

When the International Scale (IS) for measurement of BCR-ABL1 transcripts was developed they tested 30 patient samples for the level of BCR-ABL1 present.  Then they set 100% IS as the median level from that testing.

So if your PCR score is above 100% IS you have an above average expression of BCR-ABL1.

Here's an article about the subject:  https://www.tandfonline.com/doi/full/10.1080/10428194.2016.1190974

P.S. -  If I got any or all of this wrong, please feel free to correct me.  cool

The only little nugget I can add to what RC Kirk has already said is that this standard was developed as part of a clinical trial called IRIS, which was the main clinical trial that proved how much better imatinib (then named STI571) was than interferon.

David.

Awesome.  Thanks for the explanation. 

Just got my blood work back. 

- WBC went from 3.6 to 3.4.  Platelets are back to a normal range.

-----------------

Haven't had the opportunity to speak to the ONC yet.

Any cause for concern?

- Alanine Aminotransferase is 81 (range 21-72).  Did some research and it seems it probably be the TKI on my liver?  It was at a normal level 3-4 weeks prior.  Does that make sense?

- ABS Neutrophils and ABS Segmented Neutrophils were slightly lower than normal range.

 

 

Hi all, excited to let everyone know after 83 days on sprycel that my PCR went from 352% to .9%!!!!!

Cool. You're almost in the zone (not the danger zone).

Thanks Scuba! My ONC mentioned this is a EMR. Is there data that shows long term response IRT EMR?