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Severe Pulmonary Hypertension


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#21 Susan61

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Posted 26 June 2014 - 06:21 PM

Pam:  Lets keep praying for each other.  ITs God's will not ours for what we go through.  I went for my Carotid Ultrasound, and then had to wear a 24 hour holter monitor.  Carotid was Okay, but my heart flatlined well I was asleep and then started to beat again.  They found numerous stops the whole 24 hours I wore the monitor. I had to go to Deborah Hospital on Monday after I went to see the doctor to get a pacemaker put in.  Everyone said I was a miracle that I survived.  I was walking around like I was on a cloud, very weak and dizzy.  They put a pacemaker in on Monday, and it failed.  Then they redid me on Tuesday.  I am home, but they do not know if there are any other problems in the heart.  I am still very weak and scared.

     Hang in there Pam and believe something good is going to happen.  Try to keep your Faith that I know you have, as I am doing the same thing.

    Unbelievable what we are going through.  I will be in constant prayer for your lungs and heart to stabilize.  You might prove that you can come off your TKI permanently as everyone is trying to do.  GOD BLESS YOU

LOVE AND PRAYERS

susan



#22 Melanie

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Posted 26 June 2014 - 06:36 PM

Pam, so very sorry for your circumstances. My heart goes out to you for all you're going through and have to endure. My family will keep you in our prayers. Hopefully you'll be cuddling with those puppies soon!

It's a reality check for us and even though the TKI's are life savers, these are dangerous drugs for a very serious disease. Thanks for the warnings, the advice, and updates. Hang in there and hopefully you can take some comfort knowing you are cared for and we are praying for you.


Dx - 05/2011; PCR: 15.04; Fish: 87% Slow responder due to pancytopenia. Current - Bosulif - Nov: 2012, Mar 2016 lowered to 300 mg. 07/16 back to 400 mg. Clinical trial drug, Promacta, Feb 2013, for low Platelets.
CyCR - Aug 2014, Positive for 1 chromosome Sep 2015. PCR: 12.77 in Oct, 2012 to 0.04 (MDA) in Mar, 2016. 4/2016 - 0.126 (Local lab (IS); 05/2016 - 0.195 (local); 6/2016 - 0.07 (MDA); 7/2016 - 0.03 (local) 9/13/2016 - 0.16 (MDA); 9/26/2016 - 0.31 (MDA); 11/2016 - 0.012 (local); 01/2017 - 0.24 (MDA); 04/2017 - 0.09 (MDA); Cytogenetics show der(1:7)(q10;p10)7 chromosome mutation. Repeat of Sep 2015. PCR - 6/2017- 0.035 (local); 10/2017- 0.02 (MDA)

#23 pammartin

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Posted 26 June 2014 - 07:51 PM

I am still a firm believer starting the TKI's on such a high level is not good for the person, the body, pr enhances treatment unless one would be in distress.  It is so odd, I am not on a med for the pulmonary hypertension reading just like the TKI beginnings.  The side effects are just as brutal but the dosage is slowly raised as the body adjusts and as soon as the response is shown, a leveling out usually begins. 

From the beginning of my journey with CML hitting the disease as hard as possible and taking a major toll on the body is how most of the patients are treated.  I am hoping in the future, unless in major distress, the TKI's are started in low doses and response noted as low as the TKI controls the CML.  It is challenging, because it seems I walked  this journey with CML such a short time ago.

Please Note: One thing that cannot be totally proved or discredited, although my situation is perfect for the Sprycel to cause the Pulmonary Hypertension, this does not verify or guarantee  it is to blame.  As many have stated throughout their CML journey, take control of your treatment, if you do not agree, challenge the methods, and if a doctor is not willing to try a lower dose if all the signs reveal response is in place, perhaps it is time to find a second opinion.  I had a second heart cath today, confirming the original diagnosis, but I today's test included the bicycle stress test taken on one's back when the heart and lungs are damaged.  Three years ago I had a regular stress test on a treadmill and surpassed my goal for the test.  Today laying on my back I could not begin to meet the goal set because of my lack of oxygen.  I do not want to sound like the grim reaper and no disease is easy, but please pay attention to your body, I went for 2 months thinking I had a respiratory infection, I am not saying ending the Sprycel would have helped or not, but I believe a faster diagnosis and treatment may have left me with additional lung functions that may never return.



#24 pammartin

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Posted 26 June 2014 - 08:00 PM

Trey,

A question,; there are a few studies out there using Gleevec for CML but as a side treatment for Pulmonary Hypertension.  The Pitt doctors say you never go back and want me to begin the process of gaining access to the next drug above Sprycel.  The studies in Germany seem to be valid and there is some documentation the TKI helps the PH.

Any thoughts on the idea of going 'backwards' from Sprycel to Gleevec, and the potential for helping with the PH? I have signed up for two studies here at UPMC, they are documenting the frequency of PH, relation to Sprycel, and records pertaining to this subject.

Curious about your thoughts.

Thanks,

Pam



#25 CarolineCookie

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Posted 13 July 2014 - 04:43 PM

Yikes, I am so sorry Pam that you are having to deal with yet ANOTHER life threatening situation.  Seems completely unfair.  

 

And timely for me. 

 

I just stopped Sprycel 20 mg for possible PAH, found that there was some small damage, but not enough to stop taking sprycel.  I will, now, be hyper vigilent if any of the symptoms come back.

 

thank you for sharing.

 

hugs,

Caroline



#26 pammartin

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Posted 17 July 2014 - 10:44 AM

Update, I have been off Sprycel since May, I have found oncologist who is willing to try Gleevec, although I had to sit through a lenghtly lecture about the side effects. Each drug has its own unique and shared negative effects, in many ways I am trading one set for another. The oncologist will not start me on med till after my appointment with cardiologist on August 11. I have one more dosage increase for the Remodulin then a few weeks to see how effective the first target dose is. I have measurable increases with energy and breathing, although I am still struggling with the picc lines and pump. In time I will receive a port but there is concern about my compromised immune and open lines. In two weeks I will have BCR/ABL before we begin treatment and see if I have lost response over the past few months. I urge anyone who shows potential for complications with any of the TKI's to be very careful. Had I not ignored symptoms for over six months I would not have the damage I currently face. Thank you all for your support and comments. I appreciate feedback and sharing of your experiences.

#27 Guest_Alexandra Arnott_*

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Posted 17 July 2014 - 02:41 PM

How are you doing Pam? I wasn't able to post for ages and ages but was so sorry to see that you are having such a hard time.

Alex x

#28 Trey

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Posted 17 July 2014 - 03:39 PM

Pam,

Gleevec should help the PAH issue, but as I recall (new website wiped out the ability to find your previous posts easily) your drug response has not been very good.  Gleevec may not be the right drug to treat your CML, but I don't know.  Tasigna may be a better drug for you to switch to.  Again, I don't have enough of your history available.



#29 pammartin

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Posted 18 July 2014 - 09:45 AM

Trey,

You are correct, my beginning was difficult with Sprycel. I took 150 mg, almost unheard of at the time. My platelets were the problem, all other lab results were fairly unremarkable. For whatever reason I began to respond and within a few weeks my platelets went from almost 2 million to 50,000. I then had to take a month break and returned to Sprycel at 100 mg. I have been in response since then. My last BCR/ABL was in May 2014. The oncologist mentioned Tasigna also, he noted it is also being used for PH. Oct 7 2011 was my original diagnosis and I believe I had my first negative BCR/ABL early in 2012. I can't get to box holding my records yet, I am limited concerning physical movement and lifting until appointment in August with PH specialist. I appreciate your thoughts and opinions. This has been quite a journey and like the CML it is another lifetime commitment.

#30 pammartin

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Posted 18 July 2014 - 10:10 AM

Alex,
Thank you for asking, I would like to say I am feeling good and things are progressing in a positive manner. Unfortunately, the IV Remodulin has it's own difficulties and like the beginnings of a TKI it is not pleasant. I mix the Remodulin every other day and after 4 weeks of continued increases I have reached my first target goal. Hoping now as my system is not slammed with increase I will level out and begin to feel better. I have allergies to the adhesive for Picc line bandages, we are on the fourth brand, attempting to find one my skin will tolerate. I believe the emotional factor is the hardest; CML brought life changes, this new disease brings even more. I am looking for positives and trying to stay focused on the fact I can breathe, I am lucky to be here, and in many ways life will improve. I hope you are well and life is peaceful for you and your family. Thanks again!
Pam

#31 Pin

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Posted 21 July 2014 - 06:20 AM

Pam, I'm so sorry to hear this :( I'm glad that you have reached your first goal with the remodulin. Has your CML been relatively under control at your last test? I still wonder if you needed that 140mg of Sprycel, your response was so good, and very quick, hopefully one of the other drugs will work well for you and not cause too much interference with your other medication and the PH. Please let us know how you are going? I am thinking of you xx Pin


Diagnosed 9 June 2011, Glivec 400mg June 2011-July 2017, Tasigna 600mg July 2017-present (switched due to intolerable side effects, and desire for future cessation attempt).

Commenced monthly testing when MR4.0 lost during 2012.

 

2017: <0.01, <0.01, 0.005 (200mg Glivec, Adelaide) <0.01, 0.001 (new test sensitivity)

2016: <0.01, <0.01, PCRU, 0.002 (Adelaide)

2015: <0.01, <0.01, <0.01, 0.013

2014: PCRU, <0.01, <0.01, <0.01, <0.01

2013: 0.01, 0.014, 0.016, 0.026, 0.041, <0.01, <0.01 

2012: <0.01, <0.01, 0.013, 0.032, 0.021

2011: 38.00, 12.00, 0.14


#32 pammartin

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Posted 23 July 2014 - 09:23 PM

Pin!

 

Tried to post three separate times, only to lose the post.  Keeping this one to one line, hoping it completes the posting process.   



#33 pammartin

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Posted 30 July 2014 - 10:58 PM

Pin,
I am to go for BCR/ABL test this week. My car has been in shop for weeks and still there, always something! Last test in May was still undetectable but showed a rise in numbers. Not enough to be concerned over and I had labs pulled every day for three weeks while in-patient. They all looked good. About 3 weeks now since last ones drawn. I have been off Sprycel since May. Hoping to get test completed then appoinments with PH specialist/oncologist/primary care in next two weeks. I want picc line removed and port if this is how I have to live. I am not looking forward to both meds together, they each have unique side effects that will be ugly combined, the TKI seems to fade, the PH drug continues to cause symptoms. Today, I have enough energy to do small tasks and no longer gasping for air. I am lucky and very thankful. Please keep in touch!
Take Care
Pam

#34 pegetha

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Posted 01 August 2014 - 01:12 PM

Pam, I know I'm not part of the CML forum, but I wanted to thank you for your advice.  When I stumbled across this topic, I stopped and read it, and your words -- as well as some of your symptoms -- struck a chord.  Instead of a TKI, I'm taking a JKI (Janus kinase inhibitor) called Jakafi, for my myelofibrosis.  Within the last couple of months, I've started experiencing frequent heart palpitations, shortness of breath along with the palpitations, dizziness, and my energy level is in the toilet.  Although it probably isn't caused by pulmonary hypertension, nevertheless, your words of wisdom caused me to take it more seriously.  (I also don't know whether or not it has anything to do with the Jakafi, by the way.)  I first went to my pcp, who did an X-ray.  No fluid in the lungs, but results showed "hyperinflation of the lungs compatible with COPD" (I've never been a smoker, so go figure) and also "atherosclerotic cardiovascular disease" (my cholesterol is always in the normal to low-normal range, so, again, go figure).  The pcp sent me to a cardio doc, who is now running a bunch of tests to attempt to find out what is causing all of the above.  Bottom line, Pam, I might not have started down this path had I not read your words.  I don't know what the docs will find, but I just wanted to say, thank you from the bottom of my (apparently flawed) heart!

Pegetha



#35 pammartin

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Posted 01 August 2014 - 07:19 PM

Pegetha,
I wish you the best on your journey. We have chatted via Tex's posts if memory serves. I am sorry about your health but glad my post caught your attention and you are seeking treatment. One of the greatest aspects of this site is the ability to help others. Please keep me posted, you are in my thoughts and prayers. Take care
Pam

#36 pegetha

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Posted 03 August 2014 - 12:02 PM

Thanks, Pam.  I appreciate that you're always thinking of other people, in spite of the hell you're going through.  As I said, I've followed your story in this thread, and am horrified at all you're going through.  My prayers to you as well.  We can never get too much prayer!!  You're a strong lady, Pam, so hang in there.



#37 Pin

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Posted 03 August 2014 - 06:52 PM

Hi Pam,

I have my fingers crossed for you, hopefully you are just holding the bcr-abl at bay so far. I'm glad you are feeling a bit better at the moment, it's good that you have a bit more energy too. I am feeling pretty dodgy right now, going through a rough patch I guess. I have terrible tooth pain at the moment, I think the acid reflux is causing all sorts of problems, as my teeth are so sensitive now, and just ache all the time. On top of that, I recently had some dental work done and it has left me with a very sensitive tooth with nerve pain when it gets touched by anything. I am hoping to go back and get it fixed, hopefully it wont be too bad or get worse. It's nice to know that you are helping people on here like Pegatha, you are such a kind person :)

Let us know how you go with your next test?

Pin xx.


Diagnosed 9 June 2011, Glivec 400mg June 2011-July 2017, Tasigna 600mg July 2017-present (switched due to intolerable side effects, and desire for future cessation attempt).

Commenced monthly testing when MR4.0 lost during 2012.

 

2017: <0.01, <0.01, 0.005 (200mg Glivec, Adelaide) <0.01, 0.001 (new test sensitivity)

2016: <0.01, <0.01, PCRU, 0.002 (Adelaide)

2015: <0.01, <0.01, <0.01, 0.013

2014: PCRU, <0.01, <0.01, <0.01, <0.01

2013: 0.01, 0.014, 0.016, 0.026, 0.041, <0.01, <0.01 

2012: <0.01, <0.01, 0.013, 0.032, 0.021

2011: 38.00, 12.00, 0.14


#38 pammartin

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Posted 14 August 2014 - 01:31 AM

A bit early for a full update but I can't sleep so here I am. The pulmonary hypertension is responding to the remodulin, I am on 20 nanograms per 24 hour period via IV infusion. I am scheduled for a heart cath next Thursday at Presby but the echo on Monday showed internal pressure at 30, it was previously at 100. The heart cath is to confirm the internal pressure, I always become nervous with the procedure and this will be my fourth within two months. According to Dr Simon, he has only treated 9 patients with idiopathic pulomanary hypertension and somehow I got the unwanted award for achieving the worst case of the 9. This is based on my lack of oxygen and my loosing consciousness episodes along with my high internal pressures. Today is my appointment with the oncologist and after a 3 + month break I will be starting either Gleevec or Tasigna. Although there is the possibility I have not maintained my response, my initial labs are excellent so I am looking for positive news. I still have chest pain, racing heart rate, and limited air when I forget and move quickly or hike up the stairs without thinking. We are looking at oral medications for the PH since I am doing well on the IV infusion, all doctors are leery of a picc or port because I have skin issues now, once starting the TKI again they only get worse. I dislike the picc line, that has not changed but my response to the treatment is excellent, so I am thankful for my progress. I will post BCR/ABL results if they are available tomorrow and the results for the heart cath next week. It's all great news, much different from a few months ago. Continued thanks for all the support, advice, and positive feedback.

#39 Marnie

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Posted 14 August 2014 - 06:13 AM

Glad to hear things are looking up.  Thanks for keeping us posted.  Sending good vibes your way!

 

Marnie



#40 hannibellemo

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Posted 15 August 2014 - 06:07 AM

Pam,

 

So glad to hear the good news. I'm in shock that this happened so quickly and I'm keeping you in my thoughts and prayers!

 

Pat


Pat

 

"You can't change the direction of the wind but you can adjust your sails."

DX 12/08; Gleevec 400mg; liver toxicity; Sprycel 100mg.; CCyR 4/10; MMR 8/10; Pleural Effusion 2/12; Sprycel 50mg. Maintaining MMR; 2/15 PCRU; 8/16 drifting in and out of undetected like a wave meeting the shore. Retired 12/23/2016! 18 months of PCRU, most recent at Mayo on 7/25/17 was negative at their new sensitivity reporting of 0.003.<p>





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