Uptravi

I’ve just started a brand-new Pulmonary Arterial Hypertension medication called Uptravi (selexipag). It’s replacing the horrible horrible Tyvaso that I’ve been on since 2011.

Tyvaso is inhaled through a nebulizer four times a day. My dose was 18 breaths (4 times a day) and it was awful awful awful. Every breath made me cough ferociously, and the whole process left me exhausted, drained, and headachy every time. Up till now it was better than the alternative, which would be an infused medication delivered via a permanent IV pump with all the associated risks — infection, blood clots, etc. I /really/ didn’t want to deal with that, so I stuck with the Tyvaso.

Uptravi, which is the same basic type of medication as Tyvaso but delivered via tablet, was just approved by the FDA in December of 2015 and went on the market in January 2016. It took a long time for my insurance company to approve it, and for everyone to give me permission to start it, but two days ago, Friday March 11, I finally took my last Tyvaso treatment and started my new Uptravi!

Uptravi has a lot of terrible side effects, naturally, and can be hard to tolerate, so there’s a very slow titration process. They sent a nurse to my house to walk me through it and give me all the warnings in person. I started at 200mcg morning and evening (12 hours apart). Then after a week they’ll send a nurse out to check on me. If everything is good, I’ll go up to 400mcg for a week. And so on, until I reach 1600mcg “or the highest tolerated dose”.

I had some bad nausea the first night but no side effects since then. It’s so amazing to finally get away from Tyvaso and I’m incredibly grateful to the scientists who keep inventing new treatments for my terrible debilitating rare incurable illness.

They’re not really sure if Uptravi will make me feel better (every PAH patient reacts differently to medication), but it should slow the worsening of my condition. And I won’t be coughing all day!

In other medication news, I’ve switched from Tracleer to the newer, less-potentially-deadly Opsumit and also started Digoxin to help my heart beat slower and stronger. Also I’ve gone up to 20mg of Lexapro and it’s amazing. I don’t feel anxious and depressed all the time anymore! If you have anxiety and/or depression, please don’t be afraid to try some meds in addition to talking with a really good therapist.

I recorded another chapter of “Young Folks’ History of Germany” this morning :)

Category: Blog 15 comments »

15 Responses to “Uptravi”

  1. Mike Wilson

    Here’s hoping that the uptravi works out well! You face so many problems with so much good spirit, it seems like you deserve a break.

    Since you mentioned that you’ve got PAH, I’ve read a lot about it. The human body is strange; so many systems working together to keep us alive. A tiny imbalance or tiny problem or tiny infection can cause so many weird effects. I suppose I shouldn’t ask, but: nobody else in your family has this (or if they have, you’ve never mentioned it). Do you have you any idea how it is that you’ve ended up with PAH?

    Mike :)

  2. kara

    Thanks, Mike!

    No one else in my family has PAH. Mine is caused by a heart defect (ASD) that was not diagnosed until I got sick with Graves’ Disease (a thyroid condition) and they noticed some other strange stuff going on. People with that kind of heart defect can get PAH, but I don’t think it’s known why that is. It’s a rare and mysterious disease, as I’m sure you’ve discovered. :)

    I’m doing well on the Uptravi so far. I’m still having some mild gastro-intestinal issues but no nausea or headaches, or any of the other common side effects. It’s such a low dose that it’s probably not therapeutic yet, but I don’t even care because no longer having to deal with Tyvaso is so wonderful! The energy I used to expend on coughing and feeing generally ill four times a day is available for other things. I no longer have to plan my day around my Tyvaso treatments and their aftermath. Such a relief.

  3. Christy

    Thanks for posting your experiences with Uptravi. I also have PAH and am in the process of downtitrating from Ventavis (also inhaled) to Uptravi. I am currently on 400mcg twice a day. I started one week after you.

    I’m looking forward to being free from the inhaled treatments. I didn’t have a lot of coughing issues like you described, but having to plan my day around them is difficult as I am very active and work.

    My biggest side effect right now is the joint pain. Not sure if it’s from the med or from increased workouts, so please post if you are having issues with this.

  4. kara

    Hi Christy,

    You must be doing really well! I can’t imagine being active and working out. I get out of breath just doing a load of laundry.

    I understand that joint pain is a common side effect of Uptravi, but I haven’t experienced it. My only side effect is gastro-intestinal oddness. (Mostly just bloating this week) The nurse will come today to check on me, and I hope to increase to 600mcg tonight.

    I stopped Tyvaso cold-turkey (simply could not stand it any more) so I’ve been even more breathless than usual. Hoping that will get better once I reach a therapeutic dose of Uptravi.

    Take care!

  5. Rayna

    I am currently taking 600mg twice a day. The back ache and muscle ache is no picnic. I tried heating pad and hot showers but just tried Aspercreme and it helps alot

  6. kara

    Thanks for the tip, Rayna. I’ve got a strange sore shoulder now so I’ll try Aspercreme. I’ve been on 800 for almost a month now. I was having terrible headaches so decided to delay up-titration for a while. Probably going up to 1000 on Monday… wish me luck.

  7. linda

    Hi. Just found this. I too have PAH and my condition deteriorated rapidly from January through March. Then I started sildenafil + opsumit+ uptravi. I have titrated up to 1400 and will go up to 1600 tomorrow. It has made a really fantastic difference. Side effects: for me the gastro-intestinal set in days 2-4 for each upgrade, then abated. Unpleasant. Now it’s continual high back and lower hip aches, so much so that I can hardly sit on a chair for very long. Thanks for the reference to Aspercreme. So hang in there, it’s better than the other options.

  8. kara

    Hi Linda,

    I’m up to 1000mcg and will go up to 1200 in a couple of weeks. Ever since 600 I’ve been waiting about a month between up-titrations because the side effects are bad and I like to feel good for a week or two before I increase again.

    I’ve got joint pain now (knees and hands, ouch, and aspercreme doesn’t help), and facial flushing in addition to all the other stuff (see some of my more recent blog posts).

    I haven’t noticed any benefits yet but I’m glad to hear that you’re seeing some at those higher doses, so maybe I’ll get there someday and also feel better!

    Yep I’m determined to make Uptravi work for me. I’m never going back to Tyvaso and I’m not eager for IV meds.

    You hang in there, too! :)

  9. William Wall

    Hi,
    I new to this forum, but I too have PAH. I was diagnosed around 2years ago by my Doctor at Duke. I have been doing well under the combo of Adcirca and Tyvaso. My 6 min walk has improved and my abnormal Right Side Heart is back to near normal. But like a lot of you, Tyvaso had a lung side effect that is nearly unbearable. The cough and lung irritation would take nearly a hour before you could get up and do anything. My Dr is now trying to switch me from Tyvaso to Uptravi. Up to 600 has gone well but now I am at 800, I am having the headache side effect( I think). In also came down with a sinus cold and that could be the source. The cold is much better but I still seem to have nagging headache in the sinus area. Those of you having headaches, is it like a sinus type headache?? What over the counter meds seem to work best?? I want to move on to higher doses and would like to know others experiences. I can sacrifice a Minor headache compared to the Tyvaso side effects. Do after some time, your headache get better?? Let me know your experiences and I will share mine,
    Thanks

  10. kara

    Hi William,

    This is not a forum, it is just my own personal blog. But I’m happy to give you my insight.

    I am now at 1400mcg Uptravi and ready to start 1600 on Monday. I wait a month after every up-titration because I experience very bad side effects that last for a couple of weeks. I get every side effect that is possible; limb/joint pain and headaches are the most bothersome. I get two type of headaches. One is, I suppose, what I would call a “normal” headache. Not crippling, just uncomfortable. I also have gotten way more migraines since starting Uptravi — usually one after each up-titration. I have not found any OTC meds that will help either kind of headache. Sleep helps the “normal” kind, and the migraines go away after 24-36 hours.

    I encourage you to keep trying higher doses! The side effects seem to become tolerable after a few weeks, so maybe see if your doctor will let you wait a month each time, the way I do.

    Good luck!

  11. Tammy

    I ache all over , I take 600 mg uptravi in morning and 600 in the evening.
    Every part if my body hurts , restless leg syndrome all over , I hate this , driving me crazy.
    Thanks
    Also take letaris 10
    Sildenafil 20 mg 3 times a day

  12. kara

    That’s awful, Tammy. Here’s hoping you feel better soon. I’m at 1600mcg now and it’s been really tough, but worth it because I do sometimes feel better when I don’t feel terrible, if you know what I mean.

  13. Tammy

    I decided that uptravi was not for me at this time, I suppose my PAH is controlled well enough with my letairis and sildenafil .
    I was in the hospital for a right heart cath,( my 4th ) with the results from that , my lung doctor felt I needed to start the FloLan therapy, I was transported to a different hospital where they were going to begin this new drug, I am not a fan of hospital stays and questioned if this was something I really needed, after a couple of days I decided to leave against medical advice, and about a month later, my doctor recommended the uptravi. I started with one pill in the morning and one at night , had the nurse come to my home after the first week , I had slight headache , a little sore muscles but nothing I couldn’t handle, the second and third weeks became really bad and all I could do is lay in bed in pain, depressed and frustrated , I had a couple of percocets 5 mg , from a vein procedure I recently had, and I took one , it helped , for the whole day and most the night , until it wore off. I was at 800 mg twice a day , the aches and pains all over got worse, I will not live life in bed, miserable from head to toe, I called my nurse told her I could not tolerate this, and I stopped taking it and I feel fine, short of breath , but not in pain, I am hoping I never need to start this again, it was a horrible feeling , I am glad that it helps others , I just know it was not for me, YET.
    Well wishes and thanks for taking time to care.

  14. Christy

    Kara,
    I posted last March about switching to Uptravi and I was wondering how you have done on it. I was able to go up to the maximum dose of 1600 twice a da. However, in February of this year I passed out after climbing stairs. My right heart cath showed my pressures have increased 25% after switching off ventavis. My doctor wanted to put me on an Infused medicine but I begged to go back on the ventavis since I’ve done so well. I’m about to make the switch back in a week or so. I hope you and others have had better success than me, but all indications for me is that Uptravi wasn’t effective enough.

  15. kara

    Same story here. I managed to get up to 1400, and I felt a lot better November through January. I was able to turn my nighttime o2 down to 2 lpm and barely use it in the daytime at all. For the last couple of months, though, I’ve noticed a steep decline. My SOB is much much worse, and I had to turn my o2 up to 4 lpm at night and use it a lot during the day, too. My last 6-minute walk was terrible. Next step: Remodulin. :( I’m about to write a new post about all this…


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