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    • #15393
      Vanessa Vaile

        Hello all,

        I’m in my fourth week on Uptravi and would like to hear from Forum members who have been through the 8 step titration protocol. What were your experiences? What helped you deal with reactions?

        During week 1, I noticed where it making a difference but since then reactions have been overshadowing those perceptions. By now I have experienced most of side effects listed. Except for congestion and muscle/joint pain, not have been everyday or on the same days. This week, I’m spending another week step 3, taking 3 200 mg tablets 2x/day.

        Vanessa Vaile
        Yuma,, Colorado

      • #15394

          Hi Vanessa,

          I started Uptravi in March 2016. At the time I was taking Revatio (20 mg X 4, 3 times daily), Tyvaso (10 breaths, 4 x daily), Opsumit (10 mg daily), and my PAH was advancing. The plan was to eliminate Tyvaso by titrating down while titrating up on Uptravi. When I got to 800 mcg X 2 daily of Uptravi, I experienced headaches and loose stool. I was taking Advil for the headaches. I powered on to 1,000 mcg X 2 daily and the headaches were intense. We held at 1,000 mcg x 2 daily for 2 weeks. 2 Advil every 4 hours could not control headaches. Also experienced muscle pain in my legs at night and the bottoms of my feet were quiet sore. We moved back to 800 mcg with little relief. Ultimately, we discontinued Uptravi and went to infused Remodulin via Hickman port. Symptoms were difficult at this point, felt faint with one set of stairs. Infused Remodulin gave me symptom relief quickly and it has since August of 2016. Today it’s Revato, Opsumit and Remodulin with good quality of life and symptom control, although my Pulmonary pressures are still quiet high.

          • #15395
            Diana Procopio

              am also taking . I had titrated to 4 2 times a day, but had severe musclepain and weakness. I went to my GP about this. Uptravi caused a flare-up of my Polymyalgia rheunatica, so my Pulmonologist dropped me down to 3 2 times a day and my PC put me on prednisone and I’m feeling much better .

              • #15411

                It’s always the worse when it interferes or causes a flare up of another condition. And when doctors are told about this , usually it’s like “here try this other medication to help balance the side effects” . Deep inside I wish I didn’t have to balance ANY at ALL. Had to get that rant off my chest lol!

            • #15412

              I’m so glad to hear that you are managing better now and it seems like the doctors have a better grasp on the regimen for you that gives you a better quality of life. I know it must have been a headache (literally) in your case to keep titrating and trying new things. I have heard from others that headaches and joint pain were terrible. Were you given medication for this type of pain or was it just a “let your body adjust” deal?

          • #15410

            Hi Vanessa,
            I’m sorry that I can’t relate to this post but I know how hard it can be to titrate dosages to find the right one. It can be physically and emotionally so demanding. I’m so glad other forum members have (and will) be able to reach out to you in regards to this.

          • #15419
            Vanessa Vaile

              Thanks all, These insights add to my understanding about the process and what to expect — or, like the sore bottoms of my feet, sharing a reaction not on the list. This also confirms that I might not be able to tolerate Uptravi. From all the cautions and following up, I suspected as much. Right now, I’m in my no step week until Monday morning and feeling much better than last week.

              I’m also on Taldaldil and Letairis, which I started a month before the Uptravi. My GP is not particularly helpful unless you count the robocall from the local hospital’s pain management program trying to enroll me. The Accredo pharma nurses have been a good source of suggestions for dealing with reaction.

              Trucking on. Next week awaits…

              • #15421

                Best of luck to you next week Vanesssa. I know sometimes the primary care doctors can be so hard to get in touch with. Does your pulmonary team or primary care team have an online portal? Sometimes you can email them online through the portal. If you are unsure, definitely ask about it at your next appointment it has been a good tool for me especially when I don’t feel like listening to the whole “please hold , you are the 10th caller…(instruments playing in the background) lol I’m glad you have found some help though through Accredo in getting your questions answered. I’d much rather know what to expect when it comes to medications. All the best to you! Keep us updated!

            • #15702
              Vanessa Vaile

                A late — overdue — Uptravi follow up. I’m almost to the end of titration,just one more “step” or that I may spread over two weeks because I have an unavoidable but sure to be tiring day in the middle of next week that’s sure to exacerbate reactions.

                I definitely identity with Michael’s “sore feet” description and call it “Uptravi legs” ~ aching leg muscles and joints, with hot, sore soles.

                It’s been rough but not intolerable and looks like I’m going to make the optimal 1600/2x/day dose. A nurse from my PH team calls once a week, and I can call them directly. The Accredo Uptravi desk, staffed with experienced PH nurses, checks in weekly too and is available 24/7.

                More later…

                • #15705

                  Hi Vanessa,
                  It’s great to hear that your team is so on top of your care. It’s great to have a group of doctors, nurses, and staff that are accessible and call to check in. I hope that you are able to save up some energy for your “tiring day” that you have coming up and that you are able to get some much needed rest and self care afterwards ! Keep us updated on how that goes. I’m sure others can relate to your “uptravi legs” and may even get a similar reaction to medications that are vessel dilators. I know when I was on medication that acted as a vasodilator, I experienced soreness and swelling of my calves to the point where I would sit down and immediately feel the discomfort. I remember feeling it more with rest for some reason (probably because I’d be thinking about it more) . I hope you continue to feel okay though and I hope the rest of this titration goes well for you.

              • #15916
                Vanessa Vaile

                  Quick note ~ made it to day 5 of final week titration and on full dose, almost close enough to call although not side effect free. Reactions have been mostly manageable and only intermittently intolerable. I’ll want a few weeks on optimum dose to be sure though — don’t expect to be fully side effect free but do expect (hope) benefits to outweigh them. Exercise tolerance and with it, mobility and activity, are notably improved. I haven’t been able to test stairs yet since there are none where I live — on the list. Day trips with long car rides, rare and usually to Denver, are still quite draining but more manageable.

                  Good (telemedicine) support from PH and pharma teams has made a huge difference and, of course, not having to get up and out to cope with work or household on on worst days. Neither my primary nor the local rural hospital have been involved to any significant degree. I count myself very lucky not to have had any emergency. With that possibility in mind, I made sure Uptravi was on my medications list there.

                  • #15935
                    Kathleen Sheffer

                      Thank you for the update, Vanessa! I’m so glad you are seeing improvements and I expect the side effects will decrease as your body adjusts to a stable dose. Which side effects were intolerable for you and what did you do to get through them?

                  • #15924

                    It sounds like you are managing all of this so well and are trying to keep a positive attitude through the titration. I know it can’t be easy but I’m sure it will all be worth it. I hope that when you are able to do stairs you can do them with more ease! Seeing improvements is enough to drive you to keep pushing forward! Keep up the great work. I’m so glad your PH team is responsive and that we now have telemedicine to keep track of our progress and have more frequent “visits” online. Keep us posted on how things are going in the coming weeks.

                  • #16377
                    Vanessa Vaile

                      I’m glad the breathing is better. Good thing you’re not still taking ambrisenten. It gets better, Kevin. As the dose increases, you’ll have side effects, but you’ll also have good days. Take care not to overdo on those. I think my 600 week was the worst for head aches and leg pain. I stayed at 600 for two weeks before increasing to 800, then half steps up after that if side effects were still strong at the end of the week. My second week on full 1600 dose, I took a day in the middle of the week with a half step back.

                      Try taking something for your headache before you take Uptravi. My nausea was mild. Tums with meds and meals did the trick for me.

                      You mentioned taking other meds. It might help to take once a day meds in the middle of the day. I started doing that with ambrisenten and tadalafil.

                      Do you notice the weather making a difference?

                    • #16424
                      Jen Cueva

                        Hi guys! Sorry I’m just joining this Uptravi conversation. I had a hospital visit recently and been recovering.

                        As far as the Uptravi, I’m on 1600mcg 2xday , the max dose. I know when I first started I didn’t get too many bad side effects until I got to about the 600mcg 2xday, that may be because I was switched from Orenitram and that was awful for me and my body just wasn’t liking that one, lol 😂

                        Vanessa, glad you made it to the full titration! I know as my doc has told me if and when the side effects become too much, you may need to drop down a little just for awhile to see if you body can readjust! I’m hoping by now you’re seeing results and side effects are not as bad !

                        Kevin, I definitely know it’s frustrating and scary at times with these new meds! They aren’t like taking an asapirin or Tylenol by any means! I’m hoping you can adjust but remember each of our bodies are so unique and adjust different. As far as any reactions, please speak with the speciality pharmacist as they are very good to educate and catch any issues. I know we have to be careful with our meds, even with vitamins and OTC medications!

                        I definitely continue to have side effects from my Uptravi , the sore and heavy muscles and jaw pain, which always likes to surprise me, lol 😂 but I may go days without it affecting me .
                        But in my opinion and for me the Uptravi with the heavy dosages of Revatio I take as well as other meds , see,s to be working well for my PH!

                        Best of luck to you both !

                        • #16433

                          That’s awesome that you found a combo of meds that worked for you even if it must have been a long haul to get the right combo and the titration! It sounds like your doctors and YOU were right on top of the side effects that you were experiencing and they listened and lowered the doses when you needed it. Hopefully now you are seeing more improvements. Sorry to hear about your recent hospital visit and I hope you are on the mend now ! Thinking of you!

                        • #16462
                          Vanessa Vaile

                            Jen, describing intermittently recurring side effects as “surprised by” nails it more expressively and in fewer words. I’d rather be surprised by jaw pain than diarrhea, not that there’s a “choose your surprise” option, but remind myself that it’s probably a quick drive-by, not hanging around or back for the count. “Oh no, it’s back,” was my reaction to the first “surprised by.”

                            • #16466

                              It’s like your “friendly reminder” just stopping in to say “hey, I’m BAAAACK” lol! But it’s more like the annoying person that you don’t want around but can’t be avoided hahahaha. Like a pop up on a computer screen that just gets in the way. I wish there was a “choose the symptom” option! That would be wonderful!

                            • #16470
                              Jen Cueva

                                Thanks Brittany, been thinking of you as well with all of your medical issues going on the last few months! 🤗

                                Vanessa, I’m glad you are feeling some results! Yep, those side effects as well as PH likes to remind us they are still there as I say, lol

                                Kevin, I understand your frustration, please knowse are here for you ! I agree with Brittany, if you’re feeling worse with the meds, I would not increase , I would be contacting my PH team and hopefully they can even get you in to discuss your concerns and maybe able to do more testing to better help you.

                              • #16473

                                Thank you for all you support you show our members, Jen! We are so lucky to have you here!

                          • #16446
                            Vanessa Vaile

                              Oops. Tried replying to @ksmith610here before heading out for my evening walk but checked when I returned from evening walk and didn’t see it posted.

                              The short version, Kevin: do check out the polycythemia with blood tests. Hang in there, we’re here for you. fwiw I remember feeling discouraged right at the point in titration where you are now. Is there a PH pharmaceutical support line with the VA?

                            • #16481
                              Jen Cueva

                                Thanks Brittany ! I really enjoy the forums and I learn from you all and feel the su as well !

                                • #16500

                                  It makes my day (now morning) to hear that Jen! I’m so glad we have a space like this for each other !

                              • #18610

                                  Well I am about to embark on the additional medicine adventure I am on opsimit I had mild side effects but all dissipated in a month. Sildenafil didn’t work for me. They mentioned Uptravi I have low regular blood pressure, I thought Uptravi might be the one but now I don’t know😔

                                  • #18613
                                    Jen Cueva

                                      Good luck with the new meds, Ally. I’m on Uptravi currently. I’m also on Revatio(Sildenafil), sorry it didn’t work for you. Thankfully, today we have more med choices. I’m optimistic that your doctors will find a combination that will work with your body. We are all so different and often have to try a few meds before we find a good fit.

                                      Please keep us posted. I’ve been on most of the oral meds, so I’m familiar with many of them.

                                    • #18619

                                      In my personal experience with trying to get medications (not even all heart or lung related), it is hard for me to get something that works and doesn’t leave me with debilitating symptoms of low blood pressure. like you, my blood pressure is pretty low at baseline (90/60 is my normal) and that’s WITHOUT any medications. The beta blockers for my heart that I am currently on do NOT mess with my blood pressure reading, it works more on the heart rate. I know that my doctors have always been hesitant with trying anything that has a direct impact on the vascular system and blood pressure because my system is so thrown off anyways even without the medication. That’s something that has always been hard for me and my doctors to find the right balance of things. Even a simple ant anxiety med can throw my nervous system completely in shut down mode and I just SLEEP FOR HOURS

                                      • #18694
                                        Jen Cueva

                                          Brittany, our ” normal” or baseline blood pressure runs about the same. We definitely have to be careful trying new PH medications.

                                      • #18632
                                        Colleen Steele

                                          Ally, finding the best combination therapy for PH is a challenge since each patient reacts differently to treatment. Unfortunately side effects is the reality no matter what you are taking but in addition to finding the right treatment, it is also a matter of finding the right dosage. It’s a game of trial and error but hopefully in time you and your doctor will find what is best for you. Please keep us update!

                                      • #18629

                                          Thank you it’s hard for me to trust doctors but real people like y’all I feel I can trust I will keep y’all posted.

                                          • #18639

                                            I’m so glad that you’re a member of this community. It really makes a difference when you have people in your life who “get it” and a safe space to reach out to others and know you have a community of those who understand on a personal level. It’s why I love talking with everyone here so much. Please continue to update us on how you’re doing.

                                          • #18650
                                            Jen Cueva

                                              Yes, this forum is an awesome group of people. I enjoy the support as we learn from one another. Please do keep us updated.

                                              As Colleen mentioned, medications are often trial and error. Thankfully, we have many options available.

                                              • #18712

                                                So true, Jen! The encouragement is so great to see here 🙂 Especially for those that are new to a PH diagnosis, just hearing from others in a positive and supportive community is beneficial. And you’re right that because we have low blood pressure at baseline, we really need to pay attention to the way medication can impact this. If it gets too low I just become a zombie it feels like! I will start a new topic on blood pressure today !

                                          • #19772

                                              Ok guys I am to the point of not being able to walk 10 feet without resting I wear oxygen a lot more and side effects I can deal with if I am working for an end to zero quality of life. Do y’all have the disease progression in check with the med combos they said in all reality I will need a triple combo. I would do just about anything to be able to go outside and work on my landscape pond and my flowers. Or go to Walmart or the grocery store without worrying about SOB. I would love to live some kind of a somewhat normal life! I just feel like that’s come and gone because I had a bout a month or 2 of feeling pretty good on Opsumit to not even having a couple of good days a month it’s been over a month since I had a good day. Just need some reassurance there’s a light at the end THANKS GUYS

                                              • #19779

                                                hey Ally,
                                                I know so much of this is so overwhelming. especially the part you mentioned about not feeling like you are doing “normal” things. When I am stuck in bed or am having a really hard day physically or mentally, I like to make some type of checklist that involves the things I CAN do as a “break” during the day. I always feel like I’m not exacly “normal” especially if I am wearing my oxygen. Without it on, people wouldn’t even be able to tell what was really going on with my health. Keep advocating for yourself , get a second opinion of 5th opinion if you have to! You can do this and get through all of these “stuck” moments.

                                              • #19782
                                                Colleen Steele

                                                  Ally, you are not alone. We truly understand what you are going through and our hearts go out to you! I agree with Brittany, consider seeking the opinion of another doctor(s). Also, treatments can be a bit of trial and error. What works best for one might not work as well for another, and some people benefit from being in more than one treatment. My son was on many. It took a while to find not only the perfect combination for him, but also the right dosages.

                                                  Also, like Brittany, I am a believer in list making, or journaling. Every day little moments are as important as the big ones. If you washed some dishes, read a book, watching a good movie, enjoyed a phone conversation with a friend, etc., write them down. Sometimes we are so busy with what we wish we could do that we overlook what we have done.

                                                  I do understand the loss that you are feeling though and I hope that turns around for you.

                                                  • #19791

                                                    I love your idea of writing down the things that you HAVE done during the day even after the day is over. This really helps you to pause and reflect on what you were able to do. If you are going to make a goal journal for the day just to have some sort of schedule for your day then I would make things into your schedule that you KNOW you can actually do. Some “safer” things that may not make you so tired. I know that I personally work better and feel better when I have some type of schedule to follow and have my day timed out .

                                              • #19801

                                                  Yes just to know there are people who struggle like I do is in a weird way comforting because that way I don’t feel like the universe is singling me out but no seriously none of my family really understand or my friends either they think if I see more doctors or bug them they will fix me but we all know this is just as challenging for the doctors too. Like we are all learning about this together. Thank y’all for all the wonderful encouragement and just being there❤️

                                                  • #19804

                                                    you’re right Ally! It’s so good to be able to have each other here and learn from one another just as much as others learn from us. I think that sharing our experiences and talking about them so openly will even get doctors to look into the connections of some of these things. That’s what sparked one of my doctors to do a research study for PH and women who are pre-menopausal. It was all very interesting.

                                                • #19803

                                                    Yes I am very schedule oriented and I have a quick temper so sometimes when I am really mad at my doctors instead of being not so nice to them I write them a letter and say whatever I want and it’s a good release and they never know how upset I am.

                                                    • #19810

                                                      I have done this with many of my doctors too. I have left a couple primary care doctors before I found the one that I have now that I really do LOVE ! But before that there were a lot of “winners” LOL (total sarcasm) . I wrote letters about being diagnosed with PH and my heart condition and gave them a lesson in dismising patients as being “neurotic” or being “too aware of my health” .. When did self advocacy and awareness ever become a bad thing !? They could have learned a thing or two from me and they chose not to. SHAMEFUL . I’m glad you write too. It really is a good release.

                                                  • #19812

                                                      Yes and some of the doctors refused to see me because they said I already knew what I had well I might have but I don’t have access to a pharmacy lol.

                                                    • #19813

                                                        I was a veterinarian technician for years and my salary wasn’t near as much as a human nurse but I had to figure out what was wrong with an animal who can’t talk to me but I promise not one animal ever died on my watch due to negligence. Or not caring but about a paycheck! I also made sure they had their prescriptions and I bugged the doctor for them!!

                                                        • #19815

                                                          Some doctors just refuse to take things as a learning experience or they don’t have the time for it. But they NEED to make the time for it and if they don’t then that’s when we advocate for ourselves, let them know what they have done wrong to hopefully keep it from happening again, and move on to someone better and the best care we deserve.

                                                        • #19924
                                                          Jen Cueva

                                                            Hi Ally,
                                                            Sorry, I’m catching up here, so a few days behind. You are certainly not alone. Brittany and Colleen both offer some awesome support.

                                                            I can relate to you as I was a nurse prior to PH and some doctors that I’ve seen, just have no clue. I’ve had some who were unconcerned and brushed me off. It’s tough when you’re new to this PH stuff, it’s overwhelming. Having a good doctor and team can lessen some of the added stress that PH puts on us. Thankfully, I have a supportive PH team, now.

                                                            I have written emails to doctors at times to voice my concerns. I also was always a very schedule-oriented person. It’s been tough the last 14 years but doable. Learning new ways to do things and live your ” new normal” often requires trial and error. I’m glad that you found us here.

                                                            • #19933

                                                              So true Jen, it really is a lot of trial and error with medications, treatments, therapies etc. The list goes on and on and it is never a “one size fits all” way of treatment. I feel like everyone’s bodies just react so differently to all types of medications and treatments. That must be so challenging and time consuming for the doctors. Individualized treatment plans are so important ! I wish there was just a wonder drug out there that worked well for everyone but unfortunately, that’s not the case.

                                                            • #19942
                                                              Jen Cueva

                                                                I agree, Brittany. I know that often my PH team is challenged as what works for some, does not always work for me. That is the tough part. AS you mention, it would be nice if we had one “miracle “ drug that would help everyone. I can imagine our medical teams spend hours researching just for one patient.

                                                              • #19952

                                                                I’m sure they do! Only the best of the best really take the individual time with all patients and that’s exactly what we deserve. I used to feel guilty for going to see doctors because I hated to be the one to tell them “this doesn’t work for me”. But, that is part of their job and I have also learned along the way that the top doctors love a challenge and love to see any benefit from treatment at all. Even if it’s small, it’s still significant to them.

                                                              • #19960
                                                                Jen Cueva

                                                                  I have never fir into this “patient box”. It seems most of my issues are different from most “usual” patients. I, too find that doctors wanting to take the challenge speaks volumes.

                                                                  What is a “usual” patient, anyway? I sorta dislike this term as much as “normal”.

                                                                • #19970

                                                                  I totally get it. The amount of times I have heard “you’re so complex” or
                                                                  “there’s so much going on” LOL like THANKS DOC I get it hahaha!

                                                                • #19991
                                                                  Jen Cueva

                                                                    I had the same PH Doc for years. Dr. C, always told me that, “ I am not in the medical books”. Thanks, LOL – I know you can relate on so many levels. I am grateful for good doctors who are not afraid to “tackle” us. Hehe

                                                              • #19818

                                                                  Yes exactly I am an over achiever or used to be so I have always went above and beyond. I take pride in whatever I do. Some doctors just want a paycheck and does less than minimum and should have their license revoked I had 20/20 vision my whole life well due to 2 different sets of doctors misdiagnosing me I am blind in one eye and it has painful glaucoma in it not to mention it looks weird

                                                                  • #19831

                                                                    I’m so sorry to hear about your misdiagnosis, Ally. That is so hard to be able to trust a professional again after something like that. I know for me it was really hard to gain back trust in my medical team after so many others have let me down in the past. I would never wish my pain on anyone but there are some who just REALLY don’t know what it’s like. Especially if it’s “invisible illness” . I have been treated like a hypochondriac or as being paranoid in the past and I HATE when my feelings are dismissed.

                                                                • #19819

                                                                    Here’s what it really boils down to. If they felt as bad as we do they wouldn’t drag their feet for a second but they have never felt what we feel either!

                                                                  • #19893
                                                                    Jimi Mcintosh

                                                                      Thank you Brittany, you made me laugh today,it is great to vent our frustrations. Let us all have PH Vent
                                                                      Day on Sunday and start next week refreshed and ready to handle whatever comes our way.

                                                                      I find that the side effects are sometimes worse than the disease. I was once hospitalized and they didn’t know how to get my meds or allow me to take my own, headaches, sudden anxiety, depression and intense muscle pains.
                                                                      Because I had missed 3 days on most of my PH MEDS, I had to start over with titrations. I have found that some drugs do not work for everyone, severe
                                                                      Side effects, call the prescriber immediately, most side effects go away or become manageable in 2-3 weeks. The Advil, Aspirin and some other over the counter meds can cause ulcer, bleeding and even more side effects.

                                                                      Have a serious conversation with your providers, the drug manufacturers and your pharmacist. You have to advocate for yourself, drug overdoses and interactions can be fatal. I had a 2nd
                                                                      Blood clot that it’s symptoms were being hidden by a new med, had my wife not insisted on a doppler, I would have
                                                                      Stroked out in a few hours.

                                                                      • #19896

                                                                        Wow Jimi!
                                                                        I’m so glad that your wife knew to catch that. You’re right that some of the medications that we are on can sometimes mask some serious side effects. The symptoms are so hard to deal with. Some of my hardest symptoms are pain , because pain management can really be such a double edged sword. When the pain is really severe I try my hardest not to take stronger pain medication but when my body needs it, it just feels like every single part of me is tensed up and I also can’t live in that state of physical health. So I almost am forced to take them even when they can give side effects like dizziness, light headed etc.

                                                                        I think everyone needs that vent day for themselves to just throw themselves the pity party we deserve! I once saw a meme online that said “I do NOT have the time for the mental breakdown I DESERVE ! and I had to laugh because it was SO TRUE hahaha! Hope that made you laugh too.

                                                                    • #19931
                                                                      Jimi Mcintosh

                                                                        I am in constant pain from knee surgery, 2 hip replacements and 2 herniated disc in lower back. The storms and humidity causes flare-ups. I am on 325/10 Percocet or oxycodone, I manage it by taking 1 in am and pm, I can take up to 4 /day. I use mind control and tricking my brain to dull the pain. Worst day missed PH meds, COPD MEDS and late taking pain meds.

                                                                        I feel that I am in a decline with the PH, and this heat and humidity is hard.
                                                                        I realize that each change in meds is getting me closer to
                                                                        Meds that I cannot afford and that may not provide any relief. On top
                                                                        Of everything else the DEA And some drug stores treat you like an addict, when you get refills. Walgreen has refused to tell you when your meds are available, no info over the phone or by text, they will provide no assistance in locating one of their pharmacy’s that has enough meds to fill the script. Walmart and a few others will only give you 7 days of pain pills when your script says 30 days. I am so pissed that I am about to file suite under the disability act. Some doctors are not know-legible of the side effects when prescribing, and do not trust your depictions.

                                                                        I hate that Kim K has Lupus, but on the plus side she will be able to help enact changes in treatment and speedy approval of new drugs. With PH, no one knows that we exist and that the numbers of sufferers is increasing. If I hear one more person say”you do not look sick, why are you taking a handicap parking space”, I am going to lose it. We need more media coverage and a national voic

                                                                        • #19937

                                                                          So true, Jimi!
                                                                          You’re right about needing more national attention. As much as I would hate for a celebrity to have this condition, I know that sometimes it takes that famous voice to get the things that we need and the media and attention that we deserve. Congenital heart disease is the number one birth defect and the number one cause of deaths in young children, yet other things get way more funding and more attention. Last year, Jimmy Kimmel’s son was diagnosed with congenital heart disease and I saw how much more attention it was getting in the media. It pains me that it takes someone famous to spread awareness like this and raise their voices, but at the same time AT LEAST it was GETTING attention for once.

                                                                          I am sorry you continue to suffer with pain and symptoms of these conditions. It is horrible to be treated like you have an addiction or to not be told certain information or to have them withold info from you about your treatment. It’s unfair and I have seen it firsthand. I am NOT the one that will be abusing medications when I am in excruciating pain. My doctors that really know me, know that if anything I choose NOT to take them and suffer with the pain until it gets to the point of being unbearable. I do this because I have that fear of addiction so ingrained in me. That fear ends up doing more damage than taking them as prescribed. I totally get your frustration and where you’re coming from.

                                                                        • #19966
                                                                          Colleen Steele

                                                                            Jimi, your body has really been through the ringer. I am so sorry that you deal with the level of pain that you do every day. I have had my share of frustrations with pharmacies as well, especially Walgreens. What people often neglect to realize is not just the fact the PH patients are very sick but that they also carry so much worry with them. PH is very much both a physical and mental disease. It would be so helpful if it could receive more media coverage.

                                                                            • #19971

                                                                              You’re so right Colleen!
                                                                              My PH doctor always reminds me of the importance of making sure I am taking care of my mental health too. I feel like sometimes I need these reminders because my mental health can feel neglected when I am going through a physically difficult time but ironically this is when I need the most help.

                                                                        • #19985
                                                                          Muriel S.

                                                                            Hello, I am new to this forum, but have been reading topics covered in the past for awhile. I have been taking letaris, Adcirca and Tyvaso for several years. My Pulmonologist has suggested changing to Uptravi to replace the Tyvaso. He hasn’t explained much about the process for starting Uptravi except for headaches, so after reading the information on the forum regarding starting Uptravi I’m getting nervous. Also, I don’t really have a PHteam for support and to help me. Tomorrow I have an appointment with my Pulmonologist and I think I’d better get some more information on how this works. Thanks to everyone for sharing your experiences.

                                                                            • #19987

                                                                              Hi Muriel,
                                                                              Like everything else with PH, I’m sure that people’s reactions and side effects from this medication really varies from person to person. There is no “one size fits all” approach to treating this disease and it very much depends on the underlying condition that caused it in the first place. Do they know what is causing your PH or what may be contributing to it? I really hope that you can have a productive conversation with your doctor tomorrow. If you are worried about the side effects from the medication I would specifically ask them what they plan on doing to treat side effects if and when they arise. Maybe they will titrate you to a correct dosage pretty gradually. I hope that you get all your questions answered tomorrow and please keep us posted on the forums on how everything goes with your care team. Do you have a PH specialist in your area or are you comfortable with your pulmonary doctor treating you for this?

                                                                            • #20004
                                                                              Colleen Steele

                                                                                Muriel, I’m so glad you found our forums and joined. Uptravi is a treatment my son was never on so I don’t have knowledge to share but as you can see, there are many members ready to chime in and give you support. I hope your appointment goes well and that you return home feeling that your questions were well answered. Update us when you can.

                                                                              • #20042
                                                                                Jen Cueva

                                                                                  Welcome, Muriel,
                                                                                  I hope that your appointment goes well. You have been given some valuable tips from others here in this group. I wanted to add to that.

                                                                                  When I was transitioning to Uptravi, I had a home health nurse come out. She educated myself and family on Uptravi as she answered some questions on dosages, etc. She brought along some informational handouts to keep as well to keep for further references. I also spoke in great lengths before starting Uptravi with a Pharmacist at my Specialty Pharmacy. She was a helpful resource also.

                                                                                  As others mention, we react differently to Uptravi. Titration is usually slow and depends on each patient. Having the meds ready for side effects is helpful as @rockie436 mentions. I take Neurontin and Ultram for leg and muscle pains. But, if Tylenol works for you, certainly try that first. You may also need meds for constipation and/or diarrhea. I had those meds ready as I transitioned from Orenitram which was tougher on me as far as side effects than Uptravi was.

                                                                                  Please feel free to ask specific questions after your appointment.

                                                                                  • #20043

                                                                                    This is really good advice! I feel like some people don’t know the option about speaking to the pharmacy staff about these medications and side effects to look out for. I have said on other forum topics and I will say it again, pharmacists are sometimes even MORE knowledgeable about medications, side effects, medication interactions than the doctors are and will be able to look at ALL your medications you are on to try to figure out why someone is experiencing worsening symptoms. They are a great resource and it sounds like they really helped you a lot too as well as your home nurse. These options ARE available to us but sometimes we have to speak up and ask for these services or request them.

                                                                              • #19996
                                                                                Rockefeline Denis

                                                                                  Hi Muriel,
                                                                                  I’ve been on uptravi for a year now. It’s a great medication for your PAH but there’s nothing you can really do to alleviate the side effects. I experienced all the side effects listed however with time you get a break from time to time. Jaws pain is the worse. You need Tylenol to help with muscle pain,Imodium for diarrhea, stool softener for when you’re constipated. Once you have everything right there you’re good to start your Uptravi. I’m on Adempas, opsumit, and uptravi. Uptravi is really the one that helps me breathe.
                                                                                  Don’t be anxious, know that it is for your own benefit.
                                                                                  Listen to your body, when you reach the phase you cannot handle all these side effects and the migraine is too much to bear; tell your pulmonologist that you would like to stay on x-mcg.
                                                                                  Good luck!

                                                                                  • #19997

                                                                                    This is great advice. Thank you for sharing your experience on this medication. I know that for me, personally, it is comforting to hear what other people are going through and the side effects that they experience because it helps me prepare for what might be to come. I never like to be caught off guard and not know what to expect. Having that expectation of “there might be side effects that happen” helps so others can ask the appropriate questions ahead of time and hopefully be aware to help their doctors stay right on top of things ! Really great feedback. Thanks again!

                                                                                • #36281
                                                                                  Jen Cueva

                                                                                    Hi @kbogetz, check this post out, and feel free to ask questions and post comments about your experience with Uptravi.

                                                                                  • #37699
                                                                                    Jen Cueva

                                                                                      Hi @amit-ramsinghani, I wanted to tag you here to see others’ experiences with Uptravi (selexipag). Hopefully, by reading about others’ experiences with this medication, you will find support for your mother’s PH.

                                                                                    • #16039
                                                                                      Vanessa Vaile


                                                                                        I’m just finishing my second week on the full Uptravi/selexipag dose. My system is still adjusting. I expect full adjustment to take awhile.

                                                                                        My most difficult reactions were flushing, muscle and joint pain, and (mostly morning) headaches. I had mild nausea, mostly just gassy, and a few episodes of diarrhea. The jaw thing too and increased congestion. Reactions were spread out, at different times, often on different days. Some lasted longer and were more difficult than others, none intolerable. A lot of variation is possible.

                                                                                        What helps: eat first, take something for pain and/or digestive tract issues about 1/2 hour before taking selexipag. I keep Tums handy. Moving around helps with moderate muscle and pain. Sometimes you can sleep through worse ones.

                                                                                        Wish I’d known about weed; sheesh, I’m in Colorado.

                                                                                        Any time our reactions feel like too much, take a half step on the titration or stay an extra week on a level before upping your dosage.

                                                                                        This is off the top of my head. I’ll write more later. Keep us posted. More questions, just ask.

                                                                                      • #16192
                                                                                        Vanessa Vaile

                                                                                          Kevin, I’ve been thinking about your nose on selexipag. How is it doing. I know what you mean about ambrisentan and congestion because I’m on it too. I suspected a large part of my congestion problem with selexipag was the combination with ambrisentan — plus other side effects magnified by other meds.

                                                                                          Other than a bad day this week that overdoing and bad air quality might have had something to do with, my system continues to adjust — including less congestion. That much and using a CPAP have made sleeping through the night difficult, uninterrupted sleep rare until the past two nights.

                                                                                          Reactions seem to vary widely but mine didn’t really hit hard until the second week. I started feeling benefits before the reactions hit. Then, except for occasional days, they outweighed the benefits. All weeks there were good days.

                                                                                        • #16201

                                                                                          Hey Vanessa,
                                                                                          I’m so glad that you are noticing some good days within your weeks and I’m sure that makes it easier to manage with knowing that there are better days ahead and something to look forward to once the side effects start to get less severe. The congestion must be awful. Are you able to get better sleep now? It does take awhile to get used to the mask and get used to waking up and falling asleep with something on your face, but you WILL get used to that to, it just takes time, like everything else. I wish things could be more instantaneous for us, but unfortunately, even with me lately it always seems like I am in some type of “waiting period” and waiting for the next thing.

                                                                                        • #16382

                                                                                          Hi kevin,
                                                                                          I totally get the frustration of the side effects. Nobody wants a splitting headache and nausea. Luckily those symptoms are supposed to be ones that resolve as your body adjust to the medication. I have never been on this particular medication so hopefully members that have will be able to chime in. But I would definitely be vocal about the symptoms that you are experiencing and since they should be “temporary” maybe the doctors that you have can prescribe you some medication for migraines or for the nausea that works (seems like they are giving you the zofran and I’m wondering if that’s helping). Glad that you are seeing some improvements with the breathing though and are noticing differences in your sleep at night ! That’s HUGE !!! Try not to get too frustrated with the bad side effects and overlook the things that the medications are actually doing for you. Be sure to write down BOTH the positive and the negative and see if your doctors can work with you on the negative stuff! YOU GOT THIS!

                                                                                        • #16442
                                                                                          Vanessa Vaile

                                                                                            Kevin, this will be a short because I’m heading out for a walk — determined to make the most of good weather and finally being able to go more without the effort knocking me out for the rest of the week. I’ll write again when I get back,

                                                                                            Hang in there. Like Brittany says, you are not alone here.

                                                                                            My third and fourth weeks were the worst — my system adjusting to selixipag wore me out so much it was impossible see improvement. The side effects were because the med was doing what it’s supposed to — increase circulation. I was exhausted, aching from my head to my feet, and slept a lot.

                                                                                            But definitely check that out and get blood work done for polycythemia. fwiw other high RBC conditions show up in PH research case studies.


                                                                                          • #16383

                                                                                            Thanks for sharing all of that with us Kevin and Vanessa, thank you for the words of encouragement because I know that you recently wen through the titration process and would be a good person to reach out to for this because of your positive words!
                                                                                            The best place I go when I’m wondering about side effects is the pharmacist. They will be able to take a look at all your medications. I would bring your list to the pharmacy if you can and speak directly with them. They may need to look things over and you may need to go back that day or just get a phonecall back from them once they have had the chance to see about interactions with the medications. I would specifically look at the interaction between the two medications that seemed to cause your headaches ! The pharacists know best when it comes to this stuff!

                                                                                          • #16434

                                                                                            I just direct messaged you because I am thinking about you and thinking about your health and mental health right now. Please know that this is a community of support and that we are all here for you. I know how t feels when it seems like everything is too much and just beyond your control and how overwhelming and isolating that can feel. I can relate and want you to know you can reach out to me any time you need to. I hope you can find some type of hope to hang onto and even just some type of hope knowing that there is a whole community of us who is here to support you and who care about you.

                                                                                          • #16450

                                                                                            We are always here for that support for you Kevin. I would definitely bring this up to the doctors about how the medications are making you feel and not just try to “tough it out” and push through it. You know your body best. That’s the one thing that we do have control over is speaking up and letting them know when something is wrong! YOU GOT THIS!

                                                                                          • #16465
                                                                                            Vanessa Vaile

                                                                                              @ksmith610, I asked about the VA because that is how you get your Selixipag. The VA has its own distribution system and purchases pharmaceuticals directly. Actilion distributes through Accredo, which has a PH Desk and a competent intake/follow up procedure for Uptravi/Selixipag titration. Licensed nurses, experienced with PH and often certified in the specialty, staff the desk. A nurse called me every week during titration, and the support line is available 24/7.

                                                                                              I wish you had that support, but if VA does not have anything comparable anywhere in its Byzantine bureaucracy (my son has been on VA disability for 30+ years), then maybe Actelion does. Call the Tufts PH Center too. The PH team might (imo should) have a direct number you can call so you don’t have to go through the switchboard.

                                                                                            • #18689
                                                                                              Colleen Steele

                                                                                                Kevin, my son can sympathize with the vasodilation headaches. He was on Flolan and always had to titrate very slowly or else he would be down for days with a migraine. It’s that way for him with several other things too. He is post transplant now and occasionally has to have IVIG infusions. They have to go so slow on him that it takes 15 hours so he always has to be admitted. His doctor would often say in regard to his sensitivity to treatments and medications, “The good news is he is extremely responsive and the bad news is, he is extremely responsive.”

                                                                                                I’m glad you figured out a way to avoid the headaches but continue treatment.

                                                                                              • #18695
                                                                                                Jen Cueva

                                                                                                  Kevin, that’s a great tip. I know with some of my medications, I spread them out, too. I usually wait at least an hour, if possible. When I took a once a day medication, my doctor suggested I take it at bedtime to help alleviate some side effects.

                                                                                                • #18706

                                                                                                  I can sympathize with BOTH of you !! The vasodilation headaches are absolutely horrific. I remember taking medications that also worked on the blood vessels. I was on one awhile ago, I think it was called midodrine to help keep the vessels open with fluid and less floppy. But It caused terrible headaches and my blood pressure plummeted on it. I could feel pulsing in my ears and just had to stop. I was on such a small dose too. Like your son, I am super sensitive to anything that has to do with the vascular system or any medication that has an effect on the nervous system (which unfortunately most do!)

                                                                                                • #18758
                                                                                                  Colleen Steele

                                                                                                    Kevin, being that sensitive is a mixed bag. Side effects tend to be worse but, as my son’s doctor explained, it also can mean that your body really reacts well to the treatments. Some people can take medications and it have not effect on them at all. Being highly sensitive just meant you have to a slow approach to treatment and eventually you will get to the desired dose. It’s a painful challenge though and I feel for you.

                                                                                                  • #18762

                                                                                                    I understand how frustrating it can be. Some of my doctors will say “it’s just a baby dose of the medication” but for me, it could set off my entire system. Even the BABY doses I sometimes need to cut in half or cut into quarters depending on how my body reacts to it. Some people can pass through titrations of medications with flying colors, I am definitely the exact opposite and find the physical toll really challenging. What is the hardest part about the symptoms for you, Kevin?

                                                                                                  • #18792
                                                                                                    Jen Cueva

                                                                                                      Those ” baby doses” are often what I get m too. I am in a ” baby dose” of Ativan and too often the doctors want to increase it. I’m good at my dosage.

                                                                                                      @ksmith610, that jaw pain is actually a well known and experienced side effect from many of the PH meds. It’s often the first bite or two of food and is over quickly. It is definitely easy to know when you have it. I still experience it at times but I notice if I eat with my medications it’s not as bad. It was worse titrating up.

                                                                                                    • #18853
                                                                                                      Colleen Steele

                                                                                                        Best of luck with the pulmonary rehab, Kevin. Let us know how it is going for you and if it’s helping in any way.

                                                                                                      • #18861
                                                                                                        Jen Cueva

                                                                                                          Kevin, that’s so true! Accepting the decrease in our physical activity is difficult. I hope that your rehab will help some. Best of luck to you, don’t push yourself!

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