July 3, 2020 at 11:34 pm #26116
I have been dealing with pah for about 8yrs now and have had a decent amount of success,as far as walking and so on, In the past 6 months i have gone downhill to the point where i get winded walking across my yard as my right ventricle is not doing so well. My dr has me scheduled to start on remodulin in the next couple weeks. I realize the site pain will be abit rough, but am very interested to find out if people have had there breathing get better and if it is a good drug to get me back to where i was. Any help with this would be greatly appreciated. Pat
July 6, 2020 at 3:32 pm #26129
@patrickb so happy to see you jump write in an post a question. We welcome you to the forums.
My son was on Flolan for 5 years until he received a transplant. Flolan and remodulin are similar but Flolan is a bit more complicated with more responsibility. He did really well on it for all those years and it definitely gave him a better quality of life. That being said, he was on triple therapy – taking oral PH treatments as well.
Are you taking anything else or will Remodulin be your first PH treatment? If it isn’t as effective by itself your doctor might consider adding another medication or two. Side-effects will be challenging but your body will adapt and although they won’t completely disappear, they will ease up. Titrations are inevitable. Your dose and rate will probably get changed a number of times for various reasons. Titrations will cause side-effects too, especially headaches.
I have known many people on Remodulin and it has helped them. You might still need oxygen but you might not feel like you are struggling as much. I think it’s worth a try and I wish you luck. Please keep us posted on how it is going for you.
July 6, 2020 at 11:25 pm #26135
Hi, boy I wish remodulin was my only ph drug. How is your son after the transplant if I may ask? I am currently on uptravi, adempas, and tracleer, my heart and lung pressure have doubled so my dr. wants to put me on the sc form of remodulin hoping this will ease my breathing and pressure. I was on sildenafil but my insurance stopped covering it so the replacement was adempas. been a very interesting almost 8yrs. So we will see what this med does and go from there. Thanks for taking the time to write back. Pat
July 7, 2020 at 2:51 pm #26146V.R. PetersonParticipant
Patrick, does your doctor want you on Remodulin SQ (subcutaneous) or IV (with a port going directly into your heart)? My son was on the SQ, and it kept him alive. The Remodulin SQ worked for him at first, and that was the only medication he was on except for Warfarin. When it stopped working as well, his doctor added Opsumit and then Adempas. Do you mind if I ask if you have CTEPH? Adempas was developed specifically for that form of PH, which is caused by clots and scar tissue in the pulmonary arteries, but they’ve since discovered it works on other forms of PH, as well.
When those three medications stopped working, the doctor told my son that if he didn’t qualify for PTE surgery (to remove the clots and scar tissue in his pulmonary arteries), he would need to go on Remodulin IV, and possibly have a lung transplant. As luck would have it, he did qualify for PTE surgery, and the surgery was successful.
July 7, 2020 at 2:57 pm #26149
@mamabear007 that is a good question I didn’t think to ask. Whenever I think of remodulin or flolan I think IV because subcutaneous wasn’t available when Cullen had PH.
@patrickb my son took Tracleer, that was actually the first medication he was placed on, that and Revatio. I know how complicated it gets being on multiple treatments but hopefully it will be as effective for you as it was my son.
July 7, 2020 at 3:12 pm #26153
I have been on Remodulin for 14 years fir my PAH. My Dr wanted to get aggresive with this disease and put me on remodulin, adcirca and letairis. For 12 years the remodulin was subQ and yes the site pain waz no fun but the remodulin WORKS! My 6 min walks were better and I felt human again.
2 years ago the site pain did get to the point I could no longer handle it and now I have Hickman/central line – no pain, but it a be combersome and a lot more in mixing the meds etc.
If you need some hints how to make subQ a bit more easier and/or how to waterproof up without any trouble let me know and I can fill you in on what worked for me.
Remodulin does work for PAH. Good luck and let me know how you are doing if you get a chance.
July 7, 2020 at 3:47 pm #26156
V.R. I have no idea what CTEPH is so i will assume i dont have that it was never mentioned anyway. my Dr wants me to start the sub contaneous. I am on tracleer, opsumit (uptravi) and adempas. The adempas is new within the last 6 months as i was on sildenafil but my insurance stopped covering that. I am very happy that your son had a successful surgery.
July 7, 2020 at 3:50 pm #26157
Carol, any hints would be deeply appreciated!!! I am glad to hear it works. My breathing is just getting worse because of the pressure increases. My 6mwt was the same as 6 months ago as far as distance but suffered alot more breathing wise to do it lol.
July 7, 2020 at 6:30 pm #26162
Hi again Pat,
On remodulin really helped me with distance and staying power including my 6 min walk.
The device I used first on the subQ was extreemly painful – the silhouette. I switch over to the cloe – much easier and not as painful for me.
I would numb up an hour before inserting the cloe with prescription lidocaine and cover that with over the counter lidocaine, clean off with alcohol swabs then use the swab that has sticky stuff on it to help keep the cloe in place. Make sure you stay 2″ away from your navel. There are very sensitive nerve endings and getting within 2″ can be instantly painful.
I went through all their water proofing stuff and it would tear up my skin and my site would become very red and irritated. I discovered Aqua Cel Foam! Totally water proof easy to put on and take off. I not only showered with it I went swimming and snorkeling with it on. I could leave it on for about 4 days (until I could see it begin to wrinkle on an edge). When the doctor noticed how much better my site looked he made sure the special pharmacy would carry it. It still amazes me that some of the folks at the pharmacy don’t know about it until I ask them to look it up because it is there for people. I used the 4×4″.
I hope this helps Pat. If you have any questions on what or how it went for me, knowing how differnt everyone is, I would be happy to help.
Pat, be safe and take care of yourself.
July 16, 2020 at 2:44 pm #26276Elena RichParticipant
I’m 48 years old and I was diagnosed with Severe Pulmonary Hypertension in March of 2017. I was put on Adcirca & Letairis. In March of 2019, I got a second opinion and my Dr. recommended IV Remodulin, without hesitation.
The decision to move forward with IV Remodulin through a Hickman line was the best decision that I could have made. My quality of life has improved dramatically. I can walk up a flight of stairs without being winded. We are also seeing an improvement in the size of my heart, which is imperative. I know that Remodulin is not only improving my quality of life but it most certainly is extending my life as well. My heart was becoming so badly damaged that aggressive intervention was necessary.
Also, my improved health has alleviated a lot of fatigue. Before Remodulin, I was unable to stay up past about 9pm, it was very difficult. I took a nap every Saturday and Sunday, without fail. Now, I couldn’t tell you the last time I took a nap. I feel that much better!
Having a Hickman line is certainly not ideal but the reward far out-ways the risk of infection as well as the inconvenience of everything that comes along with it. Sleeping and self-care are two things that will never be the same for me. Not having taken a real shower in so long is a drag and getting tangled up in tubing while I sleep totally sucks but it is what it is. No regrets at all. I would do it again in a heartbeat.
I can only hope that Remodulin provides you with similar results and no site pain!
Best of Luck,
July 16, 2020 at 3:36 pm #26277
Pat – I agree with everything Elena said. Remodulin saved my life along with Letairis and Adcirca.
Elena – hint how to have your wonderful shower back. I use Aqua Cel Foam over the Hickman line. Totally waterproof and does not tear up your skin taking tape off. My husband put a hook just on the outside of the shower and I hang the bag with the pump there and take a shower every day. When I get out of the shower I use an alcohol swab to wipe off the connections. I usually do not have to change the Aqua Cel Foam for about 4 days – I leave it on all the time and it keeps my site safe. I use either a 3 x 3″. You can get these from your special pharmacy. They also come in othet sizes 4×4″ etc.
Thinking of you both, stay safe.
July 17, 2020 at 11:27 am #26287
Hi @patrickb, wow, good for you for posting such an important question. I think that the group has offered some helpful tips.
It sounds to me like once you switched to Adempas is when this started. I also had issues when trying to do Adempas versus sildenafil and was put back on sildenafil. I find it interesting that instead of trying Adcirca, which would be in the same treatment class as sildenafil, they added Adempas. We are all different, but this makes sense if this was when this all started. I would ask your PH team about this, too.
As far as remodulin, I have no experience. But I have been told when I need to start it, and it would be IV versus subq. I am sure that your team is knowledgeable, and it sounds like you are on triple therapy already. But this makes sense to add subq or iv as you mention your pressures have doubled, was this in the last 6 months since stopping the sildenafil?
Many have had great results with remodulin, so I am hopeful that you will, too. When is this scheduled to start?
Just some information as @mamabear007 mentioned, CTEPH, this is PH due to blood clots in the lungs that result in scarring. This is the type of PH that a person has based on the cause of the PH. I hope that makes sense.
Thanks to all of y’all who have already shared your experiences with Patrick. I know that he is appreciative. Well done!
July 17, 2020 at 11:31 pm #26294
Hello all, well I started the remodulin on tuesday, and am titrating every 3 days. The nurse says my dr has me on a very aggressive dose so we will see. Right now i have some site pain, nothing terrible but just a constant sting but not terrible. Will definetly take awhile to get use to my new friend that goes with me everywhere lol. I have been on adempas probably close to 9 months and I dont think it really helped. Also on uptravi but my dr is getting me off of that now that i am on new meds. All is good with me so far, no real side effects so to speak of. thank you all for all your help and advice. Patrick
July 19, 2020 at 6:59 pm #26297
well last nite was miserable lol. site pain went thru the roof i managed tho. question for anyone while you are on remodulin titrating did anyone feel just kind of off?? like in a haze??? My bp is normal for me but just feel kind of out of it. any help would be great. thanks
July 20, 2020 at 12:12 pm #26299
@patrickb as I mentioned, my son was on IV Flolan so I can’t speak from experience about the site pain with remodulin, but I have heard from other patients that it can get pretty bad at times. I’m sorry you had a rough night. I hope you managed at least a little sleep.
Just in general with all of his PH treatments my son would sometimes feel unbearably tired and often with titrations migraines would get beyond intense. I’m sure you already have but make certain to update your doctor about how you are feeling. Report the haze even if others tell you it is normal.
Thinking of you and hope that once titrations are complete your body starts to adjust and that you start feeling more comfortable.
July 20, 2020 at 2:52 pm #26305
@patrickb, I am so sorry to hear of the horrible pain and issues that you are dealing with in titrating your Remodulin. I know for some, when titrations are too fast for our bodies, we may need a few extra days to adjust to each dose. It especially sounds like your doctor is aggressive with titrations. If ay any time, you feel like it is too much, you may want to ask if you can take it a bit slower.
As @colleensteele mentions, report everything to your doctor. It may be a good idea to keep a journal on your phone or paper on titration and how you feel the days following. You may forget some of the side effects if you do not keep track. I know that with brain fog, note-taking is helpful for me.
Best of luck with your titration. Be patient with yourself and listen to your body.
July 21, 2020 at 3:00 pm #26322Peter G PierceParticipant
I had sc remodulin installed two weeks ago. It replaced the orenitram I had been on (5 mg x 3 daily) for 2 1/2 years. I also take daily 40 mg taladafil and 10 mg opsumit. I’m on O2 24/7, 5 liters. I had been taking letairis but switched to opsumit because of the nasal congestion letaris caused. The remodulin has been amazing. I must tolerate it well. Had no problems titrating up with orenitram and have had no problems with the sc remodulin beyond the expected site pain. For me, day 6 was the worst when I would have put the discomfort at a level 5. Today I simply don’t feel any discomfort at the site. I’m titrating every 3 days with a goal of 3 mg; at 2.4 now. My energy level is noticeably higher and I can go to the lobby to get mail without my Inogen portable. When walking with Inogen I can just keep going, no stopping, huffing and puffing; not so 6 weeks ago. I am asymptomatic and can get long with O2 level in low 80s/high 70s without feeling it. In any event, sc remodulin has made a big improvement in my quality of life. BTW, Carol: thanks for the tip on Aqua Gel Foam. Securing the hub from water has been my biggest peeve to the extent of getting a bit”ripe.” I’m looking forward to a proper shower.
July 21, 2020 at 3:45 pm #26325
@pete that is interesting that you made a connection with nasal congestion to Letaris. My son was on that and he often had problems with his sinuses but his doctor thought they were allergies. Orenitram wasn’t available at that time so he probably would have had to keep dealing with the symptoms.
Thank you for sharing your positive experiences. It is helpful to others to hear what has worked and has been tolerable for others. Every person is different and it provides hope to know that their is more than one way to react to a treatment.
July 21, 2020 at 3:48 pm #26326
Your special pharamacy should carry the Aqua Cel Foam – I have found some of the folks at the pharamacy never heard of it so I ask them to look it up on the list of items and they eventually find it! When I showed my sq site to my doctor he was amazed how un-irritated it was.
I was on sq for 12 or 13 years y til I had to go on the IV. Remodulin really does work!
Good luck with your progress!!!
July 21, 2020 at 5:47 pm #26331Peter G PierceParticipant
I was able to buy Aqua Cel Foam from Amazon. Delivery tomorrow.
July 21, 2020 at 6:49 pm #26336
Great news Peter! When I was on subQ I was even able to disconnect and go snorkling/swimming with the Aqua Cel. I was able to be disconnected for 2 hours – my Pulmonologist told me I could do that for up to 3 hours, but I kept it at 2. Stay safe and well!
July 22, 2020 at 7:26 am #26343Jennifer BeatyParticipant
I’ve been on remodulin for almost a year and a half. I take it with adcerca. I have had excellent results. I hate the site pain and itching though. However, during my last visit with my pulmonologist he stated that I have to increase (again). I thought I was leveled out at 2.4 in my syringe. I intended to ask him; but we got on another subject. So, do we build a tolerance to the remodulin? If so, what happens when we “top out”?
October 7, 2020 at 12:52 pm #27623LynnParticipant
When you add Letaris to Remodulin has anyone gotten more short of breath?
October 7, 2020 at 2:44 pm #27625
I would mention this to your PH team. When I was on Letairis, I had more swelling and shortness of breath because of that. I had to be started on another treatment. I am not on Remodulin and was not at that time, so of course, we are all different. But, increased symptoms like shortness of breath are things that your PH team needs to know. Have you noticed any increase in fluid retention?
I hope some others will share their experiences, too. Plesae do keep us posted.
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