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Do You Have Vasoreactive PH?
A member within our PH forums recently shared that she has Vasoreactive PH, which is a rare form of this disease. What I have read about this form of PH is that the small minority of patients who have it may benefit from an oral calcium channel blocker and not have to proceed with other PH treatments.
Although it might be easier to treat, the symptoms and struggles of PH are just as real for these patients.
Do you have Vasoreactive PH and if so, share your story with us. What treatments are you taking and what has been your greatest struggle?
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27 Replies
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I know several other PH friends who do have this type of PH. They usually do well on CCBs( calcium channel blockers).
The only issue I’ve heard or thought about is what if they’re not responding to CCBs. Often, that is a struggle.
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Yes,
my issue is kind of similar to this because my vessels really have a hard time responding well to medications. Usually it just goes from one extreme (either too constricted) or when they dilate I end up getting a lot of symptoms like headaches that are really hard to manage. I do well on the beta blockers that I have though and I have a new medication that I am going to begin today that hopefully will improve the strength of the muscles both vascular and the larger muscles in general.-
Brittany,
I am so happy and hopeful for you now that you can start this new medication. Keep us updated on how it is going! -
Hey Brittany, I am so excited that you are starting this new medication. I am hoping and praying that this offers you some relief so you can start to feel some better.
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I actually just got the text message that the medication got delivered to my pharmacy so I am looking forward to picking that up later today ! I will probably want to start it tonight so that the side effects don’t feel too bad in the morning. I know it may be a hard titration process and it stinks to have to start it a few days before the holiday, but when my body adjusts I am sure that it will all be worth it. I NEED to feel better than I have been lately and I KNOW that any bit does help!
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So happy that you are getting started on this med. I hope that you took it last night and do not have any negative side effects. I hope and pray this is something that will help you feel better overall. I know that you have been struggling for some time, now. You certainly need a break and fast!
Please do keep us posted on how things are going.
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Jen,
I will for SURE keep you updated! I will text you tomorrow and let you know how it’s going. I will be starting it today actually. Last night I had weird numbness in my left arm (where my bypass graft is) I called my cardiologist and we may have to do imaging on it soon depending on what my home nurse hears today when she comes here. Last time they couldn’t hear bloodflow well at all on my left side so I’m a bit concerned with this numbness. Just didn’t need anything else throwing off my body. I really hope I can start it today. It’s always hard to start a new medication when prior symptoms are so bad it’s like you question how much more symptoms and side effects you can possibly handle. -
Brittany,
I hope it all works out for you today and the side effects aren’t more than you can handle. It would be a great thing to be thankful for this holiday so I’m praying for the best news from you. -
Thank you Colleen ,
The medication that we have is certainly something to be thankful for and I consider myself privileged to be able to get access to the medicine that I have and have it covered by my dads insurance.
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I’m part of this subset of PAH patients that respond well to high-dose CCB (calcium channel blockers).
I was diagnosed almost two years ago and, similar to most of us here, I was given 2 to 3 years to live.
I was initially taking Bosentan until my second right heart Cath when a vasoreactive test was performed and it was positive. I take a very high dose of Diltiazem and the symptoms are not that aggressive anymore.
I guess I’m somehow lucky but at the end it is the exact same roller coaster we all are on, you never know how things will work.
Being vasoreactive is not definitive and the good response to CCB can end anytime and I will have to start taking specific PH meds then.-
Diego, I hope the channel blockers will continue to work well for you for a very long time. My son was also on diltiazem for a while but when he went into acute heart failure he had to stop taking it.
Thank you for sharing your personal experience! I we will continue to hear more from you.
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Hi Diego, I am happy to hear that you are doing well and responding to the CCBs. I am hoping that this will keep your symptoms at bay for some time. Thanks for sharing
Welcome to the PH forums.
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No one mentioned that the Calcium Channel Blockers could stop working. I am just on 180mg right now. It was raised after one month. My leg swelling is worse and I have gained weight which is the most aggravating symptom because I had weight loss surgery over 6 years ago so I would lose and then NOT gain it back. The fatigue is also bothersome. The dizziness is better but not gone. I cannot get my Dr. to answer me as to life span. He mentioned the three years for those who have different kinds of PH but NOT vasoreactive. He said I will live longer. OK – is it important for you to know how much longer you may have. I am getting the feeling it is important to me to know.
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Rebecca,
From reading statistics about how much “longer” I technically have with my type of congenital heart defect, I can tell you from experience that it never helped to research. It usually just made me feel worse and from what I have found, it is just not helpful especially because so many people end up defeating the odds in one way or another and live LONGER than anticipated. I am sure others on here can back up what I’m saying. Just know that everyone’s situation is different and their responses to medications are different too and need to be individualized. It is concerning that your doctors aren’t calling you back. Is there a nurse or nurse practitioner in the office that you could speak to in the meantime or do you have a primary doctor that can give the specialist office a phone call? Sometimes it helps when another doctor tries to call.
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I agree, doing internet research is pretty much futile at this point. I no longer care what I read. Only God knows the date I expire.
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Christopher,
My thought exactly! I have beaten the odds numerous times and as long as I have it left in me, I will continue to do so until it’s my time. I don’t plan on that being any time soon ! -
I totally agree with you both, Brittany and Christopher. Those websites can scare the heck out of someone. I am well past my “expiration” date. Like Brittany mentions, I have no plans to go anywhere anytime soon.
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I have had no problems with my Dr. office calling me back, I have never called them. When I asked about life span, he just kind of had a vague answer that Yes, those with vasoreactive Pulmonary arterial HTN tend to live longer. I have a feeling that his vagueness has more to do with the fact that this is a rare type plus the fact that I am new to this. As far as “research” goes, I have discovered that most articles that I have found online are many years old. When it comes to medical FACTS, when time passes, things change. New research puts whole new spins on treatments, etc. He told me not to get online. By that time he had said that, I had already been there. I used that to determine what symptoms are, tests that are done, etc. As an RN, I am well aware that things that are found online can be misleading as anyone can say or post anything. Next up for me is another Echo and 6 min walk test and a BNP lab test, all in February. I exhausted myself with Thanksgiving preparations. My son in law was helping me in the kitchen and in fact cooked the turkey on the gas grill. I think I got through it on adrenaline alone and now I am exhausted. It will be 3 days before all the dishes and stuff is put away. But…it was that way before all this.
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Hi Rebecca ,
That is great that at least your doctor’s office gets back to you. It can be frustrating though when it feels like they just don’t know ENOUGH about what you are going through or that the research for your particular type just isn’t there. I have had personal experience with the type of heart conditions that I have that doctors just don’t necessarily “know” the outcomes and what things will look like years down the road. At least some things to look out for would be a good start and like you said, information about managing symptoms etc is important to better prepare for what may lie ahead .
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Hi,
I have vasoreactive IPAH (RHC June 2019) and am also interested in hearing other people’s experience. I find it very difficult to get information. I am on ccb but was recently told to start warfarin which seems like it would be a huge lifestyle change, but then I was also told it is not thought to be as effective with earlier diagnosis. Does everyone take this, what about the newer alternatives, and how problematic is the bleeding after a bump (I do several things on the list of things not recommended)? Also, my general policy is to take as little medication as possible but I was just told I should be on the maximum dose as this relieves strain on the heart for longer. This makes sense to me, but do the side effects negate the benefit? Is it general policy to maximise the ccb dose? -
Hey @agrimsdell I am not on this type of medication but from how my vessels are with anything I believe that when I get another heart cath that they are going to find that this is probably what the underlying reason for my PH is. Is the dose of the CCB meds that you are on now causing side effects? I will post the question about Warafin for you in another post so people can comment on that medication more specifically. I will tag you in the post.
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Well, I have had a few incidents since December. My Echo and 6 min walk test in was great. The Dr. said he didn’t want to increase my CCB because my pulse was low and he didn’t want to go any lower. So all was great, then a couple of weeks later, I had another episode of “passing out” briefly, but long enough to get myself in a quandary. This time, I ended up on my side with my leg pressed against a table trying to hold on and not fall. ( HUGE bruise as a result.) Darling husband did not respond to my screaming for help fast enough to suit me ( he thought hanging up his coat took priority at the moment ) and that started a heated discussion as to what was important. Anyhow, I message the Dr. and asked if this was important. Ended up in her office the next day. She ( the chief Dr of the PAH group ) came to the conclusion that I may be having arrhymias and ordered a Holter monitor for 48 hours and sent me to a cardiologist. Holter showed some PAC’s and my Dr. seemed to think I may be having a problem in my atria and pushed the whole thing off on the cardiologist. Saw him today and he was unimpressed. Now why is it, despite everything you hear, that Doctors think only chest pain in indicative of a heart attack in women. No questions about jaw pain, arm pain, shoulder pain, sweating, etc. ( Sorry – pet peeve ) So now, I get a 30 day Holter monitor. 30 DAYS!!! Thank heavens they have improved and I will be able to remove it for showering. Alison, I certainly hope I will not be told to go on Warfarin. That almost fall I took would have turned most of my leg purple if I had been on that. BTW, Dr. Elwing, the main Dr. of the PAH clinic gave me a huge book, which she said is already dated, on PAH called Pulmonary Hypertension – A Patient’s Survival Guide. Mine is the 2016 edition. It has a ton of information but obviously most does not pertain to me. It did mention the muscle cramps, so at least I have an answer to that.
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@becca I’m so sorry. It’s the worst when you leave a clinic visit feeling satisfied with results only to have it ruined by an episode of some sort. I’ve seen my son go through that so I understand how frustrated you must feel. My son has worn the 30 day monitor as well. It is a pain to have to deal with but like you said, they are better than what they use to be. I can tell you from experience that it was worth doing because it provided his doctors important information. Let us know how it turns out for you.
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Hi Brittany glad your able to start taking something that might help you more. What is the name of the medication?
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Oh, Rebecca, I am so sorry that you are having such a frustrating time. I hope that the monitor will give them some answers. I have not had to wear one in several years but have heard they are much more user-friendly. Crossing my fingers that you get answers.
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Hi,
Brittany, thank you for reposting my question. At the moment I don’t have side effects from the ccb other than one instance of swollen ankles a couple of months ago. Thanks for your story too Rebecca, and I hope you don’t have to go on warfarin either. It seems to be not an automatic step which is good to know.
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Hi Alison,
I am glad to hear that you have not had many side effects with the CCBs. I hope that you notified your medical team of the swollen ankles. Has that resolved for you now? I have a PH friend who is a responder to CCBs and she has been on them for at least 8 years. Just some positive hope that you will respond long-term too.
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