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What Were Your Pressures At When Diagnosed?
@kygon asked what everyone’s pressures were at when they were diagnosed. It is a good question that has come up a few times in the forums so I thought it would help if I made it a topic.
Could you also share what your pressures are at now and if they are better, what treatment do you credit for lowering them?
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101 Replies
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Thank you Colleen! Just a curious cat, looking for any way I can keep my wonderful husband around as long as possible. He was diagnosed with pressures in the 60s after we repaired a hole in his heart to prevent another stroke, surprise surprise when we had a follow up to make sure the repair was set correctly there was a problem and his heart doctor returned with our daughters PAH team. He is currently on Ambrisentan, Sildenafil, and nightly oxygen and yesterday they added Uptravi and day time pulse oxygen and we ordered our inogen. We have had luck with just Ambrisentan and Sildenafil bringing his pressures back down but they are creeping up again and the right side of his heart is concerningly enlarged. Has anyone had good success with Uptravi?
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@kygon curious cats make excellent advocates!
I’ve attached two links below. One is to a previous discussion about Uptravi. You may ask more questions there and/or comment and here is fine as well. I just thought what was previously shared might be helpful. The 2nd link is to an article about Uptravi.
There are more articles and discussions available. Type in Uptravi in the search found at the top right and it will direct you to them.
Forum members – do you have anything to add to help support Ky and he her husband regarding this treatment?
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Hi @kygon, I’m hoping that the addition of Uptravi will help your husband’s symptoms and pressures. Since it was released, I’ve been on Uptravi, and I believe it has been a game-changer for me and my PH symptoms. Before we added it, my next option was IV Remodulin, so I’m incredibly grateful that it seems to be the best combination with my other PH medications.
My last pressures were in the high 40s after my COVID battle. Prior it was about 38-42 on an RHC. I plan to have an Echo in several months, so I will keep you posted.
With Uptravi, it is titrated, meaning it is gradually increased. If your husband experiences side effects that are too much, contact the doctor or nurse. When that happens, often, our bodies need a bit more time to adjust. The side effects that I experienced must be headaches, body aches, muscle pains, mainly in my legs. My PH team had medications available for these side effects before I started to keep them on hand. Taking this with or after food seems to help me.
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Colleen Thank you SO much! I just read and RE READ the forum on side effects. Very helpful information and I will pass along all of it as my husband starts experiencing it however the doctor decided to let him wait to start it until after we return from Cancun next month. We decided to start doing as much as we can while we still can.
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Aww, @kygon, that Cancun trip sounds just what you all need. I’m happy to learn that he is waiting to start until you all return; maybe a few days would be ideal after he rests. When are y’all leaving?
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@kygon happy to help! Oh my goodness Cancun sounds amazing! Safe travels and enjoy!
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Mine was 98 over 39 by the echo. When I had my cath, it was a little higher. Sildenafil did nothing for the first few months. Once I got on Adempas and endured the first 5 months of misery getting to the max dose, I have been in the 50-60’s over 30’s. Still high, but much safer and improved. I am due another echo in a couple of months.
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When I had my Cath 14 months ago I was told pulmonary pressures were 83/30. I collapsed a short while later and ended up at UCSF for 8 days. They put me on Opsumit and Tadalafil and diuretics. 5 months ago I went from a Classification 4 to a 3. Last week I was re-classified as between a 1-2. While I have not had another Cath for a real confirmation of the exact pressures, the Echo did estimate they are close to normal and the right ventricle is also only slightly enlarged. I attribute this almost exclusively to these 2 drugs and fortunately the side effects are not debilitating . Frankly, it is not always possible to determine who will respond to which drugs and who won’t, in addition to the fact that many of us have no idea how or why we contracted the disease(IPAH). I am turning 74 soon and didn’t expect to be here at all. My prayers go out to all my brothers and sisters with this disease(s) and thanks for this forum to give us an avenue for support and education.
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@mgnorwood I would like to highlight something very important that you pointed out…
“it is not always possible to determine who will respond to which drugs”
That is a good reminder for everyone here. Just because one patient doesn’t respond well to a treatment or has problematic side-effects when taking it, doesn’t meant that will be the case for other patients. Same for those doing well on a treatment – doesn’t mean everyone will.
The reason it’s good to follow how other patients are doing on treatments is because it provides you with concerns to watch out for and/or progress to hope for.
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Thank you all for the information, I didn’t even know they did classifications and our doctors never mentioned a second number, I know his current pressures on the meds is back up to 68 but I don’t know what its over.. It’s hard to understand this disease and all the complications and medications and side effects! Praying for everyone here and around the world affected with this and all other rare diseases. Sometimes I feel like the doctors and even the transplant teams don’t truly understand what they are working on because there is so few people with it and everyone is so different! I thank you all for your support!
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Well Guys, I thought my pressure was bad, as the Cardiologist said I had “serious PH and Heart Failure” – all in the one breath. I subsequently discovered that about 28 is normal, so that was my baseline for further study. I had started on Nitro-Dur patches until I got in to see a speciaist. Then it was Bosentan for 2 months,which helped a lot, but affected my liver, so I was switched to Ambrisentan 5mg, then a few years later 10mg, which I am still on. A Right Heart Cath a couple of years ago showed my pressure to be 42. I use oxygen only at night due to desaturation when in REM sleep. As an aside, I had 2 stents and a pacemaker emplanted in 2013. I had Sick Sinus Syndrome where my heart would miss a lot of beats. From how I was before diagnosis in 2012, when I believed I was dying, to now – I am fit as an 80 year old fiddle. Be your own advocate and research to understand what’s happening to you – doctors are notoriously poor communicators – unless you’re lucky enough to get one you can talk to. Good luck.
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@auntlizzie provided an excellent reminder too.
“Be your own advocate and research to understand what’s happening to you”
No relationship should be one-sided – the patient/doctor relationship should be no different. Patients and doctors must work together. BOTH need to be good communicators.
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Well said, @colleensteele! We work as a team with our medical team. Thanks for bringing this to our attention, and WTG, @auntlizzie!
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I was never told my pressures but I just had a RHC done and this is what it said:
Pressures
RA 15/12 (11)
R/V 48/7 (13)
PA 48/25 (35)
PCW 20/20 (18)Anyone knows what this means?
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Hi Debbie! Sorry for the long post, but this is a fairly complicated disscuation. Please keep in mind that I am not a Physician nor a PAH specalist. I am new to this forum, but I have been a critical care healthcare provider for a long time and I am currently a medical student as well as the caregiver for my husband who was diagnosed in 2017. I’m going to try and explain what some of these measurements mean, but if you have any questions please let me know.
First, let’s talk about the order of the numbers themself. The first number is systolic pressure. This is the pressure in that part of the heart when the muscle is contracting (pushing blood out of that part of the heart). The second number is the diastolic pressure, which is the pressure in that part of the heart when the muscle is relaxed (filling with blood). The number in the parenthesis is what is the mean pressure which is the weighted average of the systolic and diastolic pressures, and is arguability the more important number of all of them as it provides a more concise idea of the pressure throughout time.
part 1/3
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RA is short for the right atrium which is the first chamber of the heart that blood enters. This blood comes from the veins in the body and it is deoxygenated, meaning that it doesn’t carry oxygen. The normal pressure for a healthy person in this part of the heart is 6 mmHg. It is unusual for this part of the heart to have both systolic and diastolic pressure. This number is somewhat confusing as it is also the pressure of the blood in the veins. In your case, this is number is a little higher than normal, and if you are seeing increased swelling in your legs this is probably why.
RV is the right ventricle. This is the part of the heart that pushes blood through the lungs. Other than the pulmonary artery pressure, the RV is one of the most important pressures in PAH. As it becomes more difficult for the heart to push through the lungs, this pressure will increase. A healthy person will have systolic RV pressures of 15-30 mmHg, and diastolic pressures of 2-8 mmHg. Again, as is normal in PAH, your RV pressures are elevated. Most of the symptoms from PAH come from this part of the heart not working as well as it should.
PA is the pulmonary artery pressure. This is the defining measurement of PAH and it is the pressure of the main artery supplying blood to the lungs. This artery is a little tricky as it acts differently than all the other arteries in the body. When pressure increases in this vessel it gets thicker and more narrow which makes it more difficult for oxygen to move into and for CO2 to move out of the blood. The normal range for systolic pressure in the PA is 15-30 mmHg (same as RV) and the diastolic pressure ranges from 8-15 mmHg, with a mean of less than 20 mmHg. To be considered PAH the mean PA pressure typically is 25 mmHg. As is to be expected your PA pressures are elevated as well.
PCW or PCWP is pulmonary capillary wedge pressure. This is the pressure in the left atrium (or third heart chamber). Getting this pressure is somewhat complicated to understand. When they are obtaining a PA pressure they inflate a balloon at the end of the sensor that isolates the left side of the heart from the right side. This allows us to get a reading of the pressures in the left atrium (LA). This chamber of the heart works the same way as the RA does, and its pressures are equal to the pressures in the pulmonary veins which bring oxygenated blood back to the heart from the lungs. The normal pressure for the PCWP is 8-12 mmHm and is usually reported as only one number. It appears that your PCWP is increased which suggests that the left side of your heart is causing a backup of blood into your pulmonary artery.
part 2/3
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Excellent share, @tchapman, and I love how you broke the posts into 3 parts. You are learning as you go, too, I’m sure. But your experience as a caregiver and med student makes things quite helpful.
Because I worked in nursing before my PH diagnosis, I wonder if you, too, at times, want to be the HCP, not the caregiver, when dealing with PH. Do you struggle with this at times? Do you think knowing more about medical jargon makes things worse at times? I’m curious.
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@tchapman it’s nice to have a medical professional join the forums! Thank you so much for providing @debbie and all our members with this detailed information. We appreciate your taking the time to do that!
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Colleen, it is my pleasure. I am currently very busy with school, but when I get time I am happy to help however I can.
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Hi @tchapman! How are you and @dpchapman doing? I finally emailed questions off to you for a column. No rush or deadline, just wanted to give you a heads up.
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Debbie, I really wish I did .. I am so lost right now I am at the mercy of the internet. Our doctor is not great with communication and with Covid she takes advantage of the fact that I am Not Allowed at any of my husbands appointments … and honestly I feel like they let things slide with covid and had we been allowed at her appointments maybe we would have pressed them so much harder for answers and information, perhaps … possibly .. we could have saved our daughters life. Sorry hard day, I digress.. I will look into A a Pa R/v PCW and see what I can find on google but I am quite sure you have done that. (hugs)
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My numbers after last Right Heart Cath were
PA. 70/17 Mean 35. Severe baseline Hypertension
That was in August of last year and they did a Nitric Oxide challenge study and the numbers were:
PA 52/20. Mean 33Sooo…they started me on Adempas and I have been increasing titration to 1.5 MG 3x a day and I will be scheduled for a pressure test in the next month to find out if it is working. I assume it is another right heart cath, but done at UCLA, rather than Hoag Hospital in my hometown.
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Hey y’all, I wanted to share this with you for those needing a little more basic information about the right heart cath. This is from UCSF, and they also have a well-known PH Center.
This document in Adobe offers a calculation guide that may help those who want to calculate the numbers given within the RHC report. I converted this to a Google Document for better viewing. Hopefully, this isn’t too complicated.
https://docs.google.com/document/d/1lsj4Wbeq98TstCa2d8OdddqiF3nXSRqe-DqVszSUof4/edit?usp=sharing
@debbie, @kygon, and others
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my pressures when diagnosed were 96 and today they are 108 LOL.
I am on Remodulin, along with opsumit and adcirca. My pressures have not gone down, but I am doing much better and my heart shows improvement. I was in heart failure when I started remodulin and now it shows damage, but has regenerated its self….-
Hi @kgrady69, like you, I continue to have RHF but have seen improvements in my symptoms and heart function. I’m so happy to hear that you’re doing well with Remodulin. Thanks for sharing- hugs.
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I was diagnosed in April 2005 living at 7000 ft. above sea level with a pulmonary pressure of 98.
My echocardiogram last fall my pressure was 36 and I take 10 mg. of Opsumit once daily.-
Wow, @doccharli, 7,000 above sea level is high and would undoubtedly cause some increased symptoms for me. Did you relocate, or do you continue to live in the same elevation?
That’s an impressive improvement in your pressures with Opsumit. @codydaman, you may find this offers you a bit of hope with Opsumit. Thanks for sharing, Charlotte.
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Yes Jen, we relocated. We left the mountain the day after I was diagnosed and went down to 200 ft. Spent six month getting diagnosed and doing pulmonary rehab, then moved back to Western WA where we have been since late fall 2005. I’m sure getting back to almost sea level has helped my pressure quite a bit too.
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Hi @doccharli, wow, you’ve been busy moving around since your diagnosis. How is the elevation where you’re at in Western WA? I know it’s much lower than your mountainous areas when diagnosed. I do know many who have positive results when relocating to lower elevations. I’m grateful that you’re one of those who have noticed improvements. Thanks for sharing.
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Jen, we are now in Covington, just SE of Seattle at around 50-100 ft above sea level.
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Hi @doccharli, it sounds like you’ve found a perfect location for your PH and also near some excellent PH specialists, from what I’ve heard from others.
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- My pressure from RHC in August of 2021 was 72/38. I do not know what the mean was. Pressure from RHC on February of this year was 80 mean 52. I had Started on Tadalafil 20 mg in January and upped to 40mg and started on Ambrisentan 5mg. I am in Right sided heart failure
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Hi @debcgonz, were you diagnosed with right heart failure when diagnosed with PH? How are you doing with the increase and addition of Ambrisentan? Have you noticed any improvements in your PH symptoms? Thanks for sharing your experience with PH to better help others.
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When first diagnosed with ph my pressure was ‘only’ in the 27-28 range (with >25 meaning it was ph), based on a right heart cath. I’ve only ever been advised of my number as a single digit so am not sure of any other numbers. The last right side cath I had, in 2021, my pressure had dropped 6 points. This technically puts me in the normal range for pressure, took me a day or two to figure out that even with a ‘normal’ range number it’s great for the progression of ph but doesn’t affect the damage already done. I still need oxygen, still have ph, still get very winded, but it hopefully means I have some time before things progress to higher numbers.
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Hi @dawnt, how have you been? I’m happy that you shared that your last RHC was 6 points lower than previously. But as you mention, you continue to have PH, need oxygen, experience symptoms, and have a body that PH has wreaked havoc on. Unfortunately, some think when we improve at all, things go back to “normal,” meaning before PH.
When I use the term “stable” for my PH, many often take this as I no longer have PH, need oxygen, or experience symptoms. Thanks for making this critical point.
Although, I hope and pray that we all can have minor progression or more time between progression with PH.
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@jenc that is a good point! I think sometimes people hear “stable” but think “remission”. Hey, that would make a good column topic for you.
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Hi @colleensteele, this is one of the columns that I’ve started but hoped to revisit. The time to revisit this topic for me maybe soon. I’m working on another for the next one, but you’ll see it before long if thoughts come back to this one after that. Great minds, huh? 😀
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@tchapman thank you, thank you! This was so informative and will be a great way for me to track my numbers. I printed out your post so I won’t forget anything. I really appreciate the time you spent educating me. Thanks again.
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You are very welcome. I hope you get to feeling better soon.
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Yes, IKR, @debbie. I think many others will also benefit from the time that @tchapman took to break this down for everyone to understand better. Tyler, how are y’all doing this week, BTW?
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Jen, I am happy to help however I can. Unfortunately, I will be very busy with school for the next month or so, but when I get a chance I’ll hop on and see if there is anything I can help someone with.
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@tchapman I would still like to interview you for a column. What I will do is compile my questions, send them off to you and whenever you get around to answering, that is when I will write the column. No rush and no pressure. Sound good?
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Hi @tchapman, I can only imagine how busy life is for you with school and being a caregiver. Please be sure to take some quiet time out for yourself and refresh. Caregiver burnout is often common and perhaps more of a risk for you with such a busy caseload with school.
Please don’t feel obligated to post, but we like to check in and know others appreciate your helpful insights. Take care and good luck with school.
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I just want to say a big thank you to everyone for all the information and education! Hopefully I will get some clarification on what exact pressure our Dr is referencing at some point but Thank you @tchapman for breaking it down. Very good information!
And @jenc and @colleensteele yes Cancun was absolutely amazing and wonderful we were gone for a week and now we are back in the swing of work and home and just started our Uptravi adventures on Friday, Bryan says the headache and brain fog feel just like right after his stroke and the muscle aches are just like the flu, the day after he started he made it from the bed to the couch but each day gets a little bit better. Hopefully we will get a few full good days before our next titration!
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Hi @kygon, I’m grateful that you enjoy the forums and find the information beneficial.
Your Cancun trip sounds beautiful. I usually never travel for more than 3-4 days, but I did to help my parents last month. They ensured I rested in between tasks, so I wasn’t as exhausted as I thought when I returned. The first day or two was rough, but then I bounced back.
How did Bryan enjoy the trip? Were y’all able to do activities that you had planned, or did he need more downtime than expected?
Uptravi titration can be challenging. If he has more side effects after titrating than he can endure, let his PH doctor know, and they will often allow more time for his body to adjust before titrating. I needed that a few times before I reached my target dosage. For the muscle aches, mainly my legs, I do warm soaks in the bath, and my PH team ordered medication to help with that when it became too uncomfortable.
Please keep us posted on how his titration is going. I can imagine recouping from that Cancan vacation and starting it may compound the effects at the beginning. Also, I eat a snack with mine when I remember.
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@jenc Yes thankfully we were able to do everything .. he was determined to even go ziplining… I was nervous about that but it was kind of our last hoo rah before starting the new meds and now I am SO glad we did it all, and he did really well so close to sea level! (we would talk about relocating but it’s really not feasible) I will have him talk to his Dr if it gets worse with each titration and he ends up needing anything extra, the nurse just recommended Tylenol, but we have a long long ways to go! We like you are trying to avoid the Remodulin pump. Our daughter had such a hard time with the pump and site pain. He has definitely figured out the food with the pill game already but I will recommend soaking in the tub its his neck legs and back but he also complained that its odd for his legs to be so achy … not something normal for sure.
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Awesome, @kygon; that inspires me that he DID make it to do ziplining! I want to do this, too, one day. My biggest concern on most ziplines is how I get to the top to go down. I would love that! My hubby says he will find one that works for us eventually to try. Besides racing go-carts, hiking, and rock climbing a few times, I was not so adventurous before PH. But I think it makes me want to do those things even more. Hehe
If he takes the Tylenol around the clock and tries stretching and soaks with no relief, certainly contact the nurse. Unfortunately, that achiness is part of this titration. Most people taper off as their body adjusts to a dosage. But then it’s time to titrate again, and it often starts again. Maybe he will be one where that subsides after the first few titrations.
Please keep us posted and let us know how we can support y’all! Thanks for sharing!
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Jen I don’t know where you are located but in Provo Utah there is a zipline that they drive you to the top and you just climb a few stairs and then zip zip zip all the way down. There is one at the end that you walk a ways to but it isn’t bad because it is all downhill. However the elevation is somewhat high at the top so that might not work either. Unfortunately the ones in Mexico you climb up to zip down and I thought Bryan was going to pass out, and he couldn’t wear his oxygen because obviously they aren’t going to let someone on oxygen on their zipline … we basically had to lie all day or he wouldn’t have even been able to do the jeep drive through the cave.. literally everything had Do Not Ride if you have Heart or Lung issues… we were like nah he’s fit as a fiddle! Keep a look out around lakes and state parks because some of our lakes have simple ziplines that even small kids can go on, so not far up. We rarely go climbing anymore .. a few baby climbs but my hubby was always the lead climber and after his stroke it is hard for him to safely lead, with no feeling on the left side,… but we do a few top rope climbs and try to repel once in a while however getting to those locations is getting harder and harder. Our canyoneering trip last year he admitted that it was likely his last and we cried! But we go camping and love going for drives and we have a few places we just drive and walk 2 min to a nice cliff and dangle our feet over the ledge… we call this happy feet.
Perhaps rather than a zipline you can try parasailing behind a boat? That way your at low elevation, there’s no stairs, but you still get to fly over beautiful water? I haven’t tried it yet but my sister did and she said it was super amazing, if you do try it let us know what it’s like!
Thank you so much for all your advice and support! Bryan is tough but I admit this medicine has him a little overwhelmed, I have never really seen him discouraged about his medications before, but I think he is just “wow 4-6 months of this”… I am just trying to be as supportive and understanding as possible, Is there anything your husband did that was extra supportive and encouraging to help you through the roughest days?
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Hi @, I’m in San Diego, Ca. I appreciate your tips on ziplining. Manny told me last night, ironically, that his buddy at work told him about a ziplining place not far. He says he will check that one out soon.
I have seen parasailing, and our daughter has done that in Lake Havasu with her late Grandpa. It was so cool to watch. We watched several do this as we drove home from the beach on Sunday. In the bay, they do that and paddleboard, sailing, etc. Have y’all tried paddleboarding, too? Manny wants to do that, but that looks like a lot of core body strength. Plus, I’m clumsy and would probably end up sitting on it or in the water the entire time.
Manny always did and did like you, hated to watch me in pain and uncomfortable, but he would do the tiniest things that meant the most. He would ask what he could rub if it were my legs, etc. He would also remind me that we are in this together and that we will get through this.
Little things like bringing home my favorite drink or snack, and doing chores when I can’t, like grabbing take out were helpful. But most importantly, being there and learning all you can to best support Bryan is huge and means more than you’ll ever realize! You’re doing a fantastic job as a supportive partner.
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@Jenc Yay!!! I hope Manny found the right place for you to try ziplining! San Diego, that is a very exciting place to live, I have only vacationed there. Parasailing is definitely on our to do list however I have gone paragliding… Very Very top ten amazing! And yes we have been paddle boarding, we own an inflatable one and a few kayaks we just trade off who is doing what when we go with the kids .. it isn’t as core intense as you might think, especially if your on calmer water, you just start on your knees and get a feel for it before you stand up, once you are up you get really good pretty fast at going back on your knees when there are big waves. I think you would really enjoy it. Oh and the higher the weight rating the more stable and more than one person can go together on it ..because Kaylyn was so tiny she could wear her POC on the paddle board, but just not stand up because they are so expensive, not worth the risk… ( a few times while wearing the POC she would sit on the front cross legged and her dad would stand behind her and paddle and I would swim behind and if it got choppy I just grabbed the board and helped to stabilized it.) She usually wanted to enjoy standing up and just left her POC on the shore. Just some ideas for you since you are currently on oxygen 24/7 right? Hahaha maybe we should crate a chat for bucket lists and creative ways to make it happen! Hugs thanks for all you do!
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@jenc @kygon Cullen came so close to getting to zipline about a year after his transplant. We were on vacation in Utah and he was in line for it at Olympic Park. But the line was really long, it was really hot and we were with his transplant friend who was also battling cancer. The wait got to be too much so we moved on to something else. I kind of have a fear of heights so it’s not on my bucket list. LOL!
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WHAT? I am so sad to hear he didn’t get to do it! But lucky for him he has HIS WHOLE LIFE AHEAD OF HIM to try it. PS I Pray Pray Pray he has a truly long life! I understand Lung/heart transplants come with their own complications and hardships/limitations but they are ever improving on that .. so sorry I digress.. funny story my youngest did that very same zip line at Olympic Park when he was 4 yrs old (OH Wait.. Does that make me a bad mom? I am not sure! LOL) he was tall enough, wanted to do it, but he yelled all the way down “OMG IM GONNA DIE MOM” …. but he was laughing at the bottom and he lived so whatever I take that as a win.
I am so sorry to hear you have a fear of heights. I once did as a child and then I went on my first scary hike as a teen and I was on top of the world and it felt AMAZING… Fear gone, now I am obsessed with getting to the top because the view can’t be better than the top! I also met my husband on a cliff.. he was repelling, I was hiking and leaned over the edge, I said “That looks fun” he said “wanna try it?” he tossed me off a cliff the day we met and well I FELL and fell hard hahahaha
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First of all let me just admire what an amazing mom you are! Not only to your own son but other children on the transplant list… WOW.. I bow… beautiful, truly beautiful!
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When I was diagnosed in February of 2018 my pressures were at 120. I use a CPAP and oxygen at night, plus I take Opsumit and tadalafil. This treatment plan has brought my pressures down to 41.
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Hi @rayetta-meverden-johnson, how are you doing? What’s new? It’s great to see you pop into the forums.
Your journey shows how important it is to find the most effective treatments for our bodies. In 4 years, with these medications and your CPAP and oxygen, you have had a tremendous improvement in your pressures.
I’ve had some ask me if oxygen and BIPAP or CPAP help; I always say most notice a difference after using these treatments continuously for a brief period. If I go without my oxygen at night, I feel so crappy the next day, so I know it helps.
Thanks for your update and sharing with our members. Others will find it encouraging.
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Hi, my rhc at diagnosis was mean of 52. During the rhc I inhaled nitric oxide and pressures dropped instantly to 26. I was classed as nitric oxide responder. Put on sildenafil, ambrisentan and diltiazem in 2014. Pressures were around 30-35 until 2018 and then since then are more like 25-30. Believe the reduction is we increased diltiazem to 600mg per day. Had few rhc in recent years – last being 2021 which showed consistent around 26/27. Had echo a few weeks ago which showed mean of 24.
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Hi @gemsy-elizabeth, it’s nice to see you pop in and share your encouraging update. It’s incredible that you responded to the nitric oxide and have shown such improvement. I also have a close PHriend who was the same, and she is now on her calcium channel blockers (CCB) and a low dose of sildenafil. She was diagnosed about 10 years ago, so those who respond to nitric oxide and can do well with CCBs are impressive and offer hope for others.
How are you doing otherwise? Is anything new in your life you want to share with us? Thanks for sharing such a significant and positive update. Take care and enjoy your weekend.
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@Gemma, thank you for response, I have never heard of nitric oxide inhaled… I think @Dennis mentions nitric oxide as well… How interesting I am going to go on a google hunt for more information! Hopefully you can keep those pressures low enough to not need to move to a next level medicines!
Prayers for you all!
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; I would have thought Bryan’s PH team would have mentioned that some respond to the vasodilator test with nitric oxide. This is an important option that those who respond do well with. The percentage of these patients is often much lower, so maybe they didn’t mention it. Thanks, @gemsy-elizabeth, for bringing this to our attention as a reminder that some do respond to calcium channel blockers (CCB).
Here is a little more information about this, @kygon that you may find helpful. This may be a topic that we need to create so others can share their experiences with CCBs.
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Thanks Jen and Ky for your replies. Whilst my PH is stable, for the last 18 months I’ve been really struggling with other health issues. A lot of it has manifested it since I went on the drugs for ph in 2014 so I’ve always assumed that it was side effects if the medication but the more that new tings happen to me it would suspect I could have other underlying issues going on. They are exploring the option of connective tissue disorder, in particular elhers danlos and POTS. My heart rate just randomly increases from 60 to 120+ and on an adhoc basis I keep getting dizzy spells with a few fainting episodes.,When I was diagnosed with PH it was the idiopathic kind as nothing gave away why I had it. Now there is a small possibility that it’s linked to connective tissue. I also have venous reflux in my legs/ankles along with really bad dry eye/mouth, gingivitis, reoccurring pericarditis which all points to auto immune. Most weeks I feel exhausted, knackered and just out of energy. I work full time and have a full on job which I put all my energy into which means I have less in the tank for stuff outside of work. I need to start to get my work/life balance back! I am incredibly grateful though that throughout this time my PH has remained stable.
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@Gemma first of all my positive hopes and vibes and prayers for you to find balance and energy. also very happy you have remained PH stable with meds.. sometimes as disease progresses there is nothing but bad side effect medication. Happy to hear your not there. I had a sister with connective tissue disorder and a few other disorders .. she had a rough 42 yrs of life as her intestines connected together… although it isn’t what claimed her life, and she did everything she could on this planet, so no time lost. I don’t know what the other disorders are but I hope you get answers and find some reprieve! This site has done a lot for me and getting my hope back for my husband who has the same disease, PAH, that claimed our daughter in Feb. It is nice to get ideas and others take on things and not feel alone.
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My pressures were 85 when diagnosed and now 6 months later they are down to 41 due to being on Opsumit and Tadalafil every day!
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Hi @tiffany-ramirez, that’s an incredible drop in your pressures in such a short timespan. That’s such exciting news; thanks for sharing. How are you feeling overall?
Another reminder of how once we find the best treatment plan for our bodies, we will usually see improvements! What a way to start the weekend off by reading about such positive progress in pulmonary pressures.
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During the catheter, the doctor said that I reacted really well to the nitric oxide, so they knew I would respond well to the drugs they were trying to put me on. I’m so blessed that my team was able to expedite my approval process through insurance and I was able to get on Opsumit within a couple of weeks. Knowing how debilitating this disease can be, I’m so excited and feel like I can do most anything now! I still have my limitations, but it is nowhere near what it was when I was in the hospital and was out of breath just sitting on the bed.
I have read that Opsumit has really done an amazing job by cutting the numbers in half for others as well!
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It sounds like they caught your PH pretty early, @tiffany-ramirez! Plus, it sounds like you have an excellent care team. I’m grateful that you did well with the vasodilator testing and Opsumit. I was only on it briefly because it wasn’t working well with my other medications.
Your story will offer hope to all who read it! We certainly always remember the days when we were in a much worse place with PH. I know; I do, too. My heart goes out to all who have yet to find treatments and a PH team that are right for them.
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