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Advice From Experienced PH Survivors
Have you been living with pulmonary hypertension for 10 years or longer? If so, what has been the biggest help to you in battling this disease? Is there a treatment that has worked well for you long term? What has been the least effective for you over the years? What are the changes that you have experienced since your diagnosis? Share with us your experience.
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50 Replies
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Humm….I could write a book. During my 14 years with PH, I would say my faith, my family, and a good medical team has kept me going. I also want to mention other PH patients, especially the PH kids( &their caregivers), those patients who were born with PH, ( our Brittany) and you all. Watching others PHight each day is heartbreaking as well as inspiring.
As far as medications, I thank God for research and those who research. As a result of their hard work, I’ve been on only oral meds( most of them through the years) versus IV meds. Each time I’ve needed a new med, ironically, a new oral med was just released.
That’s all for now but I’m sure I can add as questions arise.
Final thought..never, ever give up…you’re ”worth the PHight”.
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What are some of the medications that have helped you? If you don’t mind me asking? And how did it make you feel better? I was diagnosed a little over 3 years ago. I have never tried any meds for my PH. But I can tell it’s getting much worse. I now use oxygen 24/7 and while walking it’s on a 10. I have several other Autoimmune diseases and I take meds for that. I just hate medicine and all the side effects and the weight gain and fluid gain. And the constant doctor appointments. I haven’t even drove in over 6 months. I hardly leave the house anymore. I can’t. And the worst is I have 4 kids. They see me struggle just to breath. PH is horrible! Your a very strong woman for fighting it all these years! Sorry so long. I am just at the end of my rope.
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My son was placed on Sildenafil and Tracleer and it was remarkable how much it started helping him almost immediately. A few months later a central line was placed and he was started on Flolan. A few years later he was doing very well and we could have switched to Remodulin but we decided to just stick with what seemed to be working. There were other medications as well, such as diuretics, beta blocker…but these I think Sildenafil, Tracleer and Flolan combined was what kept my son stable for 5 years.
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Thank you for the information I appreciate it. I wrote the medication down and am going to ask about them. Did your Son have to have a heart catheter did in order to test which meds would work the best? That’s what the Doctors want me to do, have a heart catheter up both arms and then meds will be injected to see what my heart can tolerate. Is that common? I was hoping to just try a oral medicine to see how it goes. Heart catheters scare me , I had a stoke during one when I was 36, I am now 43. I am sorry to hear your child has to go through this but I am glad to hear he does well on meds. When you say he had a central line put in, do you mean a Port? Sorry so many questions I just don’t seem to understand everything. It’s hard for me to retain information sometimes.
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Shannon, that’s how my son’s doctor discovered the right medication and dosage for his PH. First the RH cath, then starting the medication and leaving the cath in to see how his heart dealt with the titrating dosage. It actually took more hospital time to wait for the insurance company to approve Remodulin SQ (subcutaneous) than to find the right dosage. By time he was diagnosed, his situation was too dire to go the oral medication route. You could ask your doctor why he/she isn’t considering oral meds, if that’s a big concern for you.
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Thank you for telling me your experience with your Son getting his hearth catheter. I am making phone calls today and appointments getting to on top of my PH. And yes I am asking why I can’t take oral medicine. I hope your Son is doing well.
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Thank you Shannon. Yes my son is doing much better now. Two years after his PH diagnosis, he found out that he has CTEPH, a type of PH caused by blood clots. After the doctors treated the clots and scar tissue in his pulmonary arteries, it greatly reduced the PH symptoms. He’s now able to control it using oral medications instead of the Remodulin pump.
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V.R., how awesome is that…your son being off of the pump and on just oral medications! That’s a huge change in lifestyle, I know! Glad to hear he is doing well.
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Shannon, here is a link to PH treatments that you might find helpful. It includes the three that I mentioned.
https://phassociation.org/patients/treatments/
Also, here is a link that explains the central line.
http://pulmonaryhypertensionrn.com/continuously-infused-therapies-used-to-treat-ph/ -
Thanks @colleensteele, I was about to share that, lol
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Shannon, I’m sure it’s overwhelming, to say the least. You mention you also have an autoimmune disease, that may impact the medications that work well for you. It’s tough as we all react differently to these meds.
I am currently taking Revatio and Uptravi, this combination works well for me. I’ve been on Opsumit in the past, it was helpful, as well as several others.
They help me do more with fewer PH symptoms. At first, with any med, you will most likely experience the side effect train as I call it. This side effect train lessens once your body adjusts to the meds.PH is manageable and I hope that you can get started on a med that you feel some relief with. For myself, I find I have more ” good” days than bad PH days, that tells me that my meds are effective for me.
You are not alone, Shannon! We are here for support. I have a young adult daughter and it breaks my heart when she sees me on bad days. I can relate to you not wanting the kids to see you suffering. When do you have an appointment?
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Thank you for the link I appreciate all the information I can get. And I have a Doctor appointment with my Pulmonologist on September 26. That’s the soonest they can get me in. And they wanted me to do a walking, breathing test before the appointment. I can’t even walk. If I take off my air within a few minutes my O2 goes down into the 40s. I use a wheelchair. So that shows you what kind of Doctors I am dealing with in my area. They just aren’t informed enough about PH. They seem to treat it like COPD. And unfortunately with my insurance I don’t have many options. But I am going to research and get as much information as I can so I can hopefully start to feel better. This is no life staying in bed, and being wheeled around.
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Shannon, Are you talking about a 6-minute walk test? If so, most of us have had this test at least once. More often, depending on each PH doctor.
They usually have you walk with your oxygen ( if you use it for activity ) for the 6 minutes, but as you mention, you may not be able to complete the full 6 minutes. They use this to see progression before and after starting therapies. ( medications).
Here is a link for this test.6-minute walk
Also, not sure where you’re located, but I just recently wrote this column and you may find it beneficial. Doctors
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Hi Shannon, as Jen suggested, I think it’s the 6 minute walk that your doctor has scheduled for you. My son had to do them at almost every appointment. They understand that many patients won’t be able to complete the full 6 minutes and someone will be next to you monitoring and making sure you are ok during the process. It’s helpful to them to see how your lungs react to minor exercise. Especially when my son became critical he didn’t want to do the 6 minute walk but it’s a necessary evil. Let us know how it goes and what response you get from your doctor afterward.
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Shannon, it concerns me your PH has progressed so far and are not on PH meds. If the heart Cath is a hurdle to get your diagnosis, please realize that it sounds scary, but it easy a fairly easy procedure that you are awake for the whole process. I was diagnosed a year and a half ago and have had three. I was immediately put on Sildenafil, three months later Opsumit, then three months later Uptravi. You body needs to adjust to the changes, thus the stepped progression. The worst side effects that I had were stomach issues that included reflux/acid that is treated with Zantac and weekly diarrhea. Not fun, but tolerable. The pressure in my lungs has reduced from 90 to 25 in this short period of time.I have went from gasping for breath to being mildly out of breath with exertion and am very active. Note: I am on 4L of O2 during the day and 8L at night. My point in all of this is please be proactive. Get a Pulmonologist that is a PH expert, don’t live in fear of what the meds side effects might be or if a procedure is scary. Please get the help you need! You can get better! Sending you blessings, courage and hope!
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Thanks Robin for your advice and kind words. It came at a good time. I am having a rough day. But anyway, I have had heart catheter before but I stroke out during my last one a few years ago and am now terrified to have another one done. And I do have a appointment scheduled next month with a Pulmonologist. I have other health problems and Autoimmune diseases. Sceladerma being one of them and Lupus. But with Sceladerma my veins are very small and almost impossible to work with. It’s a painful struggle to even get a decent IV started. So that the main reason I don’t want a heart Cath. But at this point I feel like a fish out of water so it’s time for medicine or whatever happens next. Because this is no way to live. I use to run 5 miles a day, now I am lucky if I can get my self a cup of tea. Lol but thank you and I appreciate everyone’s advice. You all are a very understanding group! Xo
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Hi Shannon,
I know it’s hard especially when you realize that you are unable to do things that you once did with more ease. That is always the hardest part for me because it is hard to look at where I once was and where I am now. It is easy to get caught up in thinking about the things I can’t do or can’t do in the same capacity. I know it’s scary too when we have had bad experiences with caths or IVs and we just don’t want to have to deal with any more on top of what we are already going through. I hope that your new doctors can talk with you about ways to make things more comfortable and maybe put your mind at ease regarding next steps and any future procedures. -
Shannon, I’m so sorry you’re still waiting for your heart cath. I know you had a bad experience, I can tell you that my very first heart cath was terrible! I thought it was the worst feeling ever. Once I found a true PH doctor who had done thousands of caths, it was a totally different situation. It was uncomfortable but nothing like the first kind. I’ve had at least 25 by now and there are ways they can help you be more comfortable. Also, talk to your doctor about your concerns.
If you’re feeling really bad, I suggest going to the ER. If you want, maybe you can call and ask to be added to a list of someone cancels so you can see the doctor sooner.
I know it’s tougher dealing with PH and your other coexisting illnesses. Hugs to you and know you are nig alone.
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So true Jen! The first time is always the hardest because you go in not really knowing what to expect. Something that has always helped me a ton when I’m preparing for a procedure is making sure I know the details of exactly what is going to happen. I need facts and just need the truth of what to prepare myself for and what to expect. I don’t like any surprises. I also make sure there is a nurse in the room or someone that can explain each part of the procedure while they are doing it. This just allows me to mentally know what is happening and for me that knowledge makes it a little less scary and I feel like I have more control over it.
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It is much easier, well maybe less stressful if we know what we are going into. I agree, 100%, Brittany! I know for myself, in the cath lab, I often have at least a few of the same team. This makes it more comforting for me as at least one or more are familiar with me. We often catch up on our families and laugh and joke.
Since you’re having procedures so often, do you find that you often have some of the same team?
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Hey Jen,
YES this is also something that helps me SO much! Especially because it’s always good to see a familiar face and laugh and joke ! Just knowing that there are doctots or surgeons in the room that i trust and that really know me helps ease a lot of the anxiety around procedures. If there isn’t a familiar face, usually one of my own doctors will introduce me to the person that will be doing the procedure and will debrief them on anything they would need to know that’s of relevance. With new faces I worry, “do they even know my medical history “? -
So true, Brittany! Usually, at least one member is one of my, “ usual”. It helps my anxieties for sure and makes me more at ease.
I think at times some, “new” members think I am rude if I ask for my usuals, LOL
This is the same for me at the lab, especially the one I go to every 2 weeks. -
Being in the hospital is hard too, Jen! Especially when the team rounds and they have only spoken with your doctors (or so you hope so!) and then you are thinking in your head “have you even talked to my doctor? You’re NOT even my real doctor!” LOL I usually just try to stick with the teams that know me because then I don’t question their knowledge of my medical history because I know my actual doctors know how complicated things are.
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Thanks for your advice about going to the ER Jen. I did eventually give in to my pain and misery and am now admitted into my local hospital. I am glad I came in I have Pneumonia and fluid around my heart and some other things going on. I don’t have to worry about a heart Cath for at least a few days until I get more stable. So that’s fine by me lol. But seriously PH is very serious and I shouldn’t have kept putting my health last. Thank you to everyone for advice and kind words and I am ready for that heart catheter when the time comes and some medicine to start feeling better. I hope everyone is feeling well. Thanks
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Oh no Shannon! I am so sorry to hear that you have pneumonia! We can all relate to wanting to wait things out before going to the ER but thank God you took Jen’s advice and went. I’m sure you are in good hands and I hope that you start to feel an improvement soon. Are you still in a lot of pain or have they been able to help you with that? We will all be thinking of you. Update when you can.
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Shannon,
I know that it can be hard to “trust your gut” especially when it comes to your own health. It’s hard to admit that something is going on and that something is “off” with your body. It can feel scary and a lot of it probably feels out of your control. I hope you get some comfort knowing that you are in the safest place right now to receive the care that you need and they will be putting you on the road to better health and getting rid of this acute flare up for you. It’s not easy for our bodies to bounce back from things like this, but I have faith in you and know you can get through this stronger. Please reach out to all of us while you are there if you are looking for a distraction, we are always here for you if you need to chat! -
Shannon, I am sorry to hear that you have Pneumonia, but I am glad to hear that you will get the care you need. It is often difficult to decide if we should go or not. I have found in my 14 years, that I rather be safe than sorry. Although, still struggle with this if I think it is , “ just my PH”. LOL
Prayers are with you as I hope they get you some relief. Please do not hesitate to reach out for support while you are there. Please do keep us posted. I am happy that you went in when you did! Hugs
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Jen,
I get stuck with the “it’s just ______” mentality with my chronic illnesses. I’ve even said things before like “Oh. no big deal, it’s just my heart!” and people think I’m being sarcastic or joking but I’m totally serious. It’s like we just get numb to our chronic illness sometimes .Do you ever feel that way? Like we are so used to things that it’s like “ehhh just my lungs” LOL! -
Yes, Brittany, I agree. I feel at times we do become somewhat, ” numb” to our symptoms and illnesses. Then, there are days when I’m in, ” freak out” mode as my anxiety and illnesses get the best of me. Do you feel like that some days? I’m usually more positive and have high pain tolerance, but then there are days it’s all just too much.
I always remind myself that, ” tomorrow is another day ”.
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Thank you Colleen, Brittany and Jen for your kind words. I am actually feeling a little worse. It’s just hard to sleep. And my roommate snores. Lol But I should be feeling better soon after a few days of antibiotics and fluids. They did do a heart echo today and said the right side of my heart is grossly enlarged? Weird how that was worded. But that’s how it was said to me. But the left side looked to be mostly normal. Just wondering if any of you have heard that before? It’s new to me. They also have me on a soft foods only diet. My acid reflux is so bad right now. I think it’s hurting me more then anything else. They say milk makes acid reflux worse? But that seems to be the only thing helping me. And they changed all my GERD medicines around.My stomach never does well in the hospital. All the meds and stress. I am coughing less, which is great because my ribs were sore from all my coughing. And I am still on 10 oxygen and they won’t let me out of bed yet. When I am stable I will do the walking test. It’s looking like probably a long stay. Last time in December I was here for over a month. Thanks ladies for responding back and for being kind. I appreciate that. I hope all of you are feeling well and enjoying what’s left of Summer. Any good Netflix or Hulu recommendations?? Maybe I can wear my ear buds and not listen to snoring.lol
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I’m so sorry you’re feeling worse Shannon. My son’s right heart was also enlarged. Eventually, it affected his left heart, but the right heart was affected first. I heard the information about milk making GERD worse in the 1980’s. Some more information about milk… When my oldest son had pneumonia as a baby, our doctor warned me that milk would make him produce more mucus — something you definitely don’t want during a bout with pneumonia. The doctor said to keep him off dairy until the pneumonia was gone.
I pray you feel better soon.
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Great tip, @mamabear007!
Hope you are doing good this week.
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Thank you Jen. I’m doing pretty good. My son? He was feeling good enough that he started MMA classes again — and forgot that he wasn’t in the same shape as when he had to quit. He tore two tendons in his foot. As bad as that is, I’m glad his CTEPH is doing so much better. The foot is a big pain, but the PH is more dangerous.
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Oh no! I’m sorry to hear about the pain from the tendons. It’s almost always a sigh of relief when it’s not the Ph or heart related, but you still must hate seeing him suffer in any way. I hope the docs are able to help him out. Pulling tendons is no joke. That must have been really frustrating for him too, i can only imagine! Especially after getting back into it again!
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Thank you, @brittany-foster. He says as soon as the tendons heal, he will most definitely get back into it. When he was growing up, I grumbled about him being stubborn. Now I realize it was persistence that would serve him as an adult, and I’m grateful he has that trait. 💜
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It’s a great trait to have! I really hope that he is on the mend soon enough and can get back into it. I would love to take a boxing workout class one day. I used to love classes and being a part of something like that before getting to the point where I just didn’t have the energy for it. Hope he enjoys getting back to doing that and is able to enjoy it WITHOUT any more injuries ! 🙂
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Oh No, V.R! I am sorry that your som now has a torn tendon from MMA. That is a physically demanding activity. I hope that he heals quickly. It sounds like, as soon as he does, he will be back at it! LOL
How old is he again? Sorry, I suffer from, “CRS”. LOL
Have a great week. -
Jen!
You just made me laugh so hard I spit out my drink hahhahaha!!! CRS I totally know what that stands for. Can’t remember __(beeeeep)_____ hahahaha!!! Totally a good way to add some humor into the mix when you can’t recall things. I do the same thing . laughter goes a long way! -
Hehe, Brittany! I hope you had something good to drink. We definitely need some laughter dealing with these health issues.
@mamabear007, I thought he was in his 20s, so I was close.
I blame low oxygen, never age for my CRS. Hehe
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You’re right about that, Jen. As soon as he’s healed, he’s be going back to MMA! He’s in his early 30s.
I also suffer from CRS, but my excuse is that I’m in my 60s. 😁
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Shannon, I am so sorry to hear you are feeling worse. I hope and pray that the antibiotics will start helping soon. I am sure when they get some extra fluid off, it will help as well.
As far as your right side of your heart, “grossly enlarged”, PH patients do tend to have enlarged right sides of the heart as it is working, “double time” pretty much to compensate for the “ constricting” of the pulmonary artery. Does that help? I am trying to write it so you understand it better. Think of your pulmonary artery like a water hose and it is kinked off.
I just watched a movie yesterday, Otherhood, (Netflix)that my young adult daughter suggested. It was a comedy and emotional for me as well. I don’t know what types of things you watch. I would definitely try using your earbuds to drown out the roommate’s snoring and watch your shows.
I know the hospitals are no place we want to be, but it sounds like you need to be there for a bit until they can help you feel some better.
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Shannon, I am so sorry that you are struggling so much right now. All that you have shared is very familiar, especially the grossly enlarged right heart. I’m afraid that is a common occurrence with PH. My son was misdiagnosed for 2 years and by the time it was figured out that he had PH, the right side of his heart was severely enlarged.
I hope your team figures out the best reflux medication regimen for you. That is also a common problem but important that the symptom is addressed and controlled as best as possible. Reflux can do more damage to the lungs over time. Post transplant it’s taken just as seriously because it can lead to rejection.
I know how difficult long hospital stays are but it is good to hear that your team is taking your health seriously and trying to help get you more stable. Have you ever watched “Elementary”. That’s one that I really like and maybe you would enjoy too.
Update when you can. We are all thinking of you.
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Hi Shannon!
I’m so sorry to hear that you have still been in the hospital. I know how tough it is to be there and have to try to find some type of normalcy. I find that I am always so much more sensitive to sounds , smells, touch, etc when in the hospital. It is like our senses just go into overdrive. I always try to stay as distracted as possible and as Jen mentioned, even putting in some headphones just to get back in your own head and not have to worry about the sounds around you. Do more of the stuff that helps keep you in the present moment.
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Robin, you speak such true words. I’m happy to hear that your med regimen has dropped your pressures to normal, pretty much! Your experience and sharing are helpful to others.
Keep up the light!
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Hi everyone! You all are so nice and kind. Thanks so much for all the advice. I learned a lot more information today from a whole team of doctors and a “plan” that they have for me. My Pneumonia wasn’t caused by a virus but me coughing so much all the time that food got coughed up into the right lung, causing all kinds of problems. I am off of antibiotics, and basically on my normal meds and morphine for pain. My reflux is feeling better. And I am cutting back on milk. Even though I love it. But I agree it does seem to make more mucus. My kids have been so kind, each one takes a turn spending the night. And my daughter has only left me a few times. It makes me really appreciate my kids. And I have been watching a few new shows. My attention span sucks I think it’s the morphine. Do any of you have a pacemaker? They said I need one. Is that common with PH? I didn’t think it was. Sorry so long. Thanks again everyone!! Hope all is well!
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Hi Shannon!
Wow, thank you for updating us. We have been thinking about you. I’m sorry you are still in the hospital but I’m glad that you are getting good care there. Pain management is so important especially when you are dealing with the coughing that must really but a strain on your chest muscles and make everything feel even weaker. That’s so hard. I can understand the worry of food going into the lungs
Because of my reflux that is severe, I am on aspiration risks and have to get my lungs checked frequently (even if it’s just getting them listened to at the doctors). I get a lot of bronchitis and irritation from the refkux but it is mostly managed without antibiotics and more with pain management and codeine robitussin combo. I hope that you are able to get out of there soon. Are they thinking of doing the pacemaker while your are inpatient there? What did they notice where they are thinking this is going to be the next step? Just curious because I have a pacemaker and have had it since I was 21 so about 7 years. Mine got put in because I was having episodes of atrial fib and also pauses in between my heart beat like weren’t supposed to be as long as they were. It was very scary at first but I’m so glad that I got it done
It gets checked every 3 months to make sure I don’t need any adjustments to my medications. Do you have a heart condition as well on top of PH?-
Brittany you have been through so much. It’s hard growing up being ill. I also grew up sick and in and out the hospital. It’s not a easy way to grow up. But it does make us strong women! Who can survive just about anything. Yes I agree pain management is very important and it helps so much. I have developed a very high pain tolerance over the years and it takes a lot for me to ask for any help. The Cardiologist said the pacemaker would help with PH. And he said I had long pauses of heart beats while sleeping. This must be why I had about 10 people come running into my room every time I fall asleep in here. Lol I have overlapping autoimmune diseases, and a blood disease , that causes clotting and random bleeding, Chrones Disease, GERD and I am also partially blind( due to a blot clot (PE) in brain a few years ago) So I have a few things going on. But PH is the worst and it’s causing me at this time the most problems. So I do appreciate any advice and just for letting me ramble on. Colleen ….your Son is so lucky to have a great Mom like you! It’s hard when are kids are ill. We go through a lot to make sure they are comfortable and being well taken care of. It’s not easy being a caregiver. I am so glad he’s doing well. That’s great! And thanks for letting me no about him needing a pacemaker for his PH. So I guess my doctor is right then it can help with PH? And yes I have to get one before leaving here but not anytime soon. I had a blood transfusion around 5am and that went well. So I am just taking things slow and still eating soft foods! Gag me. So over it. Lol thanks ladies for your kindness it’s much appreciated during this hard time for me.
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Shannon,
We are always here for you! It definitely is hard being in and out of the hospital and sometimes feeling like you are in the hospital more than home. I hope you are doing things through the day to stay as busy as possible. I know it is easy to get a little cabin fever when in the room most of the day. I am hoping that the pacemaker they are discussing will help you out and hopefully help to improve some of the symptoms. Before I had mine in while I was having the periods of pauses in my heart, I would get very light headed and just really dizzy so easily. The pacemaker helped with that a lot and I’m so glad they ended up finding something they could treat ! Technology is so cool. When you learn more about it I’m sure it will be scary but also fascinating at the same time. Reach out any time if you have particular questions about it from my personal experience .
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Shannon, it was good to hear an update from you. I was wondering how things were going. It sounds like you have amazing support all around, from your medical team to your family! Having that kind of support makes all the difference in the world!
A pacemaker was the next step my son’s team was going to take but the day he was going to have it placed he received his transplant call. He was only 14 so the thought of giving him a pacemaker wasn’t on our radar but his doctor felt strongly that it would help him a great deal. Apparently it is something that some people with PH end up getting.
If you proceed with the pacemaker I hope it helps you and I will be anxious to hear an update.
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Shannon, so glad that you updated us. I hate that you are still at the hospital. It sounds like you have a great medical team and family support. I know that helps a ton!
I am thinking that they may try and implant the pacemaker when you are there? I’m sorry for all of the issues but so glad that you went in and they are taking good care of you.
It sounds as if the pacemaker would help you tremendously as well, so I hope they can do that soon. Please keep us posted and know we are here for support.
On a positive, my aunt just had a newer pacemaker implanted and it was a quick and much less invasive procedure than the ones I knew of years before.
Brittany and Colleen offer some great advice as well.
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