This topic contains 49 replies, has 23 voices, and was last updated by  Kathleen Sheffer 1 year, 1 month ago.

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  • #11077
     Kathleen Sheffer 
    Participant

    I had a heart-lung transplant on July 1, 2016, and I no longer have pulmonary hypertension. In the 16 years before my transplant, I was on continuous intravenous Flolan and later Remodulin. I used supplemental oxygen at night, on planes and with colds. Additionally, I tried Tracleer and later Letairis. Revatio made a big difference for me and I took Adcirca for a period in college.

    So, I have experience with a number of approved therapies, but I’m interested in knowing what medications you are taking. If you have questions about specific treatment options, feel free to create a new topic on the subject. Learning from other patients’ experience made a significant impact in my ability to manage my PH therapies, and I hope this forum can do the same for you and your loved ones.

  • #11170
     April LeMaire 
    Participant

    I’m switching from Remodulin to Flolan in the next couple weeks (a hail mary hoping something sticks). I’m nervous because it seems so much less stable than the Remodulin and my doc says the GI side effects are no joke. Any pointers?

  • #11176
     Kathleen Sheffer 
    Participant

    Hmmm I went the opposite direction with my switch and gained a lot of weight once I went on Remodulin so if you are concerned about losing weight, I’d definitely watch out for that – GI side effects probably play a role (I didn’t want to eat, especially at breakfast time).

    I think what you mean by less stable has to do with its shorter half life. I always kept an insulated bag with me with a backup cartridge and pump in case something went wrong because you do not want to be grocery shopping and have to go home to change it (which I could do with Remodulin’s longer half-life). This also prompted me to get a permanent disabled placard so I could leave it in the car and access it quickly.

    Really hoping you respond better to Flolan – the additional side-effects will be worth it if you can breathe easier!

  • #11184
     Tim Neal 
    Participant

    Hi,I am new to all this and I am on Remodulin Iv SC, can you please explain what the term ” half life” means cause I am kinda confused.

    Thank you!
    Sincerely
    Tim Neal

    • #11191
       Kathleen Sheffer 
      Participant

      Hi Tim! Great question – I sometimes forget that not everyone grew up with medical terminology like I did.

      From Google: A medication’s biological or terminal half-life is how long it takes for half of the dose to be eliminated from the bloodstream.

      Basically, a medication with a longer half-life will stay in your system longer than one with a shorter half-life. Flolan and Veletri have a half-life of approximately 6 minutes. These medications are administered continuously, but if the medication is discontinued for whatever reason, the patient will likely start feeling symptoms of withdrawal after about 6 minutes. In contrast, Remodulin will stay in the bloodstream for hours even after a discontinuation.

      Does that make any sense? I’m trying, hah!

  • #11193
     Tim Neal 
    Participant

    Thank you, yes that really makes sense now.
    I really appreciate you taking the time to answer my question and look forward to more Q and A topics with you.
    I will probably have a lot of questions for you and other PAH patients about treatment, medications…ect as time goes
    on (hopefully) for me. Its been a long hard 8 months since i was diagnosed with Sever PAH and really haven’t gotten
    any real answers for how and why i became sick other than I probably have had P.A.H for several years and my hart and lungs had finally gave out due to working so hard but to me it seemed that one day i was great and very next day i was passing out like every 20 to 45 minuets witch was extremely scary and after going to a lot of different doctors that finally a Pulmonary specialist in Pueblo Colorado diagnosed me with sever pah and told me that there was no cure for it and depending on how i reacted to the treatment that if i changed everything about me i might have 2 to 4 years left. So needless to say I’ve been looking for answers any where i could. So thank you for taking the time to answer my question about “Half-Life”.

    Once Again THANK YOU!
    Sincerely
    Tim Neal

  • #11216
     Kacie 
    Participant

    I have been taking Veletri since 2015 and added Adcirca in 2016. The Veletri is a room temperature stable (for 24 hours) version of Flolan and easier to manage but it costs more. The Adcirca added a small boost to my capabilities. There were GI issues for a long while on Veletri but I eventually overcame them with careful food choices. After 3 years on Veletri, I feel stable and I’m overall happy to be on it.

  • #11220
     Sandra Cornelius 
    Participant

    I was diagnosed in 2017 so this is new for me too. WHO class 1 pah. I am currently on Opsumit and the max dose of Uptravi. I have underlying polymyocitis and interstitial lung disease so I am also on Immuran and Prednisone. Cardiologist not sure that Uptravi is doing anything. Working with my pulmonologist on treatment plans. A bit scary. Waiting for next echo and 6 minute walk.

  • #11221
     Jen Cueva 
    Participant

    Hi everyone,
    I was diagnosed in 2015 and have taken most all of the oral meds through the years ! I currently take Revatio 20mg ( 3 pills =60mg) 3 times per day as well as the 1600mcg of Uptravi 2 times per day as well as O2 and lots of diuretics.
    I’m not familiar except from hear say on IV, SubQ, or inhaled meds, but most oral meds I’ve tried at one time.
    Welcome you all to thr forum.

    Sandra , it is a bit scary but having a good PH Specialist that you work well with as well as educating yourself as much as you will help. Remember, one breath at a time. Don’t read and believe all you read ( most ) on the internet. Pulmonary Hypertension Association site is a great start .

  • #11263
     Chris Olesen 
    Participant

    My 9 year old daughter is on sildenafil three times a day. Her pressure went from low 80’s down to low 40’s over the last two and a half years since she was diagnosed. We were doing 6 month echo’s due to the success.

    Our two most recent echo’s have seen her numbers go from low 50’s to where she was on this past Tuesday of 70’s.

    She was sick in Feb with pneumonia but our doctor wants a second opinion on her meds and is sending us to the Lurie Childrens hospital in Chicago.

    Any thoughts on our case would be greatly appreciated and congrats on the health!

    • #11271
       Kathleen Sheffer 
      Participant

      Hi Chris,

      It definitely can’t hurt to get a second opinion from a PH Specialist. I’m glad your doctor is encouraging you to do so. PH is a progressive disease, but there are treatment options. I’m thankful my parents made the decision to stay me on IV therapy right away (my pressures were above 100) and continue to choose the most aggressive treatment strategies until I was old enough to choose them for myself.

  • #11266
     Junais Bulbul 
    Participant

    I am not ready to surrender to this disease. When I was born, I was not well. I am 29 years old. I take all the car bikes without major problems I am taking medicines properly tadalafil. Cardivas amifru40 mg
    I am taking these medicines.

  • #11279
     Razelle Shulman 
    Participant

    I am on Warfarin, Slow-Mag, Spiractin, Furesemide, Sildenafil and awaiting Ambrisentan. I was diagnosed in June 2013 and am 58 years old live in Cape Town in South Africa.

  • #11283
     Diana Procopio 
    Participant

    I am 80 years old. I’m taking Adempas 1.5mg 3xday. I was on Letairis and Adempas, butdeveloped hearng loss. I had been really short of breath for a few years, pulmonary doc told me to lose weight, but it didn’t help. My family doc noticd edema in my legs and sent me to a cardio doc. Upon doing a right heart catheritization, it was discovered I had PAH. I am doing silver sneakers classesat the gym and some weight machines. Feeling much better with the Adempas.

    • #11340
       Kathleen Sheffer 
      Participant

      Great to hear you are doing better, Diana. It’s a shame how often the PH diagnosis is delayed by doctors who don’t look past obvious factors – my pediatric cardiologist attributed all my shortness of breath to asthma for years.

  • #11770
     Charlotte Gilbert 
    Participant

    I’m on veletri, sildenafil 3 times a day, lettairis and bumex ( water pills)…. my biggest side effects on veletri increases are nausea with severe dry heaves… like I pulled an all night of drinking but no good stories to tell… my biggest side effect is abdominal water weight gain. I have to get that drained every 14-17 days…. doc is hoping that cranking up the veletri and water pills will help….

    • #11771
       Brittany Foster 
      Keymaster

      Charlotte,
      I can relate to the frustrations of the extra fluid retention in the stomach. That must be awful to have to get it drained. Do they do that surgically? I’ve had fluid retention and am on water pills once a day for it but haven’t had it drained before. What do they do to test that to see how much is in there?

      • #12874
         Charlotte Gilbert 
        Participant

        They remove the extra fluid via paracentesis. Takes at the most a half an hour. They insert a small catheter after they find the pocket of fluid via ultrasound, which then drains I to a vacuum bottle… I have pain afterwards from change in size etc…I just had one yesterday… the procedure itself is not painful.. just a little lidocaine Injection…

  • #12487
     Randolph Reynolds 
    Participant

    Just yesterday I began taking Remodulin yesterday. The apparatus (pump, cleo, etc.) is something new for me and since I am rather impatient it seems it will be a while before I get accustomed to putting up with. One problem is getting anxious about it and losing my focus.

    Anyone have any suggestions for making the infusion process less cumbersome?

    Thanks

  • #12609
     Judi 
    Participant

    Is anyone taking tyvasol I
    just started up to 4 breaths
    I also have interstitial lung disease so I take Cell cept and oxygen 24/7.
    Is anyone else on these drugs?

    • #12635
       Kathleen Sheffer 
      Participant

      Hi Judi! I haven’t taken Tyvaso, but I sometimes take Cellcept to prevent organ rejection (I’m off it now, but may go back on if my white count changes). I was on oxygen before my transplant. What symptoms do you experience taking Cellcept?

  • #12815
     Judie 
    Participant

    I am new to all of this. I am newly diagnosed with PAH stage 2 because I do have symptoms. I have had right heart cath, CT of chest and lung scan. I saw my doctor today and he ordered Cialis. I am to take one dose for 2 weeks and then double the dose. I see my Pulmonologist in a month and we will begin second line of treatment. (I have systemic mast cell disease so meds have to be added with caution and 4 days of Benadryl around the clock. My reactions have always occurred in the first 4 days.
    The cost of Cialis is high. The Lilly assistance programs I do not quailify for under income. They only state for ED so I am pursuing possibly partial coverage. My insurance doesn’t cover it end of story. The good Rx brings it down to $3500 per month. I am not willing to compromise my chances of preventing progression of this disease. Any thoughts would be appreciated. I read online cealis May become generic in September. I am challenging my health insurance.
    Judie

    • #12818
       Kathleen Sheffer 
      Participant

      Definitely challenge your insurance! Your doctor can write a letter saying that the drug is medically indicated and not receiving it would be potentially life-threatening. Those are the key terms we’ve learned to use with insurance companies over the years.

      Adcirca is the brand name approved to treat PAH. You might see if they will cover Revatio (Viagra) if not Adcirca (Cialis). Revatio worked wonders for me – kept me off the transplant years for 13 years! I was on Adicrca for a bit, too. I hope this class of medications works wonders for you!

    • #13031
       Chris LaRose Mev 
      Participant

      Judie –

      When i 1st took Adcirca (Cialis), i recd financial assistance. Check out:

      http://www.rxassist.org/pap-info/company-detail?CmpId=269

      chris

      • #13033
         Brittany Foster 
        Keymaster

        Thanks for the link!!! 🙂 I’m sure it will help others too.

  • #12846
     Judie 
    Participant

    I am halfway through week one on Cealis. I can feel ease in my breathing although my symptoms are still there with exercise 🙃. I double the dose a week from Saturday, I feel ready to increase it now but of course I will follow my doctors instruction. When I return to see the Pulmonologist on 8/23 we will add a second med. I have no idea how the choice is made with so many available choices. Any insight on this would be great! It is felt I have PAH bc I have an underlying connective disease disorder, Ehlers Danlos Classical type and Rheumatoid Arthritis. I have put myself on a liver cleanse supplement bc I am detoxing so many things via my liver. I will try to investigate the different meds and come up with one I feel appropriate. My Pulmonologist includes me on decision making which a greatly appreciate. I have to say the cardiologist and Pulmonologist were given to me bc the cardiologist I had retired and Pulmonologist recommended were booked months out so I got the first available and I think he is great. The universe appears to have been on my side. Excellent knowledge and very compassionate personalities. I feel lucky.
    Judie

    • #12851
       Kathleen Sheffer 
      Participant

      I’m so glad you are starting to feel some improvement, Judie! It’s working fast! Sounds like you are taking charge of your health, and have a great team. Adding one med at a time is a good strategy to make sure you can correlate the symptoms to each one. I always find it a heavy responsibility that our doctors rely so much on how we say we feel. Helps to take note of changes as they come so we remember trends in the long term. You’re on the right track!

    • #13039
       Chris LaRose Mev 
      Participant

      Judie

      It is wonderful that Adcirca is helping you; after 6 mos on it for me, i showed no improvement, my sPAP was still in the mid-90’s. My PH Specialist (cardiologist) decided we better add IV Remodulin, which did get my numbers down a lot. So it really depends how sick you are. 2 yrs later we added Opsumit and after 6 yrs on Remodulin, transitioned to the pill version, Orenitram, which i am currently @ 3 mg tid. i believe a key in treatment is timing. PAH is progressive so it’s really not a good idea to wait too long before treating. All the stats that i’ve seen say without treatment, the ave life expectancy is 2.8 yrs.

      chris

      • #13042
         Brittany Foster 
        Keymaster

        Chris,
        You are right. When it comes to PH time is crucial ! It seems like many, including myself had a hard time getting the correct diagnosis. Once people have est that then it becomes about treatment and best options. Treatment is very individualized! I’m glad they are able to give you an oral form of that medication now. Keep pushing forward!

      • #13043
         Brittany Foster 
        Keymaster

        Chris,
        You are right. When it comes to PH time is crucial ! It seems like many, including myself had a hard time getting the correct diagnosis. Once people have est that then it becomes about treatment and best options. Treatment is very individualized! I’m glad they are able to give you an oral form of that medication now. Keep pushing forward!

  • #12862
     Randolph Reynolds 
    Participant

    Agree with Kathleen. By the way I’ve never heard of different stages of PAH. What are they?

    Cheers

  • #12863
     Judi 
    Participant

    I’d like to know too

  • #12892
     Kathleen Sheffer 
    Participant

    Hey @ripple76 and @judi – thanks for asking! The classification of PAH can get a bit confusing because there are basically two separate branches of it. This article explains it all. PH is classified by groups depending on the cause of the disease. For instance, CTEPH falls under Group 4, which refers to “cases of the disease caused by blood clots in the lungs or blood clotting disorders.”

    What @jady1958 is referring to when she says she is “stage 2” is the World Health Organization’s functional classification of PH. This is determined by the severity of symptoms and level of functional ability, on a scale of 1-4. Patients in class 1 experience very little symptoms, and patients in class 4 experience severe symptoms even at rest.

  • #12896

    Hi I’m Teresa,
    I was diagnossed with Severe PH in 2008. I started taken Tracleer, some time later I was added Revatio and sintron (anticoagulant), and then Remodulin. With these it was with the one I had some side effects, being that every time I had to punch the medicine in a different place it hurt a lot for about a whole week. I think I’ve been lucky though because i only had to change the place every three ir four months (my doctor didn’t have anyone show lasted so Kong so she didn’t lets me have it longer).
    Now I’m transplanted and feeling great! And taking cellcept, prograf and prednisone (for inmunosuppression), and azitromicine, septrim and bisoprolol…
    😊 Teresa

    • #12899
       Terry D. Blissett 
      Participant

      Theresa what events led up to your diagnosis? Other health factors?

    • #12917
       Kathleen Sheffer 
      Participant

      Hi Teresa! When and where did you have your transplant? Double lungs? So glad to have you here!

  • #12930
     Peter G Pierce 
    Participant

    I’ve traveled to high altitudes (Tibet, Machu Picchu, Santa Fe) for years without incident until March 2015 when Drs at ER in Pagosa Springs CO said I had altitude sickness. I was finally diagnosed with idiopathic WHO 1 PAH in late October 2016 by Zhudi Transplant clinic in Oklahoma City after three trips to National Jewish in Denver and elimination of interstitial disease and diastolic dysfunction. Can’t figure a cause beyond undertreated sleep apnea. Have had every test known to man including a stress cycle with arterial O2 sampling getting my BP up to 284/160 and stress right heart cath which clinched the diagnosis. Being treated by PAH clinic at Integris in Oklahoma City. I’m an unusual case because I am for the most part asymptomatic. I am comfortable and functional with O2 sat levels in high 70s-low 80s. [Use 5 liters continuous at home] I just was using O2 when diagnosed because by O2 sat level drops from 90s to low 80s with walking around. Suspicion is that I’ve had PAH for years and just adapted to it. Within 60 days after diagnosis, I was on 40 mg Adcirca (Cialis) daily. In May Letairis 10 mg daily was added. At that time my echo was 83. I began titration for Orenitram in August 2017 when echo pressure was 87 and in December reached dosage level of 3 mg three times daily 8 hours apart. I’ve experienced no side effects beyond nasal congestion. My body wants to retain water so I take 100 mg torsemide over the day; that pretty well handles the fluid problem. Echo a couple of weeks ago had a pressure of 37, quite a result that we attribute to the Orenitram combined with Adcirca and Letairis. I had a 75-minute heart MRI in March that showed no damage to right heart and normal functioning. Last 6 minute walk was 477 meters. My PAH for now is just a chronic condition that limits my activities to battery life of my Inogen One (5 liters pulse) and prevents me from flying commercial or going much higher than Denver (no more Santa Fe). No more golf. High heat (108 here tomorrow) and high humidity limit my activity but that’s pretty normal for anyone. I no longer have trouble with humidity when temperature is below 75. I’m type 2 diabetic but well-controlled (A1C 5.9). I retired in 2014 and have Medicare with United Health supplements for part B and Part D. Meds cost north of $200,000 annually but my retention is only about 10% which I am fortunately able to absorb. I’m playing the hand I’ve been dealt and with great physicians and remarkable meds and family support so far have been extraordinarily lucky.

  • #12935
     Terry D. Blissett 
    Participant

    Update:

    Just met with the doc. Specifically, the VQ scan showed that blood is no longer going to a certain area in my right lung. It also happens to be the exact same spot where my pulmonary embolism was. A pulmonary angiogram has been ordered for next week and will give them more information on exactly what is blocking that flow. They have scheduled the angiogram for Thursday morning. I will update everyone then.

    • #12941
       Brittany Foster 
      Keymaster

      Hi Terry,
      good luck and keep us posted. I know that it can be scary when given medical information like this, but at the same time it must be somewhat of a relief to have some type of idea as to what is going on. I hope they will be able to use these tests and come up with a game plan soon for you! How have you been feeling with symptoms during all of this. The waiting around can be so draining too!

      • #12944
         Terry D. Blissett 
        Participant

        Brittany I have good days and bad. Mostly good. I’m not on any meds for this. The only exercise restriction I have at this time is to reduces intensity based on shortness of breath. For the most part I can exercise. But after exercise I still get bouts of fatigue that can zap my desire to exercise for up to a week.

        Once a certain amount of time has passed, I am refreshed and ready to go again. I also am battling symptoms of ptsd that occurred with this PE. I feel like it is directly related physiologically to the residual damage from the embolism. That’s my theory anyway.

        Will keep you guys updated.

      • #12945
         Brittany Foster 
        Keymaster

        Thanks for sharing with us Terry and for keeping us posted. Remember, we know our bodies best! If you have a gut feeling about something make sure to mention it all to the doctors. The patients need to be a part of the team too!!! You are your biggest advocate.

        I can relate to the fatigue after exercise all too well. I try to do what I can, but on the bad days the motivation just isn’t there because of lack of energy. I know the zap feeling you’re talking about. Hugs to you and positive thoughts sent your way for upcoming tests and results !

  • #12936
     Judi 
    Participant

    Good Luck

  • #12938
     Judie 
    Participant

    Thanks for sharing Terry. Good luck with your upcoming test.
    Peter, so you are one 3 meds for PAH? I am in the beginning of trying to figure out what will work for me and what will not. I also have RA which I am on xeljanz for and finally have gotten it reapproved for another year. Without prescription coverage the cost is $35-40 thousand a year. The Cialis hasn’t been approved and cost about the same. My doctor I on vacation for another week. Once he returns I am going to have his office push the insurance company to pay for the Cialis. We will be adding a second drug which I am sure is also a costly one. With my systemic mast cell disease meds I am also around $200,000 Mark also. Luckily my insurance covers the bulk of it. I feel lucky that I can absorb the copays it makes me sad to think of others who can not afford the greatest new drugs that are out there, are they to sit back and die? For Cialis alone for the most 2 people in a family can make to get assistance $49,000 per year. The $49,000 is not enough to cover the drug or just barely. Unless they would get it for free. But one must send 2 years of tax returns to be scrutinized.
    My classification was the WHO criteria, II bc I do have symptoms. I feel ready to up the Cialis to 40mg but I have to wait another week.
    Judie

  • #12946
     VK 
    Participant

    Some days I fail to realize how lucky I am to not need PH meds to function in daily living. For now my lung meds are just bronchodilators and clonidine to let me actually exercise without collapisng on the floor in a heap of breathless chest pain.

    On the other hand, no need to worry that my liver is bored, because I’ve also got to take 5 other medications for other conditions ranging from premature osteoarthritis to depression. 🙂

    • This reply was modified 1 year, 3 months ago by  VK.
  • #13079
     Jan McCrory 
    Participant

    Is anyone on Opsumit 10 mg? I started it a few weeks ago, but now I am experiencing my feet and stomach to swell. Does anyone else have these symptoms?

    Thank you!

  • #13126
     Terry D. Blissett 
    Participant

    Okay so I got the results or conclusion of my VQ Scan and Pulmonary angiogram. My lung specialist said that I DO NOT have CTEPH. And not only that, she said that I do not have pulmonary hypertension. I am both shocked and thankful. All the signs were certainly pointing towards PH.

    The specialist recommended I go see the pulmonologist and cardiologist again for treatment of my ongoing shortness of breath, fatigue, etc.

    I am also being treated for PTSD, anxiety and depression all resulting from the PE. I’ve been on lexapro since January. I’m getting off of it now. Hoping that will decrease my symptoms along with exercise.

    Will keep you posted on what the pulmonologist and cardiologist says.

    • #13140
       Brittany Foster 
      Keymaster

      Thank you for the update Terry. Always good to hear from you. I’m glad that the scans didn’t show signs of this and I think that it is good that they are planning on still monitoring you and sending you to a pulmonary and cardiologist. Wishing you nothing but the best ! 🙂

  • #13145
     Michael Morale 
    Keymaster

    Hey Terry, my father had shortness of breath for a while and his cardiologist put him on Lasix. His cardiologist told him that he had a small buildup of fluid around his heart and that was causing his shortness of breath. After taking Lasix for about three months, he changed his diet and began working out and that seemed to have corrected that problem. I’m not saying that this would be right for you, but you may want to ask your doctor. I hope this helps.

  • #13543
     Kimberly van Soest 
    Participant

    @kathleen-sheffer

    Hii Kathleen, I just read your story about both taking adcirca and revatio and I was wondering how these meds worked out for you?

    I had to stop taking adcirca due to an allergic response and next week I have to start with revatio. Where there any side effects that you think are worth mentioning? And how did you feel about adcirca?
    Thank you

    -Kimberly

    • #13546
       Kathleen Sheffer 
      Participant

      Hey @kimberlyvansoest! I switched to adcirca in college because taking it once a day worked better for my schedule. When my health began to decline, I switched back to revatio and felt better. Better meaning I could walk further without stopping. Unfortunately the medication comes with some serious side-effects. It’s difficult to pinpoint which symptoms were from revatio because I was on IV therapy as well as several other medications. I think I had some dizziness from it, but the most bothersome side effect was diarrhea. I was on a VERY high dose (100mg three time a day at the end) and had diarrhea every day. So much of my time was spent in a bathroom or in bed with stomach pain. Hopefully a lower dose will help you and you won’t have the same side effects I did. Revatio significantly reduced my pulmonary pressures and allowed me to postpone transplant a couple times – I call it my “miracle med.” Let me know how it goes. I hope you don’t have an allergic response to it – that sounds awful!

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