This topic contains 13 replies, has 4 voices, and was last updated by  Kathleen Sheffer 1 month, 3 weeks ago.

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  • #13402
     Kathleen Sheffer 
    Keymaster

    Lung or heart-lung transplantation is the only treatment for end-stage pulmonary hypertension (PH). I have seen many patients resist going through with transplant evaluation, putting it off for years until their situation becomes critical. Many of my friends who have passed away from PH were on the waiting list for transplant, but missed their window.

    Having been through transplant evaluation three times myself, and seen all that goes into determining status on the waitlist, I can say for certain that transplants are not performed unless absolutely necessary (when the choice is life, or death). Evaluation can never be too early. The “worst” outcome from an early evaluation is that the transplant team will send you back to your PH team with a broadened perspective of long-term care. Transplant teams emphasize diet and exercise to build strength in preparation for the operation. They may advocate that you look into more aggressive treatment options if available (I had maxed out on all options when I had my transplant, and they will want to see that before listing you). They might recommend pulmonary rehab and/or a new diet plan, either to gain weight or lose some.

    Going through evaluation with a transplant team means you meet physicians that may handle your care in the future. This helps you build a relationship with them before you have a real emergency. I don’t know about you, but I am not at my best when I am in emergency situations. I was grateful that some of the team had met me when I was a bit more stable. You’ll also have a chance to learn more about your local transplant center. Based on your experience there, you might want to check out other programs. These decisions are more easily made when you are not in a crisis state.

    If you haven’t seen a transplant team, WHY? Has your doctor brought up the subject at all? What’s stopping you from learning more about the process? I really want to hear from you. Is it your physician who is preventing you from discussing all your long-term options? Or is something else?

  • #13421
     Kathleen Sheffer 
    Keymaster

    Thanks for the inspiration, @brian-gilbert! Would love to have you share about your experience as well.

  • #13451
     Brian Gilbert 
    Participant

    My wife, @charlotte-gilbert was diagnosed with IPAH on 9/28/2013. After diagnosis, her doctor educated us and encouraged us to learn as much as possible about the disease. We did. During our research, we learned about new meds potentially hitting the market, we read study after study about PH, we found new friends on Facebook, and joined local support groups. We felt we were as informed as we could be about PH. Nothing about transplant.

    Charlotte’s condition stayed about the same to being a little better after diagnosis and getting meds onboard for the first 2.5 years. Then over about a 1 month period, she started feeling slower. Her o2 levels dropped to low 90’s, and her walk distance became shorter. Having done the research that we did, we were close to Mayo in JAX and decided to go to their ER. They admitted her and almost immediately started her on IV therapy (Veletri). No mention of transplant. Only that Veletri is the ‘gold standard’ for this disease. Her condition improved and we were hopeful that this was the med that will keep this disease at bay. She now was on oxygen, but only while she slept and maybe during the day at home if it was hot outside.

    Being closer to Atlanta, we switched docs again. We did a few check ups, a few dosage increases on veletri and kept on trucking. No mention of transplant. Last year, she had a lot of water weight come on over a couple of months time and her dependence on oxygen grew. Her doc did a right heart cath to get updated numbers. He admitted her. They removed the fluid, increased the veletri and discharged her several days later. The only mention of transplant was when she asked, the doc said they certainly don’t take that option off the table. Nothing about evaluation or her needing one so we didn’t research. We felt confident if transplant was on the radar that her docs would’ve mentioned it.

    3 months later, water weight is becoming an issue and needs to be drained about once per month. Doc increases Veletri and water pill.

    Meanwhile with the water weight, she’s eating less because it’s harder for her to breath. She’s lost several pounds Over the past year.

    3 months and her water weight is having to be drained twice a month. We go for a check up, doc increases veletri and said it would be irresponsible not to discuss transplant. He said she should get evaluated in case the veletri doesn’t get her turned around. She’s had more weight loss.

    In July we came to Duke University Hospital for transplant evaluation week. It’s tough. Some people call it hell week, others call it hope week. In a meeting with one of the docs, his first question was “Why did you wait so long to seek transplant?” What!?!We explained we didn’t know it should’ve been an option. More than one doc said they wished she would’ve come in a year ago. The year ago was when the fluid first started coming on and the veletri was adjusted. Why would we consider transplant at that point? After meeting with many docs, social workers, and having tons of tests done, we knew we needed to move her care to this hospital regardless of the outcome of the evaluation. As expected, Duke said no. But, there were conditions she’d have to meet to get the answer turned into a yes. They wanted her under their care. We moved. Saw her new doc and he admitted her. More tests, and they’ve started to decrease her Veletri. She’ll be starting pulmonary rehab soon in hopes that she’ll improve so she can be accepted into their transplant program. They’ve already said if she can meet certain goals, they’ll take her on. She’s been malnourished which didn’t help with the abdominal fluid or her condition. She’s got to gain some weight, and be able to move more and get stronger. Nutritionists, therapists, and docs have all helped with suggestions.

    Knowing what we know now, I want to encourage PH patients to get evaluated for transplant early. You and your docs will have to define ‘early’ and it will be different for everyone. You know your body better than anyone else. The signs are there. Listen to what your body is telling you. Don’t wait until the last minute. If your health has declined beyond recovery, you will most likely not be eligible for transplant. We’re grateful that Charlotte still has a chance.

    • #13460
       Brittany Foster 
      Keymaster

      Wow Brian,
      Reading your story really has opened my eyes to how important it is to advocate for ourselves and the ones we love and care most about. It’s great that she has you on this difficult part of life. I can only imagine the stress that you are going through. For different reason, i have heard the words “i wish this was caught sooner”. It makes me think back to all the things I could have done, the signs I might have missed, the tests i didn’t get. I have to remind myself so much to stay in the present and be grateful that it was caught at all, but think about hiw fast things progressed makes me mad that I couldn’t do anything about it sooner. This is when it gets important to do EXACTLY what you are doing and spread awareness. Reach out to others so they aren’t waiting around, make sure other people you know get the early interventions they might need and ask the questions even if it feels wrong. Thank you so much for being an advocate and spreading awareness for all of us. I wish you both nothing but the best and I know how difficult and stressful this is. Try to stay as hopeful as possible and know that you have a community of support behind you!

      • #13490
         Brian Gilbert 
        Participant

        Hi Brittany! You’re right. We can’t worry about the ‘what ifs’. We have to make the best decisions we can make at the time the decision is required using the information we have. It’s definitely tough. Thoughts of ‘what am I missing now’ keep creeping up in my head. Having seen her new doc here at Duke a couple of times and getting to know the staff here, Knowing what I know now, PH patients should all understand that it really does take a team of docs/nurses/therapists to manage PH and the patient as a whole. Numbers being off don’t necessarily mean something is going wrong. They’ve put Charlotte on a new water pill and so far it’s been working incredibly well vs the previous ones she’s been on. She did her first day of rehab and loved it. Tomorrow is day 2 and she’ll go daily for 2 hours from now on. She’s consuming more calories to gain weight and is able to eat more because the water is staying off with the new pill. The lower dose of Veletri seems to be helping as well. She doesn’t feel as sluggish. Rehab monitors her progress and communicates that directly to the transplant team. As soon as she’s able to meet goals, she’ll be officially accepted into the transplant program and continue on her current path until transplant.

      • #13499
         Brittany Foster 
        Keymaster

        Hi Brian,
        It certainly does take a team effort ! And the patient should always feel as if they have a say as part of the team too (and equally as important for family members like you to feel as though they are part of the team) . I’m glad that she is benefiting from the change in the medication and the change of the dosage. I hope she continues to make good progress to move forward with the transplant. I know from personal experience how hard it was and is for me to eat when I have extra water weight especially in the stomach. Not getting in enough calories truly puts a damper on my physical as well as emotional health! So I hope increasing the calorie intake will make a positive difference. Please continue to keep us updated. I’m thinking all good thoughts for you both!

      • #13503
         Brian Gilbert 
        Participant

        For the weight gain, we found Muscle Milk Weight Gainer as well as Ensure Enlive to be high in calories while still being low in sodium. She said she can drink the Muscle Milk and not feel full (16oz) so she’ll still be able to eat a meal. I feel like we’ve been able to double her caloric intake over the past month-ish by adding these supplements.

      • #13505
         Brittany Foster 
        Keymaster

        That’s great advice Brian! Thank you! I’m a big fan of making shakes out of those supplement drinks. I like doing it for a dessert and blending in some ice, peanut butter, and some oreos for good flavor and extra calories. Plus it’s less filling than an actual ice cream shake but still just as yummy! My go to is nutrition and calorie dense shakes or smoothies when I feel like it’s hard to eat actual meals.

    • #13491
       Kathleen Sheffer 
      Keymaster

      Hey @brian-gilbert, is lowering the Veletri one of the requirements for listing? Like you and @brittany-foster, I never stop worrying I’m missing something. The paranoia comes with the territory, I guess.

      So glad Charlotte enjoyed pulmonary rehab and you are working toward the option of transplant. The nice thing is that the steps you’re taking for the transplant team are good for her overall health and have the potential to delay the need for transplant. Win-win (sort of). I know this is a stressful and emotional time. Hugs to you both!

      • #13492
         Brian Gilbert 
        Participant

        Thanks @kathleen-sheffer. They’re lowering the Veletri for two reasons. First, because her blood pressure was consistently low and secondly because the initial dosage was based on what she weighed on 4/1/16 when she started Veletri. Her weight has changed as she’s lost about 40lbs since and the dosing calculation has never been updated. They feel that because she was on such a high dose, the meds could’ve inadvertently dilated her system as a whole contributing to the low bp. Since lowering the dosage she hasn’t had the feeling of low bp and her doc feels her heart is working more efficiently now on the lower dose. They’re wanting her even lower in hopes to optimize its effectiveness allowing her to build up to transplant strength. She’s turning her pump down by 1ml per day for the next 4 days. I’m certain they’ll do a right heart cath in a month or so to measure pressures and make sure the lower dosage of Veletri isn’t having a negative impact on her PH. If they don’t, we know to ask. : ) The doc explained today that they want her heart working as best as it can to prevent the need for heart/lung transplant. He said he’d rather only do lungs if they feel they can offer that as an option. He went over the numbers, blah blah blah, you know how that goes. It’s data, but not necessarily data that will apply to any one individual. She had lab work done after seeing the doc. The lady who took her blood said she pulled blood from a lady earlier that was celebrating 7.5 years post double lung transplant. We love hearing those stories. They’re very inspiring during uncertain times.

      • #13509
         Kathleen Sheffer 
        Keymaster

        That makes sense! Thank you for clarifying why they are adjusting the dose. Again, I’m glad she is getting comprehensive care from the team at Duke. There are so many success stories, and I’m glad you’re hearing some of them! The recipients who are doing really well aren’t often present in the hospital for obvious reasons. If you don’t catch them for their annual visit, you won’t meet them. I loved getting to meet multiple recipients more than 20 years post-transplant at the Transplant Games. I have so much hope for long-term success.

  • #16779
     Sherry Berry 
    Participant

    Hey Brittany,

    I’m glad y’all are talking about Duke. I have two appts coming up in June and Sept. I’m going to talk to them again about transplants since my last visit it was unknown that I had PH,CHF,and PF. My PH went undiagnosed for 10 years. I’m on a lot of medication, but none of the meds that y’all mention. I tried silendafil, however, I had really bad headaches, I had heart palpitations, I would get dizzy and pass out. My Critical Cardiologist told me to stop taking it, yet he didn’t offer anything else, and to continue on my regular meds. So basically they are just prolonging my life. I’m on a Furosemide 160 mg twice a day, Spironolactone, Amlodipine, Ramipril, Predisone, Metropol, Potassium, Dulera, and Spriva, O2 6 to 8 ltrs 24/7, Bipap w/ O2 at night. I did go through Pulmonary rehab last year. I may need to do it again and I need to lose 60 lbs but its hard when you’re on steroids all the time. Any suggestions. My Pulmonary doctor agrees I need a transplant, reason he sent me to Duke in the first place however he’s not sure if I’ll make it through the surgery, because of my past two hospitalizations and surgery. I guess all I can do is pray.

    Best Regards,
    Sherry Berry

    • #16784
       Brittany Foster 
      Keymaster

      Sherry,
      Wow! It certainly sounds like you will be going through quite a lot in the next few months. I hope that in June they can talk about necessary steps towards transplant with you. If they don’t then please bring this up to your medical team and write down all symptoms and changed that you have noticed since last seeing them. I’m sorry that it just feels like they are prolonging your life at this point and like you’re not getting enough benefits from all the medications and oxygen that you’re on. As far as the nutrtiion and weight loss goes, I would highly recommend asking your PH team for a few names of dietitians who have worked with PH patients or patients who are on various medications that may impact weight gain/weight loss. Please keep me posted on your upcoming dates for your appointment and what they say. My thoughts and positive vibes are with you!

    • #16795
       Kathleen Sheffer 
      Keymaster

      Hi Sherry, I’m sorry you are facing the possibility of transplantation. The process can be very scary. It can also be life-changing and miraculous! Going through evaluation is a good thing and will help get you on the right track to be healthier regardless of whether you go on the list. Let me know if you have any questions about transplant as you get further along in the process. Praying for you as well. <3

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