Uptravi/Selixipag side effects

[jen-cueva]

Hi @amit-ramsinghani, I apologize for your mother’s pneumonia and low oxygen rates. I would guess this is partly because of pneumonia. That’s a lot to add to already decreased lung functions, like in most with PH.

I’ve been on Uptravi for years since it was first approved. As the PH team should inform you, it takes time to titrate or increase to the best dosage for your mother. If she recently started, this is probably partly what you are seeing in addition to the difficulties pneumonia has on her lungs and body. Have you asked her medical team their thoughts on why this is happening? I would suggest you ask them since they know much more about your mother’s PH stages and her other health concerns.

I tagged you in another post where others share their experiences with Uptravi.

I know that because CellCept is used as an antirejection drug, it lowers immunity. So, if her lungs were already impaired, this may be something to speak with her medical care team about.

As a daughter and caregiver, this must be difficult for you. Are you taking breaks for yourself? Do you have other support that can rotate the care with you? Please know we are here and want to support you as you go through this challenging time with your mom.

[Colleen]

@amit-ramsinghani I’m sorry to hear about your mother’s pneumonia and oxygen desaturation.

I don’t have experience with the treatments you mentioned except for CellCept. As @jenc mentioned, it is primarily used as an antirejection medication but is also known to be used sometimes for people with PH and/or Lupus.

My son was prescribed CellCept a few months prior to his transplant and then of course for several months after. It’s supposed to help improve breathing function.

Here is a link to case reports of PH patients who were placed on CellCept.

[Amit Ramsinghani]

1)My other question was her Pulmonary pressure was 50 this was done by the ECHO not by right heart catheterization as the Dr said she may not tolerate it.  Is the PH pressure of 50 of concern?

2)and how many years does one live on average once diagnosed with Pulmonary Hypertension?

3) Has any one done a lung transplant at 74 years old female or older?

4) Any alternative to taking steroids, I believe they weaken your immune system?

[jen-cueva]

Hi @amit-ramsinghani, I was diagnosed 18 years ago this past Valentine’s Day. So, it all depends on what stage someone is at, other coexisting conditions, and overall health. It also depends on whether treatment started early in a diagnosis or later once the PH has progressed.

A pressure of 50 on Echo is something I would suggest an RHC is done. However, it sounds like your mother may have some underlying issues like pneumonia, which would make this a higher risk at this time. Did you ask the PH team there why they thought she might not tolerate the cath? What did they say?

Steroids help breathing and lungs, but they have side effects. It’s a Catch-22.

Can you talk to a social worker or case manager for support there? It sounds like you have so many unanswered questions. We are here for you, but her medical team is more familiar with her condition.

I’m sorry because I know this must be so difficult watching your mom and having so many unknowns. I’m sending some positive thoughts and light your way. <3

[Md. Abul Kalam Azad]

I would like to know what is CellCept. Could you explain about it.

[jen-cueva]

Hi @azad, Cellcept is primarily used for anti-rejection for transplant patients. However, it’s also used for other reasons, too.

Below is a link I found at Cleveland Clinic that offers some information on this medication.
CellCept

How is your son doing this new week?

@amit-ramsinghani, how are you? How’s your mother doing? You’ve been quiet, so maybe you’ve been busy with her and talking with her medical care team.

[ky]

Hi @amit-ramsinghani, my husband has PAH and we lost our daughter to PAH a little over a year ago and around 5 years post diagnosis. I tried desperately to find answers on life span expectancy and was convinced my husband would follow our daughter right out the door, but every case is different and there is new medications being developed each year. Try to keep hopeful and continue searching for information. My husbands team didn’t know much about Uptravi until I brought it to their attention. Although our daughter was already on the pump and couldn’t get it once my husband got “bad enough” he was finally given the new drug.

As for the transplant questions once your mother is bad enough to even be considered you will meet with a transplant team and they can answer all those questions regarding transplant options. A lot depends on all underlying issues, illnesses, stability and mental health. I don’t think that just age is a consideration more overall health in general. I will say our daughter was trying to get on the transplant list with pressures between 90 and 110 for the last year and and half of her life but never qualified.  Every transplant team is different and every individual is different.

Your mom might respond well to just lower lever oral medication for a number of years before any second level medication is needed and she may never need a transplant. Sending you and your mother love and light!

[jen-cueva]

What a lovely and helpful response from @kygon to @amit-ramsinghani and your mother. This is why the forum members here are the best!

Sharing our experiences, despite the pain and heartache, we all want to help others. We learn so much from each other and are such a supportive group.

Thanks, Ky and everyone, for opening up and sharing some vulnerable moments to help others.

@amit-ramsinghani, Ky’s reminders for you and your mom are excellent. We are all vastly different, as are the medical teams caring for us.

Remaining hopeful is essential to mental health. I was diagnosed 18 years ago; the initial diagnosis was overwhelming and frightening. That’s why I do what I do to share my experiences to help offer some hope and light to others.

How are you doing this week? How are things going with your mom? Is she back at home yet? I’m sending you both extra love, hugs, and positive thoughts.

[Amit Ramsinghani]

Has anyone used a combination treatment of all 3 drugs– OPSUMIT, UPTRAVI, and ADEMPAS?  How were your results?  Did you benefit or worsen in terms of your oxygen requirements?

[jen-cueva]

Hi @amit-ramsinghani, I have been on Uptravi and Adempas together. But not Opsumit with those. I was on Opsumit before starting Uptravi.

I’m now on Uptravi and Revatio, and other miscellaneous medications. The combination of the goal of treatment, especially triple therapy, would be to improve oxygenation. However, if the treatment is not the best for that person, the oxygen needs might increase.

How is your mother doing? How are you managing this week?

[Janet Blumenshine]

I was diagnosed with PAH when I was 72 and I am now 81.  (Scleroderma caused my PAH).  I had a right heart cath and my pressures was in the 50’s plus range.  I have been on Uptravi, Opsumit, Revatio (all max doses) and also nifedipine for the last 8 years or so.  My pressures are keeping steady going up just slightly in these 8 years.  I only use oxygen when I exercise.  I asked the doctor about how long will this last and he said it will get gradually worse.  So far 8 years have been good….there is always reason to hope.  I believe these new medications for PAH are extending life.  I’ve had no real bad side effects.  I also was on Cellcept for about 6 years for my scleroderma.  I was just taken off of it now for about 3 months.

My doctor was big on oxygen and combination drugs early in the disease.  Everyone is so different but hoping for better days for all.

 

[jen-cueva]

Hi @jblum, thank you for sharing your experience with your PH treatments at your age. You are correct; we must always hold on to hope.

You mention your pressures have increased. Do you think your symptoms have worsened, too? My PH doc says how we feel more important than the numbers.

Like your PH team, mine also usually starts with combination therapy. Since I was diagnosed over 18 years, I’ve been extremely excited about the research and development of new treatments for us in the PH community. When I reached the point of needing IV treatments, a new oral medication was approved. I’m incredibly grateful to be on all oral medications with my oxygen.

How have you been? Whenever I see your name here, I think of Judy Blume, an author. Hehe. Thanks for sharing.

@amit-ramsinghani, how are you and your mother doing?