[Deleted User]

I am currently participating in a clinical trial for a drug called Pulmonary Hypertension by dilating the blood vessels in the lung.  It is inhaled and I take six puffs four times a day.  Since the study is “double blind” I don’t know if I am getting the medicine or not, at least for the first phase of the study.   The first phase lasts four months and I visit the clinic once a month for PFTs and a walk test.  Once the first phase is done I can, if I want, participate in a long term study.

If I do the long term study I will receive the real medication  – the long term study is not a double blind study, and have to do follow-up on a quarterly basis.   Since this is all done at the same place I have my regular pulmonary checkups and lab work it should not be a problem.

Aside from the inconvenience there is no cost for me.  All the tests and medications are paid for, even in the long term study.  I do get $50.00 for each “trip” I take which is supposed to help with my gas and pay for time but nothing I’ll get rich on.

I like the idea of being able to  help doctors add to the body of knowledge in fighting this disease.  When I researched the test medication I learned it had no long term ill effects so I didn’t do well with it I would expect a quick and complete reversal of any bad effects shortly after discontinuation.  Nor did I have to stop or alter taking any of my current meds.  I would say one of the biggest benefits it having access to a medication early.  If I do find it beneficial I will be able to continue taking it.

[Charlene Marshall]

Hi Alex,

Thank you for sharing…this is really fascinating to me! Were you diagnosed with PF at the same time as being diagnosed with PF, or did it come awhile later? I know sometimes it can come on after living with PF for awhile due to the pressure on the heart that poor lung function can cause. I’m assuming you’re still in the first phase of the study? How long have you been on the inhaled drug? Do you think it is making a difference (ie. do you suspect it is the drug or a placebo)? How long is the length of the long-term study? Sorry for all the questions, I’m just really interested, and agree with you completely about wanting to help Doctors add to their knowledge base when it comes to treating this disease.

So, just curious then: would dilating the blood vessels in the lungs improve oxygenation, keeping your ability to breathe more stable? I don’t imagine it would do anything or address the issue of fibrosis development in the lungs but even to be able to breathe more freely would be such a benefit. This sounds like a really promising study. Thank you so much for sharing!

 

 

[Deleted User]

I was diagnosed with PF 2012 and PH in 2016.  My PH is mild.  I started the research just before Christmas 2017 and as for it making a difference, it really is hard to tell.  Sometimes I think it is, then I just wonder if my mind is playing tricks on me…  The long term study is 2 years.

As for the dilation of the blood vessels in the lung,  that is the tricky part.    What I have learned is that the lung restricts blood flow to the parts of the lung that are damaged. Any improvement of blood flow to the damaged areas can be a bad thing.  For example one of my docs was telling me that giving dilation drugs intravenously to kids having a real bad asthma attack made their O2 levels go down.   Basically pumping blood through the parts of the lung where it won’t get oxygenated is counterproductive.

The thought is that using an inhaled drug that it will only reach the good part of my lungs and not the parts that are damaged by the fibrosis which would help the PH and improve my overall oxygenation.  That’s the thought anyway and it’s why they are testing.  Time will tell.  Thanks for your interest.

[Charlene Marshall]

Hi Alex,

Thank you so much for sharing this with us! Was there any confirmation or suspicion from your Doctor (my guess would be yes…) that the PF contributed to the development of PH? I am regularly being sent for echo’s which of course checks the pressure throughout your arteries and heart, and I’m always told this is just for “precaution”, but I suspect they are checking for PH as well. I’m glad your PH is mild though!

That makes sense re: it being hard to tell if the medication for the study is helping. If I were participating in it,  think I would be so mindful of it, that it would be easy for my mind to play tricks on me too, as you mentioned.

Wow, that is so interesting! I’m glad you (and your Doctor) explained that because I guess I wold have thought that sending oxygenated blood to damaged areas of the lung would help “repair” the damage in a way (for a visual, I thought it might be similar to  letting air at a laceration, which helps dry it up and heal) . However, I suppose if the damage is irreversible (as in the case of scarring in the lungs from PF) then sending blood to that area would be counterproductive, as you say. So, if you opt to go on the long-term study, the one that is 2 years, your progress will of course be measured by physicians but will you get to know whether it is making a difference for you throughout the study, or will you have to wait until the end? Again, sorry for all the questions, I just love research!

Have a nice day,
Charlene,