Mycaphenalate and Tyvasol-still looking

[Paul]

I am enrolled in an open label trial of Treprostinil via inhalation 4x daily. My use of the open label medication began in January 2023 following a year in a blind trial. By the end of 2022 symptoms of PAH had appeared. My diagnosis of IPF was made following a biopsy in June 2018. My symptoms first appeared in December 2015. I am in the care of a well experienced Pulmonologist and am examined/tested quarterly by the associated Research Division.

Treprostinil has resolved my PAH. My current trial is intended to test the effect of this medication upon the actual fibrosis. In recent years we have arranged annual high-resolution CT scans to observe and compare the fibrosis progression. As of March 2024 the fibrosis has shown no change as compared to the prior year, an encouraging sign.

I live alone and maintain my home and grounds (about six acres) on a tributary of the Chesapeake Bay, which affords opportunity for useful exercise.

I have no illusions with respect to my vulnerability to respiratory infections, so for all practical purposes I have discontinued all but the most essential travel. I have maintained the personal protections of COVID and I do keep my vaccinations current.

My personal impression is that Treprostinil is beneficial, and for that reason, I would intend to continue to participate in this trial if permitted to do so.

With best wishes,

Paul.

[John K. Grubb]

I WAS DIAGNOSED WITH IPF IN LATE 2021. STARTED ON OFEV IN APRIL 2022.

I WAS NOT SATISFIED WITH MY DOCTOR SO IN APRIL 2024 I SWITCHED TO A RESEARCH PULMONOLGIST. HIS ASSESSMENT IS THAT I MAY HAVE IPF OR PULMONARY HYPERTENSION — THAT IT IS TOO EARLY TO TELL. HE IS CONDUCTING A DOUBLE BLIND RESEARCH STUDY USING A TYVASOL VAPORIZER TO ADMINISTER TREPROSTINIL. I STARTED TREPROSTINIL INHALATION IN MAY OF 2024.

ONE OF THE REASONS HE SUGGESTED I PARTICIPATE IN THIS STUDY IS THAT TREPROSTINIL IS ALREADY BEING USED TO TREAT PULMONARY HYPERTENSION; THE PURPOSE OF THE CURRENT STUDY IS TO SEE IF TREPROSTINIL CAN HELP REDUCE THE EFFECTS AND DAMAGE OF IPF.

I AM 80. WORK OUT SEVERAL TIMES A WEEK. I HAVE TAKEN PULMONARY REHABILITATION CLASSES. EACH SESSON IS 44 TIMES, 1 HOUR EACH, GENERALLY TWICE A WEEK. I HAVE COMPLETE 4 SESSIONS AND AM IN THE PROCESS OF SIGNING UP FOR MY FIFTH SESSION. NOT USING OXYGEN. GENERALLY MY OX LEVEL IS 96-100. MY NORMAL FORCED VITAL AIR CAPACITY IS GENERALLY IN THE 62-66% RANGE. MY DLCO IS LOW – AROUNG 43% CAPACITY.

SINCE I AM IN A DOUBLE BLIND STUDY I DO NOT KNOW WHERE I AM GETTING TREPROSTINIL OR WATOR VAPOR.

[julian]

United Technologies or United Therapeutics? I worked for United Technologies for 28 years. A huge mostly aerospace conglomerate, but I don’t believe they ever made pharmaceuticals. They sold off most of their businesses several years ago to Raytheon.

[george-poulsen]

I may have misquoted. sorry. It is united Therapeutics Corporation (UTHR)

Here is from their website. I am looking at getting my doc to add this to my Micaphenylate regime. So far I understand that the effect of a treatment appears to be rather short term like 15 minutes or so, is that right?

[Steve Dragoo]

Hey George – that’s great what you are doing. Is the Inogen % pulse or continuous flow for you?

Steve

[george-poulsen]

I use the Inogen G5 portable during the day and a couple of the transformer/chargers (from Inogen or a lot cheaper from Amazon) that plug direct to the Inogen, The G5 is a pulse. What I like is curing my stupidity. I was buying high priced $260-$300 batteries all the time since I was constantly on the portable and changing batteries like every 2-3 hours at my daily settings of 4-5 LPM equivalent). Now I just have 4 batteries period.

That was crazy. Now since I bought several of the chargers ($30 plus on Amazon) instead of those rip off direct connect chargers for the batteries themselves. that plug into the front of the G5 unit, I can simply have a charger at my family room and another in my office and another in the shower area plugged in, in morning with a 20′ cannula so I can take the oxy cannula into the shower at a higher rate and still come back out to dry off wile using my unit at L6 and yet still have total portability to my car etc. Those plug in unit chargers both charge the battery in the unit even while running in front of the TV (with or without the battery in the unit) and just unplugging charger lets me instantly go portable with the battery still in but more charged each time to the BR or Kitchen or into the car. When I go to the car (wife usually driving) I have a cigarette lighter type charger cord in each car for instant switch to 12 volt charging) and again can even use without the battery in the unit itself making it lighter a bunch)

At night I use the Inogen At-Home constant flow. (only goes from 1-5) but I have found that recording OXY saturation on a recording watch at night I can maintain 97-98% saturation on just the 3Level (approx 3 LPM equivalent) and the special recording watch (VIHealth) still lets me print-off a neat multicolorgraph from several years ago to-now of Oxygen / lung progress or lack of. Very interesting how uplifting it can be to see kind of effect of barometric seasonal changes (weather etc) and their effect on my RA/PF/ILD. I fell worse with approaching storm fronts several days away and more quickly have a “good” day when the Barometer begins to change downward, cool stuff)

This has raised my interest in the PAH side of this and to see if I cant maybe hit the lungs and scarring with the Mycaphenolate and then go to the (Tyvasol or Treprostinol) to expand the right side heart arteries to convert more air to oxygen like they used to do 100 years ago with Nyacin and other artery openers like Albuterol etc.

No prof’l here and not very brilliant even but am dogged in researching and looking at my alternatives until I have none left. I am convinced we have many, many, many alternatives on the way down the garden path to us but the R&D phases and FDA phases deeply need another faster hand on the throttle in our best interest. (kind of like Covid resolutions, in my mind). Above all I feel someone like the Local Govts could take a hand in stopping some of these mfrs from ripping us off on pricing. I just bought my units which not everybody can do, makes me feel better, but I am convinced that Medicare is paying a very strong price and not in the interet of giving us better care products either. Maybe we just have to invest in a few of the mfrs and then attend a Board Meeting and make ourselves heard somewhere?? Wonder if Warren Buffet uses oxygen yet, or Bill Gates–Kids. Thoose guys owuld do someting if they had to breathe on 1/3rd the air for a few days or lifetimes? Maybe a special invite to one of the Annual meeting of the Foundation, or we get the Prophet to buy up a few of these groups and make some lower margin and price point units for a batch of folks with no cures—-yet? {I don’t sound like this if I get ranting so I must be feeling good right now}

I forgot who mentioned the issues about OFEV and coughing and phlegm. That’s been a major with me a northerner since born in Ohio and now Texas and allergies are a big part. I am convinced it is mostly just allergic phlegm, (which plain ol’ Benadryl seems to help when I go to bed, also helps my personal sleep) but mine also involves blood, which the ENTs and Docs have said is just exposed blood capillaries from in the nasal area up stairs and they continue seeping/ bleeding openly in bed at night from drying effect of Oxy and guess where they exit in morning when clearing throat and nose. So I use Guaffenissin at night and again in morning to thin mucous somewhat quickly and let me get rid of it for another lovely day:-) of oxygen.

Ent’s have also said they could do some procedure with a quick knock out and reduce the exposed area of bleeding (I guess they mean burn it a bit). All of this assumes there’s nothing more serious happening that could be life threatening..gads!

Forgot to say or repeat that I use a stroller walker almost everywhere to carry my Inogen concentrator since it leaves a hand or two free for doors and a cup of coffee. But a lot of folks don’t realize just how much strain a stroller(wheeled) can put on an 84 year old back either and then we wonder why we hurt somewhere else too.

That’s ok though, it keeps the Docs wondering what I’m coming back for each time :-). I’m slowly figuring this whole thing out if my progression will stay somewhere above the last DLCO of 28-32 and FVC above my last 78% Pred. At least I am mobile, and occasional CBD can be a little better than Tylenol or Aspirin. For me, any of the pain meds seem to have a numbing effect on whatever tickles my throat to make me want to cough and then start even the throat into breaking down blood vessels.

If this jumble of word salad of sorts bores you than you might just delete if but once I start, I have a lot of thoughts and things I’ve tried and its hard to make it all sound like literature. Have a good day!

[Cutter]

I was diagnosed with IPF 2 years ago and started perfenidone shortly after. Then was diagnosed 6 months later with Pulmonary Hypertension. At that point start Tyvaso 4 inhaled doses per day. Recently had appointment with my pulmonologist with a 6 minute walk and pulmonary function tests. The numbers show significant improvement so something is working for me. The pulmonologist told me that they are seeing that Tyvaso is showing some promise.

[george-poulsen]

I use a finger oxymeter to know my saturation. The Inogen pulse units put out the rough equivalent of oxygen that you would have gotten from a constant flow device but they only put it out when you breathe in triggering a repetitive series of pulses kind of like an aqualung. It charges a set of tubes with a zinc mineral powder the absorbs the Nitrogen out of our air providing a bit more enhanced Oxygen mixture is my understanding. You can look it up on the internet as to “How do oxygen Concentrators work”.

Their Constant flow units just blow the same enhanced oxy mix from Zinc filter towers constantly across the nasal area so that you waste at least half your breathing out time without breathing in any enhanced air (but now there are those medallion units that supposedly provide kind of double time oxy by storing the part blown at you while you are exhaling and feed it back to you when you breathe in.

I have seen negative feed back on comfort of those medallions, but maybe someday the mfrs will listen to the patient instead of the designer who never had to use one of these. Good day all.