pulmonary hypertension

February 29, 2008

Mike;pulmonary hypertension can be one of five different types: arterial, venous, hypoxic, thromboembolic, or miscellaneous.Given your PASP was found to be to great ,leans towards pulmonary arterial hypertensionas far as having one or the other,PF or PH, that these docs seem to be fighting overit is my understanding that you can have bothbecause one of the causes of PAH is interstitial lung disease like IPF.did you ever get a second opinion on your biopsydid more than one pathologist look at your slideswhat studies did you try to get involved inthere are studies for mid to end stage IPF but maybe your PH is causing your ineligibility where do you liveBRETT UIP/IPF 11/07 40 Mi.Hi everyone,I joined this forum last summer shortly after receiving a biopsy report confirming I have IPF. I have not been an active participant but have tried to educate myself through all of your experiences as well through a multitude of google searches. From the forum, I have noted a lot of frustration being expressed by many of you when it comes to patient care. There seems to be a wide chasm between what you expect from your pulmonologist and what you actually receive. The fact they are "practicing" medicine probably has never been truer than it is with this disease called IPF. I think it is safe to say that all of us want to be around as long as we can in hopes that maybe some "miracle" drug will surface to put this monster to rest. What has troubled me is that I have been in contact with some of the best pulmonologists in the country and it surprises me to know they are not up to speed with these same (miracle?) medications in clinical trails because they are outside their immediate domain. It goes to show that we ultimately are responsible for our well-being and to do so we need to become better educated so as to not rely so heavily on these doctors who "don't know it all" afterall. I, too, am frustrated? During the reading of my biopsy report to my wife and I, we were given the details of life expectancies etc. but were told not to worry so much because I was in the early stages - that most people are not as proactive as I was to discover what my ailment was - so by the time they are diagnosed the disease has progressed. We have been trying to figure out what was meant by "early stages" ever since. A week prior to my biopsy, my function tests were performed and revealed among other things my lung capacity to have dropped almost 20% from the previous year to a reading of 59%. I know from your experiences, many of you are far below that 59%, but have you had such a significant drop in one year. I have been out of country for the most part since then (as my lungs cannot tolerate any cold air) and have been trying to get into a number of clinical trials. I have failed to be accepted into all those studies because my fibrosis is too far advanced. Now this last week I was rejected again! This time because my PASP (pulmonary arterial pressure) was found to be too great for that study. Again, according to one of the best care centers in the country, my disease is in its early stage yet I am not being accepted into any of these studies because my disease is too advanced. How can this be? A question I have is where are they finding these people who actually are able to qualify for their studies?So much for the venting and I apologize for all that. The actual reason for this posting today is my hope that some of you can shed some light on the term "pulmonary hypertension". As I indicated above, this latest study required a PASP reading. To start with I had an echocardiogram specifically to determine my PASP. There was difficulty in getting an accurate PASP reading but the cardiologist noted I had moderate hypertension. Next, I had an Esophageal Echocardiogram performed by a second cardiologist. This revealed a PASP of 65 mm Hg and notation again of moderate hypertension. These results were sent to the clinical coordinator as well as to my pulmonologist back in the states. The clincial coordinator told me my condition was too advanced to be in the study and that I needed to contact my stateside specialists in hopes of getting on medication to treat this hypertension. Well, when I contacted my pulmonologist in the Midwest (who we are scheduled to see in June) and his come back was "I have pulmonary fibrosis - not pulmonary hypertension"! When I emailed him back and told him I was puzzled because I had two cardiologists here in Mexico and a clinical coordinator in Florida who all state I have pulmonary hypertension yet he doesn't, his response was: "Never mind would require one hour for me to explain the different forms of pulmonary hypertension and the differences in prognosis and treatment. I guess when you come we can discuss at lenght. For now I will not discuss your care further via e-mail" Can somebody shed some light on this? I was under the impression you either have pulmonary hypertension or you don't. Does anyone know of these various forms of pulmonary hypertension he refers to? I know there are no doctors on this forum (perhaps there should be) but any insight you can provide on this issue will be greatly appreciated.Mike & Sandy brett bowserbrett@...

February 29, 2008

Brett, My intial diagnosis was made by a team at the University of Washington Medical Center in Seattle to include Dr. Rhagu and Dr. Mulligan who heads up the transplant department. This was seconded by a team at the University of Wisconsin Medical Center in Madison, which included Dr. Meyer who heads up their transplant department. Initially when I met with the staff at the University of Washington, it was their belief I had some form of hypersenitivity causing the fibrosis but the biopsy said otherwise. (The pulmonologist who I wrote about in my original email is not associated with the University of Wisconsin - in fact he is located in another city altogether). I have researched a number of clinical studies. Unfortunately, a number of these are out of reach for they are in foreign countries. Others I chose to turn down myself because they

were studies with medications that had been shown to be ineffective such as the combination of Prednisone, Azathiprine and NAC as well as a studies using interferon gamma (although there is a new one applying a mist instead). I have been turned down in three - perfenidone, bosentan and sildenafil. There is one at Hopkins that uses Thalidomide to treat the chronic coughs (which I have) associated with IPF. I have contacted them to find out if they would reimburse for airfare etc. I am on a list for the NAC study (used in conjuction with a steroid and placebo) to be started this fall so I am hopeful I can get in on that one. Which other studies are you familiar with that I may be able to apply to? Mike

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March 2, 2008

My wife and I are truly surprised by the lack of response on this

forum to our inquiry regarding pulmonary hypertension. Clinical

studies for treating IPF have shown a high percentage of IPF patients

also have pulmonary hypertension (PAH). In fact, several studies have

shown that percentage to be greater 50% yet we only had two members

reply. PAH in IPF can be deadly unless properly treated. That is one

reason why some of the current treatments for PAH are also being used

with the hope the drug will somehow slow down the progression of the

IPF as well.

I'll try rephrasing my question. Could those of you on the forum who

know you have pulmonary hypertension please tell me when you found out

you had PAH, what type of treatment are you receiving for it and has

the treatment been shown to reduce your PAH?

Mike

March 2, 2008

Mike,

I was diagnosed with advanced Pulmonary Fibrosis two years ago, but only this month was found to have slight PH. So far my doctor has not prescribed anything for it and feels it is not a problem yet. Hopefully there are others who have taken meds that have helped.

Sarcoid/PF 3/2006 California

pulmonary hypertension

My wife and I are truly surprised by the lack of response on this forum to our inquiry regarding pulmonary hypertension. Clinical studies for treating IPF have shown a high percentage of IPF patients also have pulmonary hypertension (PAH). In fact, several studies have shown that percentage to be greater 50% yet we only had two members reply. PAH in IPF can be deadly unless properly treated. That is one reason why some of the current treatments for PAH are also being used with the hope the drug will somehow slow down the progression of the IPF as well. I'll try rephrasing my question. Could those of you on the forum who know you have pulmonary hypertension please tell me when you found out you had PAH, what type of treatment are you receiving for it and has the treatment been shown to reduce your PAH?Mike

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March 3, 2008

Hi Mike, Sorry you didn't get replies you wanted but very few of us

have PH that I know of. I am going to have an echo. March 12. I think

that is the first step in testing for it. Not sure. I am sure hoping

and praying I don't have it. It has been three years and nine months

since dx for me. I am feeling fine, I am excited to have Beth

and Leanne coming to visit.

You didn't put your DX, Date with your signature. When were you Dx? I

think I remember it was recently.

Stick with this Air Family, we will ALL help you in any way you can.

If we don't have an answer we don't reply

Usually someone will have the same experience and reply quickly.

God Bless and Take Care of You.

Love and Prayers, Peggy

Florida, ipf 6/04

Worry looks around.

Sorry looks back,

Faith looks up.