Guest guest Posted March 11, 2009 Report Share Posted March 11, 2009 , The work that's being done on HPS could eventually yield benefits far beyond that community. That's the thing about research, you just never know what the far reaching ramifications might be. A biomarker or two could only help in this battle and once we have that, the work can begin to find a way to prevent the development of the disease in folks who have the biomarker! How fabulous would that be?! Beth Moderator Fibrotic NSIP 06/06 Dermatomyositis 11/08 To: Breathe-Support Sent: Wednesday, March 11, 2009 2:37:11 AMSubject: Re: Just been diagnosed with PF I think more and more genes will be found for IPF - and so eventually the "I" will drop. It won't be idiopathic if you what the cause is. The really helpful thing about learning the genes etc. as we go along is that there is work being done on a biomarker for PF. The HPS patients at NIH are part of this research as we know 100 percent that those of us with an error on the HPS gene 1 or 4 will get PF. The NIH has studied us for 15 years or more and has blood samples on ice going back that far - thus they're able to test those samples for the biomarkers. Pretty cool. That means that if a gene does run in a family, it could be tested for and someday this marker could be monitored so that treatment starts at the earliest possible time, even before fibrosis is visable on a scan. It's really interesting stuff. I get sort of facinated if I can step back and forget for a moment that this is about my lungs too - grin! Hermansky-Pudlak Syndrome albinism 02 / PF 06 > > > > > > From: Jack Marshall mrshlljck (AT) yahoo (DOT) com>> > Subject: Re: Re: Just been diagnosed with PF> > To: Breathe-Support@ yahoogroups. com> > Date: Tuesday, March 10, 2009, 5:28 PM> > > > > > > > > > > > > > > > Ray, I'm a slightly different breed of IPF. I was DX in June 2005 with IPF which was subsequently confimed via biopsy in 2008. I am 80 years old, work full time managing my own business, putting in about 60 hours a week. There has been only a small progression of lung scarring. I am not on oxygen and have no need of it. I take no medications except for a cough medicine familiarly know as NAC. IPF has had no negative effects on my life, as it continues much as it always has been except for things that cause great exertion. I don't miss really heavy lifting, and I suspect that without IPF my age would make some those things unpleasant as well.. I accept that IPF is a part of my body along with all the other things that make me up. I listen to it carefully and have learned what makes it stir and so have made small modifications to keep it quiet. I know it is a terrible disease, but I do not dwell on that fact. I believe negative> > feelings are seriously unhealthy, so I have chosen to to view it more benignly and thus avoid negativism. One can say Jack, you are 80 years old and don't have many years left anway. Who knows? I could live another 10 or 20 years, and yes, I am greedy and want to, but with good health and positive attitudes. I am also fully aware that my health can change over night, which sometimes happens with IPF.> > When that happens, I will still work to be as positive as possible and keep on living.> > > > I write this to illustrate that what you read on line about very short life expectancies is not always the case. The day I was diagnosed, the pulmo said you have IPF and you may live just another three to five years. I said no, I will live until I am dead. I am nearing the end of year five and I am as healthy as I was five years ago. Put aside the notion that you have a short life expectancy and focus on living.> > > > You are in good hands here. There are many wonderul people in this group that will guide you and counsel you far better than any pulmo, and gently accept your bad times that you can vent here and enjoy your good times.> > Jack> > 79/IPF - UIP/dx06/05 Maine > > > > > > > > > > > > > > From: Beth mbmurtha (AT) yahoo (DOT) com>> > To: Breathe-Support@ yahoogroups. com> > Sent: Tuesday, March 10, 2009 3:29:57 PM> > Subject: Re: Re: Just been diagnosed with PF> > > > > > > > > > > > > > Ray,> > The internet is the best and the worst. There's tons of information on the internet but for you right now, it's all out of context. Until you have more information about your own situation most of what's out there is only going to make you feel more anxious. I'm not saying don't read it (cause I know from personal experience that's not going to happen), just take things with a grain of salt for now.> > There are over 200 different interstitial lung diseases that cause pulmonary fibrosis (lung scarring). Your prognosis very much depends on what type of ILD you have and what caused it. What is true is that treatment options are limited for most types of ILD. Life expectancy ranges from 3-15 years and beyond so it really is best not to jump ahead before you know what's going on in your own body.> > I would do two things. First, call and get an appointment with a local pulmo, even if it's 3 or 4 months out. You'll need him/her down the line. Second, call and get an appointment at Duke. The sooner you get that ball rolling the sooner you can start figure out what this is going to mean for you. > > Just as a point of reference. I am 49 years old. I was diagnosed in June of 2006 at age 46 with Fibrotic NSIP (non-specific interstitial pneumonitis. . ) I have been essentially stable since that time and though I can no longer work a full time job, I work part time and continue to lead a full and fairly active life with the help of supplemental oxygen. My life didn't end with my diagnosis, it changed course but I'm still here. Others on the board have had different experiences, some better than me, some much more difficult but we all battle on just the same.> > Welcome again! We're glad you are here!> > > > Beth> > Moderator> > Fibrotic NSIP 06/06 Dermatomyositis 11/08> > > > > > > > > > > > > > From: rayblackman86 <rayblackman86@ yahoo.com>> > To: Breathe-Support@ yahoogroups. com> > Sent: Tuesday, March 10, 2009 3:01:20 PM> > Subject: Re: Just been diagnosed with PF> > > > > > > > Thank you all for the rapid replies. After being told I had PF I immediately went on the internet and what the sites I've seen have had to say about the desease is horrible. Is the desease as bad as what is posted online? I mean could it take me away from here in the next few years?> >> Quote Link to comment Share on other sites More sharing options...
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