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Mortality high in systemic scleroderma with PH


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#1 Jeannie McClelland

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Posted 25 February 2009 - 03:21 AM

Mortality high in patients with systemic scleroderma and pulmonary hypertension. The prognosis for patients with systemic sclerosis complicated by pulmonary hypertension is "grim," particularly among African Americans and those with interstitial lung disease. Reuters. Arthritis rheumatologist 2009;60:569-577. (Also see: Pulmonary Involvement)

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#2 Jeannie McClelland

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Posted 25 February 2009 - 03:29 AM

In the article it states that:

"Survival at 3 years was 39% among the 20 patients with interstitial lung disease-associated pulmonary hypertension, and 64% among the 39 who had isolated pulmonary arterial hypertension. In multivariate analysis, those with interstitial lung disease were 5 times more likely to die during follow-up than the rest of the cohort."

I'm an odds-beater. I was diagnosed in the fall of 2006 with scleroderma and was told I already had ILD and PH~ I had a blip in the March-June 2007 PFT's and started on CellCept. The ILD and PH stabilized. Then I started on Letairis and it's improved the PH immensely. I also was started on simvastatin. My 3 years are up in 7 months and I plan on throwing a party! And since the odds were 39%, I also plan a roll-back of my age to 39! Hahaha!! :lol:

Remember our motto - never, ever give up. And motto #2? Push for the testing and care that you think you need.

Hugs to each and every one of you.
Jeannie McClelland
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#3 janey

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Posted 25 February 2009 - 05:39 AM

You go girl! It's articles like this that remind us of how serious the disease is, however, it also provides more of a challenge to beat it.

I was diagnosed in the summer of 2003 with sclero/PM, then heart involvement in January 05, then PH and ILD in fall of 06. The ILD was isolated in the lower lobes of the lungs. I was immediately put on Bosentan for the PH and have been on Cellcept for almost 2 years now as my immunosuppressant. The Bosentan stopped the PH in its tracks and my PA pressure is back to normal (as per an ECHO) - which I know is not the solid method of determining PA pressure, but the risk of a right heart cath were too high at the time. My O2 levels would drop into the 80's percentiles with any amount of walking so I was prescribed supplemental oxygen. Now I only need it when I sleep and exercise! The cellcept has stopped the ILD and in fact, the crackling noise in my lungs is hardly audible to the doctors.

So :P to the odds being against us! We're beating this beast.

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Janey Willis
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#4 debonair susie

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Posted 25 February 2009 - 11:37 AM

I like the way you think, Jeannie and Janey!

Thumbs up to the power of positive thinking!

Let's continue to be each other's cheerleaders...
I'll bring the pom-poms!! Rah! Rah! Rah!
Special Hugs,

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#5 Shelley Ensz

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Posted 25 February 2009 - 03:45 PM

Jeannie and Janey, you both set outstanding examples for us to follow, in every way. Jeannie, we will certainly all be here to cheer when you reach the 3-year mark.

Thankfully, there are some promising treatments for pulmonary hypertension now. As your examples show, it is very important to hold on tight to hope with scleroderma, because that actually helps enable healing to take place (with the mind-body connection).

You go, goils! And the same for everyone who is fighting to beat the odds with any disease. Every day is a victory and a cause for celebration!

So Susie, grab your pom-poms and give another round of rah-rah-rahs. This time, could you do your cool jump with the sideways splits where you touch your toes? But try to stop screaming in pain, will you please?! It disrupts the people who are sleeping in the back row of Sclero Forums. :blink:
Warm Hugs,

Shelley Ensz
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#6 debonair susie

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Posted 26 February 2009 - 11:06 AM

I sure second all of what you said, Shelley!

Sure will!!! Creak, crack, crackle, creak!!!! Please don't pay any attention to the noise my knees and hips are making...I'm "a little" out of "practice"!lol Ooch! Ouch! I DID it...the front to back splits! Whew!!!
(Pssst....Could you help me up please, Shelley? I can't seem to get my legs back :P )
Special Hugs,

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#7 JustME

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Posted 26 February 2009 - 11:42 AM

About how old were any of you when you diagnosed? I'm just wondering if it is being diagnosed earlier than before so we are able to live longer.

#8 Jeannie McClelland

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Posted 26 February 2009 - 12:16 PM

Age at diagnosis? 59 - a YOUNG 59!
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#9 janey

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Posted 26 February 2009 - 01:11 PM

I was diagnosed at 49 years young. I think the increased awareness of doctors and the new medications have had a great affect on decreasing the mortality rate. In fact, kidney disease use to be the cause of the highest mortality rate and that's drop WAY DOWN. You might want to take a look at our prognosis and mortality page. A lot has changed in the past 10 to 15 years.

big Hugs,
Janey Willis
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#10 debonair susie

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Posted 03 March 2009 - 10:34 AM

I just got a call from my rheumatologist.

She told me that my pulmonary function test (PFT) showed a bit of change and on the ECHO report, she said there was POSSIBLE high pressure on the right side of my heart.

She gave me the name of a doctor she wants me to see and asked that I make an appointment to see him. I did and am to get a chest xray and do the "walking test" and then will see the doctor, all on Thursday.

So, will let you know when I know what I'm supposed to know!
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#11 Jeannie McClelland

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Posted 03 March 2009 - 10:52 AM

Wow, I am ever so impressed by your rheumatologist and also your scheduling abilities!! It's Tuesday and you are getting all that done on Thursday?? Well done, you!

NOW - you aren't going to fret and stress, are you?? We'll do that for you, all part of the service.

Warm and loving hugs,
Jeannie McClelland
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#12 MajorMark

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Posted 03 March 2009 - 11:35 AM

Hello, I was just diagnosed 2 months ago with interstitial lung disease (ILD) and severe pulmonary hypertension (PH). I was non symptomatic 3 months ago with the exception of Raynaud's. I have crackling lungs and and am now on oxygen 24 hours a day. I am having problems getting a prognosis from any of my doctors and have just started to go to the Univ. of Michigan for treatment. I know that your success stories have given me hope, but I am on prednisone now which you know messes with your hormones and moods. I have been very depressed and feel that I will not be one of the 39%. I just had a small bout of bronchitis and thought I was going to not make it, I was fighting for every breath. My lung involvement is severe with sarcoidosis in 4 places in lungs. I feel as the doctors at the University know my prognosis is grim. I am a Nurse by trade and and when I entered the emergency room I was ready for admission having listened to my lungs myself before going. They sounded terrible. I may not have had pneumonia, but should have been admitted for IV antibiotics. My hope is that they have not given up due to severity of my disease which now includes scleroderma, pulmonary hypertension, sarcoidosis, Raynaud's, interstitial lung disease, and duodenal ulcer due to scleroderma esophagus.

I guess I want to know how long it took from diagnosis to treatment of the drugs you mention. I will be honest, it has gotten so bad, I have constant chest pain and severe shortness of breath with activity. My only symptom was Raynaud's for about 6-7 years prior, so I wonder if my clock started ticking back then. Any thoughts would help.

Mark

#13 Jeannie McClelland

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Posted 03 March 2009 - 12:06 PM

Dear Mark,

I am so sorry about the severe health problems you are experiencing. I hope we can give you the hope, support, and information you need to fight your battle.

Let me start by pointing you at some pages of information:

Sarcoidosis

ILD/Pulmonary Fibrosis and Pulmonary Hypertension are on our Pulmonary (Lung) Involvement page

Depression

As a nurse, a lot of this will be familiar to you but reading it again from the patient's perspective might be helpful. You may be able have a list ready of questions and suggestions for your specialists and take it from me, the minute I became a partner in managing my own care, the better I felt emotionally. Let your doctors know you aren't giving up and that you expect them not to either. :) Be your own best advocate.

You asked specifically how long it took to get from diagnosis to treatment. I guess the short answer is that it varies with everyone. I suspect your doctors at the Univ. of Michigan will want to have a good look at your particular medical situation before deciding on course of treatment and that process is hardly ever as fast as we want it to be. I'm a pushy sort of person and did what I could to facilitate the process, including hand-carrying medical records, test results, etc.; calling to ask if the doctors had reviewed them; and pushing for the earliest possible appointments. I also asked the doctors I was already working with to intervene on my behalf in order to speed things up.

Please keep in touch and let us know how you are getting on.

With best wishes,
Jeannie McClelland
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#14 MajorMark

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Posted 03 March 2009 - 12:39 PM

Thanks Jeannie. I have never been on the other side of the health care spectrum. I really appreciate your support and I will start being proactive in my care and treatment. I've got to get rid of that awe of doctors- won't be hard at U of M, they are all 20 years younger! LOL I am so glad I joined this site. I need help at times and I have already answered some questions that I knew answers to.

Thanks,

Mark

#15 Amanda Thorpe

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Posted 03 March 2009 - 02:40 PM

Hello Mark

I am sorry you are having so many health issues all at once! That you were once "in the trade" will help you be a better advocate for yourself as you know what should be done and what to insist be done for you.

As you were a nurse perhaps your doctors are being more open about your prognosis and maybe this comes across as if they're resigned to a particular outcome.

I hope that you post more because we would like to support you and get to know you better. This forum is a safe place where you can be honest about your hopes and fears as there is always someone feeling or has felt the same thing.

Take care.

Amanda
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#16 Shelley Ensz

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Posted 03 March 2009 - 03:10 PM

Dear Mark,

Hello and welcome to Sclero Forums! I'm sorry it is because of scleroderma and severe lung involvement. But this is definitely the right place to be.

At the University of Michigan, did you get in to the scleroderma center in Ann Arbor? That is a very top notch center and on the leading, cutting edge of treatments for scleroderma and pulmonary hypertension. You'd be quite hard put to be in better hands.
Warm Hugs,

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#17 Shelley Ensz

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Posted 03 March 2009 - 03:19 PM

Hi Susie,

I just caught your message that you might have flunked your ECHO. Fortunately for you, they are not a reliable test for pulmonary hypertension (PH). Which means they will probably need to run other tests, like a right heart catheterization (RHC). When it comes to that, I've heard it both ways, that the RHC has picked up things the ECHO didn't detect at all, and also that the RHC has determined pressures were fine that looked goofy on the ECHO.

I'll be sending good wishes your way. I'm pretty sure all your readings could have been off due to your acrobatic rah-rah-rahs for us all, or maybe from laughing too hard at some of the postings in Sclero Den. That surely must be it!

Let us know how things go on Thursday. We're all rooting for you!
Warm Hugs,

Shelley Ensz
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Hotline and Donations: 1-800-564-7099

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#18 Wohali

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Posted 03 March 2009 - 04:46 PM

Does it mean anything if mild pulmonary hypertension (PH) shows up twice on Echos, then the last Echo I had was normal 3 months after the last time PH showed up? I mean should I start counting down to 3 years? Are there symptoms of PH other than things that show up on Echos? Like breathing problems or something?

#19 Penny

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Posted 03 March 2009 - 04:58 PM

Hi, all,

I wanted to drop a line here about the whole percentage thing.

I have always been on the small side of the percentage wagon.

85% of people hit head on by a 18 wheeler doing 45mph while driving a sub-compact will not survive and 99% that survive will have serious injuries. I broke one bone in my hand and scratched up my forehead and was eating pizza three hours later after leaving the ER. (1989)

I was sterile, no children in my future, then the above mentioned accident and 10 months later along comes my daughter. (1990)

Birth control (two forms) left a 0.001% chance of pregnancy. Along came my son (1992)

Surgical mishap (blood pressure went up, they treated it. Blood pressure went away, they gave me a fluid overload to try to get it back filling my lungs with fluid and enlarging my heart 4x normal.) 5% chance of surviving the night in CCU and 15% chance my heart would recover. Well, I made it and my heart went back to normal size with only slight thickening to the left ventricle. (1999)

I think that percentages are a challenge and not a fact; a call to arms. I remember waking for a moment in the CCU that first night and hearing some nurses saying how I would not make it though the night. That did not scare me, it made me mad. No one tells me what to do! :lol:

Percentages are the number of people that fall into the "normal" ranges of any one thing. Well, we are not normal, so :P to percentages!

Hugs all around,

Penny

#20 debonair susie

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Posted 06 March 2009 - 08:50 AM

Thank you for your support, Shelley!

Here's the deal, so far! I did "the 6 minute walk" and did well. Walking on the flat doesn't bother me...it's the stairs that are MY culprit! They took the chest xray. The doctor is heading up a PH Clinic that covers quite a vast area, so I feel in good hands!

I was originally scheduled for the pre-admission and RHC for Tuesday and Wednesday, but there is only one doctor who performs the test and he only schedules these for Mondays. So!

I'm now scheduled for the pre-admission on Friday, March 20th and the Right Heart Catheterization on Monday, March 23rd.


I was so grateful for the down-time between now and then, as I want my mother to be doing much better than she has been, over the last few weeks.

At any rate, it will be good to find out what's what, in regard to PH. We'll know when we know it, right?!

How right you are, Penny...how right you are!
Special Hugs,

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