Jeannie McClelland

Mortality high in systemic scleroderma with PH

21 posts in this topic

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)

 

This item was posted in the ISN Newsroom. Check the Newsroom every day for the latest scleroderma medical and support information.


Jeannie McClelland

(Retired) ISN Director of Support Services

(Retired) ISN Sclero Forums Manager

(Retired) ISN Blog Manager

(Retired) ISN Assistant News Guide

(Retired) ISN Artist

International Scleroderma Network

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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

(Retired) ISN Director of Support Services

(Retired) ISN Sclero Forums Manager

(Retired) ISN Blog Manager

(Retired) ISN Assistant News Guide

(Retired) ISN Artist

International Scleroderma Network

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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.

 

Big Hugs,


Janey Willis

ISN Support Specialist

(Retired) ISN Assistant Webmaster

(Retired) ISN News Director

(Retired) ISN Technical Writer for Training Manuals

International Scleroderma Network (ISN)

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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

Founder and President

International Scleroderma Network (ISN)

Hotline and Donations: 1-800-564-7099

 

The most important thing in the world to know about scleroderma is sclero.org.

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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,

 

Susie Kraft

ISN Support Specialist

ISN Chat Host

International Scleroderma Network (ISN)

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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.

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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

ISN Support Specialist

(Retired) ISN Assistant Webmaster

(Retired) ISN News Director

(Retired) ISN Technical Writer for Training Manuals

International Scleroderma Network (ISN)

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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!


Special Hugs,

 

Susie Kraft

ISN Support Specialist

ISN Chat Host

International Scleroderma Network (ISN)

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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

(Retired) ISN Director of Support Services

(Retired) ISN Sclero Forums Manager

(Retired) ISN Blog Manager

(Retired) ISN Assistant News Guide

(Retired) ISN Artist

International Scleroderma Network

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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

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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

(Retired) ISN Director of Support Services

(Retired) ISN Sclero Forums Manager

(Retired) ISN Blog Manager

(Retired) ISN Assistant News Guide

(Retired) ISN Artist

International Scleroderma Network

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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

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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


Amanda Thorpe

ISN Sclero Forums Senior Support Specialist

ISN Video Presentations Manager

ISN Blogger

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist

International Scleroderma Network (ISN)

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