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Crest And Cardio/lung Involvement


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#1 Maddy07

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Posted 09 September 2007 - 10:57 AM

Okay, stepping in gingerly to make my first inquiry. First, hello all! In short I was diagnosed with Raynaud's and a positive ANC back in '96 and told CREST might be in my future, it might not.

I lived a busy life since then and never felt the need to be seen. I knew over the years that I have the R, some S, and lots of T going on and that I'm officially CREST or Limited as they call it now. But there was nothing to treat so I never took the time to be monitored. Besides, my dear husband is military and we move frequently so follow up can be difficult.

Anyway, my loooooong story is that a year ago we moved ot North Carolina and I lost my voice the same week. We attributed it to the new area and allergies and life long history of asthma.

It took several months (and my denial) before we had an ENT diagnose me with a paralyzed vocal cord. My left fold doesn't move at all and I had severe shortness of breath. With the vocal cord sitting in an open position all the time, shortness of breath (air wasting) was not a surprise symptom to me, the doctor, etc.. was just part of the vocal cord problem.

We did a CT scan in Jan of this year to check for growths on the nerve of that vocal fold. The scan was clear, BUT showed a mild pericardial effusion. First time I had that niggling "uh oh" feeling ya know?

We had to assume (this doctor is really really top notch in his field of voice and swallowing and I adore him) that there was just some viral damage or infection on my nerve and we had to give it time to heal.

We waited but by June we place a silicone implant in my paralyzed fold. It forces that vocal fold over to the middle so that when the other vocal cord (the working one on the right) touches it, I can produce sound again. Perfect!

Until we realize, the shortness of breath has not gotten better, and it should have. I also became one of only TWO patients of this ENT whose implant actually busted a stitch and shifted. I no longer had a voice.

In investigating my loss of voice at 4 weeks postop, we do another CT scan. The implant has shifted and has to be re-placed. AND the pericardial effusion has now gone from "mild" to "mild to moderate." And my heart is enlarged.

That started me down the path of a rheumatologist referral, a pulmonologist referral, and a cardiologist referral.

In July we did an Echocardiogram which showed some thickening of both ventricles, some valve regurgitation, and a pulmonary artery pressure that was a LITTLE high. I don't know specifics on that report but..

So we're doing all of those referrals, I have tests done/lined up such as lung perfusion scan, a right heart catheterization, a sleep study, pulmonary function tests, etc... I also redid my vocal fold surgery in mid-August. Thankfully, so far so good on that end.

For now we wait. The rheumatologist and cardio appts are at the end of September and I anxiously await those. My pulm who I really like says this is basically either --
1. An inflammation caused by the CREST and NOT pulmonary hypertension...we just have to get the rheumatologist on board so we can address this big flare I seem to be having and beat it back into remission??? Is that possible?
or
2. The very early stages of pulmonary hypertension and he has lots to treat this with and it's OKAY and he has lots of experience with this.


So I've been a mad woman doing research on PH and Cardio involvement with CREST syndrome. I feel silly saying this, but I don't know what to "hope" for. Does that make any sense? I realize it may be both... ?

Which one is the better prognosis? Which is easier to treat? I had no idea that heart involvement was a "common" occurrence with CREST. But Pulmonary Hypertension is no picnic and is it always progressive? I read and read, but I have no definitive answers.

I don't konw which one is the lesser of two evils ya know?

And I've read the comments and threads on Prednisone. I'm embarrassed to tell you how much prednisone I've been on this summer. I'll leave that for another post because I've felt for a few weeks now that I don't know if I'm coming or going and not knowing what's "flaring" and what's just that horrible drug. Again, that's a whole other post. lol

Anyway, so those of you with cardio involvement, has it been treatable? Do you feel better? What about PH? Are you doing well once treatment was established?

I hate waiting and not knowing. I hate all the testing too but I know information is power. For now I would love to hear from others who have had these internal organ complications and how any words of wisdom on them.

Thanks for reading this long post. With my past year of history it's complicated and I hope to only do all that to you once. For your sake and mine. ;)

Maddy
Maddy
38 yr old mommy to 2 boys
Raynaud's, CREST diagnosed '96
Pulmonary Hypertension diagnosed Oct '07
GERD, Inefficient Esophageal Motility diagnosed Jan '09

#2 peanut

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Posted 09 September 2007 - 01:19 PM

Maddy,
PH and cardio involvement are kinda related. PH can cause heart enlarging. The right heart cath (RHC) will help determine this further. Starting PH treatment will prolong your life and definitely help your heart issues.

I have mild PH, no cardio involvement. I have sclero related lung involvement. My RHC showed no heart enlargement, but it is important to note what your pulmonary arterial pressure is (PAP). My treatment is: Revatio, Letairis and Oxygen. I've been on it for a few months now. I feel a lot better.

Yes, prednisone is a beast drug.

You have a lot going on. Don't forget to relax and take time for yourself.

peanut

You can deprive the body but the soul needs chocolate
my HMO makes me wear a helmet...

#3 Patty

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Posted 09 September 2007 - 02:46 PM

Hi Maddy,

First let me say welcome to the forum, sorry its because you have sclero.

I have cardio . I was to have a right heart cath and got very lucky that my Dr was wonderful and not only did the right side but did the left as well. I had this because my PFT was off and Dr's thought I had PAH. After the cath I was told I have stiffening of the left side of my heart. Diastolic Dysfunction. I take a water pill/BP med for this and get a blood pressure checkup every month by my Dr and I have a blood pressure machine at home to take it every day. (I do not take it everyday because it makes me crazy if my numbers are high for a day so I find weekly is good for me or if I dont feel right I take it).

I do not have PAH or last year I didn't have it. I have SOB all th time though so who knows if things changed over the last year.

I hope that your results are good. Pleaase keep us posted.
Hugs,
Patty

If Life hands you lemons, make lemonade.

Hashimoto's thyroiditis, Limited scleroderma, Dermatomyositis. Diastolic Dysfunction, dysphagia, Fibro, restless leg. Lupus is a maybe.

#4 janey

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Posted 09 September 2007 - 05:13 PM

Welcome Maddy! We're very glad that you have joined us.

It sounds like you have fallen into some great doctors. Many times that's hard to do. So hopefully you can keep them. I have both heart and lung involvement and as peanut mentioned - they are kinda related. I have mild PH and pulmonary fibrosis. I also have heart conductive problems (complete heart block and arrhythmias). My cardiologist suspects that a lot of my arrhythmia problem is due to the lung problems, so currently we're working on the lungs. As already mentioned, PAH can cause heart enlargement if not treated. I'm on Bosentan (Tracleer) for the PAH and it's really working. My PA pressure has dropped from 45 to below 30. I'm on cellcept for my immunosuppresant and ILD. Like Peanut, I'm on supplemental oxygen which also helps, but I'm finding I need it less and less.

It sounds like you have already been doing quite a bit of research. Just in case you've missed them, we do have pages on both cardiac involvement and pulmonary involvement.

Please keep us up on how you are doing and test results. Again, welcome!

Big Hugs,
Janey Willis
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#5 Maddy07

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Posted 09 September 2007 - 05:20 PM

Thanks for the replies so far ladies! I can see how it's all related and just unsure of what's the better option. As if I have a choice. lol A girl can hope right? :P

I've heard great things about the meds for PH (Tracleer in particular) and it's nice to see that Cellcept or other Scleroderma drugs can be used in conjuction. That makes more sense to me.

Have a good week ladies!
Maddy
38 yr old mommy to 2 boys
Raynaud's, CREST diagnosed '96
Pulmonary Hypertension diagnosed Oct '07
GERD, Inefficient Esophageal Motility diagnosed Jan '09

#6 jefa

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Posted 10 September 2007 - 11:28 PM

Maddy, I was going to reply to your earlier post in the thread, but I see you have sort of answered yourself here. You asked which is better or has the better prognosis. As you say, it really doesn't make much difference. Sometimes we get so involved in trying to attach labels to everything, we lose sight of the fact that we have what we have and we need to find the right doctors (which you seem to have done) to help us get through it all.

Regarding flares - I don't think it is possible to "beat them into submission" - my doctors have all told me to lie down to a flare. It is something that happens and it will pass. I have begun to see the flare as a signal to slow down a bit.

Again, with labels, I see that you refer to your illness as CREST. It sounds like from the descriptions you have given of your symptoms you have given in this and your other posts, you may be dealing with more than one autoimmune disease. The arrival of the joint pains in your knees and hips with your current flare may be indicative of arthritis rather than sclero. You will see in our page on CREST that:

When CREST symptoms appear along with another form of Scleroderma, it is referred to as, for example, "Limited Scleroderma plus CREST" or "Diffuse Scleroderma plus CREST." A person may also have any other autoimmune disease " plus CREST."


Warm wishes,
Jefa

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#7 Maddy07

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Posted 11 September 2007 - 12:56 AM

Jefa,

Yes I think maybe it's terminology is all. I think I'm happy to hear it's a "flare" which while I know you don't ever lose the diagnosis of CREST or what have you, but a flare means it has an end point! Get through this crisis, be glad it's drawn my attention to needing treatment, and know that I won't always feel this way.

Mentally, that's what I needed to know if that makes sense.

I tapered my prednisone by 10mgs yesterday, woke up at 3am in severe knee pain. Hate not knowing if it's some new "thing" or from tapering the pred. Time will tell and I stink at waiting!

Happy Tuesday everyone!
Maddy
38 yr old mommy to 2 boys
Raynaud's, CREST diagnosed '96
Pulmonary Hypertension diagnosed Oct '07
GERD, Inefficient Esophageal Motility diagnosed Jan '09

#8 LisaBulman

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Posted 11 September 2007 - 01:27 AM

Hi Madddy,
Welcome to the forums!!! I am so glad you decided to post and join our group here. Boy have you had a whirlwind of a trip so far...fun ins't it?! (kidding) It is so difficult to go through all of the testing, worrying, appointments and still try to take care of your family. I have sclero with esophogeal, lung, liver and intestine involvement. I am 37 with two girls 9 and 7. It is not easy. The only advice I can offer is to appreciate every day, your family and try not to worry. Easier said than done, Iknow. But you can't change the test results so try to find a way to deal with what may come... Don't ever be afraid to ask for help!!!

Hugs,
Lisa
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#9 peanut

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Posted 11 September 2007 - 06:59 AM

Hey Maddy,
I take Imuran for sclero with all my PH drugs and so far so good. So yes, you can treat both or all at once. Unfortunately for me that mens I'm on a lot of meds for now. I keep a list of the meds/vitamins in my purse that way all my doctors know what's going on. Your doctors will help you weigh the pros and cons of each med.

No worries,
peanut

You can deprive the body but the soul needs chocolate
my HMO makes me wear a helmet...