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Biomarker for Diffuse Scleroderma skin has been discovered!


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Increase in Lung Scarring

Pulmonary Hypertension

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#1 miocean

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Posted 15 March 2013 - 08:06 AM

This has been a full "Not Medically Free" week between dealing with insurance, pharmacies, doctors, testing and not just for me this time but also for my husband who will be having surgery next week...we had so many CT scans and x-rays between the two of us that we must have been positively glowing!!

In a previous post I told you my pulmonary function had declined: total lung capacity went from 78% to 61%, diffusion dropped to 37%, I desaturated on the 6 Minute Walk Test. I am definitely more short of breath, especially on exertion, and am working on setting up oxygen therapy. Yesterday my scleroderma specialist called with the results of my chest CT scan. I have increased scarring on the lower lobe of my left lung. You know it's not good when you hear the words "I'm sorry" and "there's really nothing we can do." I have already tried cyclophosphamide and had a toxic reaction, ended up in the hospital for 4 days, and I am taking Myfortic for anti-rejection of my kidney transplant at the highest dose possible. The doctor said if I wasn't taking the Myfortic the scarring would probably be worse.

The next step is another Right Heart Catherization (make that #3 for me) to check for Pulmonary Hypertension. This one will be different than the others I have had, it will be without dye, part of it will be done at rest and part while I am lifting saline bags with my hands. A new medical adventure! If it turns out I can add that to my list of secondary issues it will have to be treated with a drug that is compatible with my kidney transplant medications.

Although very serious, in my mind this is overshadowed by my husband's surgery. He is having a canalplasty to open up the bones in his ear to correct a condition known as "surfer's ear" where skin and bone grow to protect the ear canal. He has lost hearing in that ear. We are going to the best Otologist in the area for this, highly recommended by a friend who had the same procedure. Although it is out-patient it will be the first time my husband is under general anesthesia and I am concerned about that.

I would be interested in hearing from anyone who has had this type of heart catherization. I know several here are being treated for PH and have been reading past posts.

The good news is my kidney is 3 years old and doing well!!!

miocean
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#2 Joelf

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Posted 15 March 2013 - 09:00 AM

Hi Miocean,

 

Goodness me, I am sorry!! As if you haven't got enough to contend with! Not only yourself to worry about, but your husband as well! :sorry:

 

I've got lung involvement and had cyclophosphamide (successfully) so can't advise you on that score from my own experience and thankfully I don't have Pulmonary Hypertension (Echo last month showed everything within normal range.)

 

However, what I can do is wish you and your husband the very best of luck with both your surgeries and hope everything goes really well for both of you. :emoticons-i-care:

 

I'm very pleased to hear that your kidney is behaving itself!! :emoticons-yes:

 

Best wishes,


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#3 Sweet

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Posted 15 March 2013 - 04:14 PM

Goodness my love, you do have a lot going on. I'm sorry to hear about your news, and about your husband. Please know I keep you in my thoughts. Hang in there.
Warm and gentle hugs,

Pamela
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#4 Shelley Ensz

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Posted 15 March 2013 - 05:43 PM

Hi Miocean,

 

Goodness gracious!  I am sorry to hear about everything you, and your husband, are going through.  I will be sending lots of extra good wishes your way, for both of you.

 

Major congrats on making it to your third anniversary with your new kidney!!  :yes: Have you gone out to celebrate that yet?

 

:emoticons-group-hug:


Warm Hugs,

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

#5 judyt

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Posted 15 March 2013 - 09:01 PM

Hi Mio,

I must add my best wishes to you and Dave for a good outcome to his surgery.   I am sure it will be successful and he will be restored to perfect (or the best it can be for his age) hearing.

 

Can't help with the Heart Catheterisation, my heart problems are due solely to Rheumatic Fever and not sclero.   The repair to my Mitral Valve has fixed the PH.   Whether it is a permanent fix or not I don't know but I hope so.

 

Best wishes to you both.

Look forward to hearing news of success

Judyt



#6 miocean

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Posted 29 March 2013 - 08:47 PM

Thank you for your support and kindness. My husband's surgery went great, he doesn't have any hearing back because the ear is packed but he came through with flying colors. He had no pain and many stories to tell about having surgery (we have been through them all but it was his first time.)

 

I contacted my scleroderma consultant regarding my recent tests and the verdict is to proceed with the right heart catherization but to also make an appointment with the pulmonologist at the scleroderma center. The decline in function and increased shortness of breath are probably due to the increased scarring but I should go ahead and rule out or be treated for pulmonary hypertension.

 

I consult with consultants who tell me to consult! :lol:

 

(I know I posted this response in a much longer version but it must have gone into the great void as I don't think it would have been flagged by the moderators.)

 

It's been a long day as we had the follow up with my husband's surgeon early this morning and it is already tomorrow. I wish every doctor could be like her, an expert but warm and caring, no ego trip.

 

miocean :emoticon-insomnia:


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#7 Joelf

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Posted 29 March 2013 - 11:34 PM

Hi Miocean,

 

I'm so pleased to hear that your husband's surgery has gone smoothly and that he's on the mend. You must be feeling very relieved!

 

I do hope that your right heart catherization goes well and that the treatment suggested will help with your shortage of breath and make you more comfortable.

 

Kind regards,


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#8 Amanda Thorpe

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Posted 30 March 2013 - 06:53 AM

Hello Miocean

 

Good news and you and your husband should have good news, always!! :balloons:  :balloons:

 

Take care.


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#9 Margaret

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Posted 31 March 2013 - 08:13 AM

Hi Miocean ,

 

Glad to hear that your husbands' surgery went well.  I'll keep you in my thoughts for your testing, too.

 

Take care, Everyone.

Margaret



#10 Shelley Ensz

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Posted 02 April 2013 - 07:13 PM

Hi Miocean,

 

I'm delighted for your husband.  :balloons: :yes: :terrific:

 

But I want to be delighted for you, too, as I know how frustrating it can be when people can't hear you.  So, can he hear yet?!

 

:emoticons-group-hug:


Warm Hugs,

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

#11 miocean

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Posted 02 April 2013 - 08:14 PM

Hi Shelley,
He has a little bit of hearing but not much as the packing is still in his ear. Right after the surgery he had no hearing so it is encouraging that he has a little! We go back in a couple of weeks to take the packing out.

Our coping skills have improved. I know to speak in his good ear to say important things, or to touch him or make eye contact. He tries to sit so that his good ear is on the side of the person he is doing the majority of talking with. He is doing better at acknowledging he hears me, especially if he is driving and I am giving directions and I sit on his bad side in the car.

We are hoping for a full recovery of his hearing. My colleague who had the same surgery for the same reason by the same doctor had his hearing restored and has been fine for over 10 years. He wears custom earplugs made by an audiologist in the water and that will be something we will do after my husband has the other ear done in the fall. In the meantime he is using silicon ear plugs and keeping the ear dry.

miocean


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#12 Shelley Ensz

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Posted 04 April 2013 - 07:36 PM

Hi Miocean,

 

I am glad to hear that your husband's recovery is progressing from his ear surgery -- and that you are both making some fine adaptations to his hearing loss in the meantime.

 

:emoticons-group-hug:


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.