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Appointment with Scleroderma Expert

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#1 miocean


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Posted 11 June 2011 - 10:27 AM

Yesterday my husband and I for a consultation with a scleroderma expert. It was a 400 mile round trip journey taking 12 hours but well worth it.

I have been receiving mixed messages from the assortment of doctors who deal with my multiple organ damage and general care and management of diffuse scleroderma. It was an honor to meet this man who has dedicated his life to finding a cure for this disease. At the end of the visit I asked him why he chose scleroderma as his field and he said he wanted a challenge and to leave his mark, that the apathy toward the disease was terrible, and that he had a personal relationship with a woman who was deaf but was a wonderful dancer, she could feel the beat through the floor. When the disease struck, everything was taken away from her. He touched his heart as he said this.

I fit the classic diffuse scleroderma profile to a T. Sudden onset with multiple organ damage, over a period of time a softening of the skin, and a plateau of lung damage. Many of you know that I had a crisis where I stopped breathing, my heart stopped and my kidneys failed. However, even though I was diagnosed with scleroderma renal failure through biopsy he doesn't think that is what cause my kidneys to fail! I don't fit the pattern, which he described as a loss of kidney function, sometimes a crisis, but afterwards an ability to produce some urine. Because I urinated for the last time when I coded and then produced very little urine (maybe a shot glass a day) he feels it was my HEART that caused the kidney failure with a sudden drop in blood pressure after it being high. With scleroderma renal failure the kidneys can come back but that was not the case with me as apparently my kidney failure was cardiac related.

As for my lung fibrosis, he still has to review the CT scans but based on everything he knew up to this point from reports, I have stabilized with around 55% of lung capacity and a diffusion rate of 45% with fibrosis on both my upper and lower lobes. There is no real treatment, drugs have been used that may halt the progression for a period of time but when they are stopped, after the same period of time with patients not on the medications, the lungs return to the same level. I am on myfortic (Mycophenolate or CellCept) for organ rejection which is becoming the drug of choice for treatment so the question is whether my dose can be increased without damage to my transplant. Basically, I can function quite well, I will get out of breath because I don't get enough oxygen. But if I function at my own pace and stop and catch my breath when needed I am fine and don't need oxygen.

On to pulmonary hypertension. This has been a back and forth of "she has it, she doesn't have it" and the bottom line is I will need to have another heart catherization, this time with a doctor at my center who will know what to look for and what numbers to get. He said the basic cardiologist doesn't really know what to look for and my scleroderma specialist has mentioned she "didn't get the numbers she needed". There is no immediate hurry for this so I will plan on having this done in the fall by the doctor he recommended. I am fortunate in that my scleroderma specialist ran the clinic at my center in the past, trained my current specialist, and is familiar with the doctors there.

Next is the use of a statin to reduce cholesterol. He doesn't believe my achy legs were caused by the statin and that current research shows that statins may actually help scleroderma! The anti-rejection medications I am on cause high cholesterol so I have to manage this some way.

One of the things he is going to do is coordinate my care, something we all have been frustrated with in dealing with different doctors and the lack of communication between them, and their basic lack of knowledge of scleroderma. This is bound to be an interesting discussion between my doctors, I wish I could be a fly on the wall! But, there is going to be discussion!!! He told me to calm down, that I am doing really well, have managed the disease well and that although he doesn't see a need for me to come to see him on a regular basis he will be in touch with me via email and that he will assist in managing my disease.

To those of you who have participated in trials, thank you :thank-you: . I don't qualify for any because of the kidney but progress is being made through these trials that helps us all.

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#2 debonair susie

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Posted 11 June 2011 - 10:50 AM

Hi miocean!

What a wonderful thread! I am really happy to know that your husband was able to go with you and I'm sure he walked away, feeling awesome relief.

I am absolutely THRILLED (for you) that he will be your care coordinator. I have NO doubt that this gives you great comfort, with knowing he will oversee your care now :terrific:

With someone who has his finger on the pulse of Scleroderma, as well as what can accompany it, I feel you will be a great "United Front"!

Enjoy your weekend! :happy-dance:
Special Hugs,

Susie Kraft
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#3 Joelf


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Posted 11 June 2011 - 11:50 AM

Hi Miocean,

How marvellous that you are under the care of a scleroderma expert; it's so lovely to be dealt with by someone in whom you can have confidence and trust. What with him and your very good lung specialist, you really are set fair for excellent care management of the disease. :woohoo:

I'm really pleased for you. :emoticons-line-dance:
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#4 Amanda Thorpe

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Posted 11 June 2011 - 01:21 PM

Hello Miocean

Interesting about the kidney failure being cause by the heart issue, mind you if the heart issue was caused by scleroderma then indirectly your kidney problems were caused by scleroderma?

What a relief it must be to have your care so well coordinated and overseen by a real specialist!

Take care.
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#5 enjoytheride


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Posted 12 June 2011 - 12:21 AM

How great to talk to someone who will explain things. I know that if I don't understand something, I am constantly worried over everything.

Congratulations on having achieved something really good with your visit.

#6 uknlv18


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Posted 12 June 2011 - 06:20 AM

That is wonderful news micean, it is always great when you can have confidence in your doctor and what he is doing for you!


#7 Margaret


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Posted 12 June 2011 - 09:13 AM

Hi Miocean ,

Thanks for sharing an uplifting, happy message! Nothing like going *straight to the top* for the best care!!!


Take care, Everyone.

#8 Shelley Ensz

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Posted 14 June 2011 - 02:13 PM

Hi Miocean,

I'm very glad that you had such a pleasant and worthwhile appointment. I have the same question as Amanda. If your heart failure was caused by scleroderma, and kidney failure was a result of the heart failure, then logically, the kidney failure is still the result of scleroderma, isn't it? I'm speculating that it would not have been precisely the same are pure ol' fashioned scleroderma renal failure, and may not have even occurred, if the heart hadn't been damaged first of all. You think, or not? I have no medical training of course, so this is just my wild guess.

Anyway, here are some flowers for your productive visit.

Warm Hugs,

Shelley Ensz
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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.

#9 miocean


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Posted 14 June 2011 - 10:31 PM

I have a lot of questions as well. What exactly happened in that CT scan room? The last thing I remember is thinking, I am going to die in this room with people staring at me and heard plastic being ripped open. I know I did not have a blood pressure cuff on at that time.

My local nephrologist did a biopsy on my kidney which came back scleroderma renal failure. When I later told my sclero doctor he did the biopsy she said it was unnecessary and she knew it was scleroderma renal failure.

However, this scleroderma expert says I don't fit the pattern and that if there was a sudden drop in blood pressure, that in itself would have caused the kidney failure. He said the biopsy would come back scleroderma even if it was the drop in blood pressure that caused it. I've learned through my dialysis/transplant support group that kidney takes heart and that heart takes kidney.

Does it matter? The end result is the same. But my curiosity has been aroused. I have some unanswered questions that will require getting records for. What caused me to code in the first place?

It's sort of like asking what caused the scleroderma?

Thanks for all your concern,

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#10 Chopper


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Posted 15 June 2011 - 12:09 PM

That was very interesting! Thank you for taking the time to share that. Your medical history is quite remarkable and it's wonderful you're doing so well today! How fortunate that your confidence in your medical team is being boosted. :emoticons-yes:

Limited Scleroderma, Hashimotos Thyroiditis, Celiac, Gastroparesis, GERD, and Gastritis.

#11 KayTee


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Posted 15 June 2011 - 07:14 PM

How wonderful that you were able to see such a dedicated specialist.
Knowledge is power (and perhaps power is part of health?).
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#12 marsha


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Posted 16 June 2011 - 07:27 PM


I am so happy that you have found such a wonderful doctor. It makes a world of difference in the way we feel about ourselves and our disease.

Big hugs,