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#1 ginatufano

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Posted 01 November 2009 - 06:53 AM

Hi all - I am new to the boards. My husband (40) was diagnosed with pulmonary fibrosis in the spring of 09. He had been coughing/short of breath nonstop for months. It started with what seemed like a bronchitis that never went away. Dr kept telling him coughs could linger. When he finally went to a pulmonologist - his PFTs showed moderate restriction and low DLCO (his #s are generally in the mid 50's-60's). His HRCT showed honeycombing in the lower lobes. His blood work was all normal at the time.

They started searching for a cause of the fibrosis. He has had pretty severe Raynaud's for 5+ years (hands and feet) - so they pushed on the autoimmune front. They began to look at some of his other symptoms (which we never even considered symptoms - but wrote it all off to getting older): GI issues - cramping, loose stools several times a week; patches of hair loss on his lower legs - that had recently tripled in size; random rash and itch on chest/back/shoulders/upper arms; red blood vessel rash on his face/neck; weight loss (30lbs over 2 years). Not including the constant getting sick (which is NOT his usual routine) - fevers, night sweats, cough, tired, etc.

His swallowing study was normal, his echo was normal. New bloodwork was drawn and his ANA was 1:2560 nucleolar pattern.

Just Friday they diagnosed him with both amyopathic dermatomyositis and limited systemic scleroderma. Having read up on PF - I understand Interstitial Lung Disease more than I ever wanted to. I know that his hours of coughing each day will not improve - what I don't know is if we will be able to stop it from progressing. The dr seems to think that the fibrotic lung is from the dermatomyositis and not the systemic sclerosis - I'd like to believe her as it has a better prognosis - however I just can't wrap my head around it. Everything that I have read about dermatomyositis - doesn't really fit him - with the exception of the rash. Everything I read about systemic scleroderma - points to diffuse not limited.

Am I missing something?

They biopsied several areas - we will have those results in a few weeks - we also have a follow up appt in Dec. to determine course of treatment.

Anyone else in this double diagnosis land? I find that I learn a lot more from patient experience than from reading journals and articles - I'd love it if you could share any knowledge/experience that you have.

This scary new world we have been in these last few months is not fun. I get so much hope from reading about people who are still active and living with this.

Thank you so much for your help!

Gina

#2 Jeannie McClelland

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Posted 01 November 2009 - 07:48 AM

Hi Gina,

Welcome to the Forum! I'm so sorry you and your husband have all this to deal with. It can be scary and there certainly times when it can seem overwhelming.

A large number of our members (or their family members) have multiple autoimmune diseases to deal with. That's not uncommon at all, so I'm sure you will be hearing from them. I have two: systemic sclerosis sine scleroderma and recently was diagnosed with autoimmune hypothyroidism. I can't comment on the dermatomyositis, but I can certainly share my experiences with pulmonary fibrosis.

I've just passed the third anniversary of my diagnosis. When I got the diagnosis, I already had developed pulmonary fibrosis (PF/ILD) and pulmonary hypertension (PH). Three years down the line, with medication, both the PF and PH are essentially stable. Posted Image

I'm not a doctor, I can't even find my way around my doctor's office, but has anyone suggested your husband's cough might be in some part due to reflux (GERD). At the time of my diagnosis I had pretty significant esophageal involvement and the GERD was more severe than even I had realized. Since I failed to improve on various medications, I ended up with a fundoplication which eliminated the reflux. That was pretty great in itself, but what was also great was that it decreased my coughing too.

It always seems that the first year/18 months is the toughest in terms of coming to grips with the diagnosis, the amount of time spent in testing and appointments, making any necessary lifestyle adjustments, and getting settled with a treatment plan. A positive attitude helps immensely. I can't speak for everyone, but this was certainly the case for me, and I am doing as much, if not more, than I did before my diagnosis.

I wish you and your husband all the best and hope you'll post often.
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#3 Amanda Thorpe

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Posted 01 November 2009 - 08:34 AM

Hello Gina

Welcome to the forum and I am sorry your husband is so unwell.

I won't be able to throw any light on whether your huband's symptoms are more diffuse scleroderma than amyopathic dermatomyocitis because I am not medically trained. What I would suggest is that you take your research, in particular that which points to diffuse as opposed to amyopathic dermatomyocitis and discuss it with your husband's doctor. Specifically ask why that diagnosis was reached in favour of diffuse scleroderma. There is nothing wrong in asking for an explanation and open discussion about a diagnosis.

As Jeannie's already said you and your husband are at the beginning of your scleroderma journeys and it is a difficult place to be with so much going on and so much information to take in all at once. It takes time to come to terms with living with a chronic illness and we have some information about emotional adjustment that you may find helpful.

I am into my second year of diffuse systemic sclerosis and have been through the initial transition period. It's tough, scary and emotionally draining but it is just a phase that you both will move out of and hopefully into better things. My life now looks nothing like I thought it would but not necessarily a bad thing as there is still good life to be had after scleroderma it's just...well...different!

Take care and keep posting.
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#4 debonair susie

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Posted 01 November 2009 - 09:19 AM

Hi Gina,

I am so glad you found your way here and have chosen to share your husband's health issues with us. I'm quite sure you will find folks here very sharing, either with their own experiences or those of their loved ones.

My diagnoses (1995)came about four years after symptoms first presented in 1991. Fifteen years prior, I was diagnosed with Fibromyalgia and Psoriasis (w/dx of Psoriatic Arthritis to be made later).

Systemic Scleroderma, along with GERD, which was difficult for me to grasp, as I had been "so healthy" before all of this "hit".
Since, further diagnoses: Angioedema (allergy-induced from a BP medication I had taken for YEARS), reacted like anaphylactic shock...throat closing up. Costochondritis, as well as Pleurisy came on about the same time.
I have like-symptoms of a hyperthyroid, but tests don't demonstrate the same.

I had many episodes of migraines. However, I believe they were caused by an allergy I had. I eliminated the "source" from my diet and have had no more migraines, to date.

It seemed my Sclero flares would last for quite some time, but they seem to be less frequent, the last year, or so.
The PFTs I receive yearly, do not indicate any sign of PF, but am so sorry to read of your husband's trouble, in this regard.
As Jeannie mentioned, GERD CAN cause coughing, as can some medications, which applies to me in this way.

You spoke of your husband's weight loss...I lost 18 lbs in one month,, as I was unable to keep food down. At first, I just thought I had the flu, but for 6 weeks?!
You also spoke of your husband's Raynaud's in his feelt and hands. Almost immediately, changes took place in my hands...bone loss in my fingers, along with a great deal of pain. Once more I am having much the same symptoms, only the pain is in shorter spurts, rather than days on end, as it was in the beginning.
My feet don't seem as bad and didn't have Raynaud's symptoms until 5 years ago. Walking barefoot is a mute point!
The fatigue your husband has experienced, was chronic for years, with me. However, since the pain had ebbed somewhat, I have more "better" days, than the "not so great" days.

Gastro issues can be most troublesome for many of us...I also suffer from looses stools, more often than not :( .

I agree with you, in that those who have personal experience are good resoures. You can also find great information from the various reliable articles that are posted in the medical area of the ISN/Newsroom.

I want to welcome you and your husband here. I'm hopeful that you both will feel as comfortable about sharing and supporting as the rest of us do!
Special Hugs,

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#5 Joelf

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Posted 01 November 2009 - 11:18 AM

Hi Gina

Welcome to this very helpful & informative forum & I'm sorry to read that your husband has been so poorly.

I have Non Specific Interstitial Pneumonia which is a form of Pulmonary Fibrosis & like your husband have the classic symptoms of coughing & shortage of breath & I was diagnosed in July of this year. At first both Scleroderma & Polymyositis were suspected but after seeing an excellent Scleroderma specialist he confirmed that I actually have Systemic (because it's affected my lungs) Limited (because I have very little skin involvment, only the tips of my fingers) Scleroderma. Prior to being put on steroids my joints (particularly my wrists, fingers, & knees) were very swollen & painful & I am just over halfway through IV Cyclophosphamide treatment for my lung involvment. My lung function tests were very encouraging which is a positive point. :) Also thankfully I don't seem to have suffered the digestive/bowel problems so many others here seem to have to cope with; although nor have I had any weight loss............I should be so lucky!!! :lol: :lol: ;)

However I am fortunate in that I have had the very best treatment imaginable & I still lead a fairly active life, although obviously I have had to tone my exercise regime down a little. ;)

I do hope you & your husband will feel encouraged by the helpful advice on this site.

Kind regards x

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#6 Snowbird

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Posted 01 November 2009 - 12:10 PM

Hi Gina

I just wanted to welcome you and your husband as well. You've come to the right place for both good and factual information. Is he under the care of a scleroderma rheumatologist/specialist?
Sending good wishes your way!

#7 janey

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Posted 01 November 2009 - 12:17 PM

Gina,
I'd like to join the others and welcome you to the Sclero forums! I am sorry to read about all of your husband's symptoms and the multiple diagnosis. Connective tissue diseases are tricky little buggers and their symptoms overlap quite a bit. I was diagnosed with systemic scleroderma and polymyositis (dermatomyositis without the rash) in August 2003.

Like Jeannie, I was diagnosed 3 years ago with pulmonary fibrosis and pulmonary hypertension (PH). The fibrosis is a complication of both sclero and poly I believe. Medications, and I think to some extent my diet, have helped to reduce the symptoms and somewhat stabilize the fibrosis and PH. I still cough, but not as bad as I use to. By keeping track of things I have found that for me, dairy products increase my cough. My GI doctor said is was due to the congestion and post nasal drip that dairy can cause with some people. The latter kicks up the reflux and it's cough, cough, cough.

BTW - the medications include Bosentan for the PH and cellcept for the immunosuppressant (which slows the progression of fibrosis).

While you are waiting for the next doctor's visit, please be sure to make a written list of all symptoms and all the questions that come up. I know I could forget a lot between now and December. Please keep us up on how the hubby is doing.
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#8 ginatufano

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Posted 01 November 2009 - 01:10 PM

Wow! Such great responses - I feel like I came to the right place!

Thank you sooooo much.

He is not seeing a Scleroderma specialist, yet. Right now we are trusting his PF specialist (out of UPenn - one of the centers of excellence for IPF). She is the "main" person involved right now, as lung issues are what started this roller coaster - we feel we are in good hands there - unfortunately, Penn doesn't have a Scleroderma center - it will be one of the discussions we will be having in Dec when we see her again.

Jeannie, Janey & Joelf: Did you have a lung biopsy? How was your PF diagnosed? Did you have any honeycombing on your HRCTs? Ground glass? Are your PFTs improving with treatment? CellCept is one of the meds that they are considering for my husband - have you seen any improvements with it?

At this point, we don't have any tests to compare yet - so we don't know if he is improving/worsening. His cough seems to wax/wane a bit. Now that it is getting colder out - he coughs much more than in the summer....He also seems to catch every little cold that comes along....Is that typical? He did get the pneumonia & flu vaccines - his pulmo's instructions were "don't get sick" and we take it seriously!

Sorry for the rapid fire questions - I am so excited to find others in the same boat!

Gina

#9 janey

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Posted 01 November 2009 - 01:57 PM

Gina,
It sounds like your pulmonary specialist is quite familiar with connective tissue diseases and the associated lung problems, so - in my opinion - if you're happy with her, then stick with her. As many of us know, a good doctor for this disease is hard to find. I have a whole team of doctors: rheumatologist, pulmonologist, cardiologist, and GI. Using a specialist in the specific problem area is the way to go.

To answer your questions, my pulmonary function tests showed a decrease in the various parameters so they did an HRCT. The first one showed ground glass in the lower lobes. I had another one in August and it show the same ground glass, but a little honeycombing laterally. (I'll have to find my results to get the exact wording.) I was put on cellcept (after 3 years on methotraxte, one month on Imuran and one month on oral cyclophosphamide). I've been on cellcept since spring of 2007. Everything has been stable until recently and now my pfts are dropping again. I have had no side effects with the cellcept nor any additional symptoms. We do have a small section on it: cellcept.

Cold weather, cold drinks, cold foods all make my cough worse! I put my soy milk and cereal in the microwave for 35 seconds before drinking or eating. As far as getting a cold - I haven't had one in more than 2 years (knock, knock, knock on wood). Of course I work out of the house and avoid large groups whenever possible. I don't fly or take trains, so I'm very conscious about avoiding the spread of things. Hope this all helps.


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#10 Joelf

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Posted 02 November 2009 - 10:02 AM

Hi Gina

To answer your questions, a lung biopsy is about the only test I didn't have; ;) but to diagnose my lung problem I've had a bronchoscopy, 2 CT scans, countless X-rays & innumerable blood tests!! The CT scans showed diffuse ground glass admixed with fine reticulation & traction bronchiectasis, with definate subpleural & basal predominance??!! :wacko: Apparently there is an element of consolidation which suggests a 'fibrosing organising pneumonia'?!! I also have positive ANA antibodies with speckled nucleolar pattern.

Like your husband I was referred to a top lung specialist before seeing a rheumatologist as my first problem was with my lungs & they have directed my treatment. As I stated previously my lung function test showed a very positive improvement; I'm not on CellCept but on Cyclophosphamide at present. When that course of treatment finishes they have suggested Azathioprine (Imuran) which I'm quite happy to take so long as it doesn't make my hair fall out (I have long, naturally curly hair & I don't do hair loss!!! ;) :lol: )

I hope that has answered some of your queries; I find the whole process rather fascinating & have spent many happy hours on the internet looking up all the details of my illness from the very comprehensive letter they sent me..........how sad is that??!! I really should get out more!!! :lol: :lol:

Kind regards x

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#11 Lori-Scleroderma Spouse

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Posted 08 November 2009 - 04:17 PM

So sorry to hear of what you are going through.  I went through the same thing with my husband and his was diagnosis when he was 40.  You go with what you believe and keep pushing the doctors.  I found out a great deal more than I ever wanted to know about all this stuff.  I was once asked by a dr if I was a nurse.  I am just an elementary school teacher but I researched the daylights out of it and knew read everything I could get my hands on.  Keep your chin up and help him stay positive.  I would be glad to talk with you anytime.  I have not been on the site in a long time but will look and see if I can send you a private message with my email address in it.  I have another great friend that I met on this site because her husband had the same thing.  We are great friends and good supporters for each other.  

Lori
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