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Daughter Ang

CREST syndrome / Limited Scleroderma with possible lung involvement and Esophageal stricture

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Hello all,

 

I sincerely hope this message finds everyone in good health. I am joining this forum because my mother (age 61) has been diagnosed with Scleorderma. I just got back from the hospital with her and possibly like everyone else here when they were first diagnosed, scared myself immensely while researching for this unheard disease online, this is also how I found this website, and what a great relieve to read your real life experiences.

 

My mother has had her fingers turn white and numb since almost 15 years ago, but it was mild and didn't affect her quality of life at all, so we didn't take it too seriously. She saw her family doctor regarding this issue in the past but was waved away diagnosed because there were no other symptoms. Recently, she has been experiencing having dry mouth and the inability to create much saliva. She has to keep hydrate her mouth by drinking water when she talks and eats. She also felt difficulty swallowing, this has actually first occurred also about 15 years ago, but very vaguely, and is becoming more noticeably recently.  

 

We saw a Rheumatologist and ran many tests. Her result indicators seem different from what I see in this Forum (maybe because we are in Canada ? ) But here is the diagnosis: 

 

Scleroderma Disease profile Positive 

ANTI CENP A : High positive 

ANTI CENP B : High positive

ANTI Ro52 : Weak Positive 

 

And the explaining says : This is associated with the limited cutaneous form of scleroderma or CREST syndrome, and is also seen in patients with Raynaud's phenomenon that may progress into scleroderma. So I am assuming  she has CREST because most of her symptoms also fit. 

 

In the meantime, we have also done a scope to see her colon and esophagus and turned out that she has a narrowing/stricture at the lower part of her esophagus. 

 

She has also done an ECHO which came out healthy ! (phrew)

 

What worried me the most is that, she had done a breathing test (PFT) and it showed "moderately reduced diffusion capacity with mid air trapping and normal airflow rates" These features would be compatible with interstitial lung disease , pulmonary vascular disease, and emphysema. I then went on researching for lung involvement in CREST and it scared me even more !

 

All the google suggests that came out were about life expectancy and the outlook for lung involvement really doesn't look too good.

 

Although my mother does not have any lung related symptoms.

 

I guess the main reason I'm here is to seek a peace of mind before we get to meet with the doctors again, and also to hear from your experience ! 

 

Thank you so much in advance for your responses, they will mean a lot to me ! 

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Hi Daughter Ang,

 

Welcome to these forums to you and your mother.

 

I'm sorry to hear that your mother has been experiencing worrying symptoms and has a possible diagnosis of scleroderma hanging over her. I can understand how anxious you're both feeling and of course, it's not possible for me to be able to predict your mother's prognosis regarding the disease. We do recommend that our members, if possible, consult a listed scleroderma expert, as this complex disease does require specialist knowledge and expertise. I've included a link to our expert in Canada.

 

Because of the very unpredictable nature of scleroderma, it does affect everyone differently; however, to give you some perspective, I have limited scleroderma with what was in the beginning serious lung involvement and after being successfully treated by a top lung hospital (I'm in the UK) I'm happy to say that my lung function is now better than the average persons' (my consultant told me they could pull people in off the streets with much worse lung function than me!:wink:)  Thankfully, I've never had any gastrointestinal problems, but I've known friends who have and they are able to lead a comparatively "normal" life, so a diagnosis of scleroderma is certainly not necessarily a death sentence.

 

I've included links to our medical pages on Lung Involvement, Gastrointestinal Involvement and Raynaud's, to give you a little light reading and hopefully allay your fears a little (googling "scleroderma" is actually not a good idea as there is a lot of inaccurate and scaremongering stories about it on the internet.)

 

It's possible that other members will be able to give you some more information about their own cases.

 

Kind regards,

 


Jo Frowde

ISN Board Member

ISN Secretary of the Board

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

International Scleroderma Network (ISN)

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Hi Daughter Ang,

 

I'm sorry, your Mum has been having problems and would echo Jo's post on the subject. However, I would like to add (from personal experience), that plain water gives limited help to swallowing problems.  I found  salt water a little more helpful, but you can buy artificial saliva which is even better.

 

A narrowed oesophagus can be stretched, though my lack of salivation resulted from mis prescribed antidepressants and stopped after I refused to take them any more.  So I am wondering whether she is taking medication where a dry mouth is a side effect.

 

Hope this is of help Dimarzio.

 

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10 hours ago, Joelf said:

Hi Daughter Ang,

 

Welcome to these forums to you and your mother.

 

I'm sorry to hear that your mother has been experiencing worrying symptoms and has a possible diagnosis of scleroderma hanging over her. I can understand how anxious you're both feeling and of course, it's not possible for me to be able to predict your mother's prognosis regarding the disease. We do recommend that our members, if possible, consult a listed scleroderma expert, as this complex disease does require specialist knowledge and expertise. I've included a link to our expert in Canada.

 

Because of the very unpredictable nature of scleroderma, it does affect everyone differently; however, to give you some perspective, I have limited scleroderma with what was in the beginning serious lung involvement and after being successfully treated by a top lung hospital (I'm in the UK) I'm happy to say that my lung function is now better than the average persons' (my consultant told me they could pull people in off the streets with much worse lung function than me!:wink:)  Thankfully, I've never had any gastrointestinal problems, but I've known friends who have and they are able to lead a comparatively "normal" life, so a diagnosis of scleroderma is certainly not necessarily a death sentence.

 

I've included links to our medical pages on Lung Involvement, Gastrointestinal Involvement and Raynaud's, to give you a little light reading and hopefully allay your fears a little (googling "scleroderma" is actually not a good idea as there is a lot of inaccurate and scaremongering stories about it on the internet.)

 

It's possible that other members will be able to give you some more information about their own cases.

 

Kind regards,

 

 

Hello Joelf,

 

Thank you so much for the response, it is very reassuring to hear your experience. Congratulations on your recovery ! I am more hopeful now after the initial shock dies down. Do you mind if I ask what kind of lung involvement you had, and what treatment was received ?

 

My mom is suspected to have scarring and the doctor said, if she has no symptoms, then they won't offer any treatment yet. I'm not sure how I feel about this because I feel like she should get her current state stabilized so she won't develop any symptom, instead of getting treatment only when symptoms surface (because that will affect her quality of life).  What are you thoughts about this ? I'd love to hear you or any one else's experience.

 

Thank you so much again ! 

 

 

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Hi Daughter Ang,

 

The type of interstitial lung disease (ILD) I have is called non specific interstitial pneumonia (NSIP), which, if you've going to get pulmonary fibrosis, is the best sort to have! :wink: The reason is that it is a fibrosing organising pneumonia which is reversible if caught early enough. 

 

Thankfully, as I was extremely fit at the time, I noticed straightaway that I had breathing problems which I shouldn't have had. I went to my general practitioner who was on the ball and after a chest X-ray she referred me to a local lung consultant (who was also on the ball) and she referred me to The Royal Brompton Hospital ( which is a top lung specialist hospital). They did every lung test known to man on me and then I had six months treatment with a rather unpleasant, but effective, drug called cyclophosphamide. I was then put on an immunosuppressant and steroids and have been on them ever since (although I'm now in the process of weaning them down with a view to coming off them completely.)

 

I would emphasise that each case of ILD is different and so is the treatment, so the fact that your mother isn't actually receiving any treatment at this time isn't necessarily a problem. The reason I had such aggressive treatment was because a) it was reversible if caught early enough and b) the inflammation was galloping on in my case and they were afraid that it would become permanent fibrosis and therefore untreatable, if not dealt with quickly. I also had lots of other symptoms which at the time I hadn't realised were scleroderma, but I was also referred to The Royal Free Hospital, where they are top scleroderma experts, so all in all I have been very lucky.

 

Kind regards,


Jo Frowde

ISN Board Member

ISN Secretary of the Board

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

International Scleroderma Network (ISN)

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