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petermeach

Hi

26 posts in this topic

I'm not sure if I qualify to be here. I had a letter from the Brompton today saying I had scleroderma related pulmonary arterial hypertension. This is not exactly a good thing apparently. I don't have any of the external symptoms people talk about except for Raynaud's but I understand it can affect just internal organs.

 

I have to go up to see them at the beginning of June which will be a nightmare journey. The letter says admit me, I think this means appointment. Hope so.

 

Not sure what to ask really. Does anyone else have this or am I in the wrong place?

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Hi Peter,

 

Welcome to these forums!

 

I'm sorry to learn that you have been diagnosed with pulmonary arterial hypertension (PAH) and you have certainly come to the right place for up to date and accurate information on scleroderma. I've included some links in this post, which I hope you'll find helpful and informative.

 

Fortunately (or unfortunately, depending on your point of view) I am able to advise you on lung involvement in scleroderma and the Royal Brompton from personal experience and I have to say that you could not be in better hands for your treatment, as they are experts in this field. Thankfully, I don't have PAH, but I do have interstitial lung disease, which has been treated very successfully by them. In my case, it was diagnosed very quickly, which meant that the treatment I received had the maximum benefit. I was diagnosed seven years ago and fortunately the disease has stablised.

 

Scleroderma can affect internal or external organs, or both and one of the main problems with it is that it has many different symptoms which affect all sufferers differently. This is what makes it a difficult disease to diagnose and why we do recommend that our members, if possible, consult a scleroderma expert. I was actually referred by the Brompton to The Royal Free Hospital, where they specialise in scleroderma, so was very fortunate on both counts.

 

The journey to the Brompton should hopefully not be too arduous for you; I'm in West Sussex and come up to Victoria Station and then walk from there to the Brompton via Sloane Square and the King's Road, which takes me about 40 minutes.

 

Please do let us know the results of your appointment with the Brompton.

 

Kind regards,


Jo Frowde

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

ISN Chat Host

International Scleroderma Network (ISN)

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Oh dear, I can't walk for 40mins, I'd have problems walking through Victoria. I get very out of breath very easily from any exertion at all. I've asked for transport. I can't drive either as I had a brain abcess at the end of August and spent a month in hospital. Not a good year all round. I get taken to hospital by patient transport. This was privatised in West Sussex from 1st April and is a disaster. I should have had 12 trips to St Richards with them since 1st April and they have managed 2, they just don't turn up.

Of course I'll tell you what happens in the hospital, it's 6th June

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Hi there Peter,

 

You are certainly not the only one who can't walk far.   Jo is a whizz so don't judge yourself by her abilities :lol: :lol: .

 

I have no heart or lung involvement although I do have SystemicSclerosis and Rheumatic Heart Disease which between them keep me well under the weather. I can't walk comfortably for more than 5 or 6 minutes.

 

Lots of folks on here ask what they should talk about at their  hospital appointments and apart from making a list of what bothers you most you will probably find that various things will come up as you talk and get results of investigations.

 

In this country (New Zealand) if I got a letter saying to admit me I would be prepared to at least stay the night.   You will probably be exhausted after the trauma of getting there so maybe you will be thankful for the opportunity of a rest.

 

Best wishes

Judyt

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Hi Peter,

 

Sorry to hear that you've been so poorly; I hadn't realised from your first post how badly you're affected.

 

We have a medical page on Preparing for Doctor Appointments, which include tips that have come from our forums as people discussed their upcoming appointments with their specialists. I hope this will be useful for you.

 

I have heard how dire the patient transport system has become in West Sussex; is it possible for you to find any alternative means to take you to hospital appointments?

 

Kind regards,


Jo Frowde

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

ISN Chat Host

International Scleroderma Network (ISN)

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It's a complicated old thing isn't it? Everyone seems to be different. The lady who rang from the Brompton said "Oh we'll arrange transport", which I was pleased to hear.

 

I read those appointment notes, very sensible and readable, a useful page. I wish there was someone who could go with me but there isn't.  I do have a possible problem with the size of the hospital. St Richards in Chichester is quite small and has lots of wheelchairs for those who need them although I don't. Southampton on the other hand is huge and I can't walk it. Also there are very few wheelchairs. I had an appointment there where I ground to a halt about halfway to the department. My taxi driver had come with me, she said no way was she going to let me go by myself, and found a wheelchair eventually in A&E. No idea how big the Brompton is, I guess quite.

 

The only way for me to get there is Brompton arranged transport, patient transport who won't turn up, or a taxi which would cost a fortune.

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Hi Peter,

 

I'm very pleased to hear that the Brompton has said they would arrange transport for you. It is a very large hospital, but hopefully, they will a) actually turn up to collect you and b) be able to take you to the right dept. I have seen patients who appear to have had hospital transport being escorted in wheelchairs by members of staff, so it could be that they will provide a wheelchair for you, if you need one. I've usually found the staff at the Brompton to be very helpful and so perhaps you could contact them and ask about wheelchairs and for directions to the correct dept.

 

if you're going to the interstitial lung disease clinic dept, then that's in the Fulham Road (the main hospital is in Sydney Street.) I had to go and stay there for a few days in the beginning, in order to have just about every lung test known to man. I stayed in the Fulham Road part of the hospital and had to go to Sydney Street for an ECHO, but they did have transport between the main part of the hospital and the Fulham Road.

 

Hope that helps. :happy:


Jo Frowde

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

ISN Chat Host

International Scleroderma Network (ISN)

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Hi Peter,

 

I don't even live in the same hemisphere as you so all I can do is sympathise with you.  We have good hospitals here and in Auckland have a Clinical Centre which is separate from our city hospital.   The clinical centre is pretty easy, I can drive myself there, park in disabled parkking - usually - and a short walk to the clinics.  

 

At the city hospital it is very different and when I have to go there I dread getting from the car to my appointment.  

 

I think Jo's advice is the most sensible, phone and talk to somebody in advance and try to make sure that they understand how hard it is for you.   I am inclined to disagree with your statement that you don't NEED a wheelchair.   I think you DO.   Not every day and all the time but in lots of instances you do.  What happens in your neck of the woods with somebody who is wheelchair-bound?   There are people who are and they must be helped to manage.  Try to imagine you are one of them and see what you come up with.   Maybe there is some organisation of volunteers who would be happy to help you.   It is easy to bury your head in the sand and decide that it is hopeless, but in reality it is not and you will find a way.

 

All my best wishes and kind thoughts are reaching out to you.

Judyt

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'stay there for a few days'

 

Ah, the letter says 'admit', I had assumed it was a one day trip. That's a bit of a problem. I have an elderly, sick and won't last long cat here so I have to get someone to look after him. The only person is my ex-wife who would reluctantly do it (cat wakes up early and howls!) but not on the day they want me to come up, it's her brother's funeral.

 

I'll ring the Brompton on Monday to clarify this. There can't be many tests I haven't had, I've had the lot and they have all the doctors.

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Hi Peter,

 

Please don't worry about a possible admittance. I had my first appointment at the Brompton; saw Professor Wells and his junior consultant and then they decided that they would admit me for a few days for further tests about 7 to 10 days afterwards (at that stage they didn't know if it was scleroderma, or not.)

 

Of course, it may be totally different in your case, so I would suggest that when you contact them about the transport etc. you could ask them if there is the likelihood of you being admitted. I would be surprised if they were to spring it on you at your appointment, without prior warning.

 

Kind regards,


Jo Frowde

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

ISN Chat Host

International Scleroderma Network (ISN)

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Well it is just a day trip. Apparently admit just means 'to the hospital'. They have also allocated me a bed which seems a bit over the top, a chair would be fine.

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Hi Peter,

 

I missed your initial post, and just want to welcome you to Sclero Forums. It sounds like your appointment will be quite an adventure, so I hope it all goes smoothly!

 

:hug-group:


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.

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Hey Peter and welcome! Don't it just get ya?! It's not enough to have a disease, we also have to have all the inconvenience the symptoms bring.

 

No one tells you when you become ill that as well as stepping out of the life you lived and waving it bye bye, everything and everyone else, carries on regardless. All we can do is struggle to keep up.

 

Please let us know how you get on and sometimes you have to raise your voice, just a tad, to be heard. Make sure you are.

 

Take care.


Amanda Thorpe

ISN Sclero Forums Senior Support Specialist

ISN Video Presentations Manager

ISN Blogger

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist

International Scleroderma Network (ISN)

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That wasn't a nice day. The Brompton was fine but the trip was awful. I was supposed to be picked up at 5.30 for an 11.30 appointment! but the person due to be picked up at 4.30 from Gosport bailed and I'm not surprised. I was given the standard set of obs, ecg, xray. The concern is the PAH and not the sclero so I was given a viagra and then had my blood pressure monitored for a couple of hours. Was also given quite a bit of stuff to read. I came home with a big bag of viagra. Hopefully I will get more used to it because I feel queasy and have a headache.

 

Drive home was awful, I was sick. Possibly car sickness but the last time I was sick was more than 40 years ago. Driver was not good. He said he was booked to take me back at 4pm and if I wasn't ready at 4.15 he would leave. His excuse was he had been up since 5. I said so had I, he was getting paid and I was seriously ill! He complained about everything, traffic, other drivers etc. Eventually I told him to shut up.

I'd rather not have to do that again!

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Hi Peter,

 

Goodness me, what a nightmare journey to and from the Brompton! I'm sorry to hear that and unfortunately, it makes a stressful day even worse. Your driver sounds a very sensitive soul! :sorry:

 

I do hope the headache and queasiness will improve once you get used to the medication.

 

Do you have any follow up appointments with the Brompton?

 

Kind regards,


Jo Frowde

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

ISN Chat Host

International Scleroderma Network (ISN)

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I sincerely hope not, at least for a long time.

 

I have to see the rheumatologists at Chichester some time, don't know when yet. I assume fairly soon, I seem to get a very prompt service, i.e. pushing in the queue.

 

He was awful, it didn't help that I wasn't feeling well but it was all so unnecessary

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Hey Peter, you'd think that people could at least be kind if they don't get it and they don't.

 

Consider reporting him, he doesn't have to be touchy feeley , you don't have to put up with him, especially the threat to leave you.

 

I have never had to use transport and never would because I couldn't physically cope with that length of time out.

 

I hope that the viagra works but make sure to phone the hospital if you have problems.

 

I am really sorry to hear about this but can't say I am surprised.

 

Take care.


Amanda Thorpe

ISN Sclero Forums Senior Support Specialist

ISN Video Presentations Manager

ISN Blogger

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist

International Scleroderma Network (ISN)

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Viagra works a treat, I barely get out of breath going upstairs. It's quite a short lived effect though, hence having 3 a day. My problem is the PAH. I'm not sure how the connective tissue disease is affecting it. The Brompton person I was talking to didn't know either. I'm going to see the lung lady in Chichester in a few weeks so I shall ask her.

I couldn't cope with the trip either, I shall have to think what to do if I have to go there again.

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Fab! So glad to hear that the medication is working!

 

Have a look at the information we have on site about PH, there are various ways in which scleroderma can cause it. The good news is that with early diagnosis and treatment, the prognosis is good.

 

Take care.


Amanda Thorpe

ISN Sclero Forums Senior Support Specialist

ISN Video Presentations Manager

ISN Blogger

(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist

International Scleroderma Network (ISN)

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I went to see the lung consultant at the hospital the other day and told her my muscles and joints are stiff and ache. This is a new thing in the last couple of weeks. She said this was normally treated with steroids and that she really didn't want to start me on steroids, I think it is sclero related.

Also I had a chest xray just before seeing her. The previous one showed voids in my lungs which have got worse. This is like tb but not tb, she thinks non-tuberculous mycobacteria. She is doing more tests. There is a treatment, 2 years on 3 different antibiotics. Apparently there are potential nasty side effects and I need councelling before deciding.

Seems a bit unfair to me!

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Hi Peter,

 

I'm really sorry to hear that you seem to be experiencing more unpleasant symptoms and problems. Having suffered with stiff and aching joints, I can empathise with the discomfort this can cause.

 

We have a medical page on Extrapulmonary Tuberculosis (EPTB), which includes a section on Autoimmunity and Mycobacterium. Unfortunately, a lot of the drugs prescribed do have the downside of nasty side effects; I think it's a question of weighing up the possible benefits of taking the medication, against the potential problems which may develop through their usage.

 

I'm afraid steroids are a case in point; we have a medical page on Glucocorticoids, Steroids (Prednisone) Warnings for Scleroderma and having taken them myself for the last seven years, I can safely say that although I thought they were nothing less than a miracle drug when I first took them, now having had just about all the known side effects with them (apart from the renal crisis, which I hope will be indefinitely postponed ! :wink: ) I am very much less ecstatic about them than I was! The worst effect of them is avascular necrosis, which has necessitated my having two total hip replacements. I can understand your lung consultant's reluctance to prescribe them for you.

 

Kind regards,


Jo Frowde

ISN Assistant Webmaster

SD World Webmaster

ISN Sclero Forums Manager

ISN News Manager

ISN Hotline Support Specialist

ISN Chat Host

International Scleroderma Network (ISN)

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Thanks for that. The links are useful and interesting even though I don't understand a huge amount of what they are saying. I get the general drift which is probably enough.

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Hi Peter,

 

I'm sorry you are having additional complications. If I were you, I'd get a second opinion before jumping straight to steroids for muscle and joint pain, especially when it is of such recent onset. First, look at all your medications to see if that is a potential side effect. A common culprit for that is statins (cholesterol medications) or many other medications with muscle and joint pain as a side effect.

 

It might also be the onset of fibromyalgia, which is very common with scleroderma. Or it could be that you are going through a stressful time, and not sleeping very well, and the fallout from that is aching from head to toe. In any event, since this is new, first look to medication side effects or any recent changes in your lifestyle (such as stress, or perhaps even stopping a regular exercise.)

 

As Jo pointed out, its not the best idea to rush to steroids, especially with scleroderma. See Warnings for Steroids and Scleroderma, because steroids strongly increase the short term risk of developing scleroderma renal crisis (sudden kidney failure).

 

So many other things could be tried first, such as improving sleep, stretching or exercising or warm pool classes, pain medications, massages, antidepressants, muscle relaxers, relaxation or meditation programs....why, the list goes on and on, and most of us use a combination of several if not many things to address muscle and joint pain.

 

You've just had the whole stress of possible lung infection and difficult questions regarding possible treatments added to your plate. That's quite a stressor, too, and probably won't do anything to improve your relaxation and sleep (and thus, it may increase your joint and muscle discomfort.)

 

I'm unable to take any steroids, even apart from scleroderma issues, and have managed widespread and chronic joint and muscle pain for decades without steroids. So, I know it's possible, even though steroids are certainly a very fast shortcut, in the long run, they can be quite troublesome, especially with scleroderma. Such as Jo, who has had to have two hips replaced just in the last year.

 

I also count myself lucky to be unable to take steroids. I'm positive a single dose would have me hooked forever, as it would be a single source for relief (rather than the more complicated steps of sleep evaluations, plus other medications and many lifestyle changes) and I imagine it would be instant, as well. So then I'd spend my remaining days incurring kidney failure and joint replacements....of that, I have little doubt.

 

Pay very close attention when you are being counseled regarding treatment, and take the time you need to make a proper decision. Some things truly are better off not treated, because sometimes the risks of treatment really are worse than the risks of the disease.  Especially as we age, we are more likely to encounter ailments that we are better off trying to just live with, as long as possible, rather than tackle them head-on.  But, you don't hear people talk of these issues much, for as a society we tend to go all-in for any treatment that exists.

 

I myself have chosen to not have some things treated, yet have gone all-in for treatment of other things. It's helped me to ask my doctor, if it was them, or their loved one, would they attempt this treatment, under these same circumstances?  A surprising number of doctors would personally forego treatments that they are still obliged to offer to their patients.

 

I'm sorry, I don't know why I'm going on and on about this.  Except to say, I appreciate that you will be faced with many decisions. Some may have easy answers, some not. Who knows, maybe you could even try the steroids at low dose for a few weeks, just to get the worst of the fires put out, while you work on getting other approaches ready, such as perhaps some good sleeping and pain meds, or enrolling in a warm pool class.  Just be aware to do your best, working with your doctors, to try to avoid relying on high dose or long term steroids for your condition -- if it's reasonable and possible, under the circumstances. 

 

And please bear in mind, none of us, particularly me, are doctors, and we should not be guiding your medical decisions; rather, hopefully just giving some food for thought or to discuss with your experts.

 

:hug-group:


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.

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Oh I am not in the slightest stressed, I don't do that. It's a bit annoying being stiff and sore but I can put up with it, it's odd suddenly being 30 years older! You should see me getting up from a chair. I have to do a 6 minute walk test, I can't see that going well.

The mycobacteria sounds worse. The hospital took a lot of blood samples and I'm to have another CT scan shortly. It sounds harder to knock off than actual TB, I think that is 6 months of pills whereas this is 2 years and might not work.

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Hi Peter,

 

It's delightful that you don't stress out over everything. Yes, non-tb mycobacteria can be very hard to treat.  I hope the hospital has some answers for you soon, to help guide your treatment decisions.

 

:hug-group:


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.

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