45 replies to this topic

[Sooty30]

Hi, my husband has diffuse scleroderma and got his CT results back last week and he has scarring in the base of his lungs and in the middle. His general practitioner said he has about 50% function and lungs of a 80 year old.

Anyway he is really struggling; always out of breath also sometimes at rest. He went to see his general practitioner yesterday as he felt the worse he has so far to be told to go and and rest and wait for his lung appointment to come through. He was also told you can't keep coming in here with this whilst you're waiting for an appointment and this could take ages with the NHS (National Health Service) anyway. I'm furious; he went up there as he was worried he had a got a cold on his chest as both myself and our 2 year old have been full of it. I think the general practitioner was really rude and I would have had plenty to say if I had been there; we pay our national insurance and tax and have every right to be there. I'm really worried as I've seen the weight fall off him and he is not eating as much either.

Not sure where to go from here. I know we have to wait for an appointment but what if he feels so uncomfortable and his doctor doesn't want to know? How bad do you have to get before I take him to hospital? Last time we went we came out 8 hours later with no results. I'm worried his lungs will get worse as he is struggling so much without any help. I know if I got stupid bad I'd call 999 (emergency number). I just want him to have a little help before it gets to that. We are back at the rhumatologist in 2 weeks time.

Any ideas what way to approach this?

[Amanda Thorpe]

Hello Sooty

A two week wait to see rheumatology should be reassuring at least and I would ask them about hurrying up the appointment for the lung specialist, are you talking about the first consultation with the specialist?

As far as the general practitioner goes, as I always suggest, put it in writing to the practice manager or the doctor. It's simply unacceptable to be so callous but I suspect it may be frustration on their part. Either way you can address it or not.

I have a friend who has had scleroderma for 30 years with just 50% lung function so anything is possible. Last time I saw her I was in a wheelchair and she was walking albeit slowly and I don't have lung fibrosis. Go figure.

As for phoning for an ambulance you might want to do that when your husband is in what they call respiratory distress, what that looks like I have no idea but I would not hesitate phoning an ambulance if you think it necessary. However, it won't necessarily jump start anything. In 2010 when diagnosed with myocardial fibrosis and serious heart failure I was scheduled for a heart MRI and left & right heart catheter test and I ended up in hospital with supraventricular tachycardia, heart rate of 230 but as I already had tests lined up they treated me, kept me in overnight and they wanted to keep me another day but they were not making any efforts to do any tests or treatment, not that I wanted them to, because it was already in hand. Of course your husband has no such treatment pending so I don't know whether a trip to A&E would speed things up for him or not, I guess that depends on whether they think he needs admitting.

I am sorry, I know how scary it is where you are, having been there myself (I waited 5 months from diagnosis of heart problems for treatment whilst I got short of breath just talking), don't hesitate to call an ambulance if you think it necessary though.

Take care.

[Joelf]

Hi Sooty30,

I'm sorry to hear that your husband has had such an unsympathetic and unhelpful response from his general practitioner; certainly not what you want to hear when you're obviously very worried about him and have a young child to care for as well. Unfortunately the average general practitioner has very little knowledge and expertise regarding Scleroderma and it's treatment and doesn't realise the seriousness of the disease. That certainly doesn't excuse his rudeness to your husband and as Amanda has suggested, I would write a very strongly worded letter to the doctor himself and also to the practice manager.

One of the most unfortunate problems with the NHS is that there is invariably a wait between your general practitioner's referral and actually getting to see a consultant. I found the waiting to be one of the worst parts of my treatment and can really empathise with you. The average time was 6 weeks between each consultant I saw, but I was extremely lucky in that my general practitioner was very understanding and did realise how worried I was.

As you're seeing your husband's rheumatologist in a couple of weeks you should certainly discuss your fears about your husband's lung condition with him; obviously the sooner he receives treatment the better the outcome is likely to be. However, to give you some encouragement and a little light at the end of the tunnel, my lung function was down to 48% at it's worst and deteriorating rapidly before I was started on my treatment. Now two and a half years later (after treatment) my lung function has increased to 85% and my consultant has told me that they could pull the average person in off the street without interstitial lung disease and find they had worse lung function tests than mine!!

As Amanda has advised, you could certainly call an ambulance if your husband was in a lot of distress; although it might help if they were to give your husband oxygen, I don't think it would necessarily mean that he would get the specialised treatment needed for pulmonary fibrosis any sooner (particularly on a Friday or Saturday night when a lot of the A & E (Accident and Emergency) departments are up to their eyes in vomiting drunks!! )

Kind regards,

[debonair susie]

Hi Sooty,

Your husband AND you can be the best judges of his distress point. Both Amanda and Jo know the system where the 3 of you live and I don't living in the United States. However, we can READ the desperation in your message and certainly empathize with you, as his wife.
I hope that by now, you have been able to make a determination as to what you both will do.

I will hold you both in my thoughts and will be watching for your posts, with regard to your husband.

[Sooty30]

Thankyou all for responding to me, I guess it is the unknown that is really worrying. The last few days he has improved more on his breathing but skin has got tighter. He has been signed off for a month now so least he can take things easy even though it's driving him mad.

We go to the Rheumatologist in 12 days, this will be his 2nd appointment with him. A month between appointments. He had injections in his legs last time and started on some medications, but last time his CT results and breathing results were not back, as they were asked to be done by a different hospital when we were not 100% sure what was wrong with him.

Thanks also for your positive stories it really has helped as when you google you just seem to get worst case etc. I guess we will soon know more.

It's our 1st wedding anniversary on Monday. Who would have thought all what has happened and changed in a year. Am hoping to get him away to Mexico next year for our 2nd but have been advised against going too far right now..time will tell but trying to stay positive

[Joelf]

Hi Sooty30,

I do hope that when your husband sees his rheumatologist he is able to get more help and advice and you'll hopefully have a better idea of how to proceed with his treatment. It is important to try and stay positive if possible ( but I know that's easier said than done! ) Do post again and let us know how he gets on, won't you?

Happy anniversary for Monday!!

Best wishes,

[Amanda Thorpe]

Hello Sooty

I so wanted to cry when you said it will be your 1st wedding anniversary on Monday, I was diagnosed on the day of my 10th wedding anniversary and that was bad enough!

May I suggest that your husband look at being signed off for more than 4 weeks at a time, for your peace of mind? Realistically you know he isn't going back to work in a month or even two so get your rheumatologist to sign him off for oh say 3 or 4 months for starters? If he goes back prior to that great but if not no worries about having to run around getting him signed off especially as you are still waiting to see the lung specialist.

Do something to celebrate Monday, however low key that may be.

One way I stay positive is to be realistic, it's great to have goals as long as they are achievable otherwise you just set yourself up for failure and disappointment. What is achievable varies daily with scleroderma which is one of the reasons why it's so hard to live with this disease because you never know what any day is going to be like until you get there! For example on Monday a good day might include making dinner, on Wednesday a good day might just be getting out of bed! Mind you I have had scleroderma now for 5 years and it takes time to find your own way of living with it and of course because it affects all of us so differently that means different things to different people.

When I first came onto the forums I used to say "there's still good life to be had after scleroderma, it just looks nothing like you thought it would but that's not necessarily a bad thing!" I still believe that even after heart failure but like I said above how you live that out looks different every single day.

Take care.

[miocean]

Amanda,

My favorite quote. It kept me going through some really rough times and I still have it in the corner of my mind.


miocean

[debonair susie]

Hi Sooty,

I see that today is Your and your husband's 1st Wedding Anniversary. May I say congratulations to you both and that each of you is very fortunate to have the other? With you as your husband's loving spouse, you are doing everything conceivable to gain as much knowledge as you possibly are able.

You are absolutely in the right place for accomplishing the afore-mentioned, as well as hearing from people (myself included), who can provide that extra emotional support, as we have lived or ARE lving what you/he are.

I'm happy to hear that his breathing has eased. Hopefully he is being treated by a doctor you both have confidence in; Since you have nearly 2 weeks before his appointment, if you aren't already, it would be very helpful for you BOTH to write down any and all questions you have for the doctor.

I'm hoping that the two of you are able to do something very special for your anniversary. These things are happening but he has an awesome caring and loving wife in you and I am very happy that he has you as his advocate.
Sending you both s to be used whenever you feel you need them.

[Amanda Thorpe]

Congratulations to Sooty and husband on their first year anniversary on Monday!!

[Sooty30]

Hello again.

We have had another appointment at Chelmsford where my husband had more injections in his legs and had his immune suppressant drugs increased. Was also told his lungs were not following the normal pattern of the disease and they are not 100% sure if some of the damage has been caused by smoking,even though it is clear there is damage there. So awaiting to see the Lung specialist.

Off to the Royal Free on 12th April. Any advice on what to expect?

Many thanks x

[Amanda Thorpe]

Hello Sooty

What a pity, I am there the week before, I'd love to have met up with you both!

Yes, expect the best treatment in the country at the Royal Free! Write your questions down to take in with you and also write down what they tell you because it all becomes a bit of a blur when you leave the room. They will also introduce you to the Specialist Nurses, Louise and/or Tani, who will thereafter be at the end of the phone for you and your husband, they are excellent nurses as well as lovely ladies.

Whatever the outcome you will know that the very best are working on your husband's behalf.

Take care.

[Joelf]

Hi Sooty,

I'm so pleased to hear that you've got your appointment at The Royal Free and as Amanda says, your husband will receive the best treatment available!

I really hope that you get on well with the appointment and do please be sure to post and let us know how it goes.

Best wishes,

[debonair susie]

Hi Sooty,

Thank you for keeping us updated on all that is being done for your husband. It isn't easy being a loving spouse to one who is enduring the symptoms your husband has been enduring. It is so disconcerting and such a helpless feeling, to be sure.

I continue to hold you both in my thoughts and will await news of your husband's appointment results.

[Sooty30]

Well we finally got seen by a lung specialist yesterday. I am totally shocked that a system such as the NHS does not share information. This specialist has ordered another CT scan and lung function test. We only had these done in Southend less than a month ago; he said he can't access them. I'm glad everything is being done to help my husband but more appointments; it's so hard trying to do my working hours and get him to all the hospitals; none are local, with a 2 year old to look after too. We had the swallow test Monday so hopefully that will be clear

They have started him on 10mg prednisolone a day. The specialist said he would see us again in a month and with the results of the tests can see exactly what we are dealing with lung wise. That will be 7 months of coughing and shortness of breath. I so feel for my husband as I can see it's wearing him down. He said to me he can live with the symptoms of the scleroderma, he just wants his lungs back. I just hope they ease a little; have already been told the damage can not be cured. On a good note he now doesn't feel in a hurry to return to work.

On one letter we have received it says the double stranded DNA results are normal and the ENA (extractable nuclear antigens) screen is strongly positive at 22 and the profile demonstrates strongly positive anti-scl-70. What does this mean? Is this the results they use to confirm diffuse scleroderma ?

[Joelf]

Hi Sooty,

I'm very pleased to hear that you've had your appointment with a lung specialist.

I'm afraid the lack of communication between different health trusts is another one of the frustrating things about the NHS. I've had similar problems as I deal with three different health trusts, none of which tend to advise the others of the overall picture. Like your husband, I did have a local CT scan and then another at The Royal Brompton Hospital; I think this is pretty standard practice, though.

The ENA is a grouping of antibodies often used to screen for autoimmune diseases and strongly positive Anti- Scl-70 antibodies are highly specific for scleroderma and are seldom present in other diseases.

Hopefully, the prednisolone will help your husband and make him feel better. There is a downside to the use of steroids for people with Scleroderma in that they can precipitate a renal crisis. However, like your husband, I was started on a dose of 10mgs of prednisolone and certainly the overall difference it made to me was remarkable. It's certainly true that once the lungs have scar tissue on them the damage is irreversible; but if the problem is caught early enough whilst the 'ground glass' present on your husband's lungs is still at the inflammation stage and hasn't yet progressed to forming scar tissue, it can be much improved, as I can testify from my own experience.

Do please let us know how your husband gets on with his appointment in a month's time.

Kind regards,

[Amanda Thorpe]

Hello Sooty

Sorry but that's a disgrace and the consultant should have phoned Southend for the results, they could have faxed them over a least.

It's funny how some doctors don't think to do the simple things that make such a difference. At the Royal Free in December 2009 a scleroderma expert said I needed to have a heart MRI but didn't say why, being claustrophobic I refused point blank so they went for another test which was a total waste of time telling nothing other that what was already know. Well in April 2010 when the ECHO showed I was in serious heart failure the MRI came up again but this time, Dr Benji Schreiber (world's best rheumatologist, in my opinion) picked up the phone, rang the Royal Bromptom and asked what could be done for a patient who needed an MRI urgently but was terrified. Turns out all sorts of things could be done and I had the MRI no sweat unfortunately by that time the myocardial fibrosis (suspected in December 2009 but not communicated to me) had become inactive so no point treating but damage done.

I am glad your husband is no longer in a hurry to go back to work, it will always be there for sure! Great that your next appointment is only 4 weeks away and it might be an idea to ring the clinic a week prior to the appointment to make sure they have the results and if not make sure your next call is to PALS (Patient Advice and Liaison Service) which every hospital has. Complain and they will do all the leg work for you ensuring results are there when you are. My mother complained to PALS at Southend Hospital about an appointment she attended but guess what, her file didn't and she hadn't been given the right tests prior to this appointment anyway. She got PALS on the case, they did everything for her and hey presto all was resolved with no effort on her part.

Take care.

[judyt]

Hi Sooty,
These stories remind me of a story of our own. In 2005 soon after we had made arrangements to go on a once in a lifetime cruise to Alaska, my husband was diagnosed with a Plasmacytoma. That is a bone tumour and his was in his pelvis. He had Radiotherapy and then was pronounced OK to travel and so off we went with him on crutches and using wheelchair access to aircraft and other transportation.

Our first stop was with friends who live on Vancouver Island. They happen to be medical doctors and the wife is into cancer therapy. She quizzed Ian about his condition and his latest Xray results. I can remember standing about in her kitchen and could see that she was not happy with Ian's responses to her questions.

She picked up the phone then and there, telephoned Auckland Hospital Radiology (on the other side of the world) and told the person on the other end of the phone who she was and that she wanted the results of the latest Xray faxed to her now. Lo and behold they arrived in minutes and she was able to advise us about what she thought. If she can do that, anybody can do what is necessary to get results that are needed.

No doubt you will take these stories on board and in the future you will be better informed about what to do when things start to turn to custard. You don't have to be bossy or get upset, just remember that you have rights and they have responsibilities and stick to your guns until you get the right response.

You have enough to do with a little one to care for as well as a sick husband and an income to protect, and people need to respect that.

Warm hugs and best wishes in the future.

JudyT

[debonair susie]

Hi Sooty,

Well, there you have it! You really ARE in the right place for gaining knowledge! As Amanda and Judy are well aware, you, your husband and the rest of us have rights, where our health records are concerned. I am SO glad you posted so that you could get such useful information from those who are in the know!

Let us know how all goes.

[Sooty30]

We are off to the Royal Free today so hopefully know a little bit more. Spoke to Ade on the phone; what a lovely women.....she let us change our appointment by 2 hours so we are able to travel in the off peak to London, bless her;otherwise it would be expensive. We asked for all medical records to take with us so now need a suitcase to carry them .

My husband had another CT scan last week and due to have another lung function test on Wednesday then lung specialist 2 days later. I'm hoping we will have a fuller picture then as he really struggles to walk anywhere.

Will keep you posted.