Sooty30

What's the best thing to do?

46 posts in this topic

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,


Jo Frowde

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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.


Amanda Thorpe

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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

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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! :emoticons-group-hug:

 

Let us know how all goes.


Special Hugs,

 

Susie Kraft

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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 :lol:.

 

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.

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Hi Sooty,

 

I'm so glad that at last you're going to get some expert advice and treatment for your husband and hope that your appointment at The Royal Free goes really well.

 

Do be sure to let us know how he gets on.

 

Kind regards,


Jo Frowde

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Hello Sooty

 

Ade is Prof Denton's secretary an when I was first ill I know my family spoke to her many times about my admission etc. By now you will be home and I hope you have a clearer picture of what you are dealing with and a way forward.

 

Did you meet the specialist nurses Tani and Louise? Whether you did or not they are now just a phone call away for you and I suggest you use them as they are excellent.

 

Take care.


Amanda Thorpe

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Hi, all went well at the Royal Free. It was a bit of information overload but we now know he has severe diffuse scleroderma so far his skin, hands, neck and feet are effected. They were also thinking his lung problems were due the scleroderma as well but at this time they didn't have the CT results. He also has Raynaud's as well.

 

We came away and I think they said they wanted to start him on the cyclophosphamide within the month. Hoping to have it done at Chelmsford but have been warned it could be London if Chelmsford can not set it up in time. I thought this was to stop the progressiveness of the disease getting any worse in his lungs. To have this once a month for 6 months. Also I thought this was the way forward we were going. My husband on the other hand thinks they will decide this when they have the CT scan results, but I thought the CT results would make them decide if my husband had to be admitted to the Royal Brompton lung unit for more tests..We so should of written things down.

 

As you can tell we both think the opposite and are totally confused. We saw the lung specialist today who had the lung function tests and CT scan results.The lung function tests have slightly got better and the CT results shows patchy ground glass infiltrate which is seen throughout both lungs. It says this has predominantly subpleural and basal distribution; also dilated airway throughout the areas of abnormal lung indicative of traction bronchiectasis. Minor reticular infiltrate can also be seen within both lower lobes.It says he has wide spread pulmonary fibrosis with diagnosis of NSIP (non specific interstitial pneumonia)

 

The lung specialist has left me feeling like I don't understand. I know it's good his lung function is slightly better but what about the other? They are forwarding results to the Royal Free as well. When I asked him about the cyclophosphamide and the lung unit he said he respects anything the Royal Free wants to do which really wasn't the answer I was after.

 

Do you think I should contact the nurses to find out if the other treatment is going to start in just over 3 weeks? I remember them saying they would write to us within about 2 weeks, but everything seems such a blur.I am stressing so much to the point my hair is falling out and as you can imagine our house is very strained too. I feel as if my husband doesn't listen to what they say or only pick out the positive bits.

 

Sorry for rambling on, thankyou for reading

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Hi Sooty,

 

Thanks for giving us the update on your husband's appointment at The Royal Free.

 

Firstly, let me give you some help and encouragement.

 

.............the CT results shows patchy ground glass infiltrate which is seen throughout both lungs. It says this has predominantly subpleural and basal distribution; also dilated airway throughout the areas of abnormal lung indicative of traction bronchiectasis. Minor reticular infiltrate can also be seen within both lower lobes.It says he has wide spread pulmonary fibrosis with diagnosis of NSIP (non specific interstitial pneumonia)

 

I had an almost identical result on the CT scan of my lungs and have a diagnosis (like your husband) of NSIP. They call mine a fibrosing organising pneumonia as there was an element of consolidation at the base of my lungs which could suggest an overlap with Polymyositis. I also had the same treatment which has been suggested for your husband with extremely good results. Obviously everybody's disease is different as is their reaction to the treatment, but you can feel confident that your husband will be receiving the very best treatment possible, particularly if he is referred to The Royal Brompton Hospital, where the Interstitial Lung Disease unit is second to none. I can't tell you whether they will refer him straight to The Brompton; I went there before I went to The Royal Free so did things back to front, but I started my treatment almost immediately after having been into The Brompton for almost every lung test known to man. ;)

 

As far as the Cyclophosphamide treatment goes, he will have 6 monthly IV infusions; it's very important that he drinks copious amounts of water throughout and after the infusion as one of the less pleasant side effects of this medication is that it can affect the bladder. However, providing he does exactly as they advise him, he hopefully should not experience the side effects. I suffered with a little nausea after the 4th or 5th infusion but they do give an anti sickness drug beforehand which deals with this and also another drug infusion to protect the bladder. The main purpose of the Cyclophosphamide treatment, as you correctly surmised, is to prevent the lung fibrosis becoming any worse; in my case because a lot of the ground glass on my lungs was inflammation the Cyclophosphamide was able to remove it before it developed into scar tissue and hopefully it might do the same for your husband. I would emphasise, though, that every case of Scleroderma is different and my excellent result was due to a number of factors. Nevertheless, it sounds as if at last your husband is getting the treatment he needs.

 

I hope that the Royal Free or the Chelmsford hospital should contact you within the next two weeks if that's what they said; I remember being slightly staggered at the speed at which things happened once I got into the Brompton loop! Obviously the sooner your husband can start the treatment the better the outcome is likely to be. Certainly, if you do feel in need of further reassurance, I would contact the very helpful nurses and perhaps they can explain things in greater detail to you.

 

I know it's easy to say but please try not to worry too much about the treatment your husband will receive; I can testify from my own experience that it's not so terrifying as it sounds and hopefully it will improve your husband's lung function and the quality of his life.

 

:emoticons-group-hug:

 

My very best wishes to you both,


Jo Frowde

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Hello Sooty

 

May I strongly suggest you get your husband treated at the Royal Brompton (if you have a choice) not a local hospital, not only are the Royal Bromptom experts at this disease and treatment but once under their wing you tend to stay there. Any queries and ring the specialist nurses at the Royal Free, that's what they are there for.

 

Now you know what you are up against and once the treatment is underway you can have hope that your husband could stabilize or even get better. The stage you are at will end and it can really get better for your husband and therefore you.

 

Take care and keep posting.


Amanda Thorpe

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Chelmsford is maybe only on offer as one of the specialists there were from the Royal Free so is used to doing these treatments. We had been told that they don't want Keith having treatment at Colchester which is our local hospital as they may not have the knowledge to treat us as well as Chelmsford or Royal Free. Chelmsford is easier for us as I can drive there and take our daughter if needs be rather than trains and tubes in London. We are happy to go where ever as long as he gets sorted.

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Hello Sooty

 

That's good news that if you do go to Chelmsford there is someone there who knows about administering the drug. Very soon your husband will be having treatment and we hope very soon feeling better.

 

Take care.


Amanda Thorpe

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Hi Sooty30,

 

It's hard to find good health professionals, at least here in Brasil, I had pass a year with a wrong diagnostic... and wrong treatment. Now that I've found a good rheumatologist, I give preference to go with some specialist that she has indicated.

 

Maybe your husband is picking out the positive bits to convince you to not get too much worry; I've done this sometimes when I saw that my husband was suffering.

 

Is he doing physiotherapy as part of the treatment?

I'm physiotherapist and can tell that (concomitant with the medication) respiratory physiotherapy it's really important to mantain the function of his lungs and to improve his breath and tiredness.

(Sorry, my English is not so good).

 

Hugs

 

Ro

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Hi Ro,

 

It's interesting that you should mention physiotherapy for lung problems as The Royal Brompton run "singing for breathing" classes to help lung patients. It's actually run by a professional singer who came into the day ward and was constantly pressganging trying to persuade the nurses and patients to join in the classes. I love singing so consequently was first in the queue! ;)

 

On a couple of occasions when I went to The Brompton for my Cyclophosphamide treatment I was able to join in; there were about 6 or 7 of us and we must have looked quite a sight; some with our cannula's in our arms and some hooked up to oxygen machines, all singing "Daisy Bell (Bicycle built for two)" and "You are my sunshine" (I was the only one who knew all the words for both verse and chorus to those songs......how sad am I?) :lol: We would spend quite a time before actually getting down to the songs doing breathing exercises and one chap used to come into the hospital just for the singing sessions; he'd been a patient for years but enjoyed it so much and it had obviously become quite a social occasion for him. He actually had COPD (chronic obstructive pulmonary disease) and asthma and the singing classes had helped him no end with his breathing problems. It was also a novel way of filling in the time between having a blood test before my treatment and waiting a couple of hours for the results to come back before the infusions could be given.

 

I don't know what we sounded like, but the singer in charge was able to find a key that we could all sing comfortably in so I don't think it was too bad! (I tentatively suggested that perhaps we could sing selections from "Oklahoma", or "The Sound of Music" as I know all the words to those musicals as well, but I think she felt that it would be a little too ambitious!!) ;) :lol:


Jo Frowde

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Hello Anotherday

 

There's nothing quite like a good rheumatologist is there? I am so glad you have one.

 

Take care.


Amanda Thorpe

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(Retired) ISN Sclero Forums Assistant Manager

(Retired) ISN Email Support Specialist

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