My wife (66) has diffuse systemic scleroderma with interstitial lung disease/fibrosis plus Raynaud's and GERD. Diagnosed in January but we are sure she's had it 2 years.. After a sea swim (despite wetsuit) she was quite unwell for 3 wks with chest and abdominal pain, which turned into an emergency after flying home (2 hrs 50 mins flight). The following day pains become much worse spreading to under left arm,. No water or food could be taken for 48 hrs, then no food a further 48 hrs. Turned out she had a fecal impaction. Only a private nurse stabliised her, should have been admitted but A&E said no beds available. Nothing like this devastating attack has happened before. tons of tests but no answers from hospital and she has not even SEEN a rheumatologist yet despite our requests as she is weak, anaemic and lost more weight.. Has anyone had this happen before? Anyone know why and what to do about it, please?
Why did this emergency happen and how... - Scleroderma & Ray...
Why did this emergency happen and how to prevent again?
Hi Pomoc, i had a somewhat similar experience, 2 years back, i have diffuse scleroderma, suddenly my intestine blocked, terrible pains and anemia, eventually they operated and removed a part of the large intestine, which was major surgery. Now after 2 years and taking medication i am doing well. Apparently scleroderma makes the intestine lazy so it gets blocked easily, also it caused adhesions which blocked my small intestine, also caused ulcers in my intestine and stomach, leading to anemia, i was given regular blood transfusions to cope. In turn stomach and small intestine do not abdorb nutrients as well as before hence the weight loss, i lost 20kv in six months. I wish your wife well, this situation needs serious treatment in hospital.
I had posted a reply and thank you for your very helpful reply but seem to have lost it. You sound like a very courageous person, and I hope the situation continues to improve. Yes, my wife went anemic in the weeks before this emergency after the swim. Out of interest, did they take you off mycophenolate or similar immunosuppressant? This is what the respiratory consultant decided after the emergency (remember, they haven't even let us see a rheumatologist yet but she has interstitial lung disease resulting from the SSD).
Hi Pomoc, i am still taking mycophenalate but in my case i have not had any lung complications yet, like your wife. The Scleroderma hit my kidneys, stomach and intestine. You definetely need to be under the care of rheumatologists as well as respiratory, they should be working together.
I forgot to mention, my rheumatologist told me mycophenalate is just a mild drug to subdue the immune system for preventitive purposes. For my case it did nothing, for me its practically useless. I havecrad that for respiratory issues they administer stronger drugs like Winrevair or Tocilizumab,these are newest treatments for scleroderma lung fibrosis.Anc be wary of the ulcers in stomach and intestine, they cause steady internal bleeding, not easily traceable. Avoid coffee and acidic foods, doctors will prescribe acidic blockers like omepraxol.
Hello, George i am also limited scleroderma patient i have no issue in stomach and intestine but i lose 13 kg in last 6 month. Do you know why?
I am sorry to hear about this weight loss. My wife suffered the same amount of kilos lost, being of average to slender build beforehand, over about a year when first falling ill. At that point it was deemed to be bronchiectasis but we know now it must have been systemic scleroderma as the primary cause, which can cause the dramatic weight loss. I am not qualified to say if limited scleroderma has caused your weight loss, but I imagine it is entirely possible. Push for help if you are not getting it. Amazingly, we still haven't seen a rheumatologist, 9 months after the diagnosis and the related emergency in June.
Hi Lion, i'm sorry to hear this, its difficult to give advice, so i will just explain what happened in my case, it was a combination of things, i think the main reason for loosing weight is that my stomach is not absorbing nutrients as well, not same as before. For this reason i stopped the low calorie diets, but no matter how much food i take in, i just can't put on weight, this i suspect is because Scl is affecting internally both stomach and intestines. Then you have the PPI blockers (omeprazol etc.) which cut appetite because they block gastric acids. In my case this eventually caused bacteria gathering inside my stomach(SIBO), as a result i completely lost all appetite, I could not digest anything, and was vomitting every night. Also Scl caused my oisofagus to loosen, and now i have acid reflux after every meal. Finally i suspect due to lack of exercise, the intestines and stomach get lazy, etc.The good news is that after treatment with antibiotic for SIBO, it killed the bad bacteria and cleaned up my the stomach and my appetite returned, i was then given immugoglobin infusions which helped a lot to restrict imflammation, and i am much better now and have started some simple exercises to keep me active.
But since as you say you have only limited Scl, so to answer properly its best to observe your particular case, for example, are you eating enough or maybe they put you on a very low calorie diet (?) have you lost your appetite (?), are you vomitting etc. and then please do consult with an experienced rheumatologist, take care.
I have just posted at length about my own experience which started with very similar issues to those your wife has recently suffered. Initially laxatives and colonic irrigation were the preferred options. Mine has gone on for years though - initially blamed on Sjogren’s and hypothyroidism and related nerve damage. But as things evolved in a more vascular manner I was retested and found to have antibody for diffuse systemic sclerosis and gastroparesis and then diagnosed officially last year with nailfold capillary changes specific to scleroderma,
To answer your questions in title - it’s tricky but I would initially request urgent gastroenterologist and/ or colorectal input and related motility testing. Depending on what tests show your wife may find that dietary changes and laxatives are enough. For myself I’m on an extremely restrictive diet which excludes almost all green veg and pulses, roughage/ fibre, red meat, high fat and processed sugar, carbs etc. Only in this way have I so far managed to avoid parenteral feeds eg NJ/ NG or TPN. I’m prescribed 1-2 ensure daily which I drink in decaf coffee and tea. This was after gastric emptying study and colon transit testing plus pictogram showed severe dysmotility throughout. 3 weeks ago I elected to have a loop colostomy - which is a halfway house measure to hopefully prevent me needing ileostomy - although my surgeon says this may be inevitable if my new stoma doesn’t work. Initially I was told I’d probably have a resection of my colon but then it was discovered that the rear outlet is knackered too and I have a large rectocele so we needed to bypass by rectum too. All of this relates to my scleroderma but I’m sure being dry from Sjogren’s with small fibre neuropathy, Raynauds, large babies and spinal osteoarthritis and being hypermobile haven’t helped.
I’m very proactive and won’t take no or shrug for an answer which has helped me get what I need from a very beleaguered NHS. It’s very hard but, having suffered both fecal impaction and years of fecal incontinence due to neglect and misdiagnosis I have learnt the hard way that I have to be very assertive with all HCPs. I now have an excellent rheumatologist but she doesn’t usually see scleroderma patients so I’m extra proactive and we work through it all together, seeking input of multiple specialists as required. This is all I can advise - keep reading up and pestering for a good rheumatologist who can work with you both and refer you appropriately and urgently when required.
Again, thank you so much for your detailed and helpful reply. You must be a very strong person and I admire how you have stood up for yourself, though what you have endured is not something I think I can even dare tell my wife who is very downcast - she had been brave up till this incident.I will have to look up several of the conditions and medications/interventions you have as I have no idea what many are. It sounds like (hopefully) you don't have intersitial lung disease which of course is another horrible burden, and she was also initially misdiagnosed as having "just" bronchiectasis. Out of interest, did I remember you rejected mycophenolate or similar immunosuppressant? If so, why? This is what the respiratory consultant decided after the emergency (remember, they haven't even let us see a rheumatologist yet).
No I don’t so far have any heart or lung complications thankfully and I credit 4 years plus on mycophenolate for this. My main risk according to my U3 RNP antibody is PAH/ pulmonary arterial hypertension rather than ILD. I was really struggling with bronchiestasis- like symptoms prior to starting Mycophenolate in 2020.
So I don’t quite understand why your wife’s respiratory consultant took her off mycophenolate nor how she got diagnosed with systemic sclerosis by any dr other than a rheumatologist? ILD is not confined to SSc - it’s quite common in mixed connective tissue disease and Sjogren’s too but mycophenolate is a good standard treatment for lung or kidney and skin involvement.
A scleroderma prof I saw last year told me that if I can’t tolerate mycophenolate or need additional immunesuppressant then Rituximab would be a very good second line treatment for me. I’ve tried all the others having initially been misdiagnosed with RA 13 years ago, then Sjogren’s. My own rheumatologist is now considering this if it’s decided that I’m not absorbing mycophenolate well enough
Many of us are under multiple specialists but usually only the rheumatologist can diagnose and treat and follow up really should be 3-4 monthly in the early days post diagnosis.
Thanks for your further feedback and deep knowledge. The anemia we think was caused by mycophenolate because the haemoglobin went down every month in the 6 months she was taking it and now seems to be raising slightly, thank God, after it stopped. Thered does seem a possible link per various studies. researchgate.net/publicatio...
Yes unfortunately, like all these immunesupressant medications, mycophenolate can affect the bloods adversely hence getting regular blood monitoring tests. Which kind of dr diagnosed and put her your wife on mycophenolate?
It was the respiratory consultant this January, whom she had reached via the mistaken diagnosis of bronchiectasis 15 months before. She is undergoing tons further tests including for lungs, GI Dr is taking another look at abdominal CT scan as they had no idea she had SSD til I told them on phone consultation. At least no ulcer as of now, that much is proven. Blood tests showed raised bilirubin, not sure what is causing that liver impact, would be rare for mycophenolate, I think. We begged after the emergency for the rheumatology professor to bring forward the first consultation he had earlier put back from July to mid-Sept but no luck.
PS Sorry, forgot to add that the antibodies for SSD were detected in bloods end of last year revealing SCL 70 positive, hence the respiratory guy knows what he is dealing with, even if the rest of the NHS Trust don't seem to unless I tell them.
So hang on scleroderma is thought likely but has she had the immunology blood tests and has a rheumatologist confirmed yet or are you still waiting for first consultation and specific scleroderma testing? I ask because a respiratory consultant can’t officially diagnose systemic sclerosis even if they have a strong suspicion of it. I can totally relate to the frustration of waiting. I’ve been under 7 or more rheumatologists over the last 13 years with changing diagnoses but had to travel from Scotland to London’s Royal Free privately to get a full diagnosis of systemic sclerosis last year. This was done by antibody, Raynauds, specific nailfold capillary changes and confirmed gastropsresis. Until then my various specialists were only allowed to refer to it as overlap connective tissue disease - although I was treated with mycophenolate and iloprost etc by my local rheumatologist regardless. I can’t believe how long you are having to wait fir a proper diagnosis but I do wish your wife the best of luck although it sounds like you’re already under a good respiratory dr. I used to have raised belibrium as part of my seronegative Sjogren’s and non alcoholic fatty liver disease. But this has reversed since by losing so much weight (unintentionally) and mycophenolate, losartan etc
I read your post, hang in there brother, i also had colostomy done and i know all these complications with intestinal and digestive problems can really be worrysome and unsettling. I just wanted to mention recently they started me on Immunoglobulin, and thid has worked wonders for both my stomach and intestine, my appetite has returned and i am starting to function normally again.
By the way i think best to keep away from coffee, it aggravates the ulcers in the stomach causing bleeding. Take care always.
Thanks so much for your lovely reply - although should correct you it’s sister rather than brother - but hard to tell with my Old Ted name I realise! I’m so glad IVIG has helped your gut dysmotility. Sadly I got two full rounds 2 years ago for this but had - severe allergic reaction leading to Steven’s Johnson so couldn’t carry on. Does this mean you were able to reverse your colostomy or do you still have it? I think mines either for keeps or else I’ll need ileostomy because my tail end is broken too and apparently beyond repair. Oh well I wouldn’t say my stoma is a disaster yet although am struggling a bit to keep up with the rapid output now! When I said ensure in coffees or teas I meant decaf only for reasons you mention! X
Dear sister Ted, yes i had my colostomy reversed thanks to God, but it was a huge surgery because they also removed a big chunk of the big istestine, the sigmoid portion, they slit me from top to bottom and cleaned me up because it was leaking. Surgery went well, so at least that is working now, it was painfull but definetely worth it. I did not expect the complications that followed, with SIBO ulcers bleeding, and loss of weight, nobody took the trouble to warn me.
Oh dear I’m sorry that you too weren’t warned of these things. I feel that my colorectal and gastro drs are always on back foot and never even mention scleroderma. I realise it’s rare but given the complications many of us suffer eg SIBO, gastroparesis, GAVE and GERD it is very disappointing that GI teams in my hospital seem so unaware. I don’t think they registered the extent of my rectocele or SIBO until recently. The colonic irrigation system stopped working so they won’t be reversing mine because I’d also need a new butt! But I did read that colostomy often works better for people like us than acresection - depending on the precise location of the damage of course.
I don’t know if you’ve found it a struggle to get departments to work together but in my case my rheumatologist hasn’t had any luck getting through to gastro or colorectal despite the severity of my GI issues. So she works closely instead with my neurologist (Erythromelalgia, migraine and spinal problems) and this was how I got offered the immunoglobulins. I have iloprost for my Raynaud’s and have portacath now and it’s my new best friend - although sadly no one on the colorectal ward was trained to use it so my veins on back a of scleroderma hands are all brutalised again now post surgery! Hey ho onwards and upwards bro!
I’m so sorry your wife is in such a bad position. I can only imagine the stress. I can’t say I’m in the same boat, but systemic sclerosis is doing a number on my entire intestinal tract. I recently had dermatomyositis added to the mix. I take mycophenolate but it didn’t improve matters so I recently had two Rituximab infusions. It’ll take some time to see if it helps.
The effects on my intestinal tract caused me to lose a great deal of weight and it’s extremely difficult to regain any ( very easy to lose it though) . At the moment I have no appetite but occasionally have what I assume is a blood sugar drop. Seeing the dietitian this week. There’s some sicca which doesn’t help when I do eat my sloppy stuff!
They’re going to do a colonoscopy to check for microscopic colitis (?) . I take Rifaximin for a week every month which works magic for SIBO. I did experience bad constipation some time ago but now it’s quite the opposite.
May I ask what treatment your wife has for Raynaud’s? I need monthly Iloprost infusions. When I researched Iloprost I found that the inhaled formula helps with ILD.
I should add that all my treatment is coordinated by Rheumatology: I lucked out with a consultant at a centre of excellence which happens to be virtually on my doorstep. I’m horrified by your A&E saying no beds available.
May I add also my admiration for those who have undergone surgery? This is something I have to read about from behind the sofa.
Thank you, Lupiknits, for your kind words and helpful thoughts. She doesn't take anything for the Raynaud's other than gloves and a heating panel for her feet when sat/resting. The Micophenolate did seem to knock it back somewhat, so now it is a bit worse a month after that was stopped. The lungs had been the main worry till now.... I alternate between fury at how poorly her situation is being managed and trying to keep her positive, which is not working at present. I intend to ask the respiratory Dr in the phone consultation next week why depts like GI don't even know that she has SSD (hence testing blind) and how unacceptable it is that she has not seen a rheumatologist, with a request to move care to the Royal Free instead of St. George's. Has anyone managed to get their care moved to Royal Free?
I do hope you have some success in getting the medical and surgical silos to join up and take a team approach. As ever, scleroderma/ systemic sclerosis is not at all common so it takes pushing from the patient and family to try and get things done.
I think others know more about London hospitals and specialists than I do. Best wishes to you both.
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