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Mars: Scleroderma with Full Gastrointestinal Involvement

New Zealand. I finally started total parenteral nutrition (TPN, or tubal feeding) and it was an absolute success!

Dog, Colored Pencil by Shelley EnszAfter four years of being told that I had psychosomatic problems, my doctor finally diagnosed scleroderma. He laughed when he told me because it was considered so rare. In 1988 a formal diagnosis was made and essentially, I just got on with life. Heavily involved with horses and shows, I kept very active even though it appears that an overlap syndrome of polymyositis (muscle inflammation) caused weaknesses in the major muscles. CREST was starting to show itself with Raynaud's, esophageal reflux, sclerodactyly appearing during the four year period.

Over the next few years, little problems started raising their heads a bit more. I began to get problems with diarrhea, sometimes for long periods and then associated dehydration. Quickly following that, anal incontinence started to make itself known. Add the incontinence to sudden diarrhea attacks things started to get a bit shaky. Eating also became a slow process with difficulty swallowing. But still not too life impacting except for the diarrhea attacks.

In 1994 I began on prednisone, and also tried things like azothioprine (which my liver played up with), methotrexate, cyclosporine E, and any other number of things tried by the general physician, all to no avail and at times to my detriment.

Finally things got so bad in 2006 that I had a colostomy as there were no interventions that would help the anal sphincter to recover and the attacks of diarrhea were frequent and deadly. By now I was moving with more difficulty from the polymyositis, and so decided quality of life was important. The operation went well, but small bacterial overgrowth began and it became quite a tyrant in itself.

In March 2007, I was formally diagnosed with small intestinal bacterial overgrowth (SIBO) and put on permanent antibiotics. Yes! Life was good at last. Walking the dog was my exercise by this point as horses had long had to be discontinued.

On January 5, 2009, I fell over the tow bar of my car onto the concrete and broke my upper right femur. They gave me strong pain medications and also a medication to offset the supposed constipation side effect, not that I suffered any of that. Ever. After a month and when nearly ready to go home, I got a bowel impaction where the bowel hooked up onto an old adhesion (old scar tissue) and I had to have an operation to free it. After ten days my intestinal tract finally woke up. And never worked right again. My sense of taste was fading, my sense of smell was acute, my appetite was nil to sheer rejection of even the thought and sight of food. I tried, but after finally getting home my ability to eat and drink became seriously compromised.

By June of 2009, I was continuously in and out of hospital with dehydration and vomiting. I kept trying to eat and drink and couldn't manage virtually anything.

In October 2009, I was sent to Christchurch Hospital for esophageal tests. Two weeks earlier my local hospital doctors said that a food tube inserted into the stomach may be in my future but not yet. In Christchurch the results came in that due to the distended small and large colon, and the lack of peristaltic motion, I urgently needed a jejunostomy (feeding tube directly into the jejunum just below the stomach). After being booked for one in two weeks, I had a bout of pneumonia which held it up another week, but despite misgivings over my general health it was deemed very urgent to go ahead.

On November 30, 2009, I had the jejunostomy inserted, but things continued to be difficult. I felt like I was drowning, and couldn't maintain the infusion rate necessary to survive. Suffice to say that the next few months were miserable to a certain extent as I battled on, determined to make it all work. I would also like to point out the throughout this time, due to serious malnutrition, the fracture in my upper leg had failed to heal. It does happen often enough, called a non-union. So I still have a pinned but broken leg. Lack of mobility was not helping.

My medical support staff, rheumatologist, local GP, hospital staff, district nurses, PEJ nurse, Stoma Nurse, and dietician have been absolutely fantastic. Dehydration was making itself known again, so we began subcutaneous fluids overnight at home with the feeding through my tube. But that didn't work as I had lost so much weight the water basically fell to my legs, or would not go into my body. Bacterial overgrowth had again raised it's ugly head but I finally, after a fight with surgeons and another test, I got the help I needed with antibiotics, thanks to my rheumatologist. Still weight loss was continuing quite quickly. My weight at time of accident was about 88 kg (chubby bubby that I was).

A final decision was reached after I had a consultation around a table with my local GP and my rheumatologist. We decided that I would have a porta-catheter for home based total parenteral nutrition (TPN) inserted. This happened mid-May 2010. It was done under local anesthetic in Nelson Hospital. It was an absolute success from the moment it went in and I started receiving TPN. It was lovely. I had no more bloatedness, no air pains, no diarrhea, and no pseudo blockages. My dehydration was under control, my feeling of well-being and stamina started to improve, my weight stabilized, and I just felt so much better. I looked at my wheelchair used for more demanding exercise and thought that I might just be able to get beyond it.

Then another blow came. Just over a week ago I got sick with severe chills. I just thought it was because I had tried to eat something and when I did that my body would reject it and cause diarrhea. After some serious testing, it was found to be pseudomonas, a particularly resistant and dastardly bug in the blood stream, which is a real risk for TPN users. So I went back into the hospital for heavy duty IV antibiotics. I got out yesterday, but it is quite possible that I will need to have the porta-catheter in my right shoulder, which is just under the collarbone, changed to the other shoulder. I will have weeks more IV antibiotics. However, I just have to roll with the punches and if this is what I need to do to get better, that is what I will do.

While this is a brief overview of my story, I would like to know if anyone else has a history of total involvement of the intestinal tract, and if you have gone onto TPN at home or in care. There is a lot more information that I am willing to share, and I would love to hear from anyone who has helpful hints. I have CREST completely now with some skin thickening on the side of my fingers. But to be honest, they don't impact very much. I also have a restrictive lung disorder which means my cough ability is limited.

Otherwise I am in very fine form and my sense of humor is still intact. We just have to roll with the punches, even though we still might have some down days.

~ Update 11-22-10 ~

I think the last I may have mentioned is that I had a bad blood bug found in the portacath in my right shoulder. It was called pseudomonas which is a particulary nasty one. My medical bods gave me an antibiotic called ceftazadime (or something like that) for a month, then I was sent to Nelson. They removed my portacath, then four days later replaced it with one in my left shoulder.

All was going well. Then we were unable to aspirate (withdraw blood) from the portacath. It was discovered that I had firbins (blood tailings) at the end of the catheter that acted as a trapdoor sealing off the end of the catheter whenever you tried to withdraw blood. After several visits to the hospitals Emergency Department, we decided to leave it alone for the time being. Thank goodness. Home again and cruising.

Three weeks later, I had three episodes, one each night, where I had bouts of septic shock, or septicaemia, or rigors or whatever they call it. The rigors can be very painful. The first night wasn't too bad. The next night I had a lot of pain from my waist to my knees as my muscles spasmed with the tremendous shaking.

If you haven't had this, it is where the body is trying to fight off the infection. The temperature rises so fast and high that you are absolutely freezing. The whole bed shakes with you as your muscles shake, and your teeth chatter really badly, almost like a cartoon of somebody coming out of ice. It lasts for about an hour.

After the second night of this, I went to my doctor. She checked with the Emergency Department who said to take my own temperature 4 hourly and see what happened. By this time I knew that I had a bad bug back in my blood as it was exactly what had happened previously. It is pretty nasty to experience. So I went home.

That night I ha d a third bout. When I took the pain medication, I ended up throwing it up as the pain was so bad and I tend to react that way to pain. My brother and I decided to call the ambulance as I have an alarm. I live about 300 metres from the hospital. It took them an hour and a half or so to arrive, and it was an ambulance team from Havelock which is 35 minutes away.

My blood pressure had dropped down to a top reading of 77 rising up to 88, before wandering all over the place and I was tachycardic, which means my heart was racing. They admitted me after taking blood cultures, and miles of blood tests. They also removed the portacath, and put a femoral vein mid-line in. We hoped it would last for four weeks but as it is a site very prone to infection they weren't sure what would happen. A day or so later, I went home from hospital.

At two weeks and a couple of days, my legs started to swell up, the right side more than the left. I had an appointment with the doctor in medical outpatients and he admitted me into the Acute Assessment Unit, which is an overnight stay ward mixed with the old ICU which is now called HDU High Dependency Unit.

They gave me an ultrasound up the right leg, particularly the femoral vein midline site, and the abdominal area looking for blood clots. They also put a urinary catheter in with intravenous frusemide to remove the water building up. They took out over 7 litres of fluid. Then I went home With 20 mg of frusemide. Over the next couple of days, I had no reaction at all to the drug for water retention but my leg got bigger. On the Friday I went to my local GP who upped the dose firstly to 40 mg then 80 mg. Still no change to fluid output.

Saturday my leg was so huge, so tight and uncomfortable, back I went to the Emergency Department. Thank goodness they know me. First thing they did after a huge amount of blood tests was to give me an ultrasound again. And there it was. A 20 cm ("massive" they called it) DVT (Deep Vein Thrombosis) running alongside the midline intravenous tube from abdominal area into the leg. So it was obviously forming earlier in the week. So onto Warfarin, Clexane stomach injections twice a day and daily blood tests to watch the warfarin clotting time. They put a PICC line in about 3 inches above my right inner arm, and once that was properly sited, they removed the femoral midline. I was readmitted to the ward.

At least I never missed any of my TPN. Even in hospital, I was responsible for and administed my own antiobiotics and TPN. By now I was also on Ciprofloxacin which is an antiobiotic which I take orally. Once I looked stable enough everywhere except my huge right leg that looked like it belonged to someone else (and still does).

The hospital uses me every so often to help in training because staff very rarely see any of the things I deal with on a day to day basis. That is good fun. You never have just one person doing anything, the other nurses and an occasional doctor all squeeze into the cubicle when anything is being done.

So here we are, I have a PICC line in. Originally it was only going to be until the beginning of December, but I think if it stays going okay they may hold off until they need to change back to a portacath. The portacath is much easier to handle and doesn't have the same (fairly low) level of discomfort. I will no doubt find out tomorrow.

Oh, and I have to wear an extremely ugly orthapaedic knee high stocking, pressure stocking I think they are called. Still haven't found anyone with fabric paints to pretty it up.

And just to let you know, my brother and I are moving my mother in with us in Blenheim until my place sells then mum and I are buying a house in Picton. I have had to go through to try and stop her (she is 80) doing too much but do some packing. Thank goodness for my aunt and cousins. I can only do so much as my leg gets badly fatigued and I have to sit down with my leg up. I feel so guilty, but have to put up with it as the last thing any of us needs is for me to have further problems. They happen pretty regularly on their own. I also have been having open home days with the real estate salespeople, and I am taking my place to auction December 1st.

I have a horse show to open on the 3rd December, another horse show to visit in Nelson (about 1 1/2 hours away) and the doctor may still want me to have the portacath inserted about then.

Life is extremely busy. Certainly not boring. My brother is just wonderful even when he is being a real MAN, and I know I can depend on him for almost anything. I have even been eating food occasionally and having a cup of coffee a day. Yay, nice change from water.

Anyway, that is pretty much me up to date in case you are interested. Hope you are all feeling as good as you can, and that you and your doctors are working together for your benefit, or that of your family. Thinking of you and if I don't have contact with you before the holidays, have a wonderful holiday season and a great New Year.

And if you can dance naked on the table, and you want to, go for it. Luckily I can't get up there without a lot of help, so not something I will indulge in. Kia kaha (stay strong).

To Contact the Author

Email: [email protected]
Story edited 07-08-2010 JTD
Story posted 07-15-2010 SLE
Story update posted 11-22-2010 SLE

Story Artist: Shelley Ensz
Story Editor: Judith Thompson Devlin
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ISN Story Editor: Judith Thompson Devlin

Photo of JudithJudith Thompson Devlin is the ISN Story Editor for this story. She is also lead editor of the ISN's wonderful Voices of Scleroderma book series!

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