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Daphne: Daughter of Scleroderma/Polymyositis/Antisynthetase Syndrome Overlap Patient

She was told her overlap syndrome was so rare that a rheumatologist may only see one case, if any, in his career.

Single Peach Blossom by Sherrill Knaggs, ISN Artist My mom's symptoms started in 2002. She began having joint pain, so much so, that she had to change jobs due to the pain.

She began to see doctors for this, and they told her she had osteoarthritis, at forty-three years old. She saw a few rheumatologists and they agreed it was arthritis. She was told that her joints were just worn out.

In 2003, fairly suddenly she was unable to raise her arms higher than the level of her shoulders. They told her she had "frozen shoulder." An operation and physical therapy were done, with no help. She had a positive ANA, or anti nuclear antibody test. Doctors thought that her problem still laid with arthritis.

Being a nurse, I took her symptoms and did some research, wondering if lupus was a possibility. She was sent to more rheumatologists, who told her "you have no connective tissue disease."

She had a lumbar fusion on her back, due to numbness in her legs. So, she took anti-inflammatory meds for quite some time.

By early 2005, she began to exhibit Raynaud's phenomenon. She was also having swelling in her face, hands and feet. By late summer, she began to have trouble swallowing.

I took her back to the doctor and insisted that this was not arthritis. Her practitioner agreed, and ran a slew of tests. She was excreting a large amount of protein in her urine, so she was sent to a nephrologist. The nephrologist did a very thorough history and physical. He told her he believed she had scleroderma.

This was a new thing, we had not heard of. He told her she was exhibiting CREST syndrome. He sent off more lab work. He reported he would refer her to a rheumatologist in Denver. The initial tests came back negative for scleroderma.

After many calls and pushing by me, we got her an appointment with the doctor in Denver. By this time, she was so weak, she could not get up from a chair alone. She was choking on everything she ate and had lost at least forty pounds. She was short of breath and was very sick.

We saw the doctor on December 12, 2005. He agreed that she had scleroderma. He reported that he was going to refer her to The University of Colorado hospital in Denver. "This may take a few months," he said, "but that's okay."

At this point, she had been seeing a pulmonologist for her interstitial lung disease, and a gastroenterologist for her swallowing issues. She had upper and lower GI scope with esophageal dilation in November, before this visit. The doctor in Denver told her to keep seeing the pulmonologist and gastroenterologist. So we took her home, still with no treatment.

By December 28th, she started spiking with a high fever, and was admitted to the hospital with aspiration pneumonia.

Finally a feeding tube was placed. I had been pushing for one the last few months. Her local doctor was able to get her an appointment at the University of Colorado. The appointment was for January 9, 2006, so the doctor discharged her from the local hospital on Jan. 8th, so we could drive her by car to Denver.

She had some nausea prior to being discharged from the hospital. She had only been on the feeding tube for two days. About halfway across town, mom vomited, and aspirated all of it into her lungs. The ambulance was called, and she was on the verge of respiratory arrest. She was transported back to the local hospital and put in the ICU. She was life flighted to Denver the next day, Jan. 9th.

When we got to Denver, she was seen by a whole lot of doctors, but the head of rheumatology did an assessment and asked us if she had ever been diagnosed with polymyositis. Of course, she had not. They checked her creatinine kinase level, or amount of muscle deterioration. The normal high number is right around 200 or a little less. Hers was 2400. She was also diagnosed with anti synthetase syndrome. She was told her overlap syndrome was so rare that a rheumatologist may only see one case, if any, in his career. The appropriate treatment was started.

Within weeks, she was pretty much up and running in the halls. Her weight was only one hundred and fifteen pounds. She had previously weighed one hundred and sixty five pounds before any weight loss. But she was getting better. Not without complication however, as she had a blood clot in her heart, and I cannot remember everything else.

By the end of January, she was able to go to a long term care facility locally for therapy. After being there for two or three days, her feeding tube got clogged. I took her to the ER. They were unable to unclog the tube. She was taken back for a simple procedure, with a scope,to replace the inner feeding tube. After the procedure, she was in pain, and her stomach was so big, she looked to be nine or so months pregnant. I took her back to rehab facility. I told her she had to walk off the air. She walked and walked, even though she was hurting bad.

After three days, her abdomen did not decrease in size, nor did the pain let up. She was directly admitted to the hospital. The surgeon pulled the feeding tube out in her room, the whole thing. He reported that the air from her stomach had escaped into the space between her organs and her abdominal wall.

I really felt strange all day. I had a terrible feeling, and I did not want to leave that day. She told me to go home and do my homework. I left, still unable to shake that feeling.

At 2:45 am, my phone rang. All my step dad said was "get here now." I came into the hospital, and she was now in the ICU. I came into the ICU to find the staff resuscitating my mom. She had gone into respiratory arrest.All I could do was just sit and stare, as I knew exactly what was happening. After a short while, she began to respond. The doctors where puzzled. They assumed the blood clot from her heart had gone to her brain.

I watched as they scanned her in CT. The CT scan showed a large amount of air between her organs and abdominal wall. They were able to find no other cause why she stopped breathing. The air had caused her to go into multiple organ failure.

When we returned to her ICU room, she was now not really with it, and on a ventilator. The surgeon cut a hole in her abdomen and the second he poked through the last layer, a large amount of air came whistling out. Her abdomen deflated like a tire.

She became septic and had a blood infection. She was very gravely ill. The doctors told us they suspected she had a necrotic bowel, or that her bowel had "died" and that she was too sick to be operated on. A necrotic bowel without surgery is a death sentence.

Somehow her temperature stabilized, and her blood pressure did not require medicine to keep going. We requested she be transferred back to Denver, due to more advanced treatment options. She was lifeflighted after a week in local ICU. She was taken off the ventilator after approximately a week in Denver. However a day after that, she generated a large mucous plug and it collapsed her right lung and her heart stopped. She had to be resuscitated again. This time damage was done to her vocal cords and throat. She had been on the ventilator for two weeks and had to have a tracheotomy done.

In the next few days, she rapidly began to decline. The doctors in Denver now suspected a necrotic bowel. The had to operate, or she would surely die. They told us that she may not have enough bowel left alive to live through the surgery. She would most likely return with a colostomy or ileostomy. Luckily, they were able to remove the small portion of dead intestine. It had perforated though, or got a hole in it. Her bowel contents had literally burned the outside of all her organs. They were unable to close the huge incision due to all the fluid she had received prior to surgery.

For the next week and a half, she was taken down to surgery every other day. Her organs had to be washed off, and liquid band aid applied to them, with packing placed to try to stop the bleeding from the outside of the organs. She was getting blood, plasma, platelets, you name it, every day. She had more tubes and drains than anyone I have ever seen. She ended up being in Denver for two to three months.

After being discharged from University Hospital, she weighed eighty-eight pounds. She was a skeleton, literally. She was sent to a rehab hospital in Denver for another month for intense therapy. Eventually, her trach was taken out. She still could not eat. She eventually began to be able to walk again.

Finally, she was released with local home health care. She had to relearn to eat. She takes an enormous amount of medicine. She has, over the rest of this year since her discharge from the rehab hospital in late April 2006, been getting better.

She was able to attend my college graduation in early May. What an emotional day for both of us. I had prayed so many times for it. It happened. She is now up to one hundred and thirty pounds. She has decreased her tube feeding significantly and eats well.

Her disease is almost in remission per the recent report from the rheumatologist in Denver. Our biggest obstacle is dealing with the depression and anxiety she suffers from. She has such a rare illness, and no one to relate to. It is a daily battle just for her to have a good day.

I try my best to help her through it. I hope everyday she will get some relief from the anxiety and depression. Sorry the story is so lengthy, but it just is not the same unless you know the whole story.

To Contact the Author

Email: [email protected]
Story edited 11-24-06 JTD
Story posted 11-29-06 SLE

ISN Senior Artist: Sherrill Knaggs
Story Editor: Judith Thompson Devlin
Anxiety and Depression
Aspiration Pneumonia
Caregiver Stories
Difficult Diagnosis
Dysphagia (Swallowing issues)
Feeding Tube
Interstitial Lung Disease
Overlap Syndrome
Raynaud's Stories
Types of Scleroderma
What is Scleroderma?

ISN Artist: Sherrill Knaggs (In Loving Memory)

Sherrill KnaggsSherrill Knaggs, ISN Artist, created the digital photo to illustrate the story on this page. Sherrill lived in New Zealand. Her story was featured in ISN's book, Voices of Scleroderma Volume 2.

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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