Thursday, December 19, 2013

Ken's Ordeal Part 12 in Which We Learn of a New Disease, RS3PE

Ken's appointment in Rheumatology was at 2.  At about 3:30 we finally met the rheumatologist, Dr. Z., a "fellow" as it turned out.  He looked very young and he was extremely serious.  English was clearly his second language.  I was not impressed. 

He hurt Ken badly, bringing him to tears during the exam.  He did apologize profusely about five times.  He asked all the usual questions, and he told us that he had actually spent several hours reviewing Mayo notes and conferring with Dr. N. the staff rheumatologist, who was, he assured us, "very good doctor and very experienced."  I thanked him for taking the time to read the notes.  He nodded seriously.  After about 15 minutes he said, "I have ideas.  But I am calling Dr. N."

More than a half hour later, Dr. N. entered the room.  Ken, meanwhile, had left.  He was too agitated and too uncomfortable on the tiny exam table, and I hadn't been able to convince him to stay put.  I called his cell phone.   Dr. N. was not amused.  A small dapper man in his 50s, he asked me questions and tapped his foot.  I wasn't too impressed with Dr. N. either.

Ken arrived.

After more questions, he said, "Dr. Z. and I independently reviewed the records sent from Mayo.  We each came to the same conclusion.  Not everything fits, but many things do.  We think you may have
a very rare disease, Remitting Seronegative Symmetrical Synovitis with Pitting Edema, or RS3PE for short."

Ken and I just stared.  What?  He went on, "Rheumatoid Arthritis, or some other rheumatic disease is really not the disease.  It is a symptom.  What really sent us in the direction of thinking you have RS3PE is the comment someone put in the Mayo notes, that your hands looked like baseball mitts when you came out of the surgery to remove the pheochromocytoma."

I nodded, saying, "Yes.  I said that to the doctor at Mayo.  Ken's hands had been badly painful and quite swollen for weeks.  But in the recovery room, well, it was shocking."

"Yes," said Dr. N.  "Very descriptive of you.  So let me tell you the symptoms of RS3PE."  And he listed them.
  • Symptoms that appear to be seronegative rheumatoid arthritis (RA).  ("Seronegative" means that the symptoms are of RA, but the lab work does not indicate RA.  The Mayo rheumatologist said they suspected seronegative RA.)
  • Extreme pain in shoulders and hands and often other joints.
  • Pitting edema (swelling) particularly in hands or feet.
  • Fatigue and general illness.
  • Severe weight loss.
The doctor went on to tell us that this disease was "discovered" in Milwaukee, Wisconsin.  And then he said, "It just seemed too coincidental that your tumor symptoms became extreme at the exact same time as your hand swelling and the severe, all-over pain began." 

We nodded.  I asked, "What do you mean, 'coincentidental? How are the pheochromocytoma and the hand swelling connected?  I have lost track of how many times one of us had said to some medical doctor, 'These two things just have to be connected, somehow.'  After a while we stopped saying it because our comment never led anywhere."

The two doctors glanced at each other, and then Dr. N. added the sentence that made my mouth drop open.  "RS3PE is associated with tumors.  Nobody knows why."

And I exclaimed, "Get out!" (I think Dr. Z. thought I literally was telling Dr. N. to get out of the room, because he looked at me in surprise.) 

And the St. Luke's rheumatologist, the doctor who saw Ken's hand and their swollen condition about a day after the tumor came out, and the Menominee Falls rheumatologist, and the Marshfield rheumatologist and the Mayo rheumatologist did not make the connection?  But two doctors at the Veteran's Administration did?  Dr. N. turned us back over to Dr. Z and left.  I wanted to hug the serious young Dr. Z, but I was trying not to cry. 

The bad part of this is that it is so rare that he admitted he really did not know what to do next.  "Usually," he said, "prednisone is the treatment that works well.  We have already been researching when we read the Mayo notes.   But you have had prednisone with no effect." 

They are going to do further research and talk to Ken in a month or so if not before.

I later did some research on the Internet.  RS3PE occurs most often among white males over the age of 60, particularly those who live in rural areas.  

Usually when prednisone does not work, a cancerous tumor is found upon further investigation. 

I do not know what to think.


LoieJ said...

I've been thinking of your both and wondering how things are going. Or not going, I guess. Sigh. Yikes. More waiting, it sounds like, unless that doc oks you to go elsewhere.

I think that the VA clinic you can go to locally is a direct VA clinic, not a clinic run by a contracting company. I don't know if that makes the staff better, but I do know that they wouldn't be jerked around by the system where the contracting companies bid for the right to run the various clinics. My husband worked "for the VA" for 7.5 years, but in reality, he worked for a contracting company and had a very good nurse-manager who really cared about the patients. Well, the contract went to a new low bidder, and there were many questionable things that this new company did in the months leading up to the changeover from the old company. My husband decided there were too many red flags, and he declined their job offer. Now he is hearing stories of difficulties, basically, that the company doesn't treat the local employees very well. He's glad he is out of there. So, when you've written before, I've read with some of his experiences in the background. I used to post as PS anAfterthought, the one whose mother was from Cleveland Wi, and I'm on FB with you as well. I've decided that two different sign in names was too complicated. I hope that Ken feels well enough that you-all can have some Christmas Grace.

Dorcas (aka SingingOwl) said...

I posted too soon! This wasn't done. Oops! It will be back up later.

Dorcas (aka SingingOwl) said...

Yep, Loie, I know who you are.
:-) And now the whole post is up. And no, Ken does not feel well at all. :(

Deb said...

Geez. I'm at a loss for words... So I'll keep praying...

Anonymous said...

I can't help but think that in spite of the fact that you (& all of us I'm sure) we're so disappointed that you couldn't continue at the Mayo Clinic and had to go back to the VA that, in retrospect, it was certainly a they are the ones who could finally add 2+2. I pray that you will be encouraged that now that they know what they are dealing with, even though it is rare, they will be on the road to researching/investigating HOW to treat it and to be pro-active about not letting any tumours grow. I am so impressed that you can even regurgitate all this information Dorcas! I had to re-read some sentences a couple of trying to grasp the meaning. I pray that your hearts will be filled with renewed hope for the future and that the promise of God's mercies being new EVERY morning will be a vibrant reality in your hearts and minds every single day. love, Maureen

Robin said...

This must be overwhelming to digest. At all. But these seem to be bright and committed doctors, bedside manner aside. This could be genuine progress.

The Rev. Terri C. Pilarski, M.Div/MSW said...

Oh my goodness. The path to get here - the determination on your part to get a diagnosis - such a terribly long road. I am stunned by the outcome - a diagnosis that finally seems to really be what it is. Holding you in prayer in new ways.

Dorcas (aka SingingOwl) said...

Thanks. Robin, that is my hope. Terri, we were stunned. I mean....there were really no words.

Dorcas (aka SingingOwl) said...

Maureen, I don't think RS3PE causes tumors, but that is the other way around. But what do I know???

Ruth said...

My father's VA docs are very good but things move extremely slowly. One of his docs is using dad and 2 of his brothers as a research project due to the 3 of them having very rare neurological diagnoses. The VA can give good care if you hit the right people.

I'll continue to keep you both in prayer. A diagnosis can at least set your feet on the right path toward treatment. ~Ruthie

Jules said...

I am so sorry for all of the pain that you and your dear ones have been through. Just so very sorry...

My mother suffered for years and years from a very strange set of symptoms that the best specialists in the fanciest hospitals in Indianapolis could not diagnose. Finally, her GP in our tiny town figured it out--by seeing an old photo of her.

I'm still holding out hope that this new diagnosis will bring some healing for you all. ((((you))))

seethroughfaith said...