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.

Wednesday, December 18, 2013

Ken's Ordeal Part 11--From the Mayo Clinic to the Veterans' Administration at Milwaukee

As I typed the title, and "Part 11," I stopped and walked away from the computer for a few minutes.  I am astonished, and not in any sort of positive way. 

When I began these posts with the label "Ken's Ordeal" I had no idea that there would be so many installments. And here I am, one year later, and I look outside, and once again the ground is snow-covered.  Spring and summer came and went with no real answers and no celebrating of restored health.

As I wrote in Part 1, Ken has been increasingly ill for some time.  No one paid much attention. 

Anyway, the tumor was discovered in December of 2012, as the urgent care doc looked for possible kidney stones.  It was, he said, "Pretty common, likely benign, and usually nothing to worry about."  He told Ken to make an appointment with his primary care doctor after the holidays.

And then Ken's rapidly deteriorating condition grew alarming, and I took him to the Emergency Room in a snowstorm, praying all the way that he would make it.  As the days in the hospital passed and tests confirmed that the nothing-to-worry-about tumor was very dangerous indeed, no one seemed too alarmed about the hand swelling he was developing nor the horrible pain in his back, shoulders, hands and neck.  It was all likely part of the strange array of symptoms caused by the tumor.  After all, they were so rare no one really knew quite what to expect from a patient who had one. 

Weeks went by as Ken took meds to prepare him for the risky surgery. The tumor was removed in February.  And Ken's erratic blood sugars became more stable than they had been in years.  And his weird "episodes," that I have written about in previous posts, ended.  And his high blood pressure became a bit on the low side--and we breathed sighs of relief.  But his pain continued, and his weight loss continued unabated.  Tests revealed nothing wrong.  He tried stronger and stronger pain meds.  Alarming loss of weight (and height) continued, along with fatigue, worsening depression, and general illness.

I won't retell the whole story.  I wrote in September that after multiple visits to Mayo Ken was told he likely had a rare kind of Rheumatoid Arthritis or some other rheumatic disease.  And his knee replacement, the one he had about ten years ago that had never been right, would have to come out.  There would be no treatment for the rheumatic disease until the knee was dealt with because treatment for the RA would increase his chance of infection, and taking the medication could even prove fatal if there was hidden infection in the knee.  The knee surgery would be extensive but needed to be done "as soon as possible."

Back in September, I wrote that Mayo doctors requested that the procedure be done there.  The Veterans' Administration denied the request.  He would need to come to Milwaukee.  Not long after that, we were informed that he could expect to soon have his "fee based" status changed.  That means any VA payment for services outside their system would end and he would be assigned a doctor in the VA clinic. 

Earlier this month Ken was finally assigned a primary care physician at a VA clinic about a half-hours drive away.  The clinic was reasonably spacious, and the staff was pleasant.  We spent nearly three hours there, and we really didn't explore anything in any depth.  The doctor, who seemed competent, was forthright in acknowledging that he had not looked at the numerous records from Memorial Hospital in Sheboygan, St. Luke's Hospital in Milwaukee nor anything from the numerous visits to Mayo. He simply didn't have time. 

Then, a few days later, we went to Milwaukee to the "big VA" for a visit with an orthopedist. We are still waiting for the knee surgery that needed to be done, "right away."  I have no desire, nor does there seem any point, in detailing the numerous frustrating reasons for delay.

I couldn't help but contrast the VA with Mayo.  The VA is overcrowded.  The buildings seem always in need of paint or repairs.  The furniture is a bit shabby.  The magazines are sometimes years old.  Scratches abound on walls and at the bottom of doors.  There is no parking ramp with elevators, just a big sprawling, always-full parking lot.  No beautiful artwork at the VA either.  Instead the walls are graced with the same posters that have been there for decades.  No music and no beautiful atriums or highly-polished floors.  No Chihuly blown glass here!  However, the people at the VA are helpful, and the majority of the staff, though obviously sometimes overwhelmed, seem to care.

The orthopedic clinic waiting room was overheated and very crowded. On a table with the usual dated magazines stood an ugly plastic rubber tree plant.  The pictures on the walls were badly faded. We sat in the waiting room for well over an hour.  When we finally got to see the doctor, he apologized, saying that the other ortho doc was out sick.  After the usual exam and review of Ken's history, the doctor told us that it was clear the knee replacement was loose and it had to come out.  He ordered more tests to try to discover any infection.  He scheduled some sort of special three-stage bone scan that will be done in January.  Then he told us that surgery would have to wait for three to four months.  The waiting list is long and they are overwhelmed--too many veterans and too few operating rooms.  He said the surgery might be able to be "fee-based" meaning it might be approved to take place outside the Veterans' Administration system.

BAM.  BAM. BAM.  That is the sound of me beating my head against the wall.  Not really, of course, but inside my own imagination.  Hadn't we all but begged for that, back in September? We waited nearly four months only to be told that we might be able to go elsewhere.

And he suggested that Ken not wait to schedule the appointment with a rheumatologist.  Even though treatment from those doctors might have to wait till the knee procedure was healed, it might be a good idea to meet.  So we  scheduled an appointment.

And, as it turned out, something surprising happened.  More about that soon.