Intro - 14 years since dx, 25 year history of PSC

Greetings, all. Since I'm new here, I figured I'd introduce myself. I'm male, 45, and live in Albuquerque, NM, USA. I work as a systems engineer, which in my case involves lots of on-site electromechanical troubleshooting of factory automation equipment and high-speed packaging machinery. Much of my current work is in a factory that makes desiccant canisters and packets for the pharmaceutical industry - the annoying little plastic cylinders or paper packets that you find in pill bottles, that invariably say "Do Not Eat" on them. It's likely that most people here have at some point handled products that came from machines that I work on. :-)

When I'm not working, I'm with my family. I have two sons and a stepdaughter, all either "tweens" or early teenagers. I got remarried about a year and a half ago, so my second marriage is still pretty new, but my wife is wonderfully supportive of me with my various medical problems.

I'm also a hot air balloon pilot. I fly in many of the local balloon festivals, including the Albuquerque International Balloon Fiesta, which is one of the largest (if not the largest) balloon events in the world. Sharing the sky with 550 other balloons is really a treat.

I was diagnosed with UC in 1988, and PSC in 2001. However, my elevated liver enzymes go back to at least 1990, so I'm pretty sure that I've had PSC since long before the diagnosis. However, by 2001 my history of elevated enzymes was long enough that my GI did an ERCP and came up with the diagnosis. I was entirely asymptomatic (apart from elevated liver enzymes) until the spring of 2011, when I started having problems with itching and fatigue. I'd have a few days of symptoms, then it would clear up for weeks or months, but I had several bouts until November 2012. I was visiting family in California for the Thanksgiving holiday, and very suddenly developed a complete biliary obstruction. Fatigue, nausea, and itching were suddenly horrible, and made the rest of the trip pretty miserable.

In December of 2012, my GI did an ERCP and stented the left and right bile ducts. Unfortunately my ducts don't follow the usual routing, and he was working largely blind because of the blockage, so one of the stents ended up puncturing the bile duct. I woke up from the anesthesia feeling OK (other than nausea, which is my routine reaction to anesthesia), but as they were getting ready to discharge me, I was suddenly in the worst pain I could imagine. In a matter of seconds I went from getting ready to go home to lying on the gurney screaming. They pumped me full of morphine, which did nothing for the pain, and I spent the next three hours in the worst agony I've ever experienced, until enough of the morphine had cleared my system that they could give me something else that finally knocked down the pain. I spent the night in the hospital and was discharged the following afternoon, but within two hours of getting home I started having increasing RUQ pain and started running a fever, so I went back to the ER and was admitted again. That was when the GI realized what had happened, and the following day did another ERCP to remove the offending stent.

I continued to have milder symptoms on and off for the next year and a half, until mid-2014 when a blocked duct led to pancreatitis. It was a bit of an awkward time - I had just gotten married, and on July 1 I was going to get on her insurance so I could stop paying for my COBRA insurance. On June 29 I ended up in the ER with severe pain and other symptoms, and they found that my pancreas was inflamed to the point that other organs (my kidneys in particular) were shutting down. I spent five days in the hospital, which got into the time period when I was supposed to be on a different insurance plan, and thus in a different hospital. Much confusion ensued, which took months to unwind, but the pancreatitis cleared up uneventfully once they took out my gallbladder.

As part of the insurance change, I had to switch GIs, from the one who'd treated me since 1988 to a new one who I'm still working to break in. I've had ongoing problems with smaller biliary blockages (not like the total blockage in 2012, but enough to kick up the itching and fatigue), and it's sometimes hard to get appointments in a timely manner. In September of this year I called for an appointment about increasing RUQ pain, and the office staff wouldn't schedule me until November. In mid-October I ended up hospitalized for five days total with ascending cholangitis secondary to the biliary obstruction that was causing the RUQ pain, and I took the opportunity to work on my new GI to get me in on some sort of priority schedule - waiting six weeks for an appointment when I have PSC symptoms is simply unacceptable. I think we may have the problem worked out; I suspect that he realizes that my recent hospitalization was directly related to his staff's unwillingness to even discuss with him whether I should be scheduled sooner than their "next available" appointment. We shall see, though. At least now I have a good relationship with both of his nurses, so I can get past the front desk scheduling people to someone who understands the need for prompt medical care.

Meanwhile, I'm continuing to have RUQ pain and PSC symptoms. He did a balloon dilation during the ERCP last month, but when I saw him last week he said that we may need to do another ERCP and put in stents. I worry about that with the increased risk of ascending cholangitis, but the fatigue I've been having is making it hard to get my job done and do things with my family, so I suppose I'll take the risk for the chance to feel better for a few months.

Greetings to you, and sorry you've had so much to deal with and having to change doctors must have been really stressful. Sounds like you have an interesting career and always love to see the hot air balloons. There is a hot air balloon event every year here at the Gregg Co. Airport. Hope you are feeling better and the doctors can get your symptoms under control. I have the good fortune to have a doctor who calls me back every time I have a problem and always sees me when necessary. I have just been diagnosed but think I've had it for a long time, just started having symptoms about a year ago but took that long to identify the problem. I'm sure they suspected what it was but went through all the testing to be sure. Take care and hope things are getting better for you.

Getting a good doc/team on your side is pretty important, and I hate bureacratic gatekeepers at doctor's offices.Having the doc's cell phone or email address is a good end run around them.

I would have hoped that the balloon dilation would have done the trick quickly of getting you some relieft. With my last ERCP, it was for a cholangitis attack. We knew the doc would have a choice of a balloon dilation or stent. He was able to get results with the balloon, and the results were ilightnening quick nad have lasted since March.

I wish yours had lasted. The thing with stents is that they are rather short term, so keep that in mind.

As for the fatigue, I have learned to pace myself at work, even if I'm slower, and have learned that weekends are for naps.

There was a 10K race that I did every year during which they had about 3-5 hot air balloons setting up near the starting line. They were impressive. I'm sure it takes a boatload of hard work and dedication to do that, not to mention your wife making sure you have some good life insurance!

Jeff

My last balloon dilation was a year ago, and it lasted until these symptoms kicked up in September. I had high hopes for this one. I’m not sure he was able to do as well this time - he dilated the common bile duct and one of the hepatic ducts, but he wasn’t able to get into the other. Perhaps we should consider another attempt at balloon dilation before stenting; that’s a conversation I’ll have to have with my GI. My only experience with stents was the ill-fated one in 2013 with the punctured duct. One of those stents stayed in place for six weeks before they removed it. Three ERCPs in six weeks wasn’t my idea of fun, though.

Ballooning is easier than most people imagine. There’s a minimum of ten hours of training, a written test (multiple guess, at that), an oral test, and a check ride with an FAA-appointed examiner. Same requirements to upgrade to a commercial certificate, which I’ll get around to doing one of these days. Much of the time I’m too fatigued to fly, so the days I feel up to it I mostly just want to get some blue sky under my feet and fly with friends instead of an instructor. I feel very fortunate that I felt good for the Albuquerque Balloon Fiesta - I flew for eleven straight mornings, and that really wears me out even when my PSC isn’t acting up. It was the week after that I came down with ascending cholangitis. But as long as I got plenty of sleep and took it easy. I did OK for the entire event. It helps that my crew takes wonderful care of me. They can set up and pack up the balloon with little input from me, so I could chat with landowners and neighbors instead of doing all the physical stuff. II couldn’t have done it without my dedicated crew.

LTA_Pilot,

Hello. I was catching up reading some old posts tonight and came across this one you started back in November. I was wondering how you have been doing lately with your PSC. That is just something you have had PSC for 25+ years. How are you doing with breaking in your new GI doctor? Have you considered switching over to a Hepatologist? If you are not satisfied with this new GI you might consider that. Have you been put on the transplant list yet and how is your MELD score doing these days?

That sounds exciting about the hot air balloons. I often seeing them flying over my home during the warmer months and they are always beautiful to see. Take care.

Mark Wilson, Moderator

PSC 2011 / Liver Transplant 7-2015

Mark,

My PSC has been good, or at least the labs indicate that - all values are in the normal range except alkaline phosphatase, although ALT is almost out of the normal range. Still, better labs than I’ve had for years.

The RUQ pain has continued, but isn’t as bad as a couple of months ago. However, I’ve been having a burning pain in the center of my upper abdomen. I was hospitalized for tests last month with it, and they did an upper endoscopy without finding anything. I have a gastric emptying study next week that may reveal something.

I just returned home this evening from a trip to Florida to buy a balloon. I flew out very early Friday and drove back in the dually pickup that came with it looking forward to flying it

Hello again LTA_pilot,

Glad to hear that your PSC has been stable now for years. Let's hope it stays that way!

Sorry you are continuing to have RUQ pain. I hope these next tests go well for you. When was the last time you had an MRCP done? I always got one annually during my years with PSC. That might be something you might consider asking about to get a good look in there at your liver and surrounding organs.

I figured hot air balloons were expensive, but to get a truck along with it, they must be mighty expensive. I hope you can recoop your investment by giving rides, doing photography or something along those lines. If you ever fly over central North Carolina let me know. I might see if you can land in my yard and give me a little ride. I've always wanted to fly in one of those things.

Take care!

Mark

PSC 2011 / Liver Transplant 2015

Mark,

I had an MRCP last year - I think in the summer. It's funny how all the tests run together, but I get them at least annually. I had my most recent ERCP in October, and they found atypical cells that they thought were a reaction to the infection I'd had and the inflammation. They want to repeat the ERCP sometime soon, but my doctor wants to send me to a different hospital that can do FISH, which is a more advanced cancer screening than typical brush cytology that looks promising. I'm waiting to get a prior authorization through my insurance company for a hepatologist visit, and the doctor wanted to wait until after that was complete before he tries to push through the ERCP, since trying to get my insurance company to do both at the same time is bound to cause them to screw up both.

My doctor is very good about trying to do everything we can for cancer screening, but it's still imperfect at best. And I still wish we could find out why I have the RUQ pain.

LTA_Pilot, glad to know your doc is on top of everything and you are getting all the necessary tests. I've heard of the FISH test although I don't believe they ever did that on me that I can recall. Hopefully you will get approval on that soon as well as the hepatologist. I'm surprised you have to get prior authorization to see a hepatologist. I just pay my co-pay at each visit and the insurance covers the rest. I realize though that all insurances are different so hopefully yours will approve soon. Stay in touch to let us know how things are going with you. Happy sailing through the sunny skies!

Mark

I need prior authorization for a hepatologist because it's an out-of-network doctor. My insurance is through a New Mexico-based health plan, and since there are no hepatologists in New Mexico I have to go to Texas, Colorado, or Arizona. I've already asked if my health plan will cover travel costs, which they won't - a hepatologist visit for me involves a few hundred dollars in plane tickets and hotels.

Dave