in addition to the PSC, josh has cholangiocarcinoma, also known as bile duct cancer or CCA. we don’t know what stage, what kind of treatment to expect, or anything really. we were just told he definitely has it. the hepatologist here in charlotte referred him to the mayo clinic in jacksonville, florida, so he has an appointment on july 14. we’re driving down on the 13th and i booked the hotel room for a week since we don’t know how long we’ll be there. my mom and josh’s parents are also going. that will be interesting since his mother has a broken leg and is on crutches. we’re taking winston with us too.

the good news is that josh’s spirits are good, he’s not depressed, and he’s determined to fight.

he seems to be getting a tiny bit better each day. yesterday he drove himself to the license tag office to turn in the CRX tag. this morning he drove himself to the orthodontist for a checkup (he’s had invisalign for about a year). of course both of those trips wiped him out, and he had no energy left by the time he got home. he’s lost about 20 pounds total but he is finally starting to eat normal amounts again, so hopefully the weight loss will stop. we still need to go to CMC to pick up the images from the two ERCPs so we can take those with us to mayo. josh will probably want to drive himself to CMC. he wants to push himself to do as much as he possibly can. i don’t blame him but i do worry about him driving when he’s so fatigued.

we’ve told our families and friends, who have all been incredibly supportive. we’ve also found some really excellent online support groups at psc-support and cholangiocarcinoma.org.

obviously we’re anxious about what we’ll find out next week, but we’re also hopeful. all we ask is that you pray. that’s all anyone can do at this point.

i’ll post more next week.

this past wednesday, we got a call from dr. russo, the carolinas medical center liver specialist, who said that josh’s liver is most definitely damaged. to what extent, no one knows because he hasn’t had a biopsy yet. i’m not sure why the doctors haven’t ordered one. dr. russo also said the brushings from josh’s latest ERCP were sent to mayo clinic for a second opinion. the pathologists at CMC felt that they were abnormal and so they sent them to mayo because mayo is where the PSC experts are. abnormal cells could mean one of three things: inflammation from the ERCP, pre-cancerous cells, or cancer. this type of cancer, cholangiocarcinoma or CCA, is rare (2 people out of 100,000), but it is not as rare among PSC patients, who have a 10 to 15% lifetime risk of developing the cancer.

dr. deal, the gastroenterologist, did a lot of work during both ERCPs, dilating as much as he could of the common bile duct. so it’s not unreasonable to assume that everything is inflamed from the trauma of the procedure. but josh is still jaundiced, itching, not hungry, and extremely tired. at this point, over a week after the ERCP, some of those symptoms should be easing up if the duct is staying open.

tomorrow is josh’s 35th birthday. hearing the big “C” from dr. russo is terrifying. 35 and cancer? it shouldn’t even be possible. so i’m praying with everything in me that it’s just inflammation. CCA statistics from cholangiocarcinoma.org:

The five-year relative survival rate (the percentage of patients who survive at least five years after the cancer is detected, excluding those who die from other diseases) for people diagnosed with early-stage cholangiocarcinoma is about 30%. However, only about 20% of cholangiocarcinoma is found at an early stage. The five-year relative survival rate decreases if cancer has spread at the time of diagnosis.

the PSC alone is bad enough. at some point josh will have to undergo a liver transplant. it’s major surgery that has continuing lifetime risks. and some people with PSC who undergo transplants actually have their PSC come back.

transplant statistics from mayo clinic:

Your chances of a successful liver transplant and long-term survival depend on your particular situation. In general, about 72 percent of people who undergo liver transplant live for at least five years. That means that for every 100 people who receive a liver transplant for any reason, about 72 will live for five years and 28 will die within five years.

People who receive a liver from a living donor have higher survival rates because having a living donor usually means a shorter wait for a liver. For liver transplants using living donors, the five-year survival rate is about 78 percent. That means that for every 100 people who receive a liver transplant using a living donor, 78 will live for five years and 22 will die within five years.

please keep josh in your prayers.

josh still feels awful. he’s actually taking a sick day today—one of the few times i’ve ever seen him do that. even when he’s sick he normally works from home, but not today.

we didn’t get out of the hospital after the last ERCP until 4 am, so we didn’t get home until almost 5 am. they had to keep him longer because he threw up right before he was going to be discharged, so they ran tests to make sure he didn’t have pancreatitis (5 to 10% of people develop pancreatitis after an ERCP). it ended up just being the anesthesia that made him sick, thank goodness. bad news is that he has to have anesthesia for every ERCP since he didn’t tolerate sedation for the first one, so we’ll most likely work it out so he stays overnight for future ERCPs. we couldn’t this time because we had no one lined up to check on winston.

but anyway, talk about a miserable night. he slept a little in the hospital but you know how they are—coming in to check your vitals right as you manage to fall asleep. i got no sleep at all and took a sick day the day after that. josh worked from home that next day.

josh’s mother broke her leg in several places this past friday night, so he spent most of saturday in the hospital waiting room while she underwent surgery to put a pin in. his dad and some of his brothers and sisters (he has 7 siblings) were there as well. apparently the siblings noticed how sick josh looks now. his mother called last night to check on josh and she said they asked if he was sick. he was hoping to keep things hidden from his siblings until he absolutely had to. but more people are starting to notice; even his boss told him yesterday he looked like he was sick and needed to get some rest. there has been no change in how he feels, but the doctor told him it could take a week or slightly longer for him to start feeling better. he’s still noticeably jaundiced, itching like crazy, and has absolutely no energy. i can’t get him to eat very much; he feels sick after he eats because his liver is producing bile that can’t flow very well through the strictured biliary tree.

my dad mowed the grass for us last weekend. he said he’d mow it again this weekend. sad to say that i haven’t mowed since i was about 14 or 15 years old, but i will be re-learning this weekend. we have a riding mower but the fenced-in portion of the yard has to be pushed-mowed because of the pool. i’m not sure i can handle the weed-eating so dad will probably still do that. there’s a bunch of stuff around the house i need to take care of, stuff that josh normally does, like changing the furnace filters and replacing burned-out landscape lights. i need to make a list.

we’re getting a break from the heat for the next week or so. highs will only be in the mid-80s instead of the mid-90s. i think we hit 90 or above for the last 3 weeks straight. the pool water is 93 degrees, which is not all that comfortable to swim in. hopefully the water will cool down some now that it’s supposed to be around 60 degrees at night.

that’s it for now. i’ll update again in a few days.

We’ve been at the hospital since 1:30 this afternoon. Josh had another ERCP. This time the doctor was able to work on both the left and the right sides of the liver. The work he did on June 15 didn’t take; the duct was almost completely closed up again. This time they put Josh to sleep (instead of just sedation) and the doc was able to dilate both sides to 6 mm. So hopefully this will stick and Josh won’t have to have another ERCP for 6 weeks. If his labs keep going up, he’ll have to have another one sooner.

He’s still very jaundiced, itching (a symptom of PSC), losing weight, and nauseated. Hopefully today’s procedure will help him feel better.

The doctor said there is no chance that Josh will live out the rest of his life with his original liver.

It’s 1:34 am and I can’t sleep. Josh has been in bed since 8 pm or so. He’s felt pretty bad since his ERCP this past Tuesday: jaundice, fatigue, nausea, no appetite. He’s been sleeping a lot, but he went to work yesterday and today.

The Urso makes him a little nauseated. He’s only been taking it two weeks, so it’s too soon to say if it’s helping. He’s also on an antibiotic (standard procedure after an ERCP). Blood work yesterday indicated his liver enzymes are back up again. Of course the doc didn’t give exact numbers, but we’ll get them at the next appointment on Monday.

The news doesn’t look good. Doc said ERCP indicated “severe PSC.” We thought we were looking at the beginnings of the disease, but it appears to be more advanced than all the doctors thought. I don’t really know what Josh thinks; he doesn’t say too much but it’s obvious he doesn’t feel well. I think he’s still shocked at the diagnosis. I struggle with feeling overwhelmed sometimes when I think about how our lives are going to change. Mostly I feel helpless because there’s nothing I can do to make Josh feel any better.

The idea that the diseased liver is slowly turning to stone inside his body is unfathomable. And I don’t think I’ve ever heard a scarier word than “transplant.” We will quite literally have to re-learn how to live our lives. And I say “we” because I’m in this with him, through whatever it takes. He’s going to be by my side at least until we’re 100. First thing I need to work on: being much, much pushier with the doctors. No more meek, head-nodding, quiet little sheep.