PSCwife or those with PSC

Hello PSCwife...
I am so very interested in what you and your DH have been though, as you seem to be further along the path than I...right now, the path is murky and obscure, but talking about it helps...

Just some background, kinda like your husband, I too have crohn's(and that's the forum I most often visit), which has hit me pretty hard, but then I noted that I was having pain in abdomen/thoracic area...the docs also noted that my liver was enlarged on CT (which I had done cuz the crohn's was flaring pretty bad) and that my LFT's were a bit abnormal (though they fluctuated, at times they were normal, then they would be high)...so we did the ultrasound and CT and MRI thinking stones, but none were observed...My GI Dr is married to a hepatologist, not to mention he treats enough patients with IBD so he was on the lookout for PSC...so he sent to me to a specialist that does the "enhanced" ERCP, in which came the definitive dx and while there they did a stitctureplasty and if I'm not mistaken a stent.(my common bile duct did not want to open).

I know that information helps me be a lot less scared (and frankly, the prospect of a transplant really scares me)...but I guess I am eager to know the path ahead for both me, and I think it would also help my husband to know what is coming (because I have already noted some issues with mental clarity, but I do not know if it is from being sick with crohn's so long or part the liver issues)

So if you don't mind, I would love to keep an open dialogue going...I might encourage my DH to log on too, because I really feel for him having to care for me(I wish I could somehow ease his pain...I wish I wasn't the bringer/bearer of it, I don't know how to comfort him)
Thanks.

Thanks for replying...I've had crohn's for over 10+ years...it's mostly perianal..but I have stomach issues too (ulcerations cause by the disease)...I've been in a pretty bad "crohn's flare" for about 3 years now, with times being worse than others, and I've had 2 surgeries (again, perianal)...I'm lucky that I haven't had to have any of my intestines removed yet...I've been on Remicade, Humira and now on Cimzia...it was actually when I was coming OFF the steroids when my liver started to cause problems (I think the steroids were helping to keep my liver in check)

I have the the inflammatory arthritis that comes with it(which can be at times more debilitating than the crohn's)...but like there are issues that I don't know if it is the crohn's or the liver or what...

Like the brain fog...I'm usually a pretty intelligent person with a good memory, but in the last year or so, the brain fog has been horrid...I can't remember a lot of things...and at times have word loss, it is sooooooo aggravating...and really I don't know what is must be like for my husband (ie, has my behaviour changed and I not know it?)

The other thing I wonder, is that I can "feel" my liver (cuz it hurts)...and I know there is transitory effects, like on certain days, I look kinda "healthy" (aka, tan...but my skin has a yellowish tone anyways, when I get sun, I don't get dark, I get "gold"), but is usually when I feel worse, and my liver feels worse...alas, my LFT's don't always show it...so I was wondering if this happened with your DH too...

And thanks for just talking to me...I don't know all that many with this disease (cuz yeah it's kinda rare)...but seeing the positive side...seeing somebody who went though transplant...it makes me feel better...

 

Undone,

 

Haven’t seen you around lately.  Hope all is well.  I wanted to address some of your questions more specifically . . .

 

Brain fog (hepatic encephalopathy) . . . yes, your behavior can change and you likely won’t be aware of it.  I’m certain your Husband understands and probably doesn’t even comment on it unless it becomes problematic.  This is when medical treatment must be stepped up.  Most of the behavioral changes are harmless and expected.  Your liver is not functioning properly and you will always have some level toxins in the bloodstream.  When Harvey would get frustrated with it, I would reassure him that I was okay with it.  It’s perfectly normal given his condition.  I would try to get him not to stress about it and help him find some humor in it.  I know it’s hard to accept, but it is simply beyond your control.  Try not to dwell on it.  Stress will only complicate things. 

 

Feeling your liver . . . right quadrant pain is normal as well.  PSC is believed to be an autoimmune disease which means liver is under constant attack by your own body.  It suffers injury and it hurts.  This will come and go throughout the process.

 

Harvey always had a yellow or golden tone.  This is jaundice caused by the buildup of bilirubin in the bloodstream.  The liver is responsible for filtering bilirubin from the bloodstream.  When the liver is diseased, it cannot do this effectively.  Some days are better than others.  Sun exposure helps so try to get in the sun at least 30 minutes a day. 

 

It sounds like your Crohn’s has kept you painfully distracted.  There is no cure for PSC and you will ultimately require a liver transplant.  It’s great that you are researching your condition and want to know what lies ahead.  However your primary caregiver, whether it is your Husband or someone else, needs to become educated on the ESLD (end-stage liver disease) symptoms and treatments in order to help you manage through the process.  ESLD can be very traumatic on the patient and caregiver. 

 

Penny

 

 

Undone,

 

Just take it one day at a time.  Some days will be better than others.  It’s great that you are ensuring the docs keep up with the blood work.  You have more than one issue and it’s important to know where you stand.  Always ask for a copy of your blood test results.  It’s important to have these when you and your husband are doing research on your condition.  You should also record your vitals and weight everyday.  Harvey now does this himself three times a day.  If he can do it, anyone can.

 

I had always allowed Harvey to be the King of his Castle, but when he got sick he wasn’t able to anymore.  I told him that I was going to have to be the “Boss” until he got through this.  He would just smile.  Since his transplant he has resumed the role.  He really stepped up his game because now he is a master at delegating!  Ha!

 

The best thing that you can do for you husband is to try to relax.  Stress is not good for you.  Besides, it’s so much easier to help you when you cooperate.  You may catch yourself doing something stupid . . . don’t get angry . . . try to laugh at yourself.  Humor is good medicine.

 

You didn’t tick off the powers that be.  Life is all about trials.  It’s how we navigate through these trials that sculpt who we are.  I think the thing that got me through was the knowledge that, “God won’t put more on you than you can handle” and “That which doesn’t kill you makes you stronger.”  We find our strength in love and faith.

 

As far as you fear of a transplant goes . . . we knew it was the only thing that would save him.  We viewed it as a goal and a blessing.  Knowing that someone had to loose a loved one in order that yours may survive is very powerful.  One family’s heartbreak is another family’s salvation.  It is a gift of life born of death.  We will never take it for granite.  Every day we have together is a day we wouldn’t have had if it weren’t for someone very special. 

 

Shand’s hospital suggested that we find a live donor for Harvey.  His sons stepped right up but it was never a consideration for Harvey.  He wasn’t about to put one of his sons at risk.  He would rather die.  They each had their own families to care for.  I wasn’t a match or would have gladly donated half of mine.

 

This ordeal has brought us so much closer.  If he ever had a doubt about how much I love him, he doesn’t anymore.  He now knows that there is nothing I wouldn’t do for him.

 

Well, that’s all for now.  Take care.  I’ll be thinking of you.

 

Penny

 

Undone,

 

Well, that explains the brain fog.  Are they prescribing Lactulose?  This will help with the immediate issues.  Ask about Xifaxin.  This will help prevent recurrences.  However, the Xifaxin is very expense.

 

Again, I’m glad you asked them to run these tests.  The results are very important in monitoring for symptoms.  It’s best to address these early on.  The doctors are extremely busy and often stick to their own specialties.  It never hurts to ask and like in this case, you caught the ammonia creeping up.  You have to be your own advocate.  Great job!

 

Don't stress about the past.  You've gotta a lot of life ahead of you and I'm sure you'll make the most of it.

 

Penny

 

 

Undone,

 

I’m glad you are investigating your condition and I’m happy to help.  When Harvey was hospitalized in 1994 with a severe bout of Crohn’s and then recovered, I was okay with it.  I figured it was a manageable disease and we would have to deal with it.  But when he was subsequently diagnosed with Primary Sclerosing Cholangitis. . . I thought, what the hell is that?!  Harvey has a brilliant GI doctor, but his bedside manner stinks.  He was very vague about the condition and didn’t discuss the course of the disease.  Up until then, Harvey would get an occasional bout of Kidney stones.  But otherwise, he was extremely healthy.  Now he has Crohn’s and PSC.  I was clueless.

 

The Internet had gone public but it wasn’t anything like it is today.  I didn’t even have access to it at the time.  I went to the library several different times trying to find information on the condition.  It was like trying to find a needle in a haystack.  I finally found one (1) small paragraph on the subject of PSC in an old medical reference book.  Simply stated this was a rare liver disease.  There was no known cause, no known cure, and that on average the patient would expire within 5 years of diagnosis due to liver failure.   I think my life flashed before me and I put the book back on the rack and walked immediately out of the library disheartened and in a daze.  Until recently, I didn’t share this experience with Harvey.  I look back on that experience and remember clearly how I felt then. 

 

Well, I have to get ready for work.  I’ll tell you later what drove me to become an informed patient advocate for Harvey. 

 

Penny