Siobhan - re: small duct psc

[Guest guest]

Dear Siobhan (is that Gaelic, btw, for Joan? And do the Celts

pronounce it the same way? I went to HS w/a girl who pronounced it

Sih-BONN...),

My husband Jim had small-duct PSC. It's hard/wrong making

generalities, but at Jim's 1996 PSC diagnosis, the doctor said Jim's PSC being

relegated to his small ducts meant it was unstentable... also, that his disease

might perhaps tend to be more chronic, progressing more slowly. In '96, at

age 42, Jim's doctor estimated roughly 7 years 'til needing transplant (which

ended up being off by about 2 years). Being that Jim's LFTs had been

elevated alllll the way back to his UC diagnosis (in 1884), despite having no

confirmation of PSC dating back to '84, the doctors nevertheless now believe

this to be true; so if you do the math on your fingers 'n' toes, for Jim, it turned

out to be 17 years 'til (living donor; our son) tx.

For Jim, his rapid successions of chills & infection (cholangitis

attacks) were initially treated with Rx Cipro. But the rapidity of occurrence

(i.e., monthly) is what finally prompted his local hepatologist to recommend

him for transplant evaluation. After 4 long months of testing (in more

ways than I ever thought possible... they even evaluated me & my ability to

be Jim's caregiver), he was eventually approved in the summer of '00. (Sidebar:

the hepatologist at that point thought Jim would be on the list for perhaps as

many as 5 more years!) This was " pre-MELD, " btw, where " the

system " utilized a " different " method of cadaveric organ allocation.

Although Jim's condition worsened progressively, we eventually came to realize:

a) that in our area (San Diego),

at that particular hospital, c) with Jim's type-A blood, & d) with so

many ahead of him on the list, his chances, sadly, were the proverbial

" slim to none. "

Toward the latter part of his pre-tx timeline, while Jim remained sedated

& in recovery from colonoscopy, his doctor & I had a blunt

heart-to-heart (re the doctor's many concerns). I threw down the gauntlet

(it's a good thing we're friends!) & challenged him to, ah, since he

reallyreally cared about Jim, to take his Hippocratic Oath seriously & give

us some brutally honest direction (since Jim was going to languish on the

cadaveric waitlist).

It was at that point the doctor advised me to get Jim heck away from

that hospital & aggressively pursue LIVING donor liver transplant,

elsewhere. As he knew Jim would have classic denial about that, we agreed

I'd bring Jim to his office later that afternoon, unsedated, for a heapin'

helpin' of verbal tough love (which did do the trick for Jim).

The doctor had just returned from some Colorado symposium & was blown away-impressed

by their living tx program, so recommended we go there, & CO wasted no time

making all arrangements. Our " Califonria " medical insurance put

the brakes on that, though, so Jim & I, not wanting to waste precious time

in any appeal, regrouped to select the best California had to offer, at the

time (in our humble opinion), for Jim's LDLTx... which we decided was USC in Los Angeles, CA.

Jim & our son were their 36th such adult-to-adult surgery (which far

exceeded other tx centers in CA back at the time).

The rest, as they say, is history.

Apologies for the length, but you did say " any comments. " One

thing's for sure, it's difficult making certain PSC comparisons -- there are so

many quirks, it's as if no 2 cases of PSC are exactly alike. But Jim

& I always found anecdotal input from this support group invaluable to " our "

process!

Maureen (wife of Jim: UC/elevated LFTs '84; PSC '96; LRLTx '01 (donor

son doing fine); post-tx incisional hernia repair 1/'03; multiple post-tx

issues with skin cancers & colon health; empty nesters & happy

grandparents to 2 + a bun in the oven, due to finish baking by the holiday

season!

small duct psc

I am intertested in any comments on small duct psc. Are there any

differences in symptoms etc? I get the chills nearly every day now and

regularly get in infections in my liver is other peoples experience?

Siobhan