how exactly were you diagnosed ?

[Guest guest]

My son has been a confusing diagnostic puzzle - to say the least. He

has a lot of unusual complicated issues going on and has puzzled

some experts in some ways. He has amazed many in other ways by how

well he is doing-considering the severity of his problems.

A short-as-possible summary of his history in order to maybe get

some input here on where he is at with his PSC. He just started

seeing a new group of drs out of state and no feedback yet from them

(since 3/6) so I am getting a little concerned that I may not have

been able to convey to them everything they needed to get a big

picture of where he is at currently. I am struggling with not

knowing at what stage things are at with my son

1. he has short bowel syndrome- had 3/4 of his small bowel removed

as an infant including having his ileocecal valve removed and part

of his colon.

2. TPN for 5+ months caused liver damage but probably ?? mostly

recoverable damage ? his bili was 14-16 (maybe higher for a while)

for many months during and after TPN

3. very large retained common bile duct stone at 4-3/4 years old

4. chronic small bowel bacterial overgrowth issues due to no

ileocecal valve (valve between small bowel and colon that help

prevent back flow of colonic bacteria into small bowel and liver)

that can reak havock on the liver/ducts

5. recurring problems with abdominal pain/leg pain/nausea/vomiting

light stools that were present before 1st stone obstruction episode

6. no furhter investigating into liver issues except for US every 6

months

7. at 7 years old another very large stone obstructing CBD in

grossly dilated common bile duct with 2 very tight strictures.

Beading and tight strictures found on ERCP ballonn dialtion done.

8. 4 weeks later per MRCP another obstruction in CBD found ERCP done

found obstruction and another balloon dialtion biopsy done

9. biopsy found small duct PSC but ERCPs and MRCPs showed strictures

beading intrahepatic dialtions pointing towards large duct PSC

10. a series of 12 more (13 altogeter) ERCPs done and stents

replaced for a year to open up common bile duct

11. obstructions were found or suspected each time with US labs and

HIDA scans.

12. an roux-en-y would have been preferred treatment if he did not

already have short bowel syndrome and absolutely no spare small

intestines.

13. an external drain was proposed by an expert out of state who we

saw and very much respected.

14. consistantly thickened- mural thickening- of common bile duct

walls went from @ 1.5 mms initially to @ 3 mms now

15. many theories proposed as to why so many stones were formed and

a lot of testing done but the combination of 2 'huge' sphincterotomy

cuts, major antibiotic doses for extended periods of time - (xifaxan

and IV gentamicin taken orally) stents replaced for a year every few

weeks in order to keep CBD open slowed down obstructions.

16. recently another huge stone was seen but seems to have passed by

the time an MRCP was done.

17. portal hypertension and hepatosplenomegaly- sizes vary though

18. ascites seen but not signifigant

19. low fibrinogen (now borderline ok)

20. elevated PT now with vit K therapy- high normal

21. low haptoglobin

22. anemia issues

23. pancreatic insufficiency and fat malbsorption

24. failure to thrive now doing better with gtube and very elemental

formula

25. consistantly elevated ALT and AST for years- now stablized

26. confusing bili elevattion- but he also has Gilbert's (that on

it's own is benign) but just confuses the picture

27. dialetd CBD and common hepatic duct & signifigantly tapered CBD

per recent US recently but not other elevated LFTs except bili

28. enlarged spleen distorting and pancreas and tail of pancreas is

folded over on itself ??

29. thickened duct walls at perihilar juncture

30. were told that all of manipulationms to his ducts have made his

problems worse but no answers on where we go from here

31. starry night appearance on ultrasound

32. duct walls appear to be fibrosing

33. ankle clonus on last exam

34. fatigue has become a huge issue

35. troubling personality changes

36. decreased appetite bloating nausea

37. there's probaly more I can't even remeber right now...

38. short gut can cause stones and a lot of his symptoms too..so it

gets confusing sorting out what is what

All we have heard so far is that since his labs are ok - except for

his bili then he is stable and only labs every 6 months are needed.

I would LOVE LOVE LOVE to believe that everything is stable but my

gut is telling me it is not ?!?!