anyone else also have Gilbert's ?

[Guest guest]

My son has Gilbert's disease - genetic inability to conjugate

bilirubin which in and of itself is not a problems but it has confused

his diagnostic course- he also has short bowel syndrome so a lot of

problems were initially blamed on the short gut. The short gut and

severe and ongoing small bowel bacterial overgrowth problems are very

likely what has caused his PSC.

His bilirubin elevations have been unconjugated even when he has had

total obstructions in his common bile duct. He has had 12 ERCPs for

stones and sludge obstructing his CBD, had stents placed often for a

year had several huge endoscopic sphincterotomies, has very thick

common bile duct walls, his intrahepatic ducts are sclerosing now not

dilated anymore, his liver is shrinking and is on urso (so that is

probably keeping his other LFTs in check...except for his bili)

His bili has only gone up to 4.0 and sometimes is only slightly

elevated at @ 1.8-2.0 or so but has been consistantly elevated for years.

If you can't conjugate bilirubin does that mean your unconjugated bili

is equivalent to what the direct part of the total bili is for other

people ??? Since the direct (conj) bili is normally only @ 10% of the

total does that mean that for a person with Gilbert's that the direct

bili would be the same as the indirect without it ???

example of what I mean- If the bili is 3.5 then the total would be 35

if the 3.5 was 10 % of the total right ??

So does that mean if you have Gilbert's and PSC the indirect bili

means more than what some drs think ???

[Guest guest]

The total bilirubin is the sum of direct (conjugated) and indirect

(unconjugated) bilirubin. Normally the direct bilirubin is 0 to 0.3

mg/dl, and the total bilirubin is 0.3 to 1.9 mg/dl.

http://www.nlm.nih.gov/medlineplus/ency/article/003479.htm

This means that the indirect (unconjugated) bilirubin would be the

total minus direct.

So, in your son's case if the total bilirubin is 4.0, and if he has

no direct bilirubin (because of Gilbert's and inability to conjugate

it), then his indirect bilirubin would also be 4.0.

Best regards,

Dave

(father of (22); PSC 07/03; UC 08/03)

>

> My son has Gilbert's disease - genetic inability to conjugate

> bilirubin which in and of itself is not a problems but it has

confused

> his diagnostic course- he also has short bowel syndrome so a lot of

> problems were initially blamed on the short gut. The short gut and

> severe and ongoing small bowel bacterial overgrowth problems are

very

> likely what has caused his PSC.

> His bilirubin elevations have been unconjugated even when he has had

> total obstructions in his common bile duct. He has had 12 ERCPs for

> stones and sludge obstructing his CBD, had stents placed often for a

> year had several huge endoscopic sphincterotomies, has very thick

> common bile duct walls, his intrahepatic ducts are sclerosing now

not

> dilated anymore, his liver is shrinking and is on urso (so that is

> probably keeping his other LFTs in check...except for his bili)

>

> His bili has only gone up to 4.0 and sometimes is only slightly

> elevated at @ 1.8-2.0 or so but has been consistantly elevated for

years.

> If you can't conjugate bilirubin does that mean your unconjugated

bili

> is equivalent to what the direct part of the total bili is for other

> people ??? Since the direct (conj) bili is normally only @ 10% of

the

> total does that mean that for a person with Gilbert's that the

direct

> bili would be the same as the indirect without it ???

> example of what I mean- If the bili is 3.5 then the total would be

35

> if the 3.5 was 10 % of the total right ??

> So does that mean if you have Gilbert's and PSC the indirect bili

> means more than what some drs think ???

>

[Guest guest]

God bless you for understanding my rambling question !

>

> The total bilirubin is the sum of direct (conjugated) and indirect

> (unconjugated) bilirubin. Normally the direct bilirubin is 0 to 0.3

> mg/dl, and the total bilirubin is 0.3 to 1.9 mg/dl.

>

> http://www.nlm.nih.gov/medlineplus/ency/article/003479.htm

>

> This means that the indirect (unconjugated) bilirubin would be the

> total minus direct.

>

> So, in your son's case if the total bilirubin is 4.0, and if he has

> no direct bilirubin (because of Gilbert's and inability to conjugate

> it), then his indirect bilirubin would also be 4.0.

>

> Best regards,

>

> Dave

> (father of (22); PSC 07/03; UC 08/03)

>

>

[Guest guest]

> >

> > The total bilirubin is the sum of direct (conjugated) and indirect

> > (unconjugated) bilirubin. Normally the direct bilirubin is 0 to 0.3

> > mg/dl, and the total bilirubin is 0.3 to 1.9 mg/dl.

> >

> > http://www.nlm.nih.gov/medlineplus/ency/article/003479.htm

> >

> > This means that the indirect (unconjugated) bilirubin would be the

> > total minus direct.

> >

> > So, in your son's case if the total bilirubin is 4.0, and if he has

> > no direct bilirubin (because of Gilbert's and inability to conjugate

> > it), then his indirect bilirubin would also be 4.0.

> > Best regards,

> >

> > Dave

> > (father of (22); PSC 07/03; UC 08/03)

Wait maybe I misunderstood what you were saying did you mean that if

you can't conjugate bilirubin then a bili of 4.0 would be the same as

someone else without Gilbert's having a direct bili of 4.0 (as part of

their total bili) ???

His bili has never been terribly high even with total obstruction in

his common bile duct- shown first w/ ultrasounds then conformed w/HIDA

scans and finally all his ERCPs that show obstructions. He has also

had the really dark cola colored urine that is 'always' a sign of

conjugated bilirubin...but if you can't conjugate bilirubin ???

> >

> >

>

[Guest guest]

>

> Hi, my son has Gilberts

You have totally managed to confuse and worry me.

When my son has a high SBr but the direct is normal [.50mg/dl and

below] the docs are happy and say its his Gilberts. When the SBr is

high and the direct is above .50mg/dl then the say its the PSC. Back in

February his SBr was 5.95mg/dl and direct was 2.46. All the other LFT's

were high too. An U/S showed constriction of the CBD and so he had an

ERCP etc etc. LAst week his SBR total was 4, but the direct was .48,

other LFTS normal{1st time in a LONG time]...he was yellow and

tired.....the doc said it was his Gilberts.

The congugated and uncongugated bit have confused me.

Sorry can't help anymore than tell you what I know at the level I

understand.

Dan UC/PSC - left for Uni 2 weeks ago and doing ok [phew].

[Guest guest]

Because I now have to understand this more I have been googling.

http://en.wikipedia.org/wiki/Gilbert%27s_syndrome

here is a good explanation of Gilberts.

I believe in most people with Gilbert there is an elevated level of

unconjugated bilirubin. So some bilirubin is conjugated. And there are

episodes when the elevation is higher than other times. Definetly seen

that in my son.

It is important that the whole liver picture is examined not just the

bilirubins because LFTs should not be effected by Gilberts.

What is new to me in this web link is the inability to metabolise

Paracetamol. I give my son paracetamol rather than ibrufen as I was

lead to believe Ibrufen was not recommended for people with PSC. Now it

looks like the paracetamol is not good for people with Gilberts. So

what does that leave me?

karen

[Guest guest]

>

> Because I now have to understand this more I have been googling.

> http://en.wikipedia.org/wiki/Gilbert%27s_syndrome

> here is a good explanation of Gilberts.

> I believe in most people with Gilbert there is an elevated level of

> unconjugated bilirubin. So some bilirubin is conjugated. And there are

> episodes when the elevation is higher than other times. Definetly seen

> that in my son.

> It is important that the whole liver picture is examined not just the

> bilirubins because LFTs should not be effected by Gilberts.

> What is new to me in this web link is the inability to metabolise

> Paracetamol. I give my son paracetamol rather than ibrufen as I was

> lead to believe Ibrufen was not recommended for people with PSC. Now it

> looks like the paracetamol is not good for people with Gilberts. So

> what does that leave me?

> karen

>

hi -

I'm sorry if I am causing you any undue worry. I had to look up what

paracetamol is (are you in Europe ? I think that's a generic name in

Europe ??..) to find out it is - acetominophen- or Tylenol brand name

I have also been told that in general ibuprofin (motrin etc) is not

good for people with liver disease and that Tylenol was a better

choice....my son gets fever often so we give him tylenol fairly often

??? Yikes better find out I guess.

Braden's LFTs were elevated for years especially when he was having so

many problems with his CBD and obstructions had so many stents and

ERCPS...but his direct (conj) bili has never been out of range and he

has had the cola colored urine proceding obstructions....There is

also a theory that his chronic infections (small bowel bacterial

overgrowth because of short gut no ileocecal valve) have deconjugated

his bili...but that is assuming he does have conjugated bili to

deconjugate AHHHHH so confusing ????