Primary sclerosing cholangitis

My BFF has it and she is with specialists all at the same place so they can consult about her.  She has over 6 inflammatory and syndromes.  PSC is serious as it affects organs. She sees a GI, Endocrinologist, Cardiac, Lung etc.  She's not able to locate to a less expensive town as they wouldn't have all the specialists working in concert like they do now.

I don't remember knowing about Jeff needing or already having a transplant of any kind. You've probably discussed it before and I don't remember - my ST memory is awful and very frustrating.  I use to have a sharper memory but that is the least of my worries lately.

Are you back from your time away from home?

Sorry about Jeff's new autoimmune disease.  When we have one more are almost a guarantee.

T E Marie, I only mentioned it a about three weeks ago.  We found out about PSC Memorial Day weekend.  We found a hematologist  at Weill Cornell in NY so I guess we are going to move all is doctors there.  As far as a transplant, he doesn't need on as of yet, but we know eventually he will.  It's been a real blow to all of us.  But as I tell him, and myself, failure is not an option!  

Hope me all is well with you.

dianne

Dr. Shukla treated me in the hospital after my takedown while my surgeon was traveling; he was a really nice guy, explained everything to me very well and was very calming.  I had some complications and developed an abscess so my hospital stay was 11 days for my takedown. 

I have never seen Dr. Bosworth but I use another GI in his practice.  They have lots of resources at the Jill Roberts IBD Center at Weill Cornell and it very well run.  

Thanks for asking Dianne.  I'm okay but am waiting to schedule a pouchoscope with my GI at Mayo's as the balloon dilation performed in Feb. didn't work.  My GI is concerned  I might need a surgical dilation under anesthesia. Since I know what the problem is I'm not freaking out like I was in Feb.!  It is what it is  

Back to my friend.  She fired her prior doctors as they kept telling her all she needed to do was loose weight.  This is why 2nd opinions are so important.  The University of Chicago is where are of her specialists are.

PSC is a nasty diagnosis.  You are right and giving up is not an alternative!  I hope you are taking care of you too.

Take care,

Paulette

I have known that I have PSC for 18 years. I discovered it along with my UC. Weill Cornell in NY has an excellent reputation and I think you can not go wrong with anyone there for treatment or a transplant. 

Ok Beth spill!  I need to know everything you know!  I'm a wreck.  I spent the 

sat 24 hours crying my eyes out because I think my son is going to die.  How many attacks have you had?  And what denotes an attack?  Can I take him back to Robert Wood if he is having an attack or get him into NYC, which for me is never easy as I don't drive on the turnpike or bridges.  Long story, don't ask!  Why meds do you take.  Eighteen years gives me hope.  This kid is only 24 and has been suffering his entire life.  I'm not sure how much more any of us can take.  I think I have aged 20 years in the last three weeks.

thanks for the opinion about Cornell.  Good to hear.  I just don't trust anything anymore.  This is too overwhelming right now. I wish it would just go away!

thank you,

dianne

Paulette, I'm sorry you are not well.  Don't any of us ever get a break?  I'm not doing well at all.  I can't stop crying, I have had a headache for a week.  And chest pains too.  I'm exhausted but can't sleep.  I feel like my life is not my own anymore.  I'm just so happy I had the time in Florida before all this hit the fan.  I'm truly greatful for that.  And Jeff had a great time in Disney World.  We all did.  

Hey, take care, feel better and breath.  I should take my own advice!  I'm here if you need me.

dianne

First off,Chill !  PSC like any disease affects each of us differently. I've had it for 16 years. Been listed as a pre transplant candidate for over 10 years. Too many details to share here,but it does NOT affect all your organs. It is an inflammitory disease of the biliary ducts. Cholangistis for me is body aches in the bone from the waist down, 10 times worse than the flu, followed by jackhammer chills even in July. Then massive fever, sometimes to the point of passing out. When that happens,I know that I've got a dominant stricture that can only be resolved by ERCP dilation and stent therapy.Then it's to the ER.  PSC Partners for a cure is a good website for information.

All I know is about my friends experiences and she has overlapping auto-immune diseases and syndromes.  Her's was undetected for years and she is 60.  She is managing all of her problems now and has everything is currently stable.  Jeff is a young man and it's good that it was diagnosed.  I didn't mean to make you worry more.  It is a nasty disease but so is UC and I'm still here since my diagnosis around 18 years ago.  I had it a good decade before then. We are all different.  Mine crept up on me and there are other's that get the diagnosis and are hit so hard they need the surgery right away.  My j-pouch is 4.5 years old and I know what the problem is which is a lot better than in February when I was clueless.  I'm going to be okay but upset that my thyroid has gone bonkers too.  I'm finally under the care of an endocrinologist but need to stay on the last dose for 3 months until she can order more testing.  It's an autoimmune disease too.  I think most of us with IBD eventually get another autoimmune disease.  I forget where I heard that from so don't take it as gospel! 

Jeff is young and has you helping him.  Please take your own advice   I had a sick son for 15 years and he is doing terrific now. Caused me my first migraines, but that comes with the territory.  I'm glad you all had fun at the Magic Kingdom. I love that place! 

Thanks everyone for your support.  I definitely need to seriously chill out.  Paulette, you take care of yourself.  Trenchwrench, that you For the information.  Beth, that you for some good news!  KS1905, thank you for the information.  I have to have faith in the doctors and know that everyone there is working to help Jeff.  

Thank nk you everyone for all you help!

dianne

Tech, those sound like some rotten symptoms to go through.

I got listed last month after a cholangitis attack in March. Lots of fatigue, skin was a neat blend of yellow and grey. Luckily, a balloon dilation did the trick on that duct.

Dianne, one very good thing about PSC (maybe the only good thing!) is that it progresses very slowly. But then, stuff can pop up out  of nowhere at anytime, just like UC.

Jeff

Been 5 years since the last ERCP.  I began  itching ( pruitis )  6 months ago and  biliruben is 3 something, so I am going in this Thursday for an ERCP. Itching is part of liver disease,but I never had the itching problem until 6 months ago. Ironically, I was on cholestramine powder for the itching, but it was constipating me, and slowing down the bowel,and causing some gas. You know what it's like as a poucher, to leave the restroom,only to have a pocket of gas fill the pouch. Then have to go back in a second time.

  Prescription anti-histamines work better for me. I learned not to take hot showers before bed. 

Spoke to the transplant people today and they told me that Jess's numbers are all fantastic!  His bilirubin is down to .6, so I'm hopeful.  When he got sick, the only symptom was jaundice.  But I think that once you get jaundice, things are pretty bad.  Jeff never gets fevers, but come to think of a few months ago, he was have mild chills.  Never gave it a thought.  But, for now, I grateful that his numbers were so good!

finally a bit of good news.  Does anyone use any of the doctors at Weill Cornell?

Update

we went to Cornell today and met with Dr. Shulka(?) and Dr. Bosworth.  Both seemed very nice and knowledable.  It was so sad however watch them talk to Jeffrey with such sadness in their eyes.  Dr. Shukla is not sure if he can save the pouch.  It depends on if the fistulia is still there.  So Much is up in the air right now.  They are going to do their own testing hopefully sooner than later.  Jeffrey has so much going on with the PSC, fistulia, micro tear in the pouch.  It's overwhelming.  We have an apt with Dr. dietz in Cleveland in September, but I'm wondering if we should go.  At this point all he wants to do is open Jeff up and poke around.  That doesn't sit well with me.  And Dr. Brown wants everything done at Cornell.  Has anyone had a j-pouch repair done at Cornell?  While we are using Dr. Shukla, Dr. Milsome will be involved.  He doesn't take insurance anymore, but does get involved in complex cases.  Basically, he picks and chooses what he wants to do.  Also, Dr. Bosworth mentioned putting Jeff on Remacade. He is also concerned that because Jeff developed this fistulia problem after connection to the j-pouch, he just might actually have Crohn's, even though all the pathology done last year said no.  Both doctors would prefer Jeffrey stay on the ostomy, but are willing to give a repair a shot.  A redo is not possible because Jeffrey doesn't have the length to do it.  But, this is really Jeffrey's decision.  Ok, I'm totally overwhelmed.  This is so serious and been going to far too long.

Hi Dianne,

No advice, ideas or information to offer but tons of good wishes, prayers and hugs....all of my thoughts are with you and Jeff...All that I can say is that no matter what happens you need to take good care of yourself, eat right, sleep at night and hydrate so that you can be strong for Jeff...it will not help him if you fall apart...go for walks with him, plan meals that he likes that are pouch healthy for the both of you and stay strong and optimistic...

Don't let those big surgeons intimidate you, ask question, be Jeff's advocate and protect your son with all of your mother love and instincts.

You are a fantastic mother and I admire you greatly...he is lucky to have you in his corner during this battle.

Sharon

Milsom did a complicated repair on my pouch. If he is involved than I would stay at Cornel. You can't get any better As far as I'm concerned. 

Ally, thank you so much.  As we get closer to surgery, I'll make sure he is n the OR with Jeff.  

Im so scared about all of this.  It's so flippin complicated with two serious illnesses that affect each other. But one day at a time!

dianne

Sharon has given you the best advice  She couldn't have said it any better.  My prayers are with you and your son     Grace

Grace, thank you.  I'm calming down a little bit and found a therapist.  This is all way too much to handle for me.   I'm afraid I'm headed for another nervous breakdown. The last one was not pretty.  At least I have a good cocktail of drugs this time.  

thanks everyone.  Today was a good day!����

dianne

One day at a time Jeffsmom! When I found out I had some small ulcers in the pouch I almost had a breakdown too. Taking the meds wasn't nearly as bad as I thought it would be but it did bring back a ton of bad memories!

Cats, thank you.  But the big issue is the fistulia.  As of now no one knows if it is still there.  And no one where it is.  Unusual situation.  You can see the fistulia on a fistulia gram but they can't see exactly where it goes in the pouch.  Most of these will connect at a seem line which is the. It's likely place for a small tear.  Not so simple with Jeff.  they think that it is in the back of the pouch through the intestine, like a puncture wound.  Nothing about any of this has been easy.

How is Jeff taking this all, emotionally as well as the pouch problems and PSC?  It's hard for us mother's to pretend everything is going to work out well when we are not sure and are so worried and emotional ourselves. Sharon and everyone is right about taking care of yourself. But you knew that.  We all know it and then over extend ourselves anyway as we want to shield our children.  

T, thank you so much for checking in.  Everyone here and n the PSC form have been wonderful.  I can't thank you enough.  Jeff's getting very depressed.  He is bing eating again and has gain 30 lbs in the last 6 weeks.  I found a therapist for me, but I may have jeff go, but he is getting very stubborn and nasty again, so it's hard to talk to him.  I'm trying to keep him a little bit busy, but like I said, his mouth is getting out of control.   And when that happens, I look for places to hide.  I'm not doing well at all.  I'm truly a glass is half full type, but this is a real challenge.  I keep think who is going to die first, me or him.  As much as I say, I can't watch him die, I don't want him to die alone without me.  But he is not dying, yet, so I don't know why I'm perseveratimg on that.  I'm too Much of a realist!  I'm having difficulty learning how to navigate the NY doctors.  I'm used to just getting what I want, when I want it!  At RWJ, I knew the powers that be, so I just had to make a phone call, and it happens!  I spoke to the doctors directly, the secretaries, etc.  at Cornell, not so much!!  But I'll learn the landscape soon enough!  

And yes, I'm trying to take care of me.  I went back to the gym for the first time in six months this week, but my back is killing me now.  I've been to yoga a few times this week too, but my poor back.  So that makes it hard to me to keep active.  I'm going to Get a massage this afternoon, so hopefully that will help my back.  

I have to say, this has been the most difficult time of my life.  I've had more than my own personal challenges, but I'd take them back in a heartbeat to make this go away.  I'd have 6 more open heart surgeries if it would make Jeffrey better.  But unfortunately, nothing is going to make him better, not even a transplant.  The PSC seems to return and attack the new liver.  But I'm way ahead of myself and I need to focus on the here and now rather than the what if's.  Hard to do!  But for today, we are ok.  Jeff is tired often but getting out of the house, so I should say, all things are good!  All things are good, today is a good day!  now if the sun would come out, it would be perfect!

ok, I'm doing better!  Thanks for allowing me to vent a little bit!  Love to you, and hope you are well.

dianne

This is the perfect place for you to vent! Get it off your chest so you don't catch yourself catastrophizing in front of Jeffrey. Remember, there is just as much (if not more) chance that the PSC will not progress for years, so you may as well encourage your thoughts to go there. Hard to do, I know.

I think that once you guys are more settled in with the new doctors and can anticipate the next steps, you will be able to relax more and make the best of each day. As you say, live in the moment. We all live heartbeat to heartbeat, but having a serious medical challenge causes us to think and brood on it more. I can't really know what you are feeling, but I do empathize...

Jan

Definitely use the Weill Cornell Connect, it's the best way to reach the doctors.  It's all in writing, you can take time wording your questions the way that you want to and then you can read the responses.  All your son's medical records are there.  It's the only way that I communicate with my GI outside of office visits.  I usually have a reply within a few hours.

https://mychart.med.cornell.edu/mychart/default.asp

I remember when Hep-C meant that you would eventually need a liver transplant, now with recent medical breakthroughs there is a 95% cure rate.  PSC is a slow moving disease, it could be many decades before Jeff would need a transplant.