Can you be symptomatic when your labs are good? My daughter is six, but this morning she claimed total exhaustion and was slightly itchy. Her liver enzymes are normal right now, and her bilirubin is only very slightly elevated. Since I am not in her skin, I don’t know how likely it is I am dealing with normal variations in behavior or her feeling tired because she is sick. She is usually fairly energetic, though. Since her liver labs have been so good I have assumed she is going to be mostly asymptomatic, but I would like to hear someone else’s perspective.
First of all, I’m sorry to hear about your daughter’s diagnosis and issues.
This is the exact same thing that is happening to me, and I asked about it here (http://forum.livingwithpsc.org/forum/topics/questions-on-itching-and-how-to-help-my-daughter, bottom posts). My bili is at normal levels, and my other test have dropped to near normal and MRCP shows that my large ducts have been in great shape since being dilated from last ERCP, yet I’ve never had such damaged skin from the constant itch. My doctors tend to come up with the same information, which is that the small ducts are still damaged and leaking those bile salts.
Also, since your daughter is pediatric, you should look into vancomycin therapy. It seems to work wonders on children.
Thanks, Nomad! I am not going to just assume she will not have symptoms right now. 
Her doc is willing to prescribe Vancomycin if it seems like what we are doing isn’t working. But for now, with her excellent labs, we are just going to continue with the Ursodiol (and azathioprine, and Flagyl for other concerns). I guess if she starts itching intensely or constantly being exhausted we will reevaluate.
Did she eat a lot of sugar the day before? That can leave me very tired. Can you trace the fatigue to any particular food?
Hi hillary,
I am so sorry to hear about your daughter. You must be sick with worry and frantic.
Regarding itchiness-
I am on cholostyromine for the itching and the high levels of bile salts. My specialist and my blood work conclude that my high or low (er) bile salts don’t relate to itching. For example I was uncontrollably itchy, in tears and exhausted but my bile salts were actually lower that day, then when I was tested a month prior month. The month prior I felt great and was surprised my levels were double than when I was itchy.
I would ask your dr. About cholostyromine and if he thinks it is appropriate for children. It helped my itching 100 percent.
I was on urso but my bile salts shot upward and my hair fell out after a few weeks of being on it. It was decided that as long as my liver function was good I didn’t have to be on it as the side effects where to great in my case.
I also had improvement with labs when I switched my diet to probiotics, and a gluten free, grain free lifestyle. If you are interested research the specific carbohydrate diet and auto immune paleo diet. I am currently doing SCD with some AIP principals. It is a lot of work but many of my symptoms have settled down.(bleeding, nerve pain, tingling sensation, quadrant pain, fatigue and so on.) My blood work is currently reading perfectly with the exception of my bile salts.
I think anything is worth a shot and although it is a lot more work I am feeling so much better. You daughter has a complicated case it may be worth reading about how these diets and how they have helped others. I found it very reassuring as far as colitis, ibs and chrones. It made me mentally feel better to knowing I wasn’t helpless and that I could play an active role in getting better. The plus side is it is incredibly healthy lifestyle. Bone broth, nothing processed etc. so if nothing else I am maximizing my nutritional intake.
Those are just my thoughts and suggestions based on what I was researching gut health and so on. My specialist said she was fine with diet and that it couldn’t negatively effect me.
Good luck and let me know if you need any more resources.
Dianne
Hi Hillary. I was diagnosed with PSC in 2006, and except for a few serious episodes where I was hospitalized, and one or two points where I felt really crummy but not hospitalized, my blood work is usually normal. But, even when my numbers are normal, I experience itchiness for weeks at a time, and generalized fatigue 90% of the time.
I have a friend who has Hep C, and had a transplant about 10 years ago. He made it a point to learn everything he could about liver disease, and he's fond of saying that the liver has over 400 different functions in the body. When my Dr runs a blood workup, he tests about 8-10 different values. What I learned is that this is just part of the disease, and a normal blood workup doesn't mean the disease is quiet. It just means that particular key, very serious things aren't wrong at that moment -- bile ducts aren't blocked, there's no infection, and so on.
Like others have said, it must be hard to be caring for a daughter that age. I hope you have good Dr care, someone who can explain stuff clearly and takes the time to answer all your questions, and that you have good emotional support around you.
Liver is a big organ with lots of reserve, just like kidneys. You can live with only one kidney without any sign of renal insufficiency. That’s one big reason why liver function test is not very sensitive. GGT and ALP are more sensitive, but not very specific, meaning abnormal results do not necessarily indicate a liver problem. In many cases, you may not see any correlation between blood work results and symptoms. As to diet and symptoms, my personal experience is that too much fat definitely makes me feel worse, if I do have any symptoms at the time.I don’t see much correlation between any other food and symptoms. Sometimes you may feel one food worked wonder on you. But pretty much next time it won’t do anything.
Thanks, everyone! Lucia seems back to her normal fairly energetic self again. I am still keeping an eye on the itching. It is so valuable to get a perspective on what the liberty labs can tell us and what they may not! I think we are on good care (pediatric hepatologist) but I don’t always know what questions to ask at her appointments. At this point I don’t see a food connection, but I will keep an eye open.
Thanks, everyone!