Hello everyone. Here is the lastest about Zac and specifically about his Mayo clinic visit. We are driving back from Mayo now so I hope all is clear in the memo it has been a bumpy ride. If you have any questions or need any clarification shot us an email or phone call and we can try to answer it..
12/16/2009 visit with Mayo doctor
Dr Harnios – Hepatology & Liver Transplantation – Mayo Clinic – woman in her late 40’s & VERY KNOWLEDGABLE.
She has been treating PSC for 15 years
There has been a growing understanding of the disease and improvements in the past 3 years. She thinks it will continue to progress.
Dr Harnios’s explanation of results from testing done by Zac’s primary and liver doctor in Lakeland
Kidney good
Liver function good – functioning normal at this point and time. (Which is the treatment goal of this disease.)
White cell count good
Auto immune inflammation test – negative
PSC is auto immune disorder but negative for Hepatitis.
Hepatitis A B and C – negative - he will get an immunization shot for Hep A &B soon.
Vitamin D is good
PSC (Primary Sclerosing Cholangitis) directly related tests:
Two types of cells in Liver
First time tested in Aug.
Type 1 cells (Phos.) – 6x’s elevated
Type 2 cells (AST & ALT) – 5x’s elevated
2nd time tested (2 months later)
Type 1 cell (Phos) – 5x’s elevated
Type 2 cell (AST & ALT) – 3x’s elevated
Doctor thought this improvement was from sugar being regulated and maybe the Urso Forte pill.
The ERCP test, completed by the Lakeland liver doctor confirmed PSC disease.
PSC the disease
Dr Harnios is currently treating 80-100 patients with PSC.
Rare disease.
Ratio is 2-1 men vs. women.
Age 17-45.
Not passed in to other children and usually not seen in siblings – no problem with having children (she mentioned this several times).
People with PSC liver bile ducts are constricted and can become blocked. Cells attack the liver bile ducts and in many cases the patient also develops inflammatory bowel disease. Zac recently had a blood test ruling out IBD but MD wants him to also get a colonoscopy.
Doctor explained that hep C attacks the entire liver which is unlike PSC – she explained PSC is like a plumbing problem
The ducts will block parts of the liver and one of the following can occur:
1. The liver will compensate and use other parts of the liver (Zac’s right side of his liver is being used more that the left right now)
2. Liver ducts will fix themselves and become unblocked – the pains zac was having in the stomach that have stopped is an example of this. (his body fixed its self)
3. Small ducts will remain blocked and Zac will develop systems such as stomach pain, fever, chills, jaundice, severe itching. The ducts may need to be ballooned or have stints put in (same as heart procedure)
4. Ducts that are going to ‘important areas’ or deep in the liver will become blocked and cannot be ballooned. This would require a liver transplant but doctor expressed that this would be a slow progression and we would know it was coming via visits and physical stressed. The doctor also kept mentioning that the need for a liver transplant may never occur.
There is no good treatment for the disease. The Urso Forte drug is like motor oil for the liver and an anti-inflammatory but the doctor does not personally think that the drug is very affective but he will continue on it for now.
Vitamins are not absorb well and many patients with PSC develop bone problems, such as osteoprosis. She suggested a bone density test on the next visit to Mayo.
Diabetes and PSC: Are they related? / How do they effect each other? - When the liver is inflamed the diabetes will get worse and if he ever needs a transplant the drugs that he would take after the transplant would affect the diabetes by possibly making it a little harder to manage.
She suggests that Zac go to a diabetes doctor (endocrinologist) – Zac has already been schedule to go to this specialist on Monday by his primary doctor.
What if questions about liver transplant
1. Q Is PSC cured after transplant? A The disease can come back but does not in the majority of PSC patients.
2. Q How long will liver transplant last A. She did not provide an exact time frame but explained 5-10 years statics are based on Hep C patients and PSC patients do better. There are too many variables and all patients are different, there isn’t an exact answer on how long it would last in him.
3. Liver transplant process procedure – in hospital 5-7 days, stay local few more weeks, 3 months of weekly lab work, every 4 month visits then after a year- once a year visits.
What’s next
1. Get a colonoscopy in Lakeland
2. See endocrinologist (diabetes specialist)
3. Hep AB shot
4. Take Vitamin D and Calcium
5. 6 month visits to Dr Harnios which will include testing to monitor the progress.

Thanks for all the support. We love you all. And Happy Holidays. I know ours will now be filled with a little less question & burden and more peace.