About ten days ago we noticed a yellowish cast to our daughters face and whites of eyes. She also has been scratching for four months to which her pediatrician attributed to dry skin. Needless to say one thing led to another and we ended up at University Hospital in Cleveland, Ohio. We are so devastated at this news but find this site so hopeful that she will be able to get help and that this is a long process and not immediate. Im not very educated about PSC but am reading all I can. Many different opinions on her situation has upset me. Her bilirubin at admission was high and continued to climb after the procedure ERCP. She was discharged for home and her bilirubin was 11.8. This concerns me. Why is it this high? Does this mean she is more advanced in her disease? This has all transpired in less than a two week time. She had been itching for four months before the jaundice. Now it has increased as well as loss of weight, yellowish skin and eyes. We are scheduled to see her hepatologist in a couple weeks. I have started her on a low fat diet with only natural foods and juices as well as the Ursodol. I am asking for any advice. My daughter has limited speech so we have no idea how long she may have been experiencing symptoms. This is a lot to take in in one week.
Thanks for any hopeful information.
Mollie
Mollie,
I’m so sorry that your dear daughter is having to go through this now on top of her other special needs. Please know there is hope and help. One of the key things now is to make sure you have her under the care of a hepatologist tied with a transplant hospital. I’m glad to read you are to see her hepatologist. Stay with them all the way through the course of this disease. Don’t even think of going to your local GI for any of her care. This is a very specialized disease that needs a lot of tender loving care and special intervention from time to time. We are here to answer any questions you might have along the way. Education is key. All of your family that are involved on a regular basis with her need to be educated about PSC. I know it’s rather scary at first, but you’ve got to know there is hope amidst all this. There are many of us that have gone through what she is going through so you have some good advice to draw from here. The high bilirubin is very concerning as well. I assume you have a copy of all her lab work. Why don’t you run her MELD score and let me know what you find. Here’s the online calculator. Not knowing if your daughter is a child or adult I’m going to paste in both links. We look forward to hearing back from you soon.
Mark
PSC 2011 / Liver Transplant 2015
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Adult (12 years and older) MELD Calculator - https://optn.transplant.hrsa.gov/resources/allocation-calculators/meld-calculator/
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Pediatric MELD Calculator (Below 12 years old) - https://optn.transplant.hrsa.gov/resources/allocation-calculators/peld-calculator/
Hi Mark,
My daughter is 20. And her MELD score is 14.
Mollie
Hi Mark,
My daughter is 20. And her MELD score is 14.
Mollie
Mollie,
Now each transplant region of the country is different, but I was listed for transplant at a MELD of 12. I would encourage you to get her in to a transplant hepatologist as soon as possible, ask them to present her to the transplant team for approval to have her evaluated and listed. Now, everyone’s situation is different, but from what you describe she has probably had PSC for quite some time and not known it which is the case with most of us. I’d be getting her labs done at least every 3 months and if her MELD continues to climb they will do labs more often. I’m not a medical professional but I can tell you my situation. I was listed for transplant with MELD 12 in January, 2015. In April, 2015 my MELD had gone to 19, and then in the middle of July I was at a MELD 36 and transplanted two days later. Some PSC patients experience elevated MELDS and then they settle back down, so we are all very different in that regards. Nevertheless, I would flat out request that she be evaluated for transplant and get on the list at least. That way, IF and when things turn for the worse she will be in a position to get bumped on up to the top of the list and get that liver when she needs it.
I know I’m speaking frank and direct, and I pray that her condition is not as bad as it sounds, but it is important that you stay on top of this, be her advocate being at every appointment, asking questions and very kindly yet firmly if need be request that she be put on the list. I wish you all well and know that we are here with you and there is hope amidst all these troubling things that she’s going through presently.
Mark
PSC 2011 / Liver Transplant 2015
Thank you for your response. In fact, these lab scores were from her first hospitalization 3 weeks ago, so this last week would have different scores so I will keep all this in mind as we go to the hepatologist on the 27th.
My biggest concern is that she has Down Syndrome and would this disqualify her but they told me no. She will be eligible. All of this so overwhelming. Keep asking myself how I could have missed this…sad…and worried…
Mollie
Mollie,
There is nothing you could have done to prevent this from occuring. Often none of us know anythings wrong until the signs of weight loss and intense itching begin. She will be ok, just stay the course, educate yourself and your family all you can. I pray the medical team that will be working with her will be blessed by her happy spirit and smiling face as I’m sure she has. I’ve met a number of Down Syndrome children and they are all so very precious and loving. Keep in touch.
Mark
fcmmark Moderator
September 15
Mollie,
There is nothing you could have done to prevent this from occuring. Often none of us know anythings wrong until the signs of weight loss and intense itching begin. She will be ok, just stay the course, educate yourself and your family all you can. I pray the medical team that will be working with her will be blessed by her happy spirit and smiling face as I’m sure she has. I’ve met a number of Down Syndrome children and they are all so very precious and loving. Keep in touch.Mark
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In Reply To
Mollie
September 15
Thank you for your response. In fact, these lab scores were from her first hospitalization 3 weeks ago, so this last week would have different scores so I will keep all this in mind as we go to the hepatologist on the 27th. My biggest concern is that she has Down Syndrome and would this disqualify …
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Awe…What a lovely young lady. You tell her Mr. Mark is praying for her and that she is going to get better and live a long and healthy life! When she feels good, just let her run and play and be the young lady she is without having to think about PSC. It will get her attention when she needs medical intervention due to more strictures in the ducts. One suggestion, please ask the doctor who does her ERCP’s, if at all possible, do the balloon dilation and avoid using stents unless absolutely critical. There are so many side effects and high-risks for infections associated with stents. She doesn’t need sepsis or other infections that arise with the use of stents. I was blessed to never have to need them although I did beg for them a few times, my doctor was the wise one. Here’s a photo of my wife and I this one taken in July, 2 years now I’m post transplant. Thanks for sharing her photo.
Mark
Mark,
You are a blessing. God is using you to touch lives. Mine especially. A great picture of you and your wife…Alana had the balloon and no stent. I will keep all this in mind…thanks!
My husband and I are a Pastor and wife. Our biological daughter Hannah, born with Downs, died from a heart defect at four years of age…were told we wouldn’t have more children so we then were given an opportunity to adopt Alana, from Seattle Washington . She was ten days old. Part Eskimo Indian. She is the sweetest girl I know. And the last person who deserves this…I’m just torn in two with this PSC. Your encouragement means the world. I know the Lord has all the answers. But the fear and “what ifs” plague me. So thank you for all the support! I know God is in control. But I’m human I guess…
Thank you again!
Mollie
fcmmark Moderator
September 16
Awe…What a lovely young lady. You tell her Mr. Mark is praying for her and that she is going to get better and live a long and healthy life! When she feels good, just let her run and play and be the young lady she is without having to think about PSC. It will get her attention when she needs medical intervention due to more strictures in the ducts. One suggestion, please ask the doctor who does her ERCP’s, if at all possible, do the balloon dilation and avoid using stents unless absolutely critical. There are so many side effects and high-risks for infections associated with stents. She doesn’t need sepsis or other infections that arise with the use of stents. I was blessed to never have to need them although I did beg for them a few times, my doctor was the wise one. Here’s a photo of my wife and I this one taken in July, 2 years now I’m post transplant. Thanks for sharing her photo.Mark
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fcmmark Moderator
September 16
Awe…What a lovely young lady. You tell her Mr. Mark is praying for her and that she is going to get better and live a long and healthy life! When she feels good, just let her run and play and be the young lady she is without having to think about PSC. It will get her attention when she needs medical intervention due to more strictures in the ducts. One suggestion, please ask the doctor who does her ERCP’s, if at all possible, do the balloon dilation and avoid using stents unless absolutely critical. There are so many side effects and high-risks for infections associated with stents. She doesn’t need sepsis or other infections that arise with the use of stents. I was blessed to never have to need them although I did beg for them a few times, my doctor was the wise one. Here’s a photo of my wife and I this one taken in July, 2 years now I’m post transplant. Thanks for sharing her photo.Mark
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I just saw these two other photos. A very happy young lady. Is the sharp looking young man your husband? I love that hat and dress. Looks like she is dressed for a celebration of sorts. Take care, and do keep in touch.
Mark
Mollie, about the only good thing about psc is that it generally progresses pretty slowly. Mark’s advise is right-get her to a hepatologist-GI docs do not have the liver as their specialty.
The more info you know about psc the better, as knowledge is power, but it can be overwhelming at first. Been there, done that. Know that this disease is a roller coaster ride.
A patient can be asymptomatic for years, and then a symptom arises out of the blue. With more knowledge, and getting used to psc, what it is, what it can do, what can be done, will go a long way to reducing your fears.
With your daughter having limited speech, you will need to be hyper-alert to her. Communication will be critical between her, you and your husband.
Bilirubin is one of the best indicators that something is happening to a person, but I don’t think it indicates how far the disease has progressed. When my bilirubin got to a 4 or 5, that got my doc’s attention.
I hope the best for your daughter.
Jeff
fcmmark Moderator
September 16
I just saw these two other photos. A very happy young lady. Is the sharp looking young man your husband? I love that hat and dress. Looks like she is dressed for a celebration of sorts. Take care, and do keep in touch.Mark
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Thank you Jeff. I’m on this every hour of the day. Just so uptight and the overwhelming feelings of fear are the worst. But her well being motivates me. Yes. Being mostly non verbal is challenging. I’m sure she has had this disease much longer than even I suspect. This alone is enough to drive me crazy. I have this GI appointment Monday which is really if no big importance. The first hospitalization set it up when they had no answers from their testing. So we will keep it but knowing this is not a hepatologist will limit us. The 27th is the important appointment. Thanks for all your insight and encouragement. You’re all so courageous. I’m so amazed. Thank you God for this site!
Mollie
Hi there. I was wondering if anyone knows if there’s a correlation with the drug methotrexate and PSC.
…my daughter diagnosed 10 days ago, was on methotrexate for two years and discontinued it four months before this diagnosis as she has arthritis.
Mollie
Mollie,
I did find an article dealing with Methotrexate in the treatment of PSC. It is a 1991 so I wouldn’t count too much on it. It was never mentioned when I had PSC and I was diagnosed in 2011. Might take a bit more probing online to find further information. I’d also encourage you to ask her hepatologist at your appointment. Hope this helps.
Mark
Hi Mark
Alana continues to be yellow despite feeling good and having no nausea or other symptoms. It’s difficult to see every day. I wish I had answers. Took her to other GI doctor specialist, who wants to give her colonoscopy soon. She says her liver is not damaged though so that as positive. And seems to think her levels are going down. Took blood yesterday. No results yet. I’m so dismayed over this every day. Hoping I get some hopeful news from liver/hepatologist next week.
Mollie
Mollie,
When you get the labs back, run the MELD score. Hopefully the bilirubin has come down from the 11.8. If it’s still high, it really sounds like there’s a blockage still that needs to be cleared up with ERCP. Ask for the balloon dilation if at all possible and avoid the stents wherever possible.
Regarding the colonoscopy, that is standard operating procedure for newly evaluated PSC patients. As you know, the most difficult part will be the preparation. I’ve learned some helps about that as well if you want a few pointers regarding which preps are the better tolerable tasting ones. They will check her colon good and may even take brushings for cancer again which is normal operating procedure. As long as she has PSC they will probably do a colonoscopy every few years. Can’t remember the frequency.
Glad to know that her liver shows no damage presently. Hopefully it will be a very long time before any problems arise there, although the high bilirubin is concerning. Just take one day at a time, be brave and trust in God’s Providence through these days. Give Alana my greetings. 
Mark