Does my 12 year old son really have PSC?

We just got the diagnosis of PSC on our 12 year old son today - after 5 weeks (3 in hospital). My son will not take URSO tablets and the liquid makes him sick. I do not believe he has PSC - am I in denial? There are inconsitencies. He was only diagnosed because of elevated LFTs, no bacterias identified, MCRP only showed bile duct infection - no specific blockages, and liver biopsy showing some fibrosis in bile ducts - liver looked great. He does not have itching. Once they saw all the infection in the bile ducts, they gave him seven days of IV antibiotics and he has responded well so they are assuming it is PSC. But how can I know for sure. I know they have found him a mystery and right form the beginning expected to find gall stones, or bactria and were confused because the pattern on the tests is not typical. Reading everyones stories they are all different but kind of connected. Only connection is that my husband does have UC. How did everyone else know for sure. We are at top teaching childrens hospital in Melbourne Australia so it is not like I have not had heaps of doctors consulting on this. But it just doesn't feel right (the diagnosis) to me. Did anyone else experience the same denial as me only to find out later they were just finding it difficult to come to terms with the truth?

is there a way to get a second opinion if you have concerns?

Hi Sofie, I am the mother of a 14 year old boy in Brisabane who was diagnosed with Auto Immune Hepatitis and PSC a year ago when he was also 12. We went through the same thing -shock,disbelief,denial.How can such a rare condition affect our son especially when there were no symptoms (picked up elevated liver enzymes on blood tests.) PSC for us is something we will be dealing with years down the track (our son is at stage 2) and along with you we hope and pray that there will be medical breakthroughs to deal with the disease, its symptoms and ultimately a cure. We have felt alone in this as it is such a rare condition not many people have it/know about it, the doctors seem to have limited experience of it in young children and it is hard to find information in Australia aout it -I'm glad to have found this support group. Good luck -you are not the only one dealing with this.

Over the last week since realising the doctors diagnosis, and having looked at our blood tests and information on the web, it is sinking in. At the same time I have finally found some research being done on Children at Stanford, that gives me hope. Up until now, it seemed all of the focus was on transplant rather than a cure, or at least maintenance. Thank-you for sharing your story. I feel like I have been just going about my life oblivious to all the pain I now see people going through at the Children's hospital. I am so sorry to hear that you have been going through the same pain we are just starting our journey on, but also comforting to know that I am not alone, and that things can always be worse. My husband does not want to talk about things much because he has UC and therefore does not want to trigger an episode through stress. He says the last thing I need is two people to look after. So this support group is my only outlet.

It sounds very much like your son went through a very similar experience to what we have just gone through. I was not given a stage or anything like that. In fact our doctor did not even tell us directly that he has PSC. That has not helped the sceptism. Over the phone, he just told me to google primary schlerosing cholangitis and we would discuss further when we had our appointment. Then my son got dehydrated and ill again so I took him back to hospital. When I saw him, I asked questions about the biopsy and asked to have his case relooked at for other possibilities. He came back saying that 6 radiologists all agreed with the diagnosis and said the biospy showed some fibrosis in the bile dcts. I asked him how severe he is, a stage, grade, score, etc. and he said that could only be determined over the next year as he is monitored. He did tell me his liver was good, so I am thinking he may be Stage 2, but no-one is telling me anything. I will be seeing a new GI, head of Hepatology, in 2 weeks because our GI is going to Canada for two years, so I will be patient and hopefully find she is more confident in her diagnosis and more liberal with providing information. The old-fashioned medical approach of here is your diagnosis and just accept it approach does not suit us.

It is a real shame there is no research being done in Australia that we can help direct money to tax deductible. I am just getting our son back to good health now, keeping him on a low fat diet, but have discovered coconut oil to still give him some yummy cake. Once he is better, I would like to find a good but sustainable diet to keep his LFTs down. If you have found anything that you find is working for you, I would be really interested.



Sofie said:

Over the last week since realising the doctors diagnosis, and having looked at our blood tests and information on the web, it is sinking in. At the same time I have finally found some research being done on Children at Stanford, that gives me hope. Up until now, it seemed all of the focus was on transplant rather than a cure, or at least maintenance. Thank-you for sharing your story. I feel like I have been just going about my life oblivious to all the pain I now see people going through at the Children's hospital. I am so sorry to hear that you have been going through the same pain we are just starting our journey on, but also comforting to know that I am not alone, and that things can always be worse. My husband does not want to talk about things much because he has UC and therefore does not want to trigger an episode through stress. He says the last thing I need is two people to look after. So this support group is my only outlet.

It sounds very much like your son went through a very similar experience to what we have just gone through. I was not given a stage or anything like that. In fact our doctor did not even tell us directly that he has PSC. That has not helped the sceptism. Over the phone, he just told me to google primary schlerosing cholangitis and we would discuss further when we had our appointment. Then my son got dehydrated and ill again so I took him back to hospital. When I saw him, I asked questions about the biopsy and asked to have his case relooked at for other possibilities. He came back saying that 6 radiologists all agreed with the diagnosis and said the biospy showed some fibrosis in the bile dcts. I asked him how severe he is, a stage, grade, score, etc. and he said that could only be determined over the next year as he is monitored. He did tell me his liver was good, so I am thinking he may be Stage 2, but no-one is telling me anything. I will be seeing a new GI, head of Hepatology, in 2 weeks because our GI is going to Canada for two years, so I will be patient and hopefully find she is more confident in her diagnosis and more liberal with providing information. The old-fashioned medical approach of here is your diagnosis and just accept it approach does not suit us.

It is a real shame there is no research being done in Australia that we can help direct money to tax deductible. I am just getting our son back to good health now, keeping him on a low fat diet, but have discovered coconut oil to still give him some yummy cake. Once he is better, I would like to find a good but sustainable diet to keep his LFTs down. If you have found anything that you find is working for you, I would be really interested.



SMick said:

Hope it gives you some comfort that we are going through the same thing with our boys and that you are not alone .If you want to contact me directly, my email is ■■■■■■■■■■■■■■■■■■■. Keep strong, I am encouraged that hopefully one day there will be a breakthrough - at least our sons have more hope for better outcomes than even 10 years ago. I know the liver research people at the RPA in Sydney are doing a lot of work on immunology in liver disease. I found a book that was positive and inspiring about an American Olympic snowboarder called Kris Klug who had PSC and ultimately received a liver transplant. He now travels promoting organ donation and is back competing.Its called 'To the edge and back".



Sofie said:

Over the last week since realising the doctors diagnosis, and having looked at our blood tests and information on the web, it is sinking in. At the same time I have finally found some research being done on Children at Stanford, that gives me hope. Up until now, it seemed all of the focus was on transplant rather than a cure, or at least maintenance. Thank-you for sharing your story. I feel like I have been just going about my life oblivious to all the pain I now see people going through at the Children's hospital. I am so sorry to hear that you have been going through the same pain we are just starting our journey on, but also comforting to know that I am not alone, and that things can always be worse. My husband does not want to talk about things much because he has UC and therefore does not want to trigger an episode through stress. He says the last thing I need is two people to look after. So this support group is my only outlet.

It sounds very much like your son went through a very similar experience to what we have just gone through. I was not given a stage or anything like that. In fact our doctor did not even tell us directly that he has PSC. That has not helped the sceptism. Over the phone, he just told me to google primary schlerosing cholangitis and we would discuss further when we had our appointment. Then my son got dehydrated and ill again so I took him back to hospital. When I saw him, I asked questions about the biopsy and asked to have his case relooked at for other possibilities. He came back saying that 6 radiologists all agreed with the diagnosis and said the biospy showed some fibrosis in the bile dcts. I asked him how severe he is, a stage, grade, score, etc. and he said that could only be determined over the next year as he is monitored. He did tell me his liver was good, so I am thinking he may be Stage 2, but no-one is telling me anything. I will be seeing a new GI, head of Hepatology, in 2 weeks because our GI is going to Canada for two years, so I will be patient and hopefully find she is more confident in her diagnosis and more liberal with providing information. The old-fashioned medical approach of here is your diagnosis and just accept it approach does not suit us.

It is a real shame there is no research being done in Australia that we can help direct money to tax deductible. I am just getting our son back to good health now, keeping him on a low fat diet, but have discovered coconut oil to still give him some yummy cake. Once he is better, I would like to find a good but sustainable diet to keep his LFTs down. If you have found anything that you find is working for you, I would be really interested.