My son Noah has been doing great the last few months. We have managed to get the AIH part of his overlap syndrome under control with normal ALT and GGT readings for the last 2 blood tests. He is taking azathioprine 62.5mg daily, prednisolone 5mg daily, Omeprazole 20mg daily, ursodeoxycholic acid 250mg twice a day, iron at night and osmolax when required.
This last month he has been really off his food, nausea and not wanting to eat. Also joint pain in his knees and back. Our most recent bloods have also shown dehydration, has anyone ever experienced this as a result of PSC?
2 weeks ago he was in a lot of pain in 4 or 5 locations around his liver and stomach area, he said it felt like someone was stabbing him. This has now subsided but he is still not wanting to eat much, it’s like he is hungry but then when you have made him the food and he smells and tastes it that he no longer feels like it.
I have spoken with his gp and he has said to increase his fluids and give him icypoles hyrdalyte etc to try and rehydrate him. We sent off a urine sample yesterday and we have also done full blood work including thiopurine metabolites and lipase activity.
I am so new to this and second guess myself constantly as we have only been diagnosed for about 8 months. At what point do I take him to hospital? What are the signs and symptoms of a cholangitis attack?
We live 2 hours from the nearest hospital so I don’t want to drive there just to be sent home.
We have an ultrasound in 3 days time and we are trying to book an MRCP for a few weeks time and hopefully all of that will provide some kind of answer.
Any advice on this would be amazing thank you in advance
Yes, his nausea is causing him to not want to eat. It got so bad with me my wife started cooking out on the back porch anything that had to be fried, like hamburgers, stir fry, etc. Just the smell of certain foods cooking made me really sick feeling. Even thinking about food, going to the grocery store, etc. would trigger the nausea. Ask his doctor about prescribing Zofran. It’s a non-drowsy anti-nausea medication. It will not get rid of it completely but it will help. Also, find out what kinds of foods and drinks he does like. This is not the time to be picky with him about eating junk foods and soft drinks, etc. With PSC I had to eat what I could eat at times. There were times I could go to McDonalds and have a hamburger, drink and fries and do just fine, but the smell of spaghetti sauce cooking at home made me so sick I couldn’t stand it.
Look for high-protein shakes like Boost and such. They come in various flavors. He needs to keep his protein levels up. As PSC progresses he will experience muscle wasting. Make sure he’s getting at least 30 minutes of some form of exercise in a day if possible. I would walk on a tread mil or just out in the yard. Nothing necessarily strenuous but just steady. He needs to keep his core body strength up to be physically fit for surgery should it come to that.
Joint pain is part of the disease progression as well. If he’s having leg cramps at night I would ask his doctor about taking magnesium chloride or some form of magnesium. That helped me a lot. Also having tonic water w/quinine on hand in the fridge is good to take several gulps when you get a bad cramp. It tastes nasty but it does help.
I think the dehydration will improve if he finds something he enjoys drinking. This is not the time to count calories, but this is the time for him to fight for all its worth. We are here for you. Please don’t hesitate to ask more.
PSC 2011 / Liver Transplant 2015
One other suggestion my wife reminded me of. We found that this child sized yogurt smoothie drink was something I could tolerate. You might try these.
Thank you so much for all of your advice it is so helpful I had zofran when I was pregnant, it was the only thing that helped me but I am not so sure he would qualify on the PBS in Tasmania for that, they are quite strict and need special authority as they can be quite expensive.
Although expense is nothing in comparison to my son’s quality of life . So I will definitely look into that.
I got him some isotonic drink at the chemist to help rehydrate and he likes the taste of that so a bonus there, he usually drinks a lot of water so I don’t know how he got so dehydrated, maybe just part of the way the disease works. It is all so new so finding his triggers and warning signs have b en a bit difficult. We eat a relatively low fat diet as we don’t live near takeaway shops and we don’t deep fry things but I have found that a lot of white bread and pastry, pies and cakes have a delayed negative impact on his stomach, it causes terrible cramps.
He gets jumpy legs at night and burning feet, he often can’t sleep because his feet are burning, weird I know but we usually have to run them under a cold water, and the jumpy legs continue through his sleep. He also has a thing with needed to crack his bones, usually his back, fingers and knees, every night before bed, he says it gets rid of the tension in his joints.
We have gotten him a similar thing called up and go liquid breakfast, it comes in a few different flavours so I got some of those yesterday to have a try when he doesn’t feel like he can eat.
At what point do I take him to hospital?
He gets lots of exercise and is a usually fit and healthy boy.
As far as the hospital a few indicators.
If he is running a fever of over 101.5-102.00 F it’s an indication of a possible infection in his bile ducts. That would need intervention with iv antibiotics most likely as soon as possible. I’d go to the emergency room for that. It would be good to have a prescription already filled on hand of a broad range antibiotic like Cipro. Go ahead and start him on that before going to the hospital. He will learn over time when it’s bad enough for that.
If his itching continues to get much worse with pain in his upper right quadrant. For me when that pain started going around my right side to the center of my lower right back I knew I needed an ERCP and that I had a major stricture in my bile ducts. Again, this is something he will know over time when it’s bad enough to seek help. For this I always contacted my specialist who scheduled the procedure.
Hope this helps.
At some point with psc, I tended to eat a lot less, and being very picky about it too. There was a tendency to not drink as much, but I fought that pretty hard. I have a pouch from losing my large intestine, and am prone to dehydration anyway.
I drank a lot of ensure, but would now recommend Premier Protein-less sugar and excellent nutrition here.
In going through my pouch recovery, my wife got me Pedialite twice when I was very dehydrated. That worked like a charm.
Agree on the temp of 101.5 to go to the ER. That was my standing instruction from my doc. Every time I did my blood work showed a bile duct infection.
During my weight loss, I ate a lot of ice cream. Guilt-free, as I needed the calories. Popsicles too.
How old is your boy? A younger child can become dehydrated much quicker and it can have more serious consequences than fo an adult. If you are in Tasmania, can you call HealthDirect and talk to the nurse there about if a hospital visit is warranted. Personally, I would think about going with the symptoms you have mentioned. Is the hospital you would take him to the same one where he is usually seen? Is there liver specialist there?
My son is on Budesonide instead of Prednisilone as it seems to be better tolerated /less side effects. It maybe worth checking about this at his next review.
You are definitely not alone with worrying all the time and overanalysing every symptom.
Hi. My name is Christina and I was just diagnosed with PSC w/cirrhosis (even though I have always been a tea totaller (sorry, spelling). Anyway, I have the same symptoms as you son…no appetite and troubles with dehydration, as well as fatigue, weakness, pain where my liver is. I’ve lost over 140lbs in under a year and a half. I feel your frustration as it is my own. I’m very new to this. I wish I had answers but I wanted to empathize with you, if nothing else. You boy must be very brave. You both are in my prayers.