Severe Pruritus

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Hi All - It has been awhile since I posted anything, although I read other's posts often and have received great advice. My sister's motor neuron progression has been significant over the past year. She is no longer able to stand or move her legs, and her arms/hands are very weak (e.g., she can no longer write her name or turn pages in a book). Despite this, she has been very courageous and continues to keep a positive attitude. However, the one thing that has been a major problem is that she has had increasingly severe, systemic pruritus for the past six months.. Even though she has limited mobility of her hands, it is so itchy that she manages to scratch her arms and wherever else she can reach until she bleeds. She has seen nurses/doctors, but no one had a reason (I think they thought it was psychosomatic). I got Benadryl and all types of OTC topical ointments, which she said helped "a little". Finally, one of her nurse caretakers (who talked to his brother, a physician, about her) suggested a liver work-up (thinking cholestatic pruritus) and started her on cholestyramine. They are doing further blood work (e.g., check for primary biliary cholangitis, etc.), but the reason I am posting is that her arm and hand motion has improved dramatically (in addition to much less severe itching). Two weeks ago, she was barely able to lift her hand to her mouth to eat, and now she is raising her arms up over her head. It is dramatic and I wanted to see if anyone else on this site had heard of any situation like this. I am not expecting a miracle, but if she can read a book and use her hands, even a little longer, I feel like some of my prayers are being answered. Thanks for taking the time to read this message.
 

affected

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Never heard of this one - I would take video to keep for evidence and go talk to her neurologist.
I'd also celebrate anything as you don't know if it will last days or for a long time.

Interesting about what has helped the pruritus as so many PALS suffer from this - did she have really high cholesterol levels?

Many of us have found that fungal issues are connected too.
 

lgelb

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Tillie, cholestatic puritis is not related to cholesterol, per se, but rather to liver disease that has slowed or stopped the flow of bile. So we wouldn't need to see high cholesterol for this to be a problem.

Besides helping itching, there is reason to believe that normalizing bile flow as cholestyramine helps to do, could improve motor function. After all, TUDCA is a related compound and it is half of AMX0035. The role of bile signaling in neurodegenerative disease is an active research area. Of course, resolving any gallbladder or liver issues is important in its own right. And, of course, jaundice can be a sign of such problems.

I'm glad for her brother's thought and her improvement as she continues through GI diagnosis, Sister.

Best,
Laurie
 

affected

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Thanks lgelb my first google search before answering was on the medication that was prescribed, rather than on cholestatic puritis - since none of us here are doctors.
"Cholestyramine is an oral medication that reduces the levels of cholesterol in the blood and improves the itching associated with liver disease" That is why I asked what I felt was a valid question. I understand that there were 2 parts to the quote I give, one is the cholesterol levels, and one is itching associated with liver disease.

PALS so often report many variations of skin itching. For my Chris, this was something that started for him more than 12 months before even his FTD symptoms and I have to add that he had the softest, supplest skin ever. As the ALS progressed, the itching areas of skin spread to many areas - and for the most part they were invisible, or could just be noticed as slightly dry areas. As the ALS progressed the itching did too and we ended up with fungal issues in so many of the areas that had started long before the ALS as simply itchy areas.

His liver functions were always completely normal and he did not take Riluzole. We had no indication of any bile duct or bile flow issues either.

So I just wanted to ask a question as it really interested me. I honestly think my response was valid.

I hope my question and the responses don't hijack this thread, I'm really interested in what you have discovered @The Younger Sister
 
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Hi lgelb and affected - It is really, really interesting to me, also. My sister never had any abnormal results on her routine screening. The only abnormal finding was a slightly. higher than normal ALT (i.e., her alkaline phosphatase, which is more likely with any kind of cholestasis, was normal!). I think (because haven't spoken to the prescribing physician directly) was that he was screening for any causes of severe, non-rash, systemic pruritus (as per google!!: among the diseases that can cause pruritus are renal insufficiency, cholestasis, Hodgkin's lymphoma, polycythemia vera, solid tumors, and many others. Other pruritic conditions appear to be iatrogenic; opioid-induced pruritus may be the most important in palliative medicine). My sister (67 years old) isn't on any medications with the exception of the lowest dose of levothyroxine, which she has been taking for years.

Despite months of trying to find out what was going with the itching (including nurse and doctor visits), a nurse who checks in on her asked her physician brother about it and asked for blood work. My guess (because I haven't spoken to him directly) is that he just decided to try her on cholestyramine to see if it helped relieve her itch. So, she has been taking it everyday for about 2 weeks. The crazy, crazy thing is that last night when I video chatted with her, she told me that she was able to lift a cup and drink from it for the first time in SIX months. I honestly can't believe how much range of motion she now has with her arms now. I think it is a great suggestion to take videos and only wish I had a video of her two weeks ago versus now because it is astounding.

I am being realistic, and take to heart your comment of, "I'd also celebrate anything as you don't know if it will last days or for a long time."

I also agree that she needs a complete work-up on her liver (....did I mention she is in quarantine for 2 weeks due to COVID exposure!!??). She is fully vaccinated and feels fine and we are almost at the end of the 2 weeks...).

Thanks for you thoughtful input. I am just blown away what is going on.....the human body is such a complex creation!
Linda (The Younger Sister by only 18 months!)
 

affected

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You are so right, the human body is incredibly complex. Full of surprises ...
 
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