PAN / Vasculitis Chat

Date: January 16th 2011

Participants:

Ed: PRSN Moderator

Dr. Eric : Guest from the American College of Rheumatology

Jeremy: PAN Group Member

Peter: PAN Group Member

Ed Moderator: Hey there you are, Peter. GREAT show on Tuesday

Peter: thank-you just made it though

Peter: how is it over there with the weather?

Ed Moderator: cold, not unbearable. UK is probably worse off than us - how about you...those were some terrible floods in BrisbaneEd Moderator:

Peter: yeah it is bad even now down here in Victoria, thousands of houses affected. Our weather is really strange of late.

Ed Moderator: How about you, escape the worst?

Peter: We are all right here in Melbourne. We have had some flash flooding at times. At present there are quite a few country towns under water

Dr. Eric: Hello, Ed! Hello, Peter!

Ed Moderator: What have you been up to Eric? Globetrotting?

Dr. Eric: Yes, I've been busy with things at the University. No travels yet this year, but in Feb I'm going to Italy

Peter: work is never done when it is research

Dr. Eric: Peter, I've been able to listen to your program the last three weeks. I enjoy it very much!

Peter: Thank you, hasn't been my best of late but at least l am getting it to air

Dr. Eric: where in MN?

Jeremy: Minneapolis. yes, 20 year old son diagnosed late last year - tough but getting through it.

Ed Moderator: What treat is your son on right now?

Jeremy: just finished Cytoxan whet well, on prednisone tapering off and on a bunch of blood pressure meds - blood pressure stubborn

Ed Moderator: so he handled the Cytoxan well?

Jeremy: Very well without a hitch. Prednisone another story - tougher

Ed Moderator: Do you know how many mg he's on?

Jeremy: 5mg three tablets three times a day

Ed Moderator: And that's down from a much higher dose, right?

Jeremy: might be less - he and his doctor spoke last week - believe they tapered off a bit - and yes down quite a bit from original dose

Ed Moderator: Thanks. Well, Dr. Eric here is the man to ask any questions When was your son diagnosed?

Jeremy: passed out in May, 2010 - diagnosed in August - angiogram with "innumerable aneurisms in both kidneys.Extensive microaneurisms in both kidneys. Being treated at Mayo. Low potassium, mild cushinoid symptoms, Creatinine 1.4, urine has small amount of protein.

Ed Moderator: sorry. What does cushinoid mean?

Jeremy: I think the round, puffy face?? Acne, redness

Ed Moderator: Dr. Eric, what does that reading on creatinine tell you?

Jeremy: Because so rare - even his doctors at Mayo reluctant to say much about the future. Difficult to tell if he will ever be off prednisone, if his kidneys will improve and heal, ever get back to competitive athletics, etc.

Dr. Eric: It's high, but not excessively so. It's an indicator of kidney function.

Jeremy: To what extent can aneurisms in kidneys "heal" or "improve"?

Dr. Eric: Jeremy that depends on how much damage has been done to the tissue. With a creatinine of 1.4 and only trace protein in the urine, it sounds like it was caught early.

The fact that he's young and strong helps, too

Ed Moderator: what would be considered a high reading for creatinine

Dr. Eric: normal goes up to 1.0 or 1.2, depending on the lab

Jeremy: When we look at the angiogram pictures, it is very prominent - hundreds of little bubbles throughout. I know this is rare, but has anyone seen kidney damage like this reverse itself a bit, or is likely to stay the same or worse - or difficult to say

Dr. Eric: difficult to say... the kidney is a very complex organ, and it depends on the specific structures that have been damaged. Dr. Eric: if it's limited to the blood vessels, it might heal with minimal long-term effect.

Ed Moderator: Eric, when there's kidney involvement does it indicate at what stage the disease is at. Like with my Mom, unfortunately for her it was at a very late stage. But that's not always the case, right?

Dr. Eric: Ed, that's right.. The disease is progressive.

Ed Moderator: So you can have kidney involvement even early on...

Dr. Eric: Ed, that's right. And the kidney damage can be extensive before there are symptoms. It's possible to live on only about 30% of normal kidney function.

Jeremy: Where does it go if it goes beyond the blood vessels?

Dr. Eric: Jeremy, the glomeruli, tubules, and other cells can all be affected.

Jeremy: There is something in a report that says renal biopsy showed focal tubular atrophy, severe, with features suggestive of a large renal artery stenosis...

Ed Moderator: Could you explain tubular atrophy? Thanks

Dr. Eric: The kidney is filled with little tubules, which is where the waste products of the blood are filtered out, and the "good stuff" is retained in the bloodstream.

When blood flow is restricted, the cells in those tubules don't get enough oxygen, and they start to die. That's called tubular necrosis or atrophy.

Ed Moderator: thanks! Understood

Dr. Eric: yes, that fits with what you told me about the renal artery stenosis. That would be my major concern.

Ed Moderator: Peter...I can't recall, did you have severe kidney involvement?

Peter: yes l did, lost my right kidney early on. l have had dialysis four times when my left kidney was distressed and had very low function

Ed Moderator: Eric, typically when is dialysis suggested. I mean, in terms of damage to kidneys. With my Mom she was just at 2% of functionality.

Dr. Eric: usually around 10-15% of normal function, but some doc's will recommend it before that much damage is done.

Jeremy: What good questions should we be asking you - and his doctors at Mayo?

Dr. Eric: You asked some good questions in your email, and I will do my best to answer them in my reply, but remember that I'm not a medical doctor, I'm a research guy. You need to ask those same questions of the docs at Mayo. If they are hesitant to answer, ask in the form of, "out of 100 patients with PAN how many have you seen that ..."

Jeremy: completely understand.

Ed Moderator: Jeremy...how is your son taking it. I mean psychologically

Jeremy: Tough stubborn kid. He likes to ice fish, trap, play hockey, date his girlfriend, read books, and hang out with his buddies. He is not in denial, but does not want to focus on it and hopes it goes away...tough

Dr. Eric: It sounds like he's fairly cooperative and compliant with his meds. That's good!

Jeremy: Yes - believe he is taking his meds, but not very organized about it - mountains of pill bottles he carries around in a bag. Smart kid - does not like checking his blood pressure. Have had a few battles with him. Peter, what questions should we be asking given your experience? I would love to get my kid connected but will need to be patient.

Peter: tough to deal with it at his age, l think we all believed we were bullet proof back then

Jeremy: Nailed it Peter

Peter: l would want them to spell it all out in a straight forward manor and give the hard diagnosis and the impact of all of the meds

Ed Moderator: http://www.vasculitisfoundation.org/vasculitismedicalprocedures........Jeremy, make note of a few links I'm going to give you. From Vasculitis Foundation. Questions to ask

Dr. Eric: Peter, I agree. There is nothing to be gained by not being completely honest and open with the patient. However, sometimes, it is not possible to predict the eventual outcome. It becomes a wait-and-see situation.

Jeremy: Think we're getting it pretty straight.

Dr. Eric: mayo is very good that way.

Ed Moderator: Who is his doctor at mayo?

Jeremy: Ken Warrington is his Rheumatologist - PAN specialist and Robert Albright his Kidney doctor. Good guys. Does PAN come and go over time - with you pretty consistently - ever go away completely?

Dr. Eric: It can go into remission. Some docs will say that the patient is "cured", but there really is no cure. Relapse can occur, but some patients have long periods of remission.

Ed Moderator: http://www.vasculitisfoundation.org/vasculitisquestionstoaskyourdoctor

Jeremy: Thanks, Ed -

Ed Moderator: that last link is excellent. It lists the key questions you should be asking your doctor!

Jeremy: You asked about energy - he does fatigue more easily, but he spent all day yesterday dragging portable ice house through the snow. Naps quite a bit.

Ed Moderator: What are your challenges as a caregiver, Jeremy? What are the toughest things about caring for a young adult with PAN?

Jeremy: Balancing trying to protect his right as a young adult who wants to make his own decisions with wanting to hammer him to take care of himself. For his mom (and me, but especially his mom) - hiding our emotions from him -

Ed Moderator: You guys are scared, obviously. It must be very hard to be encouraging to him when you have concerns

Jeremy: Great family and friends around him. They all want to offer "their thoughts and prayers" which is well intentioned - and we take them - but X does not want much of that at all..period.

Ed Moderator: You raise a good point. It's almost like you have to let him feel he is leading as normal a life as possible but you also have to be vigilant maybe FOR him at time.

He may not be picking up signals or red flags so it's good you check on how he's feeling, etc.

Ed Moderator: Dr. Eric..side note question I just came across on V F news site.

What have you heard about finding showing possible link between low levels of vitamin D and autoimmune lung disease

Ed Moderator: http://www.vasculitisfoundation.org/node/3036

Dr. Eric: low levels of vitamin D can be associated with almost any condition... lots of people have low vitamin D.

Jeremy: Low salt diet is almost unmanageable. Do what we can - he eats reasonably balanced diet - but he's 20....so not easy.

Ed Moderator: I'm sure! At 20 we're all living at McDonalds and Taco Bell. I'm on a low salt diet due to heart and it's all I can do. sort of funny story. When my mom was in the hospital with PAN she was restricted from ALL salt. I mean zero intake! She was a salt addict. She begged, bribed and threatened us to bring her salt...sneak packets in. Finally she understood how dangerous it could be for her to have salt.

Peter: I am sorry all l have to go for a scan of my stomach so l bid you all farewell. If you can join me on Wednesday, l mean Tuesday your time.

Jeremy: Thanks, guys. Really appreciate this connection. I hope I haven't

somehow stood in the way of others here. Truly appreciate your help - each of you.

Ed Moderator: Peter. take care. Sorry we didn't talk more. I will be listening on TUESDAY!

Dr. Eric: I'll do my best to be there Peter. Best wishes to you and your family.

Ed Moderator: Good luck to you. Give us updates

Peter: not a problem as l found today extremely good

Ed Moderator: It sounds like X is handling it the best he can

Jeremy: Thanks, Ed. And, thanks Dr. Eric.

Ed Moderator: I'm sorry, where are you from Jeremy?

Jeremy: From Minnesota - South Minneapolis. And you?

Ed Moderator: I'm in Pittsburgh. I was just thinking there is probably a support group for vasculitis near you if you and your wife wanted to attend

Jeremy: Would like to know about it if one exists. Thanks.

Dr. Eric: there's a chapter of the VF in Rochester

Jeremy: Take care guys. Signing out.

Ed Moderator: Yep, take care everyone. Thanks for coming to the chat tonight. Have a good week.


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