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DATE: November 15, 2009
Participants:
Ed: PRSN ModeratorEd Moderator: Let me introduce Tom, the newest member of our group. Tom is in San Francisco and was diagnosed in January
Dr. Eric: Hello, Tom. I saw your intro. I'm interested in the APL. I've seen a few patients who have both vasculitis and APL
Ed Moderator: And Dr. Eric Hoy is our chief medical advisor and immunologist. Dr. Eric, tell a little bit about APL. What you know of it. What happens exactly
Dr. Eric: In APL the antibodies react with platelets and cause blood clots DVT is common. Arterial clots can be life-threatening
Ed Moderator: So this would be a huge complication with vasculitis! Whereby the arteries are constricting or weakening already.
Dr. Eric: Yes, and it's surprising that it doesn't happen more often with vasculitis
Tom: 2 dvt's, 1 PE, HIT ...thought that was it, then the PAN diagnosis
Ed Moderator: Does APL often cause heart attacks? Or strokes?
Tom: Yes, strokes are common. I didn’t have one.
Dr. Eric: It can. It was first identified in young men (under 40) who had heart attacks with no other risk factors. If the clots occur in the cerebral arteries, it can cause stroke.
Ed Moderator: Tom, I may be asking questions from your intro, but when were you diagnosed with APL? How long ago?
Dr. Eric: So you were diagnosed with APL first?
Tom: apsl 3/09; pan 5/09
Ed Moderator: Dr. Eric...is there a possible genetic link in APL
Dr. Eric: As with all autoimmune diseases, there is probably a genetic link, but we're not sure what it is.
Tom: esr up, crp up, h&h low, plts low, not responding to cytox;on high pred
Dr. Eric: Have they tried any other drugs?
Tom: Not yet; considering rituximab; considering imuran; considering cellcept
Dr. Eric: Aspirin or anticoagulants for the APL?
Ed Moderator: You mentioned that you're really having a tough time with Cytoxan.
Tom: fondaparaneux (allergic to hep). Lw wbc (anc 1.5); anemic; mouth sores
Ed Moderator: Any complications with using thinners and the vasculitis, Dr. Eric? Actually they might help alleviate the vasc symptoms?
Dr. Eric: The danger is that if you're anticoagulated, and you have a vascular rupture, you might bleed out.
Tom: Concerned with rupturing abd aneursyms; but too risky not to not anticoagulate
Dr. Eric: Tom said the same thing... danger of clots is too great to ignore, but it does make vasculitis more risky
Ed Moderator: Tom, you mentioned that right now weakness is one of the hardest things to deal with...what else, other side effects?
Tom: Any ideas why anemic and sed rate still up.?
Dr. Eric: Still have inflammation, and probably complement activation along with that
Tom: Too neutropenic for cytoxan, now what
Dr. Eric: Is your kidney function normal?
Tom: bun/cr normal, small blood/protein in ua
Ed Moderator: Sorry...can you explain neutropenic.
Dr. Eric: Low number of neutrophils (one type of white blood cell) in the circulation. Neutrophils help with bacterial infections
Tom: How to stop inflammation - should I try rituximab? or combo immunosuppressant. Haven't been able to get below 35 mg daily of pred on 250 cytox
Dr. Eric: That is a decision to make with your doctors... your case is complex, and there are probably no simple answers.
Ed Moderator: Dr. Eric, do you think one of the disorders over the other takes priority in being treated first or more aggressively or they both need treated equally
Dr. Eric: The APL can be life threatening, so I would control that first.... some patients respond well to pred for APL, though. so one treatment may help both
Ed Moderator: Tom, as we talked about, I'll be glad to connect your doctor with a rheumy who might have more experience with these two disorders. Tom’s case sounds more challenging than even the average vasculitis patient
Dr. Eric: Tom, do you have a rheumatologist?
Ed Moderator: Dr. Eric, can you think of someone who might be good to approach. I was thinking Dr. Hoffman or Dr. Langford
Tom: That would be great... I see rheum at the VA every 2 weeks. The VA mentioned connecting to Dr. HoffmanTom:
Dr. Eric: What about Dr. Koening at University of Utah? He has the new vasculitis center in SLC.
Ed Moderator: Yes, they just opened a new vasculitis clinic there
Tom: We are going to Utah in December! I want to meet him. How to arrange?
Ed Moderator: Actually I’m hoping to have him on a guest here in a couple of weeks. Tom, I can help you. I will talk with Joyce Kullman of the Vasculitis Foundation and I can email him and share info with you. Dr. Koening is part of the Vasculitis Consortium...and doing clinical trials with PAN patients
Tom: Great! sounds good
Ed Moderator: I'll be in touch this week. Thanks Eric, I had forgotten about him. The West Coast really needs a vasculitis center doesn’t it?. In the meantime, Tom, I think I can connect you with a person at the APL patient advocacy group
Dr. Eric: Yes, I'm surprised that no one at Stanford or UCSF has jumped in to cover that area
Ed Moderator: So Tom, tell a bit about your care now. Did you go through a lot of docs before getting this one?
Tom: Do my doctors (rheum) to talk to Dr. Koening to get me seen or do they consult
Ed Moderator: I think they would prefer to have you go to the center at least initially so they can do a complete workup on you, but I'm sure they will consult with your doctor too
Tom: In the hosp for 2.5 months ; fevers/abd pain; case study, finally diagnosed angio
Ed Moderator: I can't imagine being in the hospital that long as they try to find out what was wrong. That must have been really tough
Tom: Very debilitating
Ed Moderator: Especially since you were so active.
Tom: What are my chances with remission?
Dr. Eric: Tom, remission is possible, but it involves so many factors that you need to talk to your docs about that
Ed Moderator: I told Tom one of the positive things is that he caught this early on. Before serious organ damage or something worse occurred
Dr. Eric: I've had employees that didn't spend 2.5 months in the hospital!
Tom: Hard case, learned the system too well
Dr. Eric: Yes, early diagnosis is important. Controlling the inflammation will be a big step toward remission
Ed Moderator: How many people in the group went for years undiagnosed. We’ve lost count, unfortunately. So, in that case time is on your side Tom. But you need a doctor who is adept at your unique challenges
Dr. Eric: and some people are only diagnosed when they are terminal... you're a long way from that
Ed Moderator: and that's where I hope we can help you. Dr. Eric is right, getting in touch with the new vasculitis center could be really good
Tom: Thank you. I look forward to the new vascular center. I will start gathering records
Ed Moderator: Excellent idea, Tom. That's something we really stress in our group to get as prepared as possible by keeping good records. Some members keep a daily journal that details how they feel, what they did that day
Dr. Eric: Yes, I would recommend getting copies of all your records. Whenever you have labs, get copies and keep a chronological notebook
Ed Moderator: That might be too much info for a doctor, but you're on the right track. Keep good records, lab reports... You got it. Also, Tom, It has been suggested and even done in our group.. That you have some of these records on hand to go with you on a moment's notice. If you had to go the ER...you can take a sheet describing your condition and meds you're on. Going to the ER can be a nightmarish experience for the rare disease patient. : Well, guys, I'm sorry but I have to go a bit earlier tonight. Ed Moderator:Ed Moderator:
Ed Moderator: I hope we can help you out Tom. We’ll do what we can. I have contacted some members to contact you...
Tom: Me too!! Looking forward to Utah, thank you. Thank you Ed and Dr. Eric
Ed Moderator: Thanks as always Eric!! Have a great week. : See ya'll !!!
