Input Please! ATG & CSA Trtmt: Local or Best Dr Away?

[Susan]

Hi all. I was dxed AA/PNH in 1998. My AA went away but probably came back as my ANC is now varying between 200 and 300. BMB tomorrow. Assuming SAA I'll need ATG, risk too high at my age for BMT. I'm in Chicago. Considering these docs, plse give your input, thanks!

1) Local very experienced AA spec good except doesn't do ATG much for some reason.
2) Locally are a couple of very good docs brd cert in Hem, Onc & Internal med who do many BMTs -using ATG and much experience keeping pts alive treating infections, misc side effect care. They've use ATG for MDS and a few SAA cases.

3) Dr Araten at NYU has treated extensively w ATG and has a great rep, bm failure expert. But I'm not thrilled with flying 4 -5 hours home alone with a rash, hives, maybe nausea, zero wbcs and feeling awful side effects/nervous. Plus I doubt very few of his pts have been at such high infection risk as I am.

4) Dr Maciejewski/Cleveland Clinic. He know regimen well, has all bm failure experience, has done a fair amount of ATG. CC closer than NY. But Dr Mac is super busy w/ admin, research, meetings, not sure if could get his personal attn much. Cked all these ctrs overall infection rates and BMT survival outcomes, CC was highest infection, lowest survival.

Right now leaning toward local with Dr Araten reviewing protocol w local Hem/onc. I'll probably go see him first, feel bad calling asking for advise/consult with other doc when haven't seen him since 06. But can't decide for sure. What do you guys think?

Any other suggestions from experience or word of mouth?

[Hopeful]

I like choice #2. It's nice to be local and to have the support of your family/friends. If #2 does BMTs and has experience with ATG, they will be equipped to handle any adverse reactions or infections.

When I was at the hospital for my ATG, I never even saw my hematologist who ordered the treatment! The nurses pretty much ran the show and were following a standard protocol that my doctor prescribed.

So, you definitely want a center that has done ATG frequently, knows how to administer it, and is equipped to handle any unusual reactions. They can always consult with your AA specialist if they see anything unusual. You can also request that your lab work be sent electronically to your AA specialist to keep them in the loop.

Good luck

[Susan]

Thanks Hopeful! If I stay local and don't feel well when I'm released I can just go home get in bed. Now I have to cross my fingers and hope it's AA and nothing worse. I never thought I'd be wishing to have AA.

Are you doing well? I hope so.

Wow, I hadn't considered I'd be handed off to the nurses. I do know to have multiple doses of Demerol ordered and ready for rigors.

Right now I'm using a relatively small close by facility since I get PNH Soliris infusions each 2 wks, a quick trip. It's pretty good, so is the specialist but she does mostly cancer. They only have 1 critical care pulmonologist on staff though. If I got say a lung fungal infection the larger center would have someone in an emergency for sure. Ha, thinking out how to handle any worst case scenario. So I'll likely go back to all care at the larger hospital until more stable since I'd get ATG there.

On the other hand I'm not certain I want resident and fellows determining my care. Suppose I can demand to see the attending as needed if I feel they're not involved enough.

[KMac]

Hi Suz,

I am sorry to hear your ANC has fallen down. How are your other counts doing? My hematologist often mentions that a fall in ANC alone, even a steep one, can sometimes be a viral or other infection, and not always a relapse. I hope that is the case with you!

If not though, I agree with Hopeful that it is very important to receive ATG in a hospital where they have extensive experience administering it. I actually started at a hospital where they did not have a lot of experience in ATG, I began to have a bad reaction about halfway through the first dose, and they got concerned and stopped the procedure. At that point I decided I wanted to find a more experienced hospital with respect to ATG. And this is not to put down the hospital where I began my treatment. I think they were a fine hospital. They diagnosed me very quickly, and in a very selfless professional manner, helped get me where I wanted to be once I told them of my wishes. But with something as rare as what we have, it is so crucial, as I have often heard Dr. Young at NIH say, that the patient get to a treatment center with specific extensive experience treating aplastic anemia.

Since you are already considering hospitals far from home, I can highly recommend my hospital - Colorado Blood Cancer Institute (CBCI) at Presbyterian Saint Luke's in Denver. My Dr. is Dr. Richard Nash, he is an immunologist/hematologist with extensive SAA treatment (both BMT and ATG). He is excellent. Also, if you ever needed a BMT, CBCI has very good success rates for that.

For my ATG at CBCI, I too had mainly nurses at my bedside for the majority of my treatment. But I was really OK with that, because they were so experienced with ATG admin, and just plain good at it. And I was a pretty tough case. My first dose which should have taken 4 hours, took 24 hours (due to bad allergic reactions). But they got me through it (not quite sure how), and anytime things got scary, the nurses got right on the phone with Dr. Nash, even in the middle of the night. After my treatment I thanked the department head for getting me through the process, and he just smiled and said, "oh around here, we make sure everyone makes it through the ATG".

Besides Cleveland Clinic and Dr. Maciejewski, I am not familiar with the other doctors you mentioned. But so far as him being busy, I have read this story which speaks very highly of his willingness to focus on individual patients and spend time with them:

http://www.newsweek.com/falling-medical-abyss-86537

...he sounds like quite a doctor. I personally wouldn't be too concerned about the lower BMT survival rates at Cleveland Clinic. I have heard about those too, but also heard the specific reason for that is that Cleveland Clinic sees very difficult cases, due to their excellent reputation and deep expertise.

I can tell you a bit of a story of my experience with Dr. Maciejewski, admittedly second hand, so I might get it somewhat wrong. But at my first hospital I had a wonderful hematologist that I will always hold in high esteem for getting me diagnosed properly and quickly, doing an expert job explaining to me what I had, and for her compassion and respect in helping me get where I wanted to be. But at first I had little idea what was going on, or where I wanted to be. I came across Dr. Maciejewski online and gravitated to him in my mind's eye. I asked my hematologist if she could try to contact him to review my case (informally). She managed to do that for me, and was taken by his passion, humanity, expertise, and colorful manner. While he said he really couldn't say much on my case without seeing me, he agreed with my hematologist that perhaps one way to get me through the ATG would be to bring me into ICU and attach me to a machine to keep my heart beating (one of my adverse side effects was dangerously low heart rate). But when I went to CBCI, Dr. Nash decided before trying that, we might as well slow down the infusion rate to see if that helps. And it did.

My point in the above story isn't that one way or the other was right or wrong, but rather that perhaps a doctor working in a cutting-edge research facility (with especially difficult cases) such as Cleveland Clinic, may be used to taking a more intensive treatment route. Whereas CBCI, while also excellent, is more of a treatment facility, so maybe they had a mindset of trying a less intensive route first. Which in my case worked well. So broadly speaking, in choosing your treatment center, you might want to think about whether your preference is to be somewhere that is pushing the envelope in clinical trials/new treatments (NIH also comes to mind there), or you'd be more comfortable somewhere that focuses on administering the already proven 'gold-standard' treatments.

Well, in closing, again it would be wonderful if you didn't have to go through another round of treatment. But if you do, we are fortunate to live in a place and time where the outcomes keep getting better, and we have multiple good choices.

[Susan]

Hi Kev,

Great advice. CBCI sounds excellent! I'm hearing about slowing down the treatment from others. Dr Araten put an AA friend of mine who has asthma in ICU for 1st day treatment. You can be sure I'd choose the center willing to have interventions ready, planned if needed. I had A-fib once and have asthma.

Guess what. Years ago when I was told I needed a BMT I had an evaluation at Fred Hutchinson. A doc pulled me aside and told me their current AA regimen was an MDS one. Too toxic and not necessary for AA. Well I looked him up recently and he's in Colorado: http://www.bloodcancerinstitute.com/....htm?id=254361
Not that you need his services but his honesty and patient advocacy is to be
appreciated. He'd recommend him to someone who needed him.

Well my preliminary bmb report is back and they don't find AA. But neutrophils are absent in marrow. Now they are checking for MDS and other. Hum, other doesn't sound good. But at Dx my marrow was first hyper, 1 month later it was hypo. A few months after that the marrow was 10% cellularity and a large PNH had suddenly appeared. So my point is even if I get news I don't like, I'll retest in a couple of months and perhaps the picture will be different and better. Sure I developed PNH but it crowded out the AA! PNH cells are abnormal but at least I had cells:-) Strange how the marrow can change so quickly. Mine must be quirky like my personality. Actually it must be very sensitive to something, if only I knew what.

So I have an appt with a very good local hospital, 2nd opinion. Will probably see Dr Mac for his opinion. I want at least 4 opinions:-) Not sure of his expertise on MDS. But since he trained at the NIH he must know MDS stuff. He could always run it by someone else if unsure. I'm older now so MDS wouldn't be unexpected but that was my initial Dx in 1998 and turned out to be wrong. Nearly got a BMT for it until Fred Hutch said the dysplasia is "not significant."

Guess I'm back to square one (other than known PNH) and have to track down all my past bmb slides to be checked for case reviews. That was 17 years ago when I criss crossed the county to get definitive dx. So at least I'm experienced in searching for a dx.

Just in case I have a beautiful wrought iron horse on my mantle along with a particularly cute rabbit. Lol. They've been my hope reminder for many years. Lot of other treatments out there for all kinds of diseases so not worried yet. But will get out the MDS books/sources just in case.

So happy for you that you're doing well.