Sunday, November 25, 2012

October 2011 - Familial Amyloidosis Meeting in Chicago

Back in October of 2009 before Mom or I had heard of amyloidosis, a national meeting for patients with familial amyloidosis was held in the Chicago area. This two-day meeting was sponsored by the Amyloidosis Support Groups and was held at Muriel Finkel's business, Finkel Supply. Several of the leading experts in familial amyloidosis from around the US (primarily Mayo Clinic and Boston University) were there and gave presentations, had Q & A sessions, and made themselves available for patients and caregivers to talk to. There were almost 100 attendees (patients and caregivers), and it was apparently such a successful meeting that they decided to have them every two years. Mom and I were very excited to attend the next meeting after we found out about it, so we planned our trip back when the dates were announced early in 2011, and off we went in October.

The 2011 meeting took place on Saturday and Sunday, October 29 and 30, also at Finkel Supply in the Chicago area, with an informal dinner for everyone at the hotel on Friday night. Mom and I traveled on Thursday, and then Friday morning we rode a train into downtown Chicago and took a hop on - hop off bus tour of Chicago. (Bus tours are a great way to see and learn about a new city, especially if you have a good tour guide. If you get a guide who isn't all that good, just hop off the bus [Gus] and wait for the next one to arrive at that stop.) We ended our tour with a visit to the
observation deck on the 103rd floor of the Willis Tower (formerly the Sears Tower), where we got to step out on a cantilevered ledge with a glass floor, enabling us to look straight down 1353 feet to the street below.

Skydeck Chicago
David standing 1353 feet above Chicago

We got back to the hotel in time for the dinner, and we sat at a table with some other people and introduced ourselves. I think there were four people from the same family (three men and the wife of one of them), and I believe all three of the men had at least one organ transplant. Their family had one of the ATTR variants. We told our story of how Mom was the first one diagnosed in our family, and then one of them told his story of how it took a long time to get diagnosed, and he was the first one diagnosed in his family and had never heard of it. He said when he called his mother and told her about his diagnosis of familial amyloidosis, she said something like, "Oh yeah, I got a letter from a cousin of mine about that awhile back. Since I didn't have any symptoms I assumed I didn't inherit it so I didn't think anything more about it." I have no idea how far my jaw dropped or how big my eyes got when I heard that, but it had to be a lot, given how passionate I am about informing all possibly affected family members about this hereditary disease. That's the exact scenario I'm trying to prevent, and here's a family that it happened to, simply due to a lack of communication. (Calm down David, calm down . . .)

Then another couple joined our table. It was a married couple, and he had one of the ATTR variants. This was the first glimpse Mom and I got of the effects of polyneuropathy, which begins with a numbness or tingling sensation in the extremities. I'd guess it's similar to when your hand or foot falls asleep if you cut off the circulation for awhile, except the numbness doesn't go away. Eventually the neuropathy expands to other body systems (such as the GI tract), and the patient will usually start losing feeling and/or strength in their limbs. This man's neuropathy had significantly affected his hand and arm strength, such that his wife had to cut up his food for him. I believe he also mentioned he has difficulty dressing himself. We couldn't help but feel sorry for him, and we had no idea how much more of that we would see over the next two days. At some point we also got to speak to Dr. Skinner from Boston for a bit. It was good to see her again.

Then the highlight of our evening occurred when we met Cathy Tidwell and her husband Lon. It was great meeting her after swapping emails off and on for over a year, and following her
blog as well. She seemed to be doing great, both physically and mentally. After catching each other up on our latest medical situations, we called it a night so we could be rested for the first full meeting day on Saturday.

I won't get into the various presentations that were given during the meeting. Most of them are available at the Amyloidosis Support Group web site: I think there were over 175 attendees at this meeting, and by far most of the patients had one of the ATTR variants. In fact, Cathy T., Mom and I were the only people there with fibrinogen amyloidosis as far as we know. So that was just another reinforcement of how rare our disease is, especially in the US. (It is much more common in the UK.) Some of my random thoughts on the meeting, in no particular order, are:

  • I mentioned the polyneuropathy we saw in one person at dinner Friday night. We saw many more people with various levels of polyneuropathy over the next two days, since that is one of the main symptoms with ATTR. There was one presentation dedicated to it, followed by a live demonstration by that doctor of how he goes about assessing polyneuropathy with a patient. Seeing all that honestly made us feel a little lucky that fibrinogen amyloidosis does not cause polyneuropathy, given its effects on a person's quality of life.
  • Although many of the presentations were geared specifically toward the ATTR variants, those were still interesting to listen to, especially when the various phases of clinical trials were being discussed and how that process works. It is a little disheartening to realize that the chances of any clinical trials directed specifically toward fibrinogen amyloidosis are pretty slim, given the number of potential patients. Hopefully there will be something that works on all types of amyloids.
  • A lot of the attendees had organ transplants, and it was not unusual to meet someone with two organ transplants, like we did at dinner Friday night. I think there were at least two people at the meeting with three transplants (heart, liver, kidney).
  • Speaking of transplants, the age of the transplant recipient was mentioned frequently. Since most types of familial amyloidosis get diagnosed later in life, people naturally want to know if there is an age limit beyond which a person cannot get an organ transplant. The doctors stressed repeatedly that a person's physical condition is much more important than their chronological age when considering suitability for a transplant. So there is no absolute age limit for transplant recipients. (Although I think it was mentioned that Medicare may put an age limit on heart transplants.) Since Mom was 70 at the time of this meeting, that was good to hear.
  • All of the doctors who presented at the meeting are really good speakers, very entertaining, informative, and funny at times. They know their stuff and they can explain it in an understandable way to their patients. They aren't afraid to admit when they don't know something or ask another doctor who does. It's a relatively small community of doctors who specialize in amyloidosis (concentrated at Boston University and Mayo Clinic), and they appear to work together very well and aren't territorial at all.

All in all it was an excellent meeting and we can't wait to attend the next familial meeting, which has already been scheduled for October 2013. The most uplifting part was seeing how well Cathy was doing after her liver transplant, and we came away from the meeting with the realization that we really needed to pursue a liver only transplant for Mom, since that's what Cathy had and she was on the verge of going on dialysis at the time. So we have a new mission once we get back home.

Speaking of getting back home, while waiting for our bags at DFW Airport, Mom noticed one of those plastic tubs on the conveyor that had what looked like the remains of a suitcase and its contents that had been pulled out of a shredder. She asked me if that was my bag, and I looked a little closer and recognized one of the shirts that was visible. Yep, that's my bag.

Shredded bag
David's suitcase at baggage claim

I couldn't really believe what I was seeing. After showing it to the baggage claim agent and getting a replacement bag on the way, I started going through the contents, simultaneously assessing the damage while trying to figure out how the heck this happened. Some of my clothes actually had burn marks, and one or two shirts were even stuck to melted parts of the nylon bag. One arm of the retractable metal handle that lifts up had a huge bend in it, and the other one was only slightly bent. My best guess on how it happened was that my bag fell off the cart as it was being driven from the plane to the terminal, and it got wedged under one of the wheels. It must have been pushed along the concrete tarmac by this wheel until either: A) The wheel finally rolled over the bag, getting the attention of the driver as the baggage cart jostled, or B) Somebody noticed the sparks or smoke or flames coming from my bag, and got the driver to stop. Amazingly enough, none of the contents were lost. It was all there, some of it even undamaged. I did file a claim and got promptly reimbursed. I included some pictures when I submitted the claim, mainly for the enjoyment of the people who handle claims. I'm sure they were glad to settle this one for about $200.

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