Hello, fellow fibrinogen amyloidosis patients. Today's post will be my overdue (and surprisingly lengthy) recap of the 2019 Hereditary Amyloidosis Support Group Meeting that was held in Chicago the last weekend of October. But before I start that I want to mention that two fibrinogen amyloidosis patients recently underwent combined liver and kidney transplants, one in Brazil and one in the US. We wish these patients the best in their recovery as they join the very small population of people cured of amyloidosis. [Edit: The one in the US did not actually occur because the potential donor liver was not a good enough match.]
As of September 30, 2019:
We will begin with my travel to the meeting. Since I was traveling alone this year I decided to arrive on Friday instead of a day or two early to visit Chicago. You longtime blog followers may recall that it is not unusual for something out of the ordinary to happen to me while traveling to or from these Chicago meetings. (For 2011, see here (http://www.fibrinogenamyloidosis.com/2012/11/october-2011-familial-amyloidosis.html) and for 2013, see here (http://www.fibrinogenamyloidosis.com/2013/10/getting-to-2013-familial-support-group.html). This trip's noteworthy travel moment occurred when I was standing in the security line at the airport Friday morning and realized I did not have a photo ID with me. Nope, I had scanned my driver license for something the day before and I left it on our scanner at home. Totally my fault. Panic set in. Can my wife get my ID to me in time? If I miss this flight, when is the next one?
Fortunately I learned from an agent at the American Airlines ticket counter that it is possible to get through TSA security without a photo ID if you are willing and able to do something called a secondary screening. First you have to present some things with your name on them, such as credit cards and insurance cards. If you make it through that they closely inspect everything you are carrying on, and then they closely inspect you. And I mean this is a very close inspection, probably more thorough than a police pat down. (Fortunately I have never had a police pat down so I do not have anything to compare it to.) The lesson learned is if you do not want a stranger touching you in places only your spouse or doctor usually touches you, be sure to bring your ID to the airport.
I got through security in plenty of time to catch my flight to Chicago, thankful I was not renting a car but wondering if I could check into my hotel without a photo ID. Fortunately I had a picture of my driver license on my phone, so no strip search or pat down was required to check in.
Once I got to my room I looked over the packet of material for meeting attendees that the Amyloidosis Support Groups assembled for the hotel to distribute when people checked in. In addition to the meeting agenda and a list of presenters and other medical personnel in attendance, I was saddened to see a page informing us that Dr. Steven Zeldenrust of the Mayo Clinic had passed away in July after a 10-year battle with cancer. I remembered him from the first two Chicago meetings I attended (2011 and 2013), and he was an author on over 100 articles related to amyloidosis, including two articles I have reviewed on this blog regarding organ transplantation. He was 56 years old.
Since it was still a few hours before the Friday evening Meet and Greet I went downstairs to make sure I knew where the rooms were for the meeting. Here are some pictures of the meeting room after it was set up late in the day on Friday. Compared to the ballroom at the O'Hare Hilton that was used for the 2015 and 2017 meetings, this room was much wider but not as deep front-to-back.
At the Meet and Greet I talked with a few other patients, all with hereditary ATTR, as well as someone from OneAmyloidosisVoice.com. I also met with Mackenzie Boedicker of Mackenzie's Mission (https://mm713.org/) and her mother Deb Boedicker, also of Mackenzie's Mission and the Amyloidosis Speakers Bureau (https://mm713.org/speakers-bureau/). The speakers bureau is doing some amazing work to increase the awareness of amyloidosis among the next generation of doctors, and I am excited to be one of the scheduled speakers in March at a medical school in Chicago.
The agenda for the meeting on Saturday was very similar to that of previous meetings, with some notable additions. PDF files of all of the presentations are available at this link: http://amyloidosissupport.org/support_groups/familial.html. Also at that same link is a 60 page PDF file (NOTES AND SOME Q AND A FROM THE MEETING) with notes taken by someone associated with Amyloidosis Support Groups. I have only glanced at a few pages of that document and it appears to be very well done, with summaries, bullet points, charts and other graphics. The direct link to that PDF file is: http://amyloidosissupport.org/support_groups/2019_notes.pdf.
Here is what the meeting room looked like Saturday morning:
The one presentation that dealt specifically with fibrinogen amyloidosis was the one on solid organ transplantation by Dr. Dispenzieri. The direct link to the PDF of her presentation is here: http://amyloidosissupport.org/support_groups/2019_06_dispenzieri.pdf. (Since these are PDF files on the ASG web site and not PowerPoint presentations, you don't always see the slides as they were presented due to overlapping graphic elements.)
Dr. Dispenzieri began with some information about organ transplants for patients with ATTR, since it is by far the most common type of hereditary amyloidosis. One piece of information I do not recall hearing before is that many patients who receive a liver from an ATTR patient as part of a domino liver transplant are developing symptoms of ATTR about ten years after the transplant. This was not expected because ATTR symptoms typically occur late in life, so the assumption was that a liver from an ATTR patient transplanted into a non-ATTR patient would take several decades before ATTR symptoms would appear.
She then talked about organ transplants for fibrinogen amyloidosis patients. She stressed that a kidney transplant without a liver transplant would likely fail within 1 to 7 years, and the 10-year survival rate for kidney transplants was 5%. That bullet point on the slide presentation says "5% (vs 65%)," but it does not say exactly what the 65% number is referring to. I assume that is for combined liver and kidney transplants. She then mentioned that a liver only transplant was a relatively new treatment option for AFib patients, and as you can see in the presentation she showed the chart from a Letter to the Editor in the 2016 issue of the journal Amyloid, which I reviewed in the May 1, 2016 blog post here: http://www.fibrinogenamyloidosis.com/2016/04/article-review-2016-liver-transplant.html. This chart plots the creatinine level of the first AFib patient to have a liver only transplant, starting at about a year before transplant through five years after the transplant, and clearly shows a long term, gradual improvement in the creatinine level. She did not mention how many liver only transplants have been done for AFib, but I am aware of three.
The presentation by the genetic counselor was informative as always, although she was pressed for time and had to rush through or skip some things, which was true of most of the presentations. I did learn about something new with regard to genetic testing during this meeting, though, that was mentioned elsewhere. For couples thinking of becoming pregnant and using in vitro fertilization (IVF), there is a procedure called preimplantation genetic diagnosis which can be used prior to implantation to help identify genetic defects within embryos. I won't discuss the pros and cons and other considerations of that procedure, but here is a link if you would like more information about it: https://americanpregnancy.org/getting-pregnant/preimplantation-genetic-diagnosis/.
The next presentation that got my attention and I feel is important for everyone was by Bob Gibson of Amyloidosis Support Groups. He discussed the Amyloidosis Patient Registry, which I remember being mentioned at the 2015 meeting. (http://www.amyloidosissupport.org/registry/index.html) Copying from the first paragraph of the web site:
"The Amyloidosis Patient Registry is a new resource for the entire amyloidosis community from Amyloidosis Support Groups. By compiling data on patient experiences with amyloidosis, the Registry will become the go-to resource for medical researchers and caregivers in their pursuit of earlier diagnosis, better treatment, and improved quality of life for those affected by this debilitating disease."
The registry is essentially a database of information gathered from amyloidosis patients about their symptoms, path to diagnosis, treatment, quality of life, etc. They were very concerned about privacy when developing this, so they partnered with a company called PrivateAccess that has experience in creating such things and keeping the information as private as the user desires. I registered for it on my phone and started answering the survey questions while Bob was speaking about it. I found it very easy to make the choices regarding what information could be shared with any researchers with access to the database vs. what information could only be shared with my permission. I would encourage anyone who has a confirmed diagnosis of amyloidosis or a confirmed mutation but is still asymptomatic to enter their information into the registry. I have since learned that it works better on a laptop or desktop computer than it does on phone.
One of the new topics at this year's meeting was Practical Help and Assistive Devices, presented by Sarah Boyd of Mayo Clinic. Her presentation is available here: http://amyloidosissupport.org/support_groups/2019_15_boyd.pdf. She discussed the role of occupational therapists, who are "specialists aiming to improve and restore your ability to perform daily activities within the home, hobbies, or job through restorative training or compensatory training using adaptive equipment." There was a lot of good information in her presentation regarding the kinds of help we all may need at some point in our lives, not just as a result of amyloidosis.
Another new topic that I believe everyone found interesting, even though it was late in the day and was one of the longer presentations, was on insurance and financial assistance presented by a cancer rights attorney. I will not cover her presentation here since there was so much and I cannot do it justice, but I do recommend reviewing the previously mentioned notes from the meeting (link to: http://amyloidosissupport.org/support_groups/2019_notes.pdf) and also reviewing the information at these two web sites: https://triagecancer.org/cancer-health-insurance-finances-cost and http://cancerfinances.org/ One important point from that presentation I wrote down in my notes was the fact that 60% of all insurance appeals are ruled in favor of patients.
There was a section on the agenda to discuss current clinical trials, most of which are for patients with hATTR or wild type TTR amyloidosis. Dr. Berk did speak briefly about the doxycycline trail, which was open to fibrinogen amyloidosis patients. The first thing he said was that the jury was still out regarding the effectiveness of doxycycline, then he discussed the issues with the clinical trial conducted by Boston University. A significant percentage of participants had to quit the trial early, many due to becoming hypersensitive to sunlight beyond just a bad sunburn. He believes, in hindsight, there were some issues with the way the study was set up, so there may be more doxycycline trials.
Before dinner Saturday night there were breakout sessions where patients could visit any of five different rooms for smaller group discussions with specialists devoted to five different topics: Cardiac; Neuropathy and GI; Genetic Counseling and non-TTR hereditary; Insurance questions and Issues; and Research, Treatment and Trials. I started in the Genetic Counseling room since that is one of my favorite topics. There was some good discussion there, but the only new thing I learned and took notes on was pre-implantation genetic diagnosis, which I mentioned earlier in this blog post.
The other breakout session I visited was the one on Research, Treatment, and Trials. One of the presentations earlier in the day was by Dr. Jonathan Wall of the University of Tennessee regarding a new imaging technique for amyloid he and his team are working on, which uses a synthetic peptide. This imaging technique would be similar in many ways to the SAP scan currently available from the NAC in London, but with some improvements as well. They are still in Phase 1 clinical trials with this scan, and during his presentation Dr. Wall mentioned a future goal would likely be to start imaging some hereditary amyloidosis patients who are known to have tested positive for a mutation but are still asymptomatic. Since I am in that category and live within driving distance of the University of Tennessee I wanted to talk to Dr. Wall about it. He gave me a pamphlet about the program (http://gsm.utmck.edu/research/ACTP/main.cfm) and suggested I send him my contact information so he will have it if and when I might be a candidate for imaging.
As luck would have it, initial results of this imaging study were presented at this month's ASH Annual Meeting (ASH = American Society of Hematologists). Here's a link to the abstract that was presented: https://ash.confex.com/ash/2019/webprogram/Paper128850.html and here is a link to an article about it at BusinessWire dot com: https://www.businesswire.com/news/home/20191208005058/en/Aurora-Bio-Announces-Clinical-Data-Lead-Program.
Sunday was the usual Question and Answer session with the panel of doctors answering questions submitted by the attendees. As you can imagine, these covered a wide range of topics. A summary of the questions and answers can be found toward the end of the previously mentioned notes document from the meeting: http://amyloidosissupport.org/support_groups/2019_notes.pdf. I did not submit any questions this time and actually left shortly before it was over so I could try to catch an earlier flight home. Unfortunately I was not able to catch an earlier flight due to the type of ticket I had purchased, so I had plenty of time to walk around the Chicago airport. During that time I learned the distance around entire perimeter of the publicly accessible area of Chicago's O'Hare Airport is just under 10 kilometers and can be walked in two hours.
That is all for now, so if nothing exciting happens the rest of December my next post will be the annual Happy New Year post in January.
=====Monthly Blog Status Updates=====
Total posts: 187 (1 in September)
Total pageviews: 132K (~1300 in September)
Email subscribers: 16 (unchanged)
Total number of countries that have viewed the blog: 157
One new country viewed the blog in September:
St. Vincent & Grenadines
As of October 31, 2019:
Total posts: 187 (0 in October)
Total pageviews: 133K (~1700 in October)
Email subscribers: 16 (unchanged)
Total number of countries that have viewed the blog: 157
No new countries viewed the blog in October.
As of November 30, 2019:
Total posts: 187 (0 in November)
Total pageviews: 137K (~3500 in October)
Email subscribers: 16 (unchanged)
Total number of countries that have viewed the blog: 159
Two new countries viewed the blog in November: