Monday, December 9, 2019

2019 Hereditary Amyloidosis Support Group Meeting

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.]

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 ( and for 2013, see here ( 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 I also met with Mackenzie Boedicker of Mackenzie's Mission ( and her mother Deb Boedicker, also of Mackenzie's Mission and the Amyloidosis 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: 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:

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: (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: 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:

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. ( 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: 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: and also reviewing the information at these two web sites: and 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 ( 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: and here is a link to an article about it at BusinessWire dot com:

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: 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=====

As of September 30, 2019:

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:

Caribbean Netherlands

Friday, September 27, 2019

Where has the year gone?

Greetings, loyal Fibrinogen Amyloidosis blog readers. I am still here, and I have all kinds of excuses for putting the blog on the back burner since January. They aren't great excuses and most of the major events are over, so we'll see if I can pick up the pace a little going forward. There have been a few recent journal articles related to fibrinogen amyloidosis that I need to review, so hopefully I can get one or two of those done by the end of the year.

First I should mention that the 2019 Biennial Hereditary Amyloidosis support meeting is less than a month away. It takes place at the O'Hare Hilton in Chicago and starts with a meet and greet the evening of Friday, October 25. More information can be found at this link:  If you plan on coming to the meeting, please send me an email (toe at juno dot com) so we can get together at some point. This will be my fifth such meeting to attend, and you can read my blog posts about previous meetings here, here, here and here.

I have updated the What should I do now? page of the blog for the first time since 2013. Other than some minor edits here and there, I did expand on the sections where organ transplants are mentioned. That topic is probably worthy of one or more blog posts.

A new online amyloidosis community has recently been created as a resource for people diagnosed with amyloidosis and those in the process of being diagnosed. The web site is It has not only a chat feature (called a Social Wall) for people to have discussions (similar to Facebook groups but with many additional features), it also has an ever-growing list of trusted resources that have been approved by medical professionals, plus a section called Diagnosis Educator with information regarding symptoms, medical tests used in diagnosis, and treatment options. This site is still rather new and there is not a lot of activity yet, and although most of the content will be related to AL or ATTR amyloidosis, I would still encourage everyone to take a look at it and join if you feel so inclined. If you explore the community a little you may even find a picture of me and a short bio, as I have some involvement with the ongoing development of the site.

Speaking of the amyloidosis community, our little fibrinogen amyloidosis community continues to grow outside of the US and Europe. Earlier this year I became aware of a large kindred in New Zealand with several family members diagnosed with fibrinogen amyloidosis, and another kindred in Brazil.

In organ transplant news I don't have any news, other than the fact that all the recent transplant recipients I am aware of are doing well, including the third liver-only recipient who received a liver transplant in December of 2018.

In personal health news, I had a physical exam in September and I am still asymptomatic, with normal creatinine and GFR, and no signs of proteinuria.

That's all for now. I will definitely be blogging about the Chicago meeting, so you won't have to wait another . . . (counting) . . . 8 months for the next post.

=====Monthly Blog Status Update (consolidated) for January through August, 2019=====

As of August 31, 2019:

Total posts: 186 (1 in January)

Total pageviews: 130K (~2400 per month, which includes 4500 hits from Israel in one day, and 2000 hits from Israel on another day)

Email subscribers: 16 (increased by 1 in January)

Total number of countries that have viewed the blog: 156

6 new countries viewed the blog from January through August:

St. Lucia
Cayman Islands
North Macedonia
Antigua and Barbuda

Tuesday, January 29, 2019

Happy New Year!!!!!!!

Welcome to 2019 everyone. In keeping with my tradition, there are seven exclamation points in the title (or subject line) since this is my seventh Happy New Year post in the history of this blog.

Here is what happened with the blog in 2018:

  • 4 new blog posts were written. (Down from 8 in 2017)
  • Two new articles were reviewed.
  • 5 additional countries visited the blog, bringing the total to 150.
  • The blog surpassed 100,000 all-time views in October.
  • The blog received 21 spam comments in 2018. The most interesting one mentioned Puerto Rican professional boxer Jose Pedraza, Spanish Formula One race car driver Fernando Alonso, and three national wildlife parks in India. It did all that in just three sentences.

In patient news, I know of one patient who had a combined liver and kidney transplant in 2018, and as I mentioned in the previous blog post, the third liver-only transplant for fibrinogen amyloidosis occurred in December. The use of liver-only transplants for fibrinogen amyloidosis is being covered more in the medical literature now, so that is good news. Speaking of good news, I continue to get my annual physical exam and I am still asymptomatic.

That's about it for 2018. I hope you all have a safe and healthy 2019.

=====Monthly Blog Status Update=====

As of December 31, 2018:

Total posts: 185 (1 in December)

Total pageviews: 110K (~2800 in December)

Email subscribers: 15 (unchanged)

Total number of countries that have viewed the blog: 150

No new countries viewed the blog in December.