Happy New Year!!!!!!!!!! !!!

Happy New Year greetings to all loyal followers of the Fibrinogen Amyloidosis blog. Once again there isn't much for me to report, which is a good thing. I'm still asymptomatic, based on some bloodwork taken a couple of times this past year. I'm currently about three years younger than my mother was when her first symptoms appeared.

In a repeat of last year I once again attended the American Society of Hematology convention in San Diego in December, to help set up and staff a booth for the Amyloidosis Support Groups (ASG: https://www.amyloidosissupport.org/). The primary purpose of having a booth there is to raise awareness of the ASG and the educational materials available in print and online. I'd say the turnout at our booth in 2024 was similar to the 2023 turnout.

In October of this year I plan on attending the ATTR/Hereditary Amyloidosis meeting in Chicago, which is held every two years. Although these meetings are primarily geared toward ATTR patients, some of presentations are not specific to ATTR and there is always a small contingent of patients with one of the non-ATTR types, such as fibrinogen, lysozyme, apolipoprotein, gelsolin, etc. 

So that's a wrap on 2024. Welcome to 2025!

Happy New Year!!!!!!!!!! !!

Happy New Year greetings once again to all loyal followers of the Fibrinogen Amyloidosis blog. Well, another year has passed without a blog post. I'm still asymptomatic, based on lab results from my annual physical exam and a visit to a nephrologist. No news is good news in that area.

I did attend the biennial ATTR/Hereditary amyloidosis meeting in Chicago the last weekend of October. It was another huge turnout of close to 500 patients and family members. The picture below was taken from a back corner of the main meeting room, which was very wide and not very deep, so only about two-thirds of the seating is visible.

This was the first of these meetings since 2019 because the one in 2021 was cancelled due to the COVID pandemic. I did not write a separate blog post about this meeting since there was not much to report regarding fibrinogen amyloidosis, but I did come away with a few observations:

1) The treatment options for ATTR amyloidosis, and the prognosis for those patients, have significantly improved since the first time I attended one of these meetings which was in 2011. I remember in 2011 one of the main topics of discussion at that meeting was organ transplants, and there were many patients there with double or triple organ transplants. And I believe only two or three drug treatments for ATTR undergoing clinical trials were discussed at that meeting. Compare that to the 2023 meeting, where organ transplantation was not even on the agenda yet there were presentations by six or seven different pharmaceutical companies regarding drugs they have under development or in clinical trials.

2) I did ask the doctors in one of the breakout sessions if there were any drugs on the horizon for treating any of the non-ATTR hereditary types of amyloidosis, and they were not aware of any. So right now I honestly think our best hope for a drug treatment will be a drug that clears amyloid deposits of any kind from the body. One company working in that direction is Attralus, who did present at the Chicago meeting. Here are a couple of links to pages on the Attralus web site related to this activity, which they refer to as Pan-Amyloid Removal, or PAR:

https://attralus.com/science#pan-amyloid-removal
https://attralus.com/pipeline

Although Attralus is in the very early stages of of their development, I think there is reason to be optimistic if you look at the progression of ATTR treatments over the past 10 to 15 years. Right now I think Attralus is the one to watch.

Something else I did related to amyloidosis this year was attend the American Society of Hematology convention in San Diego in December. I did this as a volunteer for Amyloidosis Support Groups (ASG web site: https://www.amyloidosissupport.org/), which is a nonprofit patient advocacy group most of you are probably familiar with. I am officially a volunteer for that group now, and at this convention I helped set up and staff a booth the ASG had to help raise awareness about the ASG and the educational materials available to patients.

This is a huge convention which usually has over 25,000 medical professionals in attendance from around the world. We had a good turnout at our booth and passed out a lot of the awareness booklets the ASG put together, and I know we had people from at least 12 countries visit and express some sort of interest.

So that's a wrap on 2023. We'll see what 2024 has in store for us.

Happy New Year!!!!!!!!!! !

Greetings once again, loyal readers of the Fibrinogen Amyloidosis blog. I hope 2023 is going well for you so far.

Since this is my eleventh Happy New Year post I have eleven exclamation points in the title. I briefly considered using something like Roman numerals and replacing ten of those exclamation points with the letter "X." But then I realized I didn't want to eliminate exclamation points altogether for years 15 and 20, or force an exclamation point in there somehow, so I decided against that. What I have settled on for now is to put a space after every tenth exclamation point to make them easier to count.

I'm still alive and kicking, and asymptomatic at the ripe old age of 60. I do have a little bit of personal health news to report, which I'll do after a quick update on some blog stats.

Since this is the third consecutive Happy New Year post, there obviously wasn't much activity on the blog in 2022. This post brings the total number of posts up to 193. The all time pageviews increased from 250K to 282K in 2022.

Only one new country visited the blog in 2022, bringing the total to 167. It was Faroe Islands, which is an autonomous territory in the Kingdom of Denmark. It's a group of islands about halfway between Iceland and the coast of Norway, with a population of approximately 54,000.

Ok, to bring you up to speed on my health news we need to go back two years, to January of 2021.

2021

January: Annual physical exam. Serum creatinine = 1.11 mg/dL; GFR > 59; Creatinine-spot Urine = 160 mg/dL

February: My doctor's office called me and said they would like to refer me to a nephrologist since my creatinine-spot number was a little high, just to be on the safe side since I have fibrinogen amyloidosis. I had not paid much attention to my creatinine-spot numbers before, and if I remember correctly from doing a little research at that time it's not a very useful measurement by itself since it can increase in the short term for a variety of reasons. But it can be an indicator that additional tests and follow-up are warranted. I don't have creatinine-spot numbers in my lab work before 2017, and that year it was 148 mg/dL. In 2018 it was 45, then 39 in 2019.

March: On the Ides of March I had two firsts: My first nephrologist appointment and my first virtual doctor appointment. This was about one year into the Covid-19 pandemic so medical facilities were still limiting patient contact as much as possible. The appointment with Dr. S went well and she asked about my genetic testing and Mom's medical history. I could tell she knew this was something other than AL amyloidosis, and she half-jokingly said I probably know more about fibrinogen amyloidosis than she does. She said she would do some basic lab work and go from there, and if everything seemed fine she would monitor my numbers every six months for now, then extend that out. Two days later I had lab work and her office let me know my kidney function was normal. It was a relief to hear that from a nephrologist, although I wasn't overly concerned. (Serum creatinine = 1.19 mg/dL; GFR normal; Creatinine-spot Urine = 106 mg/dL)

July: 6 month follow-up lab work by general physician. Serum creatinine = 1.01 mg/dL; GFR > 59; Creatinine-spot Urine = 60 mg/dL

September: Lab work with the nephrologist again. Kidney function still normal. (Serum creatinine = 1.09 mg/dL; GFR normal; Creatinine-spot Urine = 31 mg/dL)

2022

April:  In-person visit with nephrologist. Kidney function normal. (Serum creatinine = 1.09 mg/dL; GFR normal; Creatinine-spot Urine = 74 mg/dL)

November: Physical exam. Kidney function normal. (Serum creatinine = 1.16 mg/dL; GFR > 59; Creatinine-spot Urine = 35 mg/dL) 

In summary, my kidneys seem to be doing fine and my creatinine-spot urine seems to fluctuate quite a bit. No proteinuria detected, so no 24-hour urine tests. If my kidney function does start to decline it will likely be caught early since I now see a nephrologist periodically, and those appointments are staggered with appointments with my general physician where I also get lab work done.

That's all there is to report right now. Until next time, stay healthy and take care.

Happy New Year!!!!!!!!!!

Greetings once again, loyal readers of the Fibrinogen Amyloidosis blog. Long time no see. Since this is my tenth Happy New Year post, I have ten exclamation points in the title of this blog post. When I first mentioned that scheme in the 2014 Happy New Year post, I said, "Hopefully I'll be around long enough such that the number of exclamation points becomes ridiculous." If we aren't there yet, I think we're pretty close.

For those of you who subscribe by email, be sure to read to the end of this post for some important information.

So, what happened with the blog in 2021? Not much, obviously. The post before this one was the Happy New Year post for 2021, so the grand total for 2021 was . . . one post. I was going to start posting some things late in the year, but at some point I decided to just leave 2021 alone and work on making 2022 better. I know I will be doing at least one post in 2022 to cover a bit of personal health news. Nothing serious, but here's a teaser: I have a nephrologist now.

Total all time pageviews for the blog increased from 191K to 250K this year, with pageviews per month ranging from 1100 to 9400. Five new countries visited the blog in 2021, bringing the total to 166. The five new visitors were:

Myanmar (Burma) Uzbekistan Vanuatu Yemen Zimbabwe If you plot those five countries on a world map (Mercator projection) you'll see they form an upside down letter "V," with Uzbekistan at the top.

Spam comments were very sparse once again in 2021. Other than one about Russian call girls, the spam comment with the most practical information was this one: "To make sure you get paid on time, always use an invoice form that contains the right details such as payment terms, itemized charges, and your payment address."

As some of you know, the amyloidosis community lost a very important member in 2021. Dr. Merrill Benson of Indiana University School of Medicine passed away on September 11, 2021. He was instrumental in discovering many hereditary amyloidosis mutations, including the first known fibrinogen amyloidosis mutation (Arg554Leu) in 1993, followed by the most common one (Glu526Val) in 1994. I always enjoyed listening to the presentations he gave at the hereditary amyloidosis meetings in Chicago, and he will surely be missed by many colleagues and patients. Here are links to his obituaries published in the Indianapolis Star newspaper and in the most recent issue of the journal Amyloid. The one in Amyloid was written by a colleague of his and covers more details of his career and what it was like to work with him, so be sure to read them both. https://www.indystar.com/obituaries/ins126861 https://www.tandfonline.com/doi/full/10.1080/13506129.2021.1996992

Ok, here is the news for email subscribers. Since the creation of this blog, subscribing via email was accomplished by a Google service called Feedburner. That service used the email address you entered when you subscribed to the blog and sent an email when a new post was published. Unfortunately, I received notice early in 2021 that Google would no longer support Feedburner. If I understood that notice correctly, existing feeds would still work indefinitely but new subscriptions would be disabled. So I poked around on the internets and found another free service I could use instead of Feedburner, and at some point I removed the Feedburner subscribe widget from the blog and added one for the new service, called Follow.It.

Note that I have not moved any subscriptions over from the Feedburner service to the Follow.It service because I feel like people should opt in to that only if they want to. So right now, as of January 1, 2022, your Feedburner subscription is still active and if you automatically received an email with this blog post then the service is still working. (I have not tested it, so I honestly do not know yet.) If the bottom of the email has these two lines . . .

You are subscribed to email updates from Fibrinogen Amyloidosis. To stop receiving these emails, you may unsubscribe now. . . . then that email came from Feedburner, in which case you do not have to do anything to keep receiving the blog posts via email from that service. (Again, "they" say it will continue working indefinitely, which doesn't necessarily mean forever.)

But if you did receive the email from Feedburner and would like to switch to the new service (Follow.It), just visit the blog (www.fibrinogenamyloidosis.com) and click the Subscribe button on the right side of the page after entering your email address. You will get a pop-up message asking you to click the confirmation link in the email it just sent you. That email will also tell you how to log in to the Follow.It web site to manage your subscription. Once you receive a blog post from the Follow.It service you can unsubscribe from the Feedburner service using the link at the bottom of that email.

If you did not receive this blog post via email but would like to subscribe, click the Subscribe button on the right side of the page after entering your email address.

In summary, if you received this blog post via email you do not have to do anything to continue receiving them via email. If you did not receive this blog post via email and would like to receive future blog posts, click the Subscribe button on the right side.

I know that was a lot to follow, so there will probably be some more communication about it either in additional blog posts, comments on this post, or both. If you have any questions or need help with your email subscriptions, feel free to email me directly at toe at juno dot com. Hopefully we can get everything straightened out by the end of the year.

David

Happy New Year!!!!!!!!!

Greetings, loyal readers of the Fibrinogen Amyloidosis blog. Welcome to 2021, which has to be an improvement compared to 2020, right?

Here is what happened with the blog in 2020:

• 2 new blog posts were written. (Down from 3 in 2019)
• No new articles were reviewed.
• 2 additional countries visited the blog, bringing the total to 161.

In patient news, I became aware of two more families affected by fibrinogen amyloidosis in 2020, and I know of one combined liver and kidney transplant that occurred in the US. As for me, I am still asymptomatic. My creatinine was just barely above the normal range in July of 2020 but then back in the normal range again when it was checked in January of 2021. There is still no indication of protein in the urine and my eGFR is fine, so for now I'll consider the high creatinine reading to be an anomaly.

Hopefully I can catch up on some article reviews this year. (It seems like I've said that before). There have been a few articles published in the past couple of years specifically about fibrinogen amyloidosis, although at a glance they do not seem to have any significant developments with regard to treatment.

One of the benefits of fewer blog posts is that it reduces the number of spam comments, as the blog only received two this year. Neither one was particularly entertaining like some previous spam comments have been, but one got my attention when I was reviewing them for this blog post. Here is the comment (edited for typos), minus the hyperlink which is the usual tipoff that a comment is spam: "You have done a great job. I hope you will do much better in the future." Me too, spammer. Me too.


=====Consolidated blog stats for second half of 2020=====

As of December 31, 2020:

Total posts: 190

Total pageviews: 191K (significantly impacted by heavy daily traffic from Hong Kong until December)

Email subscribers: 15 (unchanged)

Total number of countries that have viewed the blog: 161

No new countries viewed the blog in the second half of 2020.
=====

Ten years?

Greetings, loyal fibrinogen amyloidosis blog readers! If you are reading this email or blog entry during the summer of 2020 I hope you are staying safe and COVID free. For those of you reading this on the blog many years after 2020, I hope the COVID pandemic of 2020 is finally over and you no longer have to view every person you get close to as potentially hazardous to your health. (Also, you citizens of the future should always have plenty of toilet paper at home. Trust me.) 

There is not much news to report from here. I am still asymptomatic, and my only recent medical issue was a little scare with some chest pain that my doctor and I are attributing to musculoskeletal stress while playing around with my grandson in the front yard one afternoon. (If playing with a six-year-old child sends you to the emergency room, does that mean you're getting old?) One benefit of this episode is that I had a stress test and echocardiogram, both of which were normal. So now I have some baseline measurements in case things start to change later.

Although I still do not have an article review ready for the blog, I wanted to get a post out today, July 30, 2020, because it was ten years ago today when Mom and I were informed by Dr. Martha Skinner of the Boston University Amyloidosis Center that genetic testing showed Mom had the fibrinogen mutation. Here's a link to my blog post where I described that call and what happened over the next few days:

http://www.fibrinogenamyloidosis.com/2012/10/july-30-2010-genetic-testing-results.html

That was the beginning of my journey into the world of fibrinogen amyloidosis, and it has certainly been an interesting ten years. That first article Dr. Skinner sent me has now grown to around 60, most of which have been reviewed on the blog. It is also worth noting that the current treatment options for fibrinogen amyloidosis are essentially the same as what Dr. Skinner told us over the phone in 2010, which is organ transplants. Hopefully that will not still be true in 2030.

I will go ahead and close out this post by once again saying I hope to start doing some article reviews soon. But I am not going to suggest that will happen once things calm down in the world. So goodbye for now, stay safe, and carry on.

= = = = =

Combined stats for January through June

Total posts: 189 (1 in January)

Total pageviews: 152K (average 2K per month)

Email subscribers: 15 (unchanged)

Total number of countries that have viewed the blog: 161

New countries:

Palau

Kosovo

= = = = =

Happy New Year!!!!!!!!

Welcome to 2020 everyone. In keeping with my tradition, there are eight exclamation points in the title (or subject line) since this is my eighth Happy New Year post in the history of this blog.

Here is what happened with the blog in 2019:

• 3 new blog posts were written. (Down from 4 in 2018)

• No new articles were reviewed.

• 9 additional countries visited the blog, bringing the total to 159.

• The blog received 14 spam comments in 2019. None were especially noteworthy, but if I had to choose a favorite it would be the one that began with this: "Hi! Someone in my Myspace group shared this website with us so I came to take a look."

In patient news, I believe I became aware of two more families affected by fibrinogen amyloidosis in 2019, and I know of one combined liver and kidney transplant that occurred in 2019 and another that almost occurred but was cancelled at the last minute. In personal news, I am still asymptomatic.

That's about it for 2019. I hope you all have a safe and healthy 2020.

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

As of December 31, 2019:

Total posts: 188 (1 in December)

Total pageviews: 139K (~2000 in December)

Email subscribers: 15 (down by 1)

Total number of countries that have viewed the blog: 159

No new countries viewed the blog in December.
=====

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 (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=====

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.
=====

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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:

Laos
Caribbean Netherlands
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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:  http://amyloidosissupport.org/support_groups/familial.html.  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 https://www.oneamyloidosisvoice.com/. 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
Reunion
North Macedonia
Antigua and Barbuda
Guatemala
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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.
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And then there were three

Happy Holidays, loyal blog readers. Hopefully you are all enjoying or recovering from the holidays.

This post was prompted not only by the fact that time is running out for me to average one post per quarter this year, but also by something exciting that recently occurred in the world of fibrinogen amyloidosis. Naturally I will cover a few less exciting things first, and save the best for last.

First, please be aware that registration is now open for the sixth Hereditary Amyloidosis Support Group Meeting that occurs every two years in Chicago. The dates for the 2019 meeting are Friday, October 25 through Sunday, October 27. Friday night is a dinner and meet-and-greet, Saturday is a full day of presentations by the doctors, and Sunday morning is a question and answer session where the doctors answer questions submitted by the attendees. 

The meeting will be held at the Hilton Chicago O'Hare Airport, which is very convenient because if you fly into O'Hare you can walk to the Hilton without even going outside. Links to register for the meeting (which is free) and to get the hotel group rate are here:

http://amyloidosissupport.org/support_groups/familial.html

This meeting is a great opportunity to see presentations by and mingle with some of the most knowledgeable amyloidosis doctors in the world. Although the bulk of the meeting is geared toward patients with ATTR amyloidosis (hereditary or wild type), I still find it worthwhile to attend for several reasons, not the least of which is being able to meet other patients with the same disease. Here are links to my blog entries about the previous four meetings I attended:

2011: http://www.fibrinogenamyloidosis.com/2012/11/october-2011-familial-amyloidosis.html

2013: http://www.fibrinogenamyloidosis.com/2013/10/getting-to-2013-familial-support-group.html

2015: http://www.fibrinogenamyloidosis.com/2015/11/2015-familial-support-group-meeting.html

2017: http://www.fibrinogenamyloidosis.com/2017/11/2017-hereditary-amyloidosis-meeting.html


In other less exciting yet good news, I had some lab work done in September and I am still asymptomatic. My creatinine was 1.05 mg/dL and my estimated GFR was over 59. I am about ten years younger than my mother was when her first symptoms appeared, so I will probably be in wait-and-see mode for many years to come, hopefully forever.

In November I attended the local amyloidosis support group meeting in Charlotte, North Carolina. There were about 25 people in attendance, including patients, caregivers, and doctors. Although I know I am likely to be the only fibrinogen amyloidosis patient at these local support group meetings and most of the topics discussed will not apply to me, I still enjoy going to them because I always find it interesting listening to the doctors and hearing the stories of other patients.

One of the doctors at the November meeting gave what I thought was a very good answer to a question someone asked about kidneys healing. The question was about how well the kidneys recover after amyloid production in the body is stopped. We know different organs respond differently to not only the buildup of amyloid deposits but also to the removal of amyloid deposits. When the kidneys are damaged (scarred) by amyloid deposits, can they recover once amyloid production stops and the amyloid deposits are cleared, or are they scarred and therefore damaged for life? The doctor gave this analogy: Imagine a multi-lane highway where traffic is flowing smoothly and the roads are in good condition. That represents your kidneys functioning normally, without amyloid buildup. Now imagine a large boulder landing on this highway and not only blocking traffic in one or more lanes but also damaging the road. Think of that boulder as amyloid deposits and the reduced traffic flow as reduced kidney function. As long as that boulder is sitting on the highway, traffic flow is going to be reduced. But what happens when the boulder is removed? That will likely allow some improved traffic flow through the areas of the road that were not as badly damaged by the boulder, but some areas are so badly damaged that traffic flow through them will not get back to normal for a long time, if ever. And so it is with the kidneys. Kidney function will usually improve somewhat and then stabilize at some point after amyloid production is stopped (perhaps with continued very slow improvement), but it typically never returns to normal because some parts will never heal.

Speaking of kidneys, there was an interesting abstract presented in October of 2018 at ASN Kidney Week, an annual gathering of kidney professionals from around the globe put together by the American Society of Nephrology. The title of this abstract was "Role of Liver Transplantation in the Treatment of Fibrinogen A alpha-chain Amyloidosis." Here is a link to the abstract:

https://www.asn-online.org/education/kidneyweek/2018/program-abstract.aspx?controlId=3020418


The abstract is not long at all, so I highly recommend reading the whole thing. It presents a case for considering liver transplantation curative for fibrinogen amyloidosis. After stating that isolated kidney transplants in AFib patients typically fail due to recurrent amyloid within 1 to 5 years, it presents data on 13 patients who received liver-kidney transplants and two who received only liver transplants. The data shows there is an obvious benefit to liver transplantation, especially for those patients who have not started dialysis. Here is the conclusion of the abstract:

The therapeutic potential of liver transplantation in fibrinogen amyloidosis is truly curative. Best transplant benefit from the addition of liver to kidney grafts was achieved in low risk pre-dialysis patients. Timely intervention in the pre-dialysis setting utilising liver transplantation alone is rational, feasible and effective. We support consideration of this approach to halt disease progression and prevent haemodialysis.

So although there is not much progress toward a drug treatment for fibrinogen amyloidosis due to the small number of patients, it is the only amyloidosis type that can be said to have a cure.

Now for the exciting news I mentioned at the beginning of this blog post. Do you remember that abstract you read about just a few seconds ago, the one presented in October during Kidney Week? It already needs to be updated because another patient received a liver transplant in early December. The only detail I will mention for now is that this patient had not started dialysis, so the prospects for a successful outcome are very good.

That makes three isolated liver transplants for fibrinogen amyloidosis patients now, with those transplants occurring in 2010, 2017, and 2018. Going forward, my hope is that as more patients are diagnosed early enough in the progression of the disease, more of them will consider a liver transplant before going on dialysis. If the doctors at the NAC in London ever start proposing liver transplants for AFib patients we will likely see many more, given the large number of patients with fibrinogen amyloidosis in the UK.

See you next year!


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

As of September 30, 2018:

Total posts: 184 (1 in September)

Total pageviews: 99,900 (~2100 in September)

Email subscribers: 16 (unchanged)

Total number of countries that have viewed the blog: 148

No new countries viewed the blog in September.
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=====Monthly Blog Status Update=====

As of October 31, 2018:

Total posts: 184 (0 in October)

Total pageviews: 103,200 (~3000 in October)

Email subscribers: 15 (down by 1)

Total number of countries that have viewed the blog: 149

One new country viewed the blog in October:

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

As of November 30, 2018:

Total posts: 184 (0 in November)

Total pageviews: 108,000 (~4700 in November)

Email subscribers: 15 (unchanged)

Total number of countries that have viewed the blog: 150

One new country viewed the blog in November:

Benin
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