Tuesday, January 2, 2024

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:


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.

Sunday, January 1, 2023

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.


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)


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.

Saturday, January 1, 2022

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.