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.

Sunday, December 30, 2018

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:

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:





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:

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.

=====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:


=====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: