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: