Wednesday, August 31, 2016

Article Review (2013) - Renal Transplantation in Systemic Amyloidosis -- Importance of Amyloid Fibril Type and Precursor Protein Abundance

Today's post will be a quick update on me, followed by an article review, ending with the always exciting blog status update at the end.

As mentioned in the previous post, on July 28 I had my second capsule endoscopy due to a malfunction with the first one. The gastroenterologist, Dr. E, called me early the next week and said no abnormalities were found. We then discussed what the next step should be, and we agreed that I would see my primary physician to discuss whether to refer me to a hematologist, have me start taking an iron supplement, or do something else.

I saw my primary physician on Thursday, August 25, and as I expected he wanted to do some blood work to see where my hemoglobin level is now. I asked him to also have my creatinine and GFR measured with that blood work and explained why. My most recent lab work was done in March of this year, so this set of numbers will give another data point 5 to 6 months after that. I do not have those results yet, so we will have to wait until the next blog post to see what happens next. Now to the article review . . .

Title: Renal Transplantation in Systemic Amyloidosis  --  Importance of Amyloid Fibril Type and Precursor Protein Abundance (1)

Authors: Pinney, Lachmann, Sattianayagam, Gibbs, Wechalekar, Venner, Whelan, Gilbertson, Rowczenio, Hawkins, and Gilmore (UK National Amyloidosis Centre, London, UK; UCL Centre for Nephrology, London, UK)

Journal: American Journal of Transplantation (February 2013)


Renal transplantation remains contentious in patients with systemic amyloidosis due to the risk of graft loss from recurrent amyloid and progressive disease. Outcomes were sought among all patients attending the UK National Amyloidosis Centre who received a renal transplant (RTx) between January 1978 and May 2011. A total of 111 RTx were performed in 104 patients. Eighty-nine percent of patients with end-stage renal disease (ESRD) due to hereditary lysozyme and apolipoprotein A-I amyloidosis received a RTx. Outcomes following RTx were generally excellent in these diseases, reflecting their slow natural history; median graft survival was 13.1 years. Only 20% of patients with ESRD due to AA, AL and fibrinogen amyloidosis received a RTx. Median graft survival was 10.3, 5.8 and 7.3 years in these diseases respectively, and outcomes were influenced by fibril precursor protein supply. Patient survival in AL amyloidosis was 8.9 years among those who had achieved at least a partial clonal response compared to 5.2 years among those who had no response (p = 0.02). Post-RTx chemotherapy was administered successfully to four AL patients. RTx outcome is influenced by amyloid type. Suppression of the fibril precursor protein is desirable in the amyloidoses that have a rapid natural history.

Here is a link to the article:

As stated in the abstract, this article reviews the results of renal transplants among UK National Amyloidosis Centre patients between January of 1978 and May of 2011. There were 111 transplants among 104 patients with various types of systemic amyloidosis. The breakdown among the five different types of systemic amyloidosis that were represented is as follows:

AL (primary) - 25 patients
AA (secondary) - 43
AFib (fibrinogen) - 19
AApo (Apolipoprotein A1) - 14
ALys (Lysozyme) - 3

The main focus of this article is to document the results of these kidney transplants and see how the type of amyloid protein and the presence of that protein post-transplant affects how well the transplanted kidney performs. In this review I will only be focusing on the results of the AFib patients.

The introduction of the article provides some basic information about the various types of amyloidosis. It mentions three important points about fibrinogen amyloidosis that differentiate it from the other types:

 - AFib presents with proteinuria and progressive decline in kidney function, usually leading to end-stage renal disease within five years of diagnosis.

 - The fibrinogen alpha-chain is synthesized only in the liver.

 - Since fibrinogen alpha-chain is only synthesized in the liver, liver transplantation completely eliminates the mutant fibrinogen molecules from being produced.

Here are some of the statistics regarding the 19 AFib patients included in this study:

 - There were a total of 21 renal transplants among the 19 patients, indicating some transplanted kidneys failed.

 - Nine patients received combined liver and kidney transplants. (One of those was after a transplanted kidney developed amyloid.)

 - Amyloid did reoccur in seven patients who received kidney transplants, but not in any of the patients who received combined liver and kidney transplants.

 - 4 of the 10 patients who received kidney transplants had died as of the writing of the article. None of those deaths were known to be due to surgical complications. 

 - 3 of the 9 patients who received combined liver and kidney transplants died due to surgical complications.

The discussion section of the article mentions again that liver transplantation removes all of the mutant fibrinogen from the blood, preventing the ongoing accumulation of amyloid in the kidneys. But it states that the risk of combined liver and kidney transplant must be considered when deciding on kidney only vs. combined liver and kidney. This article does not mention anything about liver only transplantation, which is not surprising since it was published in 2013 and came out of the NAC in London.


This article does not really give us any new information on fibrinogen amyloidosis itself, but it does provide some statistics (from a small population) on the mortality of two treatment options (kidney transplant vs. combined liver and kidney transplant). That serves as a helpful reminder that although we hear about organ transplants quite frequently in the amyloidosis community, organ transplants are certainly not without risk.

Next up will hopefully be an update on me.

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

As of July 31, 2016:

Total posts: 169 (1 in July)

Total pageviews: 44,600 (~2300 in July)

Email subscribers: 14 (unchanged)

Total number of countries that have viewed the blog: 128

One new country viewed the blog in July:




(1) Pinney, J. H., Lachmann, H. J., Sattianayagam, P. T., Gibbs, S. D. J., Wechalekar, A. D., Venner, C. P., Whelan, C. J., Gilbertson, J. A., Rowczenio, D., Hawkins, P. N. and Gillmore, J. D. (2013), Renal Transplantation in Systemic Amyloidosis—Importance of Amyloid Fibril Type and Precursor Protein Abundance. American Journal of Transplantation, 13: 433–441. 

Monday, August 1, 2016

Not so hard to swallow

Today's post will just be an update on what has been going on with me over the past two months. The last thing I mentioned was that nothing was found on the small bowel follow through exam I had on May 11, and the next test the doctor wanted to do was a capsule endoscopy (swallowing a pill camera) to examine my small intestine.

I had the capsule endoscopy on June 27. Before swallowing the pill camera, the nurse wrapped a wide belt around my waist with pads built into it, similar to the pads used on the chest when they do an EKG. This belt has a cable coming out of it that plugs into a receiver the patient wears with a shoulder strap for the duration of the test. So I looked like I was carrying a small purse all day. Then she had me activate the system by touching one gold band of the camera (shown below) with two fingers of one hand and the other gold band with two fingers of the other hand.

MiroCam MC1000-W Capsule Endoscope and US Quarter Dollar Coin

Once the system is activated it is time to swallow the camera. Although it is rather large (10.8 mm x 24.5 mm) I found it quite easy to swallow. It did not feel like there was a lump in my throat or anything like that. I had to wear the belt and receiver for 12 hours and then I could remove it and return the equipment to the doctor's office the next morning.

Three days later the doctor's office called me with the results. Drumroll please . . . 

It did not work so I have to repeat the test. Yes, I get to repeat the capsule endoscopy. I was actually not surprised when I heard that because when we were trying to activate it the nurse said she never heard the tones from the receiver that it is supposed to emit when the camera is activated. She got another nurse to try it as well, and she never heard the tones either but she believed it was already activated. I did not have a good feeling about it and walked away from the office fully expecting to have to repeat it, which meant going through the bowel prep as well, which is similar to the prep for a colonoscopy. So I really was not surprised when I heard that news. And now I know how Mom felt that year she had to have so many colonoscopies.

I had capsule endoscopy number two on July 28. The same nurse hooked me up, but once again we did not hear any tones from the receiver to indicate it was activated. She got another nurse in the office to help, and we did hear the tones that time. (And there was much rejoicing.) Exactly twelve hours later the receiver beeped 8 times and all the lights on it turned off, so I have a much better feeling about that one. I should get those results in August. I suspect it will also come back normal, so I am looking forward to seeing what the doctor wants to try next, if anything. Hopefully he will want some more blood work first so he can see what my hemoglobin is and I can see how my kidneys are doing.

In other news, a total of ten family members went on the Caribbean cruise Mom had planned for the family just a month before she died. It started out as a high school graduation gift for two of her grandsons who graduated this year, but then it evolved into Mom paying for everyone to go. After Mom's passing there was never any doubt that she would still want us to go, so we did and everyone had a great time. We sailed on the sister ship of the one we sailed on in 2013, so it brought back many memories of that trip, including taking Mom to the medical facility one night when she needed to have an emergency dialysis session due to excessive fluid. (

I even had an opportunity to visit the medical facility on this year's cruise as well. The phone in our cabin rang one night around 10 PM and it was the teen center informing me that my son needed to go to the medical facility due to a severe nosebleed caused by getting hit in the face with a soccer ball while he was playing soccer. They asked if I wanted to come up and get him or just meet them at the medical facility. I told the caller I would meet them at the medical facility since I was all too familiar with how to get there. Fortunately the nosebleed was already stopped by the time I arrived and no further medical care was needed. It was nice to visit the medical facility for a less dire situation than my previous visit.

Hopefully the next blog post will have the results of capsule endoscopy number two.

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

As of June 30, 2016:

Total posts: 168 (1 in June)

Total pageviews: 42,300 (~1000 in June)

Email subscribers: 14 (unchanged)

Total number of countries that have viewed the blog: 127

Four new countries viewed the blog in June:

Cote d'Ivoire (Ivory Coast)

Edit August 1, 2016: Changed "May" to "June" in one of the Blog Status Updates.