Tuesday, October 30, 2012

August 3, 2010 - You are not alone, Part 2

"I have this!"

That was the first sentence of an email I received less than two hours after sending my August 2 note to the support group about Mom's diagnosis of fibrinogen amyloidosis. It was from a woman on the West Coast who also had fibrinogen amyloidosis and had undergone a liver transplant just three weeks ago. She said Mom was the only other person she knew with fibrinogen amyloidosis. So although Mom has a rare mutation of a rare form of a rare disease, we were already in contact with someone diagnosed with the same thing.

Another interesting thing about that email is that it wasn't the first time we had communicated. You may recall my previous post with the title April 23, 2010 - Foreshadowing (published October 15, 2012), where I had a short email conversation with "CT," whose initial symptoms were similar to Mom's. Well, "CT" was Cathy Tidwell, the woman from the West Coast who has fibrinogen amyloidosis. She had written me in April after I sent a note to the group about our lack of success at getting Mom diagnosed locally, and as it turns out, she did have the same thing as Mom. Cathy also has a blog documenting her journey, and fortunately she didn't wait two years to start it. Here is the link to the first post on her blog: http://cathyandlon.blogspot.com/2009/09/just-beginning_22.html

Cathy and I swapped a few emails over next day or two as I continued educating myself about fibrinogen amyloidosis. While we waited for Mom's next appointment with her nephrologist, my mission was to learn about fibrinogen amyloidosis so I could start informing the family.

Sunday, October 28, 2012

July 30, 2010 - Genetic Testing Results

After I sent the note on June 19 to the online amyloidosis support group about the preliminary results from Boston, Muriel Finkel informed that there was an online support group for familial amyloidosis only. So I joined that group also.

Dr. Skinner had asked us to touch base with her in about a month, but Mom was in the middle of a two-week trip to Hawaii on that date so I waited until Monday, July 26 to send Dr. Skinner an email. She wrote me back the next day and said they were finishing up Mom's genetic testing, and we scheduled a conference call for Friday, July 30. Given the title of this blog, it should be no surprise that they determined Mom had the fibrinogen mutation. (The other three most likely possibilities were lysozome, apolipoprotein A1, and apolipoprotein A2.)

Dr. Skinner gave us some of the specifics on fibrinogen amyloidosis, and she also emailed me an article that was a study of 71 patients and their various outcomes with liver and kidney transplants. I sent an email to the family members with a 50% chance of having inherited the same mutation, which was my two younger sisters and Mom's two brothers (one older, one younger). Then on August 2, 2010, I sent the following update to both of the online support groups, which pretty well covers everything I knew about fibrinogen amyloidosis at that point:

My mother Linda (from Dallas) got her diagnosis from Boston last week. I'm posting this update in both the regular group and the familial group. To recap:

 - 69 year-old female, history of proteinuria going back to ~2007
 - Kidney biopsy December of 2009 showed amyloids, but not definitive for
primary amyloidosis
 - Bone marrow biopsy and blood work negative for primary amyloidosis (March
 - Evaluated at Boston in June of 2010
 - Fat pad biopsy negative. Second bone marrow biopsy negative. No heart issues.
 - Kidney function is estimated to be below 40%
 - Familial amyloidosis suspected, but not ATTR. Genetic testing required.

The genetic testing revealed that she has the fibrinogen mutation, which means the liver is producing amyloids and the kidneys are usually the only affected organ. Progression is much slower than primary amyloidosis, which is good news. There are no drug treatments or clinical trials available at this time, which limits the treatment options to:

 - Do nothing other than take care of her kidneys the best she can, which means the kidneys will gradually get worse and she will eventually require dialysis.
 - Get a liver transplant. The damage is already done to the kidneys, but a liver transplant means there will be no further damage due to additional amyloids.
 - Get a kidney transplant, keeping the original liver. It takes several years for a new kidney to show signs of amyloidosis.
 - Get a combination liver and kidney transplant.

So we have a lot to think about regarding an organ transplant, if she is even a good transplant candidate given her age and current medical condition. Dr. Skinner sent me a very informative article about a study in the UK that followed 71 patients with fibrinogen amyloidosis, and there is some good info in there regarding actual outcomes with and without organ transplants. If anyone would care to share their experience regarding dialysis, organ transplantation or struggling with the decision on whether to have a transplant at this stage of the disease, feel free to contact me through the group or privately at toe@juno.com.

Since a parent with this mutation has a 50% chance of passing it to a child, I am now in the process of contacting other family members who may be affected so nobody gets unnecessarily blindsided by this disease and has to go through a long process of diagnosis (or no diagnosis). Hopefully I can convince some others to proactively have the genetic testing done so we can know for certain where it is and where it isn't. My mother's mother was an only child, but my mother's father had eight siblings, six of whom have surviving children and grandchildren. It could get interesting if anyone on that side tests positive.


So that's where we were as of early August of 2010. As indicated in the last paragraph of my note to the online support group, my focus at that point became getting the word out to the rest of the family.

Friday, October 26, 2012

June 18, 2010 - Boston Phone Call

On Friday, June 18, 2010 Mom and I had a conference call with Dr. Skinner of the Boston University Amyloidosis Center (http://www.bu.edu/amyloid/) to follow-up on the meeting we had with her in Boston on Tuesday. Luckily I took notes during the phone call and then sent the following email to myself:

Conference call with Mom and Dr. Skinner at BUMC on 6-18-10:

Mom is still a puzzle.

There was no amyloid in her abdominal biopsy.

The bone marrow biopsy was clear.

No abnormal proteins in her blood, ruling out AL amyloidosis.

Her heart is fine.

Kidney – creatinine 1.9

24 hour urine: 2.3 grams of protein

There is no more kidney tissue available for staining.

The next step is gene sequencing for rare familial types. If nothing comes from that, another kidney biopsy may be necessary.

Check back with Dr. Skinner in about a month, although it will probably take longer to complete the gene sequencing.

On the pulmonary function test, the oxygenation is a little low due to smoking, but there is no sign of amyloid in the lungs.

A summary packet from BUMC will possibly be sent late next week to all of mom’s doctors.

That pretty well sums it up. Amyloid has only been found in her kidneys so far, and it's looking like it is one of the rarer familial types. So we get to wait some more while Boston does the genetic testing. The next day I sent this update to the online amyloidosis support group:

I'd like to give the group an update on my mother Linda (from Dallas), who went to Boston for her initial evaluation last week. First, some background:

 - Investigation of proteinuria eventually led to a kidney biopsy in December of 2009.

 - Kidney biopsy showed amyloids in the kidney, but could not definitively state it was primary amyloidosis.
 - Referred to oncologist/hematologist. Bloodwork did not indicate amyloidosis, so oncologist recommended a bone marrow biopsy.
 - Bone marrow biopsy was negative for amyloidosis.

That's when I decided to contact the Boston program and sent her medical records and biopsy slides there, leading to an appointment for an evaluation. So, what did we find out?

 - Bone marrow biopsy done in Boston also came up negative for amyloids.
  - Free light chains are normal. Kappa and lambda numbers are both within the normal range. (1060 / 441)
 - Fat pad biopsy was negative for amyloids.
 - No indication of amyloids in the heart.
 - Significant kidney impairment. A rough estimate of her current kidney function is 30 to 40% of normal. (Creatinine is 1.98, GFR is 25.)

So they have essentially ruled out primary and secondary amyloidosis, and they're leaning toward familial. Dr. Skinner doesn't think it's the most common familial type, ATTR, but she is instead leaning toward one of the more rare familial types. Four possibilities she listed were apolipoprotein A1, apolipoprotein A2, fibrinogen, and lysozyme. (There are many others.) Determining which one it is requires genetic testing, which will likely take a month or more, so they can't put together a treatment plan until they know what they're treating. In the meantime mom needs to work closely with her nephrologist to take care of what kidney function she has left, as the progression going forward is unknown.

It's a bit frustrating coming away from Boston without a clear diagnosis and treatment plan yet, but it's certainly understandable considering that she appears to have a rare variation of a rare type of a rare disease. The positives we came away with were that she has no heart involvement, and more than one doctor in Boston said mom's local doctors had been doing the right things up to this point. I can't say enough good things about the team in Boston, and I can't stress enough the importance of getting an evaluation at one of the centers of excellence, even if it's just to confirm a local diagnosis. As the guest speaker (Dr. B from Mayo) said at the Dallas support group meeting this month, they see patients all the time who have been on chemo for two or three years as a result of a local diagnosis of primary amyloidosis, but the Mayo evaluation shows they actually have familial. That could have been my mother's situation if her oncologist/hematologist, who does see amyloidosis in his practice, had moved forward with a diagnosis of primary earlier this year.

So now we get to wait at least another month before finding out what Mom really has. Let the waiting and thinking begin.

(Here's the link to that video, in case you can't see it: http://www.youtube.com/watch?v=u2mqqCMu-LM)

I'll take Rare Diseases for $200 Alex.

Wednesday, October 24, 2012

June 15, 2010 - Boston Evaluation Results

So it's Tuesday afternoon and Mom has had all of her tests and doctor visits in Boston. She's about to have her last appointment, where we are expecting to finally get a diagnosis on what she has and what should be done about it. I think we ended up being the last people left in the waiting room.

We were finally called back and met with Dr. Skinner, whose title at the time was, I believe, Special Projects Director. Dr. Skinner was a very nice, soft-spoken woman who explained things very well in layman's terms. She started off by going over Mom's lab results and the test results they had so far, making notes on Mom's copy of this interim report. As I recall, she specifically went over any numbers that were outside of the normal range. Looking back over that report today, some items that stand out (in retrospect) are:

  • Creatinine was 1.98  (Normal range is 0.5 - 1.1) 
  • GFR was 25  (Normal range is >60)
  • In the section for "Complete Blood Count," HGB (hemoglobin) was 10.5.
  • Also in the "Complete Blood Count" section, HCT (hematocrit) was 29.9.  (Normal is 38 to 47) Next to that Dr. Skinner wrote "may need Epogen to keep level 30-30."
  • As the nephrologist had mentioned, the kappa and lambda numbers, and the ratio, were well within the normal range.

Then Dr. Skinner started asking Mom some family history questions such as country of origin and how her parents, grandparents, aunts and uncles had died. After the family history was taken, Dr. Skinner flipped over Mom's interim report and started making notes on the back as she walked us through both a lesson in amyloidosis and the current status of Mom's diagnosis. Here is a scan of that page of Dr. Skinner's notes, followed by my transcription since some of it is hard to read, even in its original size.

Dr. Skinner Notes
Dr. Skinner's notes from our discussion on June 15, 2010


We know you have amyloid in kidney - - - ? 2 years
We know you have significant kidney impairment.
We know you have no suspicious family history.

Amyloid types

AL - Most common - problem in bone marrow - you don't have
AA - Second most common - in persons with severe inflammation/infection  --  doubt this type

Familial types

ATTR - Most common familial  --  doubt this type
  • Rarely affects kidneys
  • Causes neuropathy, cardiomyopathy
Rare familial types  --  All four of these are possibilities
  • Apolipoprotein A1
  • Apolipoprotein A2
  • Fibrinogen
  • Lysozome


  • Lec II  --  brand new  --  affects kidneys  --  most people with this have been Hispanic

  • Check fat pad biopsy  --  Try to identify amyloid type, if possible.
  • Check all your genes of rare familial types

In case you can't see it in the scan of the notes, Dr. Skinner drew a block diagram here explaining how each child has a 50-50 chance of inheriting this type of mutated gene when only one parent has the mutated gene.

What to do

Protect kidneys

  1. Watch BP (keep it normal).
  2. Don't get dehydrated.
  3. Don't take any medication without nephrologist approval.
  4. Watch blood sugar. Keep weight down to prevent onset of diabetes.

So there you have it. In summary, they have ruled out AL amyloidosis (primary), which is what about 85% of the Boston amyloidosis patients have. And they have all but ruled out AA amyloidosis (secondary). They're leaning toward familial amyloidosis (hereditary), but not the most common form of familial, ATTR, due to the symptoms she is having. The way we left things that afternoon was that we would have a phone call with Dr. Skinner on Friday after we get back home, all of the Boston test results are in, and the Boston team has had a chance to confer.

On the one hand it was disappointing to once again come away without a definite answer regarding what Mom has, but at least we had a better understanding of why and we felt very confident that we were in the right place to find out.

The next morning I sent an email to my sisters explaining the situation and that it might be a familial type of amyloidosis. Then the four of us (Mom, Cathy, Pat and I) went on a harbor cruise in the morning, followed by a hop on, hop off bus tour of Boston that afternoon. Thursday we drove back to Newark and flew home, looking forward to the Friday phone call with Dr. Skinner.

Sunday, October 21, 2012

June 14 - 15, 2010 - Boston Evaluation

I mentioned in the previous post that Mom could not attend the amyloidosis support group meeting on Saturday, June 12, 2010 because she was on a cruise to Bermuda. So how did we get her to Boston for the evaluation on Monday, June 14? Well, it just so happened her cruise returned to the Bayonne, NJ port on Sunday, June 13. And, it just so happens that Mom's cousin Pat lives in that area and was willing to help out. So my wife Cathy and I flew to the Newark, NJ airport on Sunday. Mom and Ed took an airport shuttle from the (sea) port to the airport after they departed their cruise. Ed flew home from Newark, then Pat arrived in her car, and the four of us (Pat, Mom, Cathy and I) drove to Boston. We arrived at our hotel without incident, looking forward to finally getting some answers in the coming week. Incidentally, this hotel (Best Western Roundhouse) is a circular building that used to be a reservoir for the natural gas used to light the streetlamps of Boston in the 1800's. So we were sleeping in an old gas tank. Here's a picture:

Day 1 of Boston evaluation (June 14, 2010)  --  The hotel was just a few blocks from the Boston University amyloidosis center, so Mom and I walked there. After checking in they sent her to the lab where they took her vital signs and drew a bunch of blood, then gave her the container to use for the 24-hour urine collection. (Mom was very familiar with those since she had done 24-hour urine collections several times for her nephrologist.) Medical tests that day included an echocardiogram, a bone marrow biopsy, and a chest x-ray. Although Boston had records of Mom having these tests done locally, they usually go ahead and do them in Boston to make sure they are done correctly and analyzed correctly.

The only doctor she saw that day was Dr. L, who took her medical history and did a basic physical exam. She (Dr. L) also took a fat pad biopsy, which is where they give a local anesthetic on the abdomen and then extract some abdominal fat from below the skin. It's like a miniscule amount of liposuction with a hypodermic needle. They can usually detect amyloids in the fat pad biopsy of patients with AL amyloidsis.

We did meet several other amyloidosis patients in the waiting room, so naturally everyone got around to telling their amyloidosis story. The people I remember from the waiting room were:

 - A husband and wife. She was getting her annual amyloidosis checkup in Boston, having had a stem cell transplant a couple of years ago.

 - Another husband and wife. He had had two stem cell transplants and was also there for his annual amyloidosis checkup. (A second stem cell transplant is sometimes done when amyloidosis recurs after the first stem cell transplant.) I think he was in his fifties, maybe his sixties, and did a lot of long-distance running. He appeared to be very fit. His wife mentioned that they sometimes have trouble doing a fat pad biopsy on him because he has so little body fat. 

 - A man with two or three family members. He had familial amyloidosis and was there for his first evaluation. I don't think he had any symptoms yet, but wanted to get a baseline evaluation.

 - A man with his wife and daughter, I think. I really felt sorry for this guy. He had developed very bad peripheral neuropathy over the past year, and it had progressed to the point where he had difficulty doing things like getting dressed or writing with a pen. In fact, I felt so sorry for him that I gave him the ergonomic pen I was using, a Pen Again. (www.penagain.net)

So that was Day 1 of the Boston evaluation. Rather than get pizza from the vending machine in the hotel (yes, a vending machine that delivered hot pizza), we went to a nice seafood restaurant for dinner.

Day 2 of Boston evaluation (June 15, 2010)  --  Day 2 in Boston began with a pulmonary evaluation, where they evaluated various aspects of Mom's lung capacity, both at rest and after physical exertion. At noon there was a support group meeting for the patients who were there that week. It was moderated by a social worker and none of the doctors were there, so it was more of an opportunity for patients to share their stories, and it allowed those who had already been down this road to offer some advice to us newbies.

After the support group meeting we met with the cardiologist, Dr. M. He reviewed the results of the echocardiogram and said there was no sign of amyloidosis affecting her heart, which was really good news.

Next we met with the nephrologist, Dr. S. He said her kidneys were definitely affected by amyloids, and likely by high blood pressure, too. Regarding her blood test results, he said her lambda and kappa light chains were both normal. (Those are not normal in patients with AL amyloidosis.) I remember getting the impression that Mom was a bit of a mystery to him. (He probably had a suspicion but it wasn't his place to speculate. The doctors get together and do that as a team.) We did ask him how much of Mom's kidney function was remaining, and he said her kidneys were functioning at about 30 to 40%. That was the first time either one of us had heard that number, so we were both a little shocked by that.

Our last appointment of the day was with another Dr. S, who would go over all the test results they had so far and hopefully give us a diagnosis and a suggested treatment plan. So, what did Dr. S have to say? Stay tuned . . .

[Minor edit May 19, 2013]

Friday, October 19, 2012

June 12, 2010 - Support Group Meeting

I attended another local amyloidosis support group meeting on Saturday, June 12. Mom was unable to attend because she was not yet back from a cruise to Bermuda. I volunteered to pick up an attendee at Love Field who was flying in from San Antonio for the meeting. He was a former Air Force chaplain and Presbyterian minister, and a published author. He was a neat guy to talk to, and he seemed to be doing well.

The guest speaker was Dr. B from Mayo Clinic in Rochester. There were a total of 25 people at this meeting, compared to 17 at the meeting in March. Here are the notes I took on each of the 12 patients at this meeting. (SCT = Stem Cell Transplant)

  1. Initial symptom was high levels of protein in his urine. Undergoing chemo treatment for AL amyloidosis. Currently on dialysis. Kidney function is around 15%.
  2. SCT in 2004 (The person I picked up at the airport.)
  3. Cardiac involvement. Also has multiple myeloma.
  4. SCT in March of 2008
  5. Familial amyloidosis. Swollen legs, trouble walking.
  6. Cardiac involvement. Being treated with chemo.
  7. Localized in lungs
  8. Initial symptom was getting tired while shopping at the mall. Diagnosed at Mayo in 2008, then SCT.
  9. Familial, evaluated at Boston. Part of a drug trial.
  10. Recently diagnosed. Just started chemo.
  11. Localized in throat, diagnosed at Mayo. 17 surgeries to remove it. Always comes back.
  12. Diagnosed in 2008. Kidneys affected. SCT in June of 2008. On peritoneal dialysis now. Listed for kidney transplant.

So there were two people at this meeting who were on dialysis due to amyloidosis affecting their kidneys. When it was my turn to tell Mom's story, I was happy to say: A) Mom isn't here because she was on a cruise to Bermuda, and B) We are going to Boston next week for an evaluation.

My only other notes for this meeting were the following:

"Test for suitability for treatment"  --  I'm not sure what the context of that was. I'm guessing it had something to do with how to decide which course of treatment (Stem Cell Transplant vs. chemotherapy) is best for treating primary (AL) amyloidosis in each individual patient.

"Familial  --  Liver transplant"  --  I suppose I wrote that down since so much of the discussion at these meetings focuses on the treatment of AL amyloidosis, whereas the treatment of familial amyloidosis is totally different. In fact, one thing Dr. B said stuck in my mind while he was discussing familial. He mentioned that they frequently see patients at Mayo who have been diagnosed by their local doctors as having AL amyloidosis and then started on chemotherapy. These chemotherapy treatments, when given to someone with familial amyloidosis, actually do harm and cause peripheral neuropathy, among other things.

All in all it was another informative meeting where I got to hear more patient stories, all of which indicated that amyloidosis can take a long time to diagnose and there is no consistent pattern of symptoms.

Next up: The Boston evaluation

Thursday, October 18, 2012

May 2010 - Boston or Mayo?

At this point (April of 2010) it is clear that we need to get Mom to Boston or Mayo for a proper diagnosis. Since I had never ever sought out a second opinion for anything medically related, I was pretty clueless as to how one goes about doing that, especially given the fact that we weren't simply seeking out a local doctor for a second opinion, we were hoping to go across the country for a complete evaluation. After poking around the web sites of the amyloidosis centers at Mayo Clinic (http://www.mayoclinic.org/amyloidosis/) and Boston University (http://www.bu.edu/amyloid/) it seemed like the best thing to do was just call each place and actually talk to somebody. I already had a phone number for Dr. R's office at Mayo Jacksonville, but for some reason I called Boston first.

I called the main phone number for the Boston University Amyloid Treatment and Research program on April 26. I explained my situation to the person who answered the phone, and she explained the process for sending medical records and biopsy slides to Boston for them to analyze. If their analysis confirms amyloidosis they can schedule the three-day evaluation. OK, fair enough. They want to focus on people with amyloidosis, so you need to have amyloidosis if you go there for an evaluation.

A day or two after contacting Boston I called Dr. R's office at Mayo Jacksonville. I again explained my situation to the person who answered the phone, but the phone call just didn't seem to go as well as the call with Boston. It wasn't really clear what my next step would be to get Mom into Mayo for an evaluation, and I didn't come away from that phone call with the same warm and fuzzy feeling I had after the Boston phone call. Maybe I didn't explain things as well or ask the right questions, or maybe the person I spoke with just doesn't get as much practice taking phone calls like mine, compared to the person at Boston.

In any case, I had a better gut feeling about Boston, and after talking with Mom about it we agreed to pursue getting her there for an evaluation. So we split up the tasks and spent the next couple of weeks getting Mom's medical records and biopsy slides sent to Boston. Lots of phone calls, faxes and emails involved with that, since we needed her records from at least five doctors and two pathology labs. We eventually got everything we needed to Boston, and by the end of May they had reviewed it and scheduled her for a two-day evaluation on June 14 and 15, 2010. Time to make some travel arrangements . . .

So it was starting to look like we would finally get some answers and have some idea of what treatment lies ahead, about five months after the initial diagnosis of amyloidosis from the kidney biopsy.

Monday, October 15, 2012

April 23, 2010 - Foreshadowing

I did reply to one email I received from someone in the amyloidosis support group. It was from a woman in California we'll refer to as CT. Here is the short email conversation we had over the course of 21 minutes on April 23, 2010:

CT to me:
Hi David,
I read your post on the Amy boards this morning. Your Mom's experience sounds much like mine. I was spilling a bunch of protein and got a kidney biopsy last Sept. I belong to Kaiser HMO and my nephrologist sent the biopsy to Stanford. They found the amyloid (by staining), but it didn't react the right way for primary. They (Stanford) sent the biopsy to Mayo for clarification. In the meantime I had a bone marrow biopsy and started trying on hats to wear after SCT. Early Oct. my oncologist called me with the Mayo results. I was shocked to discover it was familial amy. I am currently on the top of the list for a liver transplant at UCSF. My rare, rare type affects mainly the kidneys. My kidneys are hanging in there, but just barely.
Mayo does some kind of specialized test on the biopsy that was just published in November (if I recall correctly).
Good luck!  If I can help any more email me.

Me to CT:
Thanks for the note. In the back of my mind I have been wondering if Mom's might be familial, since I haven't looked much into familial to know how it's diagnosed, symptoms, etc. Is your liver transplant necessary because that's where the amyloids are produced, or due to damage caused by the amyloids?

CT to me:
Yes, ALL of the bummer stuff is produced in my liver. In most familial amy MOST of the bummer stuff is produced in the liver. I only had to have the one biopsy. There is a genetic blood test that can be done to see if you have the gene, but the biopsy is needed to see if you actually have the disease. I come from a large family and still can't find anyone who may have had this. The penetrance is really low with my type. I have never seen a doctor specifically for my amy--only the symptoms. I think I went through seven different diagnoses before I finally got this one.


So now we know there is someone whose initial symptoms were somewhat similar to Mom's, and hers turned out to be familial (hereditary) amyloidosis. And she's currently listed for a liver transplant.

We can also see at this point (April of 2010) that I was considering the possibility that Mom might have familial amyloidosis, but I hadn't done much research on it.

Next up: Boston or Mayo?

Sunday, October 14, 2012

April 23, 2010 - The Amyloidosis Community Responds

After sending my question to the online support group about where to go for a diagnosis (Boston or one of the Mayo Clinic locations), I started getting responses immediately via the support group and direct emails to me.

In summary, there was no clear "winner" between Boston and Mayo. Some people recommended Boston due to their experiences there, others recommended Mayo due to a positive experience at a Mayo Clinic location. More than one person mentioned it was good that we weren't rushing into any treatment since we didn't know exactly what we were dealing with. The clear message was that we needed to get to either Boston or Mayo, and we needed to do it quickly since amyloidosis can progress so rapidly.

Below are excepts from most of the replies I received over the next two days, in no particular order, with minor edits. (Note that my email to the group had been forwarded to two different doctors at Mayo Clinic.)

From PS in Florida: As the Facilitator of the Jacksonville support group meeting, I can say for a fact that Dr. R is very knowledgeable in treating amyloidosis. There are 3 doctors at Mayo Jax that specialize in Amyloidosis . . . The only thing that I am uncertain of at Mayo Jax is what other doctors are on the team....nephrologists, cardiologists, gastroenterologists, etc. Since Mayo Rochester sees more Amyloidosis patients than Mayo Jax you may want to take that into consideration when making your decision. . . . There are several patients on this list that have been treated at Mayo Scottsdale so I will let them speak up on that clinic. I can say that I have not heard any complaints about the facility.

You can have the slides of your mother's biopsies sent to Mayo for further testing. Congo red staining can be tricky if the lab does not do the test very often. False positives or false negatives can occur if too much, or too little stain is applied to the specimen. . . . I am a localized amy patient but I can say that I have not heard of any cases of Localized Kidney amyloidosis....localized bladder, lung or skin, yes...localized kidney...no. Their lack of being able to distinguish which type of amyloidosis your mother has is a concern....Please go to Mayo or Boston! They will be able to determine the type for.

From JB in Georgia: Given our experience I would choose Boston over Mayo if it is possible.  Here are my reasons but keep in mind these reasons are due to what we experienced in our given situation.

Boston is an Amyloid Clinic - they only deal with Amyloid patients, day in, day out, all Amyloid all the time. All the patients in the waiting room are Amyloid patients, at different stages or varieties but still all Amyloid. The only two down sides are; I believe that they will only accept patients who are positively diagnosed with Amyloid (someone correct me if I am wrong) and that it is further away from us (Atlanta, Georgia).

Mayo on the other hand is great but while they handle Amyloid patients they also handle everything else too (which is great if you have more than one thing going on, which for some of us this is necessary). Mayo is an amazing facility, incredibility efficient, one stop shopping if you will. . . .
We just recently returned from Boston (J. has improved greatly since diagnosis) and the difference between our local Oncologist's office/Mayo and Boston is that (I know that this may sound silly) but the waiting room has patients who are dealing with the same things you are!  We met several families there, everywhere from a 10 year SCT survivor to a couple of newly diagnosis patients. There is a sense of comfort to see others dealing with this rare disease. Also you know that these doctors eat, sleep and breathe Amyloid, even if we were to get bad/somber news I would feel that at least we know that these people are in the forefront of treatments for this disease and that we did all we could do.  

So being that we have been to both Mayo-Jax and Boston, I would choose Boston. I hope this helps.

From PW in Missouri: I have been to both Mayo (who told me in 2004 there was nothing they could do for me) and to Boston, where I am now Dr. B’s patient. I was treated so differently in Boston and in 2006 was treated for my localized amyloidosis. I will never go back to the Mayo Clinic for my Amyloidosis but would see them for other things that cannot be treated by anyone else. I was at the Mayo for 4 days and was very disappointed with the outcome of my visit.

It’s everyone’s own decision but if you are not sure, then find out who has the most experience and what their success is when treating your Mom’s type of Amyloidosis. Most of the doctors will talk to you on the phone (wait until the big hitters are back from Rome) and it won’t cost you a thing….I have found the doctors in Boston to be the most caring individuals I have ever met.

Some on this group have had great experience with the Mayo – I was just one who did not. I just won’t take, “sorry, we can’t help you” for an answer.

From BJ: Having read both JB and PW's comments, I must agree 100%. My husband RJ has been seen first at Boston for original evaluation and experienced exactly what Jill spoke about. Then in 2007 he was so ill that he was unable to fly to Boston and we drove to Mayo, Rochester. He was admitted there for 5 days and even tho' they are an excellent facility, we did not have the comfortable feeling as we did with Boston. Then in 6 months he was to be seen at the Mayo in Scottsdale, which is a long story and suffice to say, it was a disaster. 1,500 miles for a 15 minute consultation because of a mix-up in their scheduling. So I do agree that Boston is the place to go for the professionalism, and yet the way they make you feel as if you are their only patient. Truly a place "we call our Amy home."

May you be encouraged and we are all behind you in whatever decision that you make.

From KH: Having experienced some of this similar problem with my mother Dec 2008 through May 
2009, I would say Boston. Yes, I am partial because they have been a GREAT group!! I contacted them last May/June & they were willing to give a second opinion on the biopsies already done & look all her prior tests. I felt like because they see it every day & know what they are dealing with, it was appropriate. My mother was high risk & through many tests, it was felt best to treat her at home. Even though we are 10 hours away, they have answered questions from my e-mail & from doctors locally anytime. We had mom in the ER last evening & through this support website, a physician from Boston who is covering for our doctor left a message for my mother because he knew we were having issues.

If you like your doctors have them contact Boston directly, they are always willing to 
try & help the patient!!

From KW in Florida: I go to Mayo in Rochester and have since 2003. I love everything about Mayo: my doctor, the staff, facilities, etc., etc. I have been to Mayo-Jacksonville only for support group meetings . . . I have never heard of amyloid localized in the kidney and I would agree with you about letting the nephrologist be in the driver's seat until your mom gets worked up at Mayo or BU. Preservation of kidney function is of paramount importance and indeed preservation of organ function is the main objective of treatment anyway. . . . You are wise not to rush into treatment until you know what exactly is being treated and why. Meanwhile diuretics and statins will help with the nephrotic syndrome symptoms. Tell your mom to be a little fanatic about restricting her sodium which isn't too hard to do if you avoid processed foods and eat bread only in moderation, a challenge in France! All the best to you and your mom. Please keep us posted. You're going about this in all the right ways and as a result your mom will have the best possible outcome! She raised a fine son!

From a Mayo Clinic doctor: 
Don’t hesitate to contact Mayo.... or...Boston

From Muriel Finkel (same email): Would suggest Rochester over Jax unless you know it is AL..

From BF: Over many months my husband, at 66, had increasing weight loss, stamina, breathlessness and then foamy urine which got him to a nephrologist. When a kidney biopsy with Congo Red Dye stained positive (and other tests done that day) they immediately diagnosed Amy. However, by the time we found Boston BUMC (and we live in Boston!!), and went through all of the testing there, he was a borderline candidate for the SCT due to
increasing heart damage (which caused the shortness of breath in his case).

So, I agree completely with Paula-----go to either Boston or Mayo as Fast as you can!
BTW, my husband did have the SCT successfully; it's now 2 years later, and he has no Amy. There are other options equally as effective, but time could be of the essence.

Good Luck----Your Mom is fortunate to have your advocacy---it's a Big Job! Please let us know if we can help you, too.

From Jim: I would suggest going to Boston or Mayo (Rochester if you can) and get there by last week. Time works against you with this disease!

From Dr. K at Mayo Clinic in Rochester: Muriel Finkel of Amyloidosis Support Groups sent your e-mail to me. I believe that the first step is to determine the type of amyloidosis. We dissect the amyloid stained tissue and then utilize mass spectroscopy to determine the type. This has been very successful in our experience. We find that it is more accurate than immunohistochemistry.

She also needs an echocardiogram to help determine whether the heart is involved or not. 
Your oncologist is wise in going slowly and to "do no harm."
I do not see patients any longer, but if your mother is interested in coming to Mayo Clinic Rochester, I would be glad to make the arrangements. My telephone number is . . . 


Dr. K


Next up: A short email conversation with one member of the online support group.

Thursday, October 11, 2012

April 22, 2010 - Get some help, but from where?

I went with Mom to her nephrologist appointment during the week of April 19, 2010. But rather than try to recall everything that happened that week, fortunately for me I was able to retrieve the note I sent to the online Yahoo support group. Here it is, in its entirety, other than removing some names and noting a mistake I made. The first five paragraphs are basically covering what I've already written about in the blog, with a few additional details I had forgotten about.

=====Note to amyloidosis online support group. April 22, 2010=====

Greetings. I joined the group in March and I'm looking for some guidance on how to proceed with my mother's diagnosis situation. I was at the Dallas support group meeting in March, but my mother was not. I tried to be succinct with the history below, but I see I was unsuccessful. Oh well, amyloidosis is complicated.

My mother is 68, and for the last 6 to 12 months she has been seeing a nephrologist to determine the cause of excess protein in her urine (proteinuria). I don't know the extent of those tests, other than at least three 24-hour urine samples were analyzed. Eventually a kidney biopsy was performed in late December 2009, and part of the diagnosis was amyloidosis, with this comment in the biopsy report: "There is Lambda greater than Kappa staining but not significant enough to definitively state that this is AL amyloidosis. The section stained for AA amyloid is negative." There was also this sentence: "Congo Red and Thioflavin T stains for amyloid are positive."

Her only other notable medical symptoms that I am aware of now are shortness of breath with minimal exertion (possibly due to anemia, as she has experienced that before), and she bruises very easily, which started maybe six months ago but is getting better after some recent medication changes.

After the kidney biopsy, the nephrologist referred her to her oncologist, who is also a hematologist. She (nephrologist) also suggested getting an EKG [should have been "echocardiogram"]. I went with her to the oncologist appointment in March. He does treat Amy patients in his practice. He said the EKG [echocardiogram] looked fine for her age, he wasn't seeing what he would expect to see in her blood if she had primary Amy, but he suggested a bone marrow biopsy and some more blood work. So Mom got a bone marrow biopsy and then went to Europe for two weeks with her "gentleman caller," and fortunately she got home before the recent volcano activity. [Remember that volcano eruption in Iceland that disrupted air travel around Europe in 2010? http://en.wikipedia.org/wiki/2010_eruptions_of_Eyjafjallaj%C3%B6kull]

In early April we went back to the oncologist. He said the bone marrow biopsy looked fine, and the blood work still showed no indication of amyloidosis. He had already talked to the pathologist who analyzed the kidney tissue, and the pathologist reiterated that he couldn't definitively state it was primary Amy. The pathologist was going to talk to some researchers at Duke who were looking into this type of thing and get back to the oncologist. The oncologist said he would also try to contact Dr. S about it, since Dr. S has been doing amyloidosis research for years and mom's oncologist knows him. (I believe Dr. M, who was at the Dallas meeting, worked under Dr. S for awhile.) The oncologist said this would likely be a team effort among all of her doctors, and he also mentioned the possibility of going to Mayo since they have the expertise and experience. He stressed that he didn't want to rush into any aggressive treatments until he understands what is being treated and what the expected outcome is. (Do no harm.) He asked us to give him a little time to talk to some other folks and figure out where to go from here.

::: New stuff below here :::

So this week I went with mom to her nephrologist appointment. The nephrologist had spoken to the oncologist, and it sounds like the oncologist has put the ball in the nephrologist's court, at least for now. I don't mean that negatively, that's just a convenient way to put it. It does make sense to me because right now it doesn't appear to be a systemic issue, but perhaps an issue localized in the kidneys. The nephrologist said she was going to talk to the pathologist about some further analysis of the original kidney biopsy tissue, or perhaps doing another kidney biopsy. She also mentioned getting Mayo clinic involved, which mom again said would not be a problem. So the nephrologist said to give her a little time to talk to some other folks and figure out where to go from here. The next day she called mom and said she went to med school with a doctor who is at Mayo in Jacksonville (Dr. R), and she had spoken with him and found out amyloidosis is an area of research for him. We have his office phone number.

So that's where we are right now. She obviously needs to get to either Mayo or Boston, and her doctors are supportive of that. I guess I'm looking for input on Mayo in Rochester vs. Scottsdale vs. Jacksonville. I don't want to choose Jacksonville solely because her nephrologist knows someone there, although that could be a benefit at some point. Distance from the Dallas area is approximately the same to all three, so that's not a factor. Should we contact one of the Mayo clinics, give them the history, and see which one they suggest she go to? Does it even matter at this point since she would just be going for an evaluation, not a treatment plan?

Any input would be appreciated, especially if anyone has a guess as to how this diagnosis may pan out (primary, secondary, familial, localized?). Feel free to reply to the group or email me directly at toe@juno.com.



So that's a pretty good snapshot of where my mind was at the time. Next, we'll see what kind of response I got.

Sunday, October 7, 2012

April 7, 2010 - Second oncologist appointment

On April 7, 2010, I went with Mom to her second appointment with her oncologist, Dr. C, to review the results of the bone marrow biopsy and discuss the plan going forward. I came armed with pretty much the same set of questions I had at the first appointment in March, but with higher expectations for this appointment since a bone marrow biopsy is one of the main tests for diagnosing amyloidosis. Alas, we came away empty-handed again, with no answers and even more questions.

For some reason I created a Word file that day with my notes from this appointment. Here they are, almost verbatim:

  • Bone marrow biopsy looks good. No sign of amyloidosis.
  • Blood test results don’t indicate amyloidosis.
  • So far the amyloids have only been found in the kidneys, but we don’t know why yet.
  • No need to rush into aggressive treatment at this point.Dr. C will review the kidney biopsy.
  • Dr. C will consult with Dr. V (kidney doctor) and with a local amyloidosis expert, Dr. S.
  • May need to go to Mayo Clinic for evaluation

I remember Dr. C telling Mom her bone marrow looked just fine, like that of a 15-year-old. We were not expecting to hear that. I had heard of Dr. S in my internet research, and Dr. C said he knew him personally. Supposedly Dr. S is a local doctor who was a prominent researcher in amyloidosis at one time, but he's not seeing patients any more. Dr. C also mentioned having the pathologist take another look at the kidney biopsy, but he warned us that pathologists typically don't move very quickly, so it might take awhile to get anything back from them.

In a way it was frustrating to come away from this appointment without a clear indication of what Mom really has and how to treat it, but I understood Dr. C's reluctance to start any treatment without knowing exactly what is being treated and what is the expected outcome. I was also pleased to hear him mention the possibility of going to the Mayo Clinic, since that was an indication that he is willing to get help when he needs it.

So in my mind Dr. C is still the quarterback at this point, although he can't really go on the offensive just yet because he doesn't know what he's attacking. Let's be patient see what happens over the next couple of weeks . . .

Saturday, October 6, 2012

March 13, 2010 - First Amyloidosis Support Group Meeting

On Saturday, March 13, 2010 I attended my first Amyloidosis Support Group meeting. It was held at Baylor Medical Center on Gaston Avenue, north of downtown Dallas. Mom decided not to go to this meeting with me because she had either just gotten back from a trip with Ed, or they were getting ready to go on one, and she wanted to go to one or more of my nephews' soccer or basketball games. I was OK with that since we didn't know anything about these support group meetings yet.

Based on the notes I took at the meeting, there were a total of 17 people there including me and the doctor who was invited to talk to the group. There were seven amyloidosis patients and eight others who, like me, were family members of amyloidosis patients. The doctor (Dr. M) was from Baylor and spoke mainly about AL amyloidosis and his experience with treating it.

As we went around the room and people introduced themselves and talked about their initial symptoms, how they were diagnosed, what treatments they had, etc., I took short notes for each person. Here's what I wrote for each of the patients:

  1. SCT (stem cell transplant) and chemo, 2008
  2. Kidney transplant 2003, recent primary AL, age 67
  3. Primary with cardiac involvement, fatigue, chemo now, has been to Mayo
  4. Familial. Lost father, sister has it and isn't doing well, going to Boston, two adopted daughters
  5. Localized to throat, 17 throat surgeries
  6. Acute heart involvement. Long time to diagnose.
  7. Very localized to back of right calf.

So we had primary, localized and familial amyloidosis represented among those seven patients. The leader of the group (AW) had lost her husband in 2006 shortly after he was diagnosed with amyloidosis with cardiac involvement.

When I told the group where we were with Mom's diagnosis and how the kidney biopsy results couldn't say conclusively that it was primary amyloidosis, someone suggested that the kidney biopsy could be looked at by a Dr. B in Indiana. I also heard it mentioned several times the need to get to Boston or Mayo for proper diagnosis, as those are the two centers of excellence for amyloidosis. Lastly, I thought this was important enough to write down: "If diagnosed with Amy and multiple myeloma, don't treat the multiple myeloma."

After the meeting they had all the amyloidosis patients pose for a group photo. They wanted me to get in the picture as a stand-in for Mom, but I think I declined since I wasn't the patient. The leader of the group also gave me her phone number and told me to call her with any questions I had about treatments, insurance, centers of excellence, etc.

All in all it was a very good meeting and it was quite interesting listening to everyone's stories about how difficult it was to get properly diagnosed, since so many of symptoms of amyloidosis typically are caused by conditions other than amyloidosis.

So now we're waiting on the results of the bone marrow biopsy and the next appointment with Dr. C . . .

Friday, October 5, 2012

March 9, 2010 - First oncologist appointment

On March 9, 2010 I went with Mom to her first appointment with her oncologist, Dr. C of Texas Oncology. He is also a hematologist, which is why he has some experience treating patients with amyloidosis. As I previously mentioned, Dr. C treated her when she had breast cancer several years ago, and she was comfortable seeing him again for the amyloidosis. I had never met him.

I came to this appointment with a list of questions such as what treatment is recommended, how soon does she need to start, what's the likely progression if nothing is done, are any other tests needed at this point, etc. In retrospect we were probably hoping for a little too much at this first appointment, but after researching this disease on the internet for two months, reading about stem cell transplants and chemotherapy, and printing a stack of paper about two inches tall for Mom to read, we knew we needed to do something and we needed to do it quickly.

Doctor C first gave us a very simple, understandable lesson on what AL amyloidosis is. I won't even attempt to duplicate it here, but the gist of it is that you can think of the bone marrow as the factory that makes blood, and the factory has a little quality control problem. (That's my over-simplified analogy, not his.) Anyway, it was very understandable at the time. He then told us he had looked at some of Mom's bloodwork, and he wasn't seeing what he would expect to see in the blood of someone with amyloidosis. But he cautioned that you can't really make a diagnosis of amyloidosis from bloodwork alone, so the next step would be to do a bone marrow biopsy.

So although we didn't come away with a treatment plan after this appointment with Dr. C, I felt like we were in good hands and the bone marrow biopsy seemed like a reasonable next step to take. I liked Dr. C and I had no qualms about him being the "quarterback" in terms of directing Mom's treatment plan, since all of her doctors would need to be involved.

Next up, my first local Amyloidosis Support Group meeting.

Wednesday, October 3, 2012

March 8, 2010 - You are not alone

So my internet research was moving right along prior to Mom's March 9 appointment with her oncologist, and I was starting to find other people online with amyloidosis. Through the amyloidosis foundation web site I found the amyloid listserv and subscribed to it:


But the real find was on March 2, 2010 when I discovered the existence of support groups around the country, AND they have one that meets in Dallas, AND the next meeting is March 13. It's hard to believe in retrospect that it took me a month to find that, but thank goodness I did. At the time I felt like the luckiest boy in the world:

So I sent some emails to the two RSVP addresses to confirm the March 13 meeting was on, telling them my mother and I would like to attend since she was recently diagnosed. One of the local organizers recommended I inform the President of the Amyloidosis Support Groups, Muriel Finkel, that I was planning on attending. So on March 8, 2010, I sent my first email to Muriel Finkel. She wrote me back within 20 minutes and recommended I also join the online support group in Yahoo groups. Later that day I joined the online group. When a new member joins, an email is sent to the group with the following subject line:

Welcome New Member - You Are Not Alone

When you find out you have a rare disease, especially one you've never even heard of before, it is a good feeling when you realize you are not alone. I guess there had been a lot of new members joining the group lately, because Muriel replied to my New Member message with this:

So many newbie's......make sure you get a second opinion at a center of excellence and experience...on the website www.amyloidosissupport.com <http://www.amyloidosissupport.com/> under medical links....your local docs can work with these experienced doctors and learn and all will be the better for it........be sure and watch the animation on the top right of the website under the map/flag and read all of the information on all of the pages........you will be tested:-)

Take note of the phrase, "a second opinion at a center of excellence and experience." It will come up again.

So it's March 8, 2010, the day before I go with Mom to her first appointment with her oncologist since the diagnosis of amyloidosis. I have found an online support group and I'm planning on attending a local support group meeting four days after that. Things are moving along at a good pace, so maybe will start getting some answers and have a better idea of what's going to happen next. Maybe, maybe not.

Tuesday, October 2, 2012

February 2010 - What to do, where to go?

Mom's next doctor appointment regarding amyloidosis would be with the oncologist/hematologist who treated her when she had breast cancer about 10 years ago. Supposedly this doctor (Dr. C) had treated amyloidosis patients in his practice, so we felt good about going to see him. She scheduled an appointment for March 9, 2010, so I had plenty of time to search the internet to see what I could find out regarding types of amyloidosis, causes, symptoms, treatments, etc. Here are the main things I think I knew about amyloidosis as of February of 2010:

  • AL (primary) amyloidosis is by far the most commonly diagnosed form of amyloidosis. The cause of AL amyloidosis is unknown.
  • Treatments for AL amyloidosis include a stem cell transplant or chemotherapy.
  • AA amyloidosis is secondary to some other condition or disease in the body.
  • Localized amyloidosis means it is localized to a specific part of the body. Again, cause unknown.
  • Familial amyloidosis is a hereditary form of amyloidosis. There was no mention of familial amyloidosis in the biopsy report, so no need to worry about that.

It became quite clear that it could be difficult to find a doctor with any experience with amyloidosis, much less someone who could be considered a specialist. Eventually I found two very important web sites. They were the Amyloidosis Foundation at: 


and Amyloidosis Support Groups at:


I started reading through the patient stories on both of these web sites, and lo and behold there was a story by a woman (GL) in the DFW area, who mentioned a doctor at Baylor Hospital that had treated her husband. I wrote to her, told her my mom had recently been diagnosed, and thanked her for giving us some hope that we might be able to find someone in this area with amyloidosis experience. She did write back and tell me she would definitely recommend Dr. B at Baylor. Sadly her husband had passed away suddenly in May of 2009 due to a rare side effect of amyloidosis, but he had lived a normal life with amyloidosis for 8 years.

So the input rate of information about amyloidosis is starting to pick up as we head into March, and I'm starting to make contact with others who are dealing with the same thing. Hold on tight . . .

Monday, October 1, 2012

January 2010 - Kidney Biopsy Results

Here is my mother's eagerly anticipated kidney biopsy report, medical-speak and all, followed by some commentary by yours truly.

Patient: LJ
Age: 68
Date collected: 12-28-09

Specimen submitted: Kidney, biopsy


Amyloidosis (see Comment).
Interstitial Fibrosis, Moderate
Arteriosclerosis, Moderate

Comment: There is Lambda greater than Kappa staining but not significant enough to definitively state that this is AL Amyloidosis. The section stained for AA Amyloid is negative.

Clinical History:

This 68 year-old female has proteinuria.

Gross Description:
Received from Medical Center of Plano, Plano, TX, their number S09-5892, are two specimen bottles, one contains Formalin and the other Michel's fixative, labeled with the patient's name, LJ.

Received in Formalin are two pieces of tissue measuring 1.4 x 0.1 x 0.1 and 0.1 x 0.1 x 0.1 cm. Two ends are submitted for electron microscopy and the remainder of the tissue is submitted in its entirety for light microscopy.

Received in Michel's fixative are three pieces of tissue measuring 1.3 x 0.1 x 0.1 cm (bloody), 0.4 x 0.1 x 0.1 cm (bloody) and 0.3 x 0.1 x 0.1 cm. The specimen is submitted in its entirety for immunofluorescence microscopy.

Microscopic Description:

Sections of kidney show 28 glomeruli, none of which are globally sclerotic. There is diffuse replacement of the uretal architecture by expansive hyaline masses involving the mesangial region and extending into capillary loops. This is silver negative on Jones Silver stain. No hypercellularity is seen.

There is moderate tubular atrophy and dropout with interstitial fibrosis associated with mild chronic inflammation. The blood vessels show moderate intimal fibrosis. There is focal hyaline infiltration similar to the material seen in the glomeruli. Toluidine Blue stained sections show 12 glomeruli, one of which is globally sclerotic. There is diffuse toluidine blue positive material replacing the usual architecture in masses of mesangial expansion. Moderate interstitial fibrosis is seen. Congo Red and Thioflavin T stains for amyloid are positive.

Twenty-two glomeruli are available for examination, none of which are globally sclerotic. The sections are stained for IgG, IgM, IgA, C3, C4, C1q, Albumin, Fibrinogen, Kappa and Lambda light chains. Two plus amounts of Lambada (sic) light chain and one plus amounts of Kappa light chain are seen in a hazy pattern over all glomeruli. The remaining stains are essentially negative.

Two blocks and multiple Toluidine Blue stained sections are prepared. One glomerulus is examined ultrastructurally. Glomerular sections show marked mesangial matrix expansion by crossshatched fibrilla material having size characteristics of amyloid. Frequently this material extends out into the subendothelial space. Extensive effacement of foot processes is present. The capillary loops are of the usual thickness and do not contain electron dense deposits.

And then there are three black and white pictures of biopsy slides, I assume. The word "Amyloid" is in the middle of the three pictures, with a line going to each picture. There is a fourth picture with the caption "Normal glomerulus for Comparison." One of the biopsy photos is the closest match to the "normal" photo, and the biopsy photo has significantly more white spots than the normal one. I'm guessing the white stuff is the amyloid.

I think I only understand slightly more about this report than I understood back when I first looked at it in January of 2010. Later on I would learn more about Lambda and Kappa chains, and Congo Red staining. At the time, of course, we focused on "Diagnosis: Amyloidosis" and this very important sentence:

"There is Lambda greater than Kappa staining but not significant enough to definitively state that this is AL Amyloidosis."

So based on this report, they're saying she has amyloidosis but they can't definitively say it's AL Amyloidosis. So our journey begins as we try to determine what she really has, and what should we do about it.