Rapid Response Team

End-of-year greetings, loyal blog readers. Once again I am publishing the only post for the month with just a few hours to spare. Maybe that will change in 2015. Or maybe not.

Today's post will be another update on Mom, followed by the always exciting monthly blog status update for the end of November. The last update on Mom was in the October 30, 2014 blog post, and there really was not much to update at that time. She had been released from the hospital on September 25 after another GI bleeding episode, and not much had changed since then. She has been feeling very tired after most dialysis sessions, and often would need to take a very long nap in the middle of the day after coming home from dialysis since she goes early in the morning. But she did recently change her dialysis start time from the first time slot in the day to the last, which meant she could get some things done during the day before dialysis and then just come home and go to bed. That seems to be working better.

Since everything was going along smoothly in November it was time for something to happen. Sure enough, Mom had another GI bleeding episode and went to the emergency room the morning of Saturday, November 22. She had been nauseous on Thursday and first noticed some bleeding on Friday before going to dialysis Friday night. Her first hemoglobin measurement at the hospital was 7.8, which was down from 10.2 for the Monday blood draw at dialysis. I got to the hospital shortly after 2 PM to relieve my sister Laura who had taken Mom to the ER that morning, and Mom was admitted and arrived in her room at 2:40 PM. She did not think they were going to give her a blood transfusion based on what she was told in the ER, but one of the first nurses to come into her room mentioned something about a blood transfusion.

Shortly after 3 PM a doctor (Dr. W) from the same office as Mom's general physician came to the room and talked to us. She said it made sense to go ahead and give her a unit of blood because her hemoglobin had dropped so much since Monday which meant she was actively bleeding. Mom and I filled her in on the history of this GI bleeding issue and she asked Mom how she was feeling. Mom said that other than not having any energy she felt fine. No chest pain, no shortness of breath, etc. She asked Mom if she thought she needed oxygen and Mom said she didn't think so. Then they talked about diet and whether or not Mom could eat something. Dr. W looked over the hospital menu and told Mom to wait about 20 minutes to give her time to enter the orders regarding Mom's diet. I checked the time and it was 3:25 PM, so I planned on having Mom order some food around 4:00 PM.

I stayed in the room talking with Mom for awhile, figuring I would leave after Mom ordered her food since everything was stable at that point and there had not been any discussion of doing any tests this evening. At some point Mom mentioned she could tell she was having some heart palpitations, but she was not in any distress and I did not think much of it since I knew one of her medications was for heart palpitations. She was not connected to any monitoring equipment at this point, so I did not have any information about her heart rate. Then my sister Amy called me at 3:54 PM. I talked to her for a few minutes and then handed the phone to Mom so she could talk to Amy. Mom and Amy talked for a few minutes and then Mom handed the phone back to me. I was talking to Amy and telling her I was planning on leaving soon and didn't see any reason for her to come to the hospital tonight when I could see Mom was having trouble getting comfortable in bed. She told me she needed oxygen and to get off the phone.

I got off the phone with Amy and pressed the call button for the nurse. A nurse or technician walked into the room within a few seconds of me pressing the call button (probably just a coincidence) and I told her Mom needed oxygen. She turned right around, I assume to either get someone to do it or to get what she needed to put Mom on oxygen. Mom kept getting worse and said it felt similar to what she experienced on the cruise ship when she couldn't breathe because of the fluid buildup. Ok, we have a bona fide situation now. Then a nurse answered the call button and Mom told her she needed oxygen and she probably said something about heart palpitations or difficulty breathing, I don't remember exactly.

Mom's room was very close to the nurse's station, so a nurse entered the room rather quickly. I immediately told her Mom's symptoms had an acute onset, and with that and whatever she saw going on with Mom she quickly left the room and got some help. I heard someone asking about calling an RRT, which I later learned stands for Rapid Response Team. (It's not as serious as a "Code Blue.") I knew it was about to get crowded in the room, so I got the hospital phone and Mom's cell phone off her bed and stepped into the hallway. As people started filing into the room I heard over the intercom the call for RRT with Mom's room number, and someone entering the room said she was coming in with the crash cart. I also heard a nurse at the nurse's station trying to contact Dr. W.

While I was outside in the hallway I texted and called Amy to keep her informed the best I could, since I had to abruptly end our previous phone call. I could hear Mom talking to the nurses so I knew things weren't too bad. I did hear someone say her heart rate was in the 140s, which is considerably higher than her resting heart rate which I believe is typically in the 60s or 70s.

Sometime after 4:30 PM a nurse told me they were moving Mom from this floor (sixth) to the third floor, which is the telemetry floor. They can monitor her more closely there since those patients are always connected to heart monitors which I assume are monitored centrally, plus they can give more medications on that floor than they can on the sixth floor. So after they wheeled Mom out I gathered up her stuff and went down to that floor. She was settled in her new room just before 5:00 PM.

Mom's pulse rate was 137 bpm when I arrived in that room, and it gradually went down to the 120s as they gave her some medications to lower her blood pressure and pulse. Her blood pressure was fine but her pulse was still above 120 when I left shortly after 7:00 PM. She did order some food and had no difficulty eating it. Her hemoglobin at the 5:00 PM blood draw was 8.0, so it looked like that was stable and she might not need a blood transfusion after all.

Mom sent me a text the next morning (Sunday, November 23) saying her hemoglobin was down to 6.4 and she would be getting a blood transfusion. Her heart rate had finally come down to the 60s after she ate breakfast Sunday morning. She received another unit of blood Sunday night because her hemoglobin was at 6.5. It was up to 8.7 Monday night and still at 8.7 Tuesday morning, so it looked like things were holding. She had dialysis in the hospital Wednesday morning, November 26, and then she was discharged that evening (the day before Thanksgiving.)

Unfortunately we did not learn anything new during this hospital visit, since no imaging tests were done. One of the physician assistants from her gastroenterologist's office did talk to Mom about having another colonoscopy to try to locate the source of bleeding, but Mom questioned whether that was really necessary since the last one didn't show anything and the prep for colonoscopy is so difficult.

If she goes to the hospital again for this GI bleeding issue, our plan is to contact the offices of both her general physician and her gastroenterologist to let them know she is on the way to the emergency room, so hopefully those doctors can get involved as soon as possible and order whatever imaging tests might help locate the source of the bleeding. For the record, here are the dates of her emergency room visits and hospitalizations this year for GI bleeding:

May 2 (ER only, not admitted)
July 30 - August 3
September 18 - September 25
November 22 - November 26

In case you're curious, that was 89, 50 and 65 days between hospital visits, for an average of 68 days. So if nothing changes, the next episode of GI bleeding is due around January 29 of 2015.

Happy New Year!!


=====Monthly Blog Status Update===== 

As of November 30, 2014:

Total posts: 144 (1 in November)

Total pageviews: 21,500 (~800 in November)

Email subscribers: 12 (increased by 2)

Total number of countries that have viewed the blog: 101

No new countries viewed the blog in November.

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Dallas Support Group Meeting: November 8, 2014

Today's post will be about the Dallas amyloidosis support group meeting Mom and I attended on November 8, 2014. Attendance at this meeting totaled 32 people including the two guest speakers. I counted 17 patients and 13 caregivers/family members/friends. The breakdown by type was seven AL amyloidosis patients, five familial, four localized, and one person who was not typed yet but AA amyloidosis (secondary) was suspected. This was the first meeting for five of the patients, one of whom had ATTR and received a heart transplant just a month ago and seemed to be doing very well. In fact, a recurring theme for this meeting was that several patients are doing much better than they were a year or two ago, and people are surviving longer with amyloidosis. For instance, one of the attendees was an 11-year survivor (since diagnosis) who had a stem cell transplant in 2004. That kind of survival with AL amyloidosis used to be very rare.

The guest speakers were Dr. Zonder from Karmanos Cancer Institute in Detroit and Dr. K from The Binding Site, which is a company that develops blood tests used in the diagnosis and monitoring of AL amyloidosis (and many other similar diseases). Dr. K's presentation went over some of the terminology used in discussing AL amyloidosis, such as heavy chains, light chains, lambda and kappa, and how those are all related, what is important in testing as the disease is monitored, etc. Good info for AL patients, but not really applicable for AFib.

Dr. Zonder's presentation and discussion with patients included a lot of information about emerging therapies being developed and tested for all types of amyloidosis and how they worked. As I understand it, treatments can be divided into three groups:

1. Treatments that reduce or eliminate the production of the misfolded proteins. This would include stem cell transplants and chemotherapy for AL amyloidosis patients. In the case of fibrinogen amyloidosis, that is what a liver transplant does since the liver produces the mutated fibrinogen proteins.
2. Treatments that prevent the formation of amyloid deposits.
3. Treatments that dissolve amyloid deposits once they are formed so they can be eliminated by the body's normal processes. Examples of this would be CPHPC and doxycycline, both of which are in early clinical trials for all types of amyloidosis.

Dr. Zonder also said a few kind words to the group about my blog, since he became aware of it last year and has read some of it. That was nice to hear and it did make my head swell up just a little bit. We got to chat some after the meeting was over, too. I recently discovered that he also gave the blog a mention on his Twitter post later that day. So I guess I'll keep blogging (not that I was thinking about stopping any time soon.)

In the next post we may get to answer the question: What do they serve in the hospital on Thanksgiving Day?

=====Monthly Blog Status Update===== 

As of October 31, 2014:

Total posts: 143 (1 in October)

Total pageviews: 20,800 (~600 in September)

Email subscribers: 10 (no change)

Total number of countries that have viewed the blog: 101

No new countries viewed the blog in October.

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Article Reviews (1996 and 2007) - Early summary, and AFib in Italy

Hello again, loyal and patient blog readers. It is time for another monthly post, and this one will include not one, but two article reviews. As a bonus, at the end of the post you will find the always exciting monthly blog stats.

First, a quick Mom update. There really is not much to update, other than the fact that she has not been hospitalized again and things are more or less stable. It turns out this month was a good month for her to stay out of the hospital because the hospital she was in during her September visit, which is also the hospital closest to her internist and gastroenterologist, is none other than Texas Health Presbyterian in Dallas. You may recall that is the hospital where Thomas Eric Duncan, the first person diagnosed with ebola in the US, was treated. In fact, his first visit to that hospital was the same day Mom was released, which was Thursday, September 25. So yes, for awhile we were wondering what we would do if Mom's bleeding issues came back and she needed to go to the hospital. It was also a little weird watching Anderson Cooper live on CNN broadcasting from in front of that hospital, knowing I could drive to that spot myself in about 20 minutes. Fortunately we did not have to make any hospital decisions and the local ebola drama seems to be fading away.

Today I am going out of sequence with the article reviews to catch up on some older ones I acquired after starting to review articles. Both of these reviews will be rather short.

Title: Five Kindreds With Variant Fibrinogen Presenting Renal Amyloidosis (1)

Authors: T. Uemichi, JJ Liepnieks, MT Hull, MA Gertz, MD Benson (Indiana University, Indianapolis, IN; RLR VA Medical Center, Indianapolis, IN; Mayo Clinic, Rochester, MN)

Journal: Neuromuscular Disorders (1996)

This article appeared in a supplement to the journal "Neuromuscular Disorders" and is just one paragraph (ten sentences.)  It briefly describes the three mutations known at that time to cause fibrinogen amyloidosis. Those mutations were:

• Arg554Leu (one kindred with three affected members)
• Glu526Val (three kindreds with a total of 11 affected members)
• 4904DelG (one kindred with two affected members)

So that is where things stood in 1996, three years after the first article about a fibrinogen amyloidosis mutation was published. Three mutations, five kindreds, and 15 people known to be affected. Although the Glu526Val mutation was not the first one discovered, it did take the lead rather quickly. The next article will give us a sense of how prevalent AFib was in Italy about ten years later.

Title: Renal involvement in systemic amyloidosis  --  an Italian retrospective study on epidemiological and clinical data at diagnosis (2)

Authors: Bergesio, Ciciana, Santostefano, Brugnana, Manganaro, Paladini, Di Palma, Gallo, Tosi, Salvadori (Italy)

Journal: Nephrology Dialysis Transplantation (2007)

This is a much longer article (eight and a half pages, plus one page of references). But my review, which is actually just a summary of the part relevant to us AFibbers, will be shorter than the abstract. This article reports on a study of 373 patients diagnosed with some form of systemic amyloidosis (with renal involvement) in Italy between January 1995 and December 2000. Of those 373 patients, only six were diagnosed with a hereditary form of amyloidosis, and only one of those six patients had a fibrinogen mutation (Glu526Val). There really isn't enough data there to draw any conclusions regarding the prevalence of fibrinogen amyloidosis in Italy, other than to say it was rarely diagnosed before 2007. See, I told you that would be a short review.  ;-)

No, there was not much in this month's blog post but hopefully I can do some more cleanup of the article reviews and get those closer to completion in the next few months. The next blog post should be a report on the Dallas Support Group meeting in November.


=====Monthly Blog Status Update===== 

As of September 30, 2014:

Total posts: 142 (1 in September)

Total pageviews: 20,100 (~1000 in September)

Email subscribers: 10 (no change)

Total number of countries that have viewed the blog: 101

1 new country viewed the blog in September:

British Indian Ocean Territory**

**The British Indian Ocean Territory is a group of very small islands in the Indian Ocean. However, since the two-letter country code is io (as in input/output), that code is often used by technology companies.

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Citations

(1) Uemichi T, Liepnieks JJ, Hull MT, Gertz MA, Benson MD. Five kindreds with variant fibrinogen presenting renal amyloidosis. Neuromuscular Disorders. 1996;6, Supplement 1(0):S35.

(2) Bergesio F, Ciciani AM, Santostefano M, et al. Renal involvement in systemic amyloidosis--an Italian retrospective study on epidemiological and clinical data at diagnosis. Nephrol Dial Transplant. 2007;22(6):1608-1618.