Quick, quick, turn around... in more ways than one.

Last week, as J and I walked into Reagan National to fly back to Boston, I realized I had left my gloves on the NIH shuttle.

Bummer!

I decided it was a lost cause. Literally and figuratively. Oh well.

The day after we returned from Bethesda, J decided to try, on a whim, to call the NIH transportation desk to see if my gloves were found. And, surprisingly, they were still in the shuttle! I contacted the myeloma research nurse who said she would send them to me. Annnnnd... here they are.

Super quick turn around. Very nice people they have down there in Bethesda.


And, most importantly, I have the preliminary results of my bone marrow biopsy from last week. Another quick turn around.  Drum roll please...


5-10% plasma cells!


!!!!!!!!!!!!!


So, let's recap. I have had three, THREE, bone marrow biopsies since July 2012. Every time... a different reading. The results have been 10%, 10-15%, and now 5-10% plasma cells.


Stupid, stupid, stupid, patchy cells.


But. I'm thrilled, obviously.


Third time's a charm? :)

Bone Marrow Biopsy & CT Scan: NIH Day Two

Needless to say I didn't get a lot of sleep the night after the possibly "abnormal" area on my arm was discovered. The NIH team requested I not eat after midnight in case they were able to get me in for a morning PET/CT. Didn't have much of an appetite anyway! To say I had knots in my stomach is a bit of an understatement.

In the morning I received a call from the research nurse who said I could go for a CT of my arm after my bone marrow biopsy and aspiration that afternoon. There were no available slots for a PET/CT that day. The CT of my arm would only take about 20 minutes. Sweet. PET/CTs, I know from experience take at least 2 hours -  one hour for the tracer to settle, and one hour for the actual scan. I was happy with the shorter test!

J and I headed to the procedure suite for my bone marrow biopsy. I was greeted by a nurse (also named Elizabeth!) who took my temperature by swiping this wand over my forehead then back behind my ear. That was a first. Again - NORMAL temperature. She also took my blood pressure and pulse. I met the research fellow who would be performing the biopsy and aspiration. She introduced herself, "Hi, I'm Dr. so-and-so. I'm actually from the myeloma research team here at NIH. I've heard a lot about you."

Hmmm... What?

Apparently the myeloma team had discussed my case. She was going to do the biopsy on my left side since my biopsy in December was on my right. I mentioned to her how HORRIBLE my first biopsy was with the shooting pain down my leg. She said she would be very careful and that she had done many biopsies before.

I asked her how many aspirates she would be taking (I've have 4 vials for my other biopsies at Dana-Farber) and she said only ONE. Score. The aspirates are the most painful, in my opinion. The only difference between my biopsy at NIH and my biopsies at Dana-Farber was that there was a nurse, tech, and doctor, while at Dana-Farber I had just a PA and a tech with me. They also put all this crazy blue antiseptic all over my back. The procedure was fine, I feel like I'm becoming a pro at letting people suck out my marrow and bone. No big deal any more. This time the most painful part was when she injected the lidocaine - felt like a very long bee sting.

After the research fellow was finished I had to lay down for 15 minutes (never had to do that before - at DF they just sent me on my way) and I had to get a needle stick in my finger. Haven't had one of those since I was a little kid! Not really sure what that blood sample was for. The tech let me look at the biopsy - the piece of bone was tiny, smaller than my biopsies at Dana-Farber. Apparently they need less tissue at NIH? One aspirate vs. four and from my recollection a much smaller piece of bone.

Hope it was a good one. :)

After the biopsy was the CT scan of my arm. Sort of much ado about nothing. I had to wait forever in the second waiting room. Had a funny conversation with an older gentleman with the best southern accent I've ever heard. He kept saying how "cheeeeellllly" he was with his southern drawl. He was only wearing a tiny robe and slippers. Heh.

When I finally got called for the scan I was all set up but then apparently the computer froze while I was laying in this sort of weird position - almost part way off the table so my arm was more centered for the scan. After they "rebooted" the actual scan was literally 5 minutes I'm pretty sure. Probably less. Hopefully less radiation since it was just my arm? I hope so. After the CT we went up to the 12th floor clinic to meet with Dr. L, Dr. MR, and the research nurse.

So J and I were in the waiting room at the clinic when Dr. L, Dr. MR, and the research nurse came through the outside doors. I anxiously stared at them, trying to read their faces. Dr. L came over to me, smiled and whispered, "It's fine! It's fine!" The other doctor said, "Oh geez, we do respect privacy here but it's like Christmas for him, he can't wait!" HA. Kind of funny. Honestly, Dr. L really is a kind and compassionate doctor. I totally appreciate him not making me "sweat it out" and wait to hear the news until I got into the room!

They took us into a room and said the preliminary read of the CT scan was back... Apparently there is an "abnormality" on the inside of my left humerus, but it's not a lytic lesion (which I guess is on the outside of the bone). They said it would be very rare to have just one lytic lesion anyway. From what I understand, sometimes bones inside are not exactly smooth or even and my left humerus has an indentation on the inside.

The drawing of the bone on the right is what my humerus looks like inside.



The picture I posted above: the drawing of the bone on the left that has the labels of cortex and bone marrow and shows that little line coming out - that's apparently what a lytic lesion looks like. The bone on the right is what my humerus looks like. See that indentation on the inside? Yeah. The bone drawing below the other two is what my humerus looks like on the CT scan - it's a different view -  the inside part of the bone is uneven. The arrow on the top would be what a lytic lesion would look like, but mine does not have. Mine just has the unevenness inside, not outside.

The NIH team wants me to have the films from the skeletal survey and CT scan sent to Dr. R and have him compare my arm on the PET/CT from August to the CT of my arm from today. But overall, they aren't too concerned and it's just something to watch and see if it develops into anything. In the email response from Dr. R when I asked him about the PET/CT vs. CT of my arm, he said for me to "send best wishes to Dr. L" so I passed on the message - Dr. L said the same. So funny all these doctors know each other. I guess the myeloma community is pretty small.

Dr. L spoke about the gene expression profiling they are working on - apparently they have the profiles of 50 smoldering myeloma patients so far. He spoke about how important the bone marrow biopsy at the beginning of the study (now) is and then the importance of me getting a bone marrow biopsy at NIH again in 5 years. Basically he eluded to the fact that if I sign up to be in the study, these two bone marrows are really important. If I am going to be in the study, I need to be committed to coming back and, at the end, have another biopsy. Duly noted! 

Don't worry Dr. L, I won't quit part way through your study and mess up your results!

Dr. L asked what Dr. R was planning to "do" with me. He said the standard of care is to "watch and wait" which I know. From this point forward I should just be monitored and assess my "clinical symptoms" as they come. Super.

This is actually an interview with Dr. L from about a year ago: Smoldering Myeloma: What Do The Latest Research Findings Mean? A Discussion With Dr. Ola Landgren

I know that treatment of patients with SMM is still in it's very, very, early stages and is definitely not the "standard of care." I, for one, would like to hold off on treatment as long as possible as people can smolder for years and years. I don't think I'm considered high risk right now anyway. However, there is something to be said for waiting too long to start treatment. If you have bone damage and your kidneys are failing - you've waited too long. The decision of when to start treatment seems very difficult to determine. It's a fine line to walk, especially for me being on the younger side; any medication I start taking would most likely be for the rest of my life because at this time myeloma is incurable.

In closing, I share with you this quote from the interview posted above with Dr. L:

Our ultimate goal is to find a cure for myeloma. It is an exciting time to develop new strategies for myeloma and its precursor states!

For sure. I signed the consent form for the Natural History Study and will return to the National Institute of Health / National Cancer Institute in 6 months.

Dr. MR, will call me with the results of my bone marrow biopsy and m-spike next week.

Abnormal Skeletal Survey & Perfect Blood Work: NIH Day One

J and I arrived at NIH bright and early for day one of appointments and tests. We were in building 10, the NIH Clinical Center, which is HUGE.

Really bad picture of sign inside!

According to my appointment letter, I was supposed to meet a research nurse at the admissions desk in the Hatfield Building. When I got there I had to do a bunch of admissions paperwork, get my picture taken, etc. etc. etc.

I met with the research nurse and the first thing she said to me was, "Hi, it's so nice to meet you. I'm sure you already know this but we're really interested in you because of your age and we're so glad you're here."

Heh.

We chatted for a little while and she had me sign a consent form entitled, "Eligibility Screening and Tissue Procurement for the National Cancer Institute (NCI) Center for Cancer Research (CCR) and Clinical Research Protocols."

Now that's a mouthful.

The research nurse showed J and me where phlebotomy and radiology was located, and where we could get our ID badges. Having the ID badges would make entrance into NIH a little quicker in the future - if you have a badge you don't have to go through the metal detector at the security check point every time you are trying to enter the campus. Anyway, the plan was to get blood work, then a skeletal survey, then head up to the 12th floor clinic for an appointment. No EKG! I was kind of disappointed to be honest. One test I've never had before!

So I got my blood draw. I counted 15+ vials! I think that might be a record for me. I also had to give a urine sample. Good times.

After blood work I had to have the skeletal survey. The wait was REALLY long (as I had to wait for a result of a negative pregnancy test - heh) and I was worried I was going to miss my appointment in the clinic. The receptionist called a few times and the doctors did want me to get the skeletal survey prior to going to the appointment. So I waited. And waited. And waited. As you'll read later, it's a good thing I had the skeletal survey.

The x-ray tech guy said that obtaining all the images could take up to an hour. I don't remember my skeletal survey in 2009 taking that long. Mildly panicked, I told him I was concerned I was already almost TWO hours late for my appointment and he said he would move as quickly as he could. I had to wear a paper shirt and pants that were about 10 sizes too big and get into all these crazy positions, standing and laying. The x-ray guy kept saying things like, "Hmmm where are your knees in there?" and moving me all around. His hair was longer than mine and he had it in a very blond pony tail.

After the skeletal survey we headed up to the clinic for my appointment. I had my vital signs taken pretty quickly. The nurse seemed shocked with my diagnosis and kept saying "Hmmmmm" and looked at me all concerned as I gave my history. Vital signs were fine, she took my temperature in my ear (it was NORMAL - take that fevers!) and brought J and I into a room.

A few minutes later the research fellow came in.  Great news, all my blood work from earlier in the day was NORMAL. No flags at all. At least that is what he told me - he said he barely ever sees no flags. Yup. That's right. Perfect blood work. My IgG that had been climbing over the last couple months (it was 1520 last month) was down to 1340. So that was good. AND. My hemoglobin was 13.2! Whoa! It hasn't been that high in years (it was 11.3-11.8 last month).

So, research fellow asked my history, did a physical exam, and then asked me if I had any questions.

Uh, of course I did. I think he was amused at the amount and extent of questions that I did have. He was very nice and let me ask tons and tons of questions. Some of them, in particular ones that were related to treatment options he responded with, "You're not there yet." Heh. This is true. But. Still need to be prepared. When I asked about pregnancy he said there are only about 20 case studies AT ALL, EVER, in regards to MGUS/SMM/MM and pregnancy. This I knew.

This research fellow also commented on how I am considered young for the disease, but he did say there is a boy who is THIRTEEN with symptomatic multiple myeloma who just had a stem cell transplant at NIH. Yikes. Thirteen... :(

DISCLAIMER AS YOU READ ON: I had long conversations with this research fellow, Dr. OL, Dr. MR, and the other doctors. I have several pages of notes that look like this.

Bad note-taker.

Yeah. Really not very organized. I am trying my best from memory and referencing these notes to recall all the information discussed with the myeloma team. Please take everything I am writing with a grain of salt as it may not be entirely accurate! Really need to get a voice recorder for appointments like these. :)

This research fellow talked about the Natural History Study and the clinical trial they have for patients with high-risk SMM. He also spoke about the differing risk stratification models (Mayo vs. Spanish) and how there is such a discrepancy between the two. This article, Mayo, PETHEMA, And The Risk Of Progression In Smoldering Myeloma: More Disagreement Than Agreement explains this much better than I ever could. The research fellow said some patients are not willing to "watch and wait" and the NIH has this trial, Carfilzomib, Lenalidomide, and Dexamethasone for Smoldering Multiple Myeloma to see if early treatment will improve outcome for these high risk smoldering myeloma patients. These researchers believe that this treatment protocol may improve survival for these high risk patients. He said that everyone with myeloma has an altered gene sequence - the cells start to accumulate these mutations over time, the damage is irreversible, and chemo won't kill the cells because the mutations will keep coming back. They are hypothesizing that coming at these cells early with treatment may improve survival.

Well. Honestly, this may or may not be exactly what he said. You've seen my notes. They make no sense. Remember my disclaimer?  God help me.

I asked why all MGUS patients don't get a bone marrow biopsy. I didn't have one when I was first diagnosed and having had one in July has been a total game changer for me. I know according to the various support groups that I am a member of that many other MGUS patients have not had one as well. He said, like I've heard from other sources, that it is personal preference of the physician and not everyone is screened with a biopsy. It's really "hit or miss". The m-protein is really an indirect measurement of the plasma cells - some plasma cells secrete a lot and some only a little (like me). All MGUS and SMM patients that go to NIH get a bone marrow biopsy.

After I asked many, many questions the research fellow left the room and then returned with Dr. OL, Dr. MR, and four other research fellows and/or doctors. I'm not really sure who they all were. It was kind of lot of people. Seven total including the research nurse. Nine people including J and me - crammed into an appointment room.

The first thing I asked about was pregnancy, which is really my biggest concern at this time. Dr. MR said that no one would really absolutely tell me that I shouldn't have kids as there is no research that definitively says pregnancy is contraindicated in someone with MGUS or SMM. Dr. OL spoke about a research  study he was involved in in Europe where they studied women with all different kinds of cancers and compared women diagnosed with cancer who earlier in life had children and those that did not have children. He said there was no differences in women with cancer who had children and those that did not. They actually had a woman in the Natural History Study who is 35 and recently had a baby and I guess is doing "okay." Hmm. Ok? Not sure I like ok.

One thing that did suck, for lack of a better word, was that they said I definitely have smoldering myeloma based on my two biopsies of 10-15% and 10% plasma cells despite the fact that my m-spike at the time of my biopsies were .34 g/dl and .42 g/dl respectively. The team said they have seen plenty of people, like me, with low m-spikes and 10% or greater plasma cells which meets the definition of smoldering myeloma, not MGUS. The criteria for MGUS is less than 10% plasma cells and monoclonal protein less than 3.0 g/dl.  According to these physicians the m-protein really isn’t always a direct measure of the number of plasma cells because some plasma cells secrete a lot of protein and some do not. And some plasma cells don’t secrete AT ALL like in the nonsecretory patients. The number of plasma cells and the features of the plasma cells is what is really important.

Everyone was super nice and we had some good conversations. It was a little awkward having six different doctors staring at me! Dr. OL mentioned my chromosome abnormalities (13, t(11, 14) and 14q32(IGH sep)) and he said really they are just numbers and letters and not to get too bent of shape about them as scientists are not 100% clear what they mean. Obviously, I'm paraphrasing here.

So the appointment was winding down and I was all set to sign on for the Natural History Study. One of the research fellows joked that we would need to bring back New England clam chowder next time we are at the NIH. Everything was all happy-jolly. However, no one had reviewed my skeletal survey. A negative skeletal survey is required in order to sign on to the study.

As Dr. MR continued chatting with me I saw Dr. OL and one of the research fellows start to review my images on the computer in the room. Dr. OL started pointing at one of the images of my arm. They all started staring at the image and whispering together. Dr. MR was still talking and I think trying to distract me while they are reviewing the images.

I could clearly see them pointing to the image of my arm. In my mind I was thinking, what's wrong with my arm?? What's wrong with my arm??

J and I were asked to step into the waiting room as they were going to review my skeletal survey before I signed on to the study. After about 10 minutes all six of the doctors came walking out. I assumed they were going to say, "Everything is good to go! Sign here."

Unfortunately they did not. They walked right past me!

Dr. MR looked back and called to me, "Oh we're just going to take a look at the images with the radiologist."

Whaaat? This can't be good. Something is wrong with my arm.

About 10 more minutes go by and they all come back through the doorway. I again, expect them to say, "Everything is good to go! Sign here." Unfortunately, they asked me to go back into a room. This can't be good.

I start having flashbacks to when I was supposed to get a call from Dr. R saying, "Plasma cells are below 5% - good to go!" and instead I had to go back to Dana-Farber to discuss the results of my bone marrow biopsy.

We got into the appointment room and Dr. MR said that apparently there is an abnormal area on the image of my upper left arm.

No way.

I joke, "Are you sure it's not just my bulging bicep?"

Heh, it's not.

Dr. MR said it could be nothing, or it could be something - possibly a lytic lesion. Dr. OL said he wanted me to have either a full PET/CT tomorrow or at least a CT of my arm as this area needs to be investigated further. At this point it was after 5:30PM and they had no way to find out what the availablity would be for the next day. They apologized that this finding is probably causing me anxiety.

Uh. Yeah. It is.

J and I left. I was freaking out inside. I've heard skeletal surveys are worthless because they don't really show anything. How could the skeletal survey possibly show an abnormality on my arm? How could a lytic lesion form just 6 months after an entirely negative PET/CT?

I was also worried about more radiation exposure with the PET/CT or CT after already having had a PET/CT in August and then the skeletal survey as well.

When we got back to our hotel I emailed Dr. R to get his opinion of the situation. He suggested just the CT of my arm since the recent PET/CT was negative. I also emailed the NIH team. Dr. OL responded with this:



Dear Elizabeth,

Thanks for kind email.

You are asking important questions. Here are my answers:
1. Changes in bone (and other tissues/organs) can theoretically develop during short periods of time. That is the reason we want to check and make sure we are not missing anything. We are always very careful.

2. Radiation exposure is always something we consider in our daily decision making. Here are some facts: to do a skeletal survey is approx. 1 REM. To do a whole-body PET/CT is approx. 2 REM. To do a standard CT of the chest area (incl the arm) is approx. 2 REM. The reason the whole-body PET/CT and the chest CT are both 2 REM (despite whole-body vs part of the body) is because the whole-body PET/CT is a lower exposure method. CT is a better methods to assess bone than MRI. MRI is better to assess soft tissue (such as bone marrow). Bottom line: I suggest either PET/CT (whole-body), or, alt #2, a CT of the arm (if the PET/CT waiting line is too long).

Tomorrow, we will have it all sorted out. I am sorry for all anxiety due to this. I think a careful work-up is the only way to move forward from here.

Best wishes,

Ola




You know that song, "Mo money, Mo problems" by the the Notorious B.I.G.?

Well, my theme song at that moment was, "Mo DOCTORS, Mo PROBLEMS".

The more DOCTORS we come across... the more problems we see....

Yup. It was a very long night.

Travel Observations

J and I don't travel a lot. We don't have the best sense of direction. Whenever we do travel there always seems to be a lot of amusing people-watching and mishaps.

We made it to the Logan Express bus with plenty of time on Monday. When we got on the bus this is the conversation between two interesting older individuals sitting in the seat in front of us that we overheard:


I need two senior shuttle tickets!  I'm 66, does that count as a senior?

I actually slept really well last night. I just got up once for my peanut butter treat.

Oh good!

I bet Fluffy is upstairs right now. You know how he always comes down with that yellow foam in his claws? What is that?

Yellow foam from behind the piano.

Ohhhh.

It's really expensive foam.


After hearing this there are so many unanswered questions. Does 66 count as a senior?  What exactly is a peanut butter treat? Who is Fluffy? What is this yellow foam for and just how expensive is it? After hearing this conversation and pondering these questions, I silently laughed so hard I almost cried.

When we got the airport we were a little confused because the US Airways desk was labeled "Shuttle". Now. When I think of a shuttle I think of a van or a bus. Apparently they call our flight a "shuttle" because it's such a short trip from Boston to Washington D.C. Good to know.

Somehow after picking up our boarding passes J lost his. This was discovered AS we were starting to get on the plane. Somehow J and I always manage to have our seating assignments apart. Not sure if he plans this secretly? :) We got on the plane and he accidentally sat in the wrong seat and was very politely asked to move. Heh. Strike two!

During our flight a model-like girl was sitting next to me on the plane. She was diligently writing in a notebook on a list entitled, "Goals for 2013." I tried not to look, but I really couldn't help myself. Her goals include but are not limited to...

Maintain 125 pounds. (model-like girl was almost 6 feet tall)
Save $50,000 cash (apparently you can do this if you have Louis Vuitton luggage)
Run marathon #4 (cool)
Start blog: Miera Knows Best (totally going to google this blog in the future and see if she started it)


Our flight was fine, model-like girl was very entertaining, and she gave me her bag of mini-pretzels. Carbs must not be part of her diet to maintain 125 pounds. We made it to Reagan National Airport. We were going via shuttle to the NIH and then were planning to pick up a different shuttle to get to our hotel. Now, these are actual shuttles, not planes. Heh.We had a quick lunch at TGI Fridays at the airport and made it to the shuttle with plenty of time. The trip from Reagan to NIH was pretty quick, less than 30 minutes as there was no traffic, probably because of President's Day. I was able to snap a couple bad pictures of some good sights as we drove along...

We arrived at NIH for the sole purpose of switching shuttles to make it to our hotel. NIH is hardcore. It's huge and completely gated. In order to enter the grounds you have to go through a security checkpoint. At this checkpoint they inspect all vehicles and everyone has to go through a metal detector and obtain a badge. Kind of intense. I, of course, set off the metal detector about 7 times, removing an article of clothing or jewelry each time, before realizing my really cool teacher Dansko clogs must have metal in them. The security guard just laughed at me.

We arrived at our hotel, checked in, went to the fitness center to workout for a little while. After, we took the shuttle downtown - Bethesda is pretty cute! We went to a restaurant called American Tap Room and had a fun little dinner. It was pretty nice inside and the menus actually glowed when you opened them!

 You can't really tell by the picture that the menus is glowing, bummer. I've never seen anything like it in a restaurant before.

Fevers? What Fevers?

In 2009 I was experiencing fevers, joint pain, headaches, and fatigue. I was put through an extensive amount of tests through my PCP as well as a rheumatologist. The only significant finding was a very teeny tiny m-spike (.19 g/dl at the time) and I was diagnosed with MGUS. I continued to experience the fevers and intermittent joint pain. A few years after my MGUS diagnosis I was again worked up by a different rhuematolgist and the results continued to be unrevealing with the exception of elevated inflammatory markers (high sed rate and CRP).

Last spring, around the time of my biopsy that was suggestive of smoldering myeloma, I started taking the time to record all my symptoms and fevers. I recorded that I had experienced fevers in May for about 3 days (and was hospitalized for a skin infection), 3 days of fever in June, about 8 days of fever at the end of July into August, 6 days of fever in October, 8 days of fever in November, and then I had the flu in December. I know that I had experienced a similar pattern during the previous years as well and assumed that they were either due to MGUS or my nonspecific diagnosis of inflammatory joint disease (although this diagnosis has since been revoked!).

After discussing these strange fevers with Dr R, he suggested I see an infectious disease specialist to determined if these continued intermittent fevers could be due to something other than my smoldering myeloma. As I outlined in previous blog posts, I saw Dr. M, infectious disease specialist, and he speculated that I may have a periodic fever syndrome. I am still waiting for the results of the genetic testing for these syndromes. However, in the mean time, Dr. M requested that I take my temperature every morning when I wake up and in the evening between 7-8PM. For about 6 weeks I have recorded my temperature and I am supposed to go and get blood work (CBC with differential, full chemistries - electrolytes and liver function tests, a CRP, a ferritin and triglyceride levels) when I am having a fever.

And... you know what? I haven't had a fever since I started recording my morning and evening temperatures. I feel like I don't want to say this too loud as I might jinx myself. Dr. M has cured me! Praise the Lord! HA. Well, not exactly. There was one night a few weeks ago I had a temperature that was 100.5 but it was gone by the next morning. My temperature in the evening is rarely 98.6 or "normal" but it hasn't been above 100.5 so far. I have had some night sweats that I have been recording as well. But. Good news - no fevers! Dr. M gave me a journal article that described the different types of periodic fever syndrome and to be honest I really don't have many of the symptoms described as they include abdominal pain, vomiting, conjunctivitis, and rashes. I am still recording my temperature and plan to get the blood work if/when I have a fever.

I received the MD note from my appointment a month ago with Dr. R. Interesting to read his perspective on this whole fever situation.




Ms. Elizabeth ***** is seen today in followup. She is accompanied here today by her very supportive husband, and her sister. Since last seen, Elizabeth has continued to do really quite well. Excitingly, review of her most recent bone marrow aspiration and biopsy, and staining for estrogen and progesterone receptors have been entirely negative. This has provided considerable reassurance to everyone that it would be reasonable for her to plan moving forward with her family in the future, and look to monitoring her smoldering myeloma accordingly. It should be noted that right now at the present time with her bone marrow plasmacytosis being ten percent, she does meet criteria for smoldering disease. Conversely, however, she does appear to have an atypical fever syndrome, but may in fact be a variant of Hibernian fever. She has been seen in this regard by my colleague, Dr. M. In brief, please refer to Dr. M's superb consult summary from her recent visits with him. She is presently under evaluation with a number of laboratory tests for this rare condition, as a possible explanation of her fevers.

‎Most importantly, Hibernian fever may also be potentially driving some of her plasmacytosis. This may in fact mean that she does generally have monoclonal gammopathy of uncertain significance by virtue of actual plasma cell count and her M-protein measurements. Thus, to some degree, the plasmacytosis in her marrow may over represent the actual original clonal population by virtue of a reactive process in the context of the Hibernian fever. However, this all remains to be further elucidated. It is possible that she does not have Hibernian fever, and that she does in fact have smoldering myeloma characterized by a febrile syndrome, and this of course will be an important diagnosis to establish. Whilst her infectious problems are being further elucidated and her febrile picture being better understood, Dr. M has recommended that she avoid pregnancy if possible until he is clear on her febrile picture.

‎Most importantly, Elizabeth's workup for infectious disease has been entirely negative. No significant or atypical viral, fungal, or bacterial processes have been identified.

‎In herself, Elizabeth is reassured by all of the above. She is much more encouraged by the more favorable outlook of her overall picture in this context. She is without new cardiovascular, pulmonary, gastrointestinal, neurological, soft tissue, cutaneous, or other complaints. She has noticed some mild fatigue recently, but that has been in association with her current workload.

Elizabeth is clinically stable, and doing well overall. The expert input of Dr. M has been particularly useful in identifying potential etiologies for her fever. I am encouraged that her plasmacytosis is non-hormone receptor positive, which makes the decision around family planning that much easier for her and her husband. In this context, the additional workup from the Infectious Disease team will be vital, as we plan for the future. Therefore, recommended to Elizabeth we continue with a policy of careful observation. We will await to hear more from Francisco, and indeed were able to connect with him today by email between myself, him, and Elizabeth. She will return to see us in the clinic in the next several months. She will be seen in the interim also by Dr. M. She knows to call should she have outstanding issues or concerns.




Yup. It's all good. Bethesda tomorrow!

Valentine's Day Fortune

I am not a superstitious person by any stretch of the imagination, nor do I believe in psychics or fortunes.

However, last night J and I went out to celebrate a very casual Valentine's Day at PF Changs (bad, bad, bad... but so, so, good!). Normally my fortune cookies have messages that are pretty much meaningless. But last night my fortune said:

 

You will get what your heart desires.

I trust that God has a plan for us and I am very thankful for what I have now. But, MAYBE just MAYBE in 2013 I will get what my heart desires.

We'll just have to wait and see... :)

Roooad Trip!

Next week my husband and I are headed to Bethesda, Maryland. Road trip! Meh, unfortunately this is not a super "fun" road trip, per se. I am going to the National Institute of Health / National Cancer Institute to enroll in the Natural History Study of Monoclonal Gammopathy of Undetermined Significance (MGUS) and Smoldering Myeloma (SMM).

This is a "screening" visit to see if I am eligible for this particular research study. During the two days spent at the NIH there is kind of a lot going on. I sent all my test results from the last 3.5 years at Dana-Farber as a first step, but they do all of their own testing there as well. During the visit I will have...


Blood tests - to evaluate blood counts, liver and kidney function, presence of infectious diseases, presence of specific protein markers, and presences of abnormalities in a variety of genes.

Urine tests - to assess the presence of abnormal proteins (NOT a 24 hour urine. Thank you, Lord.)

Bone marrow aspiration and biopsy - my third BMB since July. Ouch, ouch, ouch...

Skeletal survey - my second, last one was in August of 2009

Electrocardiogram (EKG) - to evaluate heart rate and rhythm by measuring electrical impulses from the heart through electrodes that are placed on the skin. Never had one before, should be interesting!

I will also meet with Dr. OL, Dr. MR, and/or other members of the myeloma team.


I have to say, I truly can't think of a better way to spend my February vacation week! HA. Not. In all seriousness, I hope it will be a good experience and I will get the opportunity to hear these doctors' opinions of my current status and their recommendations for next steps moving forward. I am also looking forward to seeing the results of this third bone marrow biopsy. I wonder if I will still have 10% plasma cells. Or maybe they will hit a spot with a lower concentration! I guess, higher result is a possibility too, but I hope that won't be the case. I'm also interested to see what my m-spike will be since I am not going back to Dana-Farber until April.

If all goes well and I am accepted into the study - I'm assuming I will be - I will return to NIH in 6 months and then annually for 5 years or until the myeloma becomes active, which would then disqualify me from participating. This study is to investigate risk factors related to the progression of MGUS and smoldering myeloma to multiple myeloma, to help better understand the disease, and to develop tests that may be useful as prognostic factors. I am very excited to contribute.

J and I are trying to find fun things to do in the Bethesda/DC area to turn this medically driven trip into a little mini-vacation. We've never been to Bethesda and we're not sure what's around there, but hopefully the trip will be fun! Minus the bone marrow biopsy, of course. :)

Classical flash mob at Dana-Farber Cancer Institute

Boston's Longwood Symphony Orchestra performs at Dana-Farber. I love flash mobs and this is pretty cool! Must have been awesome to be there when it happened. :)

Waiting, waiting, waiting...

Waiting is hard. But, that's life, right? I am currently waiting for the results of the genetic testing for periodic fever syndromes. I just checked the consent form and apparently the turn around for the results can be up to 8-10 weeks. That means, if the results were sent around January 10 or 11 when I had my appointment, they probably would have made it to Maryland by maybe January 14 or so, which means...I might not get the results until almost April. Ugh. I thought waiting a week for bone marrow biopsy results was too long!

I have the note from my appointment a couple week ago with Dr. M, my infectious disease specialist. I feel like I understand a little more what he is thinking for me. Here is the summary at the end...



Mrs. M is a 29-year-old woman who has been experiencing intermittent fevers. She has associated arthalgias, but no overt arthritis has ever been documented. Evaluations of the fevers noted the detection of a IgG lambda spike that has been slowly increasing over this time. Multiple evaluations for common, seasonal, and uncommon infections as well as for rheumatologic diseases has been unrevealing. She has no constitutional symptoms of rashes during episodes and has not lost weight significantly.

The pattern she reports is unlikely to be caused by any infectious disease agent, but she gives a good story for experiencing a periodic fever syndrome. Although most of them are genetic diseases, they can present in adulthood. She has no history of recurrent serositis or ethnic background to suspect familial Mediterranean fever, but I wonder if she may have Hibernian Fever/TRAPS, relatively frequent in patients of Irish/English ascent or hyper IgD syndrome (MVK deficiency), or other related disorder.

In her particular case, it is hard to ignore the presence of the IgG lambda paraprotein.. She could indeed have 2 separate diseases. Alternatively, I wonder even though the bone marrow biopsy results are suggestive of smoldering myeloma, she may have a reactive MGUS in response to her periodic fevers and diagnosis and treatment of the appropriate illness may resolve the MGUS, as we have seen in patients with chronic infections, such as HIV. Another possibility is that the paraprotein is binding or modifying other proteins or receptors involved in temperature regulation and that it may actually be triggering the periodic fevers.

We discussed all of the above with her and her husband. We did the labs noted above. She's HIV negative. In addition, we sent a blood sample to genedx to test for common periodic fever syndrome mutations. I also offered them to contact Dr. K at NIH, who would be in the best position to study her case in case her genedx testing is negative. She agreed for me to contact him.



Waiting... Also, it feels a bit strange to not be going back to Dana-Farber until April. I do have our trip to the National Institute of Health for the Natural History Study of MGUS and SMM coming up in a couple weeks, however. After that I will hopefully be on a little mini-vacation from doctors! Woohoo!

I love free money!

This is what I got in the mail today...

 

 

Thank you Partners HealthCare! I WILL fill out your survey pertaining to my experience using Patient Gateway (patient portal where I can check my lab results) and gladly take your $5.00...or as you say in the letter, "We have enclosed a token of our appreciation as our way of saying thanks to you for completing the survey." You're welcome!

Yes, I am that cheap and that poor to get excited over 5 bucks. Ridiculous.

I've actually completed this survey a number of times. Partners just keeps sending it to me. In fact, a year ago they actually sent me TWO separate surveys, and therefore, TWO five dollar bills. I was pumped.

However, a week or two later, I got a letter in the mail explaining that the extra survey had been sent by mistake and asking me if could I please mail back the $5.00 from the duplicate survey.

And you know what? I did.

I may be cheap, but at least I'm honest. :)

Smoldering Myeloma Video

I came across this video during one of my many Internet searches on smoldering myeloma. It's from a website called, "Managing Myeloma" which I believe it is intended as a professional resource for oncologists.

The video is a little dated, and the acting is, well, a bit less than Oscar worthy, but it has some good information. I particularly like how the patient can't pronounce 'myeloma', which really, is not that unusual because most have never heard of the disease before. :)


Smoldering Myeloma - Breaking the news to your patient