M is for Myeloma is a place to chronicle my family's newest 'adventure' living with multiple myeloma to keep family and friends updated and also hopefully help others living with the disease.

Tuesday, February 1, 2011

Where to start? Let's rewind for some explanation.

It all started In June 2010 when I fractured a rib. Bear with me this is going to be a long story...

It took me a little while to realize it might actually be broken. We were (finally!) moving into our new house when the pain started so I thought I just pulled a muscle or something. It got worse instead of better so I went to the walk-in for an X-ray. Sure enough poor little L10 was fractured. Odd for sure, considering I didn't remember bumping it or anything. Moving day (and night) was totally insanity though (complete with broken down U-Haul), so I couldn't rule it out.

About a month later I was lifting my son up from the floor after giving him a bath. I stood up and turned at the same time and felt the "POP". Yowch! The pain was exactly like with the first fracture. I went to the ER that time because I was pretty freaked out. Young healthy people like me shouldn't just break ribs like that, right? The ER doc confirmed another fracture, L5 this time. She referred me to the internal medicine clinic since I (stupidly) don't have a PCP. The doctor (err, resident) did what I thought was a thorough exam, took a detailed history, asked a lot of questions, ordered bloodwork and a bone density test. He was actually the first one to speak the dreaded word...myeloma...albeit only as a remote possibility. Bloodwork was normal, bone density test showed slight osteopenia. Hmmmm. Well ok, that's weird, but I guess I'll take your "start taking calcium supplements and we'll follow up with another bone density test in 6 months".

Or maybe not.

Fast forward to end of October...I started having another painful spot in my rib area. I thought from the intensity of the pain that it had to be another fracture, but the X-ray didn't show anything. The walk-in doctor seemed concerned about my symptoms and advised me to press the doctor (err, resident) to do more tests. I was having a terrible time getting in touch with said doctor at the clinic so I decided to ditch him. I cold-called Hopkins Orthopedic Community Physicians and made the first appointment I could get. The doctor there was great. He ordered new X-rays and more bloodwork. The bloodwork was still normal, but they saw something on the X-ray. Areas of "hypolucency" (code for holes), he wanted me to go for an MRI but didn't want me to be concerned. HA! I think it's a given that when a doctor says "but I don't want you to be concerned" that's exactly when you enter total freak out mode.

The MRI gave him a better look, and that's when he decided he needed to pass me on to a different specialist. A kind of specialist you never want to hear you need. Yep, an oncologist. And although this was not the first time in the conversation myeloma came up as a possibility, having to make an appointment with an oncologist elevated me from total freakout mode to ultimate panic mode. It also sent my research oriented self poring over the medical literature for all possible differential diagnoses. I couldn't come up with anything else that really fit, and I was digging deep into the dregs of things you can't pronounce and even most doctors have never heard of. So by the time the oncologist appointment came around I had already convinced myself I knew what the answer would ultimately be. And seriously, of all the things I just had to be right about...good grief.

The oncologist was very nice, he did a great job putting us at ease...too good actually! He was so convinced that it wasn't going to be myeloma. I guess because he couldn't fathom that the seemingly healthy 34 year old woman sitting in front of him could possibly have a disease that usually strikes people twice my age. I headed to the blood test and CT he ordered actually believing him. Then 3 hours later (at 7 in the evening) he called. And that's another thing sure to send you into a panic...an after hours call from a doctor, and not just any doctor, an oncologist! The CT apparently showed I had a whole bunch of those little holes scattered around in different bones. He still wasn't believing it was going to be myeloma, but he ordered a biopsy of one of the lesions (i.e. holes). So between the blood test (called serum protein electrophoresis) and the biopsy we would have an answer.

Here is where I'm thinking I should insert the answer to the question "Wait, what the hell is myeloma anyway?" Myeloma is a type of bone marrow cancer, specifically it's cancer of the plasma cells. Bone marrow is the source of all the blood cells in your body and plasma cells are a particular type of white blood cell. Normal plasma cells are your friend. They produce antibodies that help you fight infections. Abnormal (code for cancerous) plasma cells are not your friend. They crowd up your normal bone marrow, (often) secrete large quantities of useless antibodies, and damage your bones.

The serum protein electrophoresis test I mentioned would detect the useless antibodies and the lesion biopsy would give a look at the plasma cells. The protein test came back first, and it was negative! Yay, negative! The first good news in while. The doctor did say we still needed the biopsy to be sure. So of course I had to jump on the internet and ruin my own excitement. Turns out some cases of myeloma are non-secretory, meaning no useless protein secretion and a negative protein test. Hmph. So then it was back to waiting for the lesion biopsy results.

Did I mention this was all just before Christmas? The lesion biopsy was done on the 15th of December. So my Christmas present was either going to be some amazingly good news or cancer. Ugh. The biopsy itself wasn't so bad, I mean it went about as well as having a needle stuck into a bone can go. The doctor promised he would call as soon as he had the results back from the pathologist. He kept his promise, although he admitted afterwards that he considered waiting until after the Christmas to call. I knew as soon as I heard his voice. He said he was referring me to a myeloma specialist, and he kept asking if I had any questions but I was just speechless. I was at work and it's a damn good thing Alex and I work together, because there was no way I could have driven.

The specialist worked me in the next week, just after Christmas. It was a challenge to make it through that week, but we just tried to keep the focus on making Christmas fabulous for the kids.

For the appointment I had to go to the Sidney Kimmel Cancer Center for the appointment. The clinic waiting room was one of the scariest places I had ever been (up to then). She ordered a new round of tests to help with staging, including more bloodwork, another biopsy, and a PET scan. More tests and more waiting for results. All the waiting was giving me serious anxiety. I still kept thinking she was going to tell me it was all a big mistake.

No, no mistake. No waking up from a bad dream. The surreal was definitely quite real. I was suddenly a cancer patient.

While waiting for all the tests results to come in, I realized I had acquired a third full time job... researching all things myeloma. Luckily as a research scientist I have the background to help me interpret the primary literature. There are also a huge number of fellow patients that keep really fabulous and informative blogs. Turns out the face of myeloma treatment has changed significantly in the past 10 years with the advent of newer more effective drugs. In the changing landscape there is a range of treatment strategies, some more conservative some more aggressive, with no clear cut data indicating which approach gives the best outcomes. The one thing almost no one talks about with myeloma is "cure". Fortunately that is starting to change, and more and more experts are starting to believe myeloma is becoming a manageable chronic disease.

The newer more effective drugs (thalidomide, Revlimid, Velcade) can be combined with each other and with other chemo drugs in different regimens. Higher dose therapy with melphalan supported by autologous stem cell transplant is also an important part of treatment. (The melphalan wipes out your bone marrow and your own stem cells are used to rescue bone marrow function). Some recommend transplant be done early, others say it can be saved until "later" (code for relapse after initial treatment), others yet think two transplants are better than one. After all that some docs think its a good idea to keep you on several of the drugs for a long time (so-called maintenance therapy). Very very confusing to say the least, and from what I could tell even a roomful of experts wouldn't be able to give a consensus. I was starting to think I should pursue second opinions with other key specialists to be sure I would get perspective on the range of options.

The tests results came back so it was back to the clinic to hear it all from the specialist. I was confirmed to be Stage I (for those in the know, my B2 microglobulin was 1.65 and my albumin was 4.6), so this was good news. Still no detectable "M spike" of abnormal antibodies by serum protein electrophoresis, although another test indicated slightly elevated Kappa light chain (light chains are one portion of an antibody). This means I'm classified as "hyposecretory". The secretory aspect has no significance prognostically, but does make it harder to easily track effectiveness of treatment since those abnormal antibodies provide a convenient tracking marker. The percentage of plasma cells in the marrow biopsy was 10-20% (normal is 2%, 10% or greater is myeloma). The other good news was in the cytogenetic analysis. These are tests that pick up on the genetic changes associated with the cancer cells. Certain changes are associated with higher "risk", i.e. poorer prognosis. By the standard FISH test I have only one change, a t(11,14) translocation, and it is not a high risk marker.

Her recommendation basically was that I could choose from a range of options. Hmph. The gist was "start with chemo, do an autologous stem cell transplant, consider maintenance therapy". More specifically, start with a three drug combination, but choose which drugs I wanted to use; do a transplant right after the chemo or wait until "later" to do the transplant; then consider staying on two drugs as maintenance. She was very data oriented (which the scientist in me liked), but I didn't feel any personal investment and she didn't share her gut feelings (which the patient in me didn't like). Overall her recommended approach is pretty standard in terms of aggressiveness, and while I suppose some people like having options I wasn't thrilled with the "choose your own adventure" approach to cancer treatment.

I had been hearing and reading a lot about a treatment facility in Arkansas. A facility where they treat more myeloma patients than anywhere else in the world. The treatment approach is aggressive, but with good outcomes. (Although a key point seems to be that the outcomes may not necessarily be "better" outcomes. It's just not known how the aggressive approach compares side-by-side with other standard approaches because the study hasn't been done.) Basically the approach there is to throw the kitchen sink at the disease up front in quick succession, do two transplants, then follow up with long term maintenance. A similar aggressive approach has been proven out in treating childhood leukemia. They have also developed an advanced gene expression profiling test that helps stratify patients in terms of risk. It's more sophisticated than the FISH tests I had already had done. I had asked the Hopkins specialist about it and her criticisms were that no one can say it's better and it might be unnecessarily harsh for the patient in terms of quality of life. She added though that she would love to get a look at the gene expression profiling results if I were to go for an evaluation.

We decided the only way to form an opinion was to go there for an evaluation and see things for ourselves. I also decided to make an appointment at Dana Farber in Boston...my old stomping ground...since the scientist in me wouldn't be able to make a decision without an n of at least 3. Dana Farber also has a large myeloma treatment center as well, and their research is focused on next generation drugs.

The trip to Arkansas was first. A whole weeks worth of repeating every test I had already done and adding in a whole slew of new tests. It started with 24 vials of blood and just got more fun from there. MRIs, X-rays, CT, bone marrow biopsy, EKG, cardiac echo, pulmonary function test, etc. Over the course of the week I got to know the facilities pretty well and met a lot of really nice people. Part Southern hospitality for sure, but also part just plain having a really dedicated army of people working for the myeloma patients. It definitely contrasted the more impersonal experience I had at Hopkins. After the week of poking and prodding I got to see the head honcho. I was surprised to find out that he personally sees new patients to review the results and make his recommendations. (When I called Dana Farber and asked to see the head honcho there, they told me I couldn't see him until August.)

The good doctor spent over an hour with us reviewing all my results and answering our questions. The good news was that the scenario had not really changed since the previous tests, the better news was the gene expression profiling tests showed I was NOT "high risk". This is really important because the patients they identify as "high risk" do not respond as well to treatment and relapse faster than other patients. We were very impressed by the data he showed us and the thoughtful answers he gave to our questions. No one else has the kind of long term follow up data they have. For low risk patients on their latest version of the protocol ~70% achieve remission, and of those ~90% maintain it for 7+ years (beyond that they are still being followed). He actually seemed to be really enjoying our challenging questions, and he kept asking for more (and referring to me as "doctor", which made me laugh). His passion for his work was evident and it made an impression on us both.

We left pretty convinced that Arkansas was the way to go. It made sense to both of us from all angles, well, from almost all angles...he travel angle was indeed a big BIG consideration! Biologically speaking it makes sense to me to pull out the big guns and at least TRY to cure it, rather than futz around "saving things for later". One reason you would save is so you'll have something left the cancer hasn't seen when you relapse, but since there are other new drugs close to approval in the myeloma pipeline this wasn't a big concern for us. I also really liked the idea of having a team of people working with me who are dedicated to myeloma patients. I want as many experienced eyes on the situation as possible. The biggest apprehension was really the travel though. The prospect of being away from the kids for extended periods, coordinating caretakers to help me in Arkansas, and managing the financial burden was really overwhelming. The phrase "logistical nightmare" would be a serious understatement.

I kept my appointment in Boston the following week. Not as big of an ordeal there, just one blood test and meet with the doctor. It was nice to have a chance to see my family, and my brother was brave enough to come with me to the clinic. I was impressed by the doctor there as well. He reviewed all my previous results as well and confirmed the previous characterizations. His overall recommendation was along the same lines as that of Hopkins, but was a bit more definitive. Start with a specific 3 drug regimen, do a transplant immediately after, then do maintenance with Revlimid for a year. He cited current data and gave a thoughtful explanation of his recommended approach. When I questioned him about Arkansas, he confirmed the interpretation that the criticism is no-one can say the outcomes are "better", and the only downsides are it might be overly harsh and could be "risky" not to save anything for later. He said he couldn't see any reason I would actually come to Boston for treatment (either I could get his recommendation from Hopkins of go to Arkansas), but told me to stay in touch and let him know how things are going. He also said to feel free to contact him at any time with questions.

After that it was pretty much decided. Alex and I both agreed we believed in Arkansas, and that we would work out the logistical considerations. We figured it was worth it since, as another myeloma patient wisely put it, "this is your life we are talking about".

It took a little bit of time (and a lot of stress) to work through one important logistical consideration...insurance approval. I am extremely lucky to have good health coverage, but at the out of network rate and with those pesky "reasonable and customary" and "medical necessity" clauses along with the travel/lodging expenses things were daunting. Thankfully, once we got the right people talking to each other Arkansas tandem transplant protocol was approved and we were granted in-network coverage since that service is not available in our network. Once the insurance approval was in place we could move forward and get started. So we packed up some stuff and made the (SEVENTEEN hour) drive...