Chris' Background - How we got here

This post will be a long overview of how we got to where we are today with Chris. I won't go into excruciating detail, but will try to give a brief history for those who may not be familiar with it.

December, 2007
Chris was very active and busy with many things, including tennis, a personal trainer, yoga, skiing, etc. While she felt very healthy overall, she occasionally felt tired at her training session at the health club. She felt that instead of getting stronger, that in fact she was almost "losing ground" a bit. She chalked this up to normal old-age related slowing.
January, 2008
Chris had a normally scheduled annual physical exam with her primary physician, with normal blood work, etc. Everything in the exam appeared normal, but one indicator from a urine specimen showed an unusually high level of protein.

February, 2008
Chris continued with her activities, the tennis, the long walks with the dog, her training, etc, but started to state that "things just are not feeling right". She felt tired, but had no signs of anemia.

March, 2008
Chris had a follow-up appointment with her primary physician. They discussed the need for more testing and decided to do a 24 hour urine collection along with many other tests.

Chris also read an article in the local paper about a woman in a town nearby who had been finally diagnosed with a "mystery disease". As she read the article, she kept thinking "these are the same symptoms I am starting to see". Some shortness of breath, some sores around her eyes, some swelling in her legs, etc. Here is the article: http://www.metrowestdailynews.com/homepage/x1853598606

Chris showed the article to her physician as something to possibly explore.

April, 2008
On April 4th, Chris got a call (while playing tennis) from her physician asking her if she could come in to meet with her. Her tests had shown that she had a highly elevated protein level in her urine (known as an m-spike) that was an indicator for a number of very serious diseases, including the one that she had read about in the paper - Amyloidosis. Steve was in Oneonta, NY with our daughter Abby trying to make a final college decision for Hartwick College. Abby spent the night with the Hartwick swim team and Chris and Steve each had a separate and extremely frightful night apart. One of many future nights of no sleep.

We immediately went into action, scheduling appointments with Oncologists/Hemotologists/Nephrologists (kidney), etc. Chris continued to show highly elevated proteins in her urine and a number of biopsy's were performed. One was a bone marrow biopsy which came back as negative. At this point the Hemotoligist believed Chris has either Multiple Myloma or Waldenstroms Disease. We contacted the Dana Farber Cancer Center for a second opinion and continued on with biopsies. She continued to feel tired, had significant weight loss, had edema (swelling) in her legs, and showed a few other unusual symptoms. The next biopsy was a "fat pad" biopsy, taken from the abdomen. This is the required test for amyloid. The collected specimen is stained with a "red congo" stain and looked at under a special green light. This is the only valid test for positive confirmation for amyloidosis and is hardly ever performed as the disease is so rare (about 1500 -3500 diagnosis' a year). This test was required for us to meet with the Boston Medical Amyloid Center. Steve had contacted them earlier and they looked at the results and invited us in for three days of extensive testing the first week of May.

May, 2008
We went to the Boston Medical Center for three days of tests on May 5-7. On the first day, we were still unclear whether Chris has Multiple Myloma, Waldenstrom's, or Amyloidosis. On Mayt 7th, we were told, with absolute certainty that the diagnosis was Amyloidosis. This was confirmed with the congo red fat biopsy. They explained to us the genesis of the amyloid protein, the role of the plasma cells, the bone marrow, the details of light-chain proteins, the kappa and lamda ratio's, etc. They were very blunt, this was not Myloma and not Waldenstroms. Hopefully by now you have read the sidebar in this blog that describes the basics of amyloidosis. They explained the treatment options, the success rate ( odds of complete remission, partial remission, no remission) as well as the risk factors. Treatments include a very aggressive treatment called High-dose melphalan/stem cell transplant ( HDM/SCT ), as well as oral chemotherapy(similar to cancer treatment, but this is not cancer).
They asked for additional tests (stress test, pulmonary strength, etc.) and they informed us on May 19th that Chris had been accepted into the HDM/SCT program. Very simply, HDM/SCT consists of a three step process: 1) removal of her stem cells from her body (specifically her bone marrow) after a "port" or catheter is placed in her chest with tubes to her heart, 2) doses of a very powerful chemo drug called melphalan normally given over months, that will be given in 20 minute session over two days), and 3) replant of her previously harvested stem cells. The basics of the approach is that the melphalan effectively kills the defective plasma cells that are creating the amyloid proteins that are causing the organ damage. The chemo is so strong that had the stem cells not been collected, they also would be killed by the chemo (thus the removal and the replant). The process will completely destroy her immunity system. She will have no white blood cells to fight disease for about a month or two until the replanted stem cells start producing white blood cells again. As such, the top priority for this period is to avoid all exposure to infection.

So we now have a diagnosis, and a treatment plan. Read above for the next set of activities.