Saturday Noon

Chris had a relatively good night last night and this morning (Saturday). She has been infused with her regiment of ant-infection, anti-nausea, anti-anxiety drugs, which has made her comfortable but sleepy. Generally she slept well, (but I however will be scheduling another back surgery from sleeping in this dang chair all night) and she actually ate half of a bagel and had some juice this morning. The amyloid team will be in this afternoon and may allow her to go back to the apartment tonight if she can keep her food down. She continues to lose weight as she is not eating, but we are all hopeful that will improve when her nausea stops.

Abby may come in to visit her today, but will need to wear a mask as we are now tightening down on exposure to infections. Melissa and Stephen are in Iowa as Stephen is competing in the National Junior Elite Triathlon Championships this weekend. They have been calling regularly to check in on her.

Overall, it has been a hell of a week. But now we need the melphalan to have done it's work and the replanted stem cells to wake up and start producing her new blood system. Throughout this arduous period of vomiting, nonstop nausea, aches, 100's of injections and pill taking, I have not heard her complain once. Literally, she has taken this with an amazingly stoic attitude and has not shed a single tear or even expressed a hint of frustration. She knew this was going to be a hard-fought battle and she has gone in with guns blazing. It would not be possible to state how proud I am of her. It would also not be possible to tell everyone how thankful we are for the incredible support we have been given from our extended support system. Chris' sisters have been fantastic, all of our families, friends, and colleagues have been incredibly helpful. This support has allowed me to focus on job #1.

Just as an aside, a quick comment as to how the world and God work in mysterious ways. June 20 was declared by a scientist in the UK ( http://www.ajc.com/health/content/health/stories/2008/06/20/happiest_day_0620.html)
as the Happiest Day of the Year. I couldn't agree more.

Happy Birthday Chris!!

Just a quick update, with more later, but to let interested parties know, Chris was infused with 6.2 Million of her own stem cells today. Everything went successfully!!! At 1PM this afternoon, they drained the last of 4 bags of thawed stem cells. I think there was a tube or a line for each single stem cell as it was amazing how many different things she was connnected to. This was the last of three major steps in this process 1) the stem cell collection 2) the malphalan high dose chemo and 3) the stem cell replant. When you consider the fact that we are all born with a finite number of stem cells and that those stem cells have never been outside of her body before and they took a 1 week journey to the deep freeze, it is pretty amazing. I doubt any of us truly understand the power of stem cells. I'll avoid politics here, but I am confident saying that these particualr stem cells will give Chris her new lease on life. It's her new birthday today. Happy Birthday Chris.

Thursday Evening - Re-admitted to the Hospital

Because tomorrow is a critical day and potentially one of the riskiest, the medical team has decided to admit Chris back into the hospital tonight. Her nausea has been stabilized with a list of medications that even the Doctors can't pronounce. She has been sleeping most of the day, but this is better then the non-stop vomiting. She still has no appetite at all, and to get her to eat a couple of spoons of soup requires incredible persuasion skills.

The stem cell collection will occur mid morning tomorrow (Friday). This is hopefully the start of her new life of wellness. I know a number of people who have told me already that this is their birthday (June 20) and it is a great day to have one. Her first birth date was December 27 and she always claims she gets "jipped' because she gets fewer or combined presents with Christmas. Now she will have two birthdays. I know for me, I can't wait to celebrate two birthdays with her for many many many years. I would even say that June 20 will become the most important day of our families life.

The process is incredible. Here is a link that describes the process in very simple terms:
http://www.medterms.com/script/main/art.asp?articlekey=4846

Although Chris does not have cancer, the approach is the same, but in her case the intent is to kill the defective plasma cells creating this protein.

So as of 8:00 pm Thursday night, Chris is resting comfortably, all vitals are stable, and the chemo is currently killing her blood cells. The stem cells she was born with will be replanted tomorrow and our prayer is that we have killed the bad plasma cells that are causing such damage to her heart. Thanks again to all for the countless offers of support and the incredible number of prayers.

Thurs Noon - Very Tough Night

Chris had a miserable night, basically up the entire time with non-stop vomiting and nausea. We got in early to the center today and they put her on an IV for the vomiting. She is having a very difficult stretch, but she is being very strong as well. My guess is that she will sleep most of the afternoon. They also came in and did acupuncture for the nausea and she felt that this really worked.

She really has not eaten in 2 days, and her weight this morning was 108. The good news is that her lungs have really cleared up significantly, so this is a positive.

Chemo - Day 2

Done!! Two rounds of an incredibly toxic drug designed to kill blood cells that in reality will hopefully stop this disease. Last night, while getting ready for bed, we both agreed that things had gone very well (yesterday). However, this morning, Chris did not feel well at all. She was very nauseous, a bit lightheaded, and vomited the little breakfast that she had nibbled on earlier. She is retaining fluids (3 lb's worth) and this has caused swelling in her legs and abdomen. The medical team debated this morning whether to only give her the smaller dose or continue with the stronger dose. In the end, they wanted her to make the decision. The stronger dose (200Mg) is much more toxic and the side effects much worse. But as always, Chris went the tough route. The success rate of remission is greater with the stronger dose, but she will suffer a more difficult period for a month or so. Overall Chris had a tough day today, and it is only going to get worse. I think the reality of how bad she is going to feel is going to hit sooner rather than later. Tonight, she is not feeling well, can't eat anything, and has little energy.



Tomorrow we start a much more intensive potpourri of drugs. She will be taking drugs for nausea, infection, mouth sores, etc. The regiment is incredibly scripted. This will continue for a month. Friday is the Big Day as they say around here. This is when they replant the stem cells and start her from the beginning. They call this her new birthday, and knowing all things are going to go well, it will be June 20. As I have stated earlier, she will have zero immunity system, but the newly replanted stem cells will start waking up from their deep freeze outside of her body and slowly begin producing the cells we all take for granted - the red blood cells, white blood cells, and plasma. The actual stem cell replant is extremely hard on the body, so it will take every ounce of Chris' strength. But we all know she will do this.



Chris' siblings have been a huge help. Her brother was great last week and her two sisters have just been incredibly helpful. They both have left their own families and are living at the house. Whether it is running errands, doing laundry, helping the kids, running things into Boston, it has been amazing.



Chris has been very strong so far, and she will need to be even stronger going forward. This is going to get worse over the next 5-7 days, so everyones prayers and support will be greatly appreciated. They certainly have made a difference so far, and we are both very thankful.

Chemo (Melphalan) - Day 1

One down, one to go. Today Chris did an outstanding job of taking the high dose (200mg) of the chemo. We have been told that the two day dose is the equivalent of many months of normal chemo. She was a champ today. It is a very interesting process. As the dose is so strong/toxic, it can actually create lesions on blood capillaries. The most troublesome area is the mouth, so prior to receiving the chemo (through the catheter in her chest), she has to pack her mouth with crushed ice for 30 minutes. This constricts the blood vessles and reduces/prevents the lesions which might form. The actual chemo drip takes only about 20 minutes, very quick, with all the shaved ice she can stand during this period. It is amazing to think that this small amount of drug will effectively kill every cell in her blood system over a period of a few days, including the plasma cells that are creating the amyloid protein. It will also kill all of her immune system, which will be wiped out quickly. As such, she is required to wear a surgical mask over her nose and mouth at all times outside of the apartment or the clinic itself. She can't touch a door knob, an elevator button, or breath any open air. She also has a very restricted diet. No foods that are fresh (that might have exposure to an airborne disease). This means no fruits or vegetables, no restaraunt food, nothing but packaged meals.
They apparantly had a long debate about the dosage among the doctors and decided the best approach was the stronger amount. With the heart damage, it is critical that they stop any further amyloid deposits and they thought she was tough enough and young enough to tolerate the side effects which may be very severe. As Machiavelli said, the end justifies the means. If she can tolerate the side affects for a month in order to get a higher success rate, it is worth it.

Tonights dinner was a three cheese tortolini with an alfredo sauce and some slices of cheddar cheese. All prepackaged, and of course it was overcooked by the new chief chef. But, the direction from the doctors is to overcook everything, so that is a simple instruction someone who overcooks all the time! We are generally doing well in the new apartment. The views are spectacular (21st floor) and we are close to everything. The parking is underneath the building and a straigh shot down to the car from our floor. It couldn't be easier. The concierge service keeps reminding us that they can get us reservations or tickets upon request, but unfortunately we aren't going anyhere.

Chris' spirits have been very high today as we feel like we have made great progress with one of two chemo's done. Unfortunatly this is the easy part. We are told that next week she will hit bottom. Losing all her hair, vomiting, 22 hours of restless and groggy sleep per day, etc. The trick will be to avoid all infections and to immedualty address any hint of a fever or lesion.

All the kids have been calling to check up on the day. All three of them have been great, and Chris' sisters are both at the house keeping things under control there. As of Monday evening, things are going very well considering the seriousness of what we are facing. Everybody has been willing to help, everyone has been offering their prayers, and we are incredibly fortunate to have the combination of the best medical care and expertise in the world, the support of our family, friends and colleauges, and the financial capabilty to provide a very nice living environment as we fight this disease. Day two will be another step in counquring this!

It's a GO!!!

Great news today!! They did a lung x-ray and things have improved significantly since Friday. They increased the amount of diuretics significantly over the weekend and this has done the trick. The diuretics (Lasik) reduce the edema (swelling) and you can see this in her legs and she can feel it in her breathing. The doctors were very pleased to see this and have given the go ahead for the chemo. Now the big debate is whether to use a very high dose (200 mg) of Melphalan) or a reduced scope (140mg). The higher dose has a higher success rate but it also has much more toxicity and worse side effects. The doctors are debating whether she can tolerate the higher dose, with the lung specialist stating that he believes the lower dose is the right option. So we are a GO. We had dinner at home tonight with the family and then moved to the apartment in Boston. This will be our germ free cave for the next 4 weeks. We have Lysol wipes, Purell, hand soap, etc. everywhere. Chris also starts a regimen of drugs (13 different prescriptions) which requires an advanced degree in pharmaceuticals to administer. That is Steve's job, as the caregiver, to manage all items outside of the Amyloid center. He will do all meal preparations (not a strength), all cleaning (not bad at it), all transportation, shopping, phone updates (pretty good), psychologist (better than most) and be the medical advocate (excellent).
Tomorrow will be a major milestone in beating this thing.

Long Weekend

It has been a long weekend. Stephen had a triathlon on Cape Cod (6th out of over 700) on Saturday and both he and Abby have been working as lifeguards. Chris' sister and brother (Sally and Tim) left to go back to PA on Saturday after being up here all week, and here other sister Debbie arrived. She will be taking charge of the household while we are in Boston. We went to a graduation party Saturday night, but Chris is clearly slowing down, not eating, and feeling very anxious and losing hope. The discussion on the lungs on Friday has thrown us for a loop. Things were going so positively, but now we have great concerns that things have progresses so bad that the entire course of treatment may be put on hold.
We had a private prayer session with our Pastor after todays service. He was very supportive and calming and reconfirmed that we are not alone in this very scary journey. In fact, that is obvious every day. The support from friends around town has been beyond amazing. The meals, the errands, the phone calls, all just incredible. As a former candidate wrote, "it takes a village to raise a child" but in this case it is more like " it takes a village to help those in need" and we have been on the receiving end of that.
Chris has also been trying Reiki, and that seems to have been especially successful for her. This is a Japanese approach for stress reduction and channelling "life force energy". Steve is always the ultimate skeptic, but Chris is very clear that she is getting benefit from it so we will continue for sure.
Tomorrow is going to be a difficult day. We don't know whether it will be a Go, or put on hold. We are both ready to start the chemo as soon as possible, but if her body can't tolerate it, it can be very very bad. We'll leave ourselves in the hands of the Doctors and God.

Not a great day - getting worried

We were back in the hospital for evaluations today (Friday) and we met with the pulmonary (lung) specialist as well as the program Director. They are becoming increasingly worried about the fluid in her lungs and have decided to hold off on the chemo planned for Monday. Fluid in the lungs is a concern as it is a potential indicator of congestive heart failure (the heart working too hard to pump blood around the body). They will do an x-ray of her lungs on Monday and decide one of three options: 1) start on Tuesday of next week, 2) delay a full week and try to address the lung issue, or 3) hold off indefinitely and consider other options. Of course we want to be as aggressive ans urgent as possible, but we also do not want to add risk if by waiting we can get a more optimal result. This is our first set back. We have been moving at incredible speed and have contacted the program, done the evaluations, been accepted, and done the stem cell collection faster than any other in the program, our aggressiveness and advocacy have paid off. However, now we have this issue ahead of us. We both hate going into the weekend with this hanging over our heads.

Even More

Day two of collection today. Chris has developed some side effects such as nausea, digestive issues, and fluid in her lungs, but they are treating those. Because they collected so much yesterday, they only did 3 hours on the machine today (yesterday was six hours). They collected another 3 million plus today, so about 10 million total - more than enough. They are watching a concern around fluid in her lungs, but she is able to go home. Overall good news on the collection, many many cells, some of which will be replanted next week.

Very Successful!

In order to proceed with the chemo, they need to collect at least 2.5 million stem cells. The amount collected depends on general health, blood flow, etc. They collected a whopping 6.2 million today! That is a very good result. The kids said she should get an award for best stem cell collection.
The procedure is similar to dialysis, but for this, they pump the blood from the catheter tubes into a machine, which then uses a centrifuge to separate out the red, white, plasma, and stem cells due to different densities. They place the stem cell layer in its own bag, and treat it like gold. We are all born with a certain number of stem cells, and will never get new ones, so they are very precious. They come up with a special box on wheels, wrap the bag in layers of bubble wrap and blankets, and then transport it to the blood bank. The woman who had to do this was told that if she were to see a bus coming at her, she is to save the stem cells first. It was incredible how carefully they treat this bag. In the blood bank they will freeze it and then thaw a portion of it for the replant after the chemo. So a great day for collection.

Admitted to Hospital for Stem Cell Collection

Today Chris was admitted to the hospital for her strem cell collection on Wednesday and Thursday. As she has heart damage, they want to monitor her very closely during the process. In some cases, Stem Cells are collected outpatient, but better safe than sorry. She is a bit nervous but knows this is a required step. We went out for a good dinner in Boston, and then she got admitted and into the bed. So far so good, we'll see how tomorrow goes.

Catheter Placed Today

Chris had a catheter placed in her chest today via surgery. This is really two tubes, 14 inches long that will serve as the input and output "ports" for her collections, injections, etc. The surgery went well. She now has about 2 inches of tubing hanging from her right chest that enter her heart. She was very tired after this, but we went home with no issues. So this was a major step and all went well.

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.