We heard from the doctors at Boston yesterday afternoon and it is not great news but more importantly it is not terrible news. In the end, it is progress, but will require us to continue the fight as it is not over. The results are what we actually had been predicting, as we are now smart enough to read the test results, ask the right questions, and know the options as the various meetings with doctors occur. I don't need to wait for a big report when I know the individual elements themselves. So, what I will describe is what we knew for the most part.
Lambda light chains: Normal is a number of 5-26. Chris it turns out was over 700 at one point and 660 when first diagnosed. She is now down to 39, and this represents a drop of 94% or so, but unfortunately still above 26. This means the amyloid proteins are still being produced, albeit at a significantly reduced rate. A 94% response is a long way there, but not across the finish line.
Plasma Cells in her bone marrow: Normal under 5%, Chris was over 10%. Now after the high dose chemo, she is back to under 5%. This is very good.
Serum IFE (immunoglubulin): This was down to zero, a very good story.
Flight Light Chain Ratio (Kappa/Lambda): Normal is .2-1.6. Chris is at .2, again very good.
So she still has the lambda free light chains being produced, but the biomarkers are all looking very good.
Cardio: Here is the difficult part, for both the doctors and for us. They cannot explain why the hematologic response has been so good while there continues to be further damage to her heart. We just got the analysis from the TEE (the echo through her esophagus) and it shows "severe left atrium enlargement", and "the right atrium is severely dilated". Further, there is "moderate to severe mitral regurgitation." They show an ejection fraction of 20%-25%, where normal is over 50%. Thus, as stated in earlier posts, our focus now shifts from the blood to the heart. We fully expect that there will be more work to do on the amyloidosis, as we need to stop the continued protein depostion, BUT, job #1 is now to understand and execute a plan that protects the heart. So the next activity is to meet with the Dr. Falk on December 31.
For those of you interested and looking for some technical and potentially mind-numbing/sleep inducing reading, here is a great paper written by Dr. Falk:
http://circ.ahajournals.org/cgi/reprint/112/13/2047.pdf .
I suggest only the very curious spend the time to print and read this, but it gives a detailed overview of the new battle front.
So enough of the technical stuff, lets talk about the most important element, Chris herself. Overall, she is doing very well emotionally and physically. She has been on the treadmill every day - this is her full time job, to ensure she is in top physical condition with the constraints she has. She has been very active, decorating the house, going to parties around town, shopping, etc. Outwardly, you would see little difference than the Chris you would have seen a year or two ago, except about 20 pounds lighter (she is up to a whopping 109) and very, very short hair (which is really starting to come back). She is upbeat, she is active, she is feeling good. However, there are times when we are both in the dump a bit. The worst time is late at night, when the mind has time to think a bit more. We both worry, about the future, about implications, about options, and about whether there is more we can be doing. I personally continue to ask myself; what else could we be doing? Are we missing a test? Are there better doctors to talk to? Am I prepared for all situations and outcomes? I hope so, but who knows for sure.
Overall, we are very fortunate. I can tell you when I first heard the word "amyloidosis" on Friday April 4th while touring Hartwick College with Abby, I had no idea what it is. Since then, Chris has had a stem cell transplant with high dose chemo, been "as close to hell as you can get and still being able to come back". returned home, and is living an active life. Melissa has written papers and made presentations on Amyloidosis at college, Abby has been an amazing help, cheerleader and supporter to Chris, and Stephen announced last week that for his required Senior Project at Worcester Academy, that he will be raising money for Amyloidosis research by riding from Florida to Holliston during his 2 week spring break - 1200 miles (more on that in future posts). So our family has been great. Yet, the most amazing thing has been what has occurred outside the family (which I know I state too often). Never ever, in my wildest dreams, did I ever expect a community to come together and help a friend like people have for Chris. It is the most amazing thing to see. Beyond anything I can describe. From the smallest things like a phone call to ask her how she is doing, to cards, to daily walks (sometimes in the rain and late at night in the neighborhood), the brownies, sauces, soups, meals, the errands, the interest, the listening, the learning, and most importantly the prayers and positive thoughts. It is beyond belief. There are no words strong enough or big enough to thank Chris' sister giving up her entire summer with her family to watch our family. I can't describe the incredible strength Chris has had in this fight, and I can't begin to express how optimistic I am for our future. We are going to fight two battles now (heart and blood), but I know the alignment of my tough wife, our amazing support (all of you), and the best medical care in the world will end up with success.
I'll try to update the blog occasionally over the next few weeks, and will provide a complete report after the meeting with Dr. Falk. Thanks for your continued interest.
Saturday, December 13, 2008
Thursday, December 11, 2008
Waiting for Godot (really Sanchorawala)
Chris has been very busy today and not had much time to be idle and thinking. That is a good thing. We are waiting for the big call from the medical center tomorrow afternoon where they will tell us what the indicators are for the degree of response. I know hundreds of people are waiting for the big HCR to be claimed, but based on some of the results, while I do not think we are there, I think we are close. The question I have been asked frequently, and a good one is: "At one point do they claim victory?" Is 95% close enough? Is 98%? The answer is pretty binary as I understand it. Your either have a complete response or you don't. If your body is still producing the amyloid proteins ( and as you know, this means the lambda free light chains), then we have to stop the production and the resulting deposition of them in the organs. More proteins mean more damage, and thus there is still work to be done.
My best guess is that they are going to say - "we are pleased, there is a good response, enjoy the holidays, come back in January and we'll retest and decide the action plan then".
As I thought a bit more last night about how to describe Chris' status, I would break it down into two areas: 1) Hematologically things are really looking good. 2) Cardiologically (a new word I just invented I think), things are OK but need a great deal of attention. I remain positive that if we can stop further heart damage, then Chris can move forward with a great and active (but not too active) lifestyle. Her positive attitude will make a huge difference going forward.
Chris did sleep very well last night. She has carried such a tremendous burden of stress that I know she felt as though she completed a major milestone yesterday. As she slept soundly this morning, I swear there was a smile on her face. This meant the world to me.
More tomorrow night or maybe early Saturday morning. I cannot say it enough - Thanks to all of you who care so much.
My best guess is that they are going to say - "we are pleased, there is a good response, enjoy the holidays, come back in January and we'll retest and decide the action plan then".
As I thought a bit more last night about how to describe Chris' status, I would break it down into two areas: 1) Hematologically things are really looking good. 2) Cardiologically (a new word I just invented I think), things are OK but need a great deal of attention. I remain positive that if we can stop further heart damage, then Chris can move forward with a great and active (but not too active) lifestyle. Her positive attitude will make a huge difference going forward.
Chris did sleep very well last night. She has carried such a tremendous burden of stress that I know she felt as though she completed a major milestone yesterday. As she slept soundly this morning, I swear there was a smile on her face. This meant the world to me.
More tomorrow night or maybe early Saturday morning. I cannot say it enough - Thanks to all of you who care so much.
Wednesday, December 10, 2008
Return Visit - Day 3
OK, a deep breath so I can think how to best make this understandable. Bottom line is two key messages: 1) all the test are not in yet, 2) the tests that are in show very mixed and confusing indications/results. Let me start with the bad and finish with the good.
Chris has a serious heart problem that has become worse since the spring. The number one issue, amongst many, is a significantly reduced ejection faction (EF). In simple terms, ejection faction is the % of blood pumped out of the heart with each heart beat. Normally this is in the range of 55% to 58%. In May, Chris' EF was 49%, not too bad. Today it is 35%. This has clearly worsened over the year. This is not due to the Chemo, but amyloid damage. This reduced EF is a symptom of the hardening of the left ventricle wall, which with reduced muscle elasticity has less ability to pump efficiently. There may also be some valve leakage between the atrium and the ventricle. What this all means is that Chris continues to have congestive heart failure. However, do not let the term 'failure' scare you as it did me. Failure in this case means that the heart is just not pumping at normal rates, so failure does not mean stoppage, it means reduction. The reduction in the pumping means that as her body calls for more oxygen as she exercises, climbs stairs, whatever, she will not be able to keep up with the demand. She would become easily fatigued, out of breath, and maybe have chest pounding. The cardiac story is not a good one, but she can function with normal activity and if the damage stops where it is, she can live a normal life while being careful. Nobody knows what further heart damage will occur going forward. I asked the question a hundred times, but nobody knows.
We see Dr. Falk (the most knowledgeable cardiologist in the world on this) again on December 31st. He now becomes an even bigger part of the game plan going forward since heart involvement becomes the single biggest issue we face.
OK, so now the better news. The other organs all are doing well, in fact her kidneys, which were problem number two after the heart, has actually improved since the chemo. There are a potpourri of blood and urine tests to measure kidney effectiveness. Almost all of these came back with improved results. Her kidneys are doing what they are supposed to be doing, they are seeing less amyloid proteins then they did before, and they are flushing them from the body better than before. One of the key tests is the amount of protein collected in a 24 hour urine sample (Chris hates carrying the jug around, but has now become a pro at it). Her Mg/24 hours were over 4000 in May, now they are about 2800, so this is a big drop and indicates a downward trend. But, to put it in perspective, normal is about 150 Mg/24 hours. As they explained it all today, the reduction will take as long as two years. I of course asked where we were on the reduction curve, ahead or behind what they would have expected for a good response and they said "6 months ahead of the curve". Obviously that is good.
The other key metric is the amount of lambda free light chains. Before, Chris was 660. Today she was 39. This is the single best news we have heard. This is a direct result of the chemo and indicates that the free light chain proteins that are created by her plasma cells and are floating around, combining and becoming insoluble in her organs have reduced considerably. This is about 94% reduction. Better than no reduction, better than 50% reduction, and better than 93% reduction. BUT, not a complete reduction. Normal is 15 -26. There are 4 elements that make up the criteria for a hematologic complete response (the famous HCR). Getting down to 26 or lower is one of them. The others are the Bone Marrow plasma cells at under 5% (she was at 10% in May), a Kappa/Lambda ratio between .26 and 1.65 and a good serum Immunofixation (IFE) test (too hard to explain here). The only test data we have at this point is the amount of lambda free light chains, and that is very good. The others we will get on Friday.
So now, the big question: Did we get a complete response? the answer is we do not yet know. We probably need to wait another month to see if the lambda's continue to reduce. To be honest, based on the discussions today, it is not clear if a complete response is possible. I seems to be unlikely at this point. The next big question is: what if it isn't complete response, now what?
Four options:
1) A second stem cell replant with high dose chemo (all over again), probably next spring/summer
2) An oral chemo, using a drug in trial called Revlimid
3) an oral chemo, using a drug in trial called Velcade
4) do nothing and watch.
Chris and I were very clear (more me than her) - we want to continue to be very aggressive. If they heard one word today it was "aggressive". Chris survived the pure hell of the high dose chemo this past year, and will do it again if its poison (and that is what it is) kills the rest of the bad plasma cells creating the proteins.
So here is the bottom line:
Chris has very bad heart damage, which we are seeking world class medical help to address. She will most likely be placed on an anticoagulant to prevent clots in her heart from causing a stroke.
The test results from this week are not all in, and thus we do not know what the prognosis is. The ones we do have are positive, but not where we need them to be. My guess is that they will ask her to come back in January and we will jointly decide the course of action.
So there is a lot we don't know.
Here is what I do know:
-Chris is tough as nails, an incredible fighter who is fighting the fight of her life
-We have found the best medical care possible - no second guessing on a single thing we have done
-We have the most amazing family, friends, colleagues (IBMers), and doctors we could have ever wished for
-The power of prayer, good wishes, concern, good meals, cards, phone calls, walks in the woods, etc have been the single most important factor in her success to date. All of you reading this blog are responsible for her progress to date. Thanks to everyone of you!!!
Chris has a serious heart problem that has become worse since the spring. The number one issue, amongst many, is a significantly reduced ejection faction (EF). In simple terms, ejection faction is the % of blood pumped out of the heart with each heart beat. Normally this is in the range of 55% to 58%. In May, Chris' EF was 49%, not too bad. Today it is 35%. This has clearly worsened over the year. This is not due to the Chemo, but amyloid damage. This reduced EF is a symptom of the hardening of the left ventricle wall, which with reduced muscle elasticity has less ability to pump efficiently. There may also be some valve leakage between the atrium and the ventricle. What this all means is that Chris continues to have congestive heart failure. However, do not let the term 'failure' scare you as it did me. Failure in this case means that the heart is just not pumping at normal rates, so failure does not mean stoppage, it means reduction. The reduction in the pumping means that as her body calls for more oxygen as she exercises, climbs stairs, whatever, she will not be able to keep up with the demand. She would become easily fatigued, out of breath, and maybe have chest pounding. The cardiac story is not a good one, but she can function with normal activity and if the damage stops where it is, she can live a normal life while being careful. Nobody knows what further heart damage will occur going forward. I asked the question a hundred times, but nobody knows.
We see Dr. Falk (the most knowledgeable cardiologist in the world on this) again on December 31st. He now becomes an even bigger part of the game plan going forward since heart involvement becomes the single biggest issue we face.
OK, so now the better news. The other organs all are doing well, in fact her kidneys, which were problem number two after the heart, has actually improved since the chemo. There are a potpourri of blood and urine tests to measure kidney effectiveness. Almost all of these came back with improved results. Her kidneys are doing what they are supposed to be doing, they are seeing less amyloid proteins then they did before, and they are flushing them from the body better than before. One of the key tests is the amount of protein collected in a 24 hour urine sample (Chris hates carrying the jug around, but has now become a pro at it). Her Mg/24 hours were over 4000 in May, now they are about 2800, so this is a big drop and indicates a downward trend. But, to put it in perspective, normal is about 150 Mg/24 hours. As they explained it all today, the reduction will take as long as two years. I of course asked where we were on the reduction curve, ahead or behind what they would have expected for a good response and they said "6 months ahead of the curve". Obviously that is good.
The other key metric is the amount of lambda free light chains. Before, Chris was 660. Today she was 39. This is the single best news we have heard. This is a direct result of the chemo and indicates that the free light chain proteins that are created by her plasma cells and are floating around, combining and becoming insoluble in her organs have reduced considerably. This is about 94% reduction. Better than no reduction, better than 50% reduction, and better than 93% reduction. BUT, not a complete reduction. Normal is 15 -26. There are 4 elements that make up the criteria for a hematologic complete response (the famous HCR). Getting down to 26 or lower is one of them. The others are the Bone Marrow plasma cells at under 5% (she was at 10% in May), a Kappa/Lambda ratio between .26 and 1.65 and a good serum Immunofixation (IFE) test (too hard to explain here). The only test data we have at this point is the amount of lambda free light chains, and that is very good. The others we will get on Friday.
So now, the big question: Did we get a complete response? the answer is we do not yet know. We probably need to wait another month to see if the lambda's continue to reduce. To be honest, based on the discussions today, it is not clear if a complete response is possible. I seems to be unlikely at this point. The next big question is: what if it isn't complete response, now what?
Four options:
1) A second stem cell replant with high dose chemo (all over again), probably next spring/summer
2) An oral chemo, using a drug in trial called Revlimid
3) an oral chemo, using a drug in trial called Velcade
4) do nothing and watch.
Chris and I were very clear (more me than her) - we want to continue to be very aggressive. If they heard one word today it was "aggressive". Chris survived the pure hell of the high dose chemo this past year, and will do it again if its poison (and that is what it is) kills the rest of the bad plasma cells creating the proteins.
So here is the bottom line:
Chris has very bad heart damage, which we are seeking world class medical help to address. She will most likely be placed on an anticoagulant to prevent clots in her heart from causing a stroke.
The test results from this week are not all in, and thus we do not know what the prognosis is. The ones we do have are positive, but not where we need them to be. My guess is that they will ask her to come back in January and we will jointly decide the course of action.
So there is a lot we don't know.
Here is what I do know:
-Chris is tough as nails, an incredible fighter who is fighting the fight of her life
-We have found the best medical care possible - no second guessing on a single thing we have done
-We have the most amazing family, friends, colleagues (IBMers), and doctors we could have ever wished for
-The power of prayer, good wishes, concern, good meals, cards, phone calls, walks in the woods, etc have been the single most important factor in her success to date. All of you reading this blog are responsible for her progress to date. Thanks to everyone of you!!!
Tuesday, December 9, 2008
Return Visit - Day 2
Not a great day today. I'll keep this short, as I really am not feeling like going into great detail and I am hoping that tomorrow will be better. Today was two sessions:
1) the support group. I have now been to five of these and each f them is different. Today there were a variety of experiences/stories. A woman from California was waiting on a heart and liver transplant, a man from NC had his transplant and high dose chemo 4 years ago and was doing great, another man had just localised impact in his stomach. Many different stories, but generally all good ones. So we walked out of that feeling good about these stories.
2) met with the Boston Medical cardiologist (not the one we saw two weeks ago). To be brutally honest, neither Chris or I like this guy, and we think it is more than just the message he gives. He has the sympathy of a rock and the heart of a piece of cold steel - just brutal. So here the news was not as positive as we were hoping for. There is continued decline in Chris' heart function, specifically her ejection fraction which is a measure of pumping effectiveness. There are some "biomarkers" or measurements of heart effectiveness that were not as good as they were previously. It was not a positive story and Chris and I walked out about as low as we have felt in the last 3-4 months. Tomorrow we will get the larger picture from the doctor we trust the most, but today was a huge stab in the back.
Despite the heart results, Chris continues to feel great. She honestly has a great attitude, has alot of energy, and is very positive. I think how she feels trumps what the chemical indicators show, but for me, it has been a very tough day. I'm hoping for a better day tomorrow.
1) the support group. I have now been to five of these and each f them is different. Today there were a variety of experiences/stories. A woman from California was waiting on a heart and liver transplant, a man from NC had his transplant and high dose chemo 4 years ago and was doing great, another man had just localised impact in his stomach. Many different stories, but generally all good ones. So we walked out of that feeling good about these stories.
2) met with the Boston Medical cardiologist (not the one we saw two weeks ago). To be brutally honest, neither Chris or I like this guy, and we think it is more than just the message he gives. He has the sympathy of a rock and the heart of a piece of cold steel - just brutal. So here the news was not as positive as we were hoping for. There is continued decline in Chris' heart function, specifically her ejection fraction which is a measure of pumping effectiveness. There are some "biomarkers" or measurements of heart effectiveness that were not as good as they were previously. It was not a positive story and Chris and I walked out about as low as we have felt in the last 3-4 months. Tomorrow we will get the larger picture from the doctor we trust the most, but today was a huge stab in the back.
Despite the heart results, Chris continues to feel great. She honestly has a great attitude, has alot of energy, and is very positive. I think how she feels trumps what the chemical indicators show, but for me, it has been a very tough day. I'm hoping for a better day tomorrow.
Monday, December 8, 2008
Return Visit - Day 1
Our first day back at the clinic in a long time, but it is very familiar to us and we are comfortable being there, strange as that sounds. We know may of the people, we know the building, we know the procedures, and we know all the terms. It is easy to feel like veterans having been through this previously. As before, and as we once were, there are people there that are going through this for the first time (the deer in the headlights look) and they have no idea what to expect. They have read that this is a fatal disease on the Internet and the average life expectancy. They don't know anything about the disease, the treatment, the doctors, they are just scared to death. Chris and I relate to this so well. We tried to start up conversations with the others going through their first three days and attempted to reassure them that they have found the best place to be. On the other side of the coin, we met a man today who had been diagnosed 4 years ago at the age of 70 and he also went through the stem cell transplant and high dose chemo and is doing well. So we are both givers and receivers of the all important "hope" that is the required ingredient for success.
Day 1 consisted of the following:
Blood Work and Vital Signs: Chris has many many vials of blood drawn today. They will analyze every possible aspect of this over the next 24 hours. Her blood pressure was a bit low (91/74) but not too bad. They will check her red blood cells, her white blood cells, and about 90 other unique tests. The lab results we received last time were 12 pages long.
Chest X-Ray: Chris has had many of these and to be honest I am getting a bit worried about all the radiation she must be getting. I would say she has had over a dozen x-rays in the last year on her chest/lungs. They are looking for pleural effusion - basically water in the lungs or surrounding chest cavity. This is an indication of congestive heart failure and was a problem she had pretty badly this spring. As with the blood test, we did not get any results.
Bone Marrow Biopsy: This is the dreaded big thick long needle inserted into the pelvis bone marrow. They remove a few large needles full. They are primarily looking at the % of plasma cells and the degree that their a light chain proteins in the plasma. Again, no results today.
Echocardiagram: This is the heart imaging procedure where the measure size, wall thickness, efficiency, etc. It is about a 40 minute test that they record to a DVD. Between the echo she had a few weeks ago, and the esophagus one done last Friday, they will have lots of data to look at.
Examination: This was the only meeting with a Doctor today. They asked her about a gazillion questions about her health, her medications, her activity, her emotions, and just about everything else. They were surprised to find out we went and saw the other cardiologist, but were interested in seeing his analysis. We had not met him before as he is a "fellow" new to the center, but he seemed like a good guy. I think I amazed him a bit with my knowledge of the disease, the tests, the "biomarkers", etc. I asked him questions about her BNP, her lambda numbers, her degree of left ventricle hypertrophy, etc. ( I have done a TON of research). He was a bit taken aback but acknowledged that much "homework" had been done. Nevertheless, we got no answers, only a "wait until Wednesday" response.
So the day was uneventful. We have no further insight, no good or bad results, just an ongoing level of nervousness about what we are going to hear later in the week.
I know many of you prayed a special prayer for Chris this week. It is not possible to tell you how much this means to us, but trust me, Chris feels the amount of love and good wishes being sent her way.
More tomorrow night after Day 2.
Day 1 consisted of the following:
Blood Work and Vital Signs: Chris has many many vials of blood drawn today. They will analyze every possible aspect of this over the next 24 hours. Her blood pressure was a bit low (91/74) but not too bad. They will check her red blood cells, her white blood cells, and about 90 other unique tests. The lab results we received last time were 12 pages long.
Chest X-Ray: Chris has had many of these and to be honest I am getting a bit worried about all the radiation she must be getting. I would say she has had over a dozen x-rays in the last year on her chest/lungs. They are looking for pleural effusion - basically water in the lungs or surrounding chest cavity. This is an indication of congestive heart failure and was a problem she had pretty badly this spring. As with the blood test, we did not get any results.
Bone Marrow Biopsy: This is the dreaded big thick long needle inserted into the pelvis bone marrow. They remove a few large needles full. They are primarily looking at the % of plasma cells and the degree that their a light chain proteins in the plasma. Again, no results today.
Echocardiagram: This is the heart imaging procedure where the measure size, wall thickness, efficiency, etc. It is about a 40 minute test that they record to a DVD. Between the echo she had a few weeks ago, and the esophagus one done last Friday, they will have lots of data to look at.
Examination: This was the only meeting with a Doctor today. They asked her about a gazillion questions about her health, her medications, her activity, her emotions, and just about everything else. They were surprised to find out we went and saw the other cardiologist, but were interested in seeing his analysis. We had not met him before as he is a "fellow" new to the center, but he seemed like a good guy. I think I amazed him a bit with my knowledge of the disease, the tests, the "biomarkers", etc. I asked him questions about her BNP, her lambda numbers, her degree of left ventricle hypertrophy, etc. ( I have done a TON of research). He was a bit taken aback but acknowledged that much "homework" had been done. Nevertheless, we got no answers, only a "wait until Wednesday" response.
So the day was uneventful. We have no further insight, no good or bad results, just an ongoing level of nervousness about what we are going to hear later in the week.
I know many of you prayed a special prayer for Chris this week. It is not possible to tell you how much this means to us, but trust me, Chris feels the amount of love and good wishes being sent her way.
More tomorrow night after Day 2.
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