The third day of evaluation at BU was very uneventful. I'm not sure if that is good or bad. Chris met with the Nephrologist and they went through some of her numbers which are all generally good. Chris has had some minor kidney involvement, but nothing that is significant to have a long term impact. Overall the discussion went well and then turned to Stephen's bike ride and awareness campaign. Many doctors at BU are still buzzing a little bit about and bring it up when we go and meet with them.
So hopefully today we will here the results of the team meeting. I am almost sure that the direction we will take will be wait and see. The numbers are effectively flat and she is feeling very good, so they will want to see if any thing trends in any other direction. Yet in the long term this is un unsustainable strategy from my perspective. At some point, with the knowledge that Chris did not get a complete response, action must be taken. Without a complete respoonse, further amyloid deposition occurs. It might be a slow drip, but nobody leaves a dripping faucet as is for years. I would rather err on the side of action and want to get the leak plugged. Sitting idle for another 3-4 months really does not make too much sense to me, but we'll wait to see what we hear today.
In all the meetings with the doctors, Chris asked "what should I be doing" that she may not be doing currently. This was great to hear as she is really advocating now for herself. Last year she effectively sat in a minor stupor as things passed her by. She didn't really understand what the doctors were saying and just followed the directions given. Now she is asking the questions, seeking advice, getting a better understanding. It is great to see. The answer to the question about what she should be doing is always the same: Get strong, keep exercising regularly, eat well, and try to avoid getting sick. Efffectively her primary focus is the same it has been for the last 12 months - conditioning. Chris has been very good about that, but sometimes her now busy schedule gets in the way, like all of us. She has to make it a priority.
In the past, the famous "Friday Calls" have been major milestones. Does she have amyloidosis, yes or no? Will we do a Stem Cell/High Dose Chemo regime? Have we seen any progress since the transplant? Todays call will be uneventful I believe. Wait and watch.
On Monday we see Dr Falk to get the cardio perspective. We will hopefully know the new Ejection Fraction number and the left ventricle wall thickness. IF these are improving, that will be real cause for celebration. We have asked and been asked many times - "Can the heart repair itself ?" As I have stated before, there is no clear understandfing of this, no emperical evidence, but it has happend over time. If Chris keeps exercising hard, I personally am convinced that she will be living proof that her heart has strengthened.
Chris and I continue to read the other blogs of amyloidosis patients and relatives. Our prayers are with them and their families every day. I also continue to watch the clinical trials very closely. The two that are of most interest are Pomalidomide (CC-4047) and what is reffered to as V-MD which is a cocktail of Velcade, Melphalan, and Dexemethosone. They each seem to have very positive results. For those interested you can search on these terms with amyloidosis and see much of the updated information.
Thanks again for all the interest.
Friday, July 31, 2009
Tuesday, July 28, 2009
Day 2 Visit - 1 Year Anniversary
Today went relatively well but a little disappointing for Chris and I who remain very optimistic generally. We have some of the numbers back and the important one, the lambda free light chain is now at 40. The last 4 readings have been 42, 41, 32, and today 40. Normal is 15-26 and Chris was close to 400 at one point. So the good news is it remains close to the norm vs where she was a year ago (the 94% response). The disappointing news was that her numbers did not go down to normal range. Effectively she has "plateaued" at the 40 level. Her 24 hour urine measurement is still going down, and that is good, but it also is not within the normal range - but again it is still trending down which is good. So we have come the realization that at one year out she did not get a complete response, which is what we had been praying for. But 94% is pretty close. So the big question - What do we do now? The doctors all say she is doing great clinically (direct observation) and clearly she is gaining weight, has much more stamina, and is feeling very good. The Pulminologist, Dr Burke, is very pleased overall with her lung improvement. She has no fluid in the lungs and the overall "definition" from the x-ray is very good, so he was happy. He commended her on her progress in exercising and gaining strength and her overall fitness. He did say that she has a "generous heart" which figuratively means that her heart has enlarged in order to compensate for it's reduced function over the last year. Yet we know that her heart has actually lost some of its thickness and he considers this "outstanding".
Chris also had an echocardiagram today which of course we will not have results until later in the week. We are looking for improvements in the ejection fraction (EF) from it's current reduced capacity. With her reduced BNP number and the reduction in her wall thickness, we might possibly see overall EF improvement, but that may be wishful thinking on my part. Dr Falk, the amyloidosis cardiac specialist has told us that there is no direct evidence of improved EF after Stem cell/high dose chemo treatment, but with so many positive signs we are optimistic. We meet with Dr Falk this coming Monday, with all of our tests results and a DVD of the echo. He will then give us his perspective on any improvement.
We discussed with Dr Burke the potential as to whether Chris might be a candidate for a second stem cell transplant. Of course he was non-committal (as he should be), effectively saying that we should cross that bridge when we come to it.
The normal Tuesday noon support group meeting was another reconfirmation of my now solidly held beliefs:
-Overall the medical community is grossly unaware of amyloidosis
-Having an aggressive patient advocate is critical, you can't fight this alone
-The only way to beat this thing is by working with the best in the world, such as BU and Mayo
We heard many stories where patients complained of symptoms for as long as a few years and they were all being treated for the symptoms, not the cause. If they ended up with an Oncologist they were treated for a blood disorder, if they ended up with a Cardiologist they were treated for heart conditions, if they were treated by a general practitioner they were diagnosed with fatigue, menopause, of just general aging. There was anger in the room as to the amount of misdiagnosis. Yet who is to blame? Doctors can't know everything, they are specialists at what they know, that is good. The issue is the general lack of awareness or the unwillingness to explore outside of their comfort zone. Every patient in the session today said effectively the same thing, they told their doctor that "something is wrong", in some cases many many times. Chris used to say it all the time until diagnosed and it was only the amazing luck of reading the article about Jen Gershman that Chris was diagnosed. Jen was committed to improving awareness and she was highly successful. Chris is now focused on the same thing. But again, I restate what was confirmed today: be aggressive, find a strong and organized advocate, and work tirelessly to improve awareness of this disease.
So for Chris, she has another day of test tomorrow (kidney and liver) and then we wait for the famous phone call on Friday. Yet I already know what the next step will be - wait and watch. My prediction is that the BU staff will want her back in November/December for more data points and trending (blood and urine tests). In the meantime, Chris' most important role is the same as it has been since last August - get as strong as possible. Exercise, lift weights, improve cardio conditioning, etc. If she needs another round, she will have to be very strong as this will give her the most number of options.
So overall Chris feels great, is getting stronger, and is generally living a very good quality of life. Yet it is clear we did not get the complete response we hoped for. Chris fought a very good battle and beat the enemy severely, but it is not dead yet. We don't know how badly these enemy proteins are wounded and what they are doing back to Chris, so we may need to go to battle again. So as of July '09, we wait and watch carefully for any signs of enemy movement. We will get the Chiefs of Staffs (the BU Team) back together again at the end of the year and reassess the situation. In the meantime Chris has to continue to strengthen he defense posture. (Sorry, I must have watched a war documentary recently with all these war analogies!!!). Chris and I know the war doesn't end until we get a complete response, so we can't declare success, but without any doubt in my mind, I am sure that Chris will be the victor. She is that tough.
More tomorrow, thanks for caring.
Chris also had an echocardiagram today which of course we will not have results until later in the week. We are looking for improvements in the ejection fraction (EF) from it's current reduced capacity. With her reduced BNP number and the reduction in her wall thickness, we might possibly see overall EF improvement, but that may be wishful thinking on my part. Dr Falk, the amyloidosis cardiac specialist has told us that there is no direct evidence of improved EF after Stem cell/high dose chemo treatment, but with so many positive signs we are optimistic. We meet with Dr Falk this coming Monday, with all of our tests results and a DVD of the echo. He will then give us his perspective on any improvement.
We discussed with Dr Burke the potential as to whether Chris might be a candidate for a second stem cell transplant. Of course he was non-committal (as he should be), effectively saying that we should cross that bridge when we come to it.
The normal Tuesday noon support group meeting was another reconfirmation of my now solidly held beliefs:
-Overall the medical community is grossly unaware of amyloidosis
-Having an aggressive patient advocate is critical, you can't fight this alone
-The only way to beat this thing is by working with the best in the world, such as BU and Mayo
We heard many stories where patients complained of symptoms for as long as a few years and they were all being treated for the symptoms, not the cause. If they ended up with an Oncologist they were treated for a blood disorder, if they ended up with a Cardiologist they were treated for heart conditions, if they were treated by a general practitioner they were diagnosed with fatigue, menopause, of just general aging. There was anger in the room as to the amount of misdiagnosis. Yet who is to blame? Doctors can't know everything, they are specialists at what they know, that is good. The issue is the general lack of awareness or the unwillingness to explore outside of their comfort zone. Every patient in the session today said effectively the same thing, they told their doctor that "something is wrong", in some cases many many times. Chris used to say it all the time until diagnosed and it was only the amazing luck of reading the article about Jen Gershman that Chris was diagnosed. Jen was committed to improving awareness and she was highly successful. Chris is now focused on the same thing. But again, I restate what was confirmed today: be aggressive, find a strong and organized advocate, and work tirelessly to improve awareness of this disease.
So for Chris, she has another day of test tomorrow (kidney and liver) and then we wait for the famous phone call on Friday. Yet I already know what the next step will be - wait and watch. My prediction is that the BU staff will want her back in November/December for more data points and trending (blood and urine tests). In the meantime, Chris' most important role is the same as it has been since last August - get as strong as possible. Exercise, lift weights, improve cardio conditioning, etc. If she needs another round, she will have to be very strong as this will give her the most number of options.
So overall Chris feels great, is getting stronger, and is generally living a very good quality of life. Yet it is clear we did not get the complete response we hoped for. Chris fought a very good battle and beat the enemy severely, but it is not dead yet. We don't know how badly these enemy proteins are wounded and what they are doing back to Chris, so we may need to go to battle again. So as of July '09, we wait and watch carefully for any signs of enemy movement. We will get the Chiefs of Staffs (the BU Team) back together again at the end of the year and reassess the situation. In the meantime Chris has to continue to strengthen he defense posture. (Sorry, I must have watched a war documentary recently with all these war analogies!!!). Chris and I know the war doesn't end until we get a complete response, so we can't declare success, but without any doubt in my mind, I am sure that Chris will be the victor. She is that tough.
More tomorrow, thanks for caring.
Day 1 visit - 1 year anniversary
A few things to provide updates on, all overdue. First an update on Chris' trip to California last week. This was a first big test for her as to how she would be able to handle the travel, the crowds, the time zone, etc. and overall she did amazingly well. We were heading out for Stephen's Junior Elite Triathlon Championships in Oxnard - 60 miles north of LA, for 4 days, and despite incredible troubles getting there, she did great. We finally got to bed at 2:30 am (5;30 eastern of course) and while I thought she might be exhausted from the delays, rerouting, and general trouble, she was up the next morning and taking long walks along the beach. I think she even surprised herself. She enjoyed getting away, we had a great suite right at the triathlon site on the beach, and enjoyed the great company of the other athletes and parents who also came out. For me, it was proof that she really has come a long way to getting back to normal and can easily deal with the normal bumps in the road - something we could not even fathom a year ago at this time. Chris continues to walk daily and go to the gym or yoga almost every weekday. And with three kids "mostly" home this summer, she is very busy trying to keep order in the house.
So this was the status going into our first day of three at the BY Amyloidosis Center for her one year check up. We have been so busy we really didn't spend much time preparing ourselves - for either good or bad news. We know in general she has been doing very well, but as stated, we need to know what the numbers say. Day 1 overall was reconfirmation of what we thought, but these were all based on conversations. She had her blood work done (11 vials) and her 24 hour urine collection, but we will not get those numbers back until later in the week. We are of course looking at the free light chains in the protein in the urine as the critical bio-markers.
We met with two doctors including Dr. Sanchorawala. Both evaluations were very positive. Chris has no major issues that can be observed other than an occasional bout with GI problems that occur randomly. She explained that she generally feels great, exercises often, is gaining weight. She was told last summer when she was around 92 lbs. that her ideal weight she should target is 115. Yesterday she was 114.9 - and thus she felt good about getting a Dairy Queen Blizzard on the way home (doctors orders!).
Melissa joined us all day and found the whole process interesting, the discussions with the doctors, the conversations with the other patients (including the brand new, clueless, dear in the headlight folks - we used to be them), and all the testing they do. As a graduating nursing student next summer, it was another opportunity to see more medicine in action.
So we finished the day with two major realities. First is that unless we see surprising good numbers later in the week, it is clear that Chris did not get the Hematologic Complete Response (HCR) that we were hoping for. Dr. Sanchorawala ultimately decides at the one year mark whether a patient had an "HCR" and she told us that she doubted she would be able to identify that as the result for Chris. Her numbers all need to be within normal range and as stated in previous posts, they aren't. Yet they are close. Normal for free light chains is 15-26 and she is in the high 30's (from over 400 last summer). As such, she had a 94% response and as discussed earlier, the big question is "what is the tipping point" for action? If it was a 99.9% response, probably we would d nothing. If it was a 40% response, we would jump on the next course. But what do we do at 94%? Of course I asked that question many times and it is becoming clear that the answer is frustratingly the same as before - "let's wait and watch it". I told Dr Sanchorawala that I would predict the course of action prescribed at the end of the week - I predicted "come back in 3 months for more tests and evaluation". She smiled and nodded as she knows that is exactly what we will here. That however is frustrating but also good. Frustrating in that we want to take action, "out damn spot" as Lady Macbeth would say, let's finish this thing off. Yet good that right now no action is required - "no news is good news". Thus we have the raging battle of bad quotes.
The other major outcome of the day was a new awareness or insight that her heart is getting better and stronger. The thickness of the left ventricle wall (septal) has decreased (less stiffening) from 17 mm to 12 mm. This is really surprising and good to hear. Also her BNP number (used to measure the amount of protein produced by the heart) is way down (this is good), but still above normal levels. This is heading in the right direction and is confirmation that her heart is getting stronger - no doubt to her commitment to exercise. Yet the most surprising element of this improvement is that unlike what we had heard before, Chris might be able to tolerate a second stem cell transplant. We had been told earlier "no way" as her heart would not be strong enough. Yet now, that may in fact be an option. Of course we have no idea what the future plans are until we get the numbers back later in the week and the BU team discusses options.
We will go back today for more evaluations and the famous support group discussion every Tuesday. Based on who we met yesterday, I think many people will be looking at Chris and saying "wow, she looks great" and will get a dose of hope and optimism they need.
More updates tomorrow. Thanks for being interested and caring.
So this was the status going into our first day of three at the BY Amyloidosis Center for her one year check up. We have been so busy we really didn't spend much time preparing ourselves - for either good or bad news. We know in general she has been doing very well, but as stated, we need to know what the numbers say. Day 1 overall was reconfirmation of what we thought, but these were all based on conversations. She had her blood work done (11 vials) and her 24 hour urine collection, but we will not get those numbers back until later in the week. We are of course looking at the free light chains in the protein in the urine as the critical bio-markers.
We met with two doctors including Dr. Sanchorawala. Both evaluations were very positive. Chris has no major issues that can be observed other than an occasional bout with GI problems that occur randomly. She explained that she generally feels great, exercises often, is gaining weight. She was told last summer when she was around 92 lbs. that her ideal weight she should target is 115. Yesterday she was 114.9 - and thus she felt good about getting a Dairy Queen Blizzard on the way home (doctors orders!).
Melissa joined us all day and found the whole process interesting, the discussions with the doctors, the conversations with the other patients (including the brand new, clueless, dear in the headlight folks - we used to be them), and all the testing they do. As a graduating nursing student next summer, it was another opportunity to see more medicine in action.
So we finished the day with two major realities. First is that unless we see surprising good numbers later in the week, it is clear that Chris did not get the Hematologic Complete Response (HCR) that we were hoping for. Dr. Sanchorawala ultimately decides at the one year mark whether a patient had an "HCR" and she told us that she doubted she would be able to identify that as the result for Chris. Her numbers all need to be within normal range and as stated in previous posts, they aren't. Yet they are close. Normal for free light chains is 15-26 and she is in the high 30's (from over 400 last summer). As such, she had a 94% response and as discussed earlier, the big question is "what is the tipping point" for action? If it was a 99.9% response, probably we would d nothing. If it was a 40% response, we would jump on the next course. But what do we do at 94%? Of course I asked that question many times and it is becoming clear that the answer is frustratingly the same as before - "let's wait and watch it". I told Dr Sanchorawala that I would predict the course of action prescribed at the end of the week - I predicted "come back in 3 months for more tests and evaluation". She smiled and nodded as she knows that is exactly what we will here. That however is frustrating but also good. Frustrating in that we want to take action, "out damn spot" as Lady Macbeth would say, let's finish this thing off. Yet good that right now no action is required - "no news is good news". Thus we have the raging battle of bad quotes.
The other major outcome of the day was a new awareness or insight that her heart is getting better and stronger. The thickness of the left ventricle wall (septal) has decreased (less stiffening) from 17 mm to 12 mm. This is really surprising and good to hear. Also her BNP number (used to measure the amount of protein produced by the heart) is way down (this is good), but still above normal levels. This is heading in the right direction and is confirmation that her heart is getting stronger - no doubt to her commitment to exercise. Yet the most surprising element of this improvement is that unlike what we had heard before, Chris might be able to tolerate a second stem cell transplant. We had been told earlier "no way" as her heart would not be strong enough. Yet now, that may in fact be an option. Of course we have no idea what the future plans are until we get the numbers back later in the week and the BU team discusses options.
We will go back today for more evaluations and the famous support group discussion every Tuesday. Based on who we met yesterday, I think many people will be looking at Chris and saying "wow, she looks great" and will get a dose of hope and optimism they need.
More updates tomorrow. Thanks for being interested and caring.
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