Update on Meeting with Dr. Falk

Chris and I met with the cardiologist whom we like and believe to be the world expert on cardio impact from amyloidosis. He basically told us a few things:

-Chris continues to look good "clinically", i.e. through direct observation. No edema, no lung issues, good overall appearance.

-Her heart really has not changed much in the last 9 months. No reduction in ejection fraction, no further wall thickening, no other major changes. He did confirm that echocardiagrams were highly subjective and he is becoming more convinced of that over time. I found that very interesting.

-We discussed at length the options going forward. I asked specifically what would be the plan if Chris stabilizes at this 94% response (vs a complete response). He did not want to step into the role of the hematologist, and was really stating that BU needs to decide that next step with us. He did say, that the chance of a relapse is very high if in fact there is no complete response. This could happen soon or it could be a few years, nobody really knows. So this confirmed my strong belief that we need to continue to be aggressive with treatment as we try to get a complete response. We have all come to the conclusion that her heart is not strong enough for another stem cell transplant. This leaves oral chemo as the options, either Velcade or Revlimid. Dr. Falk indicated Velcade seemed to have a good success, but was a little more toxic. Again, he is going to leave the go forward strategy to BU.

-We also discussed the option of a defibrillator being implanted. He had mentioned this in an earlier discussion and has now ruled it out as not required. This of course is good news.

So overall, things remain stable, no further decline in heart health, but as we still have an abnormally high lambda light chain number of 40 (vs 26 as normal), there is still work to determine how to get a complete response. Chris goes back for another round of blood work in May and we will see what those numbers look like.

A few other happenings:

-April 4th was one year since Chris received the phone call from her doctor while she was playing tennis. Her doctor came in from vacation to meet with her personally and explain that something was wrong. At that time we did not know what we were facing, but we were very concerned. We heard all these words that we had no idea what they meant: mgus spike, Waldenstroms, Multiple Myeloma, amyloidosis. I was visiting Hartwick overnight and spent the entire night searching the internet for what I could learn. The worst part was reading that the average life expectancy for newly diagnosed amyloidosis was 13-15 months. I cannot describe the feeling and I pray I never experience it again. It would take a book to describe all my thoughts, blurring by my head at warp speed, and the complete fear I had. I got home and Chris and I cried all night long. So one year later, I feel as if we have dodged a bullet. Shots might still be fired, but we ducked one so far. We are still in the combat zone, yet we are running fast to find safe haven (a complete response). I know how lucky we are, and I can clearly state that we are grateful everyday. There are so many other stories of courageous battles fought by others. Some have been won, but the ones that break our heart are the ones that have been lost. We heard so many stories from people who donated to Stephen's ride. People wrote, "my brother succumbed to this", or "my husband was not diagnosed early enough and lost his battle". Just as I pray and am thankful for Chris' progress, I also pray for the strength of those who have lost loved ones to this disease. In many cases we know your names, we read the blogs, we see what others are going through, and our prayers are with you.

-I pushed Chris very hard to go skiing on April 4th. I really wanted her to get back into a normal sort of life. Get out, enjoy the snow, get some sun, and have some fun. I thought this would be an emotional milestone for her on her one year anniversary. She could conquer another roadblock. Walking up the driveway was a milestone. Driving a car by herself was a milestone. Helping others with a need was a milestone. I was hoping she would really enjoy a few hours of very slow, careful skiing, which she and I have enjoyed all over the US and Europe for years. Unfortunately it did not happen. The snow was very heavy, it was spring skiing with some bare spots, and she did not feel comfortable doing it. I really wanted her to push herself to do this, but I understand why she chose not too. Hopefully next year.

-One area that Chris now has to focus on is her cholesterol. This has risen over the last few months above normal. She has been trying to put weight on and has indulged in some great but bad foods. Kim's brownies, Caroline's chocolate mousse, unlimited ice cream - all with the intent to put some meat on her bones has now raised her cholesterol (and put pounds on me as well). So now we have the difficult task of reducing cholesterol while gaining weight. The food brought by friends has been the best. These people can cook!!. Yet now it is back to reality on the food selection. I take this as a good sign. Before, the BU doctors were saying that cholesterol was the least of her problems. Now, as she needs to focus on it, it means that we are thinking longer term. This is good.

-I will not steal Stephen's thunder on his fund-rasing efforts, but I will say that he, through all of you, has blown away the objective he set by an order of magnitude. It is beyond amazing. The letters, the donations, the inspiring stories have all been just incredible.

-Chris and I hope to get a short vacation away soon. She did not get to go to Florida, and did not get on the ski's, so now we will try to do some fun travelling and look back on a hell of a year.

Thanks again for everything.

Lung Improvement

Chris had a CT scan and a number of doctor appointments last week to have her severe coughing looked at. In the end, it seems as if she had a case of both bronchitis and some pneumonia in her left lung. As an infection, she could have picked this up anywhere, but the stronger antibiotic seems to have made a difference over the weekend. She has almost stopped her continous coughing and thus is not coughing up blood either. Her doctor and the CT scan showed something called "ground glass" in her lung, which is basically scarring. In the end, this does not appear to be a long term problem and should resolve it self shortly.

Unfortunately Chris was unable to go to Florida to visit her parents. She really deserved a few days sitting in the sun, relaxing, warming herself up after the winter, but better safe than sorry. It is always a tough decision, the balance between being cautious and living your life. We can't hide in a cave the rest of her life, and she deserves to enjoy a normal existence. Yet that comes with risks of infection, and other issues. I am encouraging her to start getting back to normal, with travel (short distances for now), normal activities (hopefully some easy skiing this coming weekend), and continued daily exercise. She is anxious to get out in her gardens and I know she is looking forwared to summer, which she had none last year of course.

Chris did get a front page picture of her hugging Stephen in the local paper last week from his arrival home from the bike trip. She of course hates it, but it shows her great happiness and strong emotion as she greeted him after his incredible ride. So many peopl have been inspired by this story. Stephen received a very powerful letter, with a very nice donation to the Amyloidosis Research Foundation from a woamn who's husband passed away primarily due to lack of a diagnosis. As she stated, they kept treating the sympoms and not the cause, thus delaying possible treatment. By the time he was evaluated at BU Medical, he had lost an opportunity for aggresive treatment. This is exactly why awareness is so important. With the funds Stephen has been raising, donated by so many incredibly generous people, it is his intent to help prevent these stories from happening. Of course, like any rare disease, it will be a continuing battle.

Hopefully I can get Chris out on the slopes this weekend, and hopefully my plan to hit a few tennis balls with her comes true soon. She has been through an incredible year, and it is time to expand the horizon a little bit before we go back for the big 12 month evaluation in June.

Thanks for your ongoing support, interest, and well wishes to Chris. It means a great deal.

An amazing day....

Yesterday, March 22, was a great day for many many people. It was a great day for Chris and I, and our entire family, it was a great day for those impacted by and working to cure amyloidosis, it was a great day for an incredible community of supporters, and it was a great day for Stephen and Aislinn.

Stephen and Aislinn arrived home after a grueling 1500+ mile/15 day bike ride from Jacksonville, Fl. to Holliston, MA. It was a once in a lifetime experience for them and all those who participated. They had good days and tough days, they met many great people, they promoted awareness, and they raised a staggering amount of money. Every dollar they raised, over $40,000 and still climbing, is going directly to the Amyloidosis Research Foundation. All expenses were covered by sponsor support, or frequent flier points, or the generosity of hosting families, so not a single dollar raised will go anywhere but directly to research.

There was a crowd of over 100 people when Stephen and Aislinn arrived yesterday. Friends from Holliston, schoolmates,faculty, and staff from Worcester Academy, family from PA, NJ, NH, fellow patients, and leaders in the Amyloidosis community such as Mary O'Donnell and Dr. Comenzo, and fellow bike riders from MinuteMan Road Club. They were both stunned at the reception and had no idea how many supporters across the community they had.

Stephen and Ailsinn learned many valuable lessons that they will post in their blog when they catch their breath. The biggest lesson I think we all have learned has been the incredible generosity from so many. It is mind-numbing. In this economy, with so many people worried about their financial stability, their savings impacted, and their future unknown, the number of people who made a contribution was just incredible. Every single donation meant a great deal, whether it was $5 from a child to an incredibly large, and anonymous donation (I do not know who that was, but I pray you walk with a personal pride of your great generosity and contribution). The community that we live in, all of our friends, all of my colleagues at IBM, all of those who gave becuase they suffered or lost loved ones from this disease, all of those who gave in any way were incredibly generous. As I have posted many times, just as "it takes a village to raise a child", it takes a village, an extended community, to fight a disease. Chris so far is very fortunate, but others have lost loved ones to amyloidosis. A year ago the vast majority of us had never heard of this disease. Now we are battling it on many fronts, but with an incredible village of loving, caring, supportive, and generous people with us.

I am anxious to read Stephen's and Aislinn's postings, but I am also anxious to say THANK YOU to everyone who has helped with Chris's journey to health.

Thanks to all of you so very very much.

Home again...

Chris was sent home from her night in the hospital late Saturday afternoon. She has a combination of bronchitis and early pnuemonia caused by a viral infection. She has prescriptions for a very strong antibiotic, an inhaler for opening the bronchial tubes, and the old standby Robitussin with Codine for her cough. Basically she got a bad viral infection that we did not treat quickly enough and it got into her lungs. Her coughing has irritated her bronchial tubes so much that she was coughing up blood and the coumadine blood thinner did not help. She felt well last night and is sleeping soundly. Hopefully Chris bounces back quickly as she plans on visiting her parents down in Tampa for a few days this coming week with Abby. The sun, some sitting around the pool and some sleeeping in should help her feel better.

One interesting phenomenon which I test all the time is whether the doctors seeing Chris know anything about amyloidosis. Neither the emergency room doctor nor the internist on the floor knew anything about it other than "maybe" hearing about it in a lecture in medical school twenty years earlier. In fact the ER doctor stated that Chris really has Multiple Myeloma and not Amyloidosis which we know is completely false. I cannot blame the doctors, they can only know so much, but raising awareness is so critically important.

The lesson learned here is that when she starts to feel a cold or cough coming on, it needs to be treated. She has had such a cold free winter and I think we got comfortable that her cough would pass. Obviously a big mistake that put her in the hospital for a night.

Stephen also has a homecoming today as he completes his 1500 mile ride from FL to MA today. We expect him to be arrving at the Holliston Congregational Church at approx. 2:00 this afternoon with a good crowd to welcome him home. Abby and here friend Kim left earlier this morning to take 3 others out to join Stephen in Windsor Locks and others will be joining the ride as they get closer.

His commitment to raise funds and awareness has been nothing short of spectacular. Yet the help he has received along the way has also been amazing. Donations, sponsorships, blog postings, etc. have have all been very motivating for him. He gets to do the thing he loves (riding his bike) and helps others who have suffered or lost a loved one from this disease. His is a small contribution towards what needs to be done, but he knows he can make a difference by raisng awareness. Today will be a very special day in his life. He is very tired, he aches everywhere, he is mentally spent, but he made a plan months ago and he will complete it in great style. His friends will pull him home today, but figuratively he will be pushing research and awareness. He has met so many people and heard so many stories in the last few weeks, that he is riding with a purpose that will sustain him all the way to the finish.

I'll keep the updates on Chris coming. Getting her to Florida for a few days is the next goal. She has earned it.

Thanks for all the ongoing support and interest, it means more than I can say to Chris.

Back in the hospital...

Chris has not been feeling well over the last week with a very bad cold/cough and a very congested head. She really started feeling poorly with this bad cold more than a week ago and unfortunately delayed contacting her primary care physician. She finally did last Tuesday and was put on an oral antibiotic, but unfortunately that has not seem to do the trick. Yesterday she went for an evaluation with with her primary and they decided to go ahead and get a chest x-ray. Chris had suddenly started coughing up blood yesterday (Friday) and this and the x-ray caused her to be admitted last night to Newton-Wellesley Hospital. There is nothing serious right now as it is more for observation than anything else, but they wanted to give her a stronger antibiotic and they need to balance all the medications with what she is currently taking.

Her fantastic friend Sally spent much of the afternoon and evening (and even into early morning) with her until I was able to relieve her. I drove back from Hyde Park where I was traveling along with Stephen and Aislinn on their FL to MA ride and got to the hospital around 12:30 am.
Chris seemed to be comfortable, but was tired and still coughing up some blood. I am glad we have her being watched, as I strongly believe in the motto "better safe than sorry". We fully expect that she will be released sometime time today and that the stronger antibiotic kicks in.

Chris has been planning Stephen's return home to Holliston for Sunday afternoon and has things pretty well under control. I know she will be very excited (and relieved) to see him pulling into downtown Holliston on Sunday. Both Aislinn and Stephen have had a fantastic ride, probably a life altering trip, and have met Amyloidosis patients, raised awareness, raised funds for research, and had the adventure of a lifetime. What a journey for them!!!

I'll keep Chris' status updated here, but I do expect her out of the hospital today. I know that nobody could keep her out tomorrow.

Update from BU

Chris and I heard from BU yesterday and we heard much of what we knew as well as some new good news. Chris' biomarkers continue to go in the right direction, but as I have posted, we are not over the finish line by any means. I won't repeat all the numbers, but they are all looking good. The frustrating one is the lambda light chains kind of stuck at 40. We really want this down the the mid to low 20's and we will feel much better. The good news is that (and this is a very subjective read) is that the her left ventricle wall thickening may have reduced. BU is a currently doing research on the relationship of left ventricle wall thickness and whether there is evidence of actual reduction as patients stop further amyloid protein deposition. In other words, can the heart repair itself? We have asked doctors the various cardiologists we have met with that exact question and the answer is always something like "there is some evidence of that but it is not proven" or "that is exactly what we are trying to understand". Chris' ejection fraction came in at 37% on this reading. We have seen some readings much lower, but 37% is a number she can live a good life with if it can be maintained. What is important is that it does not get worse. Thus my aggressive pursuit of getting to a complete response, even if it means an oral chemo regiment this coming fall. Chris goes back for more blood work on April 28, the full 3 day analysis in the end of June, and then we will know much more.

In the meantime, she has a terrible cold and has had some bad GI issues the last few days. Visiting our friends in DC, she was really very disappointed as she had to lie down and was sick to her stomach often. We are trying to figure out what may have caused her digestive problems, but I am becoming convinced that the Quarter Pounder with fries on the NJ Turnpike did not help. Twice in the last month she has become ill and both times she hit fast food restaurants. This is my fault as we have learned that she can't get to a point of hunger ans when she does, we have to jump on the first food we find. We have been caught off guard when we travel and not prepared with good food. I'll get better at planning.

Her cold is very bad. Certainly the worst she has had since last year. She now is on an anti-biotic, and hopefully this will help soon. Her coughing and hacking and runny nose are very bad, but she is fighting through it.

Chris rode with Aislinn in the car from DC and was amazed at how coordinated Stephen's ride is. Aislinn has this all under control. Yet I know how hard this all is in terms of navigating Stephen's ride. You miss one turn and you can end up being miles off target. I think Aislinn is the unsung hero as she has everything ready to go, thinks ahead, plans for problems, etc. These two teenagers are learning things about managing a large project that I did not understand until well into my business experience. This ride is a potential logistics nightmare, and it is going along almost flawlessly. The focus should be on the awareness and fund raising that they are focused on, and not the ride itself. Aislinn's job is to allow that to happen without problems and she is doing it incredibly well.

I personally plan on riding with Stephen a few more times this week. The ride in DC went well ( no orthopedic surgeries required) and I think I did pretty well at keeping the pace going. The worst part was riding through a tunnel, on a 12" wide sidewalk, against oncoming traffic, with my shoes clipped in to the pedals. As Bill said, "at least I didn't soil myself". Other than that the ride was beautiful and a great deal of fun to be out riding with Stephen.

So overall, some good news from BU. Chris is heading in the right direction, more waiting to do. She needs to focus on exercise and building some muscle mass and conditioning which she will continue when she gets home. We have to get rid of this very bad sinus infection first and hopefully the anti-biotic will kick in today.

I'll post this weekend after we get Stephen and Aislinn back home. Thanks for checking in and caring so much. I can't tell you all how much everyone's support means to Chris and me.

Newspaper article - more awareness

Here is a link to a newspaper article that was published today down in NC. Aislinn, Andy, and Stephen met with Judi Collins and a reporter and this was the result.
http://www.jdnews.com/news/stephen_62967___article.html/collins_disease.html

One note of interest, Stephen normally does not ride in socks and sandals. I am sure he takes his bike shoes off as frequently as he can.

Day 3 Return Visit

We met with the Nephrologist (kidney specialist) today and went through some of the numbers that were available. Based on what she had seen come in, Chris' numbers all look good. She went through a good analysis as to how the amyloid proteins limit the kidney function and how they allow protein to get into the urine. Protein leaving the body is generally bad and an indication of amyloidosis, along with other causes. One of the indicators of protein in the urine is "foamy" urine that is very bubbly. It can also be an early indicator of diabetes so it should always be analyzed if there is foaminess (sorry to be so focused on urine tonight). The other biomarker is the amount of creatinine. Creatinine is the waste by-product of muscles and if too high indicates renal damage. Chris' number is .64 and the normal range is .5 to 1.0. So again this is very good. The doctor also expected that the 24 hour protein number would continue to decrease over time.



What we need is the total package of results which we will not have until next week. The normal procedure is that we get the call on Friday afternoon with the next steps. For this time, we will not hear until Tuesday or Wednesday.



They do want Chris to do another set of blood test (not the doctor visits) and she will do that on April 28th. Then she has her 12 month evaluation at the end of June. What we heard today was that it would not be until the June meeting that they declare what kind of response it was (complete or partial) and what the next steps are. So this is going to be slower than I would like but they need the full year to understand the total affect of the stem cell replant and chemo.



So, as I said yesterday, we are running in the right direction, but we do not know if and when we will cross the complete response finish line.

One thing that is really good is that the Director of the BU Amyloidosis Research Clinic asked us to meet with her in her office. She said that there had been a lot of talk about what our son was doing on his bike ride around the center and she wanted to tell us how amazed they are and supportive they want to be. They have invited Stephen and Aislinn to come and get the "VIP" tour of the center, meet with the doctors, see the labs, etc. I am wondering if maybe they might give a quick presentation to the medical staff on a Friday when they all meet. They will have great pictures and video's (and will get more there) and it would be a great dry-run for their presentation back at Worcester Academy.

So no real news until next week when they call with their final packaged results. We are pleased with the direction but would like to have all the biomarkers at or beyond the target. We are not there yet.

Your donations to the Research Foundation keep coming in. Thanks for all your support, your continued calls to Chris, your prayers and well wishes. We both believe that they are making a huge difference. In the meantime, Aislinn and Stephen march north. They have some great stories to tell, so their blog will be much more interesting for the next few days than mine.

More next week.

Day 2 Return Visit

More tests today and some relatively good news. Chris had another echocardiagram today ( I think this is about the 8th one in the last nine months). We have learned that reading these is clearly very subjective so each person that analyzes the echo measures, analyzes, and sees different things. Thus it is most important to a) have progressive readings over time and b) have the same person do it. In our case, we are most comfortable with Dr. Falk as he has so much experience and has written so much based on his own research. We will have todays test sent prior to when Chris meets with him in April.



We also participated in the weekly support group meeting. I have written about this before. There are many stories told around the table; people who had a stem cewll transplant six years ago to those who are being evaluated for the first time. I have attended many of these and I know all too well the feeling of being overwhelmed by the words, the science, the numbers, all this stuff that people talk about and as a new participant you just sit like a log with an internal fear that is eating you up. There has to be a better way to help the newcomers than exists today. Just like we were, they are scared to death, they are trying to understand what on earth is going on, and they bombarded with all these tests and words, and complex things that just spin around and want to make your head blow up. Chris and I were shaking as we drove home after our first few days. This can be done better, I am sure. It is very clear that the strength, the willingness to learn, and the organization of the caregiver/advocate is probably as important as anything. I have seen caregivers who love their spouses or family members dearly but in no way are they capable of navigating the maze. They are conmforting, they hold their spouses close, but they are not prepared to be advocates, at all. Yet today I saw others who were ready for the battle. They asked a ton of questions, listend very intently, asked more questions, and learned from the others who went first. They are right on top of things, keep every test result, and do their own research. They will be both great caregivers AND great patient advocates.



More specifically for Chris, here are the numbers we have and my thoughts:



Lambda Light Chains: June '08=> 600+, December '08=> 40, March '09=> 40

This means no change in the amount of lambda light chains in her body. Good news is no increase, bad news is we are not at the target yet, which is under 21. This could be better, and needs to be.

Protein - 24 Hour Urine collection: June '08=>3800, December '08=>2800, March '09=>1800

This is clear continued improvement and of course is trending in the right direction. It is expected that this will continue to improve over the next few months. The target is about 150, so while there is clear improvement, there is more to go, which should happen.

Kappa/Lambda ratio: June '08 =>N/A (off the chart). Dec '08=> .2, March '09=> .29
This is very good, the target is above .25 and she is there. There are many ways to analyze the ratio, but when the lambda number is going up and the ratio is going up, this is a good thing.

BNP: June '08=> 269, Dec '08=> 286, March '09=> 204
This is the measure of congestive heart failure (CFH). Anything above 100 is a sign of some CFH, but Chris' is going down. This is good. The higher the BNP, the lower the cardiac output and the lower the blood volume pumped. This is trending down so we are pleased.

There are just some of the many tests, but here is how I sum this up:

Things are progressing in the right direction, they are generally getting better, but we are not over the finish line of a Hematologic Complete Response (the famous HCR). We want to reach the HCR objective. Tomorrow we do some more test, primarily kidney function, and then we will get our final report next week. They have already told us that they will want her back for blood work (not all the evaluations) in 4-6 weeks.

I have told them that when they think of Chris, they need to think of one word; aggressive. I do not want further protein deposition and we know how important getting an HCR is to life expectancy. I get the sense that some of the doctors at BU are almost saying "it's really good, stop worrying". Not me, I will only be comfortable when I hear the doctors say " congratulations, you have a complete response".

If Chris does need another round of chemo, we all agree that it would be after the summer. We lost the entire summer last year and we want to enjoy one this coming season. Chris would start in September. But, let's see what the final report says when we get it next week.

Another update tomorrow I hope. Thanks for checking in, it means a great deal to us.

Day 1 at Boston Medical return visit

Things went well at BU today, for the little we learned. Chris had the 24 hour urine collection, lots and lots of blood taken, and a chest x-ray. We did not get all the results of course, but the chest x-ray results were very good. For the first time in over a year, Chris did not have any fluid in her lungs. This is really encouraging and we were glad to here it. We did meet with two doctors, one for a general review of her overall health, and then the hematologist, Dr. Sanchorawala who we both have a great deal of respect for and who runs the stem cell transplant program. She was very positive on Chris' progress, but we still need to see the results of the lambda light chains. This is the big number we are waiting to see as it will be an important determiner of the next plan of attack.

We discussed all of the various chemo options: Velcade, Revlimid, and possibly a combination of Velcade and Melphalan with dexemethesone. There is a possibility that we may have to go back in a month for more blood work to just track the data points. That may be when a final decision is made. I of course want to be as aggressive as possible and I have told every doctor I see that same thing. I am not happy with the 94% response, and Chris agrees, so we need to get 100% and pursue all potential options. We just won't know those options for awhile I guess.

The doctors were all fully aware of Stephen's ride. They have been very impressed and tell us that we must be very proud. Of course we are. There is a computer at the center and everyone was reading his web site and congratulating us.

We were very glad to spend some time with our friends from Ohio. Tim went through the stem cell transplant a month before Chris and is doing very well. He is now on an oral dose of chemo and we are learning from him all of the good and the bad. It is clearly tough on the body, but Tim is soldiering through it very well. Tim and his wife Connie have started a foundation where they will own an apartment in Boston and give it to Amyloidosis patients who are in need of financial support for their living expense while staying in Boston. Connie has this all planned out but is in need of some organization/coordination/management support from someone in Boston. Chris is very interested in helping as she has had such great support from so many people here in Holliston and donations from people all over the US. This could be a really win/win /win situation. Connie and Tim start a fantastic foundation, those in need of help get it, and Chris gets to give back (or pass forward) all the help she has received.

So bottom line, we don't know much yet, but hopefully we'll know a bit more tomorrow. Thanks so much for your interest and incredible support.

Some important days coming up....

Chris and I go in to Boston tomorrow for three days of tests tomorrow. Effectively, we will learn two things, 1) what is the current status of her amyloidosis (specifically her amyloid protein production) and 2) what the course of treatment will be. For the first question, it could be anything from worsening, to being stable, to improving. Since her response was a 94% reduction back in January, it would be great to see a total response. This would mean no second round of chemo, no further treatment other than continued medicines she is on now. If there has been no improvement, then I am sure she will have to do a second round of chemo this spring.
The process will be the same as before. Three days of tests on her heart, kidney, liver, lungs, etc. then a medical team conference on Friday followed by the famous call to us saying what the prognosis and course of action is. Chris and I are both getting a little nervous and it will hit us hard when we get there in the morning. As I have said many times, we will pray for the best, plan for the worst.

The outpouring of generosity to Stephen's quest to raise awareness and help further the level of research has been absolutely amazing. I do not know hardly any of the details of the contributions as Stephen is keeping this close to the vest, but I can tell you that many many people have been more than generous. Stephen has been amazed at the response. He is keeping a list of all of the donors and will be sending a thank you note upon is return. Everyone has been just incredibly generous in this economy. People have donated signs, shirts, biking equipment, housing, and have even hosted cocktail parties to raise money - just amazing.

I have spoken to a few people who have asked how he is doing. He has finished day two with great progress and many stories already. He and Aislin will have some incredible experiences to share when they get back to Worcester Academy. He does update his blog late every night at
www.chrisjourney.com and he is also sending quick updates during the day form his phone at www.twitter.com/chrisjourney . We can actually track his progress real time using a combination of igoogle and a blackberry. One thing that I have learned from his ride is the value of technology for communication. Between blogs and Twitter and igoogle, it is really easy to stay connected.

Chris and I cannot thank you all enough. The Holliston community, Worcester Academy friends, IBMers, people who have lost loved ones or have suffered themselves, the prayers, the love, the generosity, it is all beyond belief. It is impossible to properly thank you all.

Please continue the prayers for success this week, both for Chris and Stephen.

On their way to raise awareness......

A great send-off from the Worcester Academy students, teachers, parents, friends, and family. Many signs, hugs, and well wishes as they headed off. I won't post many pictures here as Stephen will be posting on the http://www.chrisjourney.com/ site with his daily updates.

Chris, Stephen, and Aislinn at the party. It meant a great deal to us to see such a great turnout.

Clicking on the picture should give a better view.

More Awareness for Amyloidosis

As I have posted here earlier, one of the most terrible things about amyloidosis is that it goes undiagnosed in so many people; they suffer the terrible consequences due to the lack of awareness. Chris was made aware from the pure luck of reading the newspaper article written about Jennifer Gershman, who was diagnosed a year before Chris and was also treated at Boston Medical. Jennifer promoted awareness and Stephen has continued the march with his east cost bike ride from Florida to Boston this month. Interestingly, this project that he and Aislinn Shea are doing for their senior project has benefited from some "viral marketing". That basically means that the story is spreading through word of mouth or other media without any organized or intended approach.

Today was a good example. The NBC affiliate in Boston, WBZ, interviewed Stephen and Chris today and ran a story on the news this evening. Here is the link:
http://wbztv.com/video/?id=73734@wbz.dayport.com

Here is their web site posting:
http://wbztv.com/health/east.coast.ride.2.950441.html

Also, the paper in Worcester picked up the story and ran it in today's Telegram and Gazette:
http://www.telegram.com/article/20090304/NEWS/903040449/1011

Chris is a bit worried that this is getting bigger than she wanted, but if one person is made aware or one doctor thinks of testing for this, then it is worth it all.

Stephen and Aislinn leave tomorrow for Florida from Worcester Academy. I'll try to post some pictures if at all possible on Friday.

A good and quick explanation promoting awareness

The Amyloidosis Foundation has sponsored a 5 minute video that was developed to explain Amyloidosis to physicians. It gets a little technical, but the key elements are understandable. Chris has Primary AL type, and you can hear some frightening statistics about life expectancy and number of sufferers. As the video points out, the single most important factor in successful treatment is early diagnosis. Yet diagnosis is unlikely given the lack of awareness by the medical community. As stated, often the patient suffers from many different symptoms and numerous tests are often performed without any clear pointer. In the case of amyloidosis, the only real test is a fat biopsy with a red congo stain.

Here is the url for the video:
http://vimeo.com/3358025


In the meantime, Chris is doing well but has had a very bad cold and some GI issues in the last week. Unfortunately we are learning that we just can't grab over the counter medicines to treat her symptoms as everything needs to be analyzed against the Coumadin. This slows everything down and she suffers a bit longer as a result. We want her to be as healthy as possible next week as we go in for the 3 days of evaluation.

Stephen continues to train hard for his ride. He and Aislinn leave on Thursday directly from school and will arrive in Jacksonville Friday evening with the ride north starting Saturday morning from the Mayo Clinic. They are pretty well organized, but I am sure they will have some bumps in the road. His plan is to update his blog each evening with a quick status of his location, his adventures, and his progress promoting awareness. He remains amazed as to the outpouring of support for his ride to promote awareness.

Again, thanks to all!!!

Some Good Milestones - mid February

A long overdue update. My only defense is that when there is not any significant news, I don't want to bore anyone with the mundane. Yet, I know there is so much support and interest that providing updates is important.



In the last few weeks, Chris has really "broken out" as they say. She has been going back to her personal trainer at the gym she went to prior to her diagnosis and has started a series of sessions where she will focus on her strength, building back some muscle mass, and some minor conditioning. This was an objective of hers as part of her recovery and I know that she is very happy she is back doing this. When you combine this with her regular walks and her yoga classes, it is clear she is really getting back into the swing of things. After a few more months, the next physical activity objective is to get back on the tennis court. That will have a special meaning in many ways as it is where she was when her doctor called with the bad news. I have always said we need to be back hitting balls prior to that anniversary, and I am sure she will do it.



Another milestone was getting back to church. Chris has tried to be very cautious about being in large crowds where people have potential colds and flu's and lot's of handshaking. With no immunity system, this has been a concern. But she really wanted to go and was prepared for any bugs she might pick up. Of course when she got there she was given lots of hugs by many people who were so excited to see her and she was shaking many many hands. She got home, washed her hands, and never got a thing. She was very glad to get back there for lot's of reasons.



This gave her some confidence to go to NY this past weekend for Abby's NCAA swim meet championship. We drove to Syracuse and went to the meet every morning and evening with a very large crowd, all packed in tight, with many people using door knob's, hand railings, etc. A few months ago Chris would never have attempted this, as the risk would be to high, but she did great, meeting many people. I in fact had a bad cold and did not want to risk giving it to others so I did not shake hands, while Chris was doing it for me. What a great role reversal. By the way, Abby did fantastic and was very happy.



Melissa has been calling from Australia and giving us the updates on her new escapades. Chris loves to hear the stories of her adventures in New Zealand and getting settled in Perth. I know Chris is anxious to start travelling again and hopefully will get to Florida in the next month or so after our visit to BU Medical in a few weeks. By the way, Melissa loves Perth.



So while I have been trying to stay out of the way for Stephen's bike ride, I can attest that it is coming together well. His friend, Aislinn Shea has been organizing the routes, the houses and hotels they will stay at, and the overall logistics. Stephen has been focusing on getting into shape with long rides on weekends and many hours in the basement training. Doing 100+ miles every day, for 15 days will be an incredible challenge and take a real toll on his body, but he is up for it. Aislinn will be critical in keeping the momentum going once they get started. Chris and I will be at our three days of tests in Boston while they are on week 1, so it will be a busy week for amyloidosis activity.

As I said, Stephen and Aislinn do not let Chris and I get very involved in his fund raising efforts for the research foundation, and thus I know very little. What I do know is that many many people have donated and it is incredible to see. I do not know the amounts and that is not important to me, but what is important is that he has many individuals contributing. Friends at IBM have been fantastic, friends from across town and nearby, friends of Stephen at school, it is just amazing. However, the ones that Stephen does tell me about are the ones with a letter attached to the donation. Many of them are from families who have lost loved ones to amyloidosis, or who have personally fought the battle themselves and are doing all they can to help find a cure. The stories are gut wrenching sometimes, but that is why Stephen is doing this ride. He knows he had a very ill mother, and he knows she has done very well so far, but he is painfully aware that others have lost husbands, fathers, mothers, wives, sisters and brothers. Creating awareness and finding a cure are what he and Aislinn doing this trip for.

In the meantime we will prepare for the BU appointments March 9, 10, 11. We could get great news (normal lambda light chain levels) or very bad news, but we will go in praying for the best and preparing for the worst.

I had not realized how many other amyloidosis patients read this blog, but based on the donations and the links to other sites, it is clear there are many. I can tell you, although you have not met Chris, she reads your blogs as faithfully. We both pray for you all, Amy, Kevin, Debbie, etc. We share your losses, your progress, your success.

Promoting Awareness

With amyloidosis being such a rare but brutal disease, the fact that it is so misunderstood and diagnosis is often delayed beyond treatment means that promoting awareness is critical. Chris and I remain incredibly lucky that she read the news article about Jenifer Gershman in the newspaper. http://www.metrowestdailynews.com/archive/x1853598606 It pointed Chris to the diagnosis and allowed us a strong treatment. Everyone we speak to who suffers from this disease is amazed that Chris went from her first significant symptoms in January to diagnosis in April, to stem cell transplant/chemo in June. We were very aggressive about testing and treatment, but very lucky as well. Jenifer made a commitment to promote awareness with the hope that she could help others with an early diagnosis, and with Chris she succeeded. Now it is time to help Jenifer, and that is one of the reasons Stephen has decided to do the 1500 mile bike ride. This will be an incredible accomplishment, but I am sure he will pull it off, even if he rides in snow storms in the night. But even more incredible is the response he has received from so many people. It has blown him and us away at the generosity and willingness to help to raise this money for the Amyloidosis Research Foundation. He will be updating the status of things as this is completely his initiative, but suffice it to say the outpouring has been fantastic so far. He has tremendous support from sponsors from diverse as bike retailers to cookie companies to landscape architects. The sponsors then help him raise the money by supporting the cost of the ride itself. Stephen will of course give 100% of the donations to the foundation. Those donations are coming in daily in the mail or on the foundation web site. I cannot tell you how very much appreciated these are. Some are small and some are big, but Stephen just can't believe the number of donors. Even his friends at school have been raising money through various events. Just amazing. Stephen is as much interested in the number of donors as the amount, as it means that someone was willing to spend the few minutes it took to help promote awareness of Chris' disease. Thus, even small donations are very appreciated as it means one more person helps fight the cause.

Stephen has had some good press on his campaign. The regional newspaper came by and interviewed Chris and Stephen for their Sunday edition. Here is the link:
http://www.metrowestdailynews.com/archive/x1556828605/Spitz-Son-is-riding-for-moms-life

Chris continues to work hard at regaining her strength and fitness. Unfortunately the bad weather does not encourage much outdoor exercise. Her aerobic fitness is very important to her heart, so she tries when she can, but still feels very winded with her reduced heart function. Her cocktail of pills continues morning and night, but she has not complained about it. I am becoming more convinced based on conversations with doctors and my own knowledge of the disease that Chris will be doing oral chemo this spring or summer. We need to attack the remaining proteins (lamda light chains) still being produced and prevent any further deposition in her organs, especially her heart. The March visit will be the point where we decide the next steps. Chris keeps saying, "I just want to know where I stand", as we feel like we have more to do but no definitive action plan. But in about 4 weeks we will know, just as Stephen starts his ride.

Again here is the link to his site: http://chrisjourney.com/

Thanks to all.

A very brief photo history: To Hell and Back

Chris and her sister Debbie in August of 2007 in NH. We had no idea what the next 12 months would bring at this point.

June 2008 - Getting ready to start the chemo. It took about 15 minutes to take it all. Notice the "pic" line inserted in her chest. The chemo was given via the line. The chemo is so strong that it can burn through veins close to the skin, thus Chris had to keep ice packed in her mouth for about 20 minutes to avoid severe sores. This is the equivalent of 6 months worth of oral chemo in two direct shots over 2 days.

The actual chemo, melphalan, which was given via the pic line (catheter) that had been inserted into her chest for about 6 weeks. This is the stuff that almost kills you but in the end is your best friend. Chris took the maximum (but highest risk) dose.

One of two bags, each containing 5 million stem cells. These are collected in a process similar to dialysis and separated with a centrifuge. The stems cells are collected prior to the chemo, then replanted a few days afterward.

They very carefully wrapped her stem cells up and transported them in this box to the blood bank for freezing. They can be stored for 10 years. In her case they replanted half of them back into her bone marrow. The other half, about 5 million are still at BU.

July in Boston, about 21 days after the chemo. Chris was under 95 pounds and unable to walk more than about 10 feet at a time.

On the deck in NH in August. Very frail, but happy to be done with the treatment and very slowly starting to feel a bit normal again.

Chris with Stephen, January 2009.

Another Echo today

Chris had another Echocardiagram today with Dr Falk to get his most recent perspective on her heart status. He compared the results with the November echo and saw no change, either good or bad. He is waiting to get the May echo from BU and then will compare that to the one today to see what has really occurred over the last 8 months or so. This will give us the widest possible spread of dates and provide an indication of how things have changed. The May echo was her first. So really not any significant news other then we remain concerned about the overall status of her heart and its ability to keep up with an active lifestyle.

Chris did go to Yoga again and she really feels like this is good for her. She complains that she feels very tight and yet she is pleased with her strength. Hopefully she will keep this up, as well as more cardio exercise. The weather has been very bad here and this limits the outside walks of course.

Last week I was in Rochester, MN for a business meeting and I made a trip to the Mayo Clinic which is really the only other amyloidosis center in the world, or at least the US. Rochester is a very funny place as it is about 90 miles from Minn./St Paul and really has two major employers: Mayo and IBM. The Mayo clinic of course is world renowned for it's expertise and incredible staff, but you have to wonder how it ended up in Rochester. The facility itself is amazing. I am not kidding when I say that the lobby is a beautiful three story atrium and there is a man in a tux playing a grand piano. I felt as if I was in the Four Seasons hotel. It must be hard for those without health insurance or needing to cover ongoing medical bills to see this use of money, but it certainly was an incredible place. What I really wanted to see was the Amyloidosis Center, just to see what it looked like compared to BU. We had heard of many patients who had been to Mayo for clinics or evaluation and thus it would be interesting to see it. I asked at the front desk and of course they had never heard of amyloidosis and had no idea where to direct me. Then I asked where the Hematology dept was and they said the 10th floor which I then proceeded to go investigate.

I was looking for a picture of the Center, just a nameplate or a logo or something that indicated Mayo and Amyloidosis as I wanted to give it to Stephen for his fund raiser web site (http://chrisjourney.com/) A woman that worked in the Hematology dept asked if she could help (as I wandered around aimlessly) and I explained what I was looking for. She went back to the inner sanctum of the hospital and returned saying that she had asked one of the doctors, Dr. Morie Gertz, if he knew of a sign or poster or something that I could take a picture of and the answer was simply "nope, nothing like that". Oh well. Interestingly as she mentioned Dr. Gertz' name, it hit me that I had read many many publications of his in my quest to be the expert on this disease. I brazenly asked if I could quickly meet him, just to say hello and tell him how appreciative I was of his work (and maybe get a quote for Stephen's bike ride). She went back to ask and returned with a curt "he is unavailable". My intentions were very honorable, but oh well, it just did not work out.

So our next major step is to hear about the comparison of May's echo with the one today with Dr Falk. Ideally we will get some important insight into the real level of her ejection fraction. This is a big frustration to Chris and I as we really do not have consistent answers. Hopefully we will learn more in the next few days.

Chris has recently heard from a number of friends who had not heard of her illness. She is really glad to hear from them and as always I encourage everyone to please feel free to call her. The support from all just continues to energize her. Thanks to all who have reached out in so many ways.

Brief Update on the Cardiologist meeting

Well, it is hard to say that Chris and I walked away with some incredible earth shaking news from the appointment with Dr Falk yesterday. We spent almost an hour with him reviewing his analysis, what the results show, next steps, etc. At the end, I asked him to summarize all of our discussions in just a few words. and he said, as he counted the words on his fingers: "I think she is doing very well".

Chris and I don't know exactly how to react to that. He did indicate that there has in fact been some decline in the heart function, as measured by the ejection fraction. He does not believe it is nearly as bad as the most recent BU analysis says, but he thinks there is some detioration since May. He did not know if that was due to further amyloid deposition in her left ventricle, or possibly the result of the high dose chemo itself. What confuses him (or as he says is "a contraindication") is that all of her other organs have responded so well to the treatment. Like Dr. Sanchorawala at BU, he says she clearly has had a very good partial response, but not the complete response we were all praying for. As such we asked him the likely next steps and he laid out pretty much the same 4 options we knew. 1) Another stem cell with high dose chemo, 2) oral Velcade, 3) oral Revlimid and 4) do nothing. Of course option 4 is not an option for me, so we ignore that. He doubts that any incremental benefits of the stem cell/chemo approach would be worth the risk due to her heart condition (the benefit being negligible vs a higher risk for cardiac involved patients). He thought the the oral Velcade would be his choice but he was clear to point out that he trusts BU and in the end that is a call they (and us) need to make.

He does want another (yes, yet another) echo next week. That way, they will have one more data point. One from BU in May, Dr Falk in November, BU again in December, and Dr Falk again in January. As echo's cause no radiation, I think getting more history for analysis is the right thing to do.

We did discuss mitral valve leakage and he stated that while there is probably some there, it was not a huge issue and there would be no action to take even if it was, as surgery would not be viable.

Chris asked the burning question "Can I ski ?". He was not very positive on that as he was afraid that any potential low blood pressure might cause her to faint and hit a tree. It was not a concern about stress on the heart from skiing, but a concern about fainting. Nevertheless, Chris and I did agree that on a beautiful sunny, non crowded day in March, we will in fact go ahead and ski. Chris enjoys skiing, is very good at it, and the hell with it, if we take it easy we can have a great day. So look for us in March on the slopes. I am not denying her of this and we set it as a goal.

Chris will get the results of next weeks echo right away, so we will have new news next week. I also need to add pictures here as I promised (but Stephen has beaten me to the punch on his 1500 mile ride for amyloidosis web site). Thanks for checking in and caring so much about Chris. As the doctor said, "She is doing great", but it just does not seem like we are yet able to close the book on this journey to good health. We are getting there however, I am very optimistic.

Please feel free to call Chris, she would love to hear from you and it is important for her to talk.
Pictures soon.

A small minor point: I am writing this post from Rochester MN, home of the other Amyloidosis Center. If I get a chance I may stop in to see the center.

1500 Miles (click here)

All of our children have been the best possible supporters of Chris throughout her journey to health. They have sacrificed a great deal to ensure that they can help Chris however she needs it; around the house, giving up activities, running errands, and keeping things going at home while Chris went through her Chemo in Boston.

Fortunately, our 17 year old son Stephen has a unique opportunity to help in another way. During a 2 week spring-break in March, he and some of his close friends have organized a fund-raiser with all of the proceeds going to the Amyloidosis Research Foundation. He and others (sponsors, friends) will bear all of the expenses ensuring that every dollar that is donated will go directly to the foundation. His ride will take him from Florida to Massachusetts over a 2 week period. This will mean he will need to cycle an average of over 115 miles everyday for 14 days - an incredible challenge. His goal is to raise $10,000 during this ride while promoting awareness.

A link to the web site he has created is on the right side of the blog. He is anxious to get a strong start toward his goal, and has already lined up sponsors who will support the ride. Any donation of any value will be greatly appreciated. This is fully tax deductible and will represent a very important donation towards the fight against Amyloidosis. Chris has been extremely fortunate that we have the medical care, the financial resources, a supportive community, and amazing friends who have all been an important part of Chris' journey. What Stephen wants do to is to help others who may not be so fortunate in their fight. Chris continues to fight, and we are sure she will win, but their are many others who can be helped by awareness, research, treatment, and support. This is his objective - not bad for a 17 year old kid.

Feel free to contact him with questions at: stephen@chrisjourney.com and please follow his blog as he prepares and then completes the ride. Chris and I thank you very much.