Lessons Learned from Chris' Journey through 2008

With 2008 gone, and our optimism for a much better 2009 as high as ever, I have thought for awhile now that it would be important to share my insights, perspectives, recommendations, and warnings for others who may be face a similar circumstance, I will try not to be too verbose (yeah right) but convey the essence of the thought. Some of these may be only applicable to Amyloidosis, but I am trying to think of them more generally. So here goes:

-If your family member, colleuege, neighbor, whomever says, "I'm just not feeling right" or "something is wrong but I can't tell you what" and this goes on for an extended period (more than a few weeks), get it checked out. Don't write it off to old age or work stress or a low-grade fever. I am convinced the body sends the mind clear messages that attention is needed. If something isn't feeling right, then do somehting about it. We all want to fight the battle, keep moving, go to work, etc. We don't have time for the Doctor. Lesson: If you don't feel well for more than a few weeks, see a doctor. Time may be very important. Many Amyloid patients miss their opportunity for treatment because they were not diagnosed early enough. They die from a lack of a diagnosis.

-Get a second opinion on anything more than the basics. As I have stated many times, medicine is an art not a science. What one doctor misses, the other may see. Every doctor has a different set of experiences and training. They use their judgement based on what they know. The experts based at the BU center believe that >95% of primary care physicians have never diagnosed nor treated Amyloidosis and would not even think of testing for it. Maybe they read about it in Medical School. This is why the majority of sufferers die from the disease before treatment. Doctors don't see it, they don't know what too look for, and they don't even think to test for it. A great book to read if your looking for something on this topic is a book I happened to have read about a year ago. It is very interesting reading for a caregiver or advocate:
http://www.amazon.com/How-Doctors-Think-Jerome-Groopman/dp/0618610030 (sorry I don't mean to promote Amazon). I strongly recommend it before a crisis hits and I am sure it shaped my own thinking as Chris' advocate.

Have an annual physical with complete blood and urine tests. The body is filled with bio-markers which are indicators of chemical balance. I had no idea how complicated the chemical make-up of the human body is until I looked at a 5 page blood analysis. We have every metric on Chris since May and each one tells a story: glucose, potassium, protein, lambda light chains, albumin, creatinine, etc, etc...... Always have complete blood work and a urinalysis at every checkup. and always have it if you are going in if you don't feel well. Be aggressive about getting these done, they are not needless tests, they are critical chemical indicators. Chris had a urine test, which then led to a 24 hour urine analysis, which then led to an indication of an immuniglobulin M-spike, which then led to a bone marrow biopsy, and then a fat-pad biopsy that was tested with the famous red congo stain confirming Primary Amyloidosis. Without the first urine test, the whole sequence would have been delayed and the diagnosis may never have happened.

Become an expert. Whether it is heart disease, lung cancer, pneumonia, or any strange disease nobody has ever heard of, the resources are there to become an expert. The more you know, the more you become a participant in the discussion with the medical team vs an observer to the process. You can challenge, ask intelligent questions, seek out specialists, manage the drugs, demand tests, and potentially avoid problems. I now know drug interactions, I know all clinical trials by drug companies, I know the latest testing techniques, and I have read almost every paper written in the last few years on the subject. I use Google Alerts to scan the Internet for all mention of Amyloidosis as well as any publications from selected experts in the field. I get a weekly update on all new items about the disease, including new trials, published studies, conferences, etc. I suggest reading all recently published works. In our case it comes form magazines such as Blood, Journal of the British Hematological Society, etc.

Document and get a copy of everything. I have a copy of every single test result Chris has had. I have a copy of every report written by every doctor. I have the daily blood tests results as well as the graphs of the trends over the entire period. These are documents that are your right to have as they are about you or the patient you are advocating for. I used to think there was some special underground/behind the scenes data that only the medical experts had access to. In fact you have rights to everything. There are many benefits to this:
-You can become smarter by looking up every term used, understanding every test result, and getting a very well documented position of the doctors opinion.
-You can make these available to other doctors who need to know the background, especially those providing a second opinion.
-You might be able to avoid tests. Chris has had many many lung x-rays. I am concerned about over use of these. With a copy of the x-rays, I simply hand them to another doctor and avoid the tests. As I have learned, hospitals operate as members of competitive organizations. They often network well inside of their partnership, but not well outside to other partnerships.

Hospitals are very infectious places. One surprising lesson I have learned is how hospitals are really filled with germs. Chris' immunity system was completely wiped out, and they did not want to have her stay in the hospital for fear of infection. Some hospitals do the stem cell transplant and high-dose chemo as in-patient. Boston Medical insists that all patients live off-site, but close. The doctors strongly believe that they have a lower mortality rate simply due to the number one cause of death for these patients - infections. I am not advocating that patients avoid the hospital, but I was surprised at how many stories I heard about people getting very serious infections in the hospital such as MRSA, staff infections, etc.

There are two critical roles: Caregiver and Advocate. These can be the same person or two different people, but they both need to be in place. The caregiver is focused on the patient and his/her comfort. All seriously ill patients need a caregiver, even if at the hospital. Nurses have many patients to worry about cannot give the level of comfort or attention that an ill patient really needs. The caregiver needs to do a virtual brain transplant and think in the mind of the patient. What would make them more comfortable, who might they be worrying about, what are they feeling? The caregiver needs to swallow their pride completely. As Steve G, husband of Jen (the couple whom we read about in the paper back in May) told me," be prepared to wear a loincloth, you will become a slave and will need to swallow all pride and be a servant". This is a tough and frustrating job. You may be asked to get something very small from a sound sleep, you may have to clean up some really nasty stuff, you may be sent to the store to get something only to find it was the wrong thing. My suggestion is to just acknowledge that you are there to serve. It is not about you, it is totally, 100% about the patient. You get very little sympathy, very little acknowledgement, and few people understand what you are personally going through. Suck it up, be tough, and focus only on the patient. You will have to run on the internal pride you will develop that you are making a difference.

The other key role is the medical advocate. This is the person who needs to be the brains to the caregivers heart. Write everything down, understand all medications, understand the rationale behind all tests. If there is a need for medical attention, go get it. Get the best you can, even if you have to fight a battle. Question everything -not to dispute it, but to understand it. Bring a notebook to all medical appointments and listen in on all medical rounds if in the hospital. Listen very carefully and then question for clarity. Since medicine is an art, not a science, there is no simple algorithm that doctors use to come up with the answer. The advocate needs to be a part of the decision making process if the patient cannot be. Don't let things happen around you with others making the decision, be a part of the decision. Be aggressive, actively representing the patient. This does not mean being a pain in the ass. I have asked the doctors at BU if they thought I was overly aggressive and a negative in the process. The answer is always: "active participation is always better than benign neglect". Yes, I asked alot of questions and yes I knew what I was talking about (sometimes not as much as I thought I did), but they respected me for being involved. I witnessed some spouses who did not go into an appointment with the doctor, could not even properly say the disease, had no idea what was going on, and thus could not advocate. The advocate is not about making the patient comfortable (that is the caregiver), but it is about fighting the required battles in the complex medical system we live in. It is very hard to do both, but it can be done. Just think of them as two different roles and understand which one your in. The caregiver has no pride and a big heart, the advocate has a big brain and a strong enough ego to go into battle if required. (As I right this, I know it sounds so obvious to most of us, but I witnessed such distant participation and bad care giving by family members that it shocked me).

Hospital chairs are terrible beds. It is a very small price to pay from what Chris has been through, and as the caregiver in the hospital, Chris wanted me there at night. But, these chairs are torture devices. The interesting thing is the difference in the patients. I had a very big back operation a year ago and was in the hospital for a week. The absolute last thing I would want is for Chris to be there overnight. In fact her being there for more than an hour was tough. When I am sick, just leave me alone, don't ask me how I am doing, just let me be and I will let you know when I am better and I'll call you to come pick me up. Chris is the opposite. She wanted me there every night and all day. I had to just accept it (the caregiver has no rights to personal comfort as the lowest form of life on earth) and sleep in a chair. Forget the previously cured back, but that is another story. The nurses were always very good about finding me a blanket and pillows and sneaking me a muffin or a soda, but all I can suggest is try to avoid these chairs as best you can. Again, a very small price to pay and probably my only complaint worth sharing.

Rely on religion, meditation, poems, psychologists, or denial - whatever works. The human mind is sometimes the patients worst enemy. Chris and I spent many many nights up all night, scared to death of our future, what would happen to our fantastic life, will we ever get through this, etc. Many tears, night sweats, all night pacing, it was horrendous. We both needed help, some kind of guidance. Chris reached out to many alternatives: our minister, prayer books, meditation, Reiki, massage, acupuncture, lucky bracelets from the kids, etc. Each worked a little and helped in the total picture. One thing that I found interesting was when we met with the Psychiatrist at BU as part of the normal regiment. He told us a very effective coping mechanism was the use of denial as a way to deal with this. Deny that anything could go wrong, deny that this is really happening, deny that there will be any changes in your life. He told us that this is how many patients cope. To me, it is a dangerous approach, but probably very effective. Chris found her prayer book very useful as she went to bed. It focused on hope, life, and love. She also got a bracelet from a good friend that said: Expect Miracles, Have Faith. She wears it daily and really believes that with faith, miracles can happen. One point I think is important to make is that while outwardly a sufferer and and their family may appear calm and collected, inside they are torn apart. They are scared, depressed, angry, and confused, but they fight hard not to ever show it. I was amazed at how Chris held herself together at all of Abby's graduation parties in May only to get home and breakdown. We all need to know that when others are going through this, what we see is far from the reality.

Family is critical. Both Chris' and my family have been fantastic. They have cared for our kids (with Chris' sister Debbie giving up her entire summer without hesitation), driven up numerous times from Pennsylvania (her brother Tim and sister Sally) and called almost daily (see comments below on blogs). Her parents have been fantastic. My family has also been great, always asking what they can do to help, sending love, being there when we need them.

It takes a village. I have written about this before, but more than anything this was the single largest lesson learned for Chris and I. We have been blown away at how our extended community has reached out to help us. This village, made of neighbors, life long friends, family, church members, colleagues at work, and even unknown people, have reached out to help and offer support. I cannot say enough times how valuable the meals, the rides to the doctors, the phone calls, the cards, the prayers, the small gifts offering hope have been. A great best friend coordinated all meals for many many weeks, to the point we had to say thanks but no more. Another has called up after dinner and said, "you did not walk today, lets go now", even though I know she was tired. Another drove her to Boston for entire day medical appointments on their only day off. Friends from her high school in Belgium have called and left many messages of support. Friends of our parents from all over the US have sent cards. Friends at IBM from Singapore, Sydney, Paris, all over, have sent their well wishes ( including a colleague in NJ who herself has been very sick and sent a great note of inspiration). Friends from UNH, Univ. of Delaware, a student doing a semester in New Zealand. On Chris' 100th day, we got cards from people we do not know, including a great card from a gentleman in Philadelphia who gave Chris some words of encouragement. It all has been absolutely beyond anything I had ever thought possible. For Chris, she has been so pleased to hear from friends, it means alot that people call to check on her. At this point, Chris now feels very good when she can give back, or "pass it forward". She did the breast cancer walk, she has been helping others in need, and she is really looking forward to getting back involved in much of the charity work she did prior to getting sick. It takes a village to take care of a very sick person, and this village of ours ( a worldwide village) has been the single most amazing thing for us in helping.

Communications. This is a hard one as it is complex. When we first entered this surreal world, we had no idea what to share, what to hold back, how to tell people, how to talk to our kids. Based on what I now know, the best approach is always one of complete and open communication. Tell anyone who wants to know the whole story, the good and the bad. There is no sense in hiding the reality. Some people will be very interested, others have their own issues and will listen and wish you good luck. One of the toughest decisions we had to deal with is what we would say to our children. We let them ask the questions but also tried to explain it all. We did lessen the degree of risk of the transplant/chemo. For someone with severe cardiac involvement like Chris, the chance of death during the first 100 days was about 20%. We knew this very well, but we probably hid that reality more than anything else. Maybe this was selfish on our part, but we did not want to deal with the emotional panic of our children while we needed to be focused on Chris. We wanted them to live as normal of a summer as possible, so we tried to give them only the positive. In retrospect, that was the right decision. The other lesson is the power of the blog. It has been invaluable. It has helped me give updates to hundreds at a time, it has been therapeutic for me, in fact very therapeutic, and it has let me share this ordeal with others who are or will go through it. One thing that was very hard for me was to answer the phone all evening long when Chris was unable to talk, giving an update on how she was. We would have very long days at the Center, I would make Chris a dinner and then clean both the dinner dishes as well as the food when it came back up, and then the phone would ring. I was exhausted and just wanted to rest and focus in getting her comfortable. Yet the calls were mostly from our families and I knew they were calling with their love. The blog has allowed me to give updates and people can read them at their convenience. One decision I made that I strongly feel is the right one was not to open it up for comments. This would have put pressure on me to respond, and more importantly I felt people would feel obligated to say something every time. We knew just by reading it that people cared, but by not allowing comments, I believe it was easier for both sides.
I have been told by many people that they really appreciated the blog and found it very helpful. I am not a natural writer and have never done any writing like this, but I have found that the best way of communicating my thoughts was to write from the heart. This was the therapeutic side of it for me. Probably the biggest mistake I have made is getting too focused on the medical elements - the details of the blood tests, the description of the disease, the medications, etc. Chris has read very little of this, literally a paragraph or two on a given day. When this is all over, I will somehow package these posts all together, from the beginning, and let her read them. She remembers very little, but I think she will find it very interesting. I occasionally have gone back and looked at random days. It has been an incedible ride and I am glad I have documented it. My hope is that as new patients enter into this craziness, they can at least see the details of one story. Chris and I were incredibly lucky that we had Jen and Steve Gershman to help guide us through, but there is very little documented, maybe this will help some. WHEN we are successful, then those scared to death like we were can maybe see actual hope.

Friends. Never ever try to watch the entire 10 year series of Freinds. Trust me on this one. There is clear evidence of acute brain damage to those who try. Yet as I write that, it has been our Friends who have done so much for us. Friends have been the single most important element of Chris' success and yet here I am warning people to avoid watching Friends. Not being a writer, I don't know if that is a juxtaposition, a contradiction, or the antithesis of each other, but it is at least interesting to me.

I think that at this point I have more than tried the patience of the readers on my "Lessons Learned". I apologize, but wanted to get it documented. I know I have editorialized, complained, over-described, and bored some to death with this blog, but I really appreciate your reading it. We are not done by any means with Chris' journey to health. We see Dr. Falk in 3 weeks,, we go back for a three day evaluation at BU in March, and Chris continues to work to get her body back to normal. We are trying to live a normal life now, and feel pretty optimistic. We went out for dinner last night with friends and ran into many other couples from around town. To me, it was almost like the old days (i.e. Jan. '08). We spoke about our kids, the town, real estate, jobs, etc, and little about Chris' illness. She has been through absolute hell and and here we are, less than a year from diagnosis, and trying to get back to a normal life. I look at her when she is talking to others and I feel so much pride and amazement.

OK, sorry for the far too long note. More in a few weeks, thanks for checking, please remember to look again down the road. It means so much to Chris when people call and say hello. Please feel free to call, send a note, give a prayer, or just wish her well.