Who is this guy?

My hair has grown back darker and curly. The steroids have puffed up my face a little, so it is rounder. Combined with my prosopagnosia, I can not recognize myself in the mirror anymore. At all. It is hard to express how weird this is. Intellectually, I know that the reflection in the mirror is me, and not some guy standing behind me, but I can not recognize myself. The reflection in the mirror seems like a complete stranger.

I am not the only one. Today a doctor came in that had worked with me last time I was here, (when I had no hair) but had not yet seen me for this visit. He looked right at me and asked, "Is Leif here?". He had thought that perhaps I was Leif's brother. I suppose that all of you that know me well will be able to recognize me from my picture below, but I tell you, it is weird not being able to recognize yourself in the mirror. For one thing, it makes it hard to shave. You don't want to do something like shave with someone else's image.

Not the best shot, but you get the idea.

The lymphoma seems to be responding to the light chemo better than I expected. In the past, light chemo really has had little effect because the cancer is so aggressive. I am hoping that a large part of the effect that we are seeing is because the donor cells are off immune suppression and are getting into the fight. If this is the case, then the treatment that I am getting next week (the infusion of another 5 million donor cells) is likely to be effective in bringing me into remission and keeping me there. Go donor cells!

I am feeling a little better each day. There does not seem to be any more buildup of fluid in my lungs and I can breath freely. My pulse rate is down to a place that is no longer scary. I am a little low on blood, so that tends to lower my blood pressure and increase my heart rate. Last week my pulse was in the 130+ range, but now it is around 100 beats/min. Which is still fast, but not scary fast. Last week they gave me a lot of saline solution to increase my blood pressure, which it did, but it also made my legs into swollen sausage-like tubes. This has since gotten quite a bit better and I am able to go on short walks again.

Leif

Analogies.

I have been thinking about the WWII analogy that I used the other day. I like it. It helps me visualize what is going on and I think that it helps me use visualization to help out my little hard-pressed cells. What I have been wondering about, in this analogy, is: Who is Russia? I decided that Russia represents my own unwavering tenacity. My ability to be knocked down, stomped on, hurt like no one should hurt, and get back up again and take another step forward. Zombie Nazis hate that type of tenacity.

Italy represent all the other incidental illnesses. The abscess that I had, the pneumonia, that sort of thing. Things that would be no problem if I weren't already dealing with Zombie Nazis. Irritations and illnesses that are dangerous, only because I am fighting a much more dangerous threat.

On another note: I had my 2nd round of Chemo out of 4 today. This round was Velcade and Bendamustine - both are poisons that are taken up by quick-growing cells, like the lymphoma. So my good B cells and T cells, which don't divide are completely untouched by these drugs, and the lymphoma is seriously weakened. We do 2 more rounds and then throw in 5 million T cells from my super-human donor. They will act like the D-Day invasion force and start destroying the lymphoma in winning numbers. The only down-side to the chemo is that it may be hurting my bone-marrow stem cells. They are relatively fast-dividing cells, so they are going to take a bit of collateral damage. This is why we are shoring them up with the T-Cell infusion. After the chemo, they will have to take some time before they are back up to full force and health. The infusion will cover for that period. Then, if my Chimerism stays high, I should be good to go.

We hope that the fluid in my lungs will clear up when the lymphoma starts get beaten back and is pushed into remission. We are not certain of the cause of the fluid though, so it is one of the things that we just have to wait and see how it goes.

Ending on a good note, my Graft vs Host Disease has almost completely cleared up in my skin, and does not seem to be coming up in my liver or kidney numbers. I have been having good conversations with my new blood cells, and I think that we are all on the same page now. :)

Leif

Saturday, day 1 of 11 of chemo.

The good news is that the test all show that I still have 95%+ of chimerism. This means that the donor cells are doing their job, engrafting in the marrow and producing effective white blood cells. The planned Donor lymphocyte infusion (which I have been mistakingly calling a T-cell infusion) has a good chance at helping out the donor cells fight the lymphoma.

The bad news is that my lymphoma has mostly recovered from the chemo that we gave it last year. My spleen has not grown huge again, but there are high lymphoma counts in my blood and in my "soft tissue". I think that soft tissue in this context is muscles, tendons, and ligaments. The CT scan shows numerous lymph nodes that are trying to sequester the disease. It does not appear to be in my bones, lungs, or other organs.

So, in order to give my DLI (Donor lymphocyte infusion) a better chance, we are going to hit the lymphoma with a low does of chemotherapy. The chemo drugs that I am taking are: Velcade, Rituxan, and Bendamustine. These have the benefit of being harder on the lymphoma than they will be to my new donor cells, and more importantly the stroma - which is the honeycomb structure in your bone marrow where your stem cells live.

I had my first round of chemo today, and it went very well. I have often had reactions to Rituxan in the past, but I did not have any problem this time. In fact, the drug that I took that had the most adverse reaction was Benadryl, which they gave me to ease my reaction to Rituxan. I guess it worked, so it was worth it, but I was in a fog all morning. I was trying to get out an email, and found that my brain had apparently turned off and I had a couple paragraphs consisting of nothing by the letter "n".

The other chemo drugs also went well today, with no noticeable adverse reactions.

The analogy that I have using to think about this latest round of care is from some alternate history of WWII. Where my body is Europe and the lymphoma are Nazi zombies (because they are undead B-cells; B-cells are not supposed to be able to reproduce on their own, without bone marrow stem cells), and my old immune system was the French, with their Maginot line.

I know that people mock it, but it was such a good defense that the Nazis did not even try to attack it, but instead snuck around through Belgium. I compare that to the betrayal of my own B-cells to become lymphoma cells and become invisible to any good B-cells that I might have had left. The donor cells are the British. They need more time before they get strong, and so are not able to help out the other besieged counties. They also might accidentally attack a county that they are supposed to be allied with (Graft vs Host Disease). The DLI is like the Americans. They come in way behind the time that they are needed, but with serious force. They work with the British to plan D-Day, re-take Normandy and beat the Nazis back to Berlin. Then they are done and mostly leave, leaving Europe in bad shape, but still existing.

So in this analogy, the chemo is like carpet bombing. It is hurting the Nazis more than the Brits, but there is a lot of collateral damage on both sides. It does buy time for the D-Day invasion, and it helps even the playing field between the mass-production of the Nazi war machine and the relatively green British troops.

I also like this analogy, because there have been no relapses of the zombie Nazis. Sure, there have been some minor wars in Europe since then, but nothing that threatens to make Europe as we know it cease to exist.
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Pam brought over a turnip casserole, swiss chard, and salad greens. Delicious. So far, the only thing that I have eaten from the DHMC during my stay here are salt, pepper, 2 hard-boiled eggs (how do you even get the inside of a hard-boiled egg that green?), milk, sugar, and bottled water. They did get me a fridge and containers of Brown Cow, full cream, blueberry yogurt. I am planning to have the yogurt for breakfast. At least in my analogy I am not stuck with MREs, and other fun war foods.

Becky is staying at our friends Peter and Brita tonight. She slept in the hospital the last two nights, on a cot that they brought in for her. She is planning on taking a relaxing morning and won't be coming by until the afternoon. It is important for her to get some rest and have a day where she is not bombarded by hospital stuff. I hope that I can convince her to do it more often, but of course, when she is away, she worries about me, probably just as much.

Leif

Bone marrow biopsy

Had a meeting with Dr. Meehan this morning. We don't have a plan, so much as a flow-chart. We have to balance the negative effects of Graft vs Host Disease with positive effects of Graft vs Lymphoma Effect. But only after we know how much of the disease is in my body, and what is causing the fluid in my lungs. So I am likely to be here between 1 to 6 weeks.

I had a blood biopsy yesterday, a skin biopsy today, and I just got back from having a bone-marrow biopsy. It will take a few days to analyze the results and start to get a picture of what is going on.

They started draining my lung again about an hour ago. They don't want to take out more than 2 liters per day, unless it becomes an emergency. There are issues of damaging the lung if fluid is taken out faster than that.

My mother-in-law, Pam, is up with us here at DHMC, and staying with her sister, Prue who lives just a few miles away in Vermont. She has been in charge of fixing both Becky and me delicious food. It has been really a boon - both because the food here is so bad, and also because poor Becky has really needed some TLC as well. It has allowed Becky to just relax and hang out with me at the hospital.

So, until the test come back, we are not sure what will be next or even what therapeutic route we will be taken.

Leif

Déjà vu, with ornamentation.

A lot has happened since my last post. Becky and I spent the weekend up in Brooks. I got some trees marked for felling this coming Sunday, played some cards, and had a nice dinner at Kristen's. Other than that, I rested. I seemed to be having trouble breathing. At first I thought it was just the heat. It was in the mid 80's and I don't do well in temperatures over 70.

But, when we got to Cundy's Harbor on Monday evening, I had an acute attack of shortness of breath. I had bent down and exerted myself slightly, but I had not been able to breath bent over for the last day and a half or so, and the slight exertion seemed to put me over the edge. Even after I stood back up, I could not catch my breath. It felt as I was drowning in my bedroom. I signaled to Becky that we need to go to the hospital immediately. She asked if she should call 911. Yes. I was worried about being asphyxiated in the time that it took to get to the hospital. By the time the ambulance came, I had caught my breath, gotten dressed, and packed for an extended stay. But I was glad that the ambulance had oxygen. They put me on 3 liters, and whisked me to Midcoast, in Brunswick.

After I got there, they gave me a chest X-ray, and the lung that they had drained on Thursday was more full that before they had drained it. In fact, it was nearly completely full - pushing on my esophagus and my heart. They did an emergency drain, which contained a lot of blood. A rare complication of draining a lung is that they can hit a blood vessel (less than 1%), and lucky me, it seems as if that is what happened.

After being stabilized, they put me on an ambulance and whisked me to Dartmouth Hitchcock Medical Center. I was given 2 units of blood to help make up for what was lost in the lung and monitored through the night. I slept pretty well all things considered.

They determined at Midcoast that not all the fluid in my lung was blood, so quite a bit of the other fluid had re-accumulated. We do not yet know what this other fluid is other than it is not infectious, and does not contain lymphoma. It is probably either a by-product of Graft vs Host Disease or Graft vs Lymphoma effect. We certainly hope that it is from Graft vs. Lymphoma effect.

At noon, they put in a tube that stays in, and drains off the fluid at the rate of 2 liters a day. I am not completely drained yet, but my breathing is better. I have this plastic tubing coming out of my back like some sort of tail dreamed up by Salvador Dali. The incision and the weight of the tube is less than comfortable. I would not suggest getting one installed, no matter the power of the fashion statement that it makes.

Becky and her mother Pam arrived this afternoon as I was getting in my tube. Pam is currently out getting me some actual food, since DHMC has some of the worst hospital food imaginable. They don't slice the tomatoes here for the salads. Just throw them in whole. And black olives? I wish.

Tomorrow, I am getting a bone marrow biopsy. This one should be under more sedation than my last. I hope that it goes well.

Anyway, not what I was planning to do this week, but that is what is going on. I will keep people updated as things progress.

Leif

2nd Thoracentesis

Since there was that error in the orders for draining the fluid in my lungs (thoracentesis), the scheduled me the next day. The operation when smoothly, did not hurt at all, although the muscle where they poked me is a little sore. This time, instead of draining a sample for testing, they drained out as much as they could. How much you ask? 3 liters.

During the procedure, one of the nurses walked by with a 2 liter container of the nastiest phlegm/blood product looking stuff I have ever seen. I thought "man, I hope that didn't come out of me", but even as I was thinking it, it seemed absurd, because it was a 2 liter container. Way too much to be in a lung cavity. I was wrong. I didn't see the second container, but they told me that they had drained just over 3 liters, including what they had taken off the day before.

Definitely explains my difficulty breathing.

So, the procedure helped quite a bit. It will take some time for my lungs to re-expand into that space, and as they do so, they are a little sore, so slow, careful deep breaths for the next few days. But I can walk again without getting short of breath, and life is pretty good.

Leif

Tuesday office visit.

I met with Dr. Connelly on Tuesday. This was a routine office visit, and she is mostly checking for signs of Donor vs Host Disease (none were found). However, during the last few days, I have experienced some swelling in my lymph nodes and more disturbingly, fluid in my lungs. This is not completely unexpected, because I have tapered off the steroids, and they had been helping to keep things in check. Still.

So before we met, she had me go in for a couple of chest X-rays to see how much fluid had built up. It was enough to be a concern, so she ordered some to be extracted. I was not looking forward to this, despite enjoying the ability to breath, as it involves sticking a needle into the lungs and draining off the accumulated fluid.

They were able to fit me in that day. When I got to the surgery area, the guy that runs the ultrasound and the doctor were both really nice (the nurse was really nice too). He explained that while it sounds horrible, it is not that bad of a procedure. First of all, the fluid apparently is not actually in the lung, but in the tissue around it, and the weight of the fluid is what prevents the lung from expanding. So they will not be poking a hole in my lung. The downside is that the fluid is not all in a continuous pocket. So, they will go in through my back and drain the largest cavity.

There was some confusion, because the orders were to just take a sample for testing. My understanding was that they were going to drain out most of the fluids so that I could breath better. The surgeon told me that therapeutic drainage was pretty different surgery. It was basically the same technique, but because they would have to go in multiple times, the risk was different (both of infection, and there was a greater chance of collapsing the lung) and the orders were pretty clear.  I convinced them to at least drain the largest pocket (as opposed to just take a sample), which they did.

Since to hit the other pockets, they would have to put in more needles, there was no clear disadvantage to doing the written orders at that time, and then, if there had been an error in the orders going in to do the rest of the drainage. We also thought that it that draining the largest pocket might alleviate most of my shortness of breath. It helped, I was able to sleep last night laying down - instead of propped up- but my breathing is still pretty shallow.

It turns out, after talking with Dr. Connelly, that there was an error in the orders. That the intention had been to drain as much fluid as possible, so I will be going back in on Wednesday or Thursday. On the plus side (depending on how you count these things) that was easily the least painful surgery procedure that I have had done. There was a little stick from one of the numbing shots, but other than that, I did not feel a thing, and even now that the local shot has worn off, I don't feel any pain where they went through. So it is not wrenching to think that I have to go through it again.

A social weekend.

Had a great weekend up at camp. I should probably mention, for those of you that are not from Maine, that what we call "camp" is typically what other parts of the country call a lakehouse. In Maine, though, they tend to be small, and not built for show, often out of reclaimed materials. But they usually have the necessary amenities of civilization - power, running hot and cold water, a full kitchen, a shower and a toilet. A lake cabin.

I got to see my brother's family. His two boys have grown considerably since I last saw them. I also got a chance to see my grandmother, who I had not seen since the week before I was diagnosed. The last time I saw her, she was in the hospital for a stroke, and has since recovered pretty well and is in a nursing home. She has problems remembering things, but she knew who I was, and she seemed much less worried than she has through most of her life. Perhaps, most of what she is forgetting are her fears.

There were also a score of second cousins, friends, and friends of second cousins. It was a little overwhelming, as I have been mostly sequestered for the past 10 months. After we left on the 4th, Becky and I went to her parent's camp in Brooks. We were able to see a number of our friends there, as well as spend some quite time, just reading and looking out at the lake. Just what I needed.

The next steps, more or less.

Last friday was some hard news. Both my doctors gave me a pretty short time frame, but after talking with them both earlier this week, I feel that they are not throwing in the towel yet. Statistically, at this stage, even if we get this most recent relapse into remission, it is unlikely to stay in remission. So there is a sequence of the lymphoma being brought into remission, some time goes by, it relapses, and then they try to bring it into remission again. In theory, it could go into remission and never relapse again. That would be "cured". However, in practice, with agressive lymphoma, my current relapse makes the chances of that happening are infinitesimal.

At some point, we will not be able to get the lymphoma into remission. It could be this current relapse, or it could be some relapse in the future. Each time is more difficult because the treatments are pretty hard on the body, and it limits what treatments we can use to fight the lymphoma.

I currently believe, and I think that my doctors both believe that we can get this current bout into remission. I do appreciate that they are straight shooter and did let me know a likely time-frame if we can not get this into remission.

The current plan:

I received a call from Dr. Meehan yesterday to go over my new treatment plan. What he would like to do is a T-cell infusion from my donor's lymphocytes, along with a pill called Revlimid. This is a new drug that has just been approved and looks pretty promising in cases of refractory mantel cell.

HOWEVER, we can not do the T-cell infusion, or the Revlimid while I am on my immune suppressants, so I have a new schedule to taper off the remaining suppressant as fast as we dare. The danger, of course is Donor vs Host Disease, where my donor cells decide that all my cells are foreign and attack everything. This can be a deadly disease, so we are monitoring closely for any signs of this. Also, because the Revlimid is an immune booster, it will help the donor cells, so I can not have even a touch of DVHD before I go on the Revlimid. Normally, after getting off the immune suppressants, one would wait 6 weeks to make sure there are no signs of DVHD before going ahead with the Revlimid treatment. In my case, we are going to wait a week. Perhaps not even a full week.

The T-cell infusion has the same restrictions - because it is an infusion of my donor's cells, if I have any DVHD, they will join the donor side and I will have serious problems.

I am tentatively scheduled to get the T-cell infusion and start the Revlimid on the week of the 22nd. My taper ends on 7/15.

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I did have a good talk with Dr Meehan about hitting the current relapse with a chemo regiment. We know that the last Chemo that I did was very effective against my lymphoma, and brought it down to the point where it was in complete remission. The counts low enough that they were not showing up on any test.

My thought was that I am in pretty good shape, and unlike last time, I do not have a raging infection, so I would think that the chemo would be less dangerous to me than it was last time and with my lymphoma knocked down to nearly zero, my new donor-assisted immune system would have a fair fight. Dr. Meehan believes that any chemo that I have at this point will do much more harm to my new donor immune system than to the lymphoma. The donor cells, are still settling into a damaged bone-marrow matrix and it is like tending a new garden. We were not worried about destroying my bone-marrow cells during my last round of chemo because we knew that I was getting ready for transplant. This time, we do have to take that into consideration, and because the donor cells are still filling in, repairing the bone matrix, and in an unnatural environment (ie my body instead of my donor's body), they are much more sensitive to chemo than the lymphoma. A chemo regiment would probably wipe them right out, and not bring my lymphoma into remission.

Dr. Meehan and I are also looking into the possibility of enrolling me in a Phase IV clinical study for a drug called Ibrutinib. It seems reasonably promising for relapsing mantel cell. It may be that by the time that it makes sense for me to be on Ibrutinib, it may be out of clinical trials altogether, which would mean that I would not have to drive to Massachusetts or somewhere for the drug. There are rumors that it will be approved in the spring.

I am planning to talk with another doctor to get a second opinion, because I can not see that being a bad thing. But overall, what Dr. Meehan and I discussed sounds like it makes a lot of sense. I know him to be a careful, thoughtful, and experienced doctor. My trust level in him is pretty high.

I don't have much information on T-Cell infusion, so if one of you wants to look that up and give me a summary, I would appreciate it. I know that any statistics you might find do not apply to me, but I am curious about how this treatment might have worked for other people with refractory mantel cell.

Other than that, I am back in a pretty good head-space. Feeling more or less upbeat. Enjoying one day at a time. My fatigue is not too bad - I can get in a walk, answer some emails, do a little bit of drafting on our cabin.

Tonight we are headed up to Sebec, and will see my brother and his family for the Forth, and then on Friday, we will be in Brooks for a relaxing weekend at the Jackson camp. I hope to get the canoe out and drift around the lake a bit. It should be pretty nice.

Leif