Are you kidding me?

I went in this morning and got my labs drawn. For those of you have missed a couple posts, I am trying to get into a study that will give me access to a promising new drug called Ibrutinib. The hitch is that I currently have Graft vs. Host disease and one of my liver enzymes is higher than the study parameters allow.

We are actively treating the Graft vs Host disease, and the enzyme is coming down pretty steadily. Last week I had it tested 3 times - 173, 168, and on Friday it had dropped to 143. The number that we need to hit for the study is 107.5 or lower. So, seeing that it had dropped by 25 pts between Wed and Friday, we were pretty hopeful that I would hit target today.

I did not. My lab reading this morning is 110. Arrgh! So. Since we only need another 2.5 pts, we are testing it first thing tomorrow morning. Unless there is something else going on, it really ought have dropped a good 5 to 10 points over the next 24 hrs, and everything should be able to go forward according to the MUCH delayed plan "A".

Plan "A" consists of driving to Burlington, getting into the Ibrutinib study, getting my first pills and getting the lymphoma back under control. I am really hoping that tomorrow goes without a hitch. It has been many weeks in an odd medical limbo, where I clearly need treatment - since I have been in relapse and without treatment since early August. (That is a little bit of an overstatement because the effects of the Donor Lymphocyte infusion has been doing quite a bit to keep the lymphoma from really taking off. It is just not enough to bring me into remission, or even keep me even. So I have been slowly losing ground).

So, that is what I know as far as this morning. It is likely to be different tomorrow, but that is just how this period of limbo seems to be playing out. Whatever happens tomorrow, I expect that I will be on some definite plan by the end of the week, whether that is the Ibrutinib study or we have to go back to "light" chemotherapy. The chemotherapy will prohibit me from trying again for the Ibrutinib study for at least 8 weeks, but in the scheme of things, I am sure that it will all work out.

I will try to post an update tomorrow, as I get more info.

Love you all,
Leif

Alas. Two steps back.

One Saturday, I started loosing lung capacity. Fluid in the lung. When I left for Burlington, I had a capacity of about 3.00 L. (I have a little device from the hospital that I use to test and help keep up my lung capacity).  When I got back, that had dropped to about 2.50 L. On Saturday, I lost another .50 L, and on Sunday I lost .25 L. At that point I was down to 1.75 L. I was having a issues with shortness of breath for pretty minor tasks. Don't forget, I am also down on my hemoglobin, so that doesn't help at all.

The last time I had to go to the hospital, it was for fluid in the lung, and I ended up calling 911, and getting an ambulance. I am not sure what my lung capacity was at that time, because I did not yet have my little device, but it was probably between 1.25 L and 1.00 L. So I knew that I was getting close to being in trouble. I called Dartmouth and talked to the Oncologist on call (because of course this happens on a weekend). We discussed the issue, and I was pretty confident that I would be fine until Monday, but probably not Tuesday. We made arrangements to get things rolling to get my lung drained  (the procedure is called thoracentesis) first thing on Monday.

Monday morning, I had lost a little bit more, but things got rolling and I had an appointment for 10:30am. The thoracentesis went really well. It is a much less painful procedure than it sounds. Not fun, but not as bad as a bone marrow biopsy. Honestly, it is the least painful actual surgical procedure that I have had done. They drained 2.0 L, which is the maximum they drain at a time, because the risk of the collapsing a lung climbs if they drain too much. I feel pretty good, all things considered.

The fluid comes from the fact that we have not been treating my lymphoma. I can expect to have to have another thoracentesis by the end of the week. Until I get a treatment program, I can expect to have to have a procedure about every 5 days. There is the option to get a permanent drain put in with a little spigot that I can drain when I want. While that sounds convenient, having a little

So, when can I get into the Ibrutinib study? Well, the other thing that I did today was to have my labs drawn to check my liver enzymes. There is one enzyme that needs to have a marker of about 100 in order to get into the study and the last time we drew it was 140, down from 170 a few days before. Today, beyond all reason, it tested at 173. Moving in the wrong direction. I am DEFINITELY not going to be able to get into the study this week.

Some of the issues that we are trying to balance are:
   1) I am not currently treating the lymphoma.
   2) The lymphoma has progressed to the point that my lungs are filling with fluid regularly.
   3) I have Graft vs Host disease, which bad in of itself, is preventing me from getting in the study.
   4) Ibrutinib looks to be the most promising long-term treatment currently available.
   5) If we put me on another treatment, I will not be eligible to get into the Ibrutinib study for at least 6 - 8 weeks. Perhaps, not at all.

We do not have any obvious best path. The current plan is to be assessed for thoracentesis on Wednesday by Dr. Connelly here in Waldo, and we are more than doubling the medicine I am taking to  try to fight the Graft vs Host Disease. We are going to retake my labs on Friday, and with luck, I will be within the study parameters, or at least close enough to believe that we will be with study parameters by the following Monday. If so, then off to Burlington again to get some Ibrutinib. If not, then I will probably have to go on a treatment of light chemo and hope that it controls my lymphoma and that I might be able to get into the study in a couple of months. It is definitely a poor plan B, but it is the best we have.

On the other hand, my overall prognosis has not changed. My doctors believe that the light chemo will minimize the lymphoma and my worst-case scenario is still "several years". So this setback is not too harmful in the big scheme of things.

Leif

One step forward, one step back.

Friday I went in to be officially enrolled in the Ibrutinib study, but because of my liver enzyme test, I was rejected by the company that is running the trials. The study requires my liver enzymes to be 2.5x or less of normal, and we thought that I was within that limit. The problem turned out to be that Fletcher-Allen (where the study is being run) and Janssen (the company that is running the study) have different numbers for "normal" on the liver enzymes.

There was nothing to do at that point but to drive back to Maine. I am getting my bloodwork and liver enzymes tested on Monday, and if they fall within Janssen's range, then I will head back out to Burlington to be enrolled. If they are not within range on Monday, I will be getting tested again on Friday. Everyone expects them to be within range by then. So I will be headed out to Burlington again the week after next at the latest. At this point, this is the only thing keeping me out of the study, so as soon as it is resolved there are not expected to be any more problems.

The drive back was beautiful. We took Rt. 2, which is a pretty straight shot. I think that next week the colors in the mountains will all be peaking and it is one of the most scenic drives I have been on. Next week should just be breathtaking. The weather was so clear and nice, that we took a little detour and took the auto road up Mt. Washington. I am proud to say that I climbed all the way from the parking lot to the peak. :) It was about a 200 ft rise in elevation, and I probably walked a good half mile, which are both new records for me since I got out of the hospital last time. I have lost so much muscle, and it is so slow regaining it each time. It has been especially slow this time, but I think that the Graft vs Host disease probably has strongly effected how much energy my body has to build muscle.

It was a little hazy at the top, but for Mt. Washington it was clear and beautiful weather. It was 60 degrees at the top with a very light wind. We thought we could just make out Katahdin, 330 miles away. I took some pictures, but you really need a good camera to capture that sort of majesty in the mountains. Or at least be a much better photographer than I am.

So, it was disappointing to spend most of a week trying to get into this study, and then being rejected at the last moment. It is a long trip, so I am not terribly excited to drive out there next week, but this is just part of being sick and doing what needs to be done to get better. I am confident that it will all be taken care of soon and the lymphoma seems to be pretty stable, so the week delay is not likely to be a problem with my health.

I am looking for someone to road-trip out with me next week (or the week after). It will be a day driving out, and a day coming back. I will pick up the cost of the room and meals. I may be in a position to do some or even most of the driving, but on the other hand, I may not be able to do any, so you have to be able to do 2 6-hour drives, as well as all the drop-offs and pick-ups at the hospital and probably some small errands. If we stay at Hope Lodge (which I expect) that may include cooking a breakfast.

Leif

Burlington is a nice little town.

My father-in-law drove me out here to Burlington, VT. last Tuesday. We took Rt. 2, which was a gorgeous drive. I am not big on long drives - it is a good 5 1/2 hrs - 6 hrs to get out here, but you can't beat the scenery.

Anyway, I am in Burlington in order to get access to a promising new drug called Ibrutinib. It is a targeted drug for blood cancer - lymphoma and leukemia. One side has a part that fools the lymphoma into thinking that it is glucose, and the other side has a protein that destroys an important enzyme in the lymphoma, and causes the lymphoma to self-destruct. It does very little damage to other cells in the body. As an added bonus, it appears that it may work even better on my rare, aggressive form of lymphoma than it does on other lymphomas because the type that I have is especially aggressive with the take-up of glucose.

When I went in to be approved for the study, on Wednesday, I was in the process of being treated for Graft vs Host disease, with a medicine called prednisone. It turns out that this medicine is prohibited by the study, so for a while it looked like they were not going to be able to take me after all. However, after some long discussions between the Dr Sprague (the doctor leading the study), the people funding the study, and my primary oncologist, Dr. Meehan, they were able to come to an agreement. I was immediately taken off Prednisone (which is pretty rough), and put on another drug to combat GvH disease. Unfortunately, that drug is Cyclosporine, which is my least favorite drug I have ever taken. It tastes like spoiled asian mushrooms. It is also an immune-suppressant, so I am back to being neutropenic for all practical purposes. At least I am not likely to have to take it very long - only until my GvH is under control - probably for a couple of weeks. Last time it was about 8 months.

Anyway, that seems to be all sorted out, and I am meeting with them at 9:00am to be officially accepted into the study, and given my first dose of Ibrutinib. I will be coming back to Burlington once a month until the end of the study, which for me, means either: (A) I am cured (vanishingly unlikely), or (B) the drug has become commercially available, which is likely to happen sometime early next year.

I heard through the grapevine that Dr. Meehan is considering the possibility that if the Ibrutinib gets me into full remission, that the best therapy would be another bone marrow transplant. I have not discussed this with him, and can not say that I am particularly excited by the idea. There will be plenty of time later to have that discussion and to talk about the best next steps if I do go into full remission. Right now, just the possibility of getting to full remission would be pretty great.

In other news, my fatigue, which was grueling over the last 3 weeks or so has let up somewhat, and I can mostly get through a full day. My appetite is back, and I have managed to put on about 15 pounds, so I am back up to a more reasonable 170. Probably 5 pounds of that is fluid retention from the Prednisone, but I have put on some muscle, and am able to go for *very* short walks. I lost a lot of muscle the last time I was in the hospital. Or rather, not a lot of muscle, just a high percentage of the little that I had left.

2-3 hundred yards is a pretty tough hike for me right now, but that is up from one hundred yards last week. I can also manage a flight of stairs, which I couldn't last week. Little gains, but important.  I have some hope that I will be out of the hospital long enough again to get back to walking 4 miles a day, which is what I had gotten up to earlier this summer. I love to hike, and there is still quite a bit of time before it gets too cold. My neuropathy is such that last winter, I could not really get outside much because I just don't get any heat in my hands and feet. Warmer gloves help a little, but without circulation, your hands just start losing heat until they feel like blocks of ice. I think my neuropathy is a little worse this year than it was last year, so if anyone has a good idea on what might help, let me know. I have tried the hand warmers, but they didn't work out very well. I have heard that there are gloves that actually have little battery-powered electric heaters. Has anyone tried them?

All for now,
Leif

Back from Dartmouth, off to Burlington.

Last week was pretty busy. Luckily, my fatigue seems to be easing off, so I have been able to do more of what I need to get done. I had labs taken and a dose of a light chemo drug called Velcade on Tuesday at Waldo, and then a friend and I drove out to Dartmouth and I had a PET scan and discussed the results with Dr. Meehan.

It was not any surprise that I am back in relapse. I had a little bump on the side of my neck last week that I recognized as a lymph node growth, so the DLI and the Velcade have not been able to overwhelm my lymphoma. The PET scan showed that I have many dozen highly infected nodes throughout my neck, chest, lungs, extending down into my lower intestines.

The good news, as far as it goes, is that the nodes are small. My prognosis has not changed, we are sure that we can keep this in a state where, while not in remission, will not spread very quickly. My worst-case scenario is still several years.

However, I am on my way tomorrow to be enrolled in a study with a promising new drug called Ibrutinib. It has been pretty effective on blood cancers in the Phase 3 studies. It is not a chemo drug, but instead has a little fake glucose "hook" that the lymphoma love and suck up quickly. The other side of the drug has a marker which tells one's B-cells and T-cells that the lymphoma is a foreign body and should be destroyed. As an added bonus, the drug is a pill, which beats getting stuck with a needle every day.

If it goes well, I should be starting the drug on Friday. My oncologist is pleased to have me be able to get this drug because while the other therapy will mostly control my lymphoma, this has a small chance of actually curing my disease. And, before you get your hopes up too much, I really mean small - less than 1%. But it does have perhaps as much as a 60% chance of controlling my lymphoma indefinitely. And since it is very targeted, it will not damage the rest of my body which might mean that I can eventually get back to a relatively normal life.

I do have a little bit of trepidation that I am going to share though. What if it does not control my lymphoma? I will be talking to the people running the study about when my first check-up will be in order to see if this drug is working like it should. Because I have a very aggressive form, a month can make a huge difference. I don't want to be in a position where, unbeknownst to us, my lymphoma has been growing quickly, and by the time we notice, it is too late to get me back into the state that I am currently in, where it can be controlled by a known regiment. I have done a lot of research, and I am willing to take this risk - everything that I try at this point is risky. But still, there is that little bit of trepidation.

I have beat crazy, ridiculous odds, over and over again, to still be alive after 18 months with this disease, and I know too much about probability and statistics to not have some fear over a new treatment. Still, between self-advocacy and tenacity, I am likely to make it through. And with one more dose of crazy luck, perhaps this will cure me after all.