We'll miss you.

Memorial for Leif

We'll be holding a memorial for Leif this Saturday, November 16. We apologize for the late notice, but we'd like to do it while everyone is in town. That means that we need help getting the word out. If you think someone would like to be there, they're invited.

We'll hold it at Waterfall Arts in Belfast, 256 High Street, Belfast, ME starting at 6:00pm.

Please bring your favorite Leif story to share. We'll have a microphone and a pretty easy audience.

This is a potluck. Please bring something to eat and drink, and enough to share. Family-friendly, and alcohol allowed. Special bonus points will be awarded for things that Leif liked, but anything will do. There is a kitchen on-site, so if you need to warm it up, we can accommodate.

UPDATE: Flowers are welcome tonight, but perhaps better would be donations to Big Brothers Big Sisters of Mid-Maine, the Leukemia and Lymphoma Society, or the science education nonprofit of your choice.

If you need to ask any questions, feel free to contact Mike at 410-350-5000 or Scott at 207-323-0609.

Update with hotel info compiled by Erica Buswell and Becky:

Some things you should know about getting to Belfast

Travel Arrangements

Belfast and Brooks are in lovely mid-coast Maine. If you are flying here, the major airports that serve the area are in Portland (PWM) and Bangor (BGR). Portland is located about 2 hour’s drive from Belfast; Bangor is located about 1 hour’s drive from Belfast. Flights to PWM are generally cheaper than flights to BGR. Smaller regional airports in Rockland (45 min.) and Augusta (1 hour from Belfast) are also served by Cape Air.

The Amtrack Downeaster provides rail service to Portland, Freeport, and Brunswick from Boston.

Travel Accommodations

Belfast has a number of different places to stay, ranging from cheap and simple, to more luxurious and expensive. Here’s a few suggestions.

Cheap(er):

• The Gull Motel: http://www.thegullmotel.com/
• Tke Yankee Clipper: http://www.yankeeclippermotelbelfast.com/
• Belfast Harbor Inn: http://www.belfastharborinn.com/index.html

More luxurious:

• Comfort Inn, Ocean’s Edge: http://belfastmainehotel.com/
• Alden House B & B: http://www.thealdenhouse.com/Site/Welcome.html
• The Jeweled Turret Inn B & B: http://www.jeweledturret.com/index.html

Other things to do while you are in the area

There’s lots of lovely shops and fun hangouts in downtown Belfast. Good eats, too, especially the Belfast Food Coop, and Chase’s Daily. Belfast is also a convenient distance from day trips all over Maine!

• 1.5 hours from Acadia National Park
• 30 minutes from excellent hiking in Camden Hills State Park

Visit the Penobscot Marine Museum in Searsport

Posted by Mike Aaron

Leif has died.

Leif died this morning a little after 11:00am. He's been surrounded by friends, family and lots of love. We're now sitting around telling Leif stories. We might finish in several years.

We are working on a memorial for this Saturday night in Belfast. We'll post details soon.

He was an amazing man and an amazing friend. We will all miss him deeply.

Posted by Mike Aaron

Right now . . .

Posted by Erica

We are in the 2nd Floor South Med/Surge family waiting room at MidCoast Hospital, visitor command center for the time being, mostly waiting for more details about what to do next. As many of you have been informed, it looks like Leif's liver is beginning to fail. He is jaundiced, and sometimes agitated and confused, but his pain levels have improved. Nothing is clear right now, not even a clear path forward. If you have something you are moved to share with Leif, now, right now, would be a good time to be in touch.

For the time being, it sounds like Leif may likely be up for taking brief phone calls: you may call his google voice # which is 619-4446 (this is also just the phone # to the room).

Cards and notes (but no flowers, please!) can be sent to Leif c/o MidCoast Hospital, 123 Medical Center Drive, Brunswick, ME, 04011. You can also send Leif an e-mail c/o the hospital; this is probably the best way to get in touch as Leif and Becky are not reliably checking e-mail under the circumstances. If you are interested in visiting, please call Leif's phone to check-in. There should always be someone at the other end of the line to let you know whether its a good time to do so.

Sunday afternoon doctor visit

Posted by Becky

I was mistaken about Leif's steroids; Dr. Connelly has left them at 30 mg, which was the amount they tapered down to a few days ago. She thinks there is more going on with Leif's liver than GVHD. She has a call in to Dr. Meehan to discuss this further. Specifically, his ALK and ALT levels have come down, but his bilirubin is going up. That's not typically a sign of GVHD. It could be a medication effect, or it could be caused by blockage of a bile duct (e.g. by a swollen lymph node), in which case it is possible to put a stent in. She has ordered a CT scan for tomorrow, which will give her a good look at what might be going on in his liver and also a look at the fluid around his lungs, which appears to be continuing to diminish. They've changed his antibiotics from every 8 hours to every 12 hours to see if that helps.

She was pleased to see that Leif's platelets have come up slightly, from less than 5 to 7. Although patients typically get a platelet transfusion when they are below 10, if one is not actively bleeding, it's not clear that platelet levels in this range are harmful. They are given as a preventative measure. Due to the cannula (little plastic doohickey) that delivers supplemental oxygen to his nose, plus the low platelets, Leif's had some minor nose bleeding. That's pretty common, and not the kind of bleeding they're worried about. The oxygen is bubbling through water to make it gentler on his nose, but it's still drying to have oxygen blowing up your nose 24/7. The platelet shortage is due to a recall, and is affecting all of New England. More platelets are being shipped in from around the country and should be here tomorrow.

Let's hear it for blood donors everywhere. Let's also hear it for Eliza, who generously changed her Sunday plans and is bringing Leif's cyclosporin (and my Kindle) down to us this afternoon. I am grateful that there are so many people who love Leif and who are helping us through this in ways large and small.

Otherwise, Leif has been having a quiet day resting up after seeing a lot of visitors yesterday. His pain is being controlled okay now, and mostly consists of back pain. We're using a heating pad and I've been giving him back rubs (gently, since his platelets are low). I'm also having a quiet day resting up, after a quiet day resting up yesterday.

Liver enzymes elevated, platelet shortage

Posted by Becky

A couple of days after reducing Leif's steroids, yesterday his liver enzyme levels were elevated quite a bit. This was disappointing. They have increased his steroid dose again and* the numbers have started to come down.

Plan B (which is not very good, otherwise it would be Plan A) is to hope for FDA approval of Ibrutinib sooner rather than later. I don't know how long it takes between FDA approval and drug being available to buy (assuming the company has to set pricing, scale up manufacturing, and commence distribution of drug to pharmacies). If any of my pharma friends have experience with this, please drop me a line.

His platelets are at 7 today (yesterday they were below 5). Normally they try to keep Leif above 10, but there is a platelet shortage. So they are going to hold off and see how his levels are tomorrow.

If anybody has time today to drive from Brooks/Belfast down to Brunswick - Leif's cyclosporin bottle is running dry (it has bubbles in it when he draws up a dose), and we can't tell how many doses are left in the bottle. Maybe not one, although he had enough for his morning dose. My cell phone just broke (every time I open it, it tries to speed-dial 7, and none of the other keys work), so I can't call anybody unless I have their phone # memorized, which is basically nobody. If I don't hear from someone within an hour or two, I will drive up to Brooks to get the medicine and bring it back down. Before I leave I will stop by the local US Cellular store and see if they are open on Sundays (the Belfast one isn't) to replace my phone.

*They have kept his steriods steady. See the post from later today for more information.

Quiet day getting blood and platelets

Posted by Becky

It continues to look like the bendamustine and revelimid got a response; Leif's chest xray from yesterday shows no additional fluid accumulation around his lungs, and it seems to me that the swelling in his legs is less than it was, although it's still significant. Now we are basically going to sit around and wait while he adjusts to lower steroid doses, and his platelet count comes back up. That will take an undetermined number of days; different chemo regimens suppress bone marrow for different amounts of time, and of course Leif's bone marrow has taken a beating. He'll have to hang out in the hospital until his pain is under control  by oral medication, and presumably until he doesn't need quite so many transfusions every day, and is done with the IV antibiotics.

Leif's pain level is still high, but not as bad as it was yesterday. Dr. Connelly says that it is likely due to coming off the steroids, which had been preventing him from feeling the pain so much. She increased his painkiller dose, but also says that his pain should lessen as the chemotherapy continues to take effect. He's been sleeping since he finished taking his pills this morning. He's getting two units of platelets and two of blood today, so he'll be hooked up to an IV all day anyway. 

Because his painkiller is on a PCA, and blood products can't go through the same line as anything else, he had to have an IV put in his arm this morning for the painkillers, while the blood goes into his port. He's not thrilled about that, but you've gotta do what you've gotta do.

I was up late last night keeping an eye on Leif's respiration - he was doped up and loopy, and the nurses were worried. If his respiration dropped too low, they would need to give him medication that would drop all the painkillers out of his body at once. Ouch. At about 3 AM, one of his narcotics wore off and he seemed to be just asleep, as opposed to knocked out, so I was able to get some sleep at that point. I'm going to try to take a nap now.

Pain day; update on Ibrutinib

Posted by Becky

We just met with Leif's main local oncologist, Dr. Connelly. She has spoken extensively with the people in Vermont who are involved in the Ibrutinib study. She explained to them that if Leif is going to meet the inclusion criteria, that time will be very short. He needs to be on no more than 20 mg of any steroid, so she will begin tapering Leif's prednisone starting today. His liver numbers are completely fine, so any GVHD is being managed well by the immune suppressants he's taking. And, his platelets need to be at 50. They are now around 10. Platelets are the first blood cells to recover after chemotherapy. He needs to pass the screening labs in Vermont, so the plan is to keep close tabs on his labs here, and as soon as he meets the inclusion criteria, we are jumping in the car and driving at speed to Burlington, where he will re-consent, get screened again, and, if - no, when - he passes, get the medication within 48 hours. Dr. Connelly has asked them to do what they can to have as little time lag as possible within passing the screening and getting the pills.

Leif continues to breathe okay thanks to the Bendamustine he got yesterday and the day before. He's on 3-4 liters of supplemental oxygen, down from 4-5. A noticeable node in his chest is smaller, according to Dr. Benton, Dr. Connelly's partner who is in the hospital on the days she's in Belfast.

He's having a bad pain day today, with pain in his abdomen (probably because his poor spleen keeps growing and shrinking; bad bounts of constipation are not helping there) and back, which often flares up when he can't move around much. He still has quite a bit of edema in his legs and feet as well. Dr. Connelly is prescribing a PCA pump, those lovely machines where patients can self-administer painkiller until they're on top of the pain. Leif is a pro now at knowing what works to get on top of pain and then manage if from there.

A thousand thank-yous to everyone who's putting feelers out about an alternative method of getting Leif some Ibrutinib if he doesn't squeak by the study parameters. What may help is a site that might be slightly more flexible about inclusion/exclusion criteria, or something along those lines. The stress of not knowing whether he will be able to get timely access to this promising drug has been taking its toll on us. Other than the lymphoma, he's still in good shape - young, no complications like diabetes or cardiovascular disease or out-of-control GVHD. He's super methodical and compliant taking his medications, eating healthily and exercising as he's able, and of course he has the legendary Leif tenacity. So I feel like he's a great patient to include in a study.

(Details for people with friends and colleagues in the industry - the drugmaker is Janssen, a subsidiary of Johnson and Johnson. Pharmacyclics did the early drug development. The contract research organization running the study is INC Research, and the local IRB is the Committe on Human Research at the University of Vermont. This is the study, and here is the press release describing its status as an expanded access study.)

Port issue

Posted by Becky

Leif's port is having issues. They can get flushes in, but they can't get a "blood return" out. Last night they tried accessing it several times, which of course makes Leif sore. They tried putting in a bunch of heparin to clear any clogs, to no avail. They were hoping to be able to do a port scan today to maek sure it is still in the right place, but the machine is booked all day, so instead they are soaking the port with "cath-flow," a heparin-like solvent. They can do that twice. If they still can't get the port going, they'll put an IV in Leif's arm and give him the medication he needs today though that, and scan the port tomorrow.

Leif's breathing seems about the same as yesterday.

UPDATE 1:43 EST: A second cath-flow soak did the trick. No need for a peripheral IV. Phew.

Waiting for Chemo

Posted by Becky

Leif's thoracentesis yesterday afternoon has given him some breathing relief, although he still needs suppemental oxygen. They took out as much fluid as they were able to, about 1500 ccs, which is about what they got on Friday. The remaining fluid is thick and locked up in small pockets, so they would not be able to remove it without surgery, which is not a good idea right now. The pulmonologist plans to do another xray and thoracentesis on Thursday. The hope is that the chemotherapy (Bendamustine) which Leif will be getting today will help stop the fluid from getting blocked by lymphoma and backing up into the space around his lungs. Leif has also switched to an extremely low-fat diet; some researchers think that can reduce production of the substance, chyle, that's getting backed up into his chest cavity. That's fine with Leif; he is comfortable on a very low-fat diet, so he won't feel deprived eating steamed broccoli and bean soup.

He got a unit of red blood cells and the plan is still to give him Bendamustine today and tomorrow, now that the infection is under control. The nurse is starting his pre-medications now.

Blood counts down

Posted by Becky

Leif's blood counts are down today, probably because of the lymphoma. They are going to give him a second bag of platelets, and then a throacentesis. They can't take a whole lot out, up to 2 liters, which will be helpful but probably not as much as he needs to breathe comfortably.

Due to the low blood counts, Leif is not qualifying for the Ibrutinib study. His doctor is investigating getting the drug through "compassionate use;" some pharma companies will provide drugs to very sick patients who have no other options. In the meantime, they are discontinuing Revelimid in hopes that will help his blood counts come up, and giving him Bendamustine (chemotherapy) today and tomorrow in order to hopefully see a response and buy some time. Although it is not great at keeping the lymphoma in check, he did respond rapidly (though incompletely) last time he got it, so we hope it works again somewhat.

He's having quite a bit of trouble breathing, even on supplemental oxygen. His legs are also swelling due to the lymphoma.

Baffles

Posted by Becky

Leif's infection is under control, as is the liver GVHD; the new goal is to get his platelets up to the level required to get Ibrutinib. There's always something.

His last thoracentesis was not very effective, so he's having a lot of trouble breathing. Apparently if a person has a pleural effusion (fluid building up around the lungs) for long enough, their body starts creating baffles within that cavity - which, if the fluid is coming in from a single location, is a great idea. But in Leif's case, where the fluid is coming from everywhere, it means that a thoracentesis (or a chest tube) can't drain more than one small pocket at a time. It also means that instead of having positive pressure (i.e. the fluid wants to drain on its own) you start getting negative pressure (the fluid doesn't want to come out). Once this baffle-making process starts, it can't be stopped. The pulmonologist wants him to get a surgery that will glue some membranes together with talcum powder, which will stop the process. If the baffle creation goes on too long, they can no longer do this surgery to correct the issue. However, his Maine oncologists would rather see Leif get Ibrutinib to treat the underlying cause. They feel that the surgery, although routine, is still surgery, and thus risky when his platelets are low and he's neutropenic. It's not something they can do at Midcoast Hospital - he'd have to go where there is a thoracic surgery specialist. Leif has an email in to his contacts at DHMC, asking for their opinion. We tend to want to go with the oncology team's recommendation. In the meantime, Leif is uncomfortable, breathing shallowly, and using supplemental oxygen. He's in a lot of pain and rather foggy-headed. Another thoracentesis is planned for Monday, and he may be getting platelets tomorrow as well.

I'm down at the hospital now, and Leif's parents are dog-sitting.

Oops, I posted the wrong phone number

I gave you the wrong phone number in the last post. I hope that having too many digits clued most of you in. It should have been:

(207) 619 4446

I will write more later. Very sleepy right now.

Leif

Kinetic failure of treatment.

It has been quite a while since my last post, and a lot has happened. The chemotherapy worked really well. My lymph-nodes got smaller, and my spleen shrank a little. My energy level was good. I was taking little walks every day.

The chemotherapy cycle last 21 days. I take the Revlimid that last for 14 days. The other two drugs are IV, but I only have them for the first 2 days of the cycle. Then I have a week to recover, before starting the next cycle

On day 18 of my cycle, four days after I stopped taking the RevLimid, I noticed that the lymph-node had started growing again. There were other signs that the lymphoma was shrugging off the treatment.
I had an appointment scheduled for today, Wednesday at DHMC to see Dr. Meehan, so I was not too worried about it. But on Monday, my labs, especially my white blood cells count was low enough that Dr. Connelly wanted to wait until she saw me before starting the next round of chemo.

Tuesday, I met with Dr. Connelly and mentioned that over the last two days or so, my energy levels have been dropping. We went over the blood labs that were drawn on Monday. It seems that I have picked up a fever - I am running at about 99.5 currently, but was 100 on Monday. I have some infection, but we don't yet know what it is. Probably something that something that a person with a working immune system would be able to shrug off and not even know that they had it.

The bad news is that this chemo regiment is not working. Each therapy knocks back the lymphoma, but the lymphoma gains more in the recovery week than I do. It is called kinetic failure. We currently have no back-up plan.

It is still worth staying on the treatment until we figure something out, because it at least slows the progress of the lymphoma. But I can not start my next round until I have this infection under control.
So I

I am stressed that Plan B did not work. On the bright side, such as it is, failure of treatment is one of the criterion for being accepted in the the Ibrutinib study, which is a more likely cure. It looks like my Graft vs Host disease is now under control, which is what was keeping out last time, and Dr. Meehan, Dr. Connelly and Dr. Sprague had a long conference call about the best way for me to enter the study.

But we will see what happens.  So, currently, I am it Mid-coast Hospital for an indefinite period of time. Perhaps as little as three days, probably not longer than a week. They have better resources to take care of my infection. In fact, they have moved me into ICU. I feel pretty good, all things considering. I hurt in the morning, and have a hard time fulling waking up before about 11am. The nurses down at the special hematology/oncology unit, along with my fever and low blood pressure took this to mean that I was in rougher shape than I think that I am. I am certainly the most mobile patient in the ICU, and my insistence to get out of the room for even a little walk through them all for a loop. I did manage to get my walk in finally.

I only get one bar of cell service in here, so it is dicy to call out. You can call in using my new number:
(207) 619.4446

I am typically feeling up to chatting after about llam.

Leif







.

I feel good.

I had my thoracentesis yesterday. Since Wednesday, I have had less fluid draining into my lung, so I was in much better shape than I thought I would be. Usually, I have to be pushed around in a wheelchair by this time of the week, but yesterday, I was able to walk everywhere.

And by "walking" I really mean "ambling forward". I was still struggling for air, but it was faster than going and getting a wheelchair. In fact, my doctor and I had a fairly long conversation on whether it was worth doing the draining, or whether it made sense to wait. I was not sure that I would make it all the way to Monday though, and did not want to be stuck having to come into the emergency room for it. We decided to go ahead and take off as much as we could, and if the plural effusion is slowing down, then I just get a lot longer before I need the next treatment. Plus, I get extra air all weekend.

So we went ahead and he drained 2.5 liters. Which is a lot. My follow-up x-ray showed that we had drained nearly ALL the fluid from the lung. In fact, I am a little sore because that part of my lung has not seen any air for a long long time. I can sort of feel the lung work at re-inflating with every breath I take. Yesterday was pretty much a long coughing fit, as I worked out the phlegm that had pooled down there. That was probably TMI.

Air is GREAT. I have been a little high from the extra oxygen. Also, it is fantastic that my chemo seems to be working already. I did not expect to get any real benefit for at least several treatments because my form of lymphoma is so well known not to respond to light chemo.

There are some side effects. I have been having a lot of trouble sleeping. My appetite has been suppressed, and food mostly tastes bad. My neuropathy (tingling and numbness) in my hands and feet has gotten worse, and now I have a little in my face.

And, of course, this "I feel good" is on a baseline of unbelievable crappy, but I don't care. Any improvement feels great. The way up is WAY better than the way down. It feel good to be able to breath, walk a little, and be able to do a few little things. When getting dressed for the day does not take ALL your energy for the day, it is a good day.

Leif

Dr. visit and Surprise Chemo!

Met with Dr. Connelly this morning. Got a bunch of questions resolved, and got a good visual check. It is nice to have a MD look me over and get a professional appraisal of my condition. She felt that the chemo was already having an effect vs. the last time I had seen her. I sort have thought so too, even though it usually takes a week or so to kick in. It is so easy to be a little in denial and try to look at all the good signs, that I am never quite sure if I am fooling myself or not. So having her look me over and be please felt really good.

So a visit that I thought would take no more than 45 min. or so came with quite the surprise. Apparently, there were three compounding errors in the pharmacy and the notes on Bendamustine from DHMC. For this particular therapy, I am supposed to have Rituxan on day 1, Bendamustine on day 1 AND 2, and Revlimid on days 1-14. No-one at the Oncology clinic knew about the day 2 of Bendamustine. So I had it today. No harm done at all - a day doesn't make any real difference here. I am just glad that Dr. Connelly caught it, and it was only delayed by a day.

Still, it made for a long day. I expected to get out by noon, and with the pre-treatment drugs and the Bendamustine, I did not get out of there until 4. I know that when I enter a hospital, I have no expectation of my schedule, and I brought my computer, and got just as much done there as I would have anywhere. Be that as it may, "Surprise Chemo" is low on the list that one wants to hear. :)

I am still feeling better, and it is clear to me that my plural effusion is already slowing down. I am not nearly as winded today as I expected to be. I am still getting a thoracentesis on Friday, because I will not be able to hold out over the weekend, but Dr. Connelly and I both expect that I will not have to be coming in every 4 days, but it will start stretching to 6 days next time, and after only a few more draws, may likely become resolved. I can't express how much I am hoping for that outcome.

I also learned that the stuff that they take out of my lung is not just heavy in fluid, but high in protein, so Dr. Connelly insisted that I start taking protein supplements. I went for one that has both soy and whey protein, has one of the higher protein amounts per serving, and is guaranteed to "taste great". It tastes pretty industrial to me, but it is drinkable, and as medicine, not that bad. I might get used to the taste after a while. It certainly explains why I have not put on any muscle since I have gotten home though. I had been wondering about that. I try pretty hard to get back in some sort of shape after each hospital visit, and I have not made hardly any progress for more than a month.

Leif

Long, but good day yesterday.

I feel SO much better.

I had so much going on, so many things were up in the air about what was going to happen next, and I was really low on air. Lack of oxygen was making me extremely foggy, and I had not been sleeping well as my back has been hurting when I lay down, so I was also somewhat sleep deprived. Makes for a hard morning when you need to make sure that certain very important procedures are done, and no-one else at the hospital had yet heard of my plan (to check first to see if I could get into the Ibrutinib study). It was not much different than the current plan - mostly about timing on the labs, but they had not scheduled my thoracentesis (the surgery where I get my lungs drained). So I had to be sharp to get that all taken care of by 8:30am. Whew.

So, we took my labs and they were able to schedule the thoracentesis immediately afterwards. They removed 2.8 liters of fluid. When you see it sitting in the jar  - it is a LOT of fluid. It is no wonder my back has been hurting. It weighs just over 6 pounds, and is in sort of an awkward place for you body to be carrying extra weight. It also made me think that I really ought to be drinking more liquid. They are taking that much out about every 5 days. It has to come from somewhere. My body should be USING that stuff, you know? 2.8 Liters on top of what I ought to be drinking to keep my kidneys flushed from my medications, on top of what people ought to be drinking every day. I probably should have something to sip at all day long.

Anyway, the thoracentesis went really well, and within an hour or so, my color came back, I could THINK again, I had the oxygen to walk. I don't feel "normal" of course, but I feel pretty darn good all things considering.

When I got back to Oncology, they had my test results, and I still have Graft vs. Host Disease. This was the most likely outcome, despite my plan, so I was not disappointed. I just think that if I had not given the other a try, I would have regretted not knowing if I could have made it into the Ibrutinib study. Also, it was nice that my lab was not close. I had to be under "43" and I tested at "91". The GvHD is under control - that is a lower number than my last test, but it was not a "45" or something that would have been SO close as to keep PLAN A into effect. It was a relief.

We started my chemo immediately afterwards. My chemo consists of Rituxin, Bendamustine, and a new pill called Revlimid. Historically, I have had reactions to Rituxin. Everything from full-blown rigors to relatively mild chills. This time went very smoothly. I had a few moments of slight shortness of breath, but other than that, I did not even notice that I was getting chemo. We put the Rituxin in pretty slowly - it took about 4 hrs, and we waited longer with the pre-meds. I did not get out of the hospital until 6:30pm, so it was a long day, but it went really well. This chemo plan is similar to the one that I had at Dartmouth-Hitchcock, and there it was able to get my lymphoma suppressed enough to for the DLI (the Donor-lymphocyte infusion, where they give you more donor T-Cells to help your body's immune system fight the lymphoma). So we are pretty confident that it will help considerably, although we are also pretty sure that it will not actually put me into remission - which is why trying to get into the Ibrutinib study had been so important.

We believe that this chemo regiment will hold me over until Ibrutinib is approved by the FDA and becomes available as a general treatment. That is supposed to happen in late spring, although I heard a rumor recently that it could be as early as December.

My current chemo schedule will be ever 21 days. I had thought that I was supposed to go in weekly, so that was also a pleasant surprise. Hopefully, I should see some improvement in my plural effusion (the liquid going into my lungs) in the next 2 weeks. I am looking forward to that.

I thought I would be just beat today, and I am tired. Yesterday was a long day. But so much stress has been lifted that while I am tired, and not going to tackle anything big. I feel pretty darn good.

Leif

Long morning, new thought about the Ibrutinib study.

Slow to get going this morning. I did not sleep very well. When I lay down, I get wheezing in my lungs, which is not uncomfortable, but is actually loud enough to keep me awake. I hope that this is a temporary thing caused by the constant agitation of my lungs filling with fluid and being drained, and once my plural effusion is under control, it will calm down and stop. In the meantime, it can make for a long night.

It occurred to me today that since I have been on the Prednisone for a full week since my last lab, I might be completely over my Graft vs Host Disease (GvHD), and that it still might make sense to take the lab, show that it is under control. Get off the Prednisone until Friday - send THAT lab to the Ibrutinib study people, and if it shows that I am indeed over the GvHD, drive out Monday the 13th to get on the Ibrutinib. It would mean one more week without treatment, but it might still be worth it. I would definitely need solid assurance from the Ibrutinib study director that this would be acceptable, but since it was the plan that he suggested, it is possible that the third time would be the charm.

I think that it is worth a call to Dr. Meehan monday morning. I could have the labs drawn at 8:30, get my thoracentesis, and know whether my GvHD was cured by the time I got back from the surgery. If it is not COMPLETELY cured, it is obvious to me to go ahead with the treatment that Dr. Meehan and I discussed on Friday. But if it is COMPLETELY cured, perhaps it makes sense to take one more stab at the Ibrutinib study. I could even delay taking my Prednisone until after my lab came back, so if I am clear, Monday would count as a day off Prednisone. That means that potentially, I could get a lab on Thursday sent off and get treatment on Friday.

Something to think about tomorrow. It is important to look at every possibility and think things through. Even though I did not get into the study last time, I should not close the door if the benefit of waiting 4 days outweigh going on the Rituxin treatment immediately. If I take 15 mg of Prednisone tomorrow (instead of 70 mg) , then Sunday will count as a day that it is under control and I could potentially get treatment on Thursday. You have to take your doctor's knowledge seriously, but at the same time, I am the one that is finally responsible for my health and treatment.

That is a lot to try to coordinate Monday morning before 8:30am, especially since I know that I will be a little foggy (I always am on the day of my thoracentesis because I am just not getting enough oxygen to my brain for brilliant thought). I suppose I ought to call the oncologist on call, both at Dartmouth and at Midcoast and discuss it with them. If I go ahead, I need to get a sign-off that I only took 15mg of Prednisone on Sunday. 15 mg of Prednisone is acceptable in the Ibrutinib study parameters.

I can't really see a down-side to that. One day with a lower dose of Prednisone will not make a difference with GvHD, so I am not risking anything there if i find that it is not under control on Monday. Fluctuating one's Prednisone dose has the side effect of feeling horrible, and a usual taper from 70 mg would usually be over a couple of weeks, but this is a pretty minor discomfort in the scheme of things. If it works out, I will probably be better off in the long run.

You do the best you can with what you have to work with.

Leif

Thursday 10/3, in limbo for most of the day.

Had a nice breakfast of leftover indian food, and a volenteer came in to Hope Lodge and made a nice breakfast of pancakes and eggs for everyone that was staying there that day. Real maple syrup for the pancakes. I had one even though I was already pretty full from saag paneer.

I met with Hannah (the Ibrutinib nurse coordinator) at 8:30am. She was so upset that she had called me and confirmed that I was in this study and then it turned out that I was not. She hoped that I would be able to go one more week, show that the Graft vs Host Disease (GvHD) was under control, and enter the study anyway. I explained that in my case, that was probably not an option.

Unfortunately, I could not just start a new treatment, get the lymphoma under control and then switch over to the study. The point of an access study is to get the treatment to patients that are NOT responding to other treatment. If I start a treatment, and respond to it then I can not get into the study. We have to show FAILURE of the treatment to be able to get back on list for the Ibrutinib study. She had talked to Dr. Sprague about it, and Dr. Sprague (who was not in that day), had discussed it with the study director. They had decided that they were willing to use a pretty flexible definition of "not responding" to treatment. For example, I have a reaction to Rituxin (one of the drugs that is in my plan B treatment). We mitigate it by giving me the Rituxin VERY slowly. We don't have to. We could give it to me just fast enough to elicit a reaction, and that might be construed as failure of treatment. This gives some possibility to get back on the Ibrutinib study in a couple of months. Definitely not a PLAN A sort of path, but you search for solutions when you can.

I wrote Dr. Sprague a nice note, thanking him for arguing my case to the study director. If he did all he could have, he will appreciate it, but if he weaseled a bit, I think he will try harder next time. :) The Prednisone / Ibrutinib conflict may well be more serious than I appreciate, and that it really is in my best interest not to be on both drugs at the same time. For example, brain hemorrhage is listed as a possible side effect of Ibrutinib.

It was SO nice to get my thoracentesis. I hate surgery, but this is relatively minor and painless, and I really love being able to breath. It was nice to be getting some oxygen to my head. Here I am, it is 10:30 am. I am not in a treatment program. Dr Meehan (my primary oncologist) assumed that I WOULD be in a treatment program - the Ibrutinib study, and there is no back-up plan in place ready to go. I have lived long enough with this disease to be in uncharted territory. No one know what treatment plan might work. There is no proscribed plan. I know that at this point, Dr. Meehan is calling his colleagues around the country (he excels at collaboration) and working to come up with the best possible plan, given the circumstance.

But I am in complete limbo. I definitely wanted to start treatment on Friday, if possible, rather than have to wait through the weekend and start on Monday, or even Tuesday. Tuesday (the day that Dr Connelly - my local oncologist is at my local hospital - otherwise she is at Midcoast, down in Brunswick). Do I drive to Dartmouth-Hitchcock? It is only a couple hours from Burlington. I definitely did not want to drive back to Brooks and then have to drive out to Dartmouth on Friday. Or Monday, for that matter. Did I need to go to Midcoast, down in Brunswick? That is a 6 hr drive, and I would need to get a hotel room. Do I just head back to Brooks? I had no idea. I called Kate (the nurse coordinator at Dartmouth Hitchcock), and she assured me that they were working to get something in place and would let me know as soon as possible.

For the meantime, I went ahead and reserved another night at Hope Lodge. It is free to cancer patients seeking treatment at Fletcher-Allen (the hospital in Burlington), and I decided, close enough. Also, the manager of Hope Lodge called me to let me know that a volunteer was coming in to cook indian food for supper that night. Isn't that sweet of him? Calling me to let me know because he knew that I liked indian food, so that I wouldn't make other plans without knowing about it.

Liza, my temporary caregiver for the trip, and I went and got Vietnamese food from this little food market that also has a restaurant, where the waitress is clearly the owner's 12 year-old daughter. The ambiance is horrible (being in the middle of a grocery shop with a large-screen tv permanently on the Disney channel), but the food is delicious. We took our order to go and ate back at Hope Lodge.

I got a call about 3:00 pm. While I was hoping to go to Dartmouth-Hitchcock and be seen by Dr. Meehan (I really just wanted some hand-holding here - it had been an unsettling day), the plan was to start a treatment of Rituxin, Bendamustine, and a new drug that had been released in March called Revlamid. Revlamid is a pill and has been shown to be effective with lymphoma, but no-one know the mechanism that it works on. It is related to thalidomide, so there are tons of restrictions about the dangers of getting pregnant, none of which apply to me. Thankfully. It was developed for myeloma, and just recently was found to have a benefit for lymphoma. This treatment could all be done at Waldo Hospital, and I am schedule to start on Monday at 8:30. This will be a long day because I have to take the Rituxan so slowly. Also I am having labs drawn and a thoracentesis. Unfortunately, I will not have a chance to talk with Dr. Connelly, but it better to start right away, and I will probably meet with her on Wednesday.

Dinner was good, went to bed early, and had nice drive home.  thought the scenery was nice on the way out, but on the way back it was just peak. I wanted to close my eyes and rest for a while, but everything was so gorgeous that I just stared out the window and the colors and the looming blue mountains in the backgrounds, the dark of the conifers, the sparkling little brooks. One of the most beautiful autumn scenes I have seen in the last 10 years or so - and it is not like where I live is not breath-taking. It was a lovely drive back, and it was good to get home.

Leif

Wednesday 10/2. Another day of "you have GOT to be kidding me?!!"

Wow, looked at my last post, and there is a lot to update. It has been a crazy week.

Last you all heard, I had barely made the study requirements, and we sent the blood to the independent testing lab and hoped that they would come up with the same numbers. By the time I needed to set off for Burlington, there still was no word on the lab test. So, with fingers crossed, off we went.

We had reached Farmington when I got the phone call. The study coordinator, Hannah, called to say that the liver enzyme number tested at 86! I am IN. Well within the study parameters. You can imagine the relief. It was such a good feeling to finally be out of this limbo and be getting treatment with a very promissing drug. On top of that, the drive out was just beautiful. Rt 2 winds through the mountains and the leaves were just about perfect. My good friend Liza was acting as my temporary caretaker for this trip, and we had a great time driving through the scenery, and looking forward to a pretty decent indian resteraunt in Burlington for dinner.

Not long after, Kate - my primary nurse coordinator from Dartmouth Hitchcock called to make sure that I knew the news, that Dr Sprague (the Doctor running the Ibrutinib study) had called Dr. Meehan (my primary hemo/oncologist) and that I was good to go.

At 4:30, about two hours out from Burlington, I got a phone-call from Dr. Sprague. He called to let me know that there was a problem. The study director - the Doctor that oversees the study for the company that makes Ibrutinib was concerned that I still had Graft vs Host Disease (GvHD). That by itself was not the problem, but the drug that one uses to treat GvHD, Prednisone, is restricted in the study. They allow a dose of 15mg, but I am on a dose of 140mg.

I pointed out that we knew this and had talked about it. On the day that I enter the study, I have to take 15 mg, and then the study allows for up to 10 days of a higher dose. The plan, I reminded Dr. Sprague, was that I get into the study, and then we return my dose to 70 mg. My liver enzyme numbers show a pretty clear trend that we will have successfully treated the GvHD with about another 4 days of treatment, and that I likely will not need any further Prednisone at all.

Statically though, it usually takes 6 weeks of treatment of Prednisone to cure GvHD. It is just a fluke that I respond dramatically. The study director was not buying the idea that I could possible get over the GvHD within the 10 days. And if I did not, I would be out of the study. Furthermore, I would have no possibility of being able to get BACK in the study if I had to be dropped. From his point of view, it would commit resources to a patient that clearly was going to be dropped, it would be bad for study, and it would be bad for me. There is a pretty short list of drugs that you should not be on if you are on Ibrutinib, and Prednisone is one of them.

I asked Dr. Sprague to show the Study director my numbers, talk with Dr. Meehan about how quickly I have responded to the drug before, and show that while on average it does take 60 days, in my personal case, that was highly unlikely. The most likely scenario is that by the time I started taking the drug on Thursday, my GvHD would probably already be in control (as that was 3 days from when I had my last blood draw). He agreed to call the Study director again and argue my case.

I called Dr. Meehan and gave him a heads up. I wanted to make sure that Dr. Sprague gained confidence that, in my specific case, the GvHD was going to be a non-problem.

At 5:45, Dr. Sprague called back. (We are still on the road to Burlington, about 45 minutes out at this point). He had talked to the Study director again, and the final answer was NO. The study director was NOT going to let me into the study at this point. I was going to be allowed into the study with another blood test only after I had been on 15mg of Prednisone or less for at least 5 days. That is to say, I had to prove that my GvHD was under control before I entered the study. I was upset, but in reality, this was not unreasonable. I claimed that it would be under control with 5 days, and they wanted me to prove that this was the case. This would set back my treatment by just a week - everyone would have confidence going forward, and the I would not be subject to whatever risk there is with the combination of Ibrutinib and Prednisone.

Still, I must say that I was pretty disappointed. The problem with this fairly reasonably request is that I have not had any treatment since my DLI, and my lymphoma is especially aggressive. I am having problems with plural effusion (fluid in my lung), and Dr. Meehan and I are not confident that I have an extra week before I have SOME sort of treatment. It is not like the Ibrutinib is magic - any treatment takes a while to kick in. We also want some wiggle room for a Plan B in the event that the Ibrutinib had no effect on me. I really should have started some sort of treatment 2 weeks ago, the first time that I was in Burlington. I talked to Dr Sprague for some time to see if there were any other options, to see if I could convince him to pressure the study director into moving forward anyway. I pointed out that this is not a Phase III trial, but an access trial. That is to say, a method of getting the drug to patients almost SPECIFICALLY in my situation. He felt that he had pushed his limit with the study director, and that nothing good would come out of trying to badger him into accepting me into the study.

So... after sitting by the side of the road for a bit, we decided to go ahead to Burlington. We had reservation at Hope Lodge (which is a wonderful place - I will get around to describing it at some point), and we were still looking forward to dinner at the indian restaurant.  Furthermore, even though my meeting for the Ibrutinib study was canceled, I wanted to see Hannah, and thank her for the work that she put in, talk about the possibiblity of getting into the study in a few months after I had some other treatment, and I also scheduled a thoracentesis (draining my lungs), which I desperately needed.

The indian food was pretty good, although not really worth a 6 hour drive in of itself. :)

Leif

Are you kidding me - redux.

As you know, I missed my labs yesterday by 2.5 pts. I went in this morning at 8:00am to be retested. The thought was that they would go down over the last twenty-four hours, and I would be good to get into the Ibrutinib study.

They did go down. They dropped from 110 to 10. Right in the dead center of normal. The nurses and doctor were elated, but I had a bad feeling about it. They had been dropping pretty steadily by about 10 points per day since the Monday before last. Did they REALLY suddenly drop by a full 100 pts overnight. I asked to be retested. 

They thought about it and went ahead. Turns out the sample had been contaminated. My new liver enzyme number for the day is 106. TECHNICALLY it is good enough to get into the study, which says that particular number needs to be 107.5 or less. But the sensitivity of the test is about +/- 2 pts. Also, it does not matter what the test at Waldo says, that is just a pre-test to make sure that I am within study parameters. The test that matters is the blood that is sent out to the independent testing lab. I have NO idea if they are also going to find that I am just 1.5 points below what I need, or whether, because of slightly different testing methods, they will find that I do not yet fit into the study. 

No clear resolution. Be that as it may, as far as planning, we are assuming that they will except it. So around noonish, I will be headed out to Burlington. One of the study coordinators from Fletcher-Allen will be hounding the independent lab all morning to try to get the information on whether I am in or not, and will let me know as soon as the results become available. Hopefully it will all work out, but there is still a chance that I will get a phone-call on the road letting me know that I might as well turn around.

Sigh. I am going to take an extra Lorazepam tonight to ease some of the anxiety. 96 would have been a great number. 106 - argh! I don't even know if it makes sense to continue on a couple more days and assume that my numbers will be correct by tomorrow or Friday. Or whether it is time to start something else. My doctors and I are all on the edge of our seats hoping that it works out.

Leif

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. 

You are feeling verrry sleeepy

Posted by Becky

What has Leif been up to, you ask? How is he doing? Leif has been sleeping. He gets up for an hour in the morning to eat and take medicine and deal with anything else that needs to be dealt with, then he goes and takes a nap. He does something similar at lunchtime, and tries to get some exercise. At suppertime he's usually up for 2-3 hours. His labs are looking okay; he just has a ton of fatigue. He makes an effort to exercise every day, generally by taking a walk, to build his fitness levels and combat the fatigue.

One of his doctors said that we can think of the DLI as a second transplant without the heavy-duty chemo and radiation first, and I think that he is experiencing something similar to the post-transplant fatigue he was feeling in February/March.

We have enjoyed visits from Leif's sister Fiona and friends Mike and Carol. I'm back at work and enjoying having something resembling a regular routine. Leif has been putting energy into keeping the information flowing so a crew can, at long last, finish our house. 

Out of the hospital.

I am out. Got released yesterday afternoon, which is a full week earlier than they had originally suggested. I have a meeting with Dr. Meehan on Thursday, so we are staying with my second cousins (my mom's cousins) who live in Vermont. They have a beautiful home on some beautiful land and they are two of the sweetest people you could hope to meet. Becky and I are enjoying sitting on the porch reading, looking out at the gardens. The day has been very nice. 

I am doing well enough that they did not bother to drain the rest of the fluid from my lung, but believe that it will be re-absorbed naturally. My breathing seems to be uninhibited, although it is not like I am doing windsprints or anything very challenging. The lymphoma is in remission, although it still shows up on the PET scan. The doctors and I believe that my donor cells are going to take care of it. The doctors believe that it will try to relapse eventually, but we can keep it suppressed with the chemo drug Velcade before it becomes an actual relapse. So my worst-case scenario has changed from "September" to "several years".  Basically, until my lymphoma become resistant to the Velcade. I still have high hopes that my donor cells will stay ahead of the lymphoma indefinitely. While I will never be "cured" because my lymphoma is refractory, if I never have another relapse, it is basically the same thing. As long as the donor white blood cells recognize and hunt down the lymphoma faster than the lymphoma reproduces, I will not have a relapse. I have some faith in my donor cells. So far they have responded well above typical. Go team!

The only problem I currently have is a pain in my abdomen directly below my spleen. Nothing showed up on the PET scan or on the X-ray that I had, so it is probably not anything that will lead to further complications, but it hurts quite a bit. I am on a good amount of pain killer, and it is all for this abominable pain. It has been going on for about a week. Originally we thought that it was a gas bubble, as some of the medications that I was on were causing me some problems in that area, and then the thought was that perhaps something was bruised. Although Becky just reminded me today that I had a very similar pain, in the same place when my spleen was shrinking. It is, in fact, shrinking again. So that might simply be the problem. There is probably still scar tissue in there from when it was so swollen, and is it shrinks it tugs on things and pulls in ways that are uncomfortable. It is one of the things that my doctor and I will discuss on Thursday.

If that meeting goes well, we will be back in Cundy's harbor that evening, and back in Brooks on Friday. We are planning to stay up there permanently, except for regularly scheduled trips back to Dartmouth-Hitchcock for check-ups. We are excited to finish our house, so that is the big push through the end of the summer and the fall. 

Leif

PET scan and T-cell infusion results.

Quite a bit has happened since my last post. I have been told that a number of my posts are overly technical, so I will do a quick summary, with details below for those who want them.

On Monday I got a scan that showed that my more lymphoma was killed by the chemotherapy than was expected. They also removed the tube that was draining fluid from my lung removed. Good day.

On Tuesday, I got the last dose of the light chemo. I also got the T-Cell infusion, which consists of adding 5 million of my donor's previously frozen white blood cells to my system to help fight the lymphoma and repair any damage to my bone marrow that the chemo may have caused. Both the chemo and the infusion made me feel pretty bad, and I had a mild reaction to the infusion. The reaction faded by the next day, but I have been pretty fatigued for the last few days. I am currently feeling pretty well, and doing better than the doctors expected. They may release me earlier than I was expecting. Perhaps even early next week.

Gory details:

So, the PET scan on Monday was freaking rough, not just because of the 3/4 of a liters of contrast that you have to drink before hand (and contrast is one of the worst beverages I have ever had), but because the tube into my lung, which they call a "chest tube" is pretty fricative, and it sort of got caught when the PET scanner moved me into the scanning drum. It tugged fairly hard and was excruciating. There are, of course, no technicians actually in the room when the scan is running because it is radioactive, but they heard me, turned off the machine and did take care of the problem by taping the hose directly to the table after giving me enough slack. I had repeatedly warned the technician that the hose was going to be a problem. I had intended to tuck the hose slightly under my butt to prevent exactly that sort of thing, but I got secured on the scanner board before I could do so. So I was unhappy about that. Having a tube that goes through your back, between your ribs and into your lungs tugged on feels just about as unpleasant as it sounds. It did not, however, pull out. Thank goodness. 

The result of the PET scan showed that the lymphoma responded to the light chemo better than was expected. We knew it WAS responding because my lymph nodes had stopped being swollen and had gone back to normal. This is really important, because my lymphoma has never responded to light chemo before, and the dire prognosis from June was based on that fact. If the lymphoma had not responded to this particular course of chemotherapy, there was in fact a good chance that I would not have made it past September. So, since it has responded better than expected, it is thought that we can keep it in remission by having me tested regularly, and before it actually gets to the point of relapse, hit it with a dose of Velcade and my donor T-cells and B-cells will be able to keep it at bay. 

The PET scan also showed that there was still some liquid in my lung, but it was in a different pocket than the tube, so the tube had to be repositioned. Later that day they sent me down to have that done. There were two posibilities. One was that they could fiddle around with the tube without taking it out and get the end into the other pocket. The other possibility was that they would have to take the tube out and put it back in, in a different position in order to get into that pocket. Since I am not longer symptomatic (my breathing has been deep and pretty clear), If it was the second case, I advocate fiercely for taking the tube out completely for a while, and they could put a second tube in again in a few days. Give me a break from having a tube in my back. It did turn out to be the second case, so I advocated with the nurses, who had sympathy, but voiced the opinion that it was unlikely to happen. I advocated with the doctor, who listened, paused for a while and then went to consult with her colleagues. She came back and said that they all thought that was a wonderful idea. Yay!!! So she pulled the tube (I did not get any painkillers for this, and in fact it did not hurt beyond a sharp pinch, but it was one of the weirdest, most disturbing sensations I have ever felt. The tube went more than half-way inside me, and when it came out, it felt like a rib, or part of my lung, or SOMETHING important inside me came out with it, but it was just the sensation of my body having gotten used to the tube there being surprised when it suddenly was gone.) I felt immediate relief. The tube had been hurting me more than I had realized, and in less than an hour I was feeling my usual joy at being alive. While the tube was in me, I was feeling pretty grim. It was hard to truly be upbeat. 

There was a little incident about an hour later, when the dressing over the hole suddenly was soaked with some last bit of lung fluid and blood, and it sort of ... spurted out. It did not hurt, and it probably is a measure of the roughness of this last year that I found it funny, hitting the nurse call button, trying to keep pressure on the dressing, and moving quickly away from my laptop, so not to get this nasty fluid on it. It was easily cleaned up and the dressing changed. I kept some pressure on it for about an hour and it has been fine since. 

The next day, the last of the Velcade. This drug attacks fast-growing cells, so it tends to make me nauseated, and it did. I rested until they came in to do the DLI, which is the same as the T-cell infusion. Unlike last time, the cells were frozen. The cryogenic preservative is pretty nasty and also lead to me being a little nauseated. They did not have to give me a new line, but were able to put it through my port. Or so they thought. Then at the last minute, with the nurse standing there, and the time ticking away (because after the cells are thawed they have a limited time to be put in the body - about 10 minutes), they realized that they were not POSITIVE that the cells could go through a port. A call was put into the doctor. No response. They had another nurse poring through the available information. Typically the cells are put in through a central line which is bigger than a port. The cells are fairly large and somewhat sticky, so putting them through a line that is too small might kill the cells or clog the line, or both. The other nurse came in and said that the doctor had not yet returned the call, but all the information she could find IMPLIED that a port line would work just fine. The cell technician and the attending nurse took a deep breath and went for it. Just after she had put the last ones in (it is a 5 minute push) the doctor called back and OK'd the procedure. Whew!

I had a light outbreak of Graft vs Host disease, which was not really bad, since it meant that the T-cells had made it in alive and were doing their job. The outbreak consisted of a rash around my neck, chest and upper back. It does not itch yet, but it will later, when the skin begins to peel. At least it doesn't hurt.

So the last few days I have been napping quite a bit. I have taken some walks. A loop around the hospital, on the inside works out to be about 1/2 mile according to my pedometer. I try to walk a half mile a day, and sometimes I am up for two loops. I have been having a little gastric distress, but nothing major. Otherwise, things are looking very well. 

Leif

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