Back to the hospital

Will was admitted to the hospital on Monday and is very sick. We actually went up on Sunday to the ER, but the ER doc (apparently) mis-read what was going on and sent us home. She suspected lymphoma recurrence (so did we) and told us to follow up with the pulmonary/transplant doctors and the oncologists to schedule lymph node biopsies. I was working on that Monday when Will (who was getting worse) went up for a regularly scheduled appointment with his ID doc and was dx with some form of superior vena cava syndrome, basically a series of big blood clots in the big veins in the right side of his neck, and admitted right away. (I think it's not actually, technically, in just the right spot to be SVCS, they have told me at least twice exactly where the clots are but I am not capable of remembering the names of all these vessels and all my brain can do when they start in on that part of it is think -- How do they remember all this crap? And, who cares anyway? Big, big vein in the neck near to/leading towards the heart, that's all I need to know.)

Things have gone downhill since. Will is miserable, in a lot of pain, all swollen up on the entire right side of his head, face, neck, shoulder, arm, hand, chest, etc.. (I don't think it helped that the ER gave him 2 liters of IV fluid in 2 hours on Sunday. . ..) Can't talk really, can't eat well. Can't cough, which means his lungs are filling up with crap.

Yesterday they started making noises about putting him on a ventilator, because of concern that the swelling would start to obstruct his airway. This is still a possibility but hasn't, thank God, been needed yet.

It goes on and on, the normal hospital stuff. A million doctors involved, and since it's a teaching hospital a million and a half student doctors in various stages of training. The current debate: should the portacath come out? Can he tolerate the surgery now if it needs to come out? Is it really the cause of any of this or just a bystander? (The docs have different theories on this.) If the port has to come out, Will basically will have no good access options left. The veins in his arms already are shot from picc line after picc line; he had a port on the other side pre-transplant, so they don't think they can move it there. It's a dilemma. The medical docs in charge of him and the interventional radiologist think we should treat through for a little while and least in hopes the port can stay. They don't think the port has a raging infection in it or that it is the cause of all this particularly. ENT didn't have an opinion yesterday, although they are eager to vent and a little alarmist about the airway. Pulmonary/transplant wants the port out. I don't know what ID thinks yet. And a partridge in a pear tree.

He is being treated for a blood infection. The port may be infected, or the clots(s) may be infected on their own, or the blood cultures might have been contaminated. (Only one of 6 has grown anything so far and it is growing a common skin form of staph; but blood infections are, I've been told in the past, notoriously hard to catch, which is way they keep drawing cultures, so even 1 growing is important, I guess.) He isn't spiking big fevers so that's good and means the antibiotics are working to keep that under control so far. They've pretty much ruled out lymphoma for now, which is nice. What a rabbit trail that was. The lymph nodes are not swollen and he has none of the other markers that were present for his past two lymphomas (EBV spike, fevers, weight loss.) The ER doc apparently just got it totally wrong when she did her specific examination to determine if if was lymph nodes or some other kind of swelling and decided lymph nodes. Bummer.

Will has not yet really started to respond to the treatment they're giving (heparin drip to work on the clots, increased steroids to combat the inflammation, sitting up to encourage proximal drainage). Our job is to hope the treatment starts working to clear the clots, and the work-around veins that are surely growing get bigger faster, and that Will can hold it together long enough to be able get well enough to cough and start to clear his lungs. And that the lungs hang in there too. Honestly, it's a pretty awful situation and it really doesn't convey any sense of it to say that I feel so bad for Will who doesn't deserve this (as if that's a consideration in this universe) and who is tired, frightened and in a lot of pain.

Kidney (dis)Function

Will had his regular oncology re-check today. The good news is that the oncologist doesn't see any indication of a relapse; Will is still cancer free. The not as good news is that Will's kidney function is quite a bit worse than it was when he was in the hospital. (3.1 serum creatinine compared to 2.3 or so in the hospital last week, compared to 1.8 or so, which is already high, usually. The normal range stops at 1.2 or 1.4 depending on who you ask.) So the doctors decided to discontinue the valganciclovir and the voriconazole today, for fear that they were damaging the kidneys more than they might be helping the lungs.

That's about all we know. I wasn't able to go with Will today because I had to work. A long day in Wenatchee and then a long drive home to more work and this news. He will be rechecked (a blood test) on Monday for kidney function and have his lung follow up the following (I think) Monday, and I guess then we shall have what may be an important discussion with the doctors about the risks/benefits of treating the lung stuff vs. protecting the kidneys and how all this may play out. Not unexpected, but also not happy news by a long shot. Particularly as winter cold and flu season looms, we would very much like all medications that he might need to be available to Will in case he needs them.

It's working! And, I'm working.

Will reports that he feels "significantly" better. Will is the understated type, especially when it comes to good news, so this equates roughly to shouting from the rooftops, I think. He was up before 8AM this morning and done with his breathing treatments and out working on the patio by 10:00. Tomorrow he and his father will take Liam to the zoo. (I hope they take pictures, and if they do I will post some.) This is wonderful and we hope very much that it lasts, well, forever.

My work travel is what it is. Today Hanford, tomorrow Hanford then Wenatchee, Thursday and Friday Wenatchee, and then Friday night home. Many miles to smallish towns in not so many days: it's what I imagine working in sales is like, or something. I am tired tonight and unhappy because we lost an important bid today, and even thought we lost it (I think) based in part on the competition having recently hired a former political appointee and all that blah, blah, there are always excuses, still, damn, I need to win these things.

I leave you with a photo of my crappy hotel room. I think this is exactly the same crappy hotel room I had when I was here 2 weeks or so ago. Same desk light that doesn't work and same phone message light that is broken (flicker, flicker, but no message). While I'm sure it's has some nice features, and the work has merit, on balance, I really don't like Richland; it's the city my father died in and I hate coming here. Tomorrow -- on to Wenatchee with no more whining, I promise.

Good News to Report

Finally, some good news around here. Will had his big check-up today to confirm that his PTLD is still in remission and, it still is. He remains cancer free -- which is, obviously, great. After today's visit he has no more scheduled check-up CTs. It's been long enough that he doesn't need to be monitored that way any more, so, for the cancer part of life from now on, he'll just have a regular doctor check up with his oncologist every 6 months unless something else seems needed. Hooray! Two fewer doctor appointments a year! And, as a free bonus bit of good news, today's CT showed that his lungs look better, so the new regime of high-dose Cipro and inhaled Colistin seems to be working on whatever infections process had taken hold of his lungs this time.

(Will did not have to go into the hospital on Monday; his regular pulmonary doctor seemed, confused is the nicest way to describe it, I guess, to learn that Will had been discharged with no follow up treatment when he was in 2 weeks or so ago. The set of problems he is dealing with now is almost certainly a pseudomonas flair up infection/inflammation in the small airways; the pseudomonas is always around and it jumped on the opportunity/weakness caused by the cold virus Will had three weeks or so ago. This makes me realize that maybe I should be a little more assertive with Will and his docs sometimes, since I know from past conversations with the pulmonary doctor that this type of thing is likely to happen and was pretty shocked when Will described no follow up care from his last hospitalization because: "it's viral and can't really be treated." It's never only, just viral. The bacteria are always looking for their chance. But, Will HATES the Colistin, it is awful to take, so he can sometimes be not overly motivated to question these decisions.)

For more information on PTLD, a transplant-related form of lymphoma, see here and here. It's most studied in bone marrow transplants, but also can occur in solid organ transplants (like lungs). Will is unusual in that he had two types of PTLDs, as described here. It never rains but it pours, I guess.

I'm really happy about the CT thing. Will has had so many CTs, honestly, I don't think it's exaggerating to say that in the past three years he's had in the neighborhood of 35 or 40. That's a lot a radiation, so I'm all for CTs only when really, really necessary from now on. Putting the scheduled CT behind us frees up more of my brain to worry about the pig flu. Which is good, since it requires a lot of worry. Immune suppression to maintain lung tx + pig flu = very bad news. I have washed my hands so many times in the past week it's amazing I have any skin left. The real thing will be deciding if/when to pull Liam out of his 2 day a week "school." We'll just have to play it by ear, I guess.

Liam has taken to picking all the flowers in the yard, but, so far, only the yellow ones. This is potentially useful for the dandelions; less good for the marigolds. For some reason, he likes to put the picked flowers into Mollie's outside water dish. (He knows that flowers go in water, I guess that's it.) Photos are: waiting inside the gate for Daddy and Grandpa DH to walk up, showing Mommy the flowers, and flowers in Mollie's dish. Mollie doesn't seem to mind.

Good news lymphoma re-check

Will had his quarterly re-check for lymphoma yesterday. The word is. . .he looks good. No evidence of recurrence and the blood work looked good, even his chronic anemia was some better and his kidney function was better than it has been in months. (Will suspects this is related to him being on a lower tacrolimis does, something that likely will chance this week; but, still. . .it's good to know the kidneys can bounce back when given the chance!)

So, now Will will be down to 1 chest CT a year (he'll have it in March), and semi-annual re-checks instead of quarterly. He's 3 years in remission from the transplant-related Hodgkin's lymphoma in June and 5 or 6 years remission from the regular PTLD in, I think, this fall.

The docs have now decided what we suspected all along: Will's lymphomas occurred simultaneously. The regular PTLD is so much more fast moving and easier to diagnose, it was noticed and treated first. The transplant-related Hodgkin's kept cruising along (it doesn't respond to Ritixumab, apparently) and was finally diagnosed and treated 2 years after the regular PTLD. They figure this because of the odds of the whole thing. Apparently the odds that someone would get regular PTLD and then, 2 years later, a separate occurrence of Hodgkin's lymphoma are something like 1 in a billion or so, whereas the odds that someone would get regular PTLD and that would mutate into transplant-related Hodgkin's are more like 1 in 100,000.

Will continues to recover from the pneumonia. It's a long road, but he has been really, really diligent this week about getting out and doing something every day -- walking the dog, visiting Liam at school, whatever. This is incredibly helpful to him. We hope he'll be ready to be back to rowing when the days get longer this spring.

I'm tired

I'm tired. Liam wouldn't sleep until almost 9:15 tonight; this after exactly the same routine that worked so well yesterday except that today for some reason when I took him upstairs at about 7:20 he just would not sleep. So, after almost 2 hours of rocking, squirming, fidgeting and some yelling and crying (not conducive to sleep at all) he is finally asleep and I'm tired. In the meantime, the chicken stock I was making boiled down to almost nothing and the poor dog was so ready to come inside after her 2 plus hours outside in the rain that she about broke down the door when she saw me coming downstairs. (She does have a dry dog house and a dry covered porch.) I intended to make macaroni and cheese tonight because we have a whole half gallon of milk that must be used today because Will hasn't been in the house to drink it, but I don't think I have that in me right now. No fear, because another gallon and a half will be delivered tomorrow that he also won't be in the house to drink because I forgot to email the milk delivery guy from work today.

This hospital stay has seemed particularly hard for some reason. Maybe it's because Will turned out to be so seriously ill. Maybe it's the holidays or that he was so sick at home for weeks before he finally went to the hospital. Maybe it's because Will sounds so down and subdued whenever anyone talks with him (that's when he answers the phone to talk at all; mostly I talk with the nurses). Or that we're all sick with the same or different crud to some degree. (My cough has moved into my chest and makes me sound like a smoker.) Everyone is stressed out from the driving back and forth to Seattle, or the feeling guilty (at least I do) when they can't be there, and just from worry both about Will's recovery, and about how he'll cope once he comes home if he doesn't recover as quickly or fully as we all hope he will, and about how we'll keep Liam's new school/daycare related germs away from him so he won't get sick again. Whine, whine, whine.

The actual clinical information as I understand it from just talking with Will's nurse (Tom, pretty much my favorite this stay) is: after a rocky start Will had a pretty good day today. Tom reports that Will ate a good dinner and that a picc line was successfully installed, so that should be a more comfortable way to deliver the IV antibiotics and it means Will can bring his antibiotics home with him when he's ready. Doug was there earlier today and Will ate breakfast and read part of the Sunday NYT. Will fell again last night, and had a consequent CT scan of his head this AM. Nothing is wrong with his head that shows up on CT apparently. Ha Ha. The falls are frightening; Will has had these falling episodes before and there is no clear explanation. Last night's fall did result in a physical therapy referral, which means that Will had to walk the halls today with PT and will have to every day he stays in the hospital, sometimes 2x a day. Poor Will. All this being sick and having a hundred people tell you what to do all the time must be so frightening and unpleasant. I know that all he wants is to be himself again. . .but it has been so hard finding and adjusting to his new limits after the cancer, rejection, spleen removal, pneumonia, cancer, pneumonia, aspergilious, etc., saga of the past few years. And now this set back. When I talked with him briefly around 5:00 tonight he sounded down but reported that he thought he felt better and that his oxygen was "better."

Aside from the not sleeping, Liam had a pretty good day today, and a great evening. He was interested in dinner and ate well, played well and even played some with Mollie in a way that Mollie could relate to, and he loves his new story/snuggle time.

Silly Face & Visit to the Oncologist

Liam and Will got into a silly face making contest while Liam's dinner was winding down tonight (this is the yogurt, or dessert phase of the meal). I'll spare you photos of Will's silly faces. Liam trys his hardest to get them just right, but he still has a ways to go. The word from the oncologist today was: nothing unexpected, still looks like full remission from both the regular and the Hodgkin's PTLD (although, they can't test really well for the Hodgkin's with out a bone marrow biopsy, but there's nothing to indicate it's back, so no more invasive testing is indicated for now), blood work still looks good, come back in 4 or 5 months. Let's hope Will gets a rest from worry for at least a few days.

To the Beach & Virology

We took Liam and Mollie to the county park today and after about a 10 minute walk through some nice woods you hit a big Puget Sound beach. The tide was very low. Mollie enjoyed racing around and swimming, and Liam enjoyed watching her and practicing with the ubiquitous "chucker." We are challenged by the fact that he so likes to throw things, and has been so encouraged to throw tennis balls for Mollie, that he throws everything, all the time. Sippy cup onto the floor, heavy toys at Mommy and Daddy or whoever is nearby, anything he can reach at Mollie, and last week in the pediatrician's office the toy trains at the other children. Great---I love being "that mother," the one with the out of control kid. We're trying to figure out a way to help him differentiate, but haven't come up with anything too brilliant yet. Luckily there are plenty of opportunities to throw stuff "legally" at the beach, so it was a big hit. Sorry the photos are crummy; I need to work on my photography.

Will's virology came back and his EBV count was negative and the CMV count was 400 (very low for him), so that's good news. (Both Will's cancers, like most (or all? I can't remember) PTLDs were EBV related.) He has his every 3 or 4 months follow-up cancer screening (to confirm he's still in remission) on Monday, so please send your good intentions his way. I'm feeling pretty optimistic about it since he's been able to maintain or even gain weight, fevers when they happen are mostly low, and the virology numbers are good; but Will usually gets sort of worked up and worried. (And, who can blame him -- I'll have to post sometime about the whole living with chronic illness, cancer survivor syndrome thing where every headache tends to be evaluated as a possible brain tumor and every fever might be lymphoma. . ..)

Blood Work

First, I promise to post more pictures of Liam soon. I know no one is really interested in this other stuff. But, I just got home from a week of work travel late, late last night; and finally picked Liam up tonight at dinner time after working all day today, and the battery in my camera was dead when I went to take pictures tonight. So, tomorrow.

In the meantime, we have some information that probably explains (in part at least) why Will has been feeling so crummy lately. He had his normal transplant-checking blood work earlier this week. This is a routine blood draw to check the levels of immune suppression medications in his system at the trough (that is, when the medications should be at their lowest point) and to check other odds and ends such as kidney function and overall blood health. Turns out that Will's overall blood health is not too good right now. His neutrophils (white blood cells) are about half what they should be and his hematocrit (red blood cells) also is quite low. No idea why. (Infections as you all remember from 11th grade health class generally cause more white blood cells, not fewer.)

During his chemotherapy for Hodgkin's lymphoma in 2006 Will really suffered from blood reducing side effects. Even being treated with special medications to promote production of white blood cells, he had neutropenia requiring hospitalization after virtually every treatment. Even with treatment with more special medications to promote production of red blood cells, his hematocrit took a dive each month. It got to be that we were regulars at the hospital. I could virtually plan my work commitments around knowing that he would be in the hospital for about 5 days starting about 2 weeks after each chemotherapy treatment. (Not to make light of it -- these are very serious things and cancer patients die of infections contracted while they are neutropenic. The one month we didn't go to the hospital resulted in the toughest talk I've ever received from any health-care professional ever, from Will's cancer doc. It went like this: "People die, no fooling. You have to go to the hospital. He has to have strong antibiotics and anti-fungals, he has to be monitored, this would be a stupid thing to die from.")

Ever since then, Will's had odd incursions of bad blood work for no apparent reason. So, time to watch it again and consult with the doctors next week. Hopefully someone will come up with some (relatively benign) explanation for why this is happening. Could be the transplant drugs (although he has been on higher doses before without this happening); could be the transplant drugs interacting in a weird way with something he ate, something in the air, something, whatever. Could be a side effect of treatment with Rituximab, which has been observed to result in delayed neutropenia in some people, from his first PTLD (see, e.g., which I picked because you can get the full text for free and it has many references if you want to do more research).

Could be, anything. Likely we'll never know, it just will be something else to monitor more closely for a while and worry about a little more. And, we'll be extra careful about infection control, since fewer than normal white blood cells means even less ability to fight infection. And, feed Will all the dark leafy greens and red meat he'll eat to promote red blood cell production. And, stay tuned for the results of the consultations with the docs on Monday.

The photo is again from Wikipedia, a Human blood smear. A is some erythocytes; B is a neutrophil; C is an esinophil; and D is a lyomphcyte. Makes perfect sense now -- doesn't it!

Uncertainty and gratitude

The Confessions of a CF Husband family has received the bad news that their PTLD is not responding to rituximab and will require traditional chemotherapy. I know from watching Will's experience with chemotherapy that this is likely to be a very difficult time for them. Traditional chemo is hard for anyone, I think, and particularly crummy if you health is already fragile and your immune system suppressed. I think they'd appreciate your thoughts and prayers in the days to come.

It reiterates for me how quickly one can go from feeling like things are on an even keel to complete chaos. We all know intellectually that any one of us could face the end at any moment, but if you're a person with health conditions that can turn very serious very quickly you experience the reality that your life is temporary most every day. You don't make assumptions about the future like most people do. We don't particularly focus on this in our family, but we don't ignore it either -- it's something to be respectful of. We know that even without any more cancer ever, Will's lung transplant won't last forever. Sometimes it makes us really, really sad to know this, and sometimes we can deal with it in a more matter-of-fact way, and occasionally, especially when Will is feeling well, we don't think about it for a few days or weeks at at time. We hope Will's transplant lasts a long, long time, and it may; it also challenges us to be mindfully grateful every day.

Confessions of a CF Husband

I started this (new, improved) blog after coming across and then becoming addicted to reading Confessions of a CF Husband. This is the story of a couple from North Carolina who are going through an amazing journey with stops along the way for: a very premature baby (who is now doing beautifully), a double-lung transplant, and now PTLD. When they posted about their PTLD, I realized how much families who are traveling these roads have in common and it made me want to put some of our experiences out there in case they can help anyone, in any way. So often it can make a difference just knowing that someone else has been there.

So, check it out: http://cfhusband.blogspot.com/

Pseudomonas is No Fun

Pseudomonas aeruginosa according to the Pseudomonas Genome Database V2 is "a versatile Gram-negative bacterium that grows in soil, marshes, and coastal marine habitats, as well as on plant and animal tissues." In other words, it's everywhere. For most of us, it's not a problem. But for people with cystic fibrosis, or cancer, or who have a compromised immune system, it can be a big, ugly problem. Will's on and off pseudomonas infection pre-dates his transplant. For a while after transplant it seemed gone for good, but when Will got PTLD and then Hodgkin's lymphoma, and the various types of pneumonia that went along with being sick, his pseudomonas came back and seems like it might be here to stay for a while. Will's pulmonary doc says: people after transplant get what they had before transplant. I guess that has pretty much been true for Will. It has been making his mornings, especially, tough lately.

It may be that as Will gets farther out from the various lymphomas and pneumonias and his health stabilizes more and (we hope!) improves more, he'll be able to clear the pseudomonas as he was able to pre transplant. But, it will be harder this time because of the immune suppression drugs he needs to take to prevent rejection. In the meantime, he tries to keep it under control with daily airway clearance and periodic IV and inhaled antibiotics.

It appears that the pseudomonas makes a home in Will's sinuses and goes from there into his lungs. Sinus surgery has been discussed but the results seem uncertain, especially for someone with CF. If any of you CFers out there have experience with sinus surgery one way or the other, please let me know. Thanks.

PS - I took the pseudomonas photo from the wikipedia site, which says it is P. aeruginosa colonies on an agar plate.

Maintenance

It takes a lot of daily care and attention to maintain a lung transplant. Of course there is the daily medication to prevent rejection, I'll post about that some time in the future. And the daily efforts to keep clean and prevent infection, so important when your immune system is suppressed. And, now, for Will, daily airway clearance.

Transplanted lungs are vulnerable, so if you get sick a few times as Will did (first with post-transplant lymphoproliferative disorder (a kind of cancer) then with chronic rejection, then pneumonia, then Hodgkin's lymphoma, then pneumonia again) you end up with lung damage. Among other things, the lung damage means that the lungs get infected more easily and the infections are more difficult to fully get rid of.

"Airway clearance" as far as I understand it means loosening up the gunk in ones lungs and aggressively coughing it up to get rid of it. Will doesn't like adding daily airway clearance to his routine. It takes time -- about 90 minutes or so each day -- and it makes his chest hurt. Mostly, though, I think the hard part is that it was one of the CF related obligations he had hoped to leave behind forever after transplant. But he does do it, most days, because it makes him feel better and hopefully will make hospitalizations and use of IV antibiotics less frequent. Will recently started using nebulized hyper tonic saline (a 3% solution) to help with airway clearance and it does seem to help him. It seems like inhaling the saline mist makes the gunk in his lungs more susceptible to moving around and being coughed up.

For those who haven't seen it before, the maintenance gear is, from left to right: basket of nebulizer mouth-pieces ready to be taken apart and sterilized; the compressor, which blasts air into the saline to turn it into a mist (propped up for photo purposes on an albuterol inhaler which you can barely see); box of colistimine ready to be mixed up, sometimes Will takes antibiotics by inhalation; two little ampules of the saline solution, ready to go; and the home PFT tester used to measure whether lung function is staying the same, getting better, or getting worse. (More on that some other time.)

We're grateful beyond words that these treatments exist, and that Will persists in them with (mostly!) good humor and grace.