Where to start?

After being diagnosed with the Cryptococcus on Friday, Will seemed to start to get better and then started getting worse again. Yesterday and today he has been really, really sleepy and his oxygen requirement is going up (we would expect it to go down with treatment of the infections). He's now on 5L by nasal cannula, which keeps him saturated in the mid 90s. That's about the best they can do with nasal cannula, if his oxygen requirement goes up any more it will be back to the mask or the high-flow machine.

None of the doctors really seem to have any idea why this is happening. In fact, they've been sort of arguing that Will's O2 requirement is stable or even, better, than it has been. This is so strange to me that they would look at one reading only and not what is a clear trend, but, whatever. Will was back into the mid/low 80s this morning and that seemed to get their attention. He's had sweating and some nausea yesterday and again today, which may be the fluconazole. They have (re)started another antibotic because they are concerned they don't have full coverage of both bacteria with the one Will has been on. And they are watching his white-blood cell count carefully. They think they might have "overdone" the GCSF a little, since Will's white blood cell count was in the 60,000 again today. (Normal range stops at 10,500, if I understand all this correctly.) Very high white blood cell count can make you feel like crap whether you're sick or not.

In the good news department: the spinal fluid tests were all negative which means there is no CNS involvement for the Cryptococcus infection. That means the treatment is significantly more easy to tolerate too. They thought Will lungs "sounded" clearer this morning. The nasal swab for both Rhino and Adenovirus was negative, so Will cleared them and doesn't have persistent viremia.

Liam is a Grandpa DH's and GG's until they leave on a trip tomorrow. My parents' have the dog and about 100 errands that need to be done at the house (let the cleaner in, take the trash out, etc.). We appreciate all this help beyond words.

And another thing

This hospital stay is the fifth, I think, for Will since July. It might be the sixth. First there was a bacterial infection, then (they think) CMV pneumonia, then very low sats and fevers, then the line infection (which they now sort of think might have been sample contamination) and high-dose steroids for the chronic rejection episode, and now this. So fifth. The recent idea is that there must be something else going on in addition to all the recent infections that is causing Will to never quite recover and, in fact, keep getting sick.

Yesterday they think they found the culprit: Cryptococcus. Cryptococcus is an airborne fungus. There is a particular kind that is common here in the NW. (I don’t know which kind Will has yet; the docs didn’t say and I didn’t know to ask when they first told me, but it would be reasonable to think it might be the NW kind: C. gattii) It can take different infectious courses. It commonly causes lung problems (check) and it can infect the central nervous system too. It's treatable; however, if there is CNS involvement, the treatment has some pretty serious risks to the kidneys. Treatment for the regular versions is flucytosine or fluconazole or something like it. Treatment for the CNS version is 5-7 days of amphotericin followed by flucytosine.

So, they're doing a number of examinations to see if they can tell which parts of Will are infected. Head CT (normal), eye examination (normal), and lumbar punch to collect spinal fluid to test (fluid appears normal, but it's the lab tests that will really show what is what). The lumbar punch thing is an example of, really, how tough Will is. I am about in tears if I have to have an IV put in. Will just gets through it, whatever it is. I remember when he was being treated for Hodgkin's lymphoma and the oncologist said about one of his (many) bone marrow biopsies: "you could give a class in how to tolerate these."

At any rate, the lab won't run the tests on the spinal fluid until Monday, so we won't know until then. In the meantime, they might just start the flucytosine since it often is adjunctive therapy to amphotericin. (I asked the supervising medical doc about this when he called today, and he wanted to check with ID to make sure it was a good idea.)

The blood test for EBV was negative. This is very good, because in the past EBV has always be associated with lymphoma for Will. The blood test for Adenovirus also was negative (again, good). Will still has Adenovirus in his lungs, but because they didn’t find it in his blood, it makes them feel the infection is (1) less serious and/or (2) starting to be on the way out. Nonetheless, they may decide they need to treat for that too, depending. It can be a very serious infection in someone with a suppressed immune system. The IgG treatment is on hold for a day or so until they figure out what type of cyptococcus infection they are treating and whether Will will need amphotericin. The IgG puts some strain on the kidneys and they want to have all the room they can get to treat with amphotericin if they need to.

Will also had a complete ultrasound mapping of the veins in his arms and chest this afternoon, owing to the fact that the picc line nurse couldn’t get the picc line placed yesterday. They got one placed today. He said the mapping was "interesting."

So, that's today's update. Will is stable, no fevers today/yesterday. Still on 3-4L of oxygen by nasal cannula, but they are hoping to start weaning down, if that is possible.

Adenovirus and hospital stay - updated

Will was admitted on Tuesday from the CF/transplant clinic. His oxygen saturation had gone down hill. Chest CT showed a new infiltrate in the upper left lung. It was a rough two days. Wednesday Will had a bronchoscopy and after he started running a fever (which is a typical side effect), but Will's fever just kept going up, complete with chills and shaking. I was in Texas for work Monday through Wednesday and came straight to the hospital from the airport Wednesday night, just in time to see Will's fever hit 109 degrees and his saturation crash. They couldn't get him out of the low 80s on 100% high-flow oxygen and his heart rate was 144. There was a lot of activity, lots of doctors and the stat nurse came running. Get the re-breather, no, now the high-flow. Ice packs. Rapid stripping and running the fan. Arterial draw for blood gas testing. Etc.

In the moment it's pretty easy to stay calm and just try to comfort Will, because that's what is needed, but on reflection, it was pretty scary. Luckily, his fever broke and they started giving him lots and lots of IV fluids which helped a lot. By midnight, he was down to 40% on the oxygen high-flow machine and his fever was normal. He had another (much lower) fever this morning and has been fever free since then. He's off the high-flow and back on oxygen by nasal cannula, and down to 4L, which is keeping him saturated in the mid-90s.

Today we learned a little about what might be causing a lot of this: Adenovirus. Like most viruses that Will gets, this is an "oh, never mind" type thing is most of the population, but can be very serious in people with suppressed immune systems. He also has Rhinovirus and his typical bacterial infections (psudamonous and strep). He's being treated with antibiotics for the bacteria and anti-fungals, just in case. There isn't any great treatment for the virus. He'll have more testing to see if it's an active infection and, if so, how serious it is. If the infection seams to be still active and still serious, they can investigate some treatments, and can give him IgG if his antibodies are low. Otherwise, they watch it, treat the bacteria, and hope it clears itself.

They've been worried about Will's blood pressure today, it is low, which can be an indicator of worsening infection. But, it seems to be going up with more fluids, so that's good. There also has been a lot of talk about a lymphoma recurrence -- which certainly is something serious to consider. But, as of today, the oncology doctors think it should be on the back burner: they don't have any evidence of recurrence and Will doesn't have the same symptoms (exactly) as he has with his past lymphomas. Last night and this morning there was a lot of noise and speculation about a move to the ICU, but that seems to have abated too for now and Will is, by all accounts, feeling better: lower oxygen requirement, blood pressure back in the normal range, and no fever.

Will's regular pulmonary doctor is attending in the ICU this month and stopped by to say hello, which was really nice.

Second hospital sleepover in a row tonight, and 4th night away from home and Liam. If it's a quiet night and they've got a clear treatment plan tomorrow, I'll go home in the early afternoon. Hoping that's the case. My sister is visiting and I'd really like to see her before she has to go home again on Sunday!

PS -- I forgot to write earlier. Will has been great through all of this. Steady. Not freaking out. Nice to the nurses and doctors. Cooperative. Eating. Today he even did the flutter value thing to promote coughing and coughed up a bunch of junk from his lungs.

The Mix

Today brought:

• A very sunny morning, up early and outside on the porch for coffee (mama) and yogurt with honey (Liam). Yogurt with honey is Liam's new favorite thing. We've been working on how to ask for things, and for what he really wants he is very reliable: "mor mm mm hon'ee, peese, mama" was today's expression. And, after dinner when he was having yogurt and honey for dessert it was "peese, da'dee out-si, mm mm hon'ee out-si, peese!" ("Mm mm" is his word for yogurt on account of the kind we buy has a cow on the outside of the container and cows, of course, say "mm mm;" he likes to eat outside.)


• Pee pee and the other thing in the potty at different times today for Liam. Pee pee in the morning was preceded by stripping off all his clothes on the front porch and then running inside and saying "mama bye bye, mama bye bye, etc." and then when I tried to follow him "nooo hi, nooo hi mama." I guess he wanted his privacy.


• Fever and feeling crappy for Will which took up most of the day, but by tonight he was feeling okay enough to eat dinner with us, to accommodate Liam by going outside for dessert (even though he really didn't want to), and is right now upstairs reading stories. Will thinks and I agree that despite this congestion and whatever is going on to cause it and the fever, his breathing is some better. This is wondrous because it means that, hopefully, the chronic rejection has been stopped for now and it's a matter of optimizing the lung function he has left and trying to stay healthy. We hope his doc appointment on Tuesday can bring some hopeful news. In the meantime, we'll get the results of the oximetry testing on Monday and (likely) home oxygen which, even if he uses it only at night, could be really helpful as much as no one wants it.


• Picking up much of the garden and cleaning Mollie's dog house so it's snug and warm ready for those winter days when she just refuses to come inside until after dark.


• Apple crisp warm from the oven.


• Packing for me; a work trip that cannot be avoided starts tomorrow.

Every day is a mix. That's how it goes.

A day

After a good day on Wednesday, Will's had a tough few days. Very tired, not saturating well, resting and sleeping a lot, feeling like crap. Seems a little more congested, at least to me.

Today I drove out to the next town over (it's not far) and picked up the machine for oximetry testing. It takes a continuous record of Will's oxygen saturation to see if he qualifies for home oxygen, at least for over night. Will does not want this; does not want any of it.

Tuesday he'll see his regular pulmonologist, to see if the bronchiolitis obliterans syndrome has stabilized, and what the options are from here.

Old ways

Story time #1 with Daddy tonight. Did you have these books when you were little? I have such vivid memories of the Richard Scary books. This is the one about trucks, etc.. Things that go. Will remembers them too and is actually the one who tracked down these books when Liam was younger. I didn't realize it, but Liam has learned the names of most of the characters. Mistress Mouse, the tow-truck driver. The Pig Family. Officer Flossie. Gold Bug. And, Liam's favorite, Dingo Dog. We looked at this book last night too and this morning Liam told me that Teddy Bear's new name is Dingo Dog. (Knit Kitty is still called Dory.)

Nothing has changed. Will had a somewhat better day today. I think he's trying to be more active. Still short-of-breath most of the time, but every day that goes past without further obvious decline opens the mind a little more to the possibility that his lungs may reach a new plateau, lower than before, but not totally out of luck. And maybe we can stay there for a while, and make a new normal. Maybe. I know it is my job now more than ever to just experience each day and make the best of it. Let go of the past, and release myself from the tyranny of expectations for the future. It's not a strong suit of mine; but I get to practice.

I downloaded a bunch of information on bronchiolitis obliterans syndrome today but haven't yet organized it or read it. And I got a very sweet email message from a reader offering to help with access to on-line medical stuff. Thank you, I'll be in touch once I get my act together. Thank you everyone who has visited this space and offered kind prayers or intentions. Between the help and support we get from the virtual world and the daily help we receive IRL*, I know we are not alone.

We're going to try to be more about the every-day parts of our little days, how Liam is growing, whatever the facts are with Will so people can keep up with that, and less a vomitorium for anxiety around here.

*A small sample: Grandpa DM and Grandma KM were over at the rental again today, working with the workers to figure out the floor, and then going to the store to get the new flooring, and then taking it to the house. . .Grandpa DH and GG had Liam all day today and will keep him overnight tomorrow because I have an extra early work day on Thursday. A very kind message from a cousin who has enough on her plate to not need to take the time to reach out to me. Thank you.

Surfacing

I wish I could say we were doing better. I'm stressed out and a mess. Not helping anything. Will is short-of-breath with any activity and sometimes at rest. I don't know what his lung functions are. His saturation in the upper 80s and low 90s. Lower at home than in the hospital -- and I don't know if that is progression, or the home monitor is wacky, or it's just variable or what. He's tired; wiped out feeling.

At the same time, he is incredibly steady through all this -- not freaking out in any way that he communicates to me. When asked says: I'm sure it's temporary. And he feels some better in the evening. Right now walking around eating left over pizza; dealing with the dog.

The thing is, rejection-related damage is not temporary. It's permanent.* The question is how much of what he is experiencing is that and how much is congestion or whatever that will improve with treatment. He's still on iv antibiotics for the line infection. He is scheduled to be seen on the 28th, but I would be so much happier if he could be seen on the 21st since, to begin with, we don't even know if the steroids worked to halt the BOS process. There are other treatments** he could talk with his doctors about -- all pretty strong. None very well known or proven. The most successful one as far as I can tell is a chemotherapy drug. I feel my antsy need to act, to do something, even when there's nothing to do. As if I could do anything to change any outcome. Research. Call the clinic and make sure the follow up is actually scheduled. Fret. Cry.

September was scheduled and known in advance to be a killer month at work for me. Clients want a year-long project finished two months early and we don't really have a choice. Even without out that pressure, there's only so much (not much) I can push out, send on to others, or not do. It's a small company. I own part of it. I have to keep these clients happy and earn new ones: that's how we stay in business and keep the people who work for us, well, working. It's a terrible job for our situation really except that it pays pretty well, is secure (all things considered), and has good health insurance. It holds some promise for a prosperous future, which, honestly, doesn't really seem that important right now. I have a trip next week that I'm terrified to go on and there really is no one else to do it. I miss my old government job, when you could just take a month off if you had too at the drop of a hat (I did when my father died unexpectedly), and while it wasn't considered a great thing to do, nothing bad really would happen to you. Your job wouldn't evaporate while you were gone the way clients can. And no one else would be out of work because you needed the time.

My old house, which we rent out, has renters moving out and some problem with the floor which requires a bunch of worry and attention even though Grandpa DM is handling most all of it in terms of finding the workers, and figuring out the specifics of the problem. It's going to cost a bunch of money that we don't have.

The worst part is I really have no idea if my worrying is out of proportion to the situation or not. I know only this: BOS is what most lung transplant patients who go past 5 years eventually die from. The transplants just don't last forever is the bottom line. Everyone knows this and accepts it in their minds. . .but my heart has been slow to follow. BOS can stabilize and plateau and be fine for a while and then act up again. We were in a plateau for about the past 3.5 years. Now it's acting up. We hope for another plateau, but I have no idea if we're going to hit one, or what it will look like, or how long it will last.

That's really not the worst part. The worst part is thinking of dear Will having to go through all this decline again. Having to think about the specifics of that, for real, as a result of where we are now. As if once wasn't enough. As if the fair price for 8 - ? years is this. Honestly, I cannot think about it without totally shutting down. I can't even begin to write about Liam.

*Some studies say that high does of azithromycin, over time, might recover some function. Will's on that, and has been, except that when I insisted he go to the hospital back in mid-August the not-his-regular-doctor reduced his does. That's, apparently, when all this started; something I didn't find out until today. He's back on his regular, high dose now and has been for about a week. I will lose sleep over this for the rest of my life. Other antibiotics in the same class also are being studied, with some promise.

**This is a partial reference; a section of a google book. I have to try to find the underlying studies. . .. So many of the articles and studies aren't easily available; or you have to pay for them. I think Will's doctor would give them to us, but I have to remember to write them all down and ask. It is really hard to make myself shoehorn in all this research when the last thing I feel like doing is looking up and reading terrifying things on the Internets.

Rejection

So, for his 8-year transplant anniversary, Will received a diagnosis of chronic rejection and a picc line infection. He's in the hospital being treated with high-dose steroids for the rejection and iv antibiotics for the infection. We are hopeful that both treatments will work and that his lung functions will stabilize and potentially rebound somewhat assuming that at least some of the decline is infection-related. The rejection-related losses are permanent.

The kind of rejection Will has is called bronchiolitus obliterans syndrome and it is basically an immune-injury/inflammation-related response that causes fibrosis and obstruction of the small airways. It's a rough diagnosis to receive and we're all still just trying to figure out what to do with ourselves.

Matching Lunch

Matching lunch, and fire truck play. This was preceded by Liam calling Will into the kitchen. He would yell "Daddy!" and Will would answer from the other room "Yes Liam;" and then Liam would say "woulk, woulk" (walk, walk) and then babbling. It's pretty funny.

How many male generations does it take to deal with a tiny animal candle?

It takes three generations, but at least they enjoy themselves. All the same smile, 3x over. Happy Transplant Anniversary, Will.

Family Transplant Anniversary Celebration

Family transplant anniversary celebration tonight. It was a little subdued since Will had a very high fever yesterday and a more mild fever today. Hard to tell what is going on. His regular pulmonary doctor finally weighed in on Friday and discontinued the IV ganciclovier (surprise!) since he's not convinced Will has a systemic CMV infection. There was shell vile* on the second day from the bronchoscopy lavage, and the serum level was lower than it's been in a long time; so, that doesn't scream active CMV infection to, well, anyone, or it seems like it shouldn't. They treated with iv ganciclovier because they didn't have any other idea or explanation for Will's symptoms. This is annoying to me in the extreme since iv ganciclovier is toxic to kidneys and to bone marrow -- two things that especially need protection in Will. (Also, there are many other possibilities: not yet healed from the last infection, bacterial infection, fungal infection, viral-related BOS step-off since we know that viral infections can be associated with them and we suspect Will did have a CMV infection at the end of July. This last one is very unpleasant to think about.)

At any rate. I think Will is really wanting to hold out from the hospital until he is scheduled to be seen in clinic on Tuesday. That's great, except that if he has another high fever I really think he is going to have to go in: (1) he has a picc line, so it could always be a line infection; (2) he was on iv ganciclovier for a week and may well be neutropenic which means any fever is very concerning; (3) he is congested and doesn't feel well, and these things don't tend to go away on their own. Last night I was very worried about all this. Today, I managed to mostly put it aside. Will is really congested; so maybe it's *just* a bacterial infection. We'll see.

We used our special, nifty little candle holders for dinner (Thanks Auntie L!) and after Liam practiced blowing out all the candles. About half way through he figured it out and that was it, all the rest got blown out before you could blink an eye. Darn it, I had pictures to post, but I didn't get them into the computer before the camera battery ran out. I'll post them tomorrow.

*Check out this link -- it's a google book!

Happy Transplant Anniversary

Today/tomorrow are the days. Will tells me that based on their observations during the transplant operation, the doctors said that he was probably within weeks of death when he got the call.

The transplant anniversary makes me feel the same three or four things every year.

First, gratitude. Gratitude for a family somewhere who on hearing what must have been horrible news, some version of your father, or brother, or son* is gone, didn't entirely close up on themselves, but instead reached out. That is an amazing thing. It is a daily object lesson in humility and kindness, and one that continuously challenges me to better live those values. Grateful that Will works to maintain his health and transplant. And for his doctors, and for our families who help us so much. We're grateful for another year.

Second, wonder and sadness. Will and I weren't together when he had his transplant, so I didn't go through the recovery with him. But I know it was hard. And the course lately has been hard. And chemotherapy was awful, as was the vestibular loss, the pneumonia when he had his spleen out, and all the rest of it. Throughout it, Will finds a way to keep on.. At the same time, it is hard, and I wish it weren't so. And the future is, at best, uncertain.

Third, hope. I have so much hope for the research and new discoveries that are happening all the time. That they will help people with CF be treated successfully so they can live long and productive lives without a lung transplant. That they will come up with better ways to maintain transplants, and treat post-transplant complications and infections, or better ways to do the transplants in the first place. It would be great if Will's health could be helped by some of these things and even if it is not, it is so hopeful to think about what they might mean to others.

Finally, I always feel moved to act on this day to somehow encourage others to consider organ donation and to make your intentions known to your family. So, if you haven't already, please do. Whether you choose to donate or not is deeply personal; but if you do choose, please let someone know. Write it down. And register as a donor which, in the NW you can do here.

That's all. Will and Liam are upstairs having story time, and it's my time to go up and do the rocking to sleep now and then come downstairs and enjoy a cup of tea with Will. I'll post more tomorrow with and update on Will's current health complications; he's having a very good day today for which we are incredibly grateful.

*Somehow we know Will's donor was male.

Meet Nemo and Dory

The upshot: Liam has named his stuffed Teddy Bear "Nemo" and his knit kitty "Dory" and has been swimming them all over creation and rocking them to sleep in his blue blanket. The orange one is not Nemo, she is Dory.

The back story: A few weeks ago I had the idea to institute a family Movie Night. Never mind that Liam is only two and has a two-year old attention span (which, even for things he's really interested in is not more than 10-15 minutes), and never mind that he's never really watched any amount of TV, just a few Baby Einstein videos, occasionally.* Movie Night it is -- onward. I did some online research about age-appropriate movies for two year olds and decided on Finding Nemo. Will and I did not preview it, although I did somehow know that I was supposed to be on the look-out for the "shark scene."

Sheesh. Sharks were not the half of it. In the opening scene/prologue the mother fish and almost all the baby fish (they are fish eggs at that point) are destroyed by, something, I'm not sure what, I was too horrified to notice it properly. Luckily Liam didn't really get that part because he was super-excited to be eating dinner in the living room and was focused on whatever we ate that night, I can't remember what that was either.

So, while I'm recovering from the mother fish and all the baby fish being killed and trying to figure out whether to go on with Movie Night at all, the movie starts to unfold with all the brilliant underwater-animation, songs and bright colors. Liam thinks this is very cool and watches intently. Will and I talk with him about the different fishes and what is happening. He cuddles up on the couch with us. It's going well! Then Nemo gets captured by a menacing looking diver and put in a plastic bag and brought to a fish tank in a dentist's office. The Daddy Fish (who Liam also calls Nemo) chases after the dive boat frantically. It is clear that something very bad is happening to little Nemo. Liam is worried but allows himself to be comforted.

The rest of the movie is Daddy Fish and his side-kick Dory running into all sorts of obstacles and some help in their effort rescue Nemo. In the meantime, Nemo is in the fish tank with other fish-tank bound fish, and his part of the story is all about trying to hatch escape plots so he won't be given to the dentist's fish-killing niece as a birthday present. It has a happy ending. Liam watches about 35-45 minutes. We fast forward through the shark scene, which is near the beginning. He loses interest long before the movie is over, but was interested enough to want to watch part of it again the next morning. In general I think it was too scary, or emotionally intense, or whatever, for a two year old, but what do I know.

Since then we've put movie night on the back burner until Liam is older, and I sent the Nemo movie back to Netflix. But Liam still talks a lot about Nemo ("Mee-Mo") and Dory ("Dor'ray"). This morning he started on about them as soon as he woke up and after a few minutes I realized that he was telling me that his stuffed teddy bear was Nemo. Okay. Over the course of the day, Will's mom told me he decided that knit kitty is Dory. They both like to swim by squirming along the floor, face down. Liam is very loving towards them, and has been wrapping them up in his blue blanket and rocking them to sleep. ("Mee-Mo wrock, wrock. Dor'ray.") We are very proud of his creativity and pretend play, and hoping that Movie Night hasn't somehow scared him for life. It will be interesting to see where this all goes. (In the photo below "Mee-Mo" is wrapped up entirely in the blanket and not visible.)