Thanksgiving

I'm especially thankful for my two boys this year. Will who has been through so much and has been so steady through it all. Liam, our amazing, beautiful baby, who we are so blessed to have -- a lucky IVF miracle. Thankful as always for Will's donor family. More on the dinner, etc.. later. It was a lovely day, I like very much the idea of a holiday the point of which is to feast in gratitude with those you love most.

Rain, rain all day today but warm. Warm enough to walk in just a sweater when the rain was lightest, and stomp in puddles, and visit the neighbor's friendly cat (Bunny) who was surprisingly and delightfully out in the rain too.

Home

We're all, finally, home. I had to go far up north for work this morning and was able to pick Will up at the hospital and bring him home after that. He had a portacath installed (not installed, what is the right word? placed, I guess); it is a way that they can provide IV access, hopefully easily and basically whenever needed. Please, if you have a moment, join me in praying to whatever gods or goddesses you pray to that it doesn't get infected and have to be removed on an emergency basis. That would be bad. As long as it works though, I hope it will be very good -- no more picc lines! Easier to arrange IV treatment! Faster discharges from the hospital (we hope)!

So, home, on IV ceftaz for at least another week to treat a few kinds of resistant psudamonous. Coumadin to continue treating the clot from the final picc line. And, I think that's all that's new. The nurse went over all the medications with me when Will was discharged. Holy Mother of God, it's a lot of medications. I don't see how Will keeps track of them all.

Liam was so happy to be home and see Will. And so relaxed (and tired, he was up at 5:30 AM with me this morning) that he went straight to sleep tonight. Looking forward to a day to catch up on grocery shopping, picking up, and just being around the house tomorrow. And the library, maybe; Liam told me that he wants a book about sharks (Why? I'm not interested in sharks.) and that we should "go buy one at the library." Okay.

Wind and rain; rain and wind; wind and rain. Makes one appreciate anew the idea that we're really very close to the (temperate) rainforest and get a lot of it's leftover weather.

About the Hospital

It is good and helpful to know the locations and meanings of things. I know where the patient food and nutrition room is and that some of the nurses think it is okay for you to go into, but others don't. The clean sheets and pillowcases. The warmed blankets. The place to take the tray when meals are finished. I know them all.

I know where security is to get you into the parking garage after hours and how to (usually) find a wheelchair with a full oxygen tank. Where to find the snack cart in the middle of the night after the cafeteria is closed. What time the coffee place opens, and that it is a different time on Saturday (8:30) and different still on Sunday (9:00).

I know what a rapid response is.

I know the difference between the resident, the 3rd year resident, the intern, the fellow, the attending, and the medical student. I know that you are not supposed to call the medical student "doctor" especially when the 3rd year resident or the attending is around. It makes the medical student have to explain. I know the charge nurse and the stat nurse and what they do and what it means when they show up (something has gone wrong or is getting worse).

I know how to reach physical, occupational, and nutritional therapy. I know the direct phone number to the Medical D Team team room. And somewhere I have written down the number for the Medical A Team room, from some other time, before. I know the medicine doctors call the shots officially but that you have to suck up to the consulting specialities too. That it's your job to plant seeds, ask leading questions, sometimes be direct, and---above all---keep track of what is going on. Every test, every dimension of every problem, every theory or idea; and it is your job to interject, gently in a way that makes it seem puzzling, what you know about what happened the last time, because they do not know that, and they are not looking.

I know what time blood is drawn in the morning, and roughly how many days you have to be here before then send the "spiritual care" person even if you said you didn't want one when you were admitted (10 days). I know that it takes a minimum of 56 hours to get discharged from the first time the word is mentioned. Sometimes, often, longer.

Will is stable today; some better, no worse. He is getting IVIG in hopes that it will help his immune system fend off this and future infections. It's a non-specific treatment, meant to improve the overall vigor of his immune response. Later this week if all goes according to plan he'll have a portacath placed, and after that, we hope, come home and not have to come back here for a long time. Liam and Mollie are with my parents (thank you!) and I leave tonight for work east of the mountains. Back late tomorrow.

Hospital Update

This is Will’s seventh or eighth hospitalization since August. I think in that time the longest he has been home has been about two weeks. I guess this is what moderate to severe lung disease looks like. He just can’t seem to get stable or well.

In this last series, he was discharged on October 30 on two IV antibiotics and IV ganciclovir (to treat CMV). He was taken off all those medications on November 9 because his picc line was infected and had a clot (the line was removed that day, too). By November 11 he had a junky sound cough and by the 12th he had a persistent fever of 102 and his oxygen requirement had gone from saturating in the mid 90s on 2 liters to saturating in only the upper 80s or low 90s on 5 liters. That’s a pretty fast downhill and pretty frightening.

The ID doctors now think that Will coming off IV antibiotics must have coincided with the drop in his white-blood-cell count caused by the ganciclovir. (He was very neutropenic when admitted on Friday; and we expect the wbc drop with ganciclovir.) So, he had really almost no immune system at all at that point, and that allowed the bacteria to take hold again really quickly. The pulmonary doctors think this is a good a theory as any, especially since Will is improving on the (new) IV antibiotics they’re giving now.

The second theory is that the clot in him arm from the picc line caused a transient blood-infection (the blood cultures haven’t grown anything yet, but they can be really hard to catch) or that a small piece of the clot migrated to the lung and caused the new consolidation and Will’s other symptoms. The pulmonary docs are less interested in this but say that it’s possible. (It is, I think, the medicine docs favorite theory though.) Will is being treated with a heparin drip to get rid of the clot in his arm, which is the same treatment he’d have if the embolism theory turned out to be true, so, that’s that. The only way to know for sure about an embolism is to do a CT with dye and contrast, which Will’s kidneys couldn’t tolerate right now.

Possibility number three is that it’s a return of CMV; he did have a slight positive for CMV when tested on Friday (up from a zero on Monday), so that will have to be watched carefully. The CMV is looking like more a future concern though, not the current problem. Possibility number four is that it is aspergilosis. They were getting pretty concerned about invasive aspergillius on Sunday, especially since Will had been having sensitivity to bright lights. But, since he’s improving, and that sensitivity is going away, the aspergilosis concerns have sort of fallen by the wayside. Will was successfully treated for invasive aspergillius about 18 months ago. It’s a scary thing to have since the prognosis is not great; however, since Will is known to respond to the treatment it is at least familiar.

Possibility number five, as always, is that it is something totally different and new: new bacteria, resistant bacteria, new virus, or whatever. They don’t think it’s the flu (either type).

So, the current plan is continue treating with antibiotics, continue giving fluids and hope the kidney function improves, no bronchoscopy, await further culture/sensitivity results from the sputum sample and viral culture results from the nasal swab/lavage (which they might have lost and, if so, will have to re-do), and see how things go. Will’s kidney function is improving but still not back to his baseline (his baseline for kidneys is not 100%, it’s more like 50%). His anemia is all over the place; he did get 2 units of blood over the weekend, and that seems to have improved things slightly. His white blood cells are starting to come back now that the ganciclovir is stopped and he is being given GCFS again.

The other thing that will have to be worked out before Will can come home is IV access. He can’t have any more picc lines right now. So, the access options are either peripheral IVs or a port. The pulmonary doctors want a port, but everyone will have to be really certain that Will is as infection free as possible and healthy/stable enough to tolerate the port placement, which is semi-surgical.

So, how’s Will doing you might ask? He’s tired. Tired of being sick and in the hospital, and sad about moving a step or two more down this road of progressive lung failure. A port is sort of a big deal, it says: you’re at the point where you need medical intervention most of the time. He was happy to get rid of his port after his transplant. It’s hard for him (that puts it so lightly) to be at this place again. He’s very steady, but he’s also tired and sad.

Breakfast Update

We are blessed and lucky that when I work and when I stay with Will in the hospital, Liam is cared for my either my parents or Will's. These last few days, Will's parents have had him. Here is the email I received this AM with the Liam breakfast report.

"Liam did not want a scrambled egg...until D came out with, "What, you don't want a chickenberry?" (That is a very old Southern term I haven't heard in years, and I don't know where it sprang from today.) Immediately, Liam said he wanted chickenberries RIGHT NOW. So he ate most of his, and then had a small bowl of cereal like GoGo's. [GoGo is what Liam calls grandpa.] The cereal has a modest number of raisins in it, Liam picked them out first, and then sang, "Raisin bite, raisin bite, where are you?" to the tune one of the Wonder Pets songs."

If you'd like to hear the Wonder Pets song and see how the show is made please click here. Really, why should we be the only people who have it continuously playing in our brains.Things here at the hospital are going fine. Will seems to have turned some kind of corner and is improving. Of course, it's never that uncomplicated, but all in all, things are looking better and I'll post some details soon.

65 Red Roses Documentary & Deron Arnold

65_RedRoses is the story of Eva who has CF and received a life-saving, double-lung transplant a few years ago. The documentary tells the story of her waiting for and then receiving her transplant. Since the transplant (which was about 2 years ago) she has suffered from chronic rejection (the same condition Will has), except that hers has moved much more quickly and she is now again listed and waiting. We aren't in touch with too many CFers on line, a few. It's really not Will's thing (In his words: the last time I did that everyone I liked died in the space of a few months and it was awful.) and it's not my disease. But I lurk a little, and share what little I know of the specifics of Will's experience (medication issues, treatment experiences, etc.) when it's on topic, and I'm in touch with one or two spouses of people with CF. That's how I came to hear of the 65_RedRoses movie and Eva.

At any rate, if you are at all interested in CF or organ donation I hope you have the chance to watch Eva's story. (Also, it is meant to be a great documentary and winning awards and all that; so it's good if you just like good films too.) If you get CBC it will be on Monday night, November 16. We'll be watching. And, Eva, if you find this on the Interwebs, we're thinking of you and sending love.

In other CF-related news, I am very sad to post that Deron Arnold passed away and according to his faith was undoubtedly received into heaven on November 8. Deron also suffered from CF and after waiting for more than a year received his transplant in September. Unfortunately there were many complications and Deron never fully recovered. Deron was, I think, pretty much the first not-already-known-to-me commenter on the Liam blog. He was a doctor by training (pathologist) and had lots of questions about Will's CF and transplant experiences. They "talked" occasionally and I sometimes played intermediary, relaying questions and answers back and forth. I've been thinking of Deron's death a lot this past week and it makes me really, really sad, which is why I haven't written anything about it before now.

Deron and I didn't have a lot in common in terms of our world views, but I had great respect and affection for him. He was a very likable person, even if we disagreed about, well the things people disagree about, I guess, politics, religion. I hope it's not gratuitous to say that. At any rate, we both had a lot of questions about and were pretty invested in navigating the hazy world of chronic, life-shortening, illness (him because he had one, and me because of Will). And we both had young children conceived through IVF. I admired his steadfast faith, curiosity, good humor, and seeming ability to pull up his socks and get through some really unrelenting tough stuff first while he was waiting and then after transplant. He didn't want to die. He never stopped trying to get better. He is survived by his wife and twin boys, his sister and her family, and his mother.

I invite you all to join me in remembering Deron and his family and praying for Eva and her recovery. You know that I think the world of transplant technology, and it's getting better all the time, and if you're not already, I hope you rush out and become an organ donor right now. But, what we really need is a cure for CF. If you feel moved to include the doctors, nurses, researchers, organizations, funders and countless patients contributing to CF research in your intentions, I think that would be just great too.

Here we are again. . .

Back at the hospital. I'm not exaggerating when I say I think that Will has been in the hospital fully as many days as he has been at home since August. Really. Maybe I'll get the calendar out an actually count. Maybe not.

This time it is very high, persistent fever that came on suddenly (Thursday morning) and was accompanied by increased shortness of breath and a big jump in Will's oxygen requirement. He was started on Tamiflu yesterday (just in case) and this morning we came into the hospital through the ER, as directed by the transplant clinic. The ER doctors confirmed the decision to admit him right away, and they started antibiotics, etc. Eleven hours later he was finally assigned a room and brought upstairs. Eleven hours in the ER is a long time. Especially long if you're sick and feel like crap. Also especially long if you've had about 5 hours of sleep total in the past 36 hours and still have work that must go out that day and the ER has no Internet connection.

At any rate, Liam is happy with Will's parents. My parents are kindly taking care of Mollie the dog. And I'm here at the hospital with Will who is very sick but appears to be stable. Hopefully the antibiotics will start to help and some of the many, many tests that are currently cooking in the lab will come back soon to tell us that whatever it is, it is super curable and really easy to treat.

In other news: Liam has started banging on his music toys in time to music and/or singing along with the banging. It started over the weekend last weekend and is continuing. It is very, very sweet, I'll try to take video. When he is singing along he favors "BINGO" (you know: there was a farmer had a dog, and BINGO was his name, oh, etc.) and "Baby Beluga" which is a Raffi song that you really only need to know any more about if you have a 2 year old in your life and if you do you undoubtedly already know all about Baby Beluga. (Okay, here, you can see it here.) Otherwise, he will do his best to bang away in time with whatever is playing.

No! You do it!

Liam is getting pretty good with pronouns. He uses I and me mostly correctly. Him, her, we, they, he's got those down. He still talks about himself and others in third person about half the time, but he's defiantly making progress. Except, he's a little confused about "you." I'm pretty sure he things that "you" is a synonym for "Liam." I had been wondering about this for a while and then the other night we actually had the following Abbot and Costello type exchange.

The scene: Liam and mama are playing with cars and finger puppets. Mama puts the finger puppets on her finger and they talk to the cars and run around. Liam pulls the finger puppets off and excitedly makes them hop around, chattering the whole time. Liam hands the finger puppets back to mama.

Liam: Again!
Mama: You want to play finger puppets again?
Liam: Mama do it, please mama.
Mama: Okay, should I put them on me or you?
Liam: You!
Mama: Okay, are you sure?
Liam: You! You!

Mama starts to put the finger puppets back on her fingers. Liam goes nuts.

Liam: "No mama, you do it, you do it!"
Mama: "Liam, do you want the finger puppets on mama's hand or Liam's hand?"
Liam: "Liam's hand."

Okay. Order was restored.

Halloween!

Liam re-used last year's costume for Halloween this year. Last year the lovely fuzzy little hooded dress became -- magic! -- a Polar Bear. (Click here for the story of last year's Halloween and here for Liam's related zoo adventure.) This year: Little Lamb. Although, Liam sometimes called it a Sheep. And, sometimes called it Little Angle Lamb, which, due to an unfortunate gap in Liam's ability to make certain sounds in certain words came out sounding for all the world like "A-hoo-le Lamb." Sigh.

After proclaiming all week that he didn't want to dress up and didn't want to go trick or treating, Liam rallied and went out on Halloween and really enjoyed it. All it took was an afternoon of my mother hiding in the bathroom and jumping out with a small candy bar every time Liam knocked on the door, and a clarification that we did not need to go INSIDE the houses, we could stay OUTSIDE, they would bring the candy to us. All in all, a successful outing. He likes to be out after dark, our baby.

This is, I assume, the last year that I'll be able to pocket Liam's Halloween candy and not give him any, at all, after the distribution during the actual event. He probably ate 2.5 mini candy bars total (not counting whatever my mother gave him during the training portion of the day). I have eaten many more than 2.5 mini candy bars and am ready to stop. the. madness.

Will is doing okay today. We turned down the oxygen slightly, which is going fine, and he was able to get up and come downstairs this evening to have dinner with Liam and me.

A long few weeks

It has been a long few weeks. Will started having high fevers and trouble breathing and went into the hospital two Tuesdays ago. He was treated with antibiotics (still on them) and, of course, the fluconazole continues. He got sent home on Saturday, and we were hopeful that things would start to look up, but it seemed like immediately on getting home his oxygen requirement started to go up and by last Monday we had the oxygen concentrator turned up to its maximum setting and were still having a hard time.

Then, last Tuesday he had some wacky lab results including a crazy high potassium level that resulted in the transplant clinic tracking me down at work to tell me that I had to go get Will immediately and take him to a local emergency room to recheck the blood labs and get stabilizing care. They emphasized that it would be too dangerous to take him all the way to the Seattle hospital (it's about 90 minutes away) in this condition. Except, after all that, it turned out that on rechecking it seemed like his potassium level wasn't quite so crazy high after all. In one recheck it was normal, in another it was a little high, but didn't need treatment. At any rate, considering that he was becoming more and more short of breath no matter how much the supplemental oxygen was turned up, they wanted to admit him again anyway. So, he went back into the hospital, was started on anti-viral treatment for a possible CMV infection (on top of everything else) and sent home on Friday.

That's the Readers Digest version. I'll spare you the details of which medications, the arguments and difficulties with the doctors over which tests to do. (Bronchoscopy or not? Did they really need one to know what to treat, I still think no. Ganciclovier or not, is it really an active CMV infection, we're still not sure. Overdose of GCSF and crazy high wbc count. . .again? Three iv medications, 1 every 8 hours, 1 every 12 hours, and 1 every 24 hours. . .really?) So, I guess, on reflection, I'm feeling pretty good that today is Tuesday again and we're all three -- Will, Liam and me -- tucked away cozy at home.

Will has been quite short of breath since coming home from the hospital, even on 4 liters of supplemental oxygen. It's not clear exactly what this is about. His O2 saturation is okay, but he gets really out of breath with any activity. For the first day or so home he stayed upstairs, pretty much in bed, all day. This was different and upsetting. Even when Will has felt his worst, towards the end of chemotherapy for example, he always would get out of bed and come downstairs during the day. He is feeling better today and yesterday. Able to come downstairs in the afternoon and to walk around the house a little. We're hoping for continued improvement. It all feels really fragile though. And I feel so sad and unhappy for Will, because it's not nice to be this sick and have to go through all this stuff. As awful as it is to be the one worrying about all the hospital stuff, I imagine that it is worse, much worse, to be the one in the hospital actually experiencing it. We've taken Liam out of school for now, in an effort to reduce germ transmittal.

I'll post separately about Liam's Halloween and try to be better about updating. Thank you, all for your kind good wishes and thoughts. It does help; really it does.