CF Awareness Month and Creating a Real-World Social Network

May is CF awareness month. 

You can help.  Giving money to support drug research for a cure is great.  I do that.  In my mind, though, bringing dinner by some night when someone really could use it is. . .better.  Coming over to play cards or watch a movie or just hang out with someone who would otherwise be alone, also, better.

My 90-odd year old across the street neighbor, Mr. Z, who generally still mows his own lawn God-bless him, drives out twice a week to deliver meals-on-wheels to seniors.  He tells me, otherwise they might not see anyone.  He wants to help.

He and I talked about this one day last fall when Liam and I went over to rake leaves for him.  Mr. Z protested that he could rake his own leaves, thank you very much. (This is true but it's really, really slow these days.)  He protested and protested.  Finally I took him aside.  John, I said, I'm trying to do something here, I'm trying to teach this kid about neighbors and taking care of people -- he's only going to learn that by doing it and seeing it done.  Will you help me?  He stopped protesting.  Can I give him a dollar he asked?   Sure, I said, he'd love that.  We raked leaves.  In the winter we shoveled snow.   Sometimes John gives Liam a dollar.  More often not.  Sometimes, not as often as I'd like, we bring soup over.   

You could help someone with CF, or you might help someone else.  Just get out there, be present in your world.

Check this out if you need some CF-related motivation:
Emily's entourage 
Emily's entourage, TEDx talk, about using social media to create a community of support
New medications are making are difference, these make me feel so hopeful.

Okay - thus ends the sermon.

Advances in CF drug therapy

Hello Universe -- I respectfully ask that you give a little power and attention to this; Gods and Goddesses please guide and inspire the scientists and researchers, help these medications to work -- really work -- and get them to the people who need them quickly and at a cost normal working humans can afford.

First drug ever, as far as I know, to treat the underlying salt transport problem that causes CF to be so deadly goes to the FDA with a request for accelerated review and approval this week.  Thank you God for letting it get this far.  It's a start, it's a start, it's a start.

So it goes

Today the NYT published an article about phase 3 clinical trial results for VX-770, a Vertex drug in development for treatment of the underlying genetic defect that causes cystic fibrosis.  The results are very promising -- both in the specific improvements in lung function and other measures, but, perhaps more importantly, in demonstrating that the drug is acting on the sodium transport defect that is at the root of CF.  One of the main ways CF is diagnosed is through sweat tests.  Will used to repeat the story of an old Swiss/German folk saying, something like: "Sad the child who tastes salty when kissed, for he is cursed and soon must die." People taking the the Vertex drug had less salt in their sweat.  Much less. 

This particular medication is desinged to help one of the less common CF gene mutations; but Vertex has a similar medication in development, based in part on this research, that would treat the most common mutation; the one Will had.  It's a hopeful day for the CF community.  Hopeful.

A few things

This is the picture we used on Will's memorial card; he painted it years ago and it had been put away, but I found it when trying to go through all the art supplies and preserve what we can for Liam.  It seemed perfect for the card.  On the back we put one of my favorite blessings.

May the blessing of the rain be on you—
the soft sweet rain.
May it fall upon your spirit
so that all the little flowers may

spring up,
and shed their sweetness on the air.
May the blessing of the great rains

be on you,
may they beat upon your spirit
and wash it fair and clean,
and leave there many a shining pool
where the blue of heaven shines,
and sometimes a star.

Fitting since it has rained going on four inches or so around here since Friday.

This is the link to Will's obituary.  The service was well attended and lovely, so that was a relief.  My sister when home to California for a week today; she'll be back for two weeks at Christmas and then we're well and truly on our own for a while.

The family dinner after the service went okay, I hope.  No yelling or crying, so I guess that's good.  We have lots of leftover food.

The dog is taking it very hard.  She went to dog daycare and then my parents' on Friday.  Yesterday she came home lethargic and sick to her stomach.  Finally around 5:00 I took her to the emergency vet.  Pancreatitis*, they said, probably caused by stress and grief.  Rest, IV fluids, and various medications to treat pain and nausea are needed.  Fifteen hundred dollars later (late tonight or maybe tomorrow morning) she can come home to rest here.  Pray it doesn't become recurrent.  Poor dog.  I hope it doesn't make me a completely horrible person that I find it sad, worrisome, and annoying in equal measures.

*Mildly ironic because people with CF often get this, although Will never did, at least not officially.

New bed

 

This was Will's hospital bed at home.  There's no easy way to think it and no good way to communicate it but to be out with it: Will died at home on November 27 at about 1:22 in the morning.  I know the time because I had been there with him for the past 48 hours or so and I had my head on his shoulder and my hand over his heart when it fluttered and stopped, fluttered and stopped, and then just stopped.  We had been giving him medication every hour through his IV port trying to ease his suffering. It had been a long two and a half days.

I woke Will's sister.  Called my sister downstairs.  She trained as an EMT (in addition to her zillion other useful skills) and confirmed what I already knew.  We called Will's parents. They came over (I think they had left only hours before).  We called the funeral people.  They came over.  Turns out they are neighbors (about 4 houses down) and thought this would be a good time to let me know how  they had tried to buy this house too, but we had got it right out from under them.  I said, "Oh, umm, sorry?  I'm sure your house is lovely too."  They went away and took Will with them.  And now he's just gone.

What happened you might ask.  Well, nothing really and, of course, everything.  Will was in the hospital in mid-October.  His decline in lung function was continuing and he was not recovering.  He was increasingly short-of-breath all the time.  Going up the stairs became something to be carefully planned and limited to once a day, if at all.  Will didn't want to die in the hospital.  At the end of October his doctor of going-on twenty years recommended hospice care. We tried that, but they kicked us out when the medications Will needed for palliative care proved too expensive, or something.  That part is still a little mysterious to me. 

Will went back to the hospital in mid-November.  Through the grace of God and big, big efforts on the part of Will's hospital doctor (pray you are tended by this woman or her twin if you ever get sick, really sick) and his regular doctor, we got him home with put-together palliative care and all the oxygen they can put in a house on November 22.   He had really good days on Tuesday and Wednesday.  Wednesday my sister arrived.  Will sat a the table and ate dinner, played with Liam and Chris.  Planned for Thanksgiving.  On Thursday morning he started out okay, but then almost immediately called me from the kitchen to "help."  More oxygen, he said.  We turned up all the oxygen we had.  Gave the medications we knew to give.  A nurse came over.  We were all still planning at this point -- how to make this set up work as best as possible, what would Will want from the nurse over time, that type of stuff.  We thought it was just a bad morning.  But by mid-day he was getting worse, not better.  By late afternoon we had paged the hospital doctor and got new instructions, been told this was probably the beginning of the end.  Sent Liam to my parents.  Waived off Thanksgiving dinner, half cooked.

By Friday morning he had pretty much stopped talking.  By Friday afternoon he couldn't swallow at all.   More new instructions.  We gave medication through the IV port. 

I don't know what else to write.  I wish it were different.  We're planning a memorial service for Friday.  People have been amazingly kind.  Liam is steady -- happy and sad.  The worst is when he says: Mommy! We used to play [whatever] when Daddy was still alive.  No, the worst is when he asks: Mommy? Will Daddy be back in the spring?  No, the worst is when he asks:  Mommy? Does Daddy still have the big, big sickness even though he died?  No, the worst is yet to come.  His worst days are long in the future.  So we bend towards it, trying to think of what we can do now to make those days easier, more understandable for him.

I'm not sure what I'll do with this space.  There is much going on.  Much remembering of Will.  Much growing from three towards four.  We probably will be back here in some form, someday.  In the meantime, I thank anyone reading for helping me to have a space to remember what I want to remember, in the moment but also held apart.  Thank you.

Tigers and bears

Liam requested a tiger costume for Halloween.   He's been pretty interested in tigers this year and has seen baby tigers at the zoo.  About a month ago (I need lots of time for these things.) we went to the fabric store and he choose the fleece.  Please don't tell him it is leopard.   He has declared it tiger.   I had a pattern that was a sort of footed pajama approach. . .you know, I bet you had something like that your mother made you for Halloween, I know we did.  Well, footed pajamas went by the way in favor of pants form a home traced pattern, made a month ago and worn weekly since, and a tiger "coat" which is the top of the pj pattern cut long and finished.  I don't know why the ears look so rabbit-y.  They are the tiger ears from the pattern, maybe I was supposed to gather the base or something.  I may fuss with the ears a little tomorrow.  It still needs the zipper and the hem, but that's it: basically done a whole week early.  Thank God.  Fleece fake fur is horrible to sew. 

Like most things I have time to make these days, it's pretty cobbled together.  The hood did not work exactly right and was big enough for me to wear (Cute too!) before I attached it to the jacket.  I'm going to have to take in the center seam with safety pins or something because it falls into his eyes, which he doesn't like.  There is no way I am ripping a seam on a fleece fur Halloween hood.  It is not going to happen, life it too short.  Other than the hood in the eyes thing, he thinks it's great.  I think the pants and jacket approach is going to be versatile. . .this way in a week when he wants to wear his tiger costume to school next week I can say "Yes!" and he won't be stuck in pjs all day. 

Will is sick; his is having an infectious flare up; and his pulmonary failure, and probably lung rejection, is progressing.  He was in the hospital a few weeks ago, home now on iv abx and inhaled abx and even though he's almost two weeks into treatment he started running a fever again yesterday.  His lung function is way down -- he's short of breath with any activity (i.e., sitting or standing up makes him out of breath), and sometimes at rest.   He's keeping it together but tired of being sick.

People are sweet & you can help

I got this message (as part of a longer message) from someone I went to high school with and recently re-found on Facebook. 

"I just remembered something I wanted to tell you.  At our food Co-op, there are these jars.  If you bring your own bag, you get a bean to put in a jar.  There are various and sundry jars, each bean represents a donation the Co-op will make to whichever jar: Humane Society, Food Bank, and the like.  There's one for CF research, and A [her daughter] always always puts her beans in there.  I've told her about your family, and she wants to help."

Thank you S and A.  That does help.  Every one who raises awareness and everyone who gives towards research for a cure helps.  Everyone who brings dinner, or walks the dog, or drives to doctors appointments or the hospital, or babysits, or offers their thoughts and intentions helps.  It all helps.  Thank you.

*****************
In other news, Frank Deford (NPR sports commentator) the other day did a story in which he touched on presumed consent.  This is a policy where, upon death, people are presumed to agree to transplantation of their organs unless they have left specific instructions otherwise.  It's a good idea.  In the meantime, please register to become an organ donor and make your intentions known to your family.  That helps too. A lot.

Digging

Warm rain today; sometimes lots accompanied by wind. Then bright sun. Tonight we had that funny filtered greenish light, where the sun is bright but there are lots of dark, dark clouds just waiting to get in its way.

After dinner Liam insisted that he had to go outside. Usually he wants to run around or throw the ball for Mollie. Today he wanted to plant in the garden. Digging and planting. (Mollie wanted to be inside -- she's a smart dog, it was at best 50 degrees and threatening.)

Will has been having some good days -- feeling better, had a very positive check-up on Monday. Now it is just continue this course of iv antibiotics and hope that when he stops them in mid-May the blood infection (if there ever really was one) doesn't come back. And that the dosing for his blood thinning medications can be worked out so his level isn't bouncing around quite so much. And the clots go away. At any rate -- it's a much improved picture from a few weeks ago for which we are so very grateful.

The apple tree in early spring

We're all still here. It's been a rough few weeks. Will has been very sick, although seems to be stabilizing and getting stronger now. He's a lot better than he was 2 weeks ago: the swelling in his neck is down; his pain is much decreased; and most of all he's home from the hospital, discharged Saturday. I have been traveling for work, trips long planned and not cancel-able. DC most of last week. East of the mountains tomorrow. Will's mother has been sick and unexpected confined to bedrest for most of the past 10 days. My mother came up from California to help with Liam and everything else. Everyone is tired from the fear and worry, and all the running around and trying to coordinate schedules, medications, Liam, work, the dog.

It's great to have Will home. He came home on two iv antibiotic, plus all his regular medications, plus blood thinners. He's down to one iv antibiotic but a daily dose of saline has been added to try to keep his hydration stable (he's not been feeling like eating or drinking much, although that is starting to get better), tomorrow he starts the inhaled antibiotic again. Thursday home health comes for a blood draw. Monday back to the hospital for clinic. Blah, blah.

I haven't felt like writing or taking pictures or doing any of this stuff, but it all does go on. Liam and I were outside throwing the ball for Mollie after dinner and Liam decided to be Mollie and roll in the grass. I occurred to me that this time next year he might not want to roll in the grass under the apple tree in quite this way, in early spring, when the apple flowers are just out and the birds are making nests. And I want to remember that part too.

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.

In which a big ship enters our lives. . .

In looking back over recent posts, I’m thinking we should re-title this space: Liam goes to the park.  We have been going more than usual lately, and it’s a relief to have some outside time in general and for pictures.  Indoor winter light being so, well, dim and miserable. 

But in reality we go only about once a week.  Weekdays are taken up by work and household chores for me; doctors appointments or treatments or resting and doing house stuff for Will; and child care either with his grandparents or at “school” for Liam.  Luckily between grandparents and school at least Liam gets outdoors pretty much every day.  I’m a big believer in the importance of outdoor time, natural light, and unstructured exposure to nature (even if it’s just in the playground).   And grandparent care is about the best type of child care anyone could hope for, so that’s all good.  (Of course, I still dream every day of quitting my job or reducing my house by 50% so I can spend more time with him.  As it is he generally spends more waking hours in a week with Will’s mother than with me, as he always has since I went back to work when he was 6 weeks old.)

I think I owe an update on Will’s progress.  He had pulmonary clinic last Monday and continues to be stable.  That’s sort of a double edged sword.  It means that his lung functions are not improving (which is disappointing), but they also aren’t decreasing.  He remains on 3 liters of oxygen, 24 hours a day.  We have a concentrator and lots of tubing for the house; small tanks and an on-demand delivery valve for when he goes out.  A big back-up air cylinder in the closet in case the electricity goes off.  The doctors think a plan of alternating inhaled antibiotics (maybe with a few weeks off between switches if Will feels okay) will help to keep the infection colonies under control.  He’s back on inhaled Colistin now (hateful stuff) and when he’s done with that (maybe another week) will start the new inhaled antibiotic that the CF community has been so waiting for.  It’s inhaled azetreonam called Cayston.  Not a cure, but a breakthrough nonetheless and welcome. 

That's our news.  My parents are visiting this week and have Liam during his grandparent days.  Today, wonder of wonders, a big ship arrived just as the sun was going down and Liam got to watch it travel all the way into the harbor and to the pier.  (Which can be seen from my parents house.)   Liam loves big ships, which arrive every week or so to take on logs before departing to, I think, Japan.  We can see them at the pier from the window in Liam's room upstairs and hear them clunking and clanging when we're out in the yard.  Driving home tonight, we went a different way and pulled over to look at the lights of the ship and the tug boats from across the bay.  So close it was like you could reach out to them, the ship lights getting brighter as the dark took hold.  Then we went home and Will and Liam played What Do Whales Say When They Talk? and Can Right Whale Understand Orca Whale?  (No, not really, in care you're wondering.) And that was the day.

A CF Update

It has been a while since I updated on Will's health.  He continues relatively stable on 3 liters of O2.  He is off inhaled antibotics 3 weeks on Monday (it is good to give the bacteria a break to try to reduce resistance) and he is tolerating it okay.  As is expected when he goes of antibotics, his congestion is steadily increasing.  He also had a few days of persistent low-grade fever last week, although that seems to have resolved.  Another way we know that his chronic infection is worsening is that his oxygen saturation is persistently lower than it has been; 98 is the highest it has been, and that was while sleeping.  His satruation tends to hover in the mid 90s during the day and drop into the low 80s when he does his breathing treatments.  Low 80s is pretty scary.   He's tired and feels draggy; appitite down, generally just feeling sicker than usual.  So, he will go on with this for as long as he can tolerate it (or until it gets worse) and then we'll call the clinic and ask about (probably) a corse of IV antibotics. 

In other news I'm sad to pass along that Eva Markvoot is likely in her final days of life.  Eva has CF and had a lung transplant a few years ago.  She has the same type of lung rejection as Will does, although hers has moved much faster. She was re-listed for another transplant, but it looks like she is now too sick and it won't come in time.  Eva has been a huge ambassador for CF and through her outreach on organ transplantation has doubtless saved many lives.  If you are moved to keep her and her family in your thoughts and prayers or visit her site and wish her well, I think that would be super. 

And, on St. Valentine's Day, I encourage you to search your heart and, if you haven't already, sign up to be an organ and tissue donar and make your wishes known to your family.  You can find out how to sign up here, and can register on line in many states.  I hope today brings all visitors to this place many opportunities to give and receive love. 

Apple slices with almond butter & pulmonary clinic

Apple slices with almond butter are Liam's new favorite snack. Evening story time generally consists of apple slices, almond butter, and a big glass of milk. I'm watching them read together now, snuggled up in the chair.  Liam prefers to just eat the apple slices and then scoop the almond butter out of the dish with his fingers.  He very much wants to be sure to get all of the almond butter.  Liam started his antibiotics yesterday, but still isn't noticeably better in the cough department. I got a phone message from the pediatrician today, something about check back, but I accidentally deleted it when I was trying to save it so. . .I'll have to call them tomorrow. Darn.

Pulmonary clinic all day with Will today. And, next week it will be infectious disease clinic (because of Will's immune suppression, he's followed by ID) and oncology follow up. The most exciting thing for the doctors today was that Will's INR was 7.1 and then, in the re-draw/re-test, 7.7. They want it to be below 3, so this gets their attention. Since Will was tentatively scheduled to go off blood-thinning therapy in about a week, the docs talked with one another and decided to just stop it today. And gave vitamin K to reverse the blood-thinning effects. Figuring all this out involved an unscheduled visit to the anticoagulation clinic, a repeat blood-draw, and two phone calls as we were driving back home. So, good-bye, finally, follow-up from the great picc line clot drama of 2009. Will is happy to see you go.

The most concerning things are that Will's lung functions continue to be down and his white blood cell count seems to be going up. The lung function thing may have to do with his inhaled antibiotic, at least in part, but it probably also is a result of more permanent damage from bronchiolitis obliterans syndrome and/or all the infectious complications Will has suffered over the past year or so. The white blood cell count thing has no obvious explanation. It will be something the oncologist probably will want to consider next week. In the meantime, Will is to go off his inhaled antibiotic basically to see what happens. This is welcome (it is Colistin, a crappy, unpleasant therapy), but also scary, since it seems to have kept Will out of the hospital for the past 2 months.  We are to watch closely and call at any sign of problems.  Will coughed up a sputem sample that they'll send for analysis, which is good, because if he does start to have another infection exacerbation the docs have some relatively current informaiton about what the infectious agent might be and what antibotics (if any) it is sensitive too.

That's all.  It's a long day, but it was nice to get to spend it with Will. 

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.

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.

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.

We're home

Will was discharged from the hospital on Friday evening. About twelve days in this time. My parents braved rush hour traffic to go get him so I could pick Liam up at school, for which I am eternally grateful.

Will is still feeling really, really bad. He's on 4L oxygen continuously; this keeps his saturations in the mid 90s, except that they go down to the mid 80s pretty quickly with any activity. Grandpa DM helped me rig up the O2 tubing so I think it works okay. It's only 50 feet, so we move the oxygen concentrating machine and switch to a different set of tubing for when he goes upstairs. As we figure this all out more, I'm sure we'll get a better system. He is feeling crummy enough that he wants to just sleep a lot now.

It has been beautiful fall weather which we've been trying to enjoy. Fall is my favorite season, and I'm hoping to tap into a little bit of that if I can. Liam is on a play outside kick, and all he wants to do is "dig a big hole" (like Mollie the dog) and move the dirt around out of the garden bed, into the path, up onto the porch, etc. His hands turn to ice cubes and he has a complete fit when he is made to come indoors.

Last iv antibiotics tomorrow. Then it's just the oral antibiotics and the fluconazole for the cryptococcus infection. Unless things change a lot, we'll go to clinic probably a week from Monday and see what the doctors think. In the meantime, it's just rest and try to feel better.