The Life and Lungs of Kim

A bit of a tough day, unfortunately. Kim was pretty restless in the morning, feeling very hot (although her temp was ok) and generally cruddy. She had a 30 minute bout of a-fib (this is irregular heartbeat, in her case spiking in the 170s and then dropping) which left her pretty wiped out. This, coupled with a higher white blood cell count has the doctors worried about infection and so she's being doused in wide-spectrum antibiotics while blood labs are being run. We ended up deciding bringing the kids here in the midst of all that wouldn't be the best idea, but we'll see how it goes tomorrow. On the plus side, she's done with plasmapheresis and now we're just waiting for the results to be interpreted. Also, her oxygen levels continue to look very stable, and she managed some more mobility exercises. Still, we really need to get a handle on the mystery of what's causing some of her less-great symptoms. Paging Dr. House? 2013 holds great promise for us, and ...

Short update tonight but all of it sweet. Today was much like yesterday which means more steady progress all around. The last round of plasmapheresis went smoothly, Kim rocked being off the ventilator for extended periods TWICE, her white blood cell count is down, and her epidural was taken out. It's always great to see a tube and a med being taken away rather than hung up! Mostly she's just super tired which makes sense given how much energy she's burning healing up. I snapped a few pictures for the kids' visit tomorrow. Kim's excited to see them and I know they are eager to see her too. I'm looking forward to the reunion :)

Here are the highlights of what was a pretty mellow day: - Second round of plasmapheresis down, one more to go. Then tests to see how well it worked - Switched from dilaudid to morphine, which may help with anxious feelings. Also seems to have made her less thirsty, so that's a pretty nice bonus! - Best news: Kim went three hours today with the vent support off, including while she was doing some mobility exercises. This is HUGELY positive and had staff and family smiling. Kim was pretty tuckered so I'm going to put off the pictures / video thing for another day and perhaps bring the kids in Monday. At home: - Joby and Charlotte descended on the mall for some girl time while Jack and I went to see the Hobbit. It was a nice break for both kids and gave them some much needed 1 on 1 time. - Delicious food continues to come every day for which we are so very grateful. - Alan heads home tomorrow :(

The big news today was the commencement of plasmapheresis, which involves drawing Kim’s blood into a centrifuge to separate her plasma from her blood and give her a “clean” plasma substitute.  The process takes about two hours and will be repeated once per day for three days.  Plasmapheresis can be used for several therapies, but in Kim’s case the goal is to remove antibodies from her blood stream which may be attacking her lungs on the orders of her immune system.  So those of you keeping score at home are probably thinking “rejection”, and you’re right, that’s the working theory as to why her respiratory thresholds remain low and her lungs look congested on the CT scan.  Once the antibodies are flooding her circulatory system, they don’t want to leave without some encouragement and that’s what the plasmapheresis does. At the same time, of course, the underlying reasons for the antibodies being produced needs to be addressed, and this is all about the immunosuppress...

Great rest last night followed by a mostly uneventful day of more resting. It was really great seeing her have some long stretches of dozing to regain some energy and strength. We got the results of her CT scan back, and it's apparent there is some "gunk" in the lower lobes of her lungs. This could be pneumonia, or some other type of constriction/congestion that's keeping her from reaching full capacity and thus knock the ventilator support once and for all. The biopsy showed some increase in bacterial activity, but it was mostly inconclusive, so they'll be doing some follow-up blood tests tomorrow. There is also still a concern about rejection (there will likely always be so) and although they're not seeing any obvious cell damage, the antibodies could be going a little nuts in her system. Her fever, however, remains down and her white blood cell count has been gradually lowering. Both really good signs, but until we get that gunk identified and cleared, w...

Not a lot of news today, but the news we've had has been really good! Last night was very rough for Kim, with her vent tube causing a ton of discomfort and some poor hospital communication causing quite a bit of stress, further exacerbated by her doses of dilaudid. We were up most of the night trying to keep calm, but at a couple of points that darn tube just became too much for her, and she wanted it out NOW. Eventually she got some clonazepam which helped her relax enough to get some much needed rest. When she woke up in the morning she told me she'd had some pretty good psychedelic hallucinations so I guess it wasn't all bad :) So that awful night really set up our high hopes for the tracheotomy, which she finally got this afternoon! I know it may seem odd to be cheering about a hole in your throat, but believe me, Kim feels WAY WAY better without all that junk stuck in her mouth, hindering her every movement. She can't eat, drink, or talk with it, but it's mu...

Just a short update to let you all know that Kim is doing fine, but still fighting a somewhat mysterious fever that's stuck with her for a couple days now. They are giving her a CT scan as I write this to take a look at her chest and abdomen in search of the culprit. She may have a pocket/abscess that was formed when her new lungs were tucked into her chest cavity, and sometimes an infection can take hold there. She's also still contending with a touch of pneumonia which certainly isn't helping matters. She was awake and hilarious most of the day, trying to manipulate us each in turn with big doe eyes pleading for a soda, or just some ice. Because the tube in her mouth goes straight to her lungs, the best we could offer was a little sponge spritz on her lips and mouth. Still, her spirits are really good, if exhausted. Tomorrow we should hear if she'll be getting the tracheotomy which would be a big game changer for her mobility and communication abilities. But unti...

Kim is awake, alert, and sassy! We are trying to get her overall strength up so she's doing some mobility exercises which included getting off her bed an into a chair last night. Cheryl took a picture of Kim giving us thumbs up and a hint of a smile under that tubing. Very, very encouraging. Her heart and lungs are still trying to figure each other out and so her vent support (pressure to her lungs, oxygen assistance, and volume assistance) are still in place. The team periodically lowers some settings to give her a "trial", but unfortunately thus far she's shown she still needs a lot of help. The talk has turned to giving her a tracheotomy and removing the vent from her mouth which would make her much more comfortable and allow for a bit more mobility/exercise. Because the surgeon who can make that call is away for Christmas, it won't be decided until Wednesday. She's showing signs of pulmonary edema, meaning she's accumulating a significant amount o...

Kim had a good, restful night with Cheryl giving her a pair of pigtails and a bit of a bath. She was very alert and still grumpy about the vent, but she understands why it needs to stay in for now and agrees its the right course. Still super grumpy tho, but way less than I would be. She's such a trooper. This morning she did another breathing trial to see if she's ready to go off the ventilator and once again the decision was made to delay. Shortly after her oxygen saturation dropped to the high 60s and she started turning blue. Her oxygen from the vent was immediately boosted to 100% and she was given a sedative. She slowly recovered her oxygen levels but it was a pretty scary few minutes. We still don't know what's causing her persistent fever. They've ruled out a virus, and so far they haven't identified a specific bacteria. The fever could also be caused by rejection, and the team continues to rule out what they can. The plan for today is keep her as ...

Kim is awake and grumpy. Her stats overall look great, but she's still not breathing quite easily enough to lose the ventilator. Will try again this afternoon.

Last night was not the best, with Kim feverish, a drop in blood pressure, and a bout of diarrhea. The diarrhea has cleared up, but the fever is lingering, and they suspect she’s fighting a bacterial infection and possibly pneumonia. As a result they’ve tightened down on the visiting and ask that anyone with even a hint of a cough of sniffle stay away, and those that do come in need to gown up. Unfortunately I have a slight cough related to my asthma, so I’m sitting at home today getting my updates from phones calls with the nurse and texts/pictures from Cheryl. It’s almost certain I’m not contagious but to be on the 100% safe side I started taking a Z-Pak yesterday so I should be good for a return tomorrow morning. Needless to say I’m going absolutely nuts not being able to keep an eye on her vitals and talk to her entire medical team myself, but it’s obviously best this way. The good news is her fluid levels are looking much better, and they’ve taken her off the Lasix (diuretic), E...

I can sum up today in two words: “Holding pattern”. Or maybe three words: “Need more pee”. Despite netting 5 liters (over a gallon!) of fluid loss yesterday, Kim still needs to shed quite a bit more to give her heart and lungs the relief they need to work their magic. During morning rounds, the medical team decided to pretty much hold all meds and ventilator settings steady, with the exception of adding a bit of sildenafil to help open up her pulmonary vasculature. It probably sounds a bit counterintuitive, but out of necessity her old lungs were actually better at making the most of the limited blood supply they were given than her new ones are despite the supply being ample now, and the sildenafil gives the new lungs the boost they need to be sure that blood is getting everywhere it needs to go while they continue to acclimate to their new home. If the diuretics continue to do their thing and she loses another 5 liters or so, it’s possible we could be looking at taking the venti...

Well, we started out the day with pretty high hopes of getting Kim’s ventilator out. She’d been running a fever during the night, but by morning that was under control, her heart rate was down, and all her pressures looks fantastic. The doctors were actually giddy! So began the process of bringing her back off of the sedation so she could wake up, get that tube out, and start breathing on her own. Unfortunately every time the sedation would begin to wear off she would get very restless (almost certainly due to the discomfort of the tracheal tube), and her blood oxygen saturation levels would drop to levels too low to continue. So back to sleep, stabilize, and try again. We went through three cycles of this, and by the afternoon the staff was not quite so giddy as they had been. The determination was that Kim is still holding on to too much fluid in her lung tissue, which is making breathing too strenuous. The remedy is to make her comfortable (i.e., back on the good stuff), hit her h...

End of day report for Tuesday! Today was another good day of steady progress. We had four goals at the start of the day: 1) Perform a bronchoscopy to check out the condition of her new lungs from the inside 2) Get Kim’s epidural in so that her chest pain can be mitigated enough for her to lower her sedation and give us some good breaths 3) Get the stitches on her chin taken out (from her late-night fall last Thursday) 4) Get her off sedation and wake her up! 5) Take out her ventilator The bronchoscopy showed perfect, pink, healthy lungs which of course is exactly what we wanted to see. No sign of infection and all the little bronchioles were doing their jobs just beautifully. Lungs having passed with flying colors, we slowly began working through the next three goals (the stitches came out great; virtually no scarring), but as she was slowly coming off her sedative, it was clear she was still pretty uncomfortable and that not all of her blood numbers were ideal. They were su...

Hi everyone - here's your end of day report! Kim's condition is excellent, with all her key measurements trending the right way. She was given a bit more blood today which really helped bring her blood pressure up and stabilize her oxygen levels. (I told her that must have been Portuguese blood to be so effective). This is normal, and a great sign that her body is healing! She’s still on her ventilator and sedated, but about mid-day her sedation was changed from “on-demand” (i.e., when she seemed uncomfortable) to a steady drip. This produced two advantages: 1) She stayed more comfortable consistently and as a result was 2) able to communicate more often and for longer periods. However, as Katie warned, communication is super hard! Poor Kim has been frustrated despite Alayne’s awesome signs, but we’ve learned at this point her message is either “I’m thirsty!” or “I’m sore – move me around a bit!” Unfortunately due to the ventilator there is not much to be done about her thir...

The top number is her heart rate. It's high, but that's expected as her heart works to push out extra fluid and stabilize. Next is her blood pressure. Kim generally has low BP so this number is where it should be. The yellow number is Kim's pulmonary pressure. This works like blood pressure, but measures the pressure between your heart and lungs rather than your heart and the rest of your body. Kim's pressure was over 100, and look at it now! The dark blue number is a biggie; that's her CVP (central Venus pressure) and it's down in the low single digits like yours and mine. If its above 12 you have PH. Kim's was well over 30!! Next we have another very important number, Kim's oxygen saturation level. Before surgery Kim was in the low 90's and upper 80's. As I write this she's in the upper 90s, right where a healthy person should be! The last number is her respiratory rate which doesn't really count right now because her ventilator ...

Not a lot happening here with Kim which is just how we like it. The medical staff is walking the tightropes between blood pressure and pulmonary pressure, as well as being sure she stays hydrated while shedding the excess fluids she's collected as a result of the surgery and her previously labored heart. She's roused a bit a few times, fluttered her eyes at us and even gave us a smile when the nurse complimented her Xmas manicure

I'm heading to bed, and in closing an incredible day, I want to say a few words to you all. Please humor my sappy tone; it's your fault anyhow ;) Other than the days my kids were each born, I've never felt more grateful and awestruck by life and the people I'm honored to share it with as I have been these last 24 hours. This day was much like those birth days; a new life was begun, and promise that seemed impossible or out of reach is now real, tangible, and already blooming with each rise and fall of new, perfect lungs. The possibilities are endless, and the future is radiant. I'm humbled by all of your prayers, love, meals, babysitting, texts, phone calls, and Facebook "Likes". Thank you, thank you, thank you all. I'm forever grateful for what you've done for my family. Good night, I love you all. "As each day comes to us refreshed and anew, so does my gratitude renew itself daily. The breaking of the sun over the horizon is my grat...

Kim is finally setup in ICU. She's on a ventilator and will likely remain sedated/asleep until tomorrow. We hear she lost quite a bit of blood during the procedure and had the equivalent of two complete transfusions.

Both lungs in and stabilizing. Another hour or two and then back to the ICU.

She's stable and has got one lung in! They expect to get the other in the next couple of hours and then take her off the heart/lung bypass machine. Next update then.

Just got word that Kim has had her first incision and everything is going great.

See you on the flip side!

Lung update: chillin' in Cardiac ICU in my big ol' room. Just saw doctor, right now we expect that I'll move down to pre-op at 5:30am...which means I'm looking forward to a night of sleep! Still haven't had my copious blood draws yet but that should be soon. Mom's on a flight now, she should be here around 11pm.

Checking into UW at 6pm...after last week, our expectations are low but hopeful that these are the lungs for me. Either way, it's a long night ahead!

Apparently, karma got gypped with last week's lung transplant dry run and thought I should have a new scar, somehow. About 4 in the morning, I woke up and, after tossing and turning for a while, went out the couch to see if I could grab another couple hours of shut eye.  When I awoke a little before 7am, I had to go to the bathroom and did my version of "racing" to the bathroom.  Bad move when you're short of breath all the time.  Next thing I knew, I'm on the floor of my bedroom, Scot's on the phone, and talking pretty serious-like about me.  Yup, for the first time ever (which is a rarity for someone with Pulmonary Hypertension; syncope is one of the initial symptoms in many folks), I passed out and took it on the chin. As many of you know, any wounds to the head bleed like crazy so my heart aches for Scot: the crash woke him up and as he got up to go to figure out what was going on, he finds me unconscious on the floor in a puddle of blood.  I can'...

    I'm sitting here in ICU at the UW hospital, all hooked up to monitoring equipment in my beautiful hospital gown, waiting to hear if I'm getting lungs or not!  I shall back up...     I had my standard Pulmonary Hypertension checkup appointment and echocardiogram yesterday and although my fatigue has been greater as of late, things were all pretty normal.  I was home by 2pm and a typical afternoon and evening ensued.     My cell phone rang a little after 9:30pm but I missed that call...but then my home number rang immediately and it was Christina from the lung transplant call center to say that they may have lungs for me and that I needed to be at the hospital by midnight.  That was a very surreal moment, to hear those words and try to have the presence of mind to ask pertinent questions!  I hung up and we went into action: Scot called Jessi to have her come to the house, I went to the kids' rooms (they were awake and aware someth...

Now *that* is the blog entry I've been waiting a long time to post!  I received word yesterday that UNOS granted my exception so as of yesterday afternoon, I have moved from the bottom of UW's lung transplantation waiting list to the very tippy top.  Any new lungs that come in from here on out will be run past my profile before anyone else's to see if they are a fit (literally).  Again, the main criteria they use are blood type and size and then there are other factors they will use to find the best possible match for me.  There is no way to really estimate how long it will be before I get The Call: could be days, weeks, months.  But I'm definitely on a high alert now, for the first time since I was listed last year, and I will have my phone on my person FOR SURE from now on.  And most transplant patients get at least one "dry run" call.  A dry run is when there's a set of lungs on their way to the hospital so they call the potential recipien...

Ah, September…crisp fall mornings…yellow school buses…leaves turning brilliant reds and yellows and then falling…reestablishing family routines...political signs in yards…and for me, getting back into lung transplantation mode.  I kind of took the summer off from worrying about the when and how and I backed away from my necessary hounding of doctors to get things done.  I had a blip last month and in addition to all my meds, I’ve added more.  You say you need a review of my meds?  Why, I’m happy to oblige! ·          I’m on three Pulmonary Hypertension targeted therapies, once of which is an IV medicine  (yes, that's a picture or two of me on the drug's website) so I have a central catheter in my chest that’s attached to an infusion pump I have on my person 24/7. The other 2 are oral pills.  All are designed to help open up the blood vessels in my lungs to make it easier for the right side of my heart to...

It has not been the best week ever. About 10 days ago, I noticed that I had some extra fluid around my belly so I mostly said to myself "Huh. Look at that." and kind of increased one of my diuretics as I saw fit.  Belly got bigger (at this point, Charlotte thought it fitting to call it Alexandra -- I looked 6 months pregnant -- a round, hard water baby belly) and I felt increasingly fatigued. I finally made an appointment to see Dr. Ralph, my PH specialist at UW, for Wednesday.  He prescribed another (this makes three) diuretic for me to take for 4 days to see if we could get rid of the water baby -- we were looking for at least a 5lb drop off.   I took that Metolazone for 4 days and am down at least 10 lbs. now and am happy to report that Alexandra is gone.  I feel a little...funky...still, but no more pain of  carrying around all that extra weight. My doctor also prescribed oxygen for me at night so now I'll sleep next to a big ol' concentrator and have...

I shall remain at (or very near) the bottom of the UW Lung Transplant list for the foreseeable future.  As you may recall, my transplant pulmonologist, Dr. Edelman, was appealing to UNOS to grant me an exception that would propel me to the top of this region's waiting list (into the 90th percentile).  On June 28, I had my (fifth) right heart catheterization and Dr. Edelman was hoping that the results from that test would provide convincing and irrefutable evidence that I need new lungs.  He wrote up the test results and waited to hear from UNOS.  After about a week, they said no dice.  He wrote up MORE information and after about a week, they again said no dice.  Third time's not a charm this story; I still did not get the exception so I'm going to continue my glacial pace of waiting...and waiting...and waiting... (although, UW has really upped their pace of transplants this year: they've done 26 lung transp...

I had my Right Heart Catheterization today...the procedure went very smoothly.  As I understand it, my test results still don't quite meet the threshold needed to put me at the top of the lung transplant waiting list but that's just how I interpret them; I haven't talked to my transplant pulmonologist about the results and what the next steps might be.  I'll report back here when I do!

That is the sentiment that you'll want to give me when I get The Call that there's a donor. There will be well wishes, prayers, and hopes for all the good things on this new horizon that I don't have today: watching the rest of you run your half marathons (I mean, I'm excited to be *able* to run, I just don't plan on doing much of it); sprinting from ride to ride at Disneyland with the kids; walking carefree through Nordstrom; taking up tennis, etc.  And these are all wonderful thoughts to share with me.  They reflect the love and hope of everyone out there that I will be rid of this horrible disease, Pulmonary Hypertension, and can start planning for a bright future. But I am a little consumed of late with that time between The Call and the eventual hike with my family to Snoqualmie Falls.  What exactly happens during that period is unknown but we do know I need to get much, much worse before I get better.  And it's not this current gradual...

The gradual crumminess I've been feeling reached a peak yesterday -- woke up with extra water weight (a sign that my organs are having a hard time moving fluid around) and a headache.  Tried to rest it off with morning laziness, then tried to power through it running a few errands...but by 1pm, my oxygen saturation level was  pretty low (in the mid 80s at rest -- normal people should be at 99%, I'm usually in low/mid 90s) and I was feeling absolutely horrible so Scot came immediately home from work (and I cry a lot when I feel horrible so I was super pathetic when I called him), Jen picked up niece Zella (who is staying with us for the week) and she also picked up Jack and Charlotte from school and took care of them all afternoon/evening .   Things were rough yesterday afternoon but I was finally able to keep a big dose of my diuretic down by 3:30 and was heartened by frequent peeing all evening.  For dinner, Scot ordered chinese food and we aske...

It took two weeks of titrating but I am now at my new dose of Remodulin (100 ng/kg/min).  It’s been moderately uncomfortable while my body adjusts to this new dose: foot pain, headaches, fatigue.  Or perhaps those are just the symptoms of being so tantalizingly close to the end of the  school   year BUT NOT THERE YET.  The Nuneses: not the best with patience. I’ll continue to straddle this line between the medicine providing therapeutic results (opening up the blood vessels in my lungs) versus withstanding the increasing side effects.  At some point, the line of return is diminished as the harsh medicine disrupts other body functions; it doesn’t much matter if blood can pump through your lungs marginally better when the extra fluid that collects in your body continues to put strain on your heart.  Or if you’re in constant pain. I saw Dr. Edelman, my transplant pulmonologist, last Friday (June 8 th ).  He had talked to Dr. Ralph about my ...

If you’ve been following my Adventures in Lung Transplantation, you may remember that my place on the waiting list is determined by my Lung Allocation Score (LAS).  That LAS is based upon a bunch of test results that my doctors spit into the national United Network of Organ Sharing’s (UNOS) complex formula.  Unfortunately, the tests that weigh heavily in determining the LAS are all focused on lung disease; they don’t account for heart failure, which is what's dragging me down.   There are some awesome medical folks in the Pulmonary Hypertension field that are working with UNOS to “even the playing field” and incorporate factors that show both the lung failure AND heart failure that occurs in PH patients.  UNOS is taking public comment from anyone with a connection to this issue and since you know me, that means you!  Before June 15, 2012, I’d be ever grateful if you could weigh in on these proposed changes in the following way… Click here to submit c...

Sorry about the long absence: this blog has been a testament to the adage “no news is good news.”  I had a surprisingly good winter but this spring has been a little different…not recovering from activity as quickly as I used to, more foot pain, more fatigue in general.  It hasn’t been drastic and my schedule allows for days of recovery time so while I do eventually bounce back, it’s actually less of a bounce and more like a slow pull. I saw Dr. Ralph, my PH specialist, a couple weeks ago.  My echocardiogram mirrors my slight decline in health by showing too much fluid around my heart.  Our treatment plan is to increase my IV drug over the next couple weeks and see if that helps.  I see my transplant pulmonologist (and have a slew of other tests) on June 8 and he’s been working with Dr. Ralph on my current status so I’ll know more then. In the meantime, I had the most awesome 40th birthday party ever; we’ve been enjoying Jack’s baseball games; Charlot...

I've been the leader for our area’s Pulmonary Hypertension support group for the past couple years.  Some of my duties include organizing our meetings each month; lining up sponsors for food; arranging speakers to address a variety of topics, like emergency preparedness, low sodium nutrition, PH basics, pulmonary rehabilitation.  Some of the members have Idiopathic Pulmonary Arterial Hypertension like me.  Some have PH secondary to some other disease , like scleroderma or other lung diseases – their primary disease often is manageable but it’s the PH that’s causing their health to  decline  rapidly .   Some of us are on oxygen full time, some only while sleeping, some only as needed, some not at all.   We range in age from our 30s to 80s.   We all have families and caregivers and lots of doctor appointments.   We are often tired and find frustration in many things, including trying to explain to others how serious of a condition this is ...

Yesterday, I had my 3-month check-in appointment with Dr. Edelman, my transplant pulmonologist at UW.   Mom flew up on Wednesday so she came with me and, after picking up a coffee and food at a drive-thru coffee stand, we had a beautiful and sunny drive into Seattle, complete with seeing the bald eagle that hangs out on the 520 bridge. I started at the Pulmonary Diagnostic lab, where I repeated my spirometry test , one of the pulmonary function tests. (I sit in a booth, wear nose clips and put my mouth around a tube hooked up to a computer…then big breath in, shoot that breath out as fast as possible and get all that air out – you keep squeezing it out way past the point you think you can.   Then quick breath back in.   Repeat 3 times.   Get head rush.)   This is a test that goes into that Lung Allocation Score (LAS) but typically, Pulmonary Hypertension patients score pretty well on it and I’m no different – ironically, I’ve got great lung/airway function....

Today was my last day of Evergreen Hospital’s Pulmonary Rehabilitation program , celebrated with a certificate, a graduation cap and a speech!  I started rehab back in March of last year then abruptly quit in May when I landed in the hospital with a line infection.   I then took the summer off (to play with kids…and, sadly, spent another 8 days in the hospital) and resumed the class in September.   Insurance will only pay for 36 sessions and even though I switched from “rehab”, i.e. recovering from my bad winter health to “prehab”, i.e. gearing up for a lung transplant, insurance doesn’t recognize the difference.   I did, however, make sure that after my lung transplant surgery, I can do another 36 sessions because I’m certain I will need that to get back into shape. Evergreen offers a post-rehab program called “Power Exercise Class” and that program has more people, less monitoring (i.e. no blood pressure cuff unless you’re looking really unwell),...