Thursday, October 29, 2015

Soaring...then crash

We had a few good weeks at home with marketable improvement from Julie.  She finished antibiotics on Saturday Oct. 24.  The lung infection seemed to be at bay.  Just like last time, she was able to eat more after they were done since she no longer had a wheezy stomach from the meds.  Her oxygen dependence was at it's lowest in months at 2-3 lpm resting and 4 when she was up and about.  Her energy was up more than ever and she was doing a lot more around the house.  She was regularly walking the hill by our home without getting wiped out.  In fact she was experiencing some sore muscles in that skinny little body of hers.  For one reason or another she crashed last night just 4 days after the med was done.  This was the same amount of time in the last post that she crashed and went to the hospital for neutropenia.  Yesterday she was feeling sore from all the activity, but her lungs x-ray looked good, and she was given the go-ahead to go on our postponed family trip to Hawaii in 10 days.  As the day wore on, so did she.  She was tired, and then she was exhausted.  At about 1:00 am she spiked a fever of 100.  She continued to try and sleep and at 3:30 am she woke me up with a terrible temp of 105 and a heart rate of 150.  She refused to go the the ER and took Tylenol to break the fever.   A few hours later it broke and she was back to 98 by 6 am.  She was feeling nauseous, and could not get comfortable.  She wanted to wait and just go to the infusion clinic at Huntsman.  After calling them in the morning they deferred to her transplant team.  We ended up in the ER mid-morning.  She was worse this trip with blood pressure of 60/30 putting extra stress on her kidneys with the limited blood flow.  They started fluids to bring the pressure up, all the while putting pressure on the lungs.  She was neutropenic (low white blood cells to fight infection), and her underlying infections went systemic quickly.  She was septic.  They admitted her to ICU and they offered a BiPAP with a face mask to give her some assistance with breathing.  Her ABG (Arterial Blood Gas) CO2 level was 53; Normal is 35-45.  I could see the fear in her eyes.  She was breathing 40-50+ times/minute; her baseline is around 25 right now, and normal is 12-20. Once she was on BiPAP, she was able to get comfortable.  Her x-ray still looks decent, but with really low blood pressure, comes a whole host of issues.  Blood being backed up into the lungs (pulmonary edema), pressure on the lungs, and low kidney function to name a few.  With the fluids, antibiotics, and anti-viral meds, she has improved a lot.  Her HR is back to 100 (her normal), blood pressure 95/53 (better but not normal), respiratory rate down to 25-30, temp is back to 37 C without tylenol, and her ABG CO2 is down to 47.  She is still on BiPAP, and will be through most the night.  She hasn't eaten all day in case they needed to intubate her.  By the looks of it, she won't have to go down that road.  If she can continue to get rid of CO2 and get that number closer to 40 they will be more confident she won't need intubation.

In an effort to get her treated quickly for sepsis, the ER doc prescribed her an antibiotic her Transplant pulmonologist told us to stay away from.  Tobramycin is really tough on the kidneys.  That alone is fine, but with her anti-rejection med Prograf being tough on the kidneys, you don't want to do both, especially with low blood pressure.  I asked him to verify with Dr. Cahill twice...he didn't.  They gave her two bullice doses, only to have Cahill discontinue once she rounded in the ICU.  Another example to stand your ground if you know where your doc stands on something.  I should have refused the med for her.  We were assured that pharmacy deliberated for 30-45 minutes and figured they had decided (with Cahill) that it was worth the risk.  After all it works wonders against the pseudomonas bacteria she has.  We weren't totally against it if they thought it was safe.  Turns out they didn't consult Dr. Cahill...did I mention 3 times....

Julie refused an ABG initially in the ER since she hadn't heard it from a physician that she needed it.  They are uncomfortable to get.  The tech who made the first attempt in the ICU looked up to see how she was doing and proceeded to prod around like an acupuncturist.  True to form Julie asked him to stop and get someone else.  He told her he was confident he could get it...he didn't.  The girl who tried after did so without any issues.  As Julie came to, and her CO2 dropped (be it just 5 or 6 points) she started to ask more questions through her BiPAP mask.  She is sleeping comfortably tonight here in the ICU.  This is another occasion where I am so grateful for those who practice medicine and the facilities available to us.  In many ways it is a selfless service.  I see the hippocratic oath carried out in my wife's behalf.  They put aside personal judgement and objectively care for her.  They never stop investigating on her behalf.  They stay calm under pressure, and roll with questioning.  The best ones, listen to our questions and aren't afraid to acknowledge what they don't know, or have forgotten.  For any folly mentioned here or in previous posts, they always make up for it 100 fold.  My wife is alive, being treated well, and with thoughtful care, and we live in a country where we can still access good healthcare.  I am so grateful.

So what is going on?  Why is it taking so long for her to recover now that the cancer is gone?  Well, she's a bit of a unicorn.  For one, she is fighting pseudomonas infection in her lungs, while being immune suppressed to prevent rejection of those lungs.  Second she is on meds to fight CMV (cousin to Mono) and Aspergillus (fungus) in her lungs that lower her white blood count causing neutropenia from time to time.  If she get's neutropenic, what ever infection or virus that in or around her, can move into her entire body.  This risk is similar to patients undergoing Chemotherapy.   That about sums it up in lay men's terms...sort of.  It is a balancing act of several variables, and truly takes a team effort from the docs and the patient

Pray on, hope on.  It's the best we have.  She was doing well yesterday, so no reason why we couldn't get there again soon.

Wednesday, October 7, 2015

Just Focusing on Breathing

We were back in the ER on Sunday this week.  We haven't been in the hospital since late June.  Chemo and Cancer are in the rear-view mirror so far as we can tell.  Julie has sputtered along through the end of summer trying to recover lung function lost during the last 3 rounds of chemo.  It has been one thing after another.  We found that the abscess in her spleen wasn't anything other than dead infection most likely from the fungal infection she caught during chemo.  Once she was done with the C-diff, she had a few weeks before she caught a lung infection.  With her sinuses still having Cystic Fibrosis, anytime her immune system is weakened, the underlying pseudomonas can spread to the lungs.  She picked up CMV (similar to mono) and just as she got better from another lung infection, it seemed to return a few weeks later.  She was having limited response to the very stringent anti-biotics they were giving her.  She was wondering if she had built up resistance to them.

About 2 weeks ago we found out through a CT scan and some blood work that the fungus that she caught during chemo had come back.  There had been no detectable levels in the blood a few months ago, so we stopped the treatment.  After getting on an anti fungal med, it seemed that she had turned the corner and started to improve.  We had a great week of lower oxygen dependence and better energy to spend time with the kids.  Last week however, she took a turn south.  Her oxygen dependence started to climb and over Friday night and Saturday night her underlying nausea she had experiencing for a few days turned to vomit.  Reading this while eating?  Deal with it ;).  When we took her temperature Sunday morning it was 38.5 C or 101 F.  Once we called the transplant nurse she told us we should come in.  We arrived to the hospital late afternoon and spent 4-5 hours in the ER waiting for a room and getting things ready.  I accessed her port and she slept most of the time.  They were able to give her IV zofran for nausea and she was able to rest.  That evening were moved to a room on the floor.  She had a very high resting heart rate at about 135 and was on 5 liters of oxygen.   It was a good thing we came in.  Her white blood cell and all blood counts were dropping.  Red blood cells carry the oxygen, and with lower counts the heart has to beat faster to deliver the gas.  In addition, her platelets were low so thinner blood takes more pumping to circulate.  On top of all this, just low volume of blood will make the heart work harder.  She was able to get a unit of blood, but as always it took much longer than it should have.  We knew they were dropping the night before so they did a type and match to verify her blood type.  The attending physician came in Monday morning and said that it was in the process but we found out later that day that the blood was never ordered by him.  Finally around 5:00 pm she received her unit of blood.  She had been delirious that day and nauseous.

Tuesday she was very tired and she threw up twice Monday night.  She slept most of the day but had better color.  The anti nausea meds make her sleepy but took the edge off the nausea.  They did another CT scan since her lymphnodes were very swollen.  The result was there was just fluid in them from all the fluids she is receiving.  It probably is also her body clearing the infections out with the anti fungal and antibiotics she is on.  Last night (Tuesday) she slept better.  When I saw her today she was much more interactive and in addition to just walking around the floor of the unit, she asked to go on a walk through the hospital.  We walked to the front of the hospital and sat in the wheelchairs out front since that's all there was available.  She then said, lets walk up to Huntsman.  I was surprised by her suggestion.  She was sitting on 5 liters of oxygen.  The University of Utah Hospital sits up on the bench and Huntsman Cancer institute is built on the mountain right behind it.  It is a steep climb for anyone let alone someone with sub 20% lung function.  I sensed that Julie had some of her grit back.  It reminded me of her first week after transplant when she was working so hard to recover and asking when she could start running.  We hit the hill behind the ER entrance and she was so strong.  I was bragging to the RT when we returned and of course Julie down played it by saying she needed to rest a few times.

The past few weeks have been demoralizing to Julie to say the least.  She wasn't seeing any improvement.  Two weeks of antibiotics only to need them three weeks later.  Another two weeks with nominal results.  And then the week before last seeing some improvement before this crash on Sunday.  Before she went into the hospital on Sunday she said "I'm hopeful."  "That you'll get better?" I said.  "I'm hopeful I won't die."  The fact that she was thinking about dying was telling how tired her body is.  This is a girl who has simply refused to die time after time.  She and I were both grateful to have the hospital to go to this weekend.  We weren't stoked about going up, but we were grateful once we were there.  No one likes the hospital, but you sure are glad to have it, along with talented clinicians when you need them.  On Monday, one of the few conversations we had, she whispered "Maybe I just need to change my attitude."  She is mentally depleted.  Seeing her walk and push herself today was evidence she is doing just that.  I wouldn't have guessed she would have done that two days ago.

We aren't naive to the weighty matter at hand.  With low lung function, we are on a knifes edge of the end of life.  Just one bad virus or cold can cause her to crash. That said, we are at peace with what is going on and the unknown outcome ahead.  Today I said to her "I'm so sorry you have to go through this."  She looked up at me on our walk and said with a smile of contentment, "It's okay."  I have really faced the facts lately of the possibility of losing her.  Ironically I have been at peace with what is going on.  Numbness ensues to the emotion that stress brings initially.  Though numbness connotes a disconnect of oneself, in fact it is acceptance instead.  People often say 'this is your new normal,' and that is exactly what it is.  Normal.

We have had a great few months.  On a whim at the last minute we rented an RV to go to Yellowstone in late August.  I have always been against this type of parking...I mean camping, but in an effort to include Julie on our trip, I bit the bullet.  It was a lot of fun, and honestly I kind of get why people do it now.  When you want to leave, you leave.  No packing, just chuck the stuff above the cab and buckle up!  Julie was able to walk around and see some of the sites with her boys.
We also were able to spend the weekend in Deer Valley with her family over Labor day weekend.  Most of that weekend was in the hotel for her, but it was nice to be together.  And last weekend despite how she felt, she mustered up the strength to spend the night with us at Park City's Mountainside Marriott.  It was a lot of fun for all of us.

We are still very hopeful that Julie will conquer yet another challenge.  The dark moments described above are inevitable in our search for hope.  We choose to recognize them, but not dwell on them.  We have had some of the sweetest moments in our marriage and family life these last few months with a certain feeling in our home that we cannot deny.  We are so grateful for these feelings and experiences and wouldn't trade them for the world.  We have grown closer together as we sacrifice for one another.  We have also had some colorful moments of stress...mostly coming from me.  Even when she is just focusing on breathing she remains calm.  She doesn't let me get away with my complaining for very long, and ultimately if I am upset, it is usually that I am mad at myself for being mad at the girl who can't breathe.  Who gets mad at a girl who can't breathe?  Well, I do sometimes.  She is still my wife, and we have a very normal marriage despite all the noise around us.  I do a decent job of destroying all those nice butterfly feelings I just talked about.

This experience has no doubt stretched me to carry some unthinkable amounts of stress that I never thought possible.  But that is what is incredible; with practice, and introspection (in my case prayer), we can get through anything life throws at us.  We can carry crazy amounts of stress and function very highly.  My habits around prayer frequency have really only increased a little bit.  Where things have changed is my focus when I pray.  The focus and deliberate approach to what I do each day is more thought out and planned.  I don't have time to do otherwise.  Julie on the other hand has told me the frequency of her prayers has increased.  She prayed regularly before, but I will often find her on her knees pleading with the Lord for direction.  I honestly don't know how we would do this without God and prayer.  As dark and as gnarly as this has been, there are moments that we have where we know He is there.  I am also empathetic to how this could completely shake someones faith in a higher power.  I have felt those feelings of wonder and doubt myself.  They are there because we are human.  But those feelings of doubt are hopeless, and binding.  They do not liberate your spirit the way hope does.  My Grandpa Layton reinforced an attitude that felt natural to me as a boy to "Be Positive."  Some might think that faith and hope are blind to the facts.  I believe they are an embrace of the evidence.  Facts are subjective to the understanding of the evidence in front of you.  There is so much we don't know about Julie's situation, and several "facts" end up being incorrect because they are mere assumptions presented as facts.  We can't live our lives around stats and facts; it can be hopeless.  It is limiting, and depressing.  Facts are there to inform, not to dictate.  Most of the time the toughest decisions require an act of faith.  If I had lived around facts and not hope, I wouldn't be married to my bride, I wouldn't have my twins, I wouldn't have Charles, I wouldn't have Julie alive today surviving CF, Diabetes, Transplant, Childbirth post transplant with stage 4 endometriosis, and an aggressive, rare Lymphoma.  Life is too short to doubt, especially your own impressions and experiences.  Hope on, and believe there is a higher power out there that is greater than man.  It's provided me a pretty stellar life despite the circumstances.