Monday, February 29, 2016

Beginning of the End

Last night after I left to be with my boys, things changed quickly.  Julie wanted to go to the bathroom on the commode and with some help got up.  She desaturated quite a bit down into the low 80's.  The RT came in the room and worked with settings on the BiPAP to give her some relief while she sat there.  They weren't having any luck.  He gave her 100% oxygen and increased her peak pressures among other things and she wasn't recovering.  She told her mom, "I think the end is near."  About a half hour later, she told her to have me come up because she was getting scared.  I was out for ice cream with the boys to talk to them, and more importantly get them to talk if they wanted to.  Ben was very matter of fact about everything.  He has adapted pretty easily to the roller coaster.  After he told me he thought she would die I asked them what they thought about that.  "I think it's good because she will be able to see her best friend James."  Jack was quiet and just said "I don't want her to die."  I talked to them more about how she is having a really hard time breathing and that when the time comes, she won't feel that any more; she will be able to breathe freely.  Jack listened and ate his ice cream.  They still transition to the silliness of a 7 year old during these conversations, so right now it does not consume them as it might an older kid.  I just want them to feel open to talking to me about it.

When I got home, I started the routine of getting them ready for bed.  The boys were happy as ever playing in the house with their little brother.  I had plugged my phone into my computer so I didn't see the last text that she wanted me up there.  I finally saw this text sent 30 minutes before. "She just told Dr. Beck she wanted to pass so Julie wants to talk to you about a couple things."  I kicked it into high gear and called Linda.  I gathered the boys and told them "Get your shoes on."  'Why?'  "It's time.  I think your mom is ready to leave."  They both welled up and came to me and hugged me.  Ben adapted quickly and started talking about James and her reunion.  Jack was sullen.  I think he really likes to have control of what is going on that he is reacting this way.  Ben conforms to Jack most of the time so that might be why he is adapting on the surface.  Driving up, it took everything out of me to keep it together.  I couldn't help but think of these little boys hearts.  We talked about the afterlife, resurrection and they asked some questions about mom's body when she was resurrected.  I explained to them that we will be restored to a perfect and proper frame.  Everything will be restored.  Jack asked me if she would get her old lungs back.  "Yes, but they will work just like yours and mine do.  She will be young."  I could tell they were getting done with the conversation so I asked them if they wanted to stop talking and listen to some music.  They both said yes and Ben suggested his favorite song "Radioactive."  I cranked it up for them.

When I got here I had a lot of questions for Julie, her parents and the doctors.  I was really confused and wanted to know the timeline of events.  I asked about her pressures they tried to deliver, the conversations, and I was asking Julie a lot of questions.  Initially I told her we can't quit.  She said to me, "I'm not quitting, I'm just wiped out....I don't want to leave, my body is just done."  As always she had pushed the envelope.  I quickly listened to her once she told me this and then I started to just talk to her about how she came to that conclusion.  I was comforted to find out that she had made the decision before she had talked to the doctor.  Once the doctor came in she told Julie that we were headed back towards intubation and recounted what that looked like.  Julie said she did not want to go back on the tube.   That could have lasted months.  She asked her what she had asked me on paper the day before: "How do I die comfortably?"  They explained that she would have oxycodone for pain, ativan, for anxiety (both drugs she was on low doses of since chemo), and of course morphine when she was air hungry.  They had withdrawn all meds but these when I arrived.  Julie was tired but was chatting with us.

When I walked in with the boys I anticipated she would be a wreck...she was cool as a cucumber, just like the night she was called for transplant.  She was beaming when Charles sat on her bed and said "I lob u Mom" and "Pitty Mom."  We told him to give her kisses and she was elated.  She was tired but she was very happy.  Jack wasn't too comfortable even hugging his mom.  When we took a picture he leaned away from her.  I was asking him to hold her hand or hug her and Julie just gave me this look like, 'Leave him alone.  He is fine.'  She swatted her hand at me in a gesture to say 'It's fine, and I'm fine.  Let him be.'  So I did.  I couldn't believe that even as her son was separating himself from the situation, she had the fortitude to not take it personally, and to put his feelings before hers.  She really didn't even care, because she knows how Jack really feels.  Julie put people and their feelings before herself.  Ben was a little more willing to be with her and to lay in her arms.  She told her mom later, "I was asleep, and then all of a sudden I woke up and we were all together!"  When she and I were alone, I said to her, "You have to help me with these boys.  I don't,,,I don't know what to do to help them get through this."  She looked with a grimmaced faced and said "You know what to do."  It was her way of saying, quit it.  I was once again put in my place.  She is right.  I have known what to do.  We have been doing it all along together, talking and listening to them about this life and how it interlinks with the next life.

The boys left and we spent much of the night standing by her side waiting for her to pass.  It occurred to me very early on that she was not going to die that night, but I didn't want to be the crazy husband in the room holding on to a dream.  About midnight I sent a text to Rick that I didn't think she was going to die that night.  Once it was clear to everyone that she was pretty stable on 6 liters of oxygen, they went home.  It wasn't till 3:30 am that I went to bed.  I had spent the first part of the night laying next to her at her request in case she became short of breath.  At 3:30 I decided to give her a blessing and release her from this world.  I had the distinct impression it wasn't time yet but it was a good exercise for me to hear myself letting go.  I went and crashed on the pull out chair till 6:30 am.  I woke up to a dream that I was having a BBQ and someone had a terrible cackle in the other room.  I was sshhhing them in my sleep.  Turns out it was someone in the unit talking and laughing outside my door.  What a moron.

Two things happened the last 24 hours that I think are typical of Julie.  First I had been wearing a mask because I had sneezed yesterday.  I didn't have one on when I was there with the boys and she asked me to put one on.  I double checked that she knew she had decided to go.  She said "I know, but it just shows me you still care about me."  Once again, she put me in my place, but it was so endearing.  Secondly, she asked for insulin the night before.  She started to wake up when everyone was there.  She looked at the counter and saw an apple I had bought in the cafeteria earlier that day.  I never buy an apple but I just decided to that day.  She squinted her eyes at it and said, "Is that an apple?"  We asked her if she wanted it and she said "Yeah."  She sank her teeth into this juicy red delicious apple like it was the forbidden fruit.  It made us all want an apple as we watched her enjoy the first real food since she was admitted 10 days ago.  BJ Butler mentioned he brought her cookies but wasnt sure it was approp..."You have cookies?"  We went and got her some cookies and she sank her teeth into those as well.  She was loving life.  Her family was there, Her boys were there, and she was eating some great food.  What else do you need in life??

I looked at my phone when I woke up and noticed that no one really knew what had happened.  I hadn't posted anything other than a very optimistic post, and I hadn't reached out to anyone other than my parents.  I knew I had just a few hours before the Julie circus arrived.  I started this post then.  Now it is midnight and I am finishing it up.  When she woke in the morning she knew she had a low blood sugar and asked for orange juice.  Her stomach was growling all morning.  I fed her a piece of pancake, yogurt, water, ensure, and a piece of cookie.  If she was more awake she would have kept eating.

I spoke to Julie's Lung Transplant doctor who came up to see her.  I told her how indebted I was to her, Dr's Liou, Raman, Carveth and the entire pulmonary team for keeping my wife here.  The entire team supported us as did the respiratory and nursing teams.  Julie and I had 10 years together.  Four of them were in complete bliss, free of lung illness, and the rigor that is associated with it.  We had vacations, barbecues, camping, bickering, planning for the future and just a completely normal life without any hospital visits.  Dr. Cahill is typically all business, but Julie loved her.  She knew she really cared about her.  She would put notes on her phone before her clinic visits so she wouldn't forget anything and be able to be succinct.  She deeply respected her doctors time.  I wasn't as good at this as she was.  She told me with tears in her eyes "It was an honor to care for Julie."  I told her Julie always knew she would support her.  She said, "If I gave her an inch, she'd always go a mile."  She and I were really crying together for a woman who had impressed our lives so much.  I am forever indebted to her for believing in Julie and supporting her in her "calling" to have a child.  Julie risked everything she was given to have a baby.  She saw this as the ultimate sacrifice.  It was why she was here.  As a close friend told me tonight, nothing could change her mind; fear, others opinions, worry etc.  She saw the big eternal picture. "She knew what her purpose was and she bravely and faithfully chose that road fully aware of what she was risking."

All of my siblings who hadn't come up yet were able to.  All of them were able to get to talk to Julie this week.  Many of Julies friends were able to come up and talk to her.  Each person really got some feedback from her.  We had one friend calling other friends and putting them on speaker.  I was astounded that she would wake up and say hi to them.  She was probably 50% worse late that morning.  She would be consoling people and saying "Awwwe, you're so sweet."  She told her Aunt Karen, "You're a really good person."  As the day wore on I needed to know that we were doing what she wanted.  The doctor suggested we remove the oxygen cannula.  She reiterated that Julie came to them and was very comfortable with her decision.  I had to ask myself what she was implying.  Were we prolonging what she wanted to have happen comfortably?  This was by far the hardest part of the day.  This doctor went in and spoke to her parents.  Before I knew it, she was off the oxygen.  I am so conditioned to help my wife when she is in need that watching her lips go blue and her fingertips as well was heartbreaking.  I asked Linda if this was really what we should be doing.  She assured me that it was.  I completely broke my heart to watch her.  They had given morphine to take away the sensation of being air hungry.  She took long breaths with pauses between for nearly an hour.  As I watched and as the color came out of her, I began to come to terms, (finally) that this was it.  She had spent the night doing relatively fine, but increasing in CO2.  Her mind was made up but her body was not.  During that last hour, her heart just kept going, and going, despite being starved of oxygen.  She slowly stopped breathing.  We kept thinking it was her last breath, and then she would breathe.  It was slow, but it was very comfortable.  She looked like she was in a deep sleep.  I had my eyes focused on the throat where I could see her little engine that could heartbeat.  It just kept going even when she was in between breaths that were spread by seconds.  The hour seemed to crawl, and when it was over, I thought it had been 20 minutes.  Julie passed away at 3:01 on February 28, just 3 days shy of her 36th birthday.  She couldn't wait 9 more hours so we could celebrate every 4 years.  March 5 will be her 5 year anniversary for her transplant.

Things did not turn out the way I had hoped for but then again I knew I would be surprised either way.  I know I have egg on my face, and it tastes delicious.  We were at peace all the way through.  We didn't spend this last year with Julie fretting.  Even if things aren't going to get better, that is no place to live.  We always hoped it would get better and I will never regret that deliberate decision.  We were able to enjoy each other rather than stress about if she were to die what we would do.  We would talk about what that might be like, but we planned for a future together.  I am not let down because I gave it my all.  Julie gave it her all.  I have no regrets of hoping for the impossible.  I have no regrets for praying for what seems futile.  We were happy, and we are closer to our Lord and each other because of it.  It does not change my faith.  It substantiates it.  The fact that I was able to handle today is evidence enough that there is a loving God looking out for me and my little family.  This is no doubt a result of the hundreds if not thousands of prayers that have been said on our behalf.  Last week she had so many pulling for a miracle.  We got it.  Julie was able to decide what she was going to do.  I can live with that for the rest of my life.  She was in the drivers seat with the Lord as her co-pilot.  I was torn up about making decisions I wasn't sure she would agree with.  She assured me that "I think we made the right decision" to intubate, and later, "It's time for me to go."

The funeral is slated to be this Friday March 4, at the LDS Chapel on Gregson and Kenwood St. in Salt Lake City, UT.  LDS Chapel Gregson/Kenwood  There will be a viewing the night before Thursday March 3, from 6:00-8:00, and again on Friday March 4 from 9:30 to 10:30 with funeral services from 11:00 to 12:00 and the burial service at Wasatch Lawn just after 12:00.

Please don't feel you are imposing if you do not know me but know Julie or vice versa.  There are two things you don't want to miss in life and funerals are one of them.  This will be a great day where we will enjoy telling stories about a woman who has affected us all in such a profound way.  Sidebar; many of the stories will be entertaining and humorous as you mingle with those who know Julie.  It will be a day of love and celebration.  Thank you for everything, and God Speed.


Saturday, February 27, 2016

Waking up

When Julie started to wake up from sedatives a few days ago I was faced with happiness and guilt at the same time.  I had spent Sunday and Monday preparing for the worst; my hoping for the best was waning.  I was in a split frame of mind nonetheless.  I had sat my family down including my siblings and their children and explained the gravity of the situation.  I really didn't know how things would turn out and I still don't.  Inevitably the negative thoughts can overshadow the positive ones.  It wasn't until Tuesday night when I spoke to my mom about faith that I was reinvigorated in my resolve that anything, truly anything is possible.  I recounted my resolve in November when Julie was admitted and intubation looked very possible.  In the last paragraph of Moving Along we were faced with grim possibilities, but were able to hope for the best and deal with what came.  We took the facts at face value and focused on what we hoped for.  It seemed to work.

It has become increasingly clear to me that as we all are faced with undeniably tough decisions and unknowns in our lives, we tend to focus too much on preparing for the worst.  As I have been let down many times, I have developed an opinion that this is wasted energy.  While I still think it is important hear and understand the negative possibilities or prognoses, dwelling on them and focusing on them has brought no added value when when they have come to pass.  It has brought anxiety, worry, fear, and other thought processes that can inhibit my ability to function in other aspects of my life.  I haven't found a way to prepare for a negative outcome by "facing the facts" in a way that makes it any easier if it happens.  I don't intend to keep trying.  Julie was told she may not be able to come off oxygen.  So far, they are correct, and this may ultimately be the case; but we will still push for that ceiling of coming off oxygen and being able to travel with her family and work out again.  Julie loved to go to Zumba classes, and most of all she loves trips.  This ideal is a target for us to push the envelope for.

The day after Julie was intubated, she was writing squiggles and communicating.  Her PaCO2 had dropped from 106 to 61; pH was in the normal range.  That afternoon it came up to 74 and later came back to 63 on Wednesday.  The next one we did she was back up to 73 and her pH had dropped below 7.3 again.  Ideal is between 7.5 and 8.5.  Even though these numbers were jumping around, her needed pressure to ventilate was dropping.  We started with a peak pressures in the mid 40 cmH2O range.  By today she was down to pressures around the mid 20's to ventilate.  Her ICU doc told her on Thursday that he was really impressed we didn't ever need higher pressures to ventilate.  She never needed more than 40% oxygen and she may have received too much initially.  Her PaO2 was double what it should have been by the middle of the week.  The team here has been so thorough and preemptive in their approach.  Watching her kidney function they dropped our beloved Tobramycin and replaced it for Cipro.  While her Prograf levels were coming down they restarted them at more than half the dose so she wouldn't bottom out and have further rejection of her lung graft.  They have been attentive and caring to Julie.  Always looking at the next best step.

Julie was standing on Wednesday and Thursday and on Friday she walked about 15-20 feet with the tube in her mouth.  As the week progressed, she started to ask those with a cough or sneeze to put a mask on even though it isn't policy.  She started to get more involved in her care despite being exhausted.  I knew we were on the right track. When she really woke up and was able to write on Wednesday, I asked her some questions.  I knew for a very long time that she didn't want to get intubated.  It wasn't until recently that she considered it.  I told her about the predicament we had been in Saturday and Sunday.  When we asked her she would always say yes most likely because she was air hungry.  I knew that with a CO2 in the 80's it wasn't likely she knew what she was saying.  When telling her about the conversation we had with the Dr. to intubate, I told her I really didn't know what to do.  We probably asked her 25 times if she wanted to do it.  She wrote down two things during this conversation: "I'm sorry"  (When I teared up from stress for one of the first times) and  "I think we made the right decision."  I was so relieved.  Her life had been in my hands and no matter how many times we have talked about this, it was the most difficult decision I could have made.  I knew we were on a time clock.  To be frank, she was lucky to have made it through the procedure.  I asked her if things were to get worse what we should do.  She gave me a look that took no words to describe.  "Am I stressing you out?"  I received a nod yes.  Obviously this is not something anyone wants to talk about, but it is something I want to know when she is in a coherent state of mind.

On about Thursday Julie started to ask 'how much longer?' on with her pen and paper.  She wanted ice chips or something to soothe her mouth and throat.  Tey could only swab with sponges filled with water.  She was visibly getting more and more uncomfortable.  This was to be expected since they reduce the sedatives as aggressively as they can.  Otherwise she could become dependent on the ventilator and stop working her diaphragm muscles.  We had to continue to encourage her to push through the discomfort.  Each time she had a treatment from respiratory therapy, they would push a tube into her lungs and suction out what they could.  This was extremely uncomfortable.  Last night she wrote me the following; "How do I die comfortably?"  She had had a really effective treatment bringing up a lot of the mucus that night.  So much so that it came past the cuff and out her mouth.  She wrote me "I really thought I was going to die tonight."  I had to explain to her that when she is intubated, it was near impossible she would die.  It would make her breathe even if she wasn't coughing.  This is why people can stay on a vent for a very long time.

Yesterday for about 3 hours and again today for about 6 they tried her on a spontaneous mode where she was doing the work to breathe and the machine wasn't assisting.  Initially it was hard to do but she would get used to it.  Her asking about getting the tube out became regular.  They wanted to see what the blood gas was when she was ventilating on her own.  They were impressed with how well she did.  However the CO2 came back at 73 and the pH was under 7.3.  We went back to control mode where the machine helps finish the breaths and gives a breath if she doesn't take it.  In the morning she did this again,  Two things happened; CO2 stayed the same at 74 but the pH came back up to the low 7.3 range just below normal.  Having her pH out of normal range is what causes other cells and organs to stop working correctly.  They came in and spoke with her about a plan.  They were on the fence since the CO2 hadn't dropped.  They knew she wanted to come off.  They first told her what they hoped to accomplish if she chose to be extubated; to come off to BiPAP and hopefully continue to ventilate and bring her CO2 down and pH up.  She would then be moved to Optiflow nasal cannula, and finally nasal cannula before leaving.  They talked to us about the risk if they weren't able to drop her CO2; that she may need to be re-intubated and have a tracheostomy.  That would result in being on the vent for a very long time, possibly months.  This was the road I was really unsure about where she stood.  The doctor said, "You've been dealt a really tough hand.  You have permanent lung damage."  This is where we are a little nuts.  We still know that she has lung damage, but her lungs are from someone else.  They have a different DNA structure reconstructing them.  We are walking on a dream, but its worth living for.  Who is to say it is permanent?  After all her transplant doc told her a while back "Don't listen to me. Just keep trying and pushing and living."  She knew there were risks to coming off the vent, but she followed up with "I think I need to try; Let's take it out."

So now I am watching her rest, really rest like she does at home.  Her respiratory rate is near normal in the mid teens, her heart rate is hovering around 85-90 which is the lowest it has been since the summer, and she is oxygenating well.  Her tidal volumes live in the 500 range (they were in the 300 range when on spontaneous mode on the vent).  It is very early, but she is headed in the right direction.  She has been on nasal cannula a few times and does okay for about 3-5 minutes, but she clearly needs the ventilatory support from BiPAP for a little longer.  We are still on a bit of a time clock, but not one that seems so eminent.  She knows the negative possibility, but just as with having a baby, she at least wanted to try.

We saw traffic on this blog explode last week.  There were 23000 page views in the last month.  The most we had in the past was about 16000 the week she had her transplant.  Typical traffic through chemo was 2000-6000 views/month.  There is no doubt that the countless prayers, fasting, thoughts, mojo, and positive energy pulled Julie through this week.  As far as I'm concerned, this is all from one source in God.  The incredible staff and attention they have given my wife has created an environment for her moxy to blossom along with this faith.  As you can see we are far from done.  No one is ever done.  A lot of people want to be released of this and hope she can live a good life or that we can accept if the time has come for her to leave.  One thing was made clear to me this week: Two people are in control here and I am not one of them; Julie and her Heavenly Father.  Many of us thought it would be tough for her to pull out of this.  She has chosen to fight though.  WE should support her.  No matter how long it takes.  She is pushing the odds against her...and today, she won.  On to tomorrow.  It is another day.  God Speed everyone.  My gratitude overflows.

Tuesday, February 23, 2016

We have one week

Sunday morning I was confronted with a situation that set me off.  During the night Julie had said to me, "If I don't get on IV antibiotics I am going to die."  She isn't one to cause a lot of drama, or be dramatic.  In other words, she meant every word.  She was bringing up bucket fulls of sputum.  After shift change the RT came in to tell me her supervisor told her they cannot deliver the inhaled antibiotic Cayston since it is from our home and on a device they weren't trained on.  This is all very accurate to hospital policy.  There are risks in using something you haven't been trained on.  HOWEVER, we had received 3 treatments the day before.  I told her to consider for a moment what the implications are of the said policy in this situation.  "You have a patient, who is compromised, whose only defense is a medicine you are not willing to deliver.  As far as I am concerned, following this policy in this case is unethical."  I was already on edge that Julie wasn't getting IV antibiotics and here was an individual who was not going to deliver a medicine because it was against policy; despite the fact they had already set a precedent.  I told her "You are an RT.  This is your MO to figure things out and come up with a solution.  Lets figure it out together.  We both have phones, and we have Google, I am sure we can come up with a solution to get her the medicine she needs.  (I had learned to put it together the day before while I showed the RT's how to use it).  Another nurse walked in and asked how Julie was doing.  "Like Shit.  I need to see the attending doctor!"  I was at the end of my rope.  The beauty of some doctors and clinicians who have years of experience is their ability to bring down a situation and come up with a solution.  Dr. Carveth has this uncanny ability.  She explained to me that the family agreed to do the sterilization of the nebulizer.  This was the first I had heard of  this situation.  The RT's the prior day had done everything.  So we turned to the RT and asked if she could deliver the med.  She offered to use one of their products but this medicine has a proprietary neb device and cup that it has to be used with.  In the end, I delivered the medicine.

Dr. Carveth saw how terrible Julie was and how thick her secretions were and started the ball on getting her IV antibiotics.  By the middle of the day she had her first dose of Meropenem.  She wasn't very responsive all day.  Toward the afternoon, her family started to show up.  She acknowledged them when they came in.  Linda asked if we were being too loud and she said no.  She was just glad her family was there.  She spent the time resting.  Toward 4:00 pm, they decided to move her into the medical ICU.  This was probably a bit overdue so we were grateful and apprehensive we had to go to a higher acuity unit.  We were pleasantly surprised that the attending doc who admitted her was the same ICU doc who took care of her in November.  Dr. John Boltax is highly intelligent and has great bedside manner.  He involves us in rounds or finds us when we miss them.  He is the whole package.  He immediately spoke to Julie while we were waiting outside about intubation.  He asked her if need be would she liked to be intubated.  She nodded yes.  Julie and her brother James have always been leary of intubation and James never was intubated toward the end of his life.  He had more issues with his heart on top of his CF.  Julie on the other hand has changed her stance on it in recent years.  She understood it as the last effort to get better.  She was so air hungry yesterday that I don't blame her for answering the way she did.  He talked to her about being able to suction, and give her a break from the work she was doing to breathe.  She obliged.  When he sat down with me, we had to talk about how that looked....end of life decisions.  He explained that we need to mentally go down that road and talk about what she and I would want now while she had enough ability to weigh in on things.  When he asked me and Linda if she would want to be placed into a long term care center, be both quickly said no.  He explained that if we get to a week on the ventilator and she hasn't improved enough to come off, the likelihood of her coming off was extremely low given her lung function adn disease state.  About two months ago Julie told me she didn't want me to be mad if she let go were she to get really sick like she was in November.  All the support we get on social media, and at home has really carried us through a sea of uncertainty and despair.  I think it also has it's pressure on her to get better.  This can be good in someways, if she can really get better.  In other ways, she feels like she is letting me and so many others down if she does not.  I explained to her that she has lifted so many people because she has pushed through so much. 

Dr. Boltax told Linda and I that he saw a very good chance that Julie would need to be intubated.  If she was, we had a 5-7 day window to get her improved enough to come off the vent.  He also explained they were going to try and avoid intubation as much as they could.  Delaying too much would actually put her at more risk.  His hard stops for intubation were her inability to cough up sputum, and PaCO2 levels trending upwards of 90.  If we get to those points and we delay intubation too much, it can be difficult to make it through the procedure.  She also needs reserves to fight while on the vent.  He was very candid about the low likelihood she would come off despite the constant goal to get her off the vent once she was on.  I expressed to him my desire to keep her off the vent as much as we could safely do, but that I also was on board with his parameters to put her on.  

When she was admitted to ICU that night her PaCO2 was 78.  Dr. Boltax made some early adjustments to her BiPAP and she dropped to 74.  This is the level of retained CO2 in the blood; Normal is 35-40.  It was during this time that Julie was much more interactive.  Her dad was able to give her a priesthood blessing by the laying on of hands as described in James 14:16.  It was a beautiful blessing.  He helped her realize the massive impact she had had on us and so many others, that she had fulfilled so much.  That if she is to go that her brother, and grandparents would be so happy to see her and she them.  That she would continue to experience her husband and children as they grow.  He also helped her see that if she were to be able to make it, that she would be able to have a life that she could enjoy, with her children and husband.  This was everything she and we needed to hear.  If she goes, it is time, and she will be ready; if she stays, that she will improve and have relief.  Shortly after she asked if I would like a blessing.  It was so sweet that she was thinking of me, that she had the ability to suggest this.  We spent that evening with her interacting with us on a limited but real way.  She knew everything she was saying.  She was truly there after being so out of it for a few days.  

I found myself driving to my parents that night to update my family and extended family, contemplating a funeral, death of my sweet wife, and what life might be like after with the boys she has brought me.  I have thought about this throughout my marriage, and certainly more in the last year so I have had time to process it.  This time was different though, it was profound.  I was moved to tears for one of the first times in a while that this may be her final act.  Not because of how it would affect me, but because she is such a stellar person to know and be married to.  I was on a 50/50 track.  I really had no idea how it would turn out and I still don't.  What is wild is the peace I feel with either outcome.  Selfishly I want her to stay, but hearing Rick's blessing, helps me see what everyone else sees; this is no way to live.  I have always known that and have never wanted to push her to live and be miserable, but I have seen so many incremental improvements and hurdles crossed that it is so easy to believe she will do it again.  Like I told my family, I will be surprised if she makes it, and I will be surprised if she doesn't.  If she makes it and is happy with her lifestyle, I could do this for 50 more years and not think twice.  I am so used to it and so are our kids that I'd do it for her in a heartbeat.  The boys were composed, matter of fact, and are young enough that the emotion is limited.  No doubt it is affecting them and should she die, they will mourn.  But I must say, my boys are handling this so well.  They were some of the most composed in the room last night.  The conversation I was having was one that we have had hypothetically on a regular basis their whole lives.  We have always talked about eternal life, the other side, family members deceased who are helping us, and most importantly the Atonement of Jesus Christ.  They understand the reality that because He was resurrected, never to die again in flesh and bone, everyone on earth will live again with a perfectly healthy body.  We have always addressed the elephant in the room that sometimes people die young "like mom" and sometimes people die old "like Grandma Mona".  They fill in the sentence, they know the plan.  To finish, despite the fact that they may grow up without a mom here with them, they will be forever impacted by her, and better men for it.  They are stable children because they have had to be.  Julie and her love and fight will shape them as it has their first 7 years to handle all kinds of challenges through each stage of their lives.  Having these boys was the best decision of our married lives.  I am so glad we gave the bird to the odds.

When I came back to sleep in the room with Jules, I felt guilty that I had mentally gone down the road of death.  She was responsive.  She was asking for cold towels for her feet.  She remembered that the nurse had only given her 2 enzyme pills and she needed 3 more.  This is a woman who had such high PaCO2 that she shouldn't have been able to do more than just nod yes or no.  In a weird way it is hilarious that she is still on top of her care at the end of her rope.  She also told one of her friends that she needed 6 units of insulin based on her blood sugar.  In the ICU, it is difficult to have control of your blood sugars since they are so risk averse to hypoglycemia.  Gotta love Jules.  

Sunday night she was still pretty interactive.  I helped her get up and go to the bathroom which was good.  However because she had a poor nights sleep, she was out from about 6:00 am on.  She didn't cough all day.  Her PaCO2 from Sunday night went from 74 to 84, 81 in the middle of the night, to 84 during the day.  At first glance it looked stable.  Dr. Boltax came in late in the day and made some more adjustments to her BiPAP.  It was an effort to ventilate her and bring her down.  I left to go be with my boys and put them to bed so they could have some normalcy.  I was only home for 30-45 min when I got a text that it had gone up to 91.  That was it.  I knew I'd get a call shortly thereafter to come back.  My mom had been home for about 20 minutes and I had to have her come right back to my house.  Julie was intubated Monday night at around 8:45.  The fellow did it and he was wonderful.  Her heart rate and other vitals were very stable.  We talked to her beforehand and she nodded that she knew what we were doing. 

Shortly after she had the tube, I noticed there wasn't a heater on the vent but an HME.  I started to inquire.  The company I work for innovated the first humidifiers for medical gasses.  This is all we do and is our core business.  I knew this is a standard of care and was quickly asking questions.  I pulled up the National Institute of Health guidelines for ventilation and confirmed that active humidification should "always" be used in Medical ICU situations.  It was a bit intense for a moment.  I had one nurse see that I had silenced the IV pump and she poked her head in to tell me not to touch the equipment.  I snapped back "I just silenced it.  The Toby is done."  She replied again and was pretty intense, so I snapped "Go get me a Heater!!"  This was the first time I had anxiety.  I was composed in asking for a heater for the vent, but when I was confronted on an issue that was so mundane, my apprehension that Julie was getting cold dry gas pumped in and out of her lungs was infuriating.  The reason it is so vital to deliver 100% humidity fully saturated 37 degree air is that is the natural condition of the lungs.  When you offer any ventilation but invasive nonetheless, you are bypassing the body's natural humidifier in the upper airway.  This thickens the secretions and inhibits the body's ability to move them out.  It also is a major contributor to Ventilator Associated Pneumonia or VAP.  We already have a pneumonia, we don't need more of it.  Physiologically, we are very efficient at heating and humidifying air, but not with our head out of a car window.  Shortly after the RT brought it in with another ventilator.  It wasn't my company's heater, but I didn't care for the time being.  I knew it was much better than the HME.  He was bothered and I heard him say "Serious, does he think we don't know how to do our jobs?"  to our nurse.  He was quiet when he came in.  I wanted to show my appreciation because I was very relieved they were able to deliver.  I had been on the phone with my local rep and he was ready to bring me equipment to use.  I thanked the RT and he obliged.  I told him I wanted him to pass something along.  "I know that was a little intense, and probably annoying that I was harping on this.  I am sorry.  I just have 7 days to get this right, and I only have one shot at it.  I want to give her the best chance to get off the vent and go home.  I also want you to pass something along for me.  (tears)  Julie and I are forever indebted to you and the rest of this respiratory staff.  you have carried her through some gnarly times for nearly 2 decades.  Please let them know that we are forever grateful for you and for your department.  I know we can be annoying and demand a lot when things don't seem right, but don't associate that with us not appreciating what you do.  It is a noble profession."  He noted that they sometimes have RT's who don't get things right and I cut him off and said, "But the department rally's and gets it right."  On his way out he said, "let me tell you something: Don't ever apologize for caring.  You did the right thing and you need to keep doing it.  I would do the same exact thing if I were in your shoes."  It was great that he let me know that.  I was in tears that he saw and understood why I was being such a pain.  I felt I could sit down and go to sleep...but now I have been typing for an hour or two.   

Julie has about a week.  Please don't cross that bridge yet.  I am on the edge of it, but I won't cross till we have to.  Keep praying, and hoping, and accepting what comes.  We are forever grateful for even the simple prayers.  God Speed.      

Sunday, February 21, 2016

It's been One week

Julie was discharged a week ago Thursday.  The trajectory we started on last stay continued (See He who has a WHY to live for can deal with almost any HOW. -Nietzsche post).  We were started on a regimen that Julie was leery of from the start.  She had asked to be on Tobramycin and Zosyn since it had worked during her ICU scare in November and at Christmas.  She had tolerated it well and her kidney function didn't seem to deteriorate till about day 10.  Her regimens lasted 14 and 10 days respectively due to the risk of  kidney damage.  Following the 3 week ICU scare in November, she was out of the hospital for 4 weeks.  After the 1 week Christmas lung infection she was out of the hospital for 5 weeks.  So when we went back to the ER (last post) at the end of Jan. we were on a good trajectory.  However we arrived to the hospital on a Friday.  If you can, go earlier in the week rather than later.  Nothing happens on the weekends.  Her regimen was started as Ceftazidime and Cipro.  Julie and I expressed concern that the Ceftaz would drop her WBC because her marrow was still fragile from the vicious chemo we had done the first half of 2015.  She knew she would be nauseous from the Cipro but agreed to try and push through it.  Her WBC did drop, and she was nauseous.  We found out that weekend that the foot pain and discomfort she was feeling was from another very high Prograf (anti-rejection med) level.  Anytime she is sick, the level goes crazy and insulin dependence skyrockets.  We were able to make an adjustment to Prograf, but with Ceftaz, the two drugs were affecting her WBC.  They had to drop her antibiotic on the following Monday.  She spent one day on only one antibiotic; Cipro and they dropped it for the nausea shortly after.  These lung infections she acquires, demand she be on two antibiotics at a time so they can attack the bacteria in multiple ways.  She started to lose the progress she had made in sputum reduction the first 4 days.  They added Zosyn on Wednesday to replace the Ceftaz.  Finally they added the Tobramycin with Zosyn; nearly a week after we arrived.  Toward the end of the first week, her platelets were dropping and she was asking for a platelet transfusion.  They continued to watch it and instead discontinued the Zosyn and replaced it with Meropenem.  It was a real shit show.

By the following Monday Feb. 8, Julie was looking forward to finally seeing Dr. Raman, one of her transplant docs who was coming on service.  On one hand, to defend what they had been doing, there are some  risks at hand with using the regimen she wanted, namely kidney damage.  The transplant team works closely with the attending pulmonologists, pharmacy, and the CF team to come up with a good plan for every stay.  They are in the background making the calls on what regimen to use.  Because there are so many possibilities with reactions, and disease state, I can see how there are so many directions they could go.  However, this time they should have taken the patients input more seriously.  We are not always right, in fact we aren't right a lot of the time, but the push back we give at least will substantiate their position or refute it.  We want to hear why they think they are making the right call and consider that they may not be making the right call when they speak to us.  We deserve that at the very least.  I told Julie that when she spoke to her physician she needed to let him know that she felt alone, and not listened to; that she needed him and Dr. Cahill in her corner.  She tends to suggest things in the form of questions to the attending physicians.  Often that is written off and just answered instead of addressed as a concern.  When she spoke with him he told her that he knew she wanted to be involved, but that she had to remember who had the MD's behind their names.  What I believe happened is he came in and saw all the myriad of changes that had happened and was upset.  In a frustrated moment he took it out on her.  It really upset her that the individual who is supposed to be her advocate was putting blame indirectly on her.  She told me she didn't want to see him in clinic.  After a few days I was able to help her see what he may have seen: a lot of things happened that he may not have agreed with.  We found out today that one of the attending physicians said they changed the meds because Julie thought they weren't working.  That wasn't at all the case.  She mentioned they were having different effects, but she was the lab rat, and they were practicing reactive medicine.

That final week of the stay Julie was really nervous.  She didn't feel like she was much better.  She was reluctant to go home.  She wanted to get a full 10-14 days of the current Tobramycin/Mero regimen.  They were pushing to discharge on the basis that she had 14 days of antibiotics, just different ones.  She came home with a plan to start an inhaled antibiotic Toby.  After a few days of doses she was feeling short of breath.  She had moved from 4 lpm of flow up to 8.  We drove up to Park City on Saturday to get out of the nations worst pollution that week.  She was able to drop her oxygen down to 4-5 lpm.  It was fascinating.  It may have been the clean air, and it may have been the fact that it was a tank and not a concentrator.  We don't know.  What we did know is that we had to get back up there for some R&R.  On Sunday I found a room at the Marriott PC and we left.  We spent the evening at the concierge lounge eating finger food.  Everyone was starin' hard in the room at Julie.  She was uncomfortable for a minute and then just rolled with it as she always does.  We took her back to the room and the boys and I went to the pool.

The next day we were able to lock up a room in a cottage at Hotel Park City.  We set up a massage for Julie, and headed over.  More swimming ensued, then gopro videos and treats.  The next day we had planned to come home.  We had breakfast and drove over to the magic carpet at Park City Mountain Resort.  I asked Julie if she wanted to stay another day.  She obliged and I called the hotel.  I sat on hold for a few minutes and they came back and said they had room for us to stay in our unit.  Julie was elated.  We set up another massage; this one now 80 minutes and headed back over.  I had been dying to ski with the boys so this was my opportunity.  She arranged to have some of her family come hang out when she was done.

As I arrived at Park City with the boys in tow and 45 degree early spring skiing conditions, I anticipated an epic half day with my kids on the mountain.  The snow was sticky at the bottom and I was hoping to expose them to the upper mountain for the first time.  I had Charles in tow in a baby bjorn as I always do.  He was whimpering as i was bending over to clip boots and bindings.  I don't know why, maybe it was time for a nap and the sudden rush of blood to his head?  As I started to ski down to the ticket office by the First Time lift he quieted down.  I had the boys wait on a bench next to the lift line.  At the ticket booth Charles was a little gremlin on my chest.  He had to get out.  He started to waddle around and found the closest puddle at the edge of the parking lot.  Why not?  That is what I would do if I were two.  I had the pack still on my chest and they sold me tickets for my boys and myself.  The ticket agent asked if the baby was skiing.  I replied, "No, he will be in the pack with me."  She asked if I'd like a "free" pass for him as well.  I accepted knowing what she really wanted was his name and age for her database.  I was fine with giving this to the marketing machine of Vail resorts.  I had no reason to think I wouldn't come back anyway.  After buying the pass and picking up a cute boy to place into the pack, I skate skied over to my boys only to find that cute boy had turned into a gremlin.  We put the passes in our pockets and entered the line.  A short line on a warm afternoon was in front of us.  The boys were entertained that there wasn't a gate to go through like at Alta but liftie with a hand scanner.  As we approached the chair a really important liftie that wasn't a day over 20 said "SIR I'M GOING TO HAVE TO STOP YOU RIGHT THERE AND ASK YOU TO STEP ASIDE!!  UNFORTUNATELY YOU CANNOT ENTER THE LIFT WITH THAT ON YOU."  "What?  The baby??.  "Yes it's against Vail policy"  I replied politely but firmly "You realize that every other resort in Utah wouldn't say a thing regarding this right?  They see him as a rider, but he is connected to me so they are okay with it....You realize that you just sold me a pass....FOR HIM?"  People started to get impatient with me in line.  "HOLD ON!!" I yelled in true Chris Farley fashion.  Shortly after I gave in because they simply weren't going to.  Even with my threat that I wouldn't ski there again (that may have been somewhat empty) they didn't budge.  But I was drawing straws at that point.  I couldn't believe I had been sold a pass, gone through the line, and got to the lift before someone questioned me.  How do they expect me to brainwash my kids into loving the snow if I can't do it when they are little munchkins?  What was adorable before Vail purchased PC, was now deplorable.  We spent the next few hours at the magic carpet teaching the boys to ride switch.  I expressed my frustration with just about anyone who would listen.  I was that guy...and I didn't care.  I was ticked.  They had ruined my plan to take the boys to a terrain park.  I found several locals that shared my disgust (or they were just humoring me), and one old timer liftie at the carpet mouthed "different owners."  I subsequently posted a boycott post about park city.  It took all of an hour for a hacker to get into my Instagram account and change my profile pic and name.  Weird right??? Not really.

That night we had dinner with Julies parents at the hotel restaurant.  It was great.  Julie was tired and short of breath, but she was so happy.  We went back to the room and showed grandma and grandpa the boys favorite gopro videos we had seen the night before.  When her parents were winding down to leave, Julie pressed them to stay.  These days family time is so important to her.  She soaks it up.  That night she was really starting to struggle.  She said to me in bed with tears in her eyes "I feel like Heavenly Father is letting me have this time up here with all of you..."  She knew something was brewing, and it was starting to scare her again.  We came back to Salt Lake on Wednesday morning.

We had a clinic appointment Wednesday and Julie spoke with Dr. Cahill about how she was doing.  She told her she was feeling tight and full.  Interestingly she didn't have a lot of movement and things sounded just tight.  Dr. Cahill explained that she really was a unicorn, 'short of an autopsy and a scalpel, we are limited in how much data we can get from a stethoscope, x-ray etc.'  She told her that we didn't know if it was residual inflammation from the last exacerbation, a superbug brewing inside of her or something else.  She really has a way to put Julie at ease even when there aren't clear answers.  Interestingly, we saw Dr. Raman beforehand in the foyer and he was very gracious.  I think in some way he realized he needed to let her know he was concerned about her.  We spoke briefly about her care and the plan.  We had planned to start inhaled Cayston, an antibiotic, that next day to keep her out of the hospital.  That night she really struggled.  She had a hard time breathing and woke up knowing she needed to get in...this time before the weekend.

Upon arriving Thursday, they moved her up to the floor.  They knew they needed to admit her because she had some pneumonia on the left side of her lungs.  The caveat to the last hospital stay is that since it was so recent, (one week prior) and she was exposed to many of the drugs that are effective on her current bacteria, they are leary of giving her any IV antibiotics because of bacteria resistance to them.  She didn't get a full 14 day regimen of any one regimen, but they were all used recently.  After much team deliberation behind the scenes as to what they were going to do, they told her that they weren't doing any IV antibiotics and they planned to still do the Cayston inhaled antibiotic on Friday.  She was really scared.  It didn't arrive till 4:00 pm on Thursday so I had her dad Rick run it up in the morning.  No one was there to drive the issue and they didn't actually give her her first dose till Saturday midday; this was 48 hours after we came to the hospital.  They waited on Friday because they needed a bottle sterilizer for her nebulizer since it isn't hospital property and can't be sterilized on site without risk to the device and risk of losing the specific neb cup.  Ironically there are two neb cups and they could have started treatments with yesterday.  This drives home the point that it is vital that patients have an advocate or a family member to move things along.  It is not to be a pain, but to ensure that the care happens as efficiently as possible.  If someone isn't here, it is our experience that things don't happen as quickly.  Don't leave your family members at the hospital alone when they are acutely ill.  It's not worth it.  Sometimes it is inevitable you can't be there; I know more than anyone.  Find a way to have someone there; someone familiar with the patient's care.  Julie's mom was here all day giving her love and comfort, but sometimes the biggest challenge is knowing what to question; the team said they were waiting on another part and it was just the sanitizer that could have been used Saturday morning.  Subsequently she has gotten much worse.  Her CO2 level yesterday was 69; higher than it ever was in the ICU in November.  She has been bringing up buckets of sputum, and it is dark green.  She was loopy when I spoke to her last night indicative of high CO2.  She is very sick.

Definition of torture; coming down with a cold when your immune suppressed wife is in the hospital with a lung infection.  Since I left Thursday afternoon, I came down with a cold and I wasn't able to be here till Saturday afternoon.  Last night when Julies girlfriends left they were both really scared for her.  She wanted me to come up but knew it wasn't a good idea.  I didn't sleep more than about 5 hours last night longing to be up here.  Her friends couldn't stay and I was seriously considering just going up with a mask on in the middle of the night.  Luckily she was able to get the bipap set to where she could sleep because she was able to be ventilated.  She spent the day today looking better, getting a little more sleep, but still bringing up a lot of gunk.  She believes it is a sign she is producing more.  We hope that the three treatments of Cayston today are helping her break it up.  It's probably a combination of the two.  When she is not of bipap she is on 10 lpm of flow of oxygen.  She wanted to try a higher flow with the Optiflow high flow nasal cannula so that will be what we try during the day tomorrow.  This should help wash out CO2 and keep the sputum motile because of the high physiological humidity the MR850 heater puts out.  

Tonight Julie told me she thinks she is going to die if they don't get her on an IV antibiotic regimen.  I feel completely powerless.  All I can do is share her sentiment with the nurse on staff.  They are acutely aware of the situation and my hope is that we can have a very serious conversation about how things are going tomorrow when her docs round on here.  I am finally here next to my wife.  I know I can sleep tonight with Julie next to me.  My hope is she can do the same.  I can't control the facts here.  I can't control the situation.  But I can be here to weigh in on what is happening.

Tonight I asked my in-laws if there was anything we would have done differently when we lost their son James 9 years ago.  Could he have survived any longer?  Could he have gotten better?  He certainly was a different case but I wanted to talk about it to see if we had any regrets.  My fear is that because Julie has faced her suffering with the utmost dignity and understanding, we will give up on her before she does herself; that she will be in a situation where she is not able to communicate effectively and we think she is ready to go when she is not.  Linda assured me that James was ready when he died.  He had seen his son's for the last time, was with his sweetheart, and it was clear that he was ready.  He had such high CO2 that he was in a delirium and he was hopped up on morphine to make him comfortable.  That was such a raw, but spiritual experience that will forever have an impact on me.  I will never forget the feeling in the room after we watched him pass.  It was like he was still there for a few hours.  I will never forget the feeling that I might be witnessing my own future as I watched his wife Michelle.  I will never forget that I was completely at peace with that too.  I can't explain why it felt like everything would be alright.  I don't want to speak for her, but there was a feeling that I would one day understand what she was going through.  She knew it, and I knew it.  I was about to get married just 3 weeks later; to embark on a journey that would forever teach me more than I could have imagined;  to set me on a trajectory to mold me into the man I was born to be.  The suffering I have witnessed has brought me to my knees countless times.  It has pushed me to be a little better.  I have been thrust into bold declarations of faith that at times seemed impossible that we have later realized.  This is not my doing, but is a product of how someone close to you suffers can impact how you see the world and that suffering that is so inevitable in it.  Everything will be alright.  It always is.  No matter how bad it gets.  It can always get better.  "Life is to be enjoyed, not just endured."  Stand True and Faithful - Gordon Hinckley I have so much love for Julie.  Of course I want her to pull out of this and get better; any husband would.  I have so much respect for how she quietly but boldly faces these challenges with dignity; she suffers nobly, without self pity.  It is this dignity that allows me to hope for the unthinkable time and again.  Knowing that she is comfortable accepting whatever may come allows me to shoot for the stars.  She does not accept the negative outcome before it comes; she acknowledges its reality, talks about it, and then talks about the more positive possibility.  We feed off each other.

I know how unrelenting this has been.  I am living it with Julie.  I don't know what it's like to be the one struggling.  What I do know is that suffering is much more prevalent around us than we realize.  That this is one of many examples in your personal life of suffering around you.  Pay attention, ask questions.  There is no reason people who are more introverted should suffer alone.  What I have learned from all this is we should never accept an outcome before it has come.  It is a waste of energy.  I am not advocating for blind faith.  I am not advocating for delirium or delusions of reality.  I am advocating for living.  God Speed.  Pray for Jules.  It's been working.  Thank you forever for your love and support.