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.