My aunt was my best friend, my mentor, my non-romantic soul mate. She was diagnosed with a horrific and incurable disease that very few people even know exists (Alpha-1 Antitrypsin Deficiency) in 2003. She suffered through weekly IV infusions made with human plasma, surgeries, numerous medications, and being put on oxygen. She wouldn’t quit smoking, though, so she wasn’t a candidate for a lung transplant. Sometimes I wonder if she even wanted to live, knowing that was her only chance and she didn’t take it. We knew her time was limited, and her life in the last year or so was miserable - more or less confined to her bedroom. I spent a lot of time with her, aware that she was dying and that I needed to be with her as often as I could while she was alive. She was taken to the ER for a stomach virus that made it hard for her to breathe. The night my grandma called and told me to come because she was dying, no one had told her that this was it. I cried and cried, and she tried to comfort me. I sat there with her, watching How The Grinch Stole Christmas and sharing her banana popsicle, enjoying her last bits of lucidity as she fought the morphine being pumped into her. Saying goodbye to her that night was awful. I was sure she would die before morning, but she was adamant that she was “going home - to a residence.” She didn’t want to die. They didn’t give her a choice. She was transferred to the hospice wing by the morning, and I spent the day with her after I got out of school. She had made me promise to do well on my finals that day, to try not to worry about her. Our last conversation was unexpected. She woke up as I was about to head home, after watching her sleep for hours and wondering if she’d ever open her eyes. She asked how I did on my finals. “Aced them,” I told her. She was proud. No one was ever proud of me besides her. I should have stayed with her, since she was awake. I should have stayed even though it was uncomfortable and awkward to engage in small-talk when I wanted to be telling her how much she meant to me and how I would miss her. When I told her I had to get home, I hugged her. Those were her last words to me as I shut the door and cried my way down the hall and through the night. She never saw me again. When I came back the next day, they had her on a steady drip of morphine “to keep her comfortable.” To keep her from bitching and fighting to stay alive, more like it. The day after that, I was alone in her room with her unconscious body. I held her hand and told her that I would be okay, that she could go and be with my dad and hers, that she was my best friend and I loved her so much, but that it was okay for her to leave. Her death rattle started as soon as the nurse came in and interrupted my “goodbyes,” and she died holding my hand. She was 39, and I was 17. I’m 24, now. And I still can’t think about her without crying. I miss her so much. I love you, Aunt Lori. Sleep well.
Put on my big girl pants and drove the dog to my dad’s and then drove myself to Indy. Got to see my husband for a whole 30 minutes or so 😔 before I had to leave because I wanted to be able to find my hotel before it got to be too late but I still didn’t get there before it got pitch dark. I guess I have recently knocked that fear out of driving to and from by myself. I still get super overwhelmed by all the traffic though. I’m learning though.
The liver dr that checked on him today was one of the heads to the transplant team, they said they would call interventional radiology and hopefully get what I like to call his “liver bypass” readjusted and just pray that that narrowing is what caused all this and will fix it. This is so scary but at least I know and really feel in my heart he is being well taken care of and I am glad he is there now so I don’t have to worry about him.
In the morning we go to the liver specialist again so they can check and see how my husband’s liver is holding up. It will be a really long day so if you have some thoughts, prayers, or positive vibes to share I would appreciate it.
Whenever I have a day where everything just feels like too much, the best thing just seems to be to shut the door and cry it all out, maybe write something about it… but mostly just cry it all out and then get back on with life. Big ugly cries where you feel the weight come off your back and chest. It always amuses me to be in the midst of a dramatic cry and be thinking “this feels so good to get out”… it’s like dumping poison out of your body.
Ki weathers his storm and his very life is at stake. He rarely complains. So what do I have to complain about? It’s sad that it is this way but I find the support places for people in a situation such as mine to be terribly negative places. The people who are sick are doing their damndest to live their lives and their loved ones are bitching and moaning about everything under the sun.
Yes this is hard. The constant worry takes its toll at times. But I take my wedding vows very seriously and I didn’t just say for better, for richer, and in health. It also isn’t just about some vows, it’s about loving somebody so much that you will do everything you can for them.
Going through this, I feel like I know an aspect of love I didn’t know before. You think you know all these different types of love and then something happens in life that exposes you to something deeper. I don’t know how to describe it, it just encompasses everything. It has changed for him too, he tells me that the only thing he can describe it as is he feels like he loves me unconditionally.
But yet here it is, something so challenging has strengthened us.
I have moments of fear but there is this trust growing inside of me of the eventual outcome of this bringing us to something greater than we ever imagined. It has pushed me back towards a path I blindly wandered off of long ago and it has lifted a veil from his eyes. I think we’re both here to better each other’s lives and this is really burning away all the things that aren’t important.
Tonight I’m tripping on the fact that when I left the hospital tonight I didn’t hug him for as long or as tight as I wanted to. I don’t know I was overwhelmed in my brain just didn’t think to hug tighter and longer. I left thinking everything was going to be okay and I still do but there’s always that feeling of what if…
I think that’s the thing with chronic illnesses you’re always reminded of what if this was the last time we did this or what if this was the last time we did that… And then there is always that fear of losing somebody. I think sometimes that’s the hardest part because you’re aware of your grief so often. You’re constantly grieving things that aren’t even tho person is still with you.
I want to drive over to the hospital at 3:30 AM just a hug him and then go back to my hotel.
They are supposed to do the readjustment to the device inside of his liver in the morning at some point. This time it doesn’t feel as scary as when they initially put it in but I think that’s partly because we finally realize how important it is to his overall health. Like before we felt like maybe it was almost experimental but now we know it makes his huge possibly life saving difference so that getting it back into working order is critical.
The day started out good and then went downhill. My husband is in the hospital right now, after a really good specialist appointment too. He did blood work after the appointment and then after trekking around all day, getting stuck in a massive traffic jam and then finally on our way home the university medical center calls and tells him that his blood sodium is dangerously low likely due to his medication needing tweaking and he needs to go to the er when he gets home to get iv fluids and more blood work.
So he did that and they need to keep him overnight bc they have to slowly raise the levels.
Hate this stupid fucking disease, so many ups and downs. This time both in the same day.
I knew something was coming tho, the past couple of weeks I have been carrying around that awful feeling of something not right. Every single time I have that feeling he ends up in the ER for something. I swear I can feel it in mine when something goes “off” with his body.
So Friday there was another appointment with the liver specialist. My mother-in-law came down from WI to go with us, she stayed the week with us.
I figured it would be a very long day because he was scheduled for blood work, then a CT scan, and then the doctor’s appointment but it surprisingly was all very quick in terms of them being on time and getting the stuff done… just boom, boom, boom.
So he had the blood work done, then the CT, and then we met with the doctor. I really do like his doctor a lot. He seems like he is always quite happy and positive, he always speaks with a very pleasant tone, he is organized and seems like he would know his stuff even in his sleep. By the time we met with him about 2.5 hrs after we arrived there all the reports were back on the CT scan and blood work. Wowza!
The CT scan is standard every 6 months to check for cancer. It’s a very scary prospect but people with alpha 1 are at a higher risk as are people with liver damage so they check every 6 months because if they catch it they can transplant the liver before it spreads. There was no mention of them seeing anything on it so I assume that is all good… this was what was causing me the most anxiety. They also didn’t detect any fluid in the abdominal or pleural cavities which is awesome.
The doctor seemed shocked in a very happy way that his blood work concerning all the liver stuff is staying extremely stable, in fact some of the numbers are exactly what they were when we were there in the beginning of January. He kept chuckling and going ahh that is amazing, this is very good news! The doctor said that it is possible that this could go on for quite some time.
The only downside is his kidneys are not happy with some of the meds. Because those levels are off it shot up his meld score a few points (the meld score is used to determine how sick the liver is). They start transplant proceedings at 15+. His is currently at 13 but the doctor calculated what it would be if the kidney levels were normal and it would be an 11, which is what it has been (fwiw, a normal healthy individual without liver damage is 10 and under). I asked if the kidney levels were restored to normal if the meld would go back down and the doctor said most likely. So the doctor decided to tweak some meds and have him redo blood work in 1 week and then they can recalculate the amount of meds if they need to or if he has any issues he can call them and let the doctor know. Basically they’ve just got the get these meds at a level where the kidneys are happy.
The only thing that sort of irked me is that, the last time we were there, the doctor ordered another Alpha 1 test. Now we already knew with 100% certainty that my husband has Alpha 1 Antitrypsin Deficiency and he has the ZZ phenotype, so I was kind of baffled why they ordered the test again but just figured they wanted to confirm it for their own records. This seemed to be the case but during this appointment the doctor told us that he had been doubtful of the original diagnosis. I dunno why but it irked me. I wanted to ask why he thought that but I didn’t which I kind of regret now because I just wanted to know why he doubted it, yah know? All I can figure is they so rarely see it and it is even rarer to see a liver affected Alpha, especially one in his age range (liver affected alphas are usually babies or young children and older men in the 45-55 age range).
It was a pretty short visit with mostly good news and if everything goes well we don’t have to make another trip until June 13th.
My husband has done an amazing job at maintaining a healthy lifestyle and really changing everything he does around. I know that it can’t be easy and I am so proud of him. I know that the work he is putting in is what has kept his liver stable. Not to mention he’s also been doing a lot of mental preparation for when the day arrives that he needs a transplant.
Tonight, for like the quickest split second ever, I had a moment of complete peace wash over me where I felt like it was okay, he will get to the transplant point and there will be an organ for him and everything will okay. Of course as soon as I wanted to cling to the feeling it was gone, but at least for a moment, it was there. I want to find that space again and always be there.