Tuesday, August 25, 2015

Normalcy is my priority

The gang finally started school this week. Well, if you count staggered starts and half days. They're officially all in on Thursday. We survived summer. Praise the Lord. Thanks be to God. And Amen.

Its been an exhausting week. This is the first time we've had to wake up early for treatments on any regular schedule. For preschool, Drew went in the afternoon which gave us time to wake up, eat a nice big breakfast and get our treatments done. Now, we are waking up at 6am everyday, barely getting in an Ensure Plus and a slice of bacon, and have cranky, tired kids by 6pm. My kids are 12hr a night sleepers, so for this to be sustainable we will need to start skipping dinner and going straight to bed after homework. CF be damned.

Last time I blogged, I had mentioned a CT Scan and MRI that Drew was having. Two things came out of that. The first was the idea to make IRB protocols more easily understood by kids, and that's actually happening. I got in contact with Flip the Clinic and we are doing this. More to come on that later, but i'll just say that i'm SO FREAKING EXCITED!

The other thing that came out of the CT scan was shitty looking lungs. Boo. They weren't shitty shitty, but they weren't as clear and healthy looking as they have been in the past. My initial thoughts went to that bastard achromobacter that's invaded Drew's lungs and is nearly completely antibiotic resistant. I set up time to talk with his doc about the results, and while I waited for our meeting I searched online journals, I googled everything I could possibly think of related to achromobacter and eradication, and I turned to my peers. Oh, my beloved peers. The people that I've never actually met that I trust more than some people I've known my whole live. They're the ones in the trenches with me, desperate for answers, sharing what's given them hope. They track their treatments, get into deep and thoughtful conversations about options, sharing the recommendations of their doctors and what's worked and hasn't worked for them. I took all of this learning into a conversation that my husband and I had with Drew's doctor two weeks ago.

She started by saying it wasn't that bad. There was some mild lower lobe bronchiectasis (irreversible lung damage) but that it was oddly in the lower lobes of his lungs, whereas the typical progressing of lung disease that they see in CF starts with the upper lobes. She said that the lower lobe damage looks to be more consistent with something that you might see with aspiration. Hmmmmm. Queue my mind back to our situation this spring with the weird cough that wouldn't quit that turned out to be a reflux issue. Could that have been so bad that it caused lung damage? We won't ever have a definitive answer on that, but it sure does make you wonder. And as a sidebar, it makes me crazy mad (not at anyone in particular, just at the fact) that in 2015 we don't yet have a way to look at patients by ages or genotypes or medications they're taking to identify success rates or complimentary treatment options or symptom checkers that others might have experienced that could help us to quickly identify or eliminate what might be going on. It's on my list of things to do.

Anyway, because we don't know what caused the damage and while its possible that it was from reflux, its also possible that the achromobacter treatment plan that we are currently on isn't working as well as we had hoped. There were a couple of options that we had discussed. One was trying a new combination of inhaled antibiotics. Right now Drew inhales Tobi and Ceftazadime on 14 day cycles. His bacteria is resistant to Tobi but we didn't want to put him on continuous ceftaz due to growing resistance risks. An achromobacter eradication protocol called for continuous inhaled Ceftaz with a strong oral antibiotic continuously for something like 6 months. There are risks associated with that as well; resistance to a whole new class of antibiotics, GI issues associated with an oral antibiotic, and of course the chance that it wouldn't help. Another option, and something far more novel that I had suggested, was the use of Cysteamine (if you want to be nerdy, read this - http://www.sciencedirect.com/science/article/pii/S2352396415301109). It seems to have an incredible capacity to thin mucus, disrupt biofilms and therefore allow antibiotics that were once powerless against these horrible bacteria to once again penetrate them and get rid of resistant bacteria. There hasn't been sufficient testing in people with CF yet, but theres a whole group of folks that I've met who have both use and had success treating their resistant bacteria with this medication. Drew's doctor is trying to learn more about it for us so that we can see if it could be a useful option to try for him. If I'm trusting my mom instinct and backing that up with all of the research I've done on it, I think its probably the best option that we've got at getting rid of this bacteria.

What we settled on was a bronchoscopy. We have been treating the bacteria that Drew grows when they take a culture to see what he has in his lungs. The way they do the culture is sort of like a strep test where they jam the giant q-tip looking apparatus down your through to swab it for bacteria. While this is usually a "good enough" sample, sometimes its useful to get a deeper culture from mucus way down in his lungs. Since he's not yet able to cough up sputum and spit it into a cup, the only way they can get that sample is through a bronchoscopy. The reason that we want to do this is to make sure that we are treating the right bacteria and not missing something else living in his lungs that may be causing damage because of our failure to identify and treat it.

So on Monday, the first day of school, as we were walking out of the building and I was hearing all about who sat with who at lunch and how I packed snacks that they didn't like (you're lucky you even got a snack kids, don't push it!), my phone rang and the number was Cincinnati Children's. It was someone from scheduling calling to get him set up for a bronchoscopy. I told them I was busy and would have to call back.  I know how these calls go - 15 minutes on the phone with a usually very kind woman from customer service who asks me 100 questions that I'm quite frankly shocked that she can't easily get the answers to through our medical record, and then tells me the date that they can do it. I was already not really paying attention to the woman because a ringing phone means nothing to 5yr olds so they carried on with their chatter about their day, and I simply told her I would call back to take care of this later. CF interrupts so many other parts of my life, and I wanted to hear about the first [fake, only half] day of kindergarten, uninterrupted. I didn't want to page back and forth between my calendar and my phone call, quickly and distracted, likely to give her a date that wasn't going to work anyway leading to the need for a follow up and repeat of the first phone call/questionnaire. I want to sit down and think about this, find a time that works well for us. Maybe fall break, or another day off school. Missing a day of school right now will just kill him. He's just getting comfortable going, getting to know his routine, meeting new friends to eat that snack he doesn't like with. Missing a day off to relax might kill him too. I have to weigh these things against the importance of having this done, of knowing whats next on this journey.

I think this is the thing that most infuriates me about the current system for chronic illness care. While i'm trying to make these kinds of decisions, decisions that I have to make in 1000 different ways on nearly every single day, medical professionals are trying to help us understand the importance of things like sleep, exercise and taking your medication on time. I get that that's important, but even for those of us who manage to keep our heads above water, normalcy, above all else and in whatever distorted, backwards, chronic-illness-mom-anything-but-actual-normal way we define it, normalcy is our priority. I wish we could find a way to focus on that.


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